Volume 72, Suppl 2, 2014, São Paulo, SP, Brazil
THE OFFICIAL JOURNAL OF THE ACADEMIA BRASILEIRA DE NEUROLOGIA
Vision:
Arquivos de Neuro-Psiquiatria will be the main Latin America peer-reviewed journal in neurosciences.
Mission:
Arquivos de Neuro-Psiquiatria will provide clinical neurologists and other neurocientists with sigfnificant peer-reviewed articles,
editorials, reviews and clinical reports to improve neurological practice, education, clinical research, professional improvement and
brazilian neurology international visibility.
Editors
José Antonio Livramento, SP
Luís dos Ramos Machado, SP
Special Editors
Acary Souza Bulle Oliveira, SP
Alberto Alain Gabbai, SP
Carlos Alberto Mantovani Guerreiro, Campinas SP
Paulo Caramelli, Belo Horizonte MG
Founding Editor
Oswaldo Lange
Emeritus Editor
Antonio Spina-França Netto
Arquivos de Neuro-Psiquiatria Dr. Oswaldo Lange Association
Luís dos Ramos Machado, SP, President
José Antonio Livramento, SP, Vice-President
Carlos Alberto Mantovani Guerreiro, SP, Secretary
Acary Souza Bulle Oliveira, SP, Treasure
Alberto Alain Gabbai, SP
Carlos Augusto Takeuchi, SP
José Píndaro Pereira Plese, SP
Luiz Alberto Bacheschi, SP
Maria Fernanda Mendes, SP
Paulo Caramelli, MG
Osvaldo M. Takayanagui, SP
Executive Office
Adriana Spina França Machado, SP, manager
Rua Vergueiro 1421 cj 804 – Torre Sul
04101-000 – São Paulo – SP – Brasil
Fone (5511)3884-2042; Fax (5511)2369-9721
e-mail: [email protected]
Apoio:
Publishers
Caboverde Tecnologia e Serviços Ltda.
Marketing
EPM - Editora de Projetos Médicos
phone/fax: (5511)5084-3576 / (5511)5575-3450
[email protected]
Academia Brasileira de Neurologia
Elza Dias Tosta, DF, President
Rubens José Gagliardi, SP, Vice-President
Gilmar Fernandes do Prado, SP, Secretary
Mônica Santoro Haddad, SP, Treasurer
Francisco Eduardo Costa Cardoso, MG, Delegate to WFN
www.abneuro.org [email protected]
Cataloguing
ISSN 0004-282X - paper publication
ISSN 1678-4227 - online publication
NLL (National Lending Library of Sciences and Technology, Boston, UK, 1947)
WHO/UNESCO (Word Medical Periodicals, 1949)
EMBASE - Excerpta Medica (Elsevier, Amsterdam, 1960)
MEDLINE (National Library of Medicine, Bethesda USA, 1966)
ISI (Institute for Scientific Information, Philadelphia USA, 1969)
JCR, 2003
LILACS (BIREME, São Paulo, 1982)
SCOPUS (Elsevier, 1998)
SIMAGO (Elsevier, 1998)
SciELO (1999)
QUALIS/CAPES (B2, 2009)
DOAJ
LATINDEX
Circulation
Monthly, 3000 copies
INTERNATIONAL ADVISORY BOARD
Andrew J Lees, London (UK)
Arturo Carpio R., Cuenca (Ecuador)
Christina Marra, Seattle (USA)
Gérard Saïd, Paris (France)
James F. Toole, Wiston-Salem (USA)
Jun Kimura, Kyoto (Japan)
Maria José Sá, Porto (Portugal)
Pedro L. Ponce, Caracas (Venezuela)
Roberto E. P. Sica, Buenos Aires (Argentina)
Vladimir Hachinski, London (Canada)
Editorial Advisory Board
Amauri Batista da Silva, Brasília DF
Amilton Antunes Barreira, Ribeirão Preto SP (USP-RP)
Aroldo Luiz da Silva Bacellar, Salvador BA
Ehrenfried Othmar Wittig, Curitiba PR
Elza Dias Tosta da Silva, Brasília DF
Fernando Cendes, Campinas SP (UNICAMP)
Fernando Coroneti, Botucatu SP (UNESP)
Fernando Otávio Quaresma Cavalcante, Belém PA
Gilberto Belisario Campos, Belo Horizonte MG
Gilson Edmar Gonçalves e Silva, Recife PE (UFPE)
Joaquim Pereira Brasil Neto, Brasília DF (UnB)
Marco Aurélio Lana-Peixoto, Belo Horizonte MG (UFMG)
Marcos Raimundo Gomes de Freitas, Niterói RJ (UFF)
Milberto Scaff, São Paulo SP
Newra Tellechea Rotta, Porto Alegre RS
Paulo Norberto Discher de Sá, Florianópolis SC
Ricardo Nitrini, São Paulo SP (FMUSP)
Rubens José Gagliardi, São Paulo SO (FCM-Sta Casa SP)
Sérgio Roberto Haussen, Porto Alegre RS
Vicente de Paulo Leitão de Carvalho, Fortaleza CE
ASSOCIATE EDITORS
Cerebrovascular disorders
Adriana Bastos Conforto, São Paulo SP (FMUSP)
Ayrton Roberto Massaro, São Paulo SP (Hospital Sírio Libanês and Hospital
Albert Einstein)
Fábio Iuji Yamamoto, São Paulo SP (FMUSP)
Jefferson Gomes Fernandes, São Paulo SP (Hospital Alemão Oswaldo Cruz)
Rubens José Gagliardi, São Paulo (Santa Casa - SP)
Clinical neurophysiology
Carlos Otto Heise, São Paulo SP (FMUSP)
Francisco José Carchedi Luccas, São Paulo SP (Hospital São Luiz)
João Aris Kouyoumdjian, São José do Rio Preto SP (FAMERP)
Lineu Correa da Fonseca, Campinas SP (PUC)
Clinical practice
Alberto Allain Gabbai, São Paulo SP (UNIFESP)
Eduardo Genaro Mutarelli, São Paulo SP (FMUSP)
Cognitive disorders
Jerson Laks, Rio de Janeiro RJ (UFRJ)
Jerusa Smid, São Paulo SP (FMUSP)
Paulo Caramelli, Belo Horizonte MG (UFMG)
Epilepsy
Carlos Alberto Mantovani Guerreiro, Campinas SP (UNICAMP)
Eliana Garzon, São Paulo SP (FMUSP)
Luiz Ataíde Jr, Recife PE (FMUFP)
Experimental neurology
Fulvio Alexandre Scorza, São Paulo SP (UNIFESP)
Roberta Monterazzo Cysneiros, São Paulo SP (Univ. Mackenzie)
Headache and pain
Getúlio Daré Rabello, São Paulo SP (Hospital Samaritano – SP)
José Geraldo Speciali, Ribeirão Preto SP (FMUSP – RP)
Pedro André Kowacs, Curitiba PR (UFPR)
Renan Barros Domingues, Lille, France (Université Lille)
History of neurology
Hélio Afonso Ghizoni Teive, Curitiba PR (UFPR)
Péricles Maranhão-Filho, Rio de Janeiro RJ (UFRJ)
Rubens Reimão, São Paulo SP (FMUSP)
Tarso Adoni, São Paulo SP (FMUSP)
Movement disorders
Carlos Rieder, Porto Alegre RS (UFCSPA)
Hélio Afonso Ghizoni Teive, Curitiba PR (UFPR)
Henrique Ballalai Ferraz, São Paulo SP (UNIFESP)
João Carlos Papaterra Limongi, São Paulo SP (FMUSP)
Myopathy
Anamarli Nucci, Campinas SP (UNICAMP)
Lineu César Werneck, Curitiba PR (UFPR)
Suely Kazue Nagahashi Marie, São Paulo SP (FMUSP)
Neurogenetics
Fernando Kok, São Paulo SP (FMUSP)
Iscia Lopes Cendes, Campinas SP (UNICAMP)
Laura Bannach Jardim, Porto Alegre RS (UFRGS)
Neuroimaging
Antonio José da Rocha, São Paulo SP (Santa Casa – SP)
Claudia da Costa Leite, São Paulo SP (FMUSP)
Emerson Leandro Gasparetto, Rio de Janeiro RJ (UFRJ)
Leandro Tavares Lucato, São Paulo SP (FMUSP)
Luiz Antonio Pezzi Portela, São Paulo SP (Hospital Alemão Oswaldo Cruz)
Neuroimmunology
Douglas Kazutoshi Sato, Sendai, Japan (Tohoku University)
Maria Fernanda Mendes, São Paulo SP (Santa Casa – SP)
Maria Lucia Brito Ferreira, Recife PE (HR – SSEPE)
Yara Dadalti Fragoso, Santos SP (UNIMES)
Neuro-oncology
Suzana Maria Fleury Malheiros, São Paulo SP (UNIFESP)
Suely Kazue Nagahashi Marie, São Paulo SP (FMUSP)
Neuropathology
Carmen Lúcia Lancellotti, São Paulo SP (Santa Casa – SP)
Leila Chimelli, Rio de Janeiro RJ (UFRJ)
Sérgio Rosemberg, São Paulo SP (FMUSP)
Neuropsychology
Eliane Miotto, São Paulo SP (FMUSP)
Infectious diseases of the nervous system
Abelardo Queiroz Campos Araújo, Rio de Janeiro RJ (FIOCRUZ)
Osvaldo Massaiti Takayanagui, Ribeirão Preto SP (FMUSP – RP)
Paulo Pereira Christo, Belo Horizonte MG (Santa Casa BH – MG)
Ronald Salamano, Montevideo (Uruguai)
Ronaldo Abraham, Taubaté SP (UNITAU)
Neurosurgery
Fernando Gomes Pinto, São Paulo SP (FMUSP)
Guilherme Lepski, São Paulo SP (FMUSP)
Hamilton Matushita, São Paulo SP (FMUSP)
José Píndaro Pereira Plese, São Paulo SP (FMUSP)
Mario Augusto Taricco, São Paulo SP (FMUSP)
Roberto Sérgio Martins, São Paulo SP (FMUSP)
Interventionist neuroradiology
Paulo Puglia Jr, São Paulo SP (FMUSP)
Otoneurology
Cristiana Borges Pereira, São Paulo SP (FMUSP)
Pediatric neurology
José Luiz Dias Gherpelli, São Paulo SP (FMUSP)
Marilisa Mantovani Guerreiro, Campinas SP (UNICAMP)
Umbertina Conti Reed, São Paulo SP (FMUSP)
Peripheral neuropathy
Osvaldo J. M. Nascimento, Rio de Janeiro RJ (UFF)
Wilson Marques Jr, Ribeirão Preto SP (FMUSP – RP)
Psychiatry
Antonio Egídio Nardi, Rio de Janeiro RJ (UFRJ)
Cássio Bottino, São Paulo SP (FMUSP)
Quirino Cordeiro, São Paulo SP (FMUSP)
Physiotherapy / speech therapy
Márcia Radanovic, São Paulo SP (FMUSP)
Rehabilitation in neurology
Marta Imamura, São Paulo SP (FMUSP)
Marco Orsini, Rio de Janeiro RJ (UFF)
Sleep disorders
Rosana Cardoso Alves, São Paulo SP (FMUSP)
Stella Tavares, São Paulo SP (FMUSP)
Tricks of the trade
Laura Moriyama, London UK (UCL Institute of Neurology)
EDITORIAL AREA SUPERVISORS
Cerebrovascular disorders
Charles André, Rio de Janeiro RJ (UFRJ)
Jorge El Kadum Noujaim, Rio de Janeiro RJ (HFSE – RJ)
Neurological critical care
José Manuel Rocha Fernandes, São Paulo SP (FMUSP)
Clinical practice
Sérgio Augusto Pereira Novis, Rio de Janeiro RJ (Santa Casa – RJ)
Sebastião Eurico de Mello e Souza, Goiânia GO (Instituto de Neurologia)
Neuropsychology
Alessandra Gotuzo Seabra, São Paulo SP (Univ. Mackenzie)
Experimental neurology
Antonio Carlos Guimarães de Almeida, São João Del-Rei MG (UFSJ)
Monica Levy Andersen, São Paulo SP (UNIFESP)
Neuroregeneration
Guilherme Lepski, São Paulo SP (FMUSP)
Headache and pain
Silvia R. D. Tesseroli de Siqueira, São Paulo SP (FMUSP)
Pediatric neurology
Alexandra Pruffer Queiroz Campos Araújo, Rio de Janeiro RJ (UFRJ)
Motor neuron disease
Dagoberto Callegaro, São Paulo SP (FMUSP)
Physiotherapy / speech therapy
Letícia Lessa Mansur, São Paulo SP (FMUSP)
Lúcia Iracema Zanotto Mendonça, São Paulo SP (FMUSP)
Movement disorders
Egberto Reis Barbosa, São Paulo SP (FMUSP)
Francisco Cardoso, Belo Horizonte MG (UFMG)
José Luiz Pedroso, São Paulo SP (UNIFESP)
Neuroethics
Luiz Alberto Bacheschi, São Paulo SP (FMUSP)
Neuroimmunology
Angelina Maria Martins Lino, São Paulo SP (FMUSP)
Maria Lúcia Vellutini Pimentel, Rio de Janeiro RJ (Santa Casa – RJ)
Maria José Sá, Porto (Portugal)
Psychiatry
Alexandre Shoji, São Paulo SP (FMUSP)
Rafael Bernardon Ribeiro, São Paulo SP (FMUSP)
Rehabilitation in neurology
Paulo Nicolau Borsoi Salum, São Paulo SP (FMUSP)
Sérgio Lianza, São Paulo SP (Faculdade de Ciências Médicas da Santa
Casa – SP)
Arquivos de Neuro-Psiquiatria is a periodic registered in the Departamento de Imprensa e Propaganda (11795), Departamento Nacional
de Propriedade Industrial (97414) and 1º Ofício de Títulos e Documentos de São Paulo, Brasil. Its owner and publisher is the Arquivos de
Neuro-Psiquiatria Dr. Oswaldo Lange Association, no provide society registered in the 6º Ofício de Registro de Pessoas Jurídicas de São
Paulo (12770), in the Ministério da Fazenda (CNPJ 57394579/0001-31) and in the Secretaria de Finanças do Município de São Paulo (CCM
9416334-0). It is the Official Journal of the Academia Brasileira de Neurologia / Brazilian Academy of Neurology since 1970. It is published
monthly since 2012, with absolute regularity over these 70 years since its foundation in 1943.
Permission to photocopy articles: This publication is protected by copyright. Permission to reproduce copies of articles for noncommercial
use may be obtained from the Copyright Clearing Center: www.copyright.com.
Annual subscription rates for 2015: Brazil – R$ 380,00; other countries $180.00. Subscription prices outside Brazil must be prepaid. Prices
subjected to change without notice. Information: [email protected]; phone (5511) 3884-2042; fax (5511) 2369-9721.
Manuscripts submission: www.scielo.br/anp
The announcer is entirely responsible for the advertisements included in this issue. The authors are fully responsible for the opinions and
concepts expressed in the manuscripts published in this issue, which do not necessarily reflect the opinion of the editors.
DIRETORIA ABN
Presidente: Elza Dias Tosta da Silva
Vice-presidente: Rubens José Gagliardi
Secretário Geral: Gilmar Fernandes do Prado
1ª Secretária: Márcia Maiumi Fukujima
Tesoureira Geral: Mônica Santoro Haddad
1º Tesoureiro: Luiz Henrique Martins Castro
COMISSÃO ORGANIZADORA
Presidente do congresso: Hélio A. G. Teive
Coordenadora da Comissão Científica: Viviane Flumignan Zetola
Tesoureiro: Pedro Kowacs
Presidente de Honra
Lineu Werneck
COMISSÃO CIENTÍFICA
Coordenadora da Comissão Científica: Viviane Flumignan Zetola
Departamentos Cientificos ABN
Diretor Científico: Joaquim Perereia Brasil Neto
DC de Atenção Neurológica e Neurorreabilitação
Coordenador: Cristiano Milani
Vice-Coordenadora: Carla Heloísa Cabral Moro
Secretária: Adriana Bastos Conforto
DC de Doenças Cerebrovasculares, Neurologia
Intervencionista e Terapia Intensiva em Neurologia
Coordenadora: Sheila Cristina Ouriques Martins
Vice-Coordenador: Norberto Luiz Cabral
Secretária: Gisele Sampaio Silva
DC de Cefaléia
Coordenador: Mauro Eduardo Jurno
Vice-Coordenador: Cláudio Manoel Brito
Secretário: Fernando Kowacs
DC de Doenças do Neurônio Motor/ELA
Coordenador: Marco Antonio T. Chieia
Vice-Coordenador: Mário Emilio T. Dourado Jr.
Secretário: Francisco Tellechea Rotta
DC de Neurossonologia
Coordenadora: Ana Cláudia Celestino Bezerra Leite
Vice-Coordenadora: Viviane Flumignan Zetola
Secretária: Daniela Laranja Gomes Rodrigues
DC de Neurofisiologia Clínica
Coordenador: Paulo André Teixeira Kimaid
Vice-Coordenador: Rinaldo Claudino
Secretário: Marcondes Cavalcante França Jr.
DC de Dor
Coordenador: Pedro Schestatsky
Vice-Coordenador: Jaime Olavo Marquez
Secretário: José Geraldo Speciali
DC de Neurogenética
Coordenador: Orlando Graziani Povoas Barsottini
Vice-Coordenador: Renato Puppi Munhoz
Secretária: Laura Silveira Moryama
DC de Epilepsia
Coordenador: Luciano de Paola
Vice-Coordenador: Carlos Eduardo Soares Silvado
Secretário: Vera Cristina Terra
DC de Neuroimunologia
Coordenadora: Doralina G. Brum Souza
Vice-Coordenadora: Claudia Cristina F. Vasconcelos
Secretária: Elizabeth Regina C. Frota
DC de História da Neurologia
Coordenadora: Marleide da Mota Gomes
Vice-Coordenador: Hélio Afonso Ghizoni Teive
Secretário: Francisco Eduardo Costa Cardoso
DC de Neurologia Cognitiva e do Envelhecimento
Coordenadora: Sonia Maria Dozzi Brucki
Vice-Coordenador: Norberto Anízio Ferreira Frota
Secretário: José Ibiapina Siqueira Neto
DC de Líquido Cefalorraqueano
Coordenador: Sérgio Monteiro de Almeida
Vice-Coordenadora: Cristiane Nascimento Soares
Secretário: Sandro Luiz de Andrade Matas
DC de Neurologia Infantil
Coordenadora: Marilisa Mantovani Guerreiro
Vice-Coordenardora: Umbertina Conti Reed
Secretário: José Luiz Dias Gherpelli
DC de Moléstias Infecciosas
Coordenador: Ronaldo Abraham
Vice-Coordenador: Hideraldo Luís Souza Cabeça
Secretário: Marcus Tulius Teixeira da Silva
DC de Neuropatias Periféricas
Coordenador: Susanie Amâncio Gonçalves Rigatto
Vice-Coordenador: Amilton Antunes Barreira
Secretário: Fernando de Mendonça Cardoso
DC de Moléstias Neuromusculares
Coordenadora: Cláudia Ferreira da R. Sobreira
Vice-Coordenadora: Juliana Gurgel Gianetti
Secretário: Elmano Henrique Torres de Carvalho
DC do Sono
Coordenadora: Rosana Souza Cardoso Alves
Vice-Coordenador: Raimundo Nonato Delgado Rodrigues
Secretário: Leonardo Lerardi Goulart
DC de Neuroepidemiologia
Coordenador: Tarso Adoni
Vice-Coordenador: Jefferson Becker
Secretária: Nívea de Macedo Oliveira Morales
DC de Transtornos do Movimento
Coordenador: Egberto Reis Barbosa
Vice-Coordenador: Henrique Ballalai Ferraz
Secretário: Vitor Tumas
Subcomitê Local
Subcomitê – Cefaleia/ Dor
Elcio Juliato Piovesan
Mario Luiz Giublin
Pedro Kowacs
Subcomitê – Doenças Neuromusculares/
Eletroneuromiografia/ Potencial Evocado
Cláudia Suemi Kamoi Kay
Paulo Jose Lorenzoni
Rosana Herminia Scola
Subcomitê – Desmielinizante/ Neuroimunologia
Monica Parolin
Patrícia Coral
Rosana Herminia Scola
Subcomitê – Epilepsia/ Sono/ Eletroencefalografia/
Polissonografia
Carlos Eduardo Soares Silvado
Luciano de Paola
Márcia Assis
Subcomitê – Distúrbios do Movimento
Hélio A. G. Teive
Renato Munhoz
Subcomitê – Doenças Cerebrovasculares, Neurologia
Intervencionista e Terapia Intensiva em Neurologia/
Neurossonologia
Edison Matos Nóvak
Marcos Christiano Lange
Viviane Flumignan Zetola
Subcomitê – História da Neurologia
Francisco Germiniani
Hélio A. G. Teive
Walter Oleschko Arruda
Subcomitê – Moléstias Infecciosas/ Líquido
Cefalorraqueano
Sérgio Almeida
Subcomitê – Neuroepidemiologia
Norberto Luiz Cabral
Subcomitê – Neurofisiologia Clínica
Luciano de Paola
Rosana Herminia Scola
Subcomitê – Neurologia Infantil
Ana Crippa
Isaac Bruck
Sérgio Antoniuk
Subcomitê – Neurogenética
Lineu Werneck
Salmo Raskin
Subcomitê – Neuropsicologia
Maria Joana Mader (Coordenadora)
Mauren Carneiro da Silva Rubert
Denise Ribas Jamus
Raphael Borguezan
Subcomitê – Neuroimagem
Arnolfo de Carvalho
Guilberto Mingueti
Subcomitê – Neurologia Cognitiva e do
Envelhecimento
Mauro Piovezan
Ricardo Krause Martinez Souza
ConvidadoS
Ricardo Nitrini
Ayrton Massaro
Subcomitê – Neurorreabilitação
Marise Bueno Zonta
Nilson Becker
Renato Nickel
Subcomitê – Semiologia
Edison Matos Nóvak
Elcio Juliato Piovesan
Volume 72, Suppl 1, 2014, São Paulo, SP, Brazil
Oral Presentation
Headache....................................................................................................................................13
Vestibular and Balance Disorders.............................................................................................13
Cerebrovascular Diseases.........................................................................................................14
Other Motor Neurone Disease...................................................................................................20
Muscle Disorders........................................................................................................................20
Transcranial Doppler..................................................................................................................20
Epilepsy.......................................................................................................................................21
Amyotrophic Lateral Sclerosis.......................................................................................................... 23
Neurologic Manifestations of Systemic Diseases...................................................................24
Neuroepidemiology....................................................................................................................24
Clinical Neurophysiology...........................................................................................................24
Neurogenetics.............................................................................................................................25
Neuroimaging..............................................................................................................................28
Neuroimmunology, Multiple Sclerosis and other Demyelinating Disorders..........................29
Neuroinfection............................................................................................................................31
Cognitive Neurology and Neurology of Aging...........................................................................32
Neurology of Sleep / Polysomnography....................................................................................35
Child Neurology...........................................................................................................................35
Interventional Neurology...........................................................................................................36
Peripheral Neuropathy...............................................................................................................37
Rehabilitation and Neurology Care...........................................................................................38
Neuro-ICU....................................................................................................................................39
Movement Disorders..................................................................................................................39
Videos
Child Neurology...........................................................................................................................45
Muscle Disorders........................................................................................................................45
Movement Disorders..................................................................................................................45
Neuroinfection............................................................................................................................47
Cerebrovascular Diseases.........................................................................................................48
Rehabilitation and Neurology Care...........................................................................................48
Other Motor Neurone Disease...................................................................................................48
Posters
Headache....................................................................................................................................51
Cerebrovascular Diseases.........................................................................................................61
Muscle Disorders........................................................................................................................90
Pain..............................................................................................................................................101
Epilepsy.......................................................................................................................................103
Amyotrophic Lateral Sclerosis...................................................................................................112
History of Neurology...................................................................................................................115
Neuroimaging..............................................................................................................................117
Interventional Neurology...........................................................................................................123
Neuro-ICU..........................................................................................................................124
Vestibular and Balance Disorders.............................................................................................126
Other Motor Neurone Disease...................................................................................................131
Transcranial Doppler..................................................................................................................132
Cerebrospinal Fluid....................................................................................................................133
Neurologic Manifestations of Systemic Diseases...................................................................134
Miscellaneous.............................................................................................................................151
Neuroepidemiology....................................................................................................................155
Neurogenetics.............................................................................................................................161
Neuroimmunology, Multiple Sclerosis and other Demyelinating Disorders..........................170
Cognitive Neurology and Neurology of Aging...........................................................................190
Clinical Neurophysiology...........................................................................................................200
Neuroinfection............................................................................................................................204
Neurology of Sleep / Polysomnography....................................................................................217
Child Neurology...........................................................................................................................222
Peripheral Neuropathy...............................................................................................................233
Rehabilitation and Neurology Care...........................................................................................242
Movement Disorders..................................................................................................................256
CARTA DE BOAS VINDAS
Prezados Colegas,
Chegou o grande dia! O evento máximo e bianual da Academia Brasileira de Neurologia: o XXVI Congresso Brasileiro de
Neurologia, ou, como carinhosamente o chamamos: o Neuro2014.
São todos bem vindos à Curitiba e ao Neuro2014. Aproveitem a cidade, suas belezas, seus parques e seus inúmeros restaurantes.
Um passeio de trem ao litoral, pela Serra do Mar é uma experiência inesquecível. Porém aproveitem principalmente o Congresso.
O que dizer? Foi pelo menos um ano de preparativos incessantes e cuidadosos, desde a escolha do local, dos convidados estrangeiros, dos palestrantes, dos coordenadores de sessão e do programa, o qual pensamos que está instigante.
As principais modificações que introduzimos na estrutura do congresso foram importantes, e visaram valorizar as atividades
científicas que julgamos ser o objetivo principal do evento, contemplando desde a neurociência básica às novidades na neurogenética, agregando o neurologista clínico e o pesquisador. E a NEUROCOPA 2014, outra inovação, promete ser tanto erudita
quanto divertida.
Acreditamos que nossos esforços foram recompensados. O congresso contará com 31 palestrantes estrangeiros, 349 palestrantes nacionais, em um total mais de 130 sessões, sem contar com os 12 Simpósios Satélites. Ao todo, houve a submissão de
1.445 trabalhos sendo 1.308 destes selecionados para apresentação ou pôster. Contando com mais de 3.765 participantes inscritos até o momento final.
Agradecemos o auxílio de muitos no preparo deste Congresso. Muito devemos aos Departamentos Científicos, à Comissão Local,
aos nossos Patrocinadores e às nossas famílias, as quais não apenas aceitaram que deduzíssemos nosso tempo de convívio mútuo como nos apoiaram nessa empreitada.
Aproveitem o XXVI Congresso Brasileiro de Neurologia, o Neuro2014!
Hélio Afonso Ghizoni Teive
Presidente
Viviane Flumignan Zetola
Coordenadora da Comissão Científica
Pedro André Kowacs
Tesoureiro
Comissão Organizadora do XXVI Congresso Brasileiro de Neurologia
Oral presentaTION
Oral Presentation
XXVI Congresso Brasileiro de Neurologia
AO-002
Headache
POST-TRAUMATIC HEADACHE IN PATIENTS OF
THE NEUROSURGERY SERVICE OF THE HOSPITAL
GOVERNADOR CELSO RAMOS, FLORIANÓPOLIS, SC
Bem MLP, Queiroz LP
• SERVIÇO DE NEUROLOGIA DO HOSPITAL
GOVERNADOR CELSO RAMOS
AO-001
OBESITY AND OBSTRUCTIVE SLEEP APNOEA
SYNDROME IN CHRONIC HEADACHE
Grassi V1, Stelzer FG2, Tomiozzo Junior JC1,
Fornari LHT1, Eckeli A3, Barea LM2
• 1UNIVERSIDADE FEDERAL DE CIÊNCIAS DA SAÚDE
DE PORTO ALEGRE (UFCSPA); 2IRMANDADE SANTA
CASA DE MISERICÓRDIA DE PORTO ALEGRE
(ISCMPA); 3UNIVERSIDADE DE SÃO PAULO DE
RIBEIRÃO PRETO (USP-RIBEIRÃO PRETO)
Background: Morning headache (MH) and
obesity are usually accepted as part of clinical
findings of obstructive sleep apnoea syndrome
(OSA). Obesity, snoring and sleep disorders are
considered potentially modifiable risk factors for
chronic headache. However, headache, obesity
and OSA are common disorders in general population. There is a possibility that these observed
associations are occurring by chance. Aim: The
aim of this study was to estimate obesity and
OSA prevalence between patients with chronic
headache. Methods: A transversal and prospective study including 409 adult patients (over 18
years old) referred to full night polysomnography (PSG) between January and December
2013, in our Sleep Laboratory at Hospital São
José – Irmandade Santa Casa de Misericórdia de
Porto Alegre (ISCMPA). All individuals were interviewed by neurologists to establish headache
characteristics and to diagnose sleep disorders.
The patients’ weight, height, waist circumference
and body mass index (BMI) were also registered.
This study was approved by the research ethics
committee of the ISCMPA and all patients gave
written informed consent. Results: There were
409 patients referred to PSG in the study period
and OSA was present in 318 individuals (77.7%).
In this sample, 41.2% were women (n=131), median age of 52.3±13.8 years. About 52.8% were
obese (n=168), with median BMI of 31.7 kg/m2.
Obesity was not related with higher prevalence
or frequency of headache, as expected. On the
contrary, there was a higher frequency of headache in life (91.1 versus 97.3%; p=0.030) and
in the last year (74.4 versus 84.7%; p=0.027) in
non-obese individuals. There was no association
between obesity and headache semiologic features, like pain topography, duration, frequency,
daytime period, triggers, relief factors or association with menstrual cycle. There was also no
correlation between obesity and the use of pain
killers medication or prophylactic treatment (0.8
versus 3.9%, NS). However, the mean intensity
of pain (on pain rating scale 0‐10) was higher
in the obese individuals (5.6±1.9 versus 5.0±1.9;
p=0.030). Discussion: In our study, we found
no association between obesity and headache
frequency. Higher BMI was not associated with
headache cronification or the use of pain killers
medication among OSA individuals. Disclosure:
Nothing to disclose.
Introduction: Post-Traumatic Headache (PTH)
is one of the main causes of secondary headache. It is estimated that the most common
consequence of cranioencephalic traumatism is
headache. Definitions about the epidemiology,
pathophysiology and specific headache features
of PTH are yet under construction. Objectives:
To evaluate the relevance and characteristics of
PTH in patients with head trauma, followed by
the Neurosurgery Service at the Hospital Governador Celso Ramos, in Florianópolis, SC, and to
verify the relationship of PTH with the presence
of pre-existing headache and with the severity of
the traumatic head injury (THI). Methods: Between May 2013 and October 2013, 45 patients
seen in consultation post-THI were interviewed.
The THI occurred between January 2012 and
August 2013. A structured questionnaire was applied, consisting of items about the characteristics and intensity of the THI, the presence or not
of pre-existing headache, and the characteristics
of the pre-existing headache and of the PTH.
Results: Most patients (39) with head trauma
were male, from 15 to 74 years of age (most in
the third decade of life). The most frequent (49%)
type of head trauma was due to car accident.
Pre-existing headache was present in 42% (19) of
the patients. After the THI, 75% (34) complained
of headache. THI increased 4.23 times the risk of
having a PTH (p<0.001). Of the PTH cases, 56%
had only new headaches; 20% noted a worsening
of their previous headache. The characteristics
of the PTH was similar to primary headaches in
44% of the cases (migraine 29%; tension-type
headache 15%). The presence of pre-existing
headache did not determine greater tendency
to present PTH. In 82,4% of the PTH cases, the
THI was moderate or severe. In PTH cases, headache tended to occur with higher frequency and
intensity. In 56% of the PTH cases, the frequency
of the headaches was ≥15 days/month; and in
53%, the pain was severe. Patients who lost the
conscience for ≥30 minutes, when they had THI,
reported a higher frequency and/or more severe
headaches. Conclusions: PTH is very frequent
in patients who had a THI, regardless of having
or not a pre-existing headache. Contrary to the
usually reported in the literature, the great majority of our patients had a moderate to severe
head trauma. Many PTH patients described a
headache that resembles primary headaches.
Headaches are usually very frequent and severe,
especially if patients had lost their conscience for
more than 30 minutes.
AO-003
QUINTI DIGITI SIGN AND HEMIPLEGIC MIGRAINE :
A FUNCTIONAL MAGNETIC RESONANCE IMAGING
Silva RVB, Sanchez TA, Gasparetto EL, Vincent MB
• UFRJ
Background: The Quinti Digiti Sign (QDS) has
been noted in some patients with hemiplegic
migraine interictally. This suggests that somehow these patients have motor cortex dysfunction during motor activation tasks. A Resting
State protocol of functional magnetic resonance
imaging (fMRI) of mild motor impairment after
stroke has been studied suggesting a recovery
of connectivity after rehabilitation nighty days
following stroke. Objective: The aim of this work
was to verify if patients with hemiplegic migraine
and subtle hemiparesis as demonstrated by the
presence of the QDS show distinct activations
during fMRI. Methods: Ten patients and thirteen
healthy controls were recruited at the headache
clinic of the Clementino Fraga Filho Hospital,
Rio de Janeiro, Brazil. Neurological examination
concerning motor strength, power and reflexes was carefully recorded. To access the QDS,
subjects were asked to horizontally extend both
arms forwards, parallel to the floor, with palms
facing down. A digital picture was taken perpendicularly having both hands in a single shot.
Two clinical evaluations including picture recordings separated by a week were made during
the interictal period. A blind examiner analyzed
the pictures measuring the angle formed by the
prolongations of the lines passing by the axis
of both fourth and fifth fingers. fMRI was used
to investigate brain responses during a simple
motor task of the affected finger in a blocked design consisting of 20 repetitions of 6 seconds of
activity followed by 18 seconds of rest. Analysis
was done using the general linear model (BrainvoyagerTM). Results: The quantitative analysis
showed significant differences between the two
populations. The cortical activation corresponding to the QDS’s hand was smaller as compared
to the contralateral side (p<0,038). The volume
differences between the two sides versus differences between controls hands were greater
in first group (p<0,041). The Beta values were
smaller comparing the patitents QDS hands
with the same side of controls (p<0,023) and was
also smaller when the pantient’s healthy hands
were compared with the same hands in controls
(p<0,038). BLM analysis showed a smaller amplitude in QDS hands when was compared with
the opposite hand (p<0,029). Conclusions: Mild
hemiparesis with the presence of QDS in HM is
associated with different cerebral motor cortex
activation in fMRI interictally, favoring difficulties for appropriate finger tonus and movements.
Vestibular and Balance
Disorders
AO-004
CLINICAL NEUROLOGICAL DEFICITS AND BALANCE
CONTROL IN PATIENTS WITH MULTIPLE SCLEROSIS
Vignola BA1, Pereira CB2, Callegaro D2
• 1USP; 2HOSPITAL DAS CLÍNICAS FMUSP
Balance disorders are one of the most disabling
symptoms in multiple sclerosis (MS) and may
occur even in patients with minimal neurological
signs on clinical examination. However, in these
cases, imbalance is usually underestimated. Usually, when patients complain about balance disorders, physicians focus the neurological examination on cerebellar or somatossensory signs.
The aim of this study was to describe the clinical
neurological deficits related to balance disorders
in MS patients with low disability. Methods: We
evaluated 35 MS outpatients with low disability
(Expanded Disability Status Scale – EDSS: 0 –
3,5). They were divided into two groups: without
13
Oral Presentation
balance complaint (w/oBC, n=21) and with balance complain (w/BC, n=14). We excluded patients with other neurological diseases, vertigo or
dizziness, and cognitive impairment. All patients
underwent a complete clinical neurological examination. Balance was tested with the dynamic
posturography, Pro Balance Master (NeuroCom).
We applied the modified Sensory Organization
Test (mSOT) that measures the relative contributions of visual, vestibular, and somatosensory systems during standing. Statistical analysis
was performed by the Mann-Whitney test and t
Student test. Results: Our results did not showed
differences in age, onset of disease and EDSS between groups. mSOT test proved that balance was
worse in w/BC group than in w/oBC group. We
found significant differences (p<0,05) between
groups in the clinical neurological examination
of motor strength of the legs (reduced in 42,8%
and 85%, w/oBC and w/BC groups, respectively),
spasticity (present only in w/BC group, 75%), superficial sensibility (reduced in 42,8% and 85%,
w/oBC and w/BC groups, respectively) and proprioception (impaired only in w/BC group, 75%).
Visual acuity was not difference between groups
and clinical evidence of cerebellar impairment
was absent in our sample. Conclusions: It is
common belief that cerebellar lesions are the
primary cause of imbalance in MS, for this reason imbalance is usually underestimated in MS
patients without cerebellar signs. Our results
showed that MS patients with low disability did
not have cerebellar impairment, even patients
with complaints about balance. We proved that
strength, spasticity and superficial sensibility are
also important on balance control and have to be
considered, mainly in patients with low disability
and minimal neurological impairments.
AO-005
SCREENING MIGRAINEURS WITHOUT VERTIGO
COMPLAINS WITH VESTIBULAR BEDSIDE TESTS
Maranhão ET1, Maranhão Filho P2, Vincent MB2
• 1UNIVERSIDADE FEDERAL DO RIO DE JANEIRO.
HUCFF; 2UNIVERSIDADE FEDERAL DO RIO DE
JANEIRO- HUCFF-NEUROLOGIA
Introduction: Migraine and vertigo are common
disorders with lifetime prevalence of 16% and
7% respectively; and co-morbidity around 3.2%.
Vestibular syndromes and dizziness occur more
frequently in migraine patients (30‐50%). Due
to the relatively higher prevalence of vestibular
disorders in migraineurs, clinical tests specifically designed to detect even subtle vestibular
dysfunctions are expected to be abnormal in this
condition. A systematic search for bedside clinical signs indicative of vestibular dysfunction in
migraineurs has not been performed. Objective:
To compare vestibulo-ocular (VOR) and vestibulo-spinal (VSR) responses as estimated by bedside tests in migraineurs without history of vertigo with controls. Patients and Methods: In this
cross-sectional study sixty individuals, thirty migrainous patients; 25 women, 19‐62 y-o (median
39.3 years) without vertigo complains and thirty
sex and age healthy paired controls were evaluated. Bedside tests were used to assess the VOR
and VSR reflexes. For the first, the head impulse
test (HIT), head shaking manoeuvre (HSM), dynamic visual acuity test (DVA), and the subjective
visual vertical test (SVV) were performed; and for
the second the applied tests were the clinical
test of sensory integration and balance (CTSIB),
sharpened Romberg test (SRT), Fukuda stepping
test (FST), and past pointing test (PPT). All subjects underwent a neurootological examination
14
including the minimal ice test (MIT). The Wilcoxon Signed Rank test and the McNemar chi-square
test were used for statistical comparisons. p values <0.05 were considered significant. This study
was approved by the local Ethics Committee.
Results: There was a tendency for migraineurs
to perform worse in all tests except for the VVS
test, but head to head comparisons showed that
only the SRT was statistically different between
patients and controls (p=0.039). Taken together, considering the frequency of abnormal responses, patients performed significantly worse
than controls (p=0.003, Wilcoxon). Conclusion:
Migraine patients consistently showed abnormal vestibular bedside tests when compared
with healthy controls. This indicates that the
vestibular function is impaired subclinically in
migraineurs without vestibular complains and
that bedside tests are suitable to detect such dysfunctions. Whether these changes are specific for
migraine remains to be determined.
Cerebrovascular Diseases
AO-006
ACUTE STROKE CARE: FACTORS ASSOCIATED
WITH EARLY MEDICAL ASSESSMENT IN THE
EMERGENCY DEPARTMENT OF A REFERRAL
CENTER IN NORTHWESTERN BRAZIL
Barreto ES1, Brito FS1, Farias CA2, Paranhos DR1,
Guimarães VP1, Maldonado IL3
• 1EBMSP; 2FTC; 3UFBA
Introduction: In the Brazilian public health system, a significant proportion of stroke victims
still search and/or obtain medical attention several hours or days after the initial vascular event.
In 2012, the Brazilian Ministry of Health published the decrees 664 and 665, which are official
protocols for thrombolytic treatment. Previous
data from our research unit showed that in 2013
there were a greater proportion of patients that
were admitted in the first 24 hours after onset
of symptoms in comparison with the year 2011,
which was before publication of the official protocols. Purpose: The aim of the present study
was to identify factors that were associated with
a smaller interval of time from the onset of signs
and symptoms to the hospital admission, in a referral center at Salvador-Bahia. Method: This was
a cross-sectional study with a retrospective and
observational design. The sample consisted of
patients received in an emergency unit of a public referral center. The following variables were
recorded: time of symptom onset, hospital admission (registered electronically), level of consciousness according to the Glasgow coma scale,
location of origin, age, severity of motor deficit
and presence of language disorder. Results: One
hundred thirty-three patients were included in
the study. Factors associated with first medical
assessment in the emergency department before 4h30min were ambulance transportation
and GCS ≥13. Very few patients came were from
out of the city, and all of those patients arrived
with more than 24h from the neurological deficit.
Conclusions: Rapid ambulance transportation
and a relatively preserved level of consciousness seem associated with shorter pre-hospitalar
times from the onset of neurological deficit to
hospital arrival. This strengthens the importance of an effective public pre-hospitalar care
organization
AO-007
ASSESSMENT OF RECANALIZATION IN CEREBRAL
VENOUS THROMBOSIS RELATED TO TIME IN
THERAPEUTIC RANGE OF ANTICOAGULATION WITH
WARFARIN
Suzano FL, Cordellini MF, Cavalli H, Rizelio V,
Kowacs PA
• INSTITUTO DE NEUROLOGIA DE CURITIBA
Introduction: Cerebral venous thrombosis is
an uncommon disease, whose treatment with
anticoagulation is imperative. Few studies have
investigated the process of recanalization of the
cerebral venous system, and in an unprecedented way, this study correlates this data to treatment with oral anticoagulation. Objectives: assess whether the time percentage in therapeutic
range of anticoagulation (INR ≥2.0) influenced
the degree of recanalization of the dural venous
sinuses and cortical veins in patients treated with
warfarin after a diagnosis of Cerebral Venous
Thrombosis. Methods: retrospective, descriptive
study including 17 patients. A comparative analysis was made of the extent of recanalization,
through images of brain magnetic resonance imaging (MRI) and cerebral venous magnetic resonance angiography performedin the acute phase
and after treatment for 6 months and 12 months,
respectively, with the outcome being total or partial recanalization. The International Normalized
Ratio (INR) values up to or above 2.0 were considered to calculate the time percentage in therapeutic range. Variables included age, gender,
risk factors and location of thrombosis. Results:
The rate of partial recanalization in this population was 64.7% (11). The variables age, gender
and risk factors, as well as percentage of time
in therapeutic range of anticoagulation did not
influence the final extent of recanalization. We
found partial recanalization in 83% of patients
with sigmoid sinus thrombosis (p<0.05) and in
82% with transverse sinus thrombosis (p=0.109).
All patients who spent more than 90% of the time
in therapeutic range of anticoagulation showed
partial recanalization. The treatment time for total recanalization was 6 months. No patient with
partial recanalization, evolved to total recanalization with extending treatment for 12 months.
Conclusion: total venous recanalization was not
related to the efficiency of anticoagulant treatment in this sample. It is suggested that it may
be related to risk factors and the location of the
thrombus.
AO-008
ATRIAL FIBRILLATION IN STROKE: ETIOLOGIC
PROFILE IN JOINVILLE/SC
Cunha RGD, Zalli M, Diegoli H, Schulz VC, Moro CHC
• HOSPITAL MUNICIPAL SÃO JOSÉ
Introduction: Stroke is an important cause of
morbidity and mortality worldwide. Thus, it is
clearly important in determining the etiology
of the event for secondary prevention. According to the TOAST classification, there are five
possible etiologic mechanisms: cardioembolic, atherothrombotic, lacunar, or other undetermined causes. Atrial fibrillation is the most
frequent cause of cardioembolic mechanism.
Nevertheless, it is known that many patients are
XXVI Congresso Brasileiro de Neurologia
not diagnosed at the time of etiologic investigation by the difficulty to identify paroxysmal
AF, impairing adequate secondary prevention.
Recent study showed that 2.2% of patients who
underwent ECG monitoring for 24 hours were
diagnosed with FA, while 14.8% of those who
had monitoring for 4 weeks. CRYSTAL-AF Study,
which entered implantable monitoring system
that points out the number of paroxysmal AF,
detected nearly 4 times greater than the control
study. Objectives: To determine the prevalence
of cardioembolic stroke in atrial fibrillation and
stroke of undetermined origin in Joinville/SC in
the period October 2009 to June 2014; Compare
the prevalence of cardioembolic stroke by AF
and stroke of undetermined origin in Joinville/
SC with data from other large centers. Methods:
From October 2009 to June 2014, all patients with
clinical stroke in the city of Joinville were recorded and classified using the TOAST criteria. All the
patients were asked for previous atrial fibrillation
(AF) and were submitted to at least one electrocardiogram (ECG). We considered AF present
in the patients that referred the disease or in
which the ECG showed signs of it. All data were
collected from the JOINVASC Study. Results:
From October 2009 to June 2014, were recorded
in Joinville/SC 2789 cases of ischemic stroke,
of which 676 (24.24%) were atherothrombotic;
808 (29.01%) were cardioembolic; 598 (21.44%)
were lacunar; 573 (20.54%) were of undetermined origin; and 133 (4.77%) had other causes.
Of the 808 cardioembolic, 322 (39.85%) are due
to AF and 486 (60.15%) are other cardioembolic
sources. The FA rate in the sample was 13.4%.
Conclusion: Atrial fibrillation is the cause of a
significant proportion of cardioembolic strokes,
even in our service that uses only electrocardiogram (ECG) and Holter 24h in selected cases. It is
concluded that the implementation of more sensitive methods for diagnosis of paroxysmal AF in
the etiology of ischemic stroke is needed.
AO-009
BASILAR ARTERY OCCLUSION: CALL FOR A MORE
AGRESSIVE TREATMENT?
Dias FA, Cougo-Pinto PT, Alessio-Alves FF, CastroAfonso LH, Camilo MR, Santos-Pontelli TEG, Abud DG,
Pontes Neto OM
• HCFMRP-USP
Background: Acute basilar artery occlusion
(BAO), which accounts for only about 1% of
acute ischemic strokes, usually leads to severe
disability and mortality (80‐90%). Intravenous
(IV) thrombolysis within 4.5 hours was proven
safe and effective for the treatment of acute ischemic stroke in randomized, phase 3 trials. Endovascular therapy has been tested up to 8 hours
for ischemic stroke. However, the number of patients with posterior circulation ischemia – more
specifically with BAO – included in these studies was small. Therefore, the most appropriate
recanalization strategy in patients with BAO is
still controversial. Objective: To assess the clinical outcomes of patients with BAO within each
modality of recanalization therapy in a tertiary
academic hospital in Brazil. Study methods: This
is a retrospective analysis of patients with BAO
selected from the hospital-based, prospective
stroke cohort (REAVER) at Ribeirão Preto Medical School. Primary outcomes were mortality
and non-severe disability [modified Rankin Scale
(mRS)<4] at 90 days. Results: Between August
2004 and August 2014, 26 patients (61.5% male),
were diagnosed with BAO, based on transcranial
ultrasound, computed tomography angiography
or magnetic resonance imaging angiography. The
most common stroke risk factors were hypertension (69.2%); diabetes (26.9%); smoking (26.9%);
and 9 (34.6%) had a previous stroke. The median
National Institutes of Health Stroke Scale was 26
[interquartile range (iQR): 14‐38], and the median time from symptoms onset to admission was
4.5 hours (IQR: 2.5‐8.2). Ten patients received no
recanalization therapy, 7 received IV thrombolysis and 9 received endovascular therapy. Patients
treated conservatively had 80% mortality and no
patient achieved a mRS<4 at follow-up. Patients
treated with IV thrombolysis and endovascular
therapy had 57.1% and 44.4% of mortality and
14.2% and 22.2% of mRS<4 at follow-up, respectively. Only one patient in the whole sample had
an excellent outcome (mRS=1) after being treated with IV thrombolysis. There was no significant
statistical difference between the outcomes in
each group. Conclusions: BAO is a catastrophic
event with high morbidity and mortality. Current
recanalization therapies did not seem to alter
the dramatic course of disease in our sample.
There is an urgent need for randomized studies
to evaluate the best therapeutic strategy for this
condition.
AO-010
DABIGATRAN ETEXILATE VERSUS WARFARIN
IN MANAGEMENT OF NON-VALVULAR ATRIAL
FIBRILLATION IN PATIENTS FOLLOWED AT A
REFERENCE CENTER OF SALVADOR, BAHIA,
BRAZIL
Monteiro JMC, Sousa TRM, Pinto BM, Costa BAL,
Campana IG, Bouza CB, Martin DLS, Jesus PAP
• UFBA
Introduction: the RE-LY study showed superiority of the use of dabigatran in patients with
non-valvular atrial fibrillation (NVAF) compared
to warfarin for prevention of thromboembolic events, particularly stroke, and reduction of
bleeding. However, there are few data about the
use of dabigatran in brazilian population. This
study aims to evaluate the effectiveness of these
medications in a group of patients with NVAF.
Methods: case-control study with patients with
NVAF and oral anticoagulation therapy with dabigatran (cases) or warfarin (controls) followed
at a reference stroke center in Salvador, Brazil,
between January/2012 and August/2014. Clinical characteristics and number of consultations
were compared between the groups. A non-parametric test was performed to assess differences
between groups related to ratio variables. Results
are described using mean ± standard deviation,
median [interquartile range] or proportion. The
p-value is shown for a two-tailed and exact test.
Results: 58 patients were collected (21 cases,
42.9% females and 37 controls, 56.8% females)
with similarity between cases and controls for
age’s distribution (69.7±10.6 vs 63.8±18.6 years,
p=0.078) and gender. All cases and 12 controls
had stroke. The CHA2DS2Vasc was higher in cases than controls (5[4‐5.5] vs 3[2‐4.5], p<0.001).
Small bleeding was found in five patients of control group and two patients of case group. Stroke
occurred in two controls during the treatment.
The number of appointments adjusted by time
of follow-up in years (AA) was lower in cases than
in controls (3.4[2.4‐5.5] vs 9.2[8.2‐10.2], p<0.001).
The number of AAs on which was necessary reduction or interruption of medication was higher
in controls compared with cases (1.9[1.3‐3.4] vs
0[0‐1.4], p<0.001). On the sub-groups analysis
with stroke patients, the number of AAs was lower in cases compared with controls (1.5[0.8‐2.6]
vs 9.7[7.2‐10.4], p=0.001), as well as the number
of AAs that were necessary reduce or interrupt
the medication (0[0‐1.4] vs 3.4[2.4‐5.5], p=0.007),
being necessary to do more consultation in controls for RNI evaluation when compared to main
group. Conclusion: the data indicate the usage
of dabigatran is safe and allow better therapeutic
control reducing number of appointments and
adjust of dose on patients with NVAF. Patients
with stroke’s history can present better thrombotic risk control and lower intervention when
using this medication.
AO-011
EFFECT OF THROMBOLYTIC THERAPY ON THE
OCCURRENCE OF MILD COGNITIVE IMPAIRMENT
AND DEMENTIA AFTER STROKE
Silva ES1, Dalpizol L2, Rodrigues F1, Röhers P1,
Martins SCO1, Chaves ML1
• 1HCPA; 2HCA
Introduction: Thrombolytic therapy reduces
functional dependence and mortality after acute
stroke but its action upon cognitive condition
is still unknown. Objectives: To evaluate the influence of the thrombolytic therapy on the incidence of cognitive impairment and dementia
after stroke. Methods: Quasi-experimental study
with post acute ischemic stroke patients of the
neurovascular unit of a public hospital in southern Brazil. The thrombolytic therapy followed
the standard protocol. A questionnaire was administered to patients or family members in the
post ischemia period to assess pre-stroke functional and memory conditions, and depression.
Patients with cognitive impairment, depression
and aphasia were excluded. The first assessment was carried out between 30 and 90 days
after ischemia and the second after 6 months.
The primary outcome was cognitive impairment
or dementia defined by the CDR scale. The instruments used were the NIHSS scale, modified
Rankin and Barthel, Mini Mental State Examination, semantic verbal fluency, clock drawing
test, Boston Naming Test, digit span forward and
reverse, basic and instrumental activities of daily
living scale, Fulg-Meyer scale and Beck Depression Inventory. Results: Two hundred and thirty
two (232) patients and/or their relatives were interviewed, from whom 90 (38.8%) were included
in the study (27 pertained to the thrombolized
group). Thrombolized patients were significantly
more severely affected (according to the stroke
scales). In the first assessment, thrombolized patients had more depressive symptoms and poorer cognitive, functional and motor performance.
At 6 months of follow-up, 28 patients met criteria
for mild cognitive impairment (MCI) or mild/
moderate dementia, from whom 12 (42.9%) pertained to the thrombolized group and 16 (57.1%)
from the non-thrombolized group (p=0.074). The
non-thrombolized group showed greater stability of the cognitive status at 6 months compared
to the first assessment, while the thrombolized
group showed significant migration either for
enhancement and for deterioration (p=0.02).
Conclusion: Patients submitted to thrombolytic therapy showed higher incidence of MCI/
dementia at 6 months of follow-up, but showed
greater category migration both for enhancement and for deterioration than the non-thrombolized patients. A longer follow-up is in progression to clarify these findings.
15
Oral Presentation
AO-012
HOSPITALIZATIONS AND DEATHS BY VASCULAR
BRAIN INJURY IN SOME CAPITAL OF BRAZIL: AN
ANALYSIS OF SECONDARY DATA
Guimarães VP, Pires MS, Figueirôa F
• ESCOLA BAHIANA DE MEDICINA E SAÚDE PÚBLICA
Introduction: The stroke is the second major
cause of death in the world, and also an importante cause of hospitalization and parcial or
complete disabillity between elderly and middle-age adults. The brazilian epidemiological
profile is marked by a high increase of deaths
for cerebrovascular diseases and also for a higher number of people with chronic diseases and
incapacities, mainly because of the increased
populational aging. Thereby, it becomes necessary the analysis and comparison of the epidemiological profile at the main brazilian capitals,
in a way to understand the profile of the pacients
most affected by this clinical condition for the
creation of more incisive and directed public
policies. Objective: Describe and analyze the
profile of hospitalization and deaths by stroke
on some brazialian capitals, according to gender
and age. Methods: A descriptive study was conducted using secondary data from the Hospital
Information System of the Unified Health System
(SIH / SUS), deaths in the Mortality Information
System (SIM / SUS) and resident at the IBGE
Census 2010 population, based on available
Datasus of the Ministry of Health. The study was
conducted in the main capital of each one of the
five regions of Brazil: Manaus, Salvador, Sao Paulo, Brasilia and Curitiba. Results: The proportion
of hospitalization for stroke in Brazil showed the
most significant increase in the Northeast. The
Southeast region decreased the proportion of
such admissions over this period, from 49.78%
in 2008 to 45.99% in 2011. Most regions showed
that there was a higher frequency of hospitalizations among males, but in Salvador and Brasilia
was a reversal of these values​​. The number of
hospitalizations was higher in individuals 40‐49
years, but the death rate tends to increase with
increasing age of patients, reaching 18,192 cases
in individuals aged 80 or over in 2011. Discussão:
Higher rates of hospitalization was observed in
men, although the number of deaths is higher in
females, which may be associated with increased
life expectancy of women, which also seek medical care in greater proportion per year, translating into better health prevention.
AO-013
INTRAVENOUS THROMBOLYSIS IN VERY ELDERLY
PATIENTS WITH ACUTE ISCHEMIC STROKE
Camilo MR, Pinto PTC, Barreira CMA, Dias FA, Alves FFA,
Rocha LJA, Libardi MC, Martins Filho RKV, Santos RSA,
Pontelli TS, Abud DG, Pontes Neto OM
• FMRP-USP
Background: The population of elderly individuals is growing worldwide and advanced age is a
strong predictor of stroke. However, the routine
use of intravenous thrombolysis (IVT) remains
debated in very elderly patients, mostly duo to
safety concerns. The aim of this study was to
determine the rate of symptomatic intracranial hemorrhage (SIH) and in-hospital mortality
among elderly patients with ischemic stroke
treated with IVT in a public Brazilian academic
hospital. Methods: Consecutive stroke patients
aged 80 years old and older were retrospectively selected from a single-center, hospital-based
prospective registry of stroke patients. Collected
16
data included treatment with IVT, demographics,
cerebrovascular risk factors, comorbidities, National Institutes of Health Stroke Scale (NIHSS)
at admission and in-hospital mortality. Brain
computed tomography scans were blindly reviewed to assess the occurrence of SIH (defined
according to the ECASS 3 criteria). Results: Between January 2006 and July 2014, 285 patients
aged ≥80 years old were admitted with ischemic
stroke. Among those, forty-five (15.8%) received
IVTaccording to our institutional protocol. Seven (15.5%) of those also received endovascular
rescue treatment. The mean age was 83.9±3.8
years with predominance of females (62.2%).
Arterial hypertension was the most frequent risk
factor for stroke (80%) followed by atrial fibrillation (44.4%). The mean time between symptoms
onset and IVT was 203.6±69 minutes. Thirty-one
(68.9%) patients had total anterior circulation
syndrome on admission. The median baseline
NIHSS was 18 (IR: 13‐21.5). There were 14 (31.1%)
in-hospital deaths and only two (4.4%) cases of
SIH. Six (85.7%) of the seven patients who underwent endovascular therapy died. Conclusions:
IVT was safely administered in very elderlyacute
stroke patients and was not associated with an
increase risk in SIH at a high volume academic
stroke center in Brazil. Further studies should
addressthe safety of endovascular rescue treatment for this population.
AO-014
IS THERE A CORRELATION BETWEEN RIGHT-TOLEFT SHUNT SIZE WITH STROKE PHENOTYPIC
CLASSIFICATION IN DETERMINING THE
CARDIOEMBOLIC EVENTS?
Chamma JF, Zetola VF, Hadas TC, Lange MC, Novak EM
• UFPR
Background: ASCOD is a new system to phenotype patients with ischemic stroke to better describe the overlap between diseases underlying
a cerebral ischemic event. The finding of FOP
has better causal correlation when associated
with other cardiac anatomical abnormalities and
cryptogenic stroke. Objetive: We hypothesized
that the larger size of the shunt D/E and the passage of microbubbles without Valsalva detected
by DTC may have better correlation with cardioembolic event. Methods: Retrospective analysis
of patients with RLS selected through the database of reports of Neurosonology Laboratory
associated with the review of medical records
with permission of the physcian assistant. The
RLS size was classified according to the following
criteria obtained by DTC: below 10 microbubbles (MES) was considered small, and greater
than 10 MES was considered significant. We also
correlate the passage of MES without Valsalva.
The ASCOD classification was obtained from
medical records. For data analysis we excluded
all patients with rating of C1 with cardiac findings unrelated to shunt. Results: A total of 124
patients were included divided in two groups
according to the classification of ASCOD (C): 55
patients in group 1 classified as “C2” and 69 in
group 2 as “C3”. In 38 (69%) patients of group 1
and 46 (66%) in group 2 were classified as significant RLS we found no correlation. In 44 (80%)
patients of group 2 and 45 (65%) in group 3 also
had a MES during the rest test we found a statistical trend (p=0.069). Conclusion: At date there
is no evidence linking the size of the shunt or
the rest passage of MES with higher risk of cerebrovascular events. The FOP is usually classified
as probable or simply a finding depends on the
clinical and EET exam. Probably further information evaluated in additional tests (TCD) may
bring future considerations for decision making.
AO-015
IS THERE A CORRELATION BETWEEN THE
ROPE SCORE AND THE RECURRENCE OF
CEREBROVASCULAR EVENTS?
Zetola VF, Chamma J, Lange MC, Hadas TC, Bruch T,
Novak EM
• UFPR
Background: The RoPE score (Risk of Paradoxical Embolism) was recently published with the
goal of identifying the probability of the detection of patent foramen oval represent ´the cause´
of cryptogenic cerebrovascular event or just an
incidental finding. According to the literature
it is possible that this score can also be used to
demonstrate low recurrence rate when the isquemic event is related to the FOP. Objetive: Apply
retrospectively score the Rope in patients with
cryptogenic stroke and correlate with the time of
follow-up for recurrence. Methods: Selected 105
patients in outpatients with cerebrovascular diseases without defined etiology (CS = cryptogenic) but with the presence of shunt D / E detected
by transcranial Doppler or echotransesophageal
exam. We classified in two groups. The group 1=
probable PFO-related stroke once the RoPE score
>6 points and group 2= probable PFO incidental finding once RoPE score ≤6. The recurrence
rate was obtained by the own patient‘s records
during their follow-up. For all patients we performed a phone call in July 2014 for the latest
information. We excluded patients who died by
unknown cause and those who could not obtain
information or reliable information. Results: Of
total 75 patients, 24(32%) were been followed for
less than 2 years, 32 (42%) between 2 and 8 years
and 19 (25,3%) more than 8 years. There was no
statistically difference among the three follow-up
groups and rates of recurrence (p=0,20), as well
for the values assigned in RoPE score (p=0,27).
In group 1 (n=38) we found 18% and in group
2 (n=39) 25% of recurring events, no significant
difference (p=0,79). Conclusion: In this retrospective study there was no significant correlation between the rates of recurrent stroke and
the values assigned by the RoPE score. Further
studies are necessary to validate the RoPE score.
AO-016
IS THERE A GOOD CORRELATION
BETWEEN TRANSCRANIAL DOPPLER WITH
TRANSESOPHAGEAL ECHOCARDIOGRAPHY TO
DETECT RIGHT-TO-LEFT SHUNT (RLS)?
Chamma JF, Zetola VF, Mendes DC, Lange MC,
Novak EM
• UFPR
Background: Right-to-left shunt (RLS) can be
identified by transesophageal echocardiography
(TEE) or by transcranial Doppler (TCD) during
the monitorization. Both techniques use intravenous injection of agitated saline with air in a peripheral vein as a contrast agent. Once uniform
protocols for detection and interpretation are
not established, each service seeks to improve its
own agreement. Objetive: Assess agreement between cTEE and cTCD examinations in detecting
RLS. We also evaluated the correlation between
the tests to qualify between small and large shunt
considering the literature data Methods: We retrospectively analyzed a database from Neurossonology Laboratory of the Hospital de Clinicas
XXVI Congresso Brasileiro de Neurologia
of the Federal University of Paraná that showed
a presence of RLC in at least one of the exam.
We consider the descriptive reports of both examinations to qualify the size of the shunt. Regarding the TCD report the detection over than
10 microembolus was considered large. We excluded patients who had undergone just one
test. Results: Of the total of 106 selected patients,
93 (87.7%). had positive results in both tests. No
agreement was found in 13 of the tests. In 2 patients the RLS was evidenced only by the TEE and
11 only by TCD in patient. We assumed 100% of
positivity in each exam and found that TCD had
a 98,12% sensitivity and TEE had a 89.62%. When
the descriptive data for each test were compared,
we could not find any information regarding
the RLS size in 33.96% of the reports in the TEE.
The agreement between the tests was obtained
in only 52.5% of the cases where both tests were
reported correctly. Conclusion/Comments: Our
comparative study confirms literature data that
show high sensitivity of both TCD and TEE for
detect RLS. The goal of performing both tests
includes support decision making involving the
consideration of a large or small shunt. Although
the literature reinforces the difference between
the operators and the technics of examiners, our
work reveals that there is also a necessity to adjust the report prior to comparing. Our work calls
for urgent guideline activity in the areas of TEE
and DTC so that the results may contribute to
clinical decision making.
AO-017
MEASURES TO REDUCE THE DOOR-TO-NEEDLE
TIME IN STROKE THROMBOLYSIS: A BRAZILIAN
EXPERIENCE
Tansini G, Ducci RD, Nóvak EM, Germiniani FMB,
Zetola VF, Lange MC
• UFPR
Background: The door-to-needle time (DNT) is
an important goal in order to reduce the time
to treatment in intravenous thrombolysis (IVT).
The use of simple measures could reduce significantly the DNT. Aims: To analyze if the inclusion of some of those measures, in addition
to specific local actions could reduce DNT in a
Brazilian hospital. Methods: Patients admitted
for emergency neurological evaluation with ischemic stroke who were submitted to IVT within
4.5 hours after symptoms onset were separated
in two groups: in the first group, patients arrived
in the CT room for neurological evaluation and
brain image and were admitted in the Emergency Room for IVT (ER Group); in the second group,
after arrival in the CT room, patients were admitted in an exclusive bed [“Thrombolysis Bed”
(TB)] in the general neurology ward (TB Group)
for IVT. Median DNT for both groups was analyzed. We also evaluated the results of a subset of
patients treated within 60 minutes of admission.
Results: Patients from the ER Group [45(31.5‐61)
min] had a higher median DNT when compared
to the TB Group [37(26‐49.5) min], p=0.008. Sixty-eight (86.0%) patients from TB Group were
treated in the first 60 minutes of arrival as compared to only 48 (67.6%) in the ER Group ER
(p=0.011). Conclusion: The introduction of a TB
associated to the previous implementation of
simple measures in a general hospital setting can
markedly reduce the DNT, thus allowing more
than 85% of patients to be treated within the first
hour of admission.
AO-018
The admission in a Stroke Unit (SU) is one of
the most important measures that modify
the prognosis of patients with ischemic
stroke (IS)
Ferreira LFT, Ducci RD, Lange MC, Zetola VF,
Nóvak EM
• UFPR
Introduction: The admission in a Stroke Unit
(SU) is one of the most important measures that
modify the prognosis of patients with ischemic
stroke (IS). Objective: Analyze the complications
and the mortality rate in patients admitted in a
SU compared to patients admitted in a general
ward of neurology (GW). Methods: Patients with
IS were selected in two different periods, 91 patients before (GW group) and 91 patients after
the establishment of the SU (SU group). Those
groups were compared in relation to their mortality rate, their level of independence and their
following in-hospital complications: pneumonia, urinary tract infection (UTI), deep venous
thrombosis (DVT), pressure ulcer (PU) and hemorrhagic transformation (HT). Results: There
were no statistical differences between the studied groups for all the variables analyzed. There
were three deaths in the GW compared to eight
deaths in the SU, p=0.212. Forty-seven of the GW
patients were independent in the discharge compared to 55 SU patients, p=0.119. Pneumonia occurred in 10 GW patients versus 12 SU patients,
p=0.720. UTI occurred in five patients from the
GW and in three patients from the SU, p=0.821.
There were no cases of DVT in both groups. Two
patients had PU in the GW and one in the SU,
p=1.00. The number of HT cases were the same
in both groups, three cases, p=1. Conclusion:
The SU implemented in our hospital used the
same facilities and professionals as the previous
GW of neurology. In addition, the groups were
similar in previous risk factors related to bad
outcome after IS. These facts might explain the
results observed in the study.
AO-019
PAEDIATRIC NEUROVASCULAR CONSULTATION IN
SICKLE CELL DISEASE
Silva RL, Manita M, Conceição C, Maia R, Kjollerstrom P
• CENTRO HOSPITALAR LISBOA CENTRAL
Introduction: Cerebrovascular disease strongly
contributes to mortality and morbidity in Sickle Cell Disease (SCD) patients. Since the STOP
Study (published in 2008) systematic transcranial doppler (TCD) is recommended in order to
stratify stroke risk and implement regular blood
transfusions if elevated blood flow velocities are
present. Our Hospital is a SCD reference center
and a Pediatric Neurovascular Consultation was
created in articulation with the Hematology and
the Neurosonology Units to improve the neurological care of these children and adolescents.
Objectives: To evaluate the neurological surveillance and stroke prevention programme of the
paediatric SCD patients followed in our Hospital. Materials and methods: Data were collected
from SCD children/adolescents followed in the
Haematology Unit with at least one appointment since January 1st 2013. Variables analysed
were age, gender, TCD and MRI findings, formal
neurological evaluation and the occurrence of
stroke, learning disability and headache in this
population. Results: The Paediatric Haematology Unit currently follows 96 patients with SCD
(91% HbSS), 55% male, 64% between 7 and 18
years and 29% between 2 and 7 years old. Positive stroke history was present in 4 (4%) patients,
occurred from 3,5 to 12 years of age, mainly in
the middle cerebral artery territory and with
recurrence in one patient. Of the 91 patients
older than 2 years of age, 88% had performed
TCD according to the International Guidelines,
94% with normal blood flow velocities and 2,5%
with pathologically elevated values. MRI was
performed in only 25 (26%) patients and 52%
of them had pathological findings, namely 2
with intracranial arterial stenosis, 5 with isolated silent infarcts and 4 with stroke (2 with silent
infarcts and 2 also with moyamoya pattern). A
formal Paediatric Neurology evaluation was obtained so far in 55 (57%) children/adolescents
with SCD. Among these, headaches were present
in 23 (41%) and learning disability in 19 (41%).
Conclusions: Our SCD patients are followed
according to the International Guidelines, 88%
of them had performed TCD regularly and 4%
have a positive history of stroke. The Paediatric
Neurovascular Consultation had an important
role, promoting the communication between
the Haematology and Neurosonology Units, preventing cerebrovascular disease and improving
the care of these children and adolescents.
AO-020
PILOT STUDY OF CARDIAC MAGNETIC RESONANCE
IMAGING IN CRYPTOGENIC ISCHEMIC STROKE
Ferreira IL, Barreto-Neto N, Torreão JA, Fernandes RD,
Gonçalves BM, Andrade AL, Reis CC, Resende LL,
Jesus AA, Lordelo MJ, Abbehusen C, Reis F, Jesus PA,
Oliveira Filho J
• UFBA
Background: Defining the underlying mechanism of ischemic stroke (IS) is one of the cornerstones of secondary prevention, but up to 30%
of all IS are cryptogenic. We aimed to determine
the added clinical value of cardiac magnetic resonance imaging (CMRI) in patients with cryptogenic stroke. Methods: Consecutive patients
admitted to an outpatient university-based
stroke clinic were investigated for stroke etiologic subtyping using the Causative Classification
of Stroke. Patients with undetermined cause of
stroke were further investigated using CMRI.
Chagas disease (CD) status was also determined
in these patients using an ELISA assay. Results:
We studied 38 patients, mean age 54±15 years,
18 (47%) female, 10 (29%) with CD. A potential
source for cardioembolism was found in eight
(22%) patients: eight (22%) with wall fibrosis,
four (11%) with wall edema and two (5%) with
intracardiac thrombus. CD was associated with a
greater number of abnormalities on CMRI when
compared to non-CD patients (median zero,
range 0‐3 versus zero, range 0‐1, respectively,
p=0.027). Conclusions: Potential sources of cardioembolism are found on CMRI in a significant
proportion of patients previously classified as
having cryptogenic stroke, especially among patients with CD.
17
Oral Presentation
AO-021
POLYPHARMACY IN INDIVIDUALS ATTENDING AN
OUTPATIENT REFERRAL OF STROKE IN SALVADOR,
BAHIA, BRAZIL
Passos Neto CEB, Echegaray MVF, Matias LG, Pinto BM,
Souza IFB, Fukuda TG, Jesus PAP
• UFBA
Introduction: Polypharmacy (PP) is defined as
the use of five or more medications. Patients who
have suffered stroke deserve attention regarding
the use of drugs, since there is a need to control
risk factors and the frequency of comorbidities
is high. Objectives: To describe the frequency of PP in an outpatient referral for secondary
prevention of stroke in Salvador, Bahia, Brazil.
Methods: This study was a cross-sectional study
of 219 patients attending an outpatient referral
of stroke, assessed from April 2014 to August
2014. It is an analytical study, with data collected
from medical records and through pre-defined
interview. Measures of central tendency and frequency were used for description. For univariate
analysis, chi-square tests (categorical variables)
and Mann-Whitney test (numerical variables)
were made; logistic regression by backward
conditional method was used for multivariate
analysis. The data were analyzed using the software SPSS v21. Results: The sample included
219 patients. 119 subjects (54.3%) were women
and 100 (45.7%) were men. The average age was
59.45 years old. 170 subjects (77.6%) have hypertension, 168 (76.7%) dyslipidemia, 60 (27.4%)
type 2 diabetes mellitus, 41 (18.7%) post-stroke
epilepsy, 28 (12.8%), congestive heart failure, 21
(9.65%) atrial fibrillation and 18 (8.2%) coronary
artery disease. 5 (2.3%) patients uses only one
medication, 69 (31.5%) uses 2 to 4, 130 (59.4%)
uses 5 to 8, and 12 (5.5%) uses 9 to 13. Thus, 142
have PP (65%). Univariate analysis found a statistically significant correlation between PP and
age (p<0.001), hypertension (p<0.001), diabetes
mellitus (p<0.001), dyslipidemia (p<0.001) and
congestive heart failure (p<0.072) and smoking
(p<0.113). In multivariate analysis, there was
an association between PP and hypertension
(p<0.001, OR: 7.757, CI: 3.2163 to 18.729) and diabetes mellitus (p<0.001, OR: 6.547, CI: 1.352 to
9.304) and a trend towards association with dyslipidemia (p<0.070) and congestive heart failure
(p<0.129). Age (p<0.669) showed no association.
Conclusions: PP is a problem in stroke patients,
represented by 65% of the patients in this study,
surpassing more than 2 times its occurrence in
the SABE Study (31.5%) and about 5 times its
occurrence in the elderly in the city of Fortaleza
(13.6%). The study confirms the need for careful
and judicious selection in prescription medications for this population.
AO-022
PREDICTORS OF HEMORRHAGIC
TRANSFORMATION IN PATIENTS TREATED
WITH INTRA-ARTERIAL THROMBOLYSIS AND
MECHANICAL THROMBECTOMY: A REAL WORLD
EXPERIENCE
Silva GS, Massaud RM, Vaccari AMH, Miranda RCAN,
Cendoroglo Neto M
• HIAE
Introduction: Hemorrhagic transformation
(HT) is a feared complication of intra-arterial
(IA) thrombolytic therapy in patients with acute
ischemic stroke (AIS). Predictors of HT in the era
of mechanical thrombectomy are not well established. The aim of our study was to evaluate
18
the frequency of HT and its predictors in a series of patients with AIS treated with either IA
thrombolysis and/or mechanical thrombectomy
outside a clinical trial. Methods: We evaluated a database of consecutive patients admitted
to a Brazilian tertiary hospital with AIS treated
with either IA thrombolysis and/or mechanical
thrombectomy from January 2009 to December
2013. Categorical comparisons were made by the
chi-square or Fisher exact test. We used logistic
regression analysis to investigate predictors of
any HT. Results: Forty-eight patients were treated. The median baseline National Institutes of
Health Stroke Scale score was 16 [11‐21], and
mean age was 68.8±16.5 years. A total of 33.3%
of the patients were treated with IA thrombolysis
and mechanical thrombectomy (solitaire and/
or penumbra devices used), 24.9% received only
IA thrombolysis, 22.9% only mechanical thrombectomy, 12.5% intravenous (IV) rtPA followed
by mechanical thrombectomy, 6.4% IV rtPA
followed by mechanical thrombectomy and IA
thrombolysis. Any HT occurred in 14 patients
(29.8%). The rate of any HT was similar in patients treated with pure IA thrombolysis, versus
combined IV-IA thrombolysis and mechanical
thrombectomy. Systolic blood pressure at admission (OR 1.01 [1.01‐1.08, p=0.02), ASPECTS
scores (OR 0.44 [0.23‐0.82, p=0.01), platelet count
(OR 0.98 [0.97‐0.99], history of diabetes (OR 4,5
[1.14‐17.73] and hyperthermia in the first 48
hours peri-procedure (OR 5.5 [1.1‐27.8], p=.03)
were univariate predictors of HT. On multivariate analysis, only the ASPECTS score remained
as an independent predictor of HT. Conclusions:
In this real world series of patients treated with
IA thrombolysis and/or mechanical thrombectomy the burden of ischemic lesion at admission
measured using the ASPECTS scores was the only
independent predictor of HT.
AO-023
RELEVANCE OF ENDOTHELIAL NITRIC OXIDE
SINTASE, ELASTIN, ENDOGLIN AND COLLAGEN
GENETIC VARIANTS IN FAMILIAL INTRACRANIAL
ANEURYSM
Gregório ML1, Neiva CM1, Pinhel MAS2, Nakazone MA3,
Madureira LS3, Lauletta LFM3, Santos MLT3,
Ferraz Filho JRL3, Souza DRS3, Tognola WA3
• 1UNIFRAN; 2USP; 3FAMERP
Background: Intracranial aneurysm (IA) risk
factors can be environmental like smoking and
alcoholism, and genetic like variants of endothelial nitric oxide sintase (eNOS), elastin (ELN),
endoglin (ENG) and collagen (COL). Objectives:
Analyze eNOS, ELN, ENG and COL polymorphisms, environmental risk factors and the association with IA. Methods: 836 individuals divided in 6 groups: G1- 40 (familial IA); G2- 176
(G1 family); G3 - 113 (sporadic IA); G4 - 277 (G3
family); G5 - 104 (controls); G6 - 126 (G5 family).
Polymorphisms analysis eNOS, ELN, ENG and
COL was done by PCR (polymerase chain reaction). Significance level P<0.05. Results - eNOS:
allele A more prevalent in G1 (0,93), G2 (0,83),
G3 (0,79), G4 (0,89) than G5 (0,61) and G6 (0,75;
P<0,0001). A/A genotype more frequent in G1
(86%); G2 (77%) G3 (79%) and G4 (78%), than
G5 (26%) and G6 (50%; P<0,0001). ELN: similarity between groups (P>0,05). ENG: allele Wt
more prevalent in G5 (0,81) than in G1 (0,61;
P=0,01); allele I more prevalent in G2 (0,30) than
G6 (0,19; P=0,003) and in G3 (0,34) than G5 (0,24;
P=0,027). Genotype -/Wt more frequent in G5
(89%) than G1 (69%; P=0,009) and in G2 (77%)
than G4 (88%; P=0,003). Genotype II prevailed
in G2 (22%), rather than G6 (8% P=0,001). COL:
C/C genotype more frequent in G2 (25%) than
G6 (13,2%) (P=0,016). C/G genotype more prevalent in G3 (69,6%) than G5 (49,1%) (P=0,003) and
G4 (67,9%) than G6 (44,9%) (P<0,0001). Smokers
and alcoholics more prevalent in G1 (79%; 40%,
respectively) and G3 (61%; 36%), than G5 (29%;
20%; P<0,05). Conclusions: eNOS, ENG and COL
genetic polymorphisms are associated with IA,
differentiating familial or sporadic IA and controls, as well as smoking and alcoholism.
AO-024
ROPE SCORE, PATENT FORAMEN OVALE AND THE
ETIOLOGY OF STROKE AMONG YOUNG BRAZILIAN
PATIENTS
Libardi MC, Fábio SRC, Camilo MR, Martins Filho RKV,
Rocha LJA, Santos RSA, Coletto FA, Barreira CMA,
Dias FA, Pinto PTC, Alves FFA, Pontes Neto OM
• FACULDADE DE MEDICINA DE RIBEIRÃO PRETO FMRP-USP
Introduction: The etiology of stroke in young patients may be a diagnostic challenge and many
cases remain classified as cryptogenic. Patent
foramen ovale (PFO) is present in 25% of the
general population. Among stroke patients with
PFO, paradoxical embolism is a possible etiology.
The “Risk of Paradoxical Embolism” (RoPe) trial
has developed a score to identify stroke-related
vs incidental PFO in cryptogenic strokes through
clinical variables. Nevertheless, the Rope score
has never been validated in a population of
young Brazilian patients with stroke. Objectives:
to study the clinical profile and the clinical utility of the RoPe score among young patients with
cryptogenic and non-cryptogenic stroke admitted to the emergency department of a public tertiary academic hospital in Brazil. Methods: Retrospective analyses of 71 young stroke patients
with 50 years-old or less admitted to our emergency unit over the period of one year. Patients
were blindly evaluated with a Transcranial Doppler Bubble Test (BT) to screen for PFO, which
was subsequently confirmed by Transesophageal
Echocardiography. According to the TOAST system, patients were classified as cryptogenic and
non-cryptogenic strokes. RoPe score was blindly
determined. A ROC curve was built and the area
under the curve (AUC) was used to estimate the
accuracy of the RoPe score for cryptogenic stroke
among patients with PFO. Results: In non-cryptogenic stroke group (n=40), the mean age was
37.9 years (SD:6,9), 57.5% were female, 52.5%
smoked and 42.5% had hypertension. According
to the TOAST classification: small vessels (15%),
large vessels (27.5%), embolic source (20%) and
75% other causes. We found 35% of patients with
PFO and 60% had superficial lesions. The RoPe
score median was 7.5 (QI 5‐8). On the cryptogenic stroke group (n=31), the mean age was 36
(SD: 8,6), 64.5% female, 38% smoked, 42% had
dyslipidemia and 29% hypertension. The BT was
positive in 61% (p=0.03) of patients. The RoPe
score median was 8 (QI 7‐9) (p=0.06) and its AUC
was non-significantly predictive for cryptogenic
etiology among patients with PFO (AUC: 0.64;
p=0.19). Conclusion: In a small sample of young
patients with stroke in Brazil, we found a high
prevalence of cerebrovascular risk factors. The
RoPe score was not accurate to predict cryptogenic stroke among young patients with PFO. Regional characteristics and epidemiologic profile
may have impact on the utility of the RoPe score
when applied to young stroke patients.
XXVI Congresso Brasileiro de Neurologia
AO-025
STROKE INCIDENCE FALL IN BRAZIL OVER 16
YEARS: HAEMORRHAGIC STROKE FASTER THAN
ISCHEMIC STROKE?
Cabral NL1, Longo A1, Moro CH1, Vivian 22, Garcia AC2,
Venancio VG2, Goncalves AR1
• 1UNIVERSIDADE REGIAO DE JOINVILLE; 2JOINVILLE
STROKE REGISTER
Background: From 1990 to 2010 a non-significant 6% increase in the incidence of first-ever
ischemic stroke (IS) and a significant 22% increase in the incidence of first-ever haemorrhagic stroke (HS) was reported in low and middle-income countries. In contrast, we previously
showed one-third fall in the incidence of first ever
stroke in Joinville, Brazil from 1995 to 2005‐6.
Now, we aim to expand these trends in all strokes
and between IS and HS until 2011. Methods: Using multiple overlapped sources, we ascertained
the changes in stroke incidence in 1995, 2005‐6
and 2010‐11, in Joinville, Brazil. The premorbid
medication in patients with first ever and recurrent strokes was ascertained in the last 6 years.
Results: We registered 320 first-ever strokes in
1995, 759 in 2010‐11 and 859 in 2010‐11. From
1995 to 2011, the age-adjusted incidence of
first-ever stroke fell by 33% (relative incidence
0.67, 95% CI, 0.61‐0.72) and in young people (≤44
years) fell by 39% (RI 0.61, 0.42 to 0.84). In the last
six years, HS incidence decreased 53% (RI 0.47,
0.26 to 0.78). IS and subarachnoid haemorrhage
(SAH) incidences did not change significantly.
Obesity, use of drugs for diabetes and dyslipidemia has doubled in patients under primary and
secondary prevention in the last six years. Use of
drugs for hypertension, antiplatelets, anticoagulants and active smoking didn´t changed significantly in patients under primary prevention
and secondary prevention. Conclusions: Stroke
incidence has decreasing in Joinville been over
the last 16 years. HS might be more responsible
for the rates fall than IS. We found an alarming
increase in obesity prevalence and in proportion
use of lower lipid and anti-diabetes drugs.
AO-026
THREE-YEAR SURVIVAL AND RECURRENCE AFTER
FIRST EVER STROKE: THE JOINVILLE STROKE
REGISTER
Cabral NL1, Muller M1, Franco SC1, Nagel V2, Longo A1,
Moro CH1, Costa G1, Gonçalves AR1
• 1UNIVERSIDADE REGIAO DE JOINVILLE; 2REGISTRO
DE AVC DE JOINVILLE
Background and Purpose: Despite continuous
mortality fall over the last three decades, stroke
remains the first cause of death in Brazil. We had
shown that incidence, case-fatality and mortality
have been decreasing over the last 18 years in Joinville, Southern of Brazil. We aim to determine the
recurrence and survival rates and cause of death
over 3 years after stroke in a population-based
setting. Methods: From Joinville Stroke Registry,
we ascertained all first ever stroke ocurred in Joinville, Brazil between 2009‐10. Multiple overlapping sources of information were used to ensure
completeness of case ascertainment. Patients
were followed-up prospectively at 1 month, 6,
months, 1 year, 2 years and 3 years after the index event. Results: We registered 406 first-ever
events who were followed-up at 3 years by which
time 148 (36%) had died. In the first year of stroke
the risk of death was 34.9% (95% CI, 29.6% to
40.43%). Beyond the first year, approximately
3 to 4% of survivors continued to die each year.
Over the follow-up, we registered 7.9% (32/406)
of stroke recurrence. Among IS, the proportion
of surviving free of recurrent stroke or death was
80.4% (74/92; 95% CI, 70.9‐88.0) in small artery
oclusion; 73% (8/11; 95% CI, 36.7‐93.9) in other
determined; 61.4% (35/57; 95% CI, 47.6‐74.0)
in large artery oclusion; 47.3% (43/91; 95% CI,
36.7‐58.0) in undetermined and 42% (36/84; 95%
CI, 32.1‐54.1) in cardioembolic. First stroke, recurrent stroke or miocardial infarction was the
most common causes of death in 30 days (96%)
and in 3 years (67%). Conclusions: In Joinville,
one third of patients died in 3 years after first
stroke. This finding is similar as other series done
one decades ago in high income countries. The
most common cause of death after a first stroke
is cardiovascular disease. Long-term survival after stroke may be improved by early, active, and
sustained implementation of effective strategies
for preventing subsequent cardiovascular events.
AO-027
THROMBOPHILIA SCREENING IN YOUNG ADULTS
WITH ISCHEMIC STROKE: A SYSTEMATIC REVIEW
OF LITERATURE AND INVESTIGATION PROPOSAL
BASED ON EVIDENCE
Monteiro JMC, Martin DLS, Rodrigues Neto VF,
Souza IFB, Carmo CO, Sousa TRM, Jesus PAP
• UFBA
Introduction: Ischemic stroke (IS) in young
adults (less than 45 years) is an important cause
of morbidity and usually presents an extensive
and expensive etiological evaluation. In some
of these individuals, an evaluation for prothrombotic states is usually performed to identify possible thrombophilias (Ts), taken as a risk
factor. However, evidence of the presence of this
condition and its possible causality with stroke
is contradictory between different studies. The
request of the tests should be performed focusing on current evidence, due to the high cost of
research, varying from R$910 ($406) to R$4,000
($1,786) among different state capital cities in
Brazil. This study aims to review the literature on
Ts as a risk factor for stroke, proposing a rational flowchart to request these examinations especially in Brazilian public health system (SUS).
Methods: Systematic review of the literature according to the PRISMA guidelines, performed in
Medline and CAPES Journals in the last 20 years.
The terms thrombophilia and stroke were used
in the search. Pediatric population was excluded. Results: 479 articles were found in Medline
and 1,081 in CAPES Journals and 62 articles were
selected, most of these case-control type. There
was high variation among the different conditions evaluated and important methodological
limitations. The most frequent Ts among cases,
after adjusting for the control groups, were antiphospholipid syndrome (APS), protein C and
S deficiency and hyperhomocysteinemia. The
methylenetetrahydrofolate reductase (MTHFR)
mutation was extremely common among several studies in both controls and cases. Factor V
Leiden mutation (FVL) also had high frequency
in the study controls, but associated with venous thrombosis. A mutation in the prothrombin gene, despite having been implicated as risk
factor for stroke in young adults, presents high
heterogeneity between studies. There are insufficient data on deficient anti-thrombin III and
stroke risk among different studies. Conclusion:
in young adults with IS, a directed research
should be done starting with intra-and extracranial vessels study and a cardiology research before deciding if order tests for Ts. In the absence
of clinical signs suggestive of a probable cause,
it is advisable to divide the screening into four
stages, 1º evaluation for APS; 2º protein C, S and
homocysteine; 3º resistance to activated protein
C or FVL and MTHFR mutation; 4º prothrombin
mutation and antithrombin III activity.
AO-028
WHAT DOES IT KILL MORE IN BAHIA SEMI ARID,
ACUTE MYOCARDIAL INFARCTION OR STROKE? AN
ECOLOGICAL AND ECONOMIC ANALYSIS
Santos AAO, Souza AS, Faria GAS, Cardoso E,
Nascimento MA
• UEFS
Introduction: The main cause of death and permanent disability in Brazil are cardiovascular
diseases. These diseases comprise a broad spectrum of clinical syndromes, such as cerebrovascular disease and acute myocardial infarction
(AMI) with high morbidity and impact on quality
of life and in the productivity of afected population. Therefore is important a proper training
of health professionals who treat these medical
conditions. Methodology: An ecological study
including hospitalized inpatients of both genders in the Cleriston Andrade General Hospital
(the largest reference hospital of the Bahia countryside) from January 2012 to December 2013.
The hospitalization inpatient data were provided from DATASUS and have included hospitalization, death, mortality, length of stay, gender,
age and average length of stay. Results: Of all the
hospitalizations, it was identified 757 cases of
AMI and 1506 of stroke. Of all the AMI patients,
a greater frequence of male was observed: 58%
(444), while between those with stroke, the variation between gender was more slight, with approximately 51% (768) of female. The age groups
with the highest frequence of hospitalizations
for stroke were 70‐79 years (312) and 80 and over
(302). For AMI were 60‐69 years (185), 50‐59 years
(170) and 70 to 79 years (170). The length of stay
for AMI were 7,270 with average stay of 9.6 and
AVE way to 14,619 and 9.0 days of stay and average length of stay, respectively. Concerning the
hospitalizations, 124 admitted with AMI have
died, conferring a mortality rate of 16.38, while
the number of death by stroke were 487, with
mortality rate of 32.45. The total amount spent
on AMI was R $713,462.41 and on stroke was R
$1,653,120.65 Conclusion: Results show a higher cost with stroke (the value is 131.70% more
than the AMI cost). In spite of the higher costs,
the number of days stayed, deaths and mortality
rates of stroke are greater than that of AMI. So, it
was identified an incompatibility among the cost
and effectiveness of treatment. For this reason it
is necessary improving the quality of patient care
through of creating measures of performance in
meeting these diseases and especially of stroke.
AO-029
YOUNG PATIENTS WITH HYPERTENSION IN
SECONDARY STROKE PREVENTION. A NEGLECT
AND A HIGH-RISK GROUP?
Diegoli H1, Garcia VP1, Starling F2, Moro CHC2, Longo AL2,
Gonçalves AR2, Cabral N2
• 1HOSPITAL MUNICIPAL SÃO JOSÉ DE JOINVILLE;
2
UNIVILLE
Introduction: Hypertension is the highest population-attributable risk for stroke. Reducing
the prevalence of uncontrolled hypertension is
a worldwide challenge. Objectives: In a secondary prevention cohort of patients who survived
a stroke event in Joinville, Brazil, we aim to
19
Oral Presentation
evaluate which variables are related to uncontrolled hypertension and if it is related to death.
Methods: We extracted demographic, socio-economic and clinical data from patients in the Joinville Stroke Registry who survived 6 months after
a first-ever stroke from October 2009 to July 2012.
These patients were followed-up through regular
telephone calls for over 2 years, in which their
last blood pressure (BP) was asked. The exclusion
criteria were non-ischemic stroke, death within 6
months after admission, less than 45 years old,
and no phone contact in at least half of the attempts. We calculated the mean blood-pressure
(BP) reported in the phone calls, which was labeled uncontrolled if systolic >139 or diastolic
>89mmHg. We compared the number of deaths
in the follow-up between patients with controlled and uncontrolled hypertension. Results:
The cohort had 1130 patients in secondary prevention, where 255 (22,5%) had uncontrolled
hypertension and 59 (5.2%) didn´t inform their
BP in at least half of the interviews. Over the 2
years of follow up, 9.3% (76/816) patients with
controlled BP died whereas 10.2% (26/255) died
in the group of uncontrolled BP. Variables related to controlled BP were functional dependency
(mRankin 4‐5; relative risk [RR]: 0.55; 95% CI,
0.39 to 0.77 p<0,001) and age above 65 years (RR:
0.62, 0.50 to 0.78; p<0,001). In the multivariate
analysis, the uncontrolled BP group had higher mortality than the controlled group (hazard
ratio [HR]: 1.57, 1.00 to 2.47; p=0,047), and the
group who didn’t inform their BP had even higher mortality than the controlled group (HR: 4.63,
2.8 to 7.68; p<0,001). Conclusions: As expected,
uncontrolled hypertension is related to higher
mortality rates. Despite having a higher potential
benefit from BP control, younger and less dependent patients have higher prevalence of uncontrolled hypertension.
Other Motor Neurone Disease
AO-030
SPINAL CORD ATROPHY IN HEREDITARY SPASTIC
PARAPLEGIA CAUSED BY SPG4 MUTATIONS
Branco LMT, Lamas GM, Bergo FP, Lopes-Cendes I,
França Junior MC
• UNICAMP
Background: HSP is a very heterogeneous group
of neurodegenerative disorders. SPG4-HSP is
the most prevalent autosomal dominant HSP,
especially in adults. Its core features are spastic paraplegia and progressive course. Although
corticospinal tracts are known to be damaged
in SPG4-HSP, there are few MRI-based studies that specifically investigated the SC in the
disease. Objectives: To investigate spinal cord
(SC) atrophy in patients with Hereditary Spastic
Paraplegia caused by SPG4 mutations (SPG4HSP). Methods: Eleven patients with molecular
confirmation of SPG4-HSP and 22 age-andgender-matched healthy controls underwent
MRI on a 3T Achieva PHILIPS scanner. We used
T1-weighted 3D images covering the whole
brain and the cervical SC to estimate cervical SC
area and eccentricity at C2/C3 level based on a
semi-automatic image segmentation protocol
20
using a validated software (Spineseg). Acquisition parameters were: TE=3.2ms, TR=7.1ms, flip
angle=8o, voxel size=1.0 x 1.0 x 1.0 mm3 and FOV
=240x240. SC areas and eccentricity of patients
and controls were compared using Mann-Whitney test. P values <0.05 were considered significant. Results: Mean age of patients was 49 years
(range 15‐68) and there were 7 men. The two
groups were significantly different regarding SC
areas (67.5±7.8 mm2 vs 54.7±5.3 mm2, p<0.001).
However, eccentricity values were similar in
both groups (p=0.917). SC areas did not correlate
with age of the patients (p=0.336). Conclusions:
Patients with SPG4-HSP have SC atrophy, but
no flattening. Further studies are needed to determine the clinical relevance of these abnormalities. Supported by: Fundação de Amparo à
Pesquisa do Estado de São Paulo (FAPESP)
Muscle Disorders
AO-031
ANTILIPEMIC AGENTS AND CREATINE
PHOSPHOKINASE: EPIDEMIOLOGIC STUDY AND
CLINICAL ANATOMICAL ANALYSES OF 55 PATIENTS
Carvalho AAS, Delgado PO, Koch ME, Pagura JR, Feder D
• FACULDADE DE MEDICINA DO ABC
Introduction: Lipid-lowering drugs, such as simvastatin, rosuvastatin, atorvastatin calcium etc.,
have been occasionally associated with neuromuscular symptoms and morphological changes in the muscle biopsy. Material and methods:
We studied 453 muscle and nerve biopsies from
a private center from 2007 to 2014 may. We selected the muscle biopsies from patients with
hyperlipoproteinemia treated with lipid -lowering drugs (statins / fibrates) and we analyzed the
clinical, epidemiological and anatomical pathologic features of these patients. No patients had
neuromuscular disorder. Results: We selected
55 (12,1%) patients using antilipemic drugs. The
patients had myalgia and proximal muscle weakness and cramps or elevated creatine phosphokinase(CPK). The CPK remained elevated although
the drugs where withdrawn. The anatomical
pathologic features found were: (1)variation in
fibers diameters, (2) necrosis of fibers, (3) inflammatory infiltration, (4)the presence of vacuolated fiber (5) ragged-red fibers (6) COX negative
fibers. Conclusion: The long-term use of statins
± fibrates can induce a chronic myopathy even
in the absence of symptoms , although the muscle biopsy findings were nonspecific. Keywords:
Statins , myopathy and muscle biopsy.
AO-033
STUDY OF A COHORT OF BRAZILIAN
CENTRONUCLEAR MYOPATHY PATIENTS
Abath Neto OL1, Martins CA1, Carvalho M1, Oliveira ASB2,
Reed UC1, Bönnemann C3, Laporte J4, Zanoteli E1
• 1FMUSP; 2UNIFESP; 3NIH; 4IGBMC
Introduction: Centronuclear myopathy (CNM)
is a rare clinically heterogeneous congenital
myopathy characterized by a prominence of
centralized nuclei in the muscle biopsy. So far,
7 genes have been associated to different forms
of the disease: MTM1, DNM2, BIN1, RYR1, TTN,
CCDC78 and SPEG. Objectives: To establish the
molecular diagnosis and correlate clinical, histological and genetic features in a cohort of Brazilian patients with CNM. Methods: We identified
muscle biopsy reports taken from two large biopsy banks of Sao Paulo, Brazil, in the last ten years.
We then recruited families for clinical assessment and drawing of blood. For the molecular
studies, we used a sequential approach involving
Sanger sequencing, Multiplex Ligation-dependent Probe Amplification (MLPA), X inactivation
studies, a gene panel for neuromuscular diseases, and exome sequencing. Results: A total of
24 patients from 21 families were recruited. We
established a molecular diagnosis in 16 families.
In 6 out of 7 families with boys affected by the
severe X-linked neonatal form, which constituted a clinically and histologically homogeneous
group, we found known and novel mutations in
the MTM1 gene. This gene was also implicated
in a milder manifesting carrier girl, in which a
heterozygous macrodeletion was detected using
MLPA. The girl was found not to have skewed X
chromosome inactivation. Two sporadic families presenting the “spokes of wheel” histological
marker showed mutations in the DNM2 gene,
one of which had never been described. The
RYR1 gene was the culprit in 7 families, all of
which had compound heterozygous mutations,
most in the form of a severe null mutation associated with a milder pathogenic missense, distributed throughout the length of the gene. This
clinically heterogeneous group of patients had in
common the finding of focal disruptions in the
intermyofibrillar architecture of muscle fibers.
The TTN gene is probably implicated in one sporadic family with a single affected boy harboring
a compound heterozygous null mutation. In this
Brazilian cohort, we did not find mutations in the
rarely affected genes BIN1, CCDC78 and SPEG.
Ongoing studies in the remaining families seek
to find potential novel genes implicated in CNM.
Conclusions: Features of Brazilian CNM patients
follow findings described in the literature. Mutation distribution in our cohort suggests RYR1 is a
frequently involved gene, while DNM2 is not as
recurring as in other works. (CAPES 1286/51‐2)
Transcranial Doppler
AO-034
TRANSCRANIAL DOPPLER ASSESSMENT AND
CLINICAL OUTCOME FOLLOWING PERCUTANEOUS
CLOSURE OF PATENT FORAMEN OVALE
IN PATIENTS WITH PREVIOUS ISCHEMIC
NEUROLOGICAL EVENT
Richartz M, Rizelio V, Leal AG, Selig FA, Balbi Filho EM,
Kowacs PA
• INC
Introduction: Right-to-left shunt due to patent
foraman ovale (PFO) is associated with ischemic
neurological events, especially in young individuals. Transcranial Doppler (TCD) with injection
of gaseous contrast is a useful technique to identify this type of shunt (paradoxical embolism).
In this context it is important to determine if
XXVI Congresso Brasileiro de Neurologia
residual shunt after PFO closure represents a risk
factor for adverse clinical outcome. Objective:
this study aimed to compare the results of TCD
after percutaneous closure of PFO with clinical
outcomes in patients with history of ischemic
neurological event. Method: a retrospective
study was conducted in a Transcranial Doppler
Service. Inclusion criteria were: patients with
history of neurological ischemic event (stroke or
transient ischemic attack), PFO closed percutaneously and TCD performed before (TCD1) and
after (TCD2) the closure procedure. The primary
outcome was recurrence of ischemic stroke or
TIA after PFO closure. The analyzed variables
were residual shunt (identified by the presence
of paradoxical embolism in TCD2) and thrombophilia. Results: the sample was constituted of 23
patients (mean age 39.6 years; 56.5% female). All
of them had positive result for paradoxical embolism in TCD1. The mean time between PFO
closure and TCD2 was 12.57 months (SD 4.49).
In TCD 2, 7 patients (30.4%) presented paradoxical embolism (57.1% with isolated microemboli
and 42.9% with shower pattern in Valsalva maneuver). The mean time of follow-up after the
closure was 16.57 months (SD 8.15). Two participants (8.69%) presented an ischemic neurological event during the follow-up, both with negative results in DTC2. No statistical significant
difference was found when the groups with positive and negative results in TCD2 were compared
in relation to the primary outcome. In contrast,
patients with thrombophilia (n=8) had more adverse outcomes than the ones without the condition (p<0.05, x2 =4.107). Conclusion: No relation
was found between presence of paradoxical embolism in DTC performed after PFO closure and
clinical outcomes (ischemic stroke or TIA). On
the other hand, thrombophilia was a risk factor
for recurrence of neurological ischemic events in
our sample.
Epilepsy
common causes of human focal epilepsy may
occur. Methods: Analysis of a series of 191 consecutive patients surgically treated for MTLE-HS
at CIREP (Center for Epilepsy Surgery at Ribeirao
Preto) from 1995 to 2000. The variables studied
included demographic, clinical neuroimaging
and surgical data. Binary logistic regression using SPSS Statistics v.17.0 established independence of variables and odds-ratio adjustments.
Results: Seventy-one patients (37.2%) presented chronic findings of NCC (cNCC). MTLE-HS
plus cNCC was significantly more common in
women (O.R.=2.45; 95%CI=1.30‐4.60; p=0.005),
in patients with no history of classical forms of
IPI (O.R.=2.67; 95%CI=1.37‐5.18; p=0.004), and in
those with bi-temporal interictal spikes on video-EEG (O.R.=2.00; 95%CI=1.07‐3.73; p=0.03).
Single cNCC lesions were observed to occur more
often on the same side as hippocampal sclerosis, a finding perhaps suggesting an anatomical relationship between NCC and MTLE-HS.
Conclusions: We suggest that NCC contributes
to or causes MTLE-HS in some patients. Based
on our findings, we propose two distinct, non-excluding, and potentially synergistic mechanisms
involved in the development of MTLE-HS in
NCC, one of them being inflammatory-mediated, while the other is electrogenic-mediated. Our
observations may improve the current knowledge about the pathogenesis of MTLE-HS and
about epilepsy associated with NCC. In our view,
these aspects are important because they open
new opportunities for epilepsy research and may
contribute to further elucidating the pathogenesis of epilepsy worldwide. This work was supported by CNPq, FAPESP and FAPERGS.
AO-036
CONTRALATERAL INTERICTAL AND ICTAL EEG
DISCHARGES ARE ASSOCIATED WITH LESS
EXTENSIVE WHITE MATTER TRACT INVOLVEMENT
IN UNILATERAL MTS
Coelho AQ, Yamaki ML, Passarelli V, Lima Filho HC,
Lyra K, Chaim KT, Otaduy CG, Leite CC, Jorge CL,
Valerio RM, Martucci CH, Ramos GE, Castro LHM
• FMUSP
AO-035
CHARACTERISTICS OF MESIAL TEMPORAL LOBE
EPILEPSY ASSOCIATED WITH HIPPOCAMPAL
SCLEROSIS PLUS NEUROCYSTICERCOSIS
Bianchin MM1, Velasco TR2, Wichert-Ana L2,
Alexandre Junior V2, Araujo Junior D2, Santos AC2,
Carlotti Junior CG2, Takayanagui OM2, Sakamoto AC2
• 1UFRGS; 2USPRP
Introduction: Neurocysticercosis (NCC) is
caused by Taenia solium, an infestation which
is endemic in Latin America, Asia, India, and Africa, and affects millions of individuals. It is one
of the most common causes of CNS infection
and acquired epilepsy worldwide. In developed
countries, NCC is less common but is still a public health problem. Recent observations suggest
that NCC might act as an initial precipitating injury (IPI), causing mesial temporal lobe epilepsy
associated with hippocampal sclerosis (MTLEHS) in some patients. Objectives: We evaluated
differences in demographic, clinical neuroimaging and surgical outcome of 191 consecutive
MTLE-HS patients in order to better understand how an interplay between two of the most
Introduction: Magnetic resonance imaging (MRI)
studies of temporal lobe epilepsy (TLE) with Diffusion Tensor Imaging (DTI) technique evidenced
white matter tract abnormalities that extend beyond the temporal lobe. It it is unknown if inter
or ictal epileptiform discharges contralateral to
the structural lesion are associated with contralateral tract abnormalities. Objective: To evaluate
the relation between contralateral epileptiform
discharges and tract integrity in ipsi and contralateral hemispheres in patients with unilateral
hippocampal sclerosis. Methods: A consecutive
series of patients with TLE associated with unilateral mesial temporal sclerosis (MTS) diagnosis that
underwent an MRI protocol including DTI, who
were studied with videoelectroencephalographic
monitoring. Patients were classified according to
ictal and interictal EEG findings as Concordant
(Co) – more than 70% interictal discharges and
ictal EEG activity restricted to the MTS side or Discordant (Di) – less than 70% interictal discharges
or independent ictal involvement in the contralateral side. MRI DTI data were analyzed with
tract based spatial statistics (TBSS). We compared
fractionated anisotropy (FA) of 20 white matter
tracts of right(R) and left(L), C and D groups with
controls using 2 sample T student test with p<0.05.
Results and conclusion: We studied 38 patients: 21
L (47% Co) 17 R (59% Co) and 25 healthy controls.
Groups did not differ in relation to age (mean 33
years), gender (54% woman) and epilepsy duration (mean 21 years). Co LMTS patients disclosed
20 involved tracts, while Co R MTS disclosed 12 involved tracts compared to control. Both L and R Co
groups disclosed more ipsi and contralateral tract
involvement than L and R Di groups. Conclusion:
We found extensive white matter tract involvement within and outside the temporal lobe in L
and R MTS patients. WMT involvement was more
pronounced and bilateral in L compared to R MTS
patients. Surprisingly, Co groups showed more extensive and bilateral WMT involvement than Di,
compared to C. The relation between contralateral
epileptiform activity and less pronounced WMT
involvement is unclear. We hypothesize that more
extensive disease may restrict epileptiform activity
within the ipsilateral hemisphere.
AO-037
DO CONTRALATERAL EEG ABNORMALITIES
NEGATIVELY IMPACT ON IPSI AND
CONTRALATERAL BRAIN VOLUMES IN UNILATERAL
MESIAL TEMPORAL SCLEROSIS (MTS) PATIENTS? A
WHOLE BRAIN VOLUMETRIC STUDY IN RIGHT AND
LEFT MTS PATIENTS
Yamaki ML, Coelho AQ, Passarelli V, Lima Filho HC,
Lyra KP, Chaim KT, Leite CC, Otaduy MCG, Jorge CL,
Valério RM, Martucci CH, Ramos GE, Castro LHM
• FMUSP
Seizure onset and Interictal epileptiform discharges may occur ipsi or contralaterally to the
lesion in Mesial Temporal Sclerosis (MTS) associated epilepsy. Seizures may also initiate on the
MTS side and propagate to involve contralateral mesial temporal structures. It is not known if
contralateral EEG abnormalities are associated
with structural lesion severity in the contralateral temporal and extratemporal structures.
Objectives: To investigate if contralateral ictal
and interictal EEG abnormalities are associated with severity of volumetric changes in both
hemispheres in unilateral MTS associated epilepsy patients. Methods: A consecutive series of
TLE patients with uMTS underwent video-EEG
monitoring with registration of at least three
seizures with an EEG correlate. Patients also underwent a volumetric 3T MRI study, with whole
brain volumetry processed with FreeSurfer software. Corrected volumes (structure/whole intracranial volumes) were calculated for all structures. Patients were classified according to ictal
and interictal EEG findings as Concordant (Co)
– more than 70% interictal discharges and ictal
EEG activity restricted to the MTS side or Discordant (Di) – less than 70% interictal discharges or
independent ictal involvement in the contralateral side. We compared CV for all structures for
Co and Di L and R MTS groups and C, using 2
sample t-student test, with a p<0.05. Results:
We studied 38 patients: 21 left (47% Co) 17 right
(59% Co) and 25 healthy controls. Groups did not
differ in relation to age (mean 33,1), gender (46%
men) education years (mean 11,3) and epilepsy
duration (mean 21). As expected, ipsilateral hippocampal volumes were significantly decreased
compared to C. L thalamic volumes were decreased in the L Co and Di MTS patients compared to controls. III ventricle volumes were increased in L Di compared to C (inferring thalamic atrophy). We also found decreased right and
left midtemporal volumes in R Di, as well as increased R inferior lateral ventricle volume in R Di
MTS patients compared to C. Corpus Callosum
volumes were consistently found to be decreased
in all groups, specially in the midanterior, central
and midposterior regions. Changes were more
21
Oral Presentation
pronounced and extensive in the L MTS and in
the Co groups. Conclusion: Contralateral interictal and ictal activity did not negatively impact on
ipsi or contralateral volumes in uMTS patients.
Corpus callosum volumetric changes appear to
be more widespread in the Co groups.
AO-038
ELECTROCARDIOGRAPHIC FEATURES AND SUDEP
RISK IN PATIENTS WITH EPILEPSY: A CASECONTROL STUDY
Sousa JMB, Fialho GL, Lin K
• UFSC
Objective: Sudden death in epilepsy (SUDEP) is
a major cause of mortality in young patients with
epilepsy (PWE). Although their mechanisms are
still poorly understood, they may be related to respiratory ± cardiac dysfunction. We investigated
interictal 12-lead electrocardiogram (EKG) abnormalities in PWE, in comparison with healthy
controls. Methods: Standard 12-leads EKGs were
obtained from all participants: 62 PWE diagnosed according to ILAE‘s criteria, followed in
Eplepsy’s Clinic in HU-UFSC, without seizures
in the date of EKG, and 57 controls matched for
sex, age and body mass index (BMI). We used
Burdick/Spacelabs carts, at a paper speed of
25mm/s and at 1 mV/cm. All EKG were manually assessed by a blinded cardiologist, member of the Brazilian Cardiac Society, according
to the AHA/ACCF/HRS Recommendations for
Standardization and Interpretation of the EKG.
The QT interval was corrected by Bazett‘s and
Framingham‘s methods. Results: 85.4% patients
were diagnosed with symptomatic focal epilepsy, of which 56.4% had temporal lobe epilepsy.
Patients had a mean of 12.30±15.64 years of epilepsy. We observed longer P wave (p=0.0001) and
PR interval (p=0.02) in patients than in controls.
Additionally, we observed longer QT intervals
(average and corrected) among patients than in
controls (p≤0.01) and pathologic QT dispersion
was also observed predominantly among PWE
(p<0.001). Early repolarization was seen in 3.5%
of controls and 10.5% of patients (p=0.27). Likewise, changes in ventricular repolarization was
more frequent among PWE. Conclusion: The
electrocardiographic profile of PWE is not the
same as the general population. Findings such
as pathologic QT dispersion, longer Qtc interval
and PR interval could be a subtrate for SUDEP.
AO-039
ETOMIDATE WADA TEST IS USEFUL TO PREDICT
SEIZURE OUTCOME AFTER ANTERIOR TEMPORAL
LOBECTOMY
Pinto LF, Passarelli VO, Jorge CL, Valerio RMF, Tzu WH,
Puglia Junior P, Castro LHM
• HOSPITAL DAS CLINICAS DA FACULDADE DE
MEDICINA DA UNIVERSIDADE DE SÃO PAULO
Objective: To study the usefulness of the etomidate Wada test to predict seizure outcome following anterior temporal lobectomy (ATL) in unilateral mesial temporal sclerosis (MTS). Methods:
This prospective study was part of a research
program approved by the ethics Committee of
our institution. Patients were selected in our epilepsy service between 2009 and 2013, and included in the study if they met the following criteria:
1) Drug resistant epilepsy as defined by ILAE
criteria; 2) Unilateral MTS (no dual pathology)
determined by 3.0 Tesla MRI ; 3) 18 years or older.
4), eight or more education years. A twelve-item
recognition paradigm was used for Etomidate
22
Wada memory testing. Memory reserve (MemRes) was defined by the number of recalled items
(spontaneously or with multiple choice) after
ipsilateral injection (to the lesion). Memory capacity (MemCap) was defined as the number of
recalled items (spontaneous or cued) after contralateral injection. Wada memory asymmetry
(MemAsy) score was calculated for each patient
subtracting MemRes from MemCap. No patient
was denied surgery based on Wada results. Receiver operator characteristic curves and predictive values of Wada memory scores to determine
seizure-free outcome (one year follow-up) were
calculated. Results: 47 patients (28, 59.5% leftMTS; mean age: 33.9±8.7 years) formed the study
group. 42 (89.3%) patients were seizure free after
one year. Areas under the curves (AUC) for Memcap (0.77±0.09; p=0.006) and MemAsy (0.74±0.07;
p=0.004) were significant to predict seizure-free
outcome, indicating its usefulness in individual
patients. AUC for MemRes was similar to chance
(0.51, p: 0.9). Memcap (cut off:≤9) and MemAsy
(cut off:≥2) were associated with high positive
predictive (PPV) and low negative predictive values (NPV) for one year seizure-free status (MemCap: PPV 96.7% 95 CI 82.8‐99.9; NPV 20.8% 95 CI
7.2‐42.2; MemAsym: PPV 100% 95 CI 85.2‐100;
NPV: 23.5% 95 CI 6.9‐49.9). Conclusions: Etomidate Wada test is useful to predict seizure outcome following ATL in unilateral MTS.
AO-040
PROSPECTIVE MEMORY AFTER TEMPORAL LOBE
EPILEPSY SURGERY
Adda CC, Santos APP, Jorge CL, Valerio RMF, ValottaSilva A, Tzu WH, Castro LHM
• FMUSP
Introduction: Prospective memory (PM) refers
to a set of cognitive abilities that allow recall of
a previous intention to perform in the future, in
the appropriate setting. This function is not usually evaluated in neuropsychological batteries
used to evaluate people with epilepsy. Objective:
We evaluated the impact of epilepsy surgery on
the prospective component of PM, in people
undergoing unilateral temporal lobectomy to
treat medically refractory epilepsy. Methods: We
compared performance in PM in people with
left or right mesial temporal sclerosis (MTS) in
the pre and postoperative periods with that of
two control groups. One group was composed of
people without epilepsy (normal controls), and
another group was composed of people with epilepsy associated with mesial temporal sclerosis
that underwent neuropsychological testing and
retesting without undergoing surgery (clinical
test/retest control group). Results: We studied
42 people without epilepsy, 20 clinical controls
(test/retest group), and 39 patients that underwent epilepsy surgery (pre and postoperative
testing). We compared groups performances
and changes in individual performances with
the reliable change index. Groups did not differ
in age, education, and intelligence quotient. We
found decreased preoperative PM performance
for the clinical and surgical groups (p<0.01) (lesion effect), without a difference between right
and left groups (laterality effect). Postoperatively,
there was a significant (p<0.05) decrease in number of seizures, a small, but significant reduction
in antiepileptic drug load, stable prospective
memory, verbal and visual memory for right
mesial temporal sclerosis, and decreased verbal
memory in the left mesial temporal sclerosis
group. Conclusion: In spite of a shared evocation
system for episodic and prospective memory,
resection of pathologically involved mesial temporal structures does not impact on prospective
memory performance, even in the setting verbal memory decline in the left MTS group. The
finding of dissociated verbal memory decline
and PM stability after epilepsy surgery suggests a
different role of mesial temporal lobe structures
in these memory systems. The role of extratemporal and nonmesial temporal lobe structures in
prospective memory in MTS patients should be
evaluated in future studies.
AO-041
USE OF FOLIC ACID IN WOMEN WITH EPILEPSY:
POSSIBLE PREDICTORS OF ADHERENCE IN AN
EPILEPSY SERVICE
Angst DBM1, Figueiredo NSV1, Baldocchi MA2,
Rocha MSG3, Passarelli V4
• 1RESIDENT IN NEUROLOGY AT SANTA MARCELINA
HOSPITAL; 2EPILEPSY SECTOR‘S PRECEPTOR AT
NEUROLOGY SERVICE FROM SANTA MARCELINA
HOSPITAL; 3CHIEF AT NEUROLOGY SERVICE FROM
SANTA MARCELINA HOSPITAL; 4EPILEPSY SECTOR‘S
PRECEPTOR AT NEUROLOGY SERVICE FROM SANTA
MARCELINA HOSPITAL
Introduction: Antiepileptic drugs (AEDs) interfere in folate metabolism and are one of the
most frequent teratogenic exposures in pregnant
women. Supplementation with folic acid (FA) is
related to lower rates of fetal malformations and
recommended by neurological societies in all
women with epilepsy (WWE) on childbearing
age. Objectives: Determine the percentage of
WWE taking FA in an outpatient epilepsy tertiary hospital at São Paulo, Brazil, and try to identify possible predictors of adherence. Methods:
576 medical records of patients followed in our
service were reviewed. 66 WWE in use of AEDs,
with age between 16 and 52, but not in the menopausal period, sexually active, not pregnant and
not sterilized and/or with vasectomized partners
were selected. They underwent direct interviews
using a pre-structured questionnaire. The groups
were compared according to: 1) age (above vs.
below 35 years), 2) education (up to 10 years
versus greater than 10), 3) category of presented epilepsy (partial vs. generalized), 4) absolute
number of AEDs currently in use (monotherapy
vs. polytherapy), 5) the used AEDs (inducing vs.
non-inducing cytochrome P450, and, further,
the use or not of VPA specifically), 6) control of
seizures (seizures occurring in last 6 months or
not), 7) use of contraceptive method (concurrent or non-use of hormonal vs. non-hormonal
method). Statistical analysis was performed using Fisher‘s exact test and statistical significance
was accepted when p<0.05. Results: In this study,
we found a reduced percentage (42.40%) of supplement intake. The adherence was significantly
higher in patients with generalized epilepsy than
in patients with focal seizures (p=0.03). Another
significant association was between adherence
to FA supplementation and use of non-hormonal birth control method (p=0.03). It was additionally performed another statistical analysis in two
separately groups (≤35y and >35y). The analysis
significantly demonstrated a greater FA intake
in people above 35 years who have at least 10
years education level, p=0.04. The others characteristics surveyed did not show statistical significance. Conclusion: The preconception care
of this population should be more specific and
involve a more effective supplementation of FA.
In addition, health care professionals should give
information to WWE about all delicate and intricate aspects involved in this pathological condition to improve WWE’s quality of life.
XXVI Congresso Brasileiro de Neurologia
Amyotrophic Lateral Sclerosis
AO-042
BRAZILIAN EXPERIENCE WITH DIAPHRAGM
PACING SYSTEM IN ALS PATIENTS
Rotta FT, Gurski RR
• HMV
Introduction: Diaphragm pacing is a technic
that stimulates electrically the muscle motor
point and has been used in amyotrophic lateral sclerosis (ALS) patients since 2006, with the
objective of preventing atrophy and ventilatory
insufficiency. Objectives: Report the Brazilian
experience with ALS patients using diaphragm
pacing. Methodos: Patients with diagnosis of
ALS (probable or definite according to El Escorial
revised criteria) were evaluated clinically, neurophysiologically and had pulmonary function
tests performed prior to intervention. Diaphragm
pacing system (DPS) was implanted through
laparoscopic surgery, under general anesthesia.
One patient had his procedure performed in the
United States (Case Western University Hospital
- Cleaveland, OH) and the remaining at Hospital Moinhos de Vento (Porto Alegre, RS). Results:
From October 2010, nine ALS Brazilian patients
were submitted to DPS implantation. This group
consists of 6 men and 3 women, 32 to 74 years
old at time of implantation. Forced vital capacities ranged from 42% to 60% of predicted and
clinical involvement was either of mixed upper
and lower motor neuron or predominantly upper
motor neuron type. All patients had detectable
compound muscle action potential on phrenic
nerve conduction studies, except for one who
had clear diaphragm voluntary activity clinically
and was not submitted to this test. All patients
had visible muscle contraction on transoperatory motor point stimulation. There were no significant surgical complications and all patients
tolerated stimulation sessions. Two patients died
since receiving the DPS (26 and 41 months later),
one because of disease progression and the other
secondary to complications from colon cancer
surgery. Conclusion: DPS implantation was safe
and well tolerated in this group of patients.
AO-043
CORTICOSPINAL TRACTOGRAPHY IN PATIENTS
WITH AMYOTROPHIC LATERAL SCLEROSIS (ALS)
Albuquerque M, Andrade HMT, Campos BM,
França Junior MC, Nucci A
• UNICAMP
Introduction: Amyotrophic Lateral Sclerosis
(ALS) is the prototypical motor neuron disease,
but usual MRI sequences often fail to detect upper motor neuron compromise. Diffusion-tensor
imaging is a novel technique that enables the
evaluation of white matter tracts in the brain,
and a very promising tool to investigate neurodegenerative disorders. Objective: To evaluate
the usefulness of corticospinal tractography as
neuroimaging marker in ALS. Methodology: Diffusion tensor images (DTI) were acquired on a
3T MRI (Philips Achieva) of 46 ALS patients (27
men, mean age 53.8±10) and 48 healthy controls
(32 men, mean age 53.0±11). Disease severity
was quantified with the ALS functional rating
scale (ALSFRSs) and ALS severity scale (ALSSS).
Tractography of the corticospinal tracts was performed through a semiautomatic deterministic
approach using the ExploreDTI software. Fractional anisotropy (FA), mean diffusivity (MD),
radial diffusivity (RD) and the number of tracts
were obtained for each side. Patients and controls were compared through parametric tests
using age as a covariate. Correlation between
DTI-based parameters and ALSFRs was investigated through Spearman coefficient. Results:
Patients had significantly smaller FA values
than controls (0.563 x 0.599, p<0.0001. MD and
RD were also higher in the ALS group (0.00076
x 0.00073, p<0.0001 and 0.00049 x 0.00045
p<0.0001, respectively). In addition, the number
of fibers was significantly different in both groups
(119.41 x 218.22, p<0.0001). Corticospinal FA did
correlate with ALSS scale (ρ=0.413). Conclusion:
Corticospinal tractography is able to detect and
quantify upper motor neuron damage in ALS. It
correlates with clinical parameters, thus suggesting it might be a reliable neuroimaging marker
for this disease.
AO-044
EXPRESSION PROFILE OF MICRORNAS IN
THE SKELETAL MUSCLE OF PATIENTS WITH
AMYOTROPHIC LATERAL SCLEROSIS (ALS)
Andrade HMT, Albuquerque M, Avansini SH, Dogini DB,
Rocha CS, Nucci A, Lopes-Cendes I, França Junior MC
• UNICAMP
Introduction: ALS is a rapidly progressive neurodegenerative disease. Diagnosis and prognosis
estimation are often delayed because there are
no reliable biomarkers. In this scenario, microRNAs may be good candidates because they are
released into the circulation by pathologically
affected tissues and display remarkable stability
in body fluids. Objectives: to identify microRNAs
differentially expressed in the skeletal muscle of
ALS patients when compared to healthy controls.
Method: We perfomed biopsies of Biceps brachii
muscles from five patients with ALS (El Escorial
criteria) and five healthy controls. MicroRNAs
enriched fraction was isolated with miRNeasy
Kit (Qiagen). The expression profile of microRNAs was evaluated using the GeneChip miRNA
Arrays platform (Affymetrix). For background
correction, normalization and summarization of
data, we employed the RMA (Robust Multi-array
Average) algorithm. Differential expression of
microRNAs between patients and controls was
evaluated through RankProd with correction for
multiple comparisons (FDR p<0.05). The results
were validated by qPCR. Results: Our preliminary
analysis identified 11 differentially expressed microRNAs when patients and control group were
compared. Among them we observed a significant up regulation of hsa-miR‐424, hsa-miR‐503
and hsa-miR‐886‐3p (4x overexpression), hsamiR‐542‐5p and hsa-miR‐34a (3 x overexpression), hsa-miR‐886‐5p, hsa-miR‐146b‐5p, hsamiR‐504 and hsa-miR‐21 (2x overexpression),
hsa-miR‐214 and hsa-miR‐183 (1x overexpression). Hsa-miR424 and hsa-miR214 were validated by qPCR. Conclusion: We identified a set of
microRNAs overexpressed in the skeletal muscle
of ALS patients. These are promising candidate
biomarkers that need to be validated in larger
samples with available clinical data.
AO-045
QUANTITATIVE MRI OF THE SPINAL CORD IS
A RELIABLE MARKER TO MONITOR DISEASE
PROGRESSION IN ALS
Branco LMT, Albuquerque M, Andrade HMT, Nucci A,
Bergo FP, França Junior MC
• UNICAMP
Background: Biomarkers are urgently needed
to help in the diagnosis and long term care of
patients with ALS. Spinal cord (SC) damage
is a pathological hallmark of the disease, but
there are few studies that investigated quantitative MRI of the SC in a longitudinal setting in
ALS. Objectives: To evaluate whether SC area
declines over time in patients with ALS and to
determine the clinical correlates of such progressive atrophy. Methods: Forty-seven patients with ALS confirmed by El Escorial criteria were enrolled and underwent at least once
clinical evaluation and a MRI acquisition on a
3T Achieva PHILIPS scanner. Of these, 28 and
20 performed a second evaluation within 6 and
12 months of follow-up, respectively. We used
T1-weighted 3D images covering the whole
brain and the cervical SC to estimate area and
eccentricity (ECC) at C2/C3 level based on a
semi-automatic image segmentation protocol
using a validated software (Spineseg). Acquisition parameters were: TE=3.2ms, TR=7.1ms,
flip angle=8o, voxel size=1.0 x 1.0 x 1.0 mm3
and FOV =240x240. We determined ALSFRS-R
scale in each evaluation. Both imaging and
clinical parameters were compared at different time points using paired Student´s t test.
We also used Pearson coefficient with Bonferroni correction to investigate correlations
between percentage variation of clinical and
imaging parameters. P values <0.05 were considered significant. Results: Mean age of the
patients and disease duration at baseline were
52,4 years and 35,0 months, respectively. Mean
ALSFRS score at baseline was 30,3. SC areas
showed a significant decline both at 6 and 12
months of follow-up (63.2±9.2 mm2 vs 61.4±9.0
mm2, p=0.001 and 63.1±8.1 mm2 vs 60.0±8.4
mm2, p=0.001). Interestingly, percent change
of SC areas at 6 months and 12 months did correlate with clinical deterioration at the same
time points, expressed by change in ALSFRS
(r=0.539, p=0.004 and r=0.501, p=0.024, respectively). SC ECC did change at 12 months, but
not at 6 months (p=0.001 and 0.184, respectively). ECC change over 12 months did not
correlate with clinical parameters (p=0.099).
Conclusions: Patients with ALS have progressive SC atrophy and clinical worsening amenable to detection after 6 months of follow-up. Interestingly, both parameters appear decline in
parallel, thus suggesting that quantitative MRI
of the spinal cord is a reliable marker to monitor disease progression in ALS. Supported by:
Fundação de Amparo à Pesquisa do Estado de
São Paulo (FAPESP)
23
Oral Presentation
Neurologic Manifestations of
Systemic Diseases
AO-046
COGNITIVE IMPAIRMENT IN PATIENTS OF A FABRY
DISEASE’S FAMILY
Mendonça ACR1, Zenha EFG1, Carneiro MAD1,
Ataídes TL2, Veloso VSP2, DIniz DS1
• 1CRIEM; 2FM
Introduction: Inborn errors of metabolism lead
to a heterogenic group of rare genetic disorders,
Fabry disease being one of them. It is a lysosomal
adult disease related to X-linked and enzymatic
changes cause the accumulation of globotrialosilceramide (Gb‐3) inheritance. Its main manifestations are cutaneous, neurologic, cardiac and
renal. Objective: Because the neurological disorders, we evaluate possible cognitive impairment
in this group of patients. Case report: A six female patients’ family, was referred to the Neurology Service of the Hospital das Clinicas de Goiás
by the Nephrology Service of the same hospital.
Neurological examination and cognitive assessment was done in all of them. Methodology: The
patients underwent neurological examination
by a neurologist and for neuropsychologists
who applied the BRB-N battery that assesses
verbal memory (learning), visuospatial memory
(learning), attention, processing speed, working
memory and semantic verbal fluency. MSFC was
also applied to evaluate the agility of upper limb
motor function, walking speed and the attention,
processing speed and working memory. All procedures took place at the Centro de Referencia,
Investigação e Tratamento em Esclerose Múltipla
(CRIEM) / FM / HC / UFG for a visit of approximately 2 hours. Results: All patients showed cognitive impairment in at least two tests (33.4% at 2,
50% at 3 and 16.6% at 5). The worst performance
(100%) was the Paced Auditory Serial Addition
Test (PASAT) test that assesses attention, processing speed and working memory and better
performance on the Word List Generation (WLG)
(83.4%) which assesses verbal learning memory. Conclusion: According to our results we can
state that the patients of Fabry disease, even
if asymptomatic, early show cognitive impairments, confirmed by performance in the PASAT
(already validated for the Brazilian population).
The performance at Nine-Hole Peg Test (9HPT)
and Timed 25-Foot Walk, were relatively good.
Half of the subjects assessed by 9HPT and 66.6%
in the Timed 25-Foot Walk showed performance
above normal that the engine point of view, most
had no motor commitments.
AO-047
NEUROLOGICAL MANIFESTATION IN AUTOIMMUNE
RHEUMATIC DISEASES: A REVIEW OF 170 CASES
Toso FF, Dutra LA, Barsottini OGP
• UNIFESP-EPM
Introduction: The involvement of central nervous system (CNS) in autoimmune rheumatic
diseases (ARD) is frequent and increases mortality. Patients may present variable symptoms
such as psychosis, epilepsy, cognitive decline,
stroke and neuropathies. Objective: To describe
24
a series of ARD patients with neurological involvement from the General Neurology Division
at UNIFESP. Methods: 170 charts of patients
with systemic erythematosum lupus (SLE) and
other ARD were reviewed. Epidemiological data,
comorbidities, clinical and neurological manifestations, laboratory and imaging results were
compiled. Risk factors for SLE neuropsychiatric
manifestation were studied in logistic regression
model. Results: Mean age was 43,77±13,62 years
and 87,57% were female. Most common diseases were SLE (31,36%), Neuro-Behçet’s disease
(NBD) (11,83%), APS (8,28%) and sarcoidosis
(5,92%). Most common symptoms were stroke
(31,95%), headache (28,99%), seizures (27,22%),
mood disorders (27,08%), cranial neuropathy
(18,93%), cognitive decline (12,21%), meningitis
(11,83%), psychosis (10,06%), peripheral neuropathy (9,47%), movement disorders (7,10%), myelopathy (4,73%). Among patients with stroke the
diagnosis were: SLE, APS and NBD. In myelopathy we found 3 SLE, 2 NBD and 1 APS. Seizures
were found in 18 SLE, 4 APS and 6 NBD. In cranial neuropathy, 5 sarcoidosis, 3NBD and 1 SLE.
LES+APS patients received more steroids than
the other patients. Most SLE patients presented
seizures (39,22%), stroke (23,56%) and psychosis (23,46%). Patients with seizures were youger
(p=0,0012). We found no association between
SLE neurological manifestations and hypertension, diabetes, dislipidemia, age at neurological
manifestation, disease duration and APS overlap.
Conclusion: In our population most common
ARD with CNS involvement are SLE, APS, NBD
and sarcoidosis. The high prevalence of NBD
reflects a selection bias. Patients with stroke and
suspected ARD should be tested for APS and patients with cranial neuropathy should be tested
for other ARD besides SLE, especially sarcoidosis. We did not find risk factors for CNS involvement in SLE in this series, probably reflecting
the small number of patients enrolled. Although
CNS manifestations are variable in ARD, the neurological picture may guide the investigation toward diagnosis.
the Brazilian regions. Methods: This is a study
based on secondary data, with descriptive approach between the years of 2007 to 2012. The
information was provided by Diseases Information Notification System (SINAN) and obtained
from a query in the electronic database of the
SUS Informatics Department (DATASUS). Based
on these data, we calculated the standardized
incidence by age and the fatality rate of dengue
cases and its neurological complications. We performed a comparative analysis of morbidity and
mortality caused by neurological complications
associated with dengue in the country regions.
Results: Among the Brazilian regions, the North
has the highest incidence in the country of neurological manifestations in dengue patients: 20
cases/10.000 dengue patients, followed by the
Midwest (1.42), Northeast (1.18), South (0.83)
and Southeast (0.82). We performed a comparison by region between the lethality of patients
who had no neurological manifestation (L) and
lethality of dengue patients who had neurological manifestation (LN): North (L=0.06 =1.38%
and LN %), Northeast (L=0.08%; LN =23.7%),
Midwest (L=0.08%; LN =34.1%), Southeast
(L=12:06%, LN =31, 3%) and South (L=0.03%; LN
=44%). Conclusion: The North shows the highest
incidence among the regions in the country. In
all regions, the fatality rate of dengue patients
who had neurological manifestation was higher
than the fatality rate of those patients without
these complications. It is essential to emphasize
that the death risk of a patient with dengue neurological manifestations is greater than one who
has not. Given this, we must strengthen financial
and social incentives to campaigns against dengue and alert the public to the ascendancy of the
neurological complications associated with dengue, which can kill or promote severe sequelae.
Clinical Neurophysiology
Neuroepidemiology
AO-049
INDEX OF ALPHA/THETA RATIO OF THE
ELECTROENCEPHALOGRAM: A NEW MARKER FOR
ALZHEIMER’S DISEASE
AO-048
INCIDENCE EVALUATION OF THE NEUROLOGICAL
COMPLICATIONS ASSOCIATED WITH DENGUE IN
BRAZIL, 2007-2012
Lessa KP1, Vilasbôas ÍGM2, San Martín DL2,
Vasconcelos TCM2
• 1UNEB; 2UFBA
Introduction: Dengue is a viral disease transmitted by the Aedes aegypti mosquito, presenting serotypes 1 to 4. In the last decade, 700.000
cases in Brazil were reported per year and the
incidence took a rising trend. This disease may
have symptomatic characteristics or not, reaching neurological system and many others. Such
complications, considered as rare, like encephalitis and meningoencephalitis, have increased
in recent years, making it a challenge for clinical
practice. Objective: To analyze and compare the
spatial distribution and the incidence of neurological complications associated with dengue in
Schmidt MT1, Basile LFH2, Lopes HFS1, Baratho R3,
Demario JLC3, Jorge MS1, Nardi AE4, Ianof JN1, Nitrini R1,
Anghinah R1
• 1HOSPITAL DAS CLÍNICAS DA USP; 2HOSPITAL DAS
CLÍNICAS DA USP E UNIVERSIDADE METODISTA DE
SÃO PAULO; 3PONTIFÍCIA UNIVERSIDADE CATÓLICA
DE SÃO PAULO; 4UNIVERSIDADE FEDERAL DO RIO DE
JANEIRO
Introduction: The inclusion of clinical neurophysiology techniques, or more specifically,
electroencephalography, in diagnostic research
protocols for AD is wholly justified given EEG’s
wide availability, low cost and high sensitivity,
allowing serial exams and neurological evolution
follow-up to be performed. Objective: We evaluated quantitative EEG measures to determine
a screening index to discriminate Alzheimer’s
disease (AD) patients from normal individuals.
Methods: Two groups of individuals older than 50
years, comprising a control group of 132 normal
volunteers and a study group of 136 patients with
probable AD, were compared. EEG recordings
XXVI Congresso Brasileiro de Neurologia
were obtained from subjects in a wake state with
eyes closed at rest for 30 min. Logistic regression
analysis was conducted. Results: Spectral potentials of the alpha and theta bands were computed
for all electrodes and the alpha/theta ratio calculated. Logistic regression of alpha/theta of the
mean potential of the C3 and O1 electrodes was
carried out. A formula was calculated to aid the
diagnosis of AD yielding 76.4% sensitivity and
84.6% specificity for AD with an area under the
ROC curve of 0.92. Conclusion: Logistic regression of alpha/theta of the spectrum of the mean
potential of EEG represents a good marker discriminating AD patients from normal controls.
AO-050
LOCOMOTOR ARREST ELICITED BY SPINAL
ELECTROMAGNETIC STIMULATION IN AWAKE
RATS: A STUDY ON DOPAMINERGIC PATHWAYS
Sales PMG, Andrade LMS, Araújo DF, Rôla FH,
Gondim FAA
• UFC
Introduction: The repetitive ElectroMagnetic
Stimulation (rEMS) is an expanding research
field. To date, its applications focus over the stimulation of encephalic structures, known as transcranial stimulation. Few studies tried to explore
the effects of rEMS over the spinal cord, despite
strong evidence linking spinal cord modulation
and locomotor changes. Objective: The present
study aims to elucidate the behavioral changes
elicited by rEMS over the spinal cord of awake
rats and the roles of dopaminergic pathways
in these phaenomena. Methods: Thirty male
naïve Wistar rats were restrained in a non-ferromagnetic apparatus developed by our research
group. The experimental protocol was developed
in two days: Day 1 – exposure to an audio record
similar to the sound produced by the stimulation equipment; Day 2 – exposure to stimulation
trains over the spinal cord with a power equal to
2x the animal’s motor threshold in a frequency
of 20 Hz during 5 minutes. The exploratory behavior of the animals in a wooden box (100x50x50cm) was recorded for 10 minutes in both days
and behavioral parameters were quantified. The
animals were divided in 6 subgroups with 5 animals each: Lumbar rEMS, Thoracic-Lumbar
rEMS, Levodopa/Benserazide for 2 or 7 days,
Haloperidol 0.1 or 0.3 mg/kg. Differences among
subgroups were compared using 2-Way-ANOVA
(Day x Subgroup) with an alpha of 0.05. Results:
The rEMS over Lumbar and Thoracic-Lumbar regions reduced the spontaneous walking behavior
(p<0.01), but only Thoracic-Lumbar rEMS was
capable of increasing the time spent in the cage’s
corner (p<0.01). The treatment with Levodopa/
Benserazide significantly reduced the walking and climbing durations (p<0.001), but only
the 7-days treatment was capable of increasing
time spent on grooming and in the cage’s corner (p<0.05). The treatment with Haloperidol
significantly reduced the time spent in walking,
but only low-dose Haloperidol was capable of
reducing grooming (p<0.01). Conclusions: The
Thoracic-Lumbar rEMS significantly reduced
the exploratory behavior and increased the time
spent in the cage’s corner. These effects were
potentialized by an induced pro-dopaminergic
state, which was capable of provoking a new increase in the time spent on grooming after rEMS.
Further studies are necessary to elucidate the
mechanisms involved catalepsy and stereotypies
induced by rEMS.
AO-051
SLEEP SPINDLES IN OBSTRUCTIVE SLEEP APNEA:
A SUMMARY OF RESULTS
Neurogenetics
Schönwald SV1, Carvalho DZ1, Dellagustin G1, Lemke N2,
Santa-Helena EL3, Rybarczyk Filho JL2, Segal AZ4,
Gerhardt GJL5
• 1HOSPITAL DE CLÍNICAS DE PORTO ALEGRE;
UNIVERSIDADE ESTADUAL PAULISTA JULIO DE
2
MESQUITA FILHO; 3UNIVERSIDADE FEDERAL DE
SERGIPE; 4WEILL CORNELL MEDICAL COLLEGE, NY;
UNIVERSIDADE DE CAXIAS DO SUL
5
Sleep spindles are markers of thalamo-cortical
integrity. Obstructive sleep apnea (OSA) has an
impact on central nervous system structure and
function. Sleep spindle frequency-topography
characteristics were investigated in OSA, including internal frequency modulation. Previously,
we showed that spindles tend to decelerate before termination in healthy subjects (negative
chirp). Here we summarize our results from JNM
2011, 197:158–164; BMC Neuroscience 2012,
13:89; ClinN 2014, 125:306–312; and SCI 2014
(in press). Seven non-OSA subjects and 21 OSA
patients (11 mild and 10 moderate) contributed
with 30 min of non-REM sleep stage 2, subjected
to a Matching Pursuit procedure with and without Gabor chirplet functions for automatic sleep
spindle detection. As the night progressed, Moderate OSA patients showed higher proportions
of slow (<13Hz) spindles in frontal regions compared to Non-OSA subjects. Frontal slow spindle
percentage in night section III predicted OSA
with good accuracy - OSA likelihood increased by
12.1% for every slow spindle percentage unit increase (OR 1.121, 95% CI 1.013 - 1.239, p=0.027).
Central region spindle density (using a fixed 20%
top amplitude threshold) was similar among
groups. However, median voltage was lower in
moderate OSA [39.30 (12.56)μV] compared to
non-OSA [44.64 (19.15)μV] and mild OSA [43.69
(27.28)μV] (K −W=77.014; df 2; p<0.001), consistent with former reports of visually reduced spindle number in OSA. Further tests with fixed vs.
individualized amplitude thresholds favored an
adaptive amplitude criterion for spindle detection, reducing intersubject variability. Concerning internal frequency modulation, Moderate
OSA patients showed an inferior percentage of
negative chirp when compared to Mild and NonOSA groups in frontal and parietal regions, specially for slow spindles (rs = ‐0.519, p=0.005 for
percentage of slow spindles with negative chirp
on the parietal region). Frequency-topography
results are consistent with diffuse, predominantly frontal thalamo-cortical dysfunction during
sleep in OSA, while more posterior brain regions
appear to maintain some physiological spindle
frequency modulation across the night. Loss of
physiological sleep spindle deceleration may
either represent a disruption of thalamocortical
loops generating spindle oscillations or some
compensatory mechanism, an interesting venue for future research in the context of cognitive
dysfunction in OSA.
AO-052
BEHAVIORAL RESPONSES IN ANIMAL MODEL OF
CONGENITAL MUSCULAR DYSTROPHY 1D -LARGE
Soares JA1, Ventura L1, Freiberger V1, Cassol Junior OJ2,
Vainzof M3, Quevedo J2, Comim CM1
• 1UNISUL; 2UNESC; 3USP
Congenital Muscular Dystrophies 1D (CMD1D)
present mutated gene in the LARGE and it is
characterized by an abnormal glycosylation of
α-dystroglycan (α-DG), strongly implicated as
having a causative role in the development of
Central Nervous System abnormalities such as
cognitive impairment seen in patients. However, in the animal model of the pathophysiology
of CMD1D the behavior responses and the brain
involvement remains unclear. Therefore, the objective of this study is to evaluate the cognitive
involvement in the Largemyd mice. To this aim,
we used adult homozygous, heterozygous, and
wild-type mice. The mice separately underwent
six behavioral tasks: habituation to an open field,
step-down inhibitory avoidance, continuous
multiple trials step-down inhibitory avoidance
task, object recognition, elevated plus-maze and
forced swimming test. In summary, it was observed that Largemyd presented deficits on the
habituation to the open field, stepdown inhibitory avoidance, continuous multiple-trials stepdown inhibitory avoidance, object recognition
and forced swimming. This study shows the first
evidence that abnormal glycosylation of α-DG
may be affecting the memory storage and restoring process in animal model of CMD1D - Large.
AO-053
BILATERAL CAUDATE ATROPHY DISTINGUISHES
DYSTONIC VS NON-DYSTONIC PATIENTS WITH
MACHADO-JOSEPH DISEASE
Rezende TJR, Martinez ARM, Nunes MB, Guimaraes RP,
D‘Abreu A, Cendes IL, França Junior MC
• UNICAMP
Objective: to compare atrophy patterns of
MJD/SCA3 patients with (dMJD/SCA3) and
without (c MJD/SCA3) dystonia when compared to healthy controls using VBM analyses. Background: MJD is the most common
spinocerebellar ataxia and represents a neurodegenerative disease. On clinical grounds
it is marked by pleomorphic characteristics
including movement disorders. Dystonia is
frequent in MJD patients and little is known
about central nervous system patterns of involvement in this subgroup of patients. Design/methods: Patients were evaluated regarding demographic data (age, age at onset,
gender), SARA (Scale for the Assessment and
Rating of Ataxia) scores and (CAG) expansions. Matched age and sex healthy controls
were enrolled. The Marsden-Fahn rating scale
(MFR-S) was applied to quantify dystonia severity in dMJD/SCA3. Volumetric T1W images
were acquired on a 3T device using 1mm slices, TE=3.2ms, TR=7.1ms, flip angle 8°, isotropic voxels of 1mm3, FOV=240x240. We used
25
Oral Presentation
SPM software with VBM toolbox to assess
differences in gray matter volume between
controls and cMJD/ SCA3 and, also, between
controls and dMJD/ SCA3. We performed a
general linear model using age and gender as
covariates to assess the groups. We employed
a t-test to carry out the group analyses and
the final statistical maps for two groups were
overlaid on MNI152 template to compare
the different atrophy patterns between the
groups. Results: We included 33 cMJD/SCA3
(mean age 52.7±9.2y, 16 men) and 19 dMJD/
SCA3 (mean age 38.7±14.1y, 11 men). dMJD/
SCA3 patients had longer (CAG) expansions
(75±3.9 vs 70±2.9, p<0.001) and earlier onset (29.6±13.3 vs 42.8±9.2 years, p=0.001) in
comparison to patients in the cMJD/SCA3
group. Dystonic patients also had higher SARA
scores (18.2±6.9 vs 12.9±6.9, p=0.017). Ten out
of 19 dMJD/SCA3 had generalized dystonia,
2 blepharospasm and 7 focal limb dystonia;
mean MFR-S score was 16.5±12.6. VBM analyses demonstrated significant volumetric reduction for both groups in the cerebellum and
occipital lobes when compared to controls.
However, bilateral caudate nuclei and left precentral gyri atrophy were only found in the
dMJD/SCA3 group. Conclusions: Caudate nuclei and precentral damage underlie dystoina
in MJD/SCA3. These structures are potential
therapeutic targets to relieve dystonia through
neuromodulation.
AO-054
CEREBROSPINAL FLUID FLOW IMAGING BY
USING PHASE-CONTRAST MR TECHNIQUE IN
MUCOPOLYSACCHARIDOSIS
Corte AD1, Souza CFM2, Vairo F2, Anés M2, Vedolin LM2,
Ferreira MM2, Aguzzoli AAG2, Perrone SGP2,
Federhen A2, Giugliani R2
• 1UFRGS; 2HCPA
Introduction: Cerebrospinal fluid (CSF) flow
study is a noninvasive imaging technique that is
useful for the evaluation of patients with hydrocephalus, which is a common finding in mucopolysaccharidosis (MPS). Objective: To evaluate
the CSF flow using magnetic resonance imaging
(MRI) in 15 patients with MPS presenting neurological manifestations or not. Methods: We analyzed CSF flow study by phase-contrast MRI in 15
patients: MPS type I in 3 patients, MPS type II in
7 patients, MPS type III in 3 patients, MPS type
IV in 1 patient and MPS type VI in 1 patient. The
age range was 1 to 21 years, 13 males and 2 females. Results: The most frequent findings were
pyramidal signs in 7 patients and macrocephaly
in 8 patients. Only three patients had no cognitive impairment. The MRI abnormalities were
observed in all patients. The most frequent MRI
findings were dilated perivascular spaces in 10
patients, white matter changes in 10 patients,
ventricle enlargement in 8 patients and craniovertebral junction stenosis in 5 patients. Four patients with suspected hydrocephalus showed hyperdynamic aqueductal CSF flow. Conclusions:
Neuroradiological changes in MPS have been described before, although there are no published
articles on the CSF flow study as an adjunct for
the diagnosis and therapy planning of abnormalities like hydrocephalus and high intracranial
pressure in MPS patients.
26
AO-055
COENZYME Q10 DEFICIENCY CAN MODULATE
MITOCHONDRIAL BIOGENESIS AS ADAPTIVE
RESPONSE IN PRIMARY HUMAN FIBROBLASTS
Barros CCF1, Ravagnani FA1, Barros MH2, Arita JH3,
Sanches LR1, Masruha MR3, Barsottini OGP3,
Pedroso JL3, Picosse FR3
• 1HOSPITAL ISRAELITA ALBERT EINSTEIN;
2
UNIVERSIDADE DE SÃO PAULO; 3UNIFESP
Introduction: Mitochondrial disorders are the
main causes of inborn errors of metabolism.
Among all described mitochondrial diseases,
coenzyme Q10 (CoQ10) primary deficiency has
a good response to oral supplementation. Ubiquinone or CoQ10 is a mobile lipophilic electron carrier localized in the inner mitochondrial
membrane and has a critical role in the electron
transfer from complex I and II to complex III in
respiratory chain and also works as a potent antioxidant cellular. Coenzyme Q10 deficiencies are
heterogeneous, with variable clinical manifestation. DNA mutations directly related to the biosynthetic pathway of CoQ10 characterize the primary form of the disease. The secondary form of
the disease is characterized by DNA mutations in
intermediary metabolic pathways related genes
that affect mitochondrial respiratory chain.
Objectives: The aim of this study is to understand
the pathophysiological mechanisms related to
variable CoQ10 levels in primary culture skin fibroblasts in patients with suggestive clinical for
CoQ10 deficiency. Methods: Skin biopsies were
taken from 12 voluntaries normal individuals
and 27 patients. Fibroblasts were cultivated under culture medium to passage 4 and evaluated
for CoQ10 levels by HPLC with electrochemical
detection, enzymatic analysis for complexes I+III
and II+III, citrate synthase to access mitochondrial mass, ROS production by flow cytometry.
Mitochondrial biogenesis was analyzed by Real
Time PCR for PGC‐1 alpha, SIRT3 e SOD2 expression. Results: Ten patients (37%) had low CoQ10
levels. Five patients (18.5%) showed high levels of
CoQ10 content and twelve patients had normal
levels comparing to control group (24.10±3.84
pmol/mg protein, mean ± SD, p<0.05%). The
biochemical analysis showed variable enzymatic
activity for CI+III from 50.21 – 123.63% activity of
control and CII+III from 36.64 – 226.25% activity
of control, correlated to CoQ10 levels measured
by HPLC. We observed increased enzymatic activity for CII and citrate synthase in some patients suggesting an adaptive compensatory
mechanism to maintain mitochondrial homeostasis. In addition, PGC‐1 alpha expression was
higher in patients compared to control group
as well higher production of superoxide radical
in most of patients. Conclusion: The variable
CoQ10 content associated (or not) to other mitochondrial respiratory complexes deficiencies can
modulate an adaptive response involving mitochondrial biogenesis and redox signaling.
AO-056
DIFFERENTIATING HYDROCEPHALUS FROM
ATROPHY IN MUCOPOLYSACCHARIDOSIS WITH
COMBINED CEREBROSPINAL FLUID FLOW AND
LUMBAR PRESSURE STUDIES
Corte AD1, Souza CFM2, Vairo F2, Anés M2, Vedolin LM2,
Ferreira MM2, Aguzzoli AAG2, Perrone SGP2,
Federhen A2, Giugliani R2
• 1UFRGS; 2HCPA
Introduction: Hydrocephalus in patients with
mucopolysaccharidosis (MPS) is remarkable
for two reasons: 1) it is one of the few treatable
causes of intracranial hipertension, and 2) neuroradiologists, neurologists and neurosurgeons
are usually involved in making the diagnosis
which radiographically is not easily distinguishable from atrophy. Objective: To distinguish
ventricles dilatation from sulcal enlargement in
MPS patients and characterize hydrocephalus by
using cerebrospinal fluid (CSF) lumbar pressure
manometry and aqueductal CSF flow magnetic
resonance imaging (MRI) study. Methods: We
performed a CSF flow study by phase-contrast
MRI followed by a standart lumbar puncture
with the CSF opening pressure assessment in 14
patients: MPS type I in 3 patients, MPS type II in 7
patients, MPS type III in 3 patients and MPS type
IV in 1 patient. The age range was 1 to 20 years, 12
males and 2 females. Results: The most frequent
findings were pyramidal signs in 7 patients and
macrocephaly in 7 patients. Only two patients
had no cognitive impairment. The MRI abnormalities were observed in all patients. The most
frequent MRI findings were dilated perivascular
spaces in 9 patients, white matter changes in 9
patients, ventricle enlargement in 7 patients and
craniovertebral junction stenosis in 4 patients. Of
the 7 patients who were suspected of hydrocephalus, hyperdynamic aqueductal CSF flow was obtained in 4 of them and 3 patients showed CSF
lumbar pressure values above 200 mm H2O. On
the other hand, in 3 patients with no typical ventriculomegaly elevated CSF pressure values were
obtained. Conclusions: A better understanding
of the pathophysiology of hydrocephalus in MPS
patients will undoubtedly lead to better patient
selection and treatment. Although this is the first
description using CSF flow MRI and CSF lumbar
pressure manometry to diagnose high intracranial pressure in MPS patients larger studies must
be done to best determine which patients will
respond positively to shunting.
AO-057
FREQUENCY AND CLINICAL PROFILE OF BRAZILIAN
PATIENTS WITH LATE-ONSET FRIEDREICH’S
ATAXIA
Martinez ARM1, Borges C2, Abrahão Junior A3, Silva CB1,
Moro A2, Rezende TJ1, Moscovich M2, Munhoz RP4,
Segal SL5, Arruda WO2, Saraiva-Pereira ML5, Karuta S2,
Pedroso JL3, D‘Abreu A1, Jardim LB5, Lopes-Cendes Í1,
Barsottini O3, Teive HA2, França Junior MC1
• 1UNICAMP; 2UFPR; 3UNIFESP; 4UNIVERSITY OF
TORONTO; 5UFRGS
Objective: To evaluate the frequency and clinical
profile of Brazilian patients with late-onset Friedreich’s ataxia (LOFA). Background: Friedreich’s
ataxia (FDRA) is the most common inherited
ataxia worldwide, caused by homozygous GAA
expansions in the FXN gene. Patients usually
have early onset ataxia, areflexia, Babinski sign,
scoliosis and pes cavus, but at least 25% of cases
have atypical phenotypes. Disease begins after
the age of 25 in occasional patients (LOFA). Little
is known about the frequency and clinical profile
of LOFA patients. Design/methods: One hundred
and six patients with molecular confirmation of
FDRA and followed in three Brazilian outpatient
centers were enrolled. General demographics,
GAA expansion size, age at onset, cardiac, endocrine and orthopedic manifestations were evaluated and compared between LOFA and classic
FDRA (cFDRA) groups. We used Mann-Whitney and Fisher tests to compare means and
proportions between groups, p values <0.05
were considered significant. Results: LOFA patients accounted for 17%(18/106) and cFDRA
for 83%(88/106) of the patients. There were
13 and 48 women in each group, respectively.
XXVI Congresso Brasileiro de Neurologia
Mean ages at onset and disease duration for
LOFA and cFDRA were 30.6±6.7vs.13.5±5.0
years (p<0.001) and 18.2±8.6vs.14.8±8.8 years
(p=0.13), respectively. GAA quantification was
available for 63 patients, patients with LOFA
had shorter GAA expansions than patients with
cFRDA (GAA1:328.6±180vs.492.8±263.7 p<0.001;
GAA2:761.8±198.8vs.923.5±83.1, p<0.001). Clinically, LOFA group had a tendency towards lower
frequency of diabetes/impaired glucose tolerance (5.5%vs.19%, p=0.29) and cardiomyopathy
(16.6%vs.31.6%, p=0.25). Orthopedic abnormalities were significantly less frequent in LOFA
(scoliosis:25%vs.65.8%, p=0.004 and pes cavus: 18.7%vs.74.5%, p<0.001). Another relevant
clinical difference is spasticity and sustained
reflexes, which was found in 22% of LOFA patients but none of the cFDRA patients(p=0.001).
Conclusions: LOFA accounts for 17% of Brazilian FDRA patients evaluated herein. Clinically,
orthopedic features and spasticity with retained
reflexes are helpful tips to differentiate LOFA
from cFDRA patients. LOFA phenotype may be
easily misinterpreted as hereditary spastic paraplegia or other chronic myelopathies, so that clinicians should be aware of this FRDA variant in
their differential diagnosis. Study partially supported by Fundação de Amparo à Pesquisa do
Estado de São Paulo, FAPESP:2013/01766‐7 and
2012/17494‐3.
AO-058
MAGNETIC RESONANCE IS MORE SENSITIVE
THAN ECHOCARDIOGRAPHY TO DETECT
CARDIOMYOPATHY IN FRIEDREICH’S ATAXIA
Martinez ARM, Faber I, Silva CB, Venâncio T,
Martins Júnior CR, d‘Abreu A, Lopes-Cendes Í,
Coelho Filho OR, França Junior MC
• UNICAMP
Objective: To evaluate the usefulness of cardiac
magnetic resonance imaging (cMRI) to investigate heart damage in Friedreich’s ataxia (FDRA).
Background: FDRA is the most common inherited ataxia worldwide and is caused by homozygous GAA expansions in the FXN gene. Besides
its overt neurological features, FDRA is a multisystem disorder with endocrine, orthopedic and
cardiac involvement. Cardiomyopathy is often
progressive and the main cause of death in the
disease, so that sensitive diagnostic methods
would be helpful to improve long-term prognosis. Design/methods: General demographics,
GAA expansion size, age at onset and clinical
characteristics were evaluated. For each patient,
echocardiography and cMRI were performed
within one year interval. We used a 3T scanner
to obtain cMRI, and the acquisition protocol included: cine CMR images for LV volumes, function and mass assessment; late gadoliniun enhancement for scar assessment and T2* images
for iron overload assessmet. Results are detailed
with descriptive statistics. We used Fisher exact
test to compare the sensitivity of both methods
in our cohort. P values ≤0.05 were considered
significant. Results: We enrolled 10 FDRA patients, 6 of which were men. Mean age and age
at onset were 22.2±5.3 and 11.4±2.3 years, respectively. GAA-expansion lengths were: GAA11178.3±282.63 and GAA2- 1020±205.26. All patients had scoliosis, 4/10 (40%) pes cavus and
5/10 (50%) had diabetes/impaired glucose tolerance. Echocardiography findings were normal in
6/10 (60%) and showed concentric left ventricle
hypertrophy in 4/10 (40%) patients. In contrast,
cMRI proved more sensitive to detect abnormalities (cMRI: 90% vs echo: 40%, p=0.05). The most
frequent finding was left ventricular hypertrophy
with preserved ejection fraction. Evaluation of
T2* weighted images was performed in 4 patients
with normal echocardiograms and demonstrated
signs of iron accumulation in 75%. Conclusions:
cMRI is a robust tool to identify cardiomyopathy in FDRA and more sensitive than standard
echocardiography. Another relevant advantage
is that it enables in vivo demonstration of iron
accumulation in the heart which is a major feature of FDRA. Supported by: FAPESP - Fundação
de Amparo à Pesquisa do Estado de São Paulo:
2013/01766‐7.
AO-059
MME GENE MUTATION PROTECTS AGAINST
ALZHEIMER DISEASE
Simão-Silva DP1, Antunes A2, Batistela MS2, Dias PFR2,
Josviak ND2, Bono GF2, Piovezan MR2, Teive HAG2,
Souza RKM3, Alle LF2, Souza RLR2
• 1PUC-PR; 2UFPR; 3INC
Introduction: Alzheimer disease (AD) is a neurological disorder with profound and progressive cognitive decline, including memory loss,
by the functional and morphological deterioration of the hippocampus and temporal lobe
with accumulate of aggregates of the neurofibrillary tangles (NFT) and amyloid-β peptide (Aβ).
Neprilysin (NEP), coded by MME (Membrane
Metalloendopeptidase) gene, has been identified as an extracellular Aβ degrading enzyme in
the brain. Several reports have found that MME
is associated with AD. Objective: We have performed a case-control study in order to detect
the MME variant (rs2016846; G/T) which has not
yet been evaluated, is associated with Alzheimer
disease. Methods: Genomic DNA was extracted
from whole blood from 112 AD patients and 118
elderly controls (EC) by a salting out method.
Both groups constituted mainly of euro-Brazilian from Southern Brazil, being AD patients
from Clinical Hospital of the Federal University
of Paraná (HC-UFPR) and Curitiba Neurology
Institute (INC), and diagnosed according to the
NIA-AA (National Institute on Aging and Alzheimer’s Association) criteria for probable AD. Clinical Dementia Rating (CDR) was used to classify
the stage of dementia in mild (CDR1), moderate
(CDR2) and severe (CDR3). Genotyping of the
rs2016846 was performed with the kit TaqMan®
genotyping assays (Applied Biosystems). Results:
In the examined samples the genotype distributions were in Hardy–Weinberg equilibrium. The
genotype and allele distribution of rs2016846
did not show significant differences between
cases and controls (χ2=3.34; p=0.10) if consider
all AD stages. The genotype distribution differed
significantly between the stages CDR1 and CDR2
and CDR3 (χ2=10.75; p=0.02). The genotype frequencies of rs2016846 was not different between
CDR1 and controls (χ2=0.89; p=0.64) but, the
comparison between AD in CDR2 plus CDR3 and
EC showed a significantly decreased frequency
of the non usual allele (T) in the homozygote
genotype (χ2=8.01; p=0.01). Conclusions: Thus,
we found that the rs2016846 protect against
AD. Intriguingly, CDR1 patients have the same
proportion of the variant in study that EC. This
found leads to questions about the clinical and
diagnostic characteristics of these patients. Considering that NEP plays a key role in regulating
the level of Aβ peptide in the brain, this research
opens for investigation of the protective role of
rs2016846 mutation in the brain.
AO-060
PHENOTYPE VARIABILITY AND POSSIBLE
MODIFIER GENES
Monte T1, Coelho LL1, Silva AS1, Reckziegel E2, Olchik M3,
Pereira MLS2, Pereira FS3, Barsottini O4, Pedroso JL4,
Vargas FR5, Jardim LB2
• 1HOSPITAL DE CLINICAS DE PORTO ALEGRE; 2HCPA;
3
UFRGS; 4UNIFESP; 5UFRJ
Introduction: Spinocerebellar ataxia type 2
(SCA2) is an autosomal dominant cerebellar
ataxia caused by the abnormal expansion of a
CAG repeat, characterized by an adult-onset,
progressive cerebellar syndrome associated with
saccadic slowness and other findings. Although
SCA2 presents a large variability of clinical signs,
there is no explanation for this heterogeneity.
Objective: to test the potential modifier effects
of six genes and one mitochondrial polymorphism on the determination of five neurological findings in SCA2: cognitive deterioration,
sensory loss, amiotrophy, parkinsonism and
dystonia. Material and methods: After consent,
symptomatic subjects with molecular diagnosis of SCA2 done by Rede Neurogenetica, were
recruited from Porto Alegre, Sao Paulo and Rio
de Janeiro. The following scales were applied:
SARA , NESSCA, SCAFI, CCFS, Mini Mental Examination, MOCA, and BDI. The CAG repeats
at ATXN1, ATXN2, ATXN3, CACNA1A, ATXN7,
and RAI1 genes, as well as the A10398G mitochondrial polymorphism, were determined.
The distribution of CAG repeats and of the mitochondrial alleles were compared between
phenotypic groups (with and without the five
neurological findings) by Mann-Whitney U test
and chi-square, respectively. Since this is an exploratory study, a p<0.05 was chosen. Results:
37 SCA2 patients were examined. Age at onset
correlated with the expanded repeat at ATXN2
(r=0.73, p<0.0001, Spearman). Cognitive decline
was associated with the mitochondrial allele G
at A10398G (p=0.01), while dystonia was associated with larger expansions in ATXN2 (p=0.021).
Discussion: We were not able to confirm earlier
suggestions of association between parkinsonism and shorter expansions at ATXN2, in SCA2.
In contrast, two modifiers of SCA2 neurological
phenotype were suggested. Dystonic movements
are associated with more severe expansions at
ATXN2, a phenomenon similar to what was seen
in SCA3/MJD and its causal expanded repeats.
Finally, cognitive deterioration was associated
to allele G of the mitochondrial polymorphism,
a variant already associated to early ages at onset
of SCA2.
AO-061
UNRAVELING THE DIAGNOSIS OF THE
CEREBELLAR ATAXIAS WITH RETAINED TENDON
REFLEXES
Pedroso JL1, Noreau A2, Souza PVS1, Pinto WBVR1,
Albuquerque MVC1, Rouleau G3, Barsottini O1
• 1UNIFESP; 2MONTREAL, QC; 3MONTREAL
NEUROLOGICAL INSTITUTE AND HOSPITAL
Introduction: Autosomal recessive cerebellar
ataxias are a heterogeneous group of neurological disorders. In 1981, a neurological entity
comprised by early onset progressive cerebellar
ataxia, dysarthria, pyramidal weakness of the
limbs and retained or increased upper limb reflexes and knee jerks was described. This disorder is known as early onset cerebellar ataxia with
retained tendon reflexes. It is uncertain, however, whether early onset cerebellar ataxia with retained tendon reflexes is a homogeneous disease
27
Oral Presentation
or a group of phenotypically similar syndromes
represented by different genetic entities. Further
molecular studies were required to provide definitive answers to the questions that remained
regarding early onset cerebellar ataxia with retained tendon reflexes. Objectives: To present a
series of 39 patients with the classic phenotype
of cerebellar ataxia with retained tendon reflexes. To describe 4 new mutations in SYNE1 gene
in this sample, that causes cerebellar ataxia with
retained tendon reflexes. Methods: We evaluated 39 patients in the Ataxia Unit, Department
of Neurology, UNIFESP, from 18 to 50 year-old,
that presented with progressive cerebellar ataxia
with retained tendon reflexes and had negative
tests for Friedreich ataxia and the most common
SCAs. Detailed clinical features were described.
We also evaluated brain imaging and electroneuromyography. Blood biomarkers also excluded
other recessive ataxias. 190 primer pairs were
designed to cover the 144 coding exons of SYNE1
(NM_182961) gene and loaded on the Fluidigm
Access Array, a microfluidic array in which a
PCR was performed (AccessArray™ System User
Guide, Fluidigm Corporation, San Francisco,
CA). We amplified 39 DNA samples of Brazilian
origins and performed MiSeq sequencing runs.
These two steps were performed at the Genome
Quebec Innovation Centre (Montréal, Québec,
Canada). Results: We found 4 new truncating
mutations, all homozygote and other missense of
unknown pathogenicity (Figure). Conclusions:
Patients with cerebellar ataxia with retained
tendon reflexes that presented negative tests for
Friedreich ataxia, for other recessive ataxias and
for the most common SCAs should be genetically
tested for SYNE1 gene.
Neuroimaging
frontal Meningioma. Case 3: Poodle, 12 years
old, with signs of syncope during a month that
has progressed to torpor and coma. CT scan
findings: large fauces meningioma. Case: 4,
Pittbull, 7 years old, it was reported the history
of behavioural changes, sleep disorders, ataxia,
muscular tremors. Mri findings: pilocytic astrocytoma. Case 5: Shitzu, 4 years old, with lingual
and jaw paralysis, difficulty swallowing. Mri findings: large quadrigeminal cyst. Conclusion: The
clinical cases above reported illustrate very similar imaging features in different types of brain
tumor between humans and animals.
AO-063
COMPARISON OF TEMPORAL LOBE EPILEPSY WITH
LEFT OR RIGHT HIPPOCAMPAL ATROPHY USING
QUANTITATIVE ANALYSIS OF DIFFUSION (DTI)
Sanches P, Fujisao EK, Yamashita S, Betting LE
• UNESP
Medial temporal lobe epilepsy (MTLE) with left
hippocampal atrophy (HA) probably has different clinical behavior when compared with the
right side. This study aims to quantify structural changes in the brain white matter of patients
with MTLE and left or right HA compared with
controls. Eight patients with right MTLE, seven
with left MTLE and 30 controls were evaluated.
All patients underwent 3T MRI (Siemens Verio).
Echo planar imaging (EPI) sequence was used
to obtain the images. To analyze diffusion tensor
images (DTI) obtained, FSL program and TBSS
(Tract-Based Spatial Statistics) technique were
used. Fractional anisotropy (FA) maps were extracted, processed and submitted to statistical
analysis comparing the groups. MTLE with right
HA: FA reduction with the center of gravity in the
fusiform gyrus (x=39, y= ‐4, z= ‐18; 1271 voxels,
T=5.13). MTLE with left HA: more significant
and diffuse FA reduction with center of gravity
in the thalamus (x= ‐1, y= ‐13, z=15; 56282 voxels,
T=6.32). The present data shows changes in the
white matter of patients with MTLE with more
significant and diffuse involvement in patients
with left HA.
AO-062
AO-064
BRAIN TUMOR LESIONS IN ANIMALS DIAGNOSED
BY RESONANCE IMAGING AND COMPUTED
TOMOGRAPHY
INVESTIGATION OF BRAIN ATROPHY LATERALITY
IN PARKINSON‘S DISEASE USING VOXEL-BASED
MORPHOMETRY
Machado VMV1, Amorim RM1, Yamashita S2,
Vulcano LC1, Fernandes TD2, Resende LAL2
Santos MCA, Campos L, Guimarães R, Piccinin CC,
Azevedo P, Piovesana L, Campos B, Amato Filho A,
Lopes-Cendes I, Cendes F, D‘Abreu A
• 1FMVZ - UNESP; 2FMB - UNESP
Introduction: Technologies in advanced imaging modalities as computed tomography and
magnetic resonance were acquired by the School
of Veterinary Medicine and Animal Science - São
Paulo State University (FMVZ-UNESP- Botucatu-SP) in 2007 and 2012, respectively, enabling
the diagnosis of many different types of brain
tumors. It was also possible to stablish advances and improvement in the treatment and prognosis of several diseases. The aim of the present
study was to describe canine brain tumor lesions
in order to emphasize the similar features of human brain tumors. Methods: Clinical and imaging descriptive study of patients admitted to
FMVZ-UNESP presenting brain tumor. Results:
Case 1: a Boxer, 11 years old, was referred to FMVZ-UNESP and intermittent seizures and generalized apathy was reported. CT scan findings:
frontal oligodendroglioma and brain ventricular
system enlargement. Case 2: Boxer, 12 years old
presenting sporadic seizures. CT scan findings:
28
• UNICAMP
Introduction: Increasing neuroimaging studies
have reported extensive gray matter (GM) atrophy in idiopathic Parkinson‘s disease (IPD), involving cortical and subcortical structures. Nevertheless, the majority of evidence disregards
the laterality of disease presentation, which has
a knowingly heterogeneous onset and progression. Objective: To investigate GM loss in IPD by
voxel-based morphometry (VBM) considering
the side of disease onset. Methods: Sixty patients
with IPD (57.87±10.27 years), in accordance
to the UK PD Society Brain Bank criteria, were
compared to 80 healthy controls (HC) (57.1±9.47
years). Among IPD group, 26 had a right-sided
disease onset (RDO) and 34 a left-sided disease
onset (LDO). We acquired all T1-weighted images
on a 3T scanner. MRIcro software flipped the images right/left of the 26 RDO patients, so that all
images had the contralateral side of IPD onset at
the right hemisphere. We flipped 35 HC images as
well, since the hemispheres are not equivalent. A
secondary analysis involved RDO patients versus
HC and LDO patients versus HC. VBM8 (SPM8/
Dartel) on Matlab R2012b processed the images,
after exclusion of three images by a homogeneity test. For statistics, we performed two-sample
t-test with family-wise error correction (p<0.05),
using an extent threshold of 20 voxels. Significant
decrease of GM was reported only within clusters ≥100 voxels. Results: Flipped-whole brain
VBM showed GM atrophy predominantly in the
left olfactory cortex, left amygdala, left parahipocampal gyrus and bilaterally in the putamen, rectus gyrus, insula, frontotemporal cortex, cuneus,
precuneus and calcarine fissure. In LDO, we
observed GM reduction in the insula, putamen,
anterior cingulate, frontotemporal cortex bilaterally and in the right caudate; while in RDO group,
we found GM loss, bilaterally, in the anterior cingulate gyrus, insula, frontotemporal and occipital cortex. Conclusion: LDO patients are more
severely affected than RDO group, based on the
number of atrophic voxels. Previous studies associated the left side of IPD onset with advanced
symptoms, such as cognitive dysfunction. Flipping the images, in order to evince the laterality
of disease, enhances the sensitivity of the method and it might allow for a better evaluation of
IPD progression in whole-brain analyses.
AO-065
INVESTIGATION OF CORTICAL THICKNESS IN
PATIENTS WITH HIV/AIDS AND WITH STRUCTURAL
ABNORMALITIES IN THE BRAIN
Fujisao EK, Sanches P, Mendes MB, Barbosa AN,
Yamashita S, Betting LE
• UNESP
Background: Thinning of the cerebral cortex is
associated with the progression of the disease
in people living with HIV/AIDS and reflects
CD4 lymphocyte decline. After the highly active
antiretroviral therapy era these findings were
expected to be less frequently observed. Other
structural abnormalities of the brain were also
described with HIV infection including cerebelar, vascular and white matter abnormalities. In
this investigation the cortical thickness of people
living with HIV/AIDS and with the coexistence
of several major brain structural abnormalities
was investigated. Methods: Eleven people living with HIV/AIDS (4 women, mean age 46±10,
range 30‐58) were retrospectively selected when
hospitalized for HIV treatment. All patients (18
women, mean age 32±11, range 22‐57) and a
group of 36 controls were submitted to 3T MRI.
Volumetric 3D T1 sequences with 1mm isotropic
voxels were used for the evaluation. Freesurfer
software was used for the analysis. All images
were submitted to the same process. Basically, images were automatically corrected for inhomogeneities, normalized and segmented.
Cortical thickness was calculated as the closest
distance from the gray/white boundary to the
gray/CSF boundary at each vertex on a tessellated brain surface. Statistical analysis was performed using general linear model. A p<0.05 was
considered significant after correction for multiple comparisons (False Discovery Rate, FDR).
Results: Conventional visual analysis showed
that all patients presented at least one brain lesion. They were located in the left medial frontal
gyrus, right basal ganglia, right cingulate gyrus,
right and left parieto-temporo-occipital cortex,
fronto-polar region, right cerebellum and left
midbrain. Most of the patients (9) presented with
toxoplasmosis, 1 had gliosis and 1 was diagnosed
XXVI Congresso Brasileiro de Neurologia
with brain tumor. 8 patients were receiving antiretroviral therapy. Structural analysis revealed
areas of decreased cortical thickness in the patients group localized mainly in the cingulate
gyrus and insula (right hemisphere: max value
-log10(p) 10.3, size 7149 mm2, Talairach coordinates x=4.7, y=‐24, z=38; left hemisphere: max
value -log10(p) 11, size 6297mm2, coordinates
x=‐36, y=‐23, z=‐3). Conclusion: In this preliminary investigation, people living with HIV/AIDS
and with several major brain structural abnormalities also presented cortical thinning mainly
in limbic structures and independently of the
primary site of the lesion.
AO-066
PROGRESSION OF GRAY MATTER ATROPHY
THROUGH SEVERITY STAGES OF PARKINSON‘S
DISEASE
Santos MCA, Campos L, Guimarães R, Callegari C,
Azevedo P, Piovesana L, Campos B, Amato Filho A,
Lopes-Cendes I, Cendes F, D‘Abreu A
• UNICAMP
Introduction: Braak stages define an ascending
progression of Parkinson’s disease (PD), beginning at olfactory nucleus and brainstem towards
all neocortex. Regardless of the reliability of this
post-mortem evidence and the clinical correlations in discussion, no in vivo study has definitively validated the distribution of gray matter
(GM) atrophy according to disease severity.
Objective: To identify a pattern of GM abnormality in PD using voxel-based morphometry (VBM)
across severity stages determined by HY scale.
Methods: Sixty-six patients diagnosed with idiopathic PD (57.94±10.25 years) following the UK
PD Society Brain Bank criteria, were compared
to 80 healthy controls (HC) (57.1±9.47 years). We
stratified patients as mild PD (HY 1‐1.5), moderate (HY 2‐2.5) and severe (HY 3‐5). Anatomical
T1-weighted MRI images were acquired on a 3T
scanner. VBM8 toolbox of the SPM8/Dartel software on Matlab R2012b platform processed the
aligned images, after exclusion of 1‐5 patients
and one HC by a homogeneity test using covariance. We evaluated the subsequent groups compared to HC: all PD (66), mild PD (14), moderate
PD (31) and severe PD (16). The statistical analysis comprised two-sample t-test with family-wise
error correction (p< .05) and an extent threshold
of 20 voxels. Results: Whole-sample VBM showed
GM atrophy predominantly in the olfactory cortex, putamen, amygdala, anterior cingulate gyrus, insula and frontotemporal areas. For the
subgroup analysis, there was no GM reduction in
mild PD patients. In moderate PD, we identified
GM loss in the insula and inferior frontal gyrus
while in severe PD we found atrophy in the insula,
inferior frontal gyrus, putamen, medial superior
frontal gyrus, anterior cingulate, median cingulate and paracingulate gyrus. Conclusions: The
study revealed a somewhat ascending pattern of
GM progression, since the severe PD atrophy expands beyond the areas affected in the moderate
group. However, we did not find any GM changes
in early PD, even in brainstem or olfactory bulb,
which might be due to a method limitation. Anyhow, our findings are in accordance with previous reports on advanced symptoms in PD, such
as depression, hallucinations and dementia. Further longitudinal analyses accessing both motor
and non-motor scales might offer a better understanding of disease progression.
AO-067
QUANTITATIVE BRAIN MAGNETIC RESONANCE
IMAGING FINDINGS IN CHAGAS DISEASE
Fernandes RD, Gonçalves BM, Barreto Neto N,
Andrade AL, Reis CC, Ferreira IL, Resende LL,
Jesus AA, Lordelo MJ, Abbehusen C, Reis F, Jesus PA,
Oliveira Filho J
• UFBA
Background: Chagas disease (CD) has been
associated with cognitive dysfunction, but the
brain structural mechanism is largely unknown.
We aimed to determine which magnetic resonance imaging (MRI) findings differentiate CD
from non-CD subjects. Methods: Cross-sectional
study of a cohort of patients from a Heart Failure
(HF) clinic. Stroke-free patients underwent brain
MRI to quantify several volumetric imaging parameters (brain volume, cerebellar volume,
white matter hyperintensity volume – all corrected for intracranial volume) and MRI spectroscopy to quantify N-acetyl-aspartate to creatine
ratio (NAA/Cr), choline to Cr ratio (Cho/Cr) and
myoinositol to Cr ratio (mI/Cr). Linear regression
models adjusted for age, sex and left ventricle
ejection fraction were used to test the association
between CD and each imaging marker. Results:
We studied 145 subjects, mean age 55±12 years,
81 (55%) female, 63 (46%) with CD. Both NAA/Cr
and Cho/Cr ratios were significantly lower in CD
patients when compared to non-CD (1.74±0.16
vs 1.82±0.20, p=0.011; and 0.57±0.10 vs 0.60±0.09,
p=0.023, respectively). In the multivariable model, CD remained independently associated with
lower NAA/Cr (effect/standard error=‐0.12/0.04,
p=0.001), but not Cho/Cr (effect/SE=‐0.03/0.02,
p=0.086). In the 95 subjects with available volumetric data, we found no significant association
between CD and any of the measured volumes.
Conclusions: Chagas disease is independently
associated with MRI markers of neuronal depopulation. In a smaller sample, we were not able to
confirm previous observations on the association between CD and brain atrophy.
Neuroimmunology, Multiple
Sclerosis and other
Demyelinating Disorders
AO-068
ANALYSIS OF BONE MICROARCHITECTURE IN
WOMEN WITH MULTIPLE SCLEROSIS
Guerreiro CT1, Brum DG2, Marques VD1, Carmo SS1,
Barreira AA1, Iannetta O1
• 1SCHOOL OF MEDICINE OF RIBEIRÃO PRETO,
UNIVERSITY OF SÃO PAULO – USP; 2BOTUCATU
MEDICAL SCHOOL, SÃO PAULO STATE UNIVERSITY
- UNESP
Background: Women with multiple sclerosis (MS)
can have increased risk of developing osteoporosis due to secondary factors, such as reduction of
the mobility and the treatment with steroids that
interferes with calcium absorption and building
up of bones. Objectives: The aims of this study
were to verify the bone profile of women with
MS through phalangeal quantitative ultrasound
(QUS) parameters and compare it to healthy
women. Methods: The present study included
115 females (57 MS patients, 58 healthy controls)
with mean age of 33.45±6.09 and 35.74±7.64 respectively. The mean Expanded Disability Status
Scale (EDSS) was 3.42±1.46 (1.5‐6.5). The weight
and height were evaluated by anthropometric
methods and the Body Mass Index (BMI) was
calculated. The evaluation of bone quality (Ultrasound Bone Profile Index, UBPI) and quantity
(Amplitude Dependent Speed of Sound, AD-SoS)
was measure by phalangeal quantitative osteosonography (DBM Sonic BP). DBM Sonic BP is
a method for measuring the ultrasound velocity
through the proximal phalanges of the hand. All
analyses were performed using the SPSS Statistics (SPSS Inc., Chicago, IL, USA) with level of
significance set at p<0.05. Results: Significant
differences were found in UBPI values (p=0.001),
AD-SoS values (p=0.022), and T-score (p=0.022).
A negative strong correlation between EDSS and
low bone quantity (AD-SoS <2054 m/s; r=‐0.870;
p=0.130) were found. When correlated to inadequate quality of the bone (UBPI <0.69) a negative moderate correlation was found (r=‐0.588;
p=0.057). Conclusion: DBM Sonic BP indicated a
difference of bone profile and the degree of bone
loss in MS women. This is a fast, reliable and easy
to perform method that can be used to evaluate
MS patients in an outpatient basis.
AO-069
ANTI-NMDA RECEPTOR ENCEPHALITIS: CLINICAL
ANALYSIS OF A SERIES OF PEDIATRIC CASES
Ferreira THS1, Paranhos DR1, Leite NPS2, Mattos AM2,
Souza VMA3, Lima Filho HC3
• 1EBMSP; 2HOSPITAL SÃO RAFAEL; 3HSR
Introduction: Anti-N-methyl-D-aspartate encephalitis (rNMDA) is a severe neurological
disease, however with high probability of recovery, characterized as autoantibody production against the NR1 subunit rNMDA. The main
clinical symptons include cognitive-behavioral
disorders, memory deficits, language disorders,
seizures, and movement disorders. Objective: To
discuss and highlight the main clinical features
of encephalitis anti-rNMDA in three pediatric patients, as well as alert to the possibility of
this diagnosis in pediatric and adult population.
Methodology: A retrospective observational
study conducted through analyzing the medical
records of three pediatric patients in a hospital
in Salvador, Bahia, with a diagnosis of encephalitis anti-rNMDA confirmed by autoantibodies
against rNMDA present in cerebrospinal fluid
(CSF). Results: The mean age of patients was 13
years (11‐14 years), all female. All patients initially presented with psychiatric symptoms such as
agitation, aggression, mood disturbances and
hallucinations. Subsequently, they progressed
to presenting dyskinesias (predominantly orofacial), dysarthria, seizures and Intensive Care Unit
(ICU) admission. The patients received steroids,
intravenous immunoglobulin or plasmapheresis
alone or in combination, as well as Rituximab
and Cyclophosphamide. All patients had a score
between 0‐1 in the modified Ranking Scale at
discharge from hospital. Conclusion: A well-defined set of clinical characteristics are associated
with the anti-rNMDA encephalitis. The diagnosis
of encephalitis anti-rNMDA should be thought
in patients with suggestive clinical presentation
and after exclusion of other common causes of
encephalitis in children and adults, especially
by the severity of the disease and its potential
treatment.
29
Oral Presentation
AO-070
AO-072
DISABILITY VERSUS PROGRESSION IN MULTIPLE
SCLEROSIS AND CLINICALLY ISOLATED
SYNDROMES: A SOUTH AMERICA MULTICENTER
STUDY
ELECTRICAL STIMULATION OF THE TIBIAL NERVE
AS PHYSIOTHERAPEUTIC PROPOSAL OF URGENCY
URINARY INCONTINENCE IN WOMEN WITH
MULTIPLE SCLEROSIS
Alvarenga RMP1, Vasconcelos CCF1, Alvarenga MP1,
Campanella L1, Calmon A1, Grzesiuk AK2,
Pessanha Neto S1, Batista E1, Hamuy F3,
Sotto I4, Brito ML5, Freitas MCDNB6, Rocha MS7,
Pimentel MLV8, Parolin M9, Marinho P6, Siqueira HH10,
Camargo S1, Machado S11, Neri VC1, Fleitas CV12,
Fragoso YD13, Diniz D14, Alvarenga MP6, Cabeças H15,
Gonçalves MVM16, Thuler LCS17
• 1UNIRIO/HOSPITAL DA LAGOA; 2CUIABÁ;
3
PARAGUAY; 4VENEZUELA; 5RECIFE; 6REDE
SARAH DE REABILITAÇÃO; 7HOSPITAL SANTA
MARCELINA; 8SANTA CASA RJ; 9CURITIBA; 10UFMT;
11
FLORIANÓPOLIS; 12ASSUNCION; 13SANTOS; 14GOIÁS;
15
BELEM; 16JOINVILLE; 17UNIRIO/INCA
Background: Population studies on the progression of disability in MS are difficult to perform
because the relationship between disability and
disease duration does not follow a linear pattern
due to considerable individual variation in the
clinical course of the disease. The MS severity
score (MSSS) was proposed for epidemiological
studies to analyze progression. Few studies analyzed MS progression in South America Methods:
A descriptive, multicenter, cross-sectional study
was performed including individuals with IIDD
followed in 2011 in 21 neurological MS centers
in South America. Only CIS and MS cases were
selected for this study. Data collected ethnicity [white and non-white], age at onset, EDSS
[0‐3=mild], [4.5=moderate], [≤6=severe]; MSSS:
[benign <0.45], [mild-moderate 0.46 – 5.00], [advanced-accelerated 5.00– 8.23], [aggressive-malignant >8.24. The data were forwarded through
the Internet and analysis performed using the
SPSS software. Results: CIS was described in 70
patients, mainly white women; disease onset
below 18 years (10.1%) and after 50 (7.2%); optic neuritis (47.8%), transverse myelitis (24.6%),
multifocal (14.5%) and brain stem syndrome
(8.7%). After 3.56±3.82 years, disability was
mild and progression mild-moderate. MS occurred in 1252 patients [84.42% RR, 15.57 SP%;
7.1% PP]. MSRR pediatric forms 6.6% and late
forms 6.0%; disability was mild (66%), severe
(18.6%) or moderate (15.1%); progression was
mild-moderate (55.56%), advanced-accelerated
(25.21%), aggressive malignant (9.73%) or benign (9.48%). Among MSPP pediatric forms did
not occur and 19% had late onset; the majority
had severe disability (64.55%) and advanced-accelerated (26.9%) or aggressive malignant progression (44.03%). Discussion: A mild disability
in 66.0% of the patients with MS with relapsing
remitting course at onset and 15% of SP might
suggest a more benign course of MS in South
America. However, this hypothesis can only be
confirmed through prospective follow-up of
this cohort, accounting for confounding factors
such as the use of disease-modifying therapy, age
at onset, and ethnicity. Among cases with mild
disability occurred benign, mild-moderate and
advanced-accelerated progression. In addition
the MSSS index showed higher rate of progression of MS in non-white patients, as previously
described in African-Americans and Afro-Brazilians. Conclusion: the pattern of disease progression in MS is broader than showed by EDSS
scores.
30
Cursino MP1, Anezio A1, Barbalho A1, Souza KES2,
Navega FRF1, Barbosa AMP1
• 1UNESP; 2FISIOTERAPEUTA UROGINECOLÓGICA
Introduction: Urinary incontinence cause social,
physical and hygienic problems. Objective: Analyze the effectiveness of transcutaneous electrical stimulation of the tibial nerve as a therapeutic
proposal to urgency urinary incontinence (UUI)
in women with multiple sclerosis (MS). Method:
Six women with MS and UUI. Complaints related
to UUI were listed, applied quality of life questionnaires King‘s Health Questionnaire (KHQ),
Overactive Bladder Questionnaire (OAB-V8),
specific MS Scale for the Functional Assessment
of MS quality of life instrument (FAMS) and
Expanded Disability Status Scale (EDSS), functional evaluation and electromyographic assessment of the pelvic floor (PF). Thirty sessions of
twenty minutes of bilateral electrical stimulation
were performed, with parameters 200μs / 10 Hz,
three times per week. For every 10 sessions and
at the end of the protocol, the participants were
reevaluated. Shapiro Wilk normality test was
applied; for normal data, paired Student T test
was applied and to not normal data, Wilcoxon
test, results were considered significant when
p<0.05. Results: In the daily loss of urine, most
participants had the volume and frequency of
loss decreased and ranged from daily to weekly, and two women reported no loss in the last
two weeks; voiding diary showed decreased urinary frequency with a tendency to be significant
(p=0.058). Significant difference was positive in
OAB-V8 questionnaire (p=0.030). The functional evaluation of the PF by Perfect showed significant difference for improvement in Muscle
Strength item (p=0.021), for the variables E, R
and F (p=0.168, p=0.054, p=0.330 respectively)
there was no difference significant. In KHQ there
was no significant difference for the variables
1, 3, 4, 6, 7 and 9 (p=0.101, p=0.265, p=0.412,
p=0.287, p=0.201, p=0.165 respectively). There
were significant differences for improvement between the initial and final moments in questions
2 – How much the bladder problem disrupts your
life (p=0.002), 5 - Social limitation (p=0.020) and
8 - Sleep/Energy (p=0.037). In FAMS (p=0.116),
EDSS (p=0.055), electromyography slow fibers
(p=0.42) and fast fibers (p=0.44), no significant
differences were found. Conclusion: Transcutaneous electrical stimulation of the tibial nerve
was effective to decrease the symptoms of urgency and urgency urinary incontinence in women
with multiple sclerosis, improve muscle strength
and quality of life.
AO-073
MYASTHENIA GRAVIS – THE REFERENCE CENTER
EXPERIENCE WITHIN A 19 YEARS FOLLOW-UP
Lins SPS, Moreira ÁJP, Cruz NSR, Machado ÍMM,
Ferreira MLB
• SERVIÇO DE NEUROLOGIA - HOSPITAL DA
RESTAURAÇÃO
Introduction: The neuromuscular transmission
diseases have immunological, toxic or genetic origins and myasthenia gravis is the most frequent.
Objective: To describe the clinical presentation
and follow up of myasthenia gravis cases, during
19 years at a Reference Center. Methods: In a descriptive, observational, individual, prospective,
cross sectional study, we included a population
of 33 patients, followed from January 1995 to
April 2014. To data obtained at the beginning of
management (clinic manifestations, exams and
diagnosis), we added clinic classification, comorbidities and therapeutic evolution. Results: We
identified predominance of females (1.54:1.00 female/male). First symptoms were more frequent
on forth decade for females (median=41.0 years),
and fifth decade for males (median=50.0 years).
Female patients, aging up to forth decade, had
myasthenia gravis associated with autoimmune
diseases, as relapsed optic neuritis, hypothyroidism, thyroiditis, polymyalgia and fibromyalgia.
The exception was penfigo with myasthenia
gravis and thymus neoplasia, diagnosed in a
69 years old patient, surgically treated. Among
males, the autoimmune diseases were restricted to ulcerative rectocolitis associated to Herpes
zoster infection. There were 10 (30.3%) cases of
thymoma, a frequency almost three times greater than referred in literature. The 7 (21.2%) cases
of hypertrophic thymus were diagnosed by image in 25% of females and 15% of males and this
distribution differed from the literature. Twenty
(60.6%) patients among 25 tested, had increased
anti acetylcholine receptor antibody titles. Within the comparison of patients with and without
thymoma, there was a significant increase in diagnose time (1.2±0.5 years against 0.5±0.3 years;
p=0.025), anti acetylcholine receptor antibody
titles (14.4±8.00 against 2.62±0.76; p=0.096) and
disease duration (6.12±0.86 against 6.04±1.21;
p<0.001). According to the clinical classification
of myasthenia gravis, there was a predominance
of grades IIa and V. Therapeutic management
included piridostigmine isolated or associated
with corticosteroid, azathioprine or immunoglobulin. Conclusion: This first description of
our reference center permitted to identify major
incidence of thymoma, frequent association with
immune mediated disorders. These clinic characteristics may help to increase suspicion of this
diagnose by neurologists, as well as to perform
faster attendance to myasthenic crisis of patients
already diagnosed.
AO-074
PSYCHIATRIC DISORDERS IN BRAZILIAN PATIENTS
WITH MULTIPLE SCLEROSIS
Cerqu AC1, Andrade PS2, Godoy-Barreiros JM2,
Teixeira Júnior AL3, Nardi. AE1
• 1UNIVERSIDADE FEDERAL DO RIO DE JANEIRO;
2
UNIVERSIDADE DO ESTADO DO RIO DE JANEIRO;
3
UNIVERSIDADE FEDERAL DE MINAS GERAIS
Introduction: The aim of this study was to evaluate the frequency of psychiatric disorders in a
sample of ambulatory patients with MS in Brazil,
and correlate with clinical and sociodemographic data. Methods: A cross-sectional study was
conducted with MS patients in Rio de Janeiro.In
this study, all patients who had diagnosis of MS
according to the McDonald criteria were invited
to participate. Patients who had exacerbation
of the disease in the last three months,under
18 years of age and more than 65, EDSS >7.5,
less than four years of schooling, and those
with other neurological diseases were excluded.
Complete medical history focusing on the disease, as well as socio-demographic data were
collected concurrently. The Mini-International
Neuropsychiatric Interview, a short structured
interview designed to explore each of the main
necessary criteria for the diagnoses of DSM-IV,
was the main diagnostic instrument.The severity
of depressive symptoms was assessed using the
XXVI Congresso Brasileiro de Neurologia
Beck Depression Inventory,and the severity of
symptoms of anxiety through the Beck Anxiety
Inventory. Patients signed a voluntary written informed consent to Participate in the study, which
was approved by our Local Research Ethics Committee. Results: The sample n=60 was divided
into genders and included 76.7% female patients, 23.3% were male.The frequency of mood
disorders, current depressive episode was found
in 18.5% history of depressive episode 18.5%.
Hypomanic episode 10%, mania 3.3%, so 13.3%
had bipolar disorder. Regarding the frequency of
anxiety disorders 16.7% had generalized anxiety
disorder and 3.3% had panic disorder. Of the 60
patients 8.3% had attempted suicide, and 8.3%
had current suicide risk.All patients were at risk
of suicide had current depressive episode.The
severity of depressive symptoms measured by
the BDI showed that 24.9% of patients had depressive symptoms ranging from mild to severe.
The severity of anxiety symptoms assessed by the
BAI showed that 34% of the patients had symptoms of anxiety, ranging from minimal to severe.
The results of this study showed that differences
duration of the disease and severity of symptoms
in MS no appears to be related to major depression. Conclusion: The results suggest that major
depression is an important risk factor for suicide
and should be evaluated systematically in this
population.
Neuroinfection
AO-075
CLINICAL AND LABORATORY CHARACTERIZATION
OF NEUROGENIC BLADDER ASSOCIATED WITH
HTLV-1
(p=0.01). Urinary incontinence was found in all
HAM/TSP individuals and EDSS was higher in
this group (p<.01). Conclusion: The neurogenic
bladder associated with HTLV‐1 may be an incipient state of myelopathy and the proviral load
and the CSF may be good research parameters.
AO-076
DETECTION OF SCHISTOSOMA MANSONI DNA
IN THE CSF OF PATIENTS WITH SPINAL CORD
SCHISTOSOMIASIS
Bruscky IS1, Correia CC1, Medeiros ZM2,
Albuquerque FF3, Wanderley LB2, Melo FL2
• 1UPE; 2FIOCRUZ; 3SDN
Introduction: The diagnostic methods currently available for spinal cord schistosomiasis have
low specificity and sensitivity. In recent decades,
a more specific and sensitive diagnostic tool
was developed based on the use of polymerase
chain reaction. This strategy was only applied to
the blood, feces and urine of patients. However,
with the aim of achieving a more accurate diagnosis, a new diagnostic method has been developed based on polymerase chain reaction and
applied in the study of Schistosoma mansoni
DNA in the cerebrospinal fluid (CSF). Objective:
The objective of this study was to assess the detection of Schistosoma mansoni DNA in CSF
using the Nested PCR technique in a single tube
(STN-PCR) for diagnosis of spinal cord schistosomiasis. Methods: This is a cross-sectional
study carried out from March 2013 to January
2014 at the Aggeu Magalhães Research Center/
FIOCRUZ (Pernambuco state, Brazil). STN-PCR
was used to detect Schistosoma mansoni DNA
in CSF samples from 20 patients with spinal cord
schistosomiasis and 30 controls. Results: STNPCR was positive in 16 patients with spinal cord
schistosomiasis and none from the control group
(sensitivity 80%; specificity 100%). Conclusion:
The STN-PCR technique is highly sensitive and
specific for diagnosis of spinal cord schistosomiasis and can be an important diagnostic tool,
particularly in cases with negative serology.
Barboza ICF1, Costa DT2, Santos ALMA2
• 1ESCOLA BAHIANA DE MEDICINA E SAÚDE PÚBLICA;
2
SERVIÇO DE IMUNOLOGIA, UNIVERSIDADE FEDERAL
DA BAHIA
AO-077
Introduction: The human T-lymphotropic virus
type‐1 (HTLV‐1) was the first human retrovirus
discovered. It causes a severe chronic neurological dis¬ease called HTLV‐1 associated myelopathy/tropical spastic paraparesis (HAM/TSP). The
infection is endemic in Brazil. Salvador, Bahia,
has the highest prevalence of infected individuals in the country (1.76%). Urinary symptoms are
present in 90% of patients with HAM/TSP and
can occur in isolation in patients without paraparesis, may precede it in some cases. Objective:
To characterize the clinical and laboratory neurogenic bladder associated with HTLV‐1 compared with patients with myelopathy associated
with HTLV‐1 (HAM/TSP). Methods: Cross-sectional study of 20 patients with neurogenic
bladder isolated and 20 patients with HAM/TSP.
Clinical analysis was based on the Expanded
Disability Functional Scale (EDSS) and laboratory investigation proviral load and cerebrospinal fluid testing. Results: The group HAM/
TSP, resulted in longer clinical evolution, greater disease progression, higher proviral load and
increased global cytology in cerebrospinal fluid
testing (p=0.05, 0.04, <0.01 and 0.01 respectively).
In the group with isolated bladder was identified
greater monocyte counts in cerebrospinal fluid
Maia BBV, Endres DC, Ruiz AV, Oliveira CR, Duarte DGB,
Orellana HB, Costa KS, Lucena LA, Fais VRM, Dib VBE,
Rabelo YD, Silva HM
EPIDEMIOLOGICAL AND ETIOLOGICAL PROFILE OF
BACTERIAL MENINGITIS IN TOCANTINS STATE
• UNIRG
Introduction: The Bacterial Meningitis is a high
morbidity and mortality disease in childhood,
38% of cases presents acute neurological complications. The inflammation of one or more meninges by bacteria characterizes the disease; it
can be acute or chronic. While the Koch bacillus
cause the Chronic Meningitis, pyogenic bacteria
cause the Acute Bacterial Meningitis (ABM). The
first symptoms of ABM are headache, irritability
and neck stiffness. Objectives: verify the occurrence of ABM in Brazil and in Tocantins State.
Besides the incidence in Tocantins, our study
evaluates the most prevalent microorganisms,
sex and age of affected people by ABM between
2009 and 2012. Methodology: retrospective descriptive study, using the database’s recorded
in “Sistema de Informação de Agravo de Notificações” of Brazil Health Ministry, saved in the
online base Tabnet (Epidemiological data tab).
The study considers the absolute number of
cases recorded in Tocantins and Brazil between
the four years. The Etiological profile (delimited
and nonspecific bacterial, ignored etiology or
aseptic infections), age groups (up to 1-year-old,
1‐9, 10‐19, 20‐39 and over 40) and sex were tabulated. Results: Brazil recorded 84622 cases in
four years, 270 of the sample were from Tocantins. The incidence per year average in Tocantins
was 67,5 cases, the biggest number of confirmed
cases was in 2009 (74) and the lower in 2010 (62).
During the four year of analysis, ABM affected at
most males, with incidence from 54 to 65%. On
2009 and 2010, most cases were in childhood
(1‐9 age group), in 2011 the incidence of this age
group was similar to the 10‐19 year old group,
but in 2012 the occurrence was bigger in the
10‐19 year group. Etiological analysis found that
the Bacterial Meningitis is the most common
in Tocantins, 61% (165) cases. Nonspecific and
other bacteria occupied the first most prevalent
group that causes ABM (bacteria different from
pneumococcus, Haemophilus sp and meningococcus), the second place was the other etiology
or aseptic group. Meningococcus and pneumococcus were the most prevalent bacteria of AMB.
In spite of, 2009 and 2010, registered cases of
BM caused by Haemophilus sp and tuberculosis
bacillus, each one with two cases. Conclusion:
Tocantins represents 0,32% of all cases reported
in Brazil. After analysis, we verified a change in
the most affected age group on the last two years.
ABM was more common in males and bacteria
were the main responsible for meningitis.
AO-078
MINI MENTAL STATE EXAMINATION AND
INTERNATIONAL HIV-DEMENTIA SCALE IN
ASSESSING NEUROCOGNITIVE MANIFESTATIONS
OF HIV/AIDS
Souza EM, Buoniconti CS, Valim FC, Moura AS
• UNIFENAS BH
HIV infection can cause neurocognitive disorders of different intensities, from mild to moderate until dementia. To assess neurocognitive
disorders from different etiologies, including
those caused by HIV, the MiniMental State Examination (MMSE) is widely used. However, it has
shown significance for cortical dementias (such
as Alzheimer’s), but not the same to subcortical
(as the ones caused by HIV). The aim of this study
was to compare the performance of Mini Mental
State Examination (MMSE) and the International
HIV Dementia Scale (IHDS), proposed by Sacktor
et al. in 2005 and validated in Brazil in 2013 by
Rodrigues et al. Methodology was based on the
consecutive recruitment of patients, who were
infected by the HIV, after the clinical evaluation
at the outpatient service at CEASC-BH. They answered first the Beck Depression Inventory (BDI)
and right after the MMSE and the IHDS were applied. Socio-demographic and clinical data were
obteined from medical records. As results, of the
63 enrolled patients, 45 were male, 62% were
black or mixed race, 44.4% were single and 60.3%
had completed primary education only. From
these, only five patients were not taking antiretrovirals medication. The average of the last CD4
cells count before the neurocognitive assessment
was 556.6cells/mm3, with two patients (3.2%)
under 200cells/mm3. Neurocognitive assessment
by the MMSE has shown that only two patients
would be below their cutoff point. Furthermore,
with the evaluation of IHDS 34 (54%) of the analyzed individuals had scores less than 10 (which
is the cutoff point for this test). The two patients
with MMSE underscore were also underscored
with IHDS, but 32 patients had IHDS below 10
with MMSE above the cutoff for schooling. Only
31
Oral Presentation
age was associated with IHDS, in patients over 50
presenting higher prevalence of abnormalities
(OR 7,36; IC95% 1,49‐36,5). IHDS was not associated with sex, viral loa dor BDI score. 58 patients
were on retrovirals, of those 30 (51.7%) were on
use of Efavirenz.The prevalence of changes in the
MMSE was too low to analyze possible associations with other factors. We conclude that more
than half of HIV-infected patients showed neurocognitives disorders according to IHDS and age
was being asssociated with a worse performance.
The MMSE seems to underestimate the meeting
of changes in this population.
AO-079
NEUROCOGNITIVE PROFILE OF HIV INFECTED
SUBJECTS IN COMPARISON TO A HEALTHY
POPULATION
Toledo PVM, Bertucci Filho DC, Miglorini EC, Favaro MG,
Olinek W, Krum LK
AO-081
Cognitive Neurology and
Neurology of Aging
Miyoshi E, Schamne MG, Latyki CL, Santos AFC
• UNIVERSIDADE ESTADUAL DE PONTA GROSSA
AO-080
ANALYSIS OF CLINICAL PROFILE OF PATIENTS
WITH ALZHEIMER’S DISEASE (AD) CONFIRMED
BY THE PRESENCE OF BIOMARKERS IN THE
CEREBROSPINAL FLUID (CSF)
Toledo AASF, Cunha NDS, Faraco CMF,
Seraidarian MBF, Resende EPF, Maia RD, Souza LC,
Guimarães HC, Beato RG, Figueiró M, Barbosa IG,
Caramelli P
• UFMG
• UEPG
Introduction: HIV infection is the most common
cause of neurocognitive deficits in the population
under the age of 50 years. According to the intensity of such neurological deficits, the HIV-associated neurocognitive disorders (HAND) can be
classified into a disease spectrum. HIV‐1-associated asymptomatic neurocognitive impairment
(ANI); HIV‐1-associated mild neurocognitive
disorder (MND) and HIV‐1-associated dementia
(HAD). In the present study we aimed to evaluate
the prevalence of cognitive deficits, as defined
by the IHDS, in addition to depression, cognitive complaints and level of day-to-day functioning among HIV-positive patients (HIV+) and
compare to a HIV negative population (HIV-), in
southern Brazil. Beyond that, we sought to determinate factors associated with a low IHDS score
in the HIV+. Objectives: To assess neurocognitive characteristics of adults infected by HIV
and acknowledge aspects associated with worse
cognitive performance. Method: Cross-sectional
study evaluating 101 HIV+ and 51 controls, >18
years old, without confounding neuropsychiatric
disorder. Data were gathered using a socio-demographical questionnaire, International HIV
Dementia Scale (IHDS), Beck Depression Inventory-II (BDI-II), Lawton Instrumental Activities
of Daily Living (IADL) Scale, mini–mental state
examination (MMSE) and a modified version of
the Patient Assessment of Own Functioning Inventory (PAOFI). Results: Of the HIV+, 56.43%
had an IHDS≤10. HIV+ subjects were less likely
to be in a stable union and to use alcohol, more
likely to use marijuana, antidepressants and
benzodiazepines, had lower MMSE scores and
higher BDI-II and PAOFI scores. Conclusions:
The cognitive performance of the HIV+ patients
is clearly worse than the control`s and the prevalence of low IHDS scores in this population is
high, especially among those on public health
system, although the majority of patients is experiencing the milder forms. Depression and
cognitive complaints were more important at
the HIV+ group. Finally, more studies are necessary to access the effects of the different HAART
schemes on neurocognitive function. Keywords:
HIV, AIDS Dementia Complex, Central Nervous
System, Highly Active Antiretroviral Therapy.
32
AVALIAÇÃO DO EFEITO NEUROPROTETOR E
COMPORTAMENTAL DOS INIBIDORES DA HMGCOA REDUTASE EM MODELO ANIMAL DA DOENÇA
DE PARKINSON
Introduction: Patients with diagnostic suspicion of AD are becoming more frequently. The
search for early diagnosis led to discovery of
biomarkers enabling the detection of central
pathological aspects of the disease. Neuropathology of AD includes accumulation of senile
plaques and neurofibrillary tangles in brain
tissue, which have as molecular counterparts
β-amyloid (Aβ42) and tau proteins, respectively. Because of the direct contact between
CSF and adjacent space to the brain, certain
concentrations of these proteins in this environment can be considered accurate markers
of AD pathophysiology. Objectives: To analyze
the clinical profile of patients with positive
biomarkers for AD in CSF. Methods: Statistical non-parametric analysis of AD biomarkers
concentration (Aβ42, total tau or T-tau and
phosphorylated tau or P-tau) in CSF of a group
of patients, comparing with their clinical profile. The research project was approved by the
institution’s ethics committee and all patients
signed term of consent to take part in the
study. Results: Twenty-four patients had been
evaluated (15 women and nine men) with positive results for AD biomarkers in CSF. The average age of onset of symptoms was 57.1±3.6
years in the pre-senile group and 73.0±5.4
years in the senile group. No significant correlation has been found between age of onset of symptoms and Aβ42, P-tau, T-tau concentrations in the whole sample. Correlation
between age of onset of symptoms and Aβ42
concentration in pre-senile group was almost
null (r=0.049), with moderate positive correlation in senile group (r=0.397). There was a very
weak positive correlation between age of onset of symptoms and P-tau concentration in
the pre-senile group (r=0.139), being moderate in the senile group (r=0.385). Correlation
between age of onset of symptoms and T-tau
concentration in the pre-senile group was almost null (r=–0.090), whilst the same correlation was strong in the senile group (r=0.524).
Conclusion: With advancing age, particularly
from 65 years, CSF biomarkers concentration
in AD show increasing tendency and positive
correlation, which supports a more accurate
determination of diagnosis.
Introduction: Parkinson‘s disease (PD) is a hypokinetic disorder due to decrease of dopaminergic
neurons at the nigroestriatal via. Statins (inhibitors of HMG-CoA) possibly have the ability to
modulate neurodegeneration because their anti-inflammatory, anti-oxidative and suppression
of aggregation of alpha-sinucleína effect. The
forced swimming test is a behavioral test that
can be used to evaluate the depression in an animal model of this disease. Therefore, this study
aims to assess the behavioral effects of statins
in animal models of PD induced by intracerebral administration of 6-OHDA by forced swimming test. Materials and methods: Male Wistar
rats were divided in two groups: sham-operated
controls treated with vehicle, sham-operated
treated with simvastatin or pravastatin 10 mg /
kg, nigrostriatal via injured and treated with vehicle, and nigrostriatal via injured and treated
with simvastatin or pravastatin 10 mg / kg. The
intracerebral injection of the toxin 6-OHDA was
performed by stereotactic surgery, which was
administered bilaterally 4μg toxin, dissolved in
0.2% ascorbic acid. The treatment of animals
was conducted orally and started 3 days before
surgery and lasted for 7 days. Fourteen days after surgery, animals were placed in a plastic cylinder over 15 minutes to a pre-test and after 24
hours were submitted to the test for 5 minutes.
The time of immobility, swimming and climbing
were quantified to assess depressive behavior.
Results and discussion The 6-OHDA + vehicle
group remained immobile a longer time when
compared to sham + vehicle, and the 6-OHDA
+ treatment showed lower time of immobility when compared to untreated injured group
(p<0.001). The animals of group 6-OHDA + vehicle showed a decrease in swimming time when
compared with SHAM + vehicle group (p≤0.001).
This reduction was reversed by treatment with
simvastatin or pravastatin at a dose of 10 mg / kg.
About the climbing time there was no significant
diferences between the groups. The results of this
study confirm what has already been shown by
other studies regarding the neuroprotective effect of estatinas. Conclusion: The results showed
that treatment with 6-OHDA was able to reproduce the cognitive impairments and that PD
treatment with statins is responsible for reverse /
prevent much of depressive-like behavior.
AO-082
CEREBROSPINAL FLUID “TAP - TEST” IN
OUTPATIENTS WITH CLINICAL IDIOPATHIC
NORMAL-PRESSURE HYDROCEPHALUS: A NEW
OBJECTIVE APPROACH
Okamoto IH, Vaccari AMH, Miranda RCAN, Lacerda SS,
Soares CAS, Bernal MMB, Specialli DS, Silva GS,
Tanuri CC, Cendoroglo Neto M, Massaud RM
• HIAE
Introduction: The “TAP-TEST” as a therapeutic proof of ventriculoperitoneal shunt efficacy
for idiopathic normal pressure hydrocephalus
(iNPH) has been used in many different ways
with few standardization, leading to difficulties
in evaluating the accuracy of the test. We present a series of patients with a clinical diagnosis of iNPH evaluated with a new standardized
XXVI Congresso Brasileiro de Neurologia
method, using a gait laboratory and a structured
neuropsychological evaluation. Methods: We
described the results of the TAP-TESTs of 68 patients with a clinical and imaging diagnosis of
iNPH, evaluated by the Neurology Program of a
tertiary hospital in Sao Paulo, from July 2012 to
July 2014. The “Tap-Test” was performed with a
complete neuropsychological assessment and
evaluation at a gait laboratory before and after
CSF puncture. Neuropsychological assessment
included evaluation of attentional / executive
processes, verbal memory, visuospatial skills,
motor processing speed and fine motor precision. At the gait laboratory we evaluated cadence
of steps, walking speed, step length, single support and kinematic data. The evaluations were
conducted about 24 hours before lumbar puncture and 2 to 4 hours after the exam. The CSF
was drained until the intracranial pressure was
halved. Results: We evaluated 68 patients (63.3%
men). The mean age was 77.5 (±6,02) years old
and the mean educational level 12.4 years (±4,45)
A total of 56 patients (82.4%) had a positive response to the “tap - test” (improvement in at least
one of the tests): 58% with improvement in one of
the items of the neuropsychological assessment,
53.1% with improvement in at least one of the
items of gait assessment and 28.8% improved in
both evaluations. Six months after the test, 47%
of the patients with a positive test underwent a
ventricular peritoneal shunt and 87.5% of those
patients had a significant clinical improvement.
We had no complications related to the exam.
Conclusions: The “tap - test” performed using
a new standardized method was very important
to support the clinical decision of the indication
of a ventricular peritoneal shunt in patients with
iNPH. It objectively measured the major changes in patients with iHPN pre and post puncture,
probably increasing the accuracy of the test for
detecting CSF shunting responsiveness. Further
evaluations comparing this new standardized
approach to the regular tap-test should bring
insights regarding the accuracy of both methods.
AO-083
CHANGES IN COGNITIVE FUNCTIONING AFTER
CEREBROSPINAL FLUID TAP TEST IN PATIENTS
WITH CLINICAL IDIOPATHIC NORMAL-PRESSURE
HYDROCEPHALUS
Lacerda SS, Boschetti WL, Kernkraut AM, Vacari AMH,
Okamoto IH, Silva GS
• HOSPITAL ISRAELITA ALBERT EISNTEIN
Introduction: Idiopathic normal-pressure hydrocephalus (iNPH) is a neurological syndrome
characterized by a triad of clinical symptoms
consisting of gait disorder, cognitive and urinary
dysfunctions. The cerebrospinal fluid (CSF) tap
test is recommended as a key step in the diagnosis of iNPH. Cognitive improvement after CSF
Tap Test can predict which patients with iNPH
will improve after CSF shunting Objectives: To
evaluate the results of CSF Tap Test in cognitive
functioning of patients with a clinical diagnosis
of iNPH. Methods: 64 patients with a clinical diagnosis of iNPH (mean age=77.55±7.80 years old;
years of schooling 12.74±5.53) underwent neuropsychological assessment before and 2 hours
after CSF Tap Test and the results were analyzed
using the t test for repeated measures. Results:
The results indicated significant performance
improvements after CSF-Tap Test related to attentional span (Digits Forward mean=4.87±1.24
and post CSF Tap Test mean=5.28±1.36,
p<0.001), sustained attention (Trail Making part
A mean=135.98±101.82 and post CSF Tap Test
mean=122.71±106.49, p=0.030), divided attention (Trail Making part B mean=288.22±150.21
and post CSF Tap Test mean=259,96±157.42,
p=0.009), immediate memory (RAVLT A1
mean=3.20±1.89 and post CSF Tap Test
mean=3.63±1.56, p=0.019), visuospatial skills
(Block design mean=17.73±9.31 and post CSF
Tap Test mean=20.08±10.21, p=0.002) processing speed (Digit Symbol mean=24.17±15,78 and
post CSF Tap Test mean=27.24±18.12, p=0.001
and Searching Symbols mean=11.14±7.35 and
post CSF Tap Test mean=13.12±8.99, p<0.001).
For working memory, delayed memory, verbal
learning, verbal fluency and fine motor precision
no significant differences pre-and post CSF Tap
Test were observed. Conclusion: Comparison
of the cognitive performance of patients with a
clinical diagnosis of iNPH pre and post-CSF Tap
Test demonstrated that participants experienced
significant improvements in attentional skills,
immediate memory, and visuospatial processing
speed but not in working memory, delayed memory, verbal learning, verbal fluency and fine motor precision. The implications of those findings
in the long-term outcome after CSF shunting
procedure have to be further evaluated.
AO-084
INTER-HEMISPHERIC ACTIVATION ASYMMETRY
AND TRIAL-TO-TRIAL FLUCTUATIONS IN
ALERTNESS: AN ELECTROPHYSIOLOGICAL AND
PUPILLOMETRIC INVESTIGATION
Newman DP1, Loughnane GM2, Abe R3, Zoratti MTR3,
Kelly SP4, O‘Connell RG2, Bellgrove MA1
• 1MONASH UNIVERSITY; 2TRINITY COLLEGE DUBLIN;
3
UNIVERSIDADE FEDERAL DE MATO GROSSO; 4CITY
COLLEGE OF THE CITY UNIVERSITY OF NEW YORK
Introduction: Corbetta and Shulman’s model of
networks for attention suggests that alertness is
underpinned by a right-hemisphere-lateralized
ventral network, which receives slightly asymmetric (right > left hemisphere) input from the
brainstem locus coeruleus/norepinephrine (LC/
NE) system. Supporting this, the well characterised increase in parieto-occipital alpha (8‐14Hz)
with decreased alertness may occur in concert
with a rightward shift in the inter-hemispheric balance of alpha power (alpha asymmetry),
such that alpha increases more over the right
relative to the left hemisphere with time-on-task.
Such time-on-task manipulations capture tonic
decreases in alertness but fail to account for trial-to-trial fluctuations in alertness which are also
influenced by LC/NE. Thus we sought to test the
hypothesis that parieto-occipital alpha asymmetry is related to pre-target pupil diameter on a trial-by-trial level. Methods: Healthy participants
(50) were exposed to a repetitive random dot
motion task lasting ~40mins while pupillometry,
EEG and behaviour were recorded. Participants
were required to make a speeded button press if
they detected coherent motion in the left or right
hemifield. Results: As expected the increase in
alpha power with time-on-task occurred with a
significant rightward shift in hemispheric alpha
asymmetry and a significant decrease in behavioural accuracy. Further, reaction time was
significantly slower on trials with more rightward
pre-target alpha asymmetry (particularly for
left hemifield targets) and on trials with smaller
pre-target pupil diameter. Interestingly, participants displayed significantly larger pre-target
pupil diameter during trials with more rightward
pre-target alpha asymmetry. Conclusion: We
show that greater alpha activity over the right
relative to the left hemisphere is linked with
decreased alertness and slower reaction times
to left-hemifield targets. Pre-target hemispheric alpha asymmetry was modulated by tonic
alertness (time-on-task) as well as trial-to-trial
fluctuations in alertness (as measured by pupil
diameter). These results support Corbetta and
Shulman’s model which explains the common
coupling of spatial and alertness deficits after
right hemisphere damage.
AO-085
IS THE IMPAIRMENT OF THE VERB PRODUCTION IN
NON-FLUENT VARIANT OF PRIMARY PROGRESSIVE
APHASIA ASSOCIATED WITH DAMAGE IN THE
MOTOR SYSTEM?
Beber BC1, Mandelli ML2, Santos MA2, GornoTempini ML2, Chaves MLF1
• 1HCPA; 2UCSF
Introduction: Patients with the nonf-luent variant of Primary Progressive Aphasia (nfvPPA) can
have more difficult to produce verbs than nouns
and this can be due to the grammar impairments
or to the atrophy in some brain regions involved
in the motor system. Indeed, according with the
theory of embodied cognition, motor brain areas
are engaged in the semantic representation of
action words (as the verbs). Aim: to test the hypothesis of the impairment in verb production
due to the damage in the motor system we assessed the production of verbs in the nfvPPA using a naming test with an effect of manipulability.
Methods: We elaborated a naming test, with an
action naming (AN) and an object naming (ON).
The AN was composed of 16 action pictures
(verbs), and the ON of 16 object pictures (nouns).
The groups of AN and ON pictures were matched
to psycholinguistic features. Half of each group
of pictures was composed of pictures involving
manipulable content. We evaluated a group of
patients with diagnosis of nfvPPA (Gorno-Tempini et al., 2011) and a group of healthy control subjects at the Memory and Aging Center at UCSF.
All participants were native American English
speakers. The total score was the total number
of words named correctly in the AN and in the
ON. We performed a Voxel-based Morphometry
on T1-weigthed images of the brain of these subjects to investigate the neural correlates of AN
and ON. Eleven patients with nfvPPA (5 male, 8
right handed, age range of 69.1±8.56, education
of 16.1±2.68) and 9 control subjects (6 male, 8
right handed, age range of 66.38±4.69, education
of 17.44±1.42) were evaluated. Results: When the
performance of the nfvPPA patients was compared to the control subjects, the patients performed significantly fewer in both naming test,
but the statistical difference was stronger for the
AN(p=.006) than the ON(p=.019). Besides that,
the AN was significantly worst than the ON into
the nfvPPA group (p=.004) and we did not find a
manipulability effect. We found a correlation between the performance in the AN and the brain
atrophy in Broadmann area 44 and in the precentral gyrus. Conclusion: We did not find effect of
manipulability in the performances, but the difficulty to name actions might be associated with
the motor system due the correlations found between the performance and the atrophy in brain
areas involved in the motor system. Further studies are needed using additional motor effects in
the pictures of the naming test.
33
Oral Presentation
AO-086
MR-SPECTROSCOPY MARKERS OF NEURONAL
DEPOPULATION MEDIATE CHAGAS DISEASEASSOCIATED COGNITIVE DYSFUNCTION
Resende LL, Fernandes RD, Dias JS, Jesus AA,
Andrade AL, Gonçalves BMM, Reis CC, Ferreira IL,
Lordelo MJ, Barreto Neto N, Abbehusen C, Reis F,
Jesus PA, Oliveira Filho J
• UFBA
Background: Chagas disease (CD) is an important cause of stroke and cardiac disease. However,
cognitive dysfunction may occur independently
of these factors. We aimed to evaluate the effect
of CD on cognitive function and to investigate
the brain mechanisms underlying CD-associated
cognitive dysfunction. Methods: Cross-sectional
study of a cohort of patients from a Heart Failure
(HF) clinic. Stroke-free patients underwent prospective clinical data collection including a battery of cognitive tests, used to generate Z-scores
on each cognitive domain (visuospatial, executive function and long-term memory). Brain
magnetic resonance imaging (MRI) was performed to quantify several volumetric imaging
parameters (brain volume, cerebellar volume,
white matter hyperintensity volume – all corrected for intracranial volume) and MRI spectroscopy to quantify N-acetyl-aspartate to creatine
ratio (NAA/Cr), choline to Cr ratio (Cho/Cr) and
myoinositol to Cr ratio (mI/Cr). Linear regression models adjusted for age, sex left ventricle
ejection fraction and educational level were used
to test the association between CD and cognitive
dysfunction; and to examine potential mediation by each imaging marker. Results: We studied 281 patients, mean age 55 (± 13) years, 157
(54%) female, 124 (47%) with CD. MR spectroscopy data was available in 145 patients and MR
volumetric data in 95 patients. Patients with CD
performed significantly worse compared with
non-CD patients on visuospatial (p<0.001) and
executive function domains (p=0.008), but not
memory (p=0.567). In the multivariable model,
CD was significantly associated with visuospatial function (effect/standard error=‐0.23/0.097,
p=0.021), but not executive function or memory
(p>0.5). CD was associated with lower NAA/Cr
(p=0.011) and Cho/Cr (p=0.023), both decreasing the effect of CD on visuospatial function by
>80% when added to the model. Conclusions:
CD is associated with cognitive deficits mostly
in the visuospatial domain, independently of
main confounders. The effect of CD on cognition
seems to be mediated by neuronal depopulation
without significant white matter disease or total
brain/cerebellar volume loss.
AO-087
NEUROPSYCHIATRIC SYMPTOMS OF PATIENTS
WITH BEHAVIORAL VARIANT FRONTOTEMPORAL
DEMENTIA COMPARED WITH PATIENTS
WITH ALZHEIMER’S DISEASE: FREQUENCY,
CORRELATION AND CAREGIVER DISTRESS- BRAZIL
Bahia VS1, Lima-Silva TB1, Carvalho VA2, Guimarães HC2,
Caramelli P2, Balthazar MLF3, Damasceno B3,
Bottino CMC4, Nitrini R1, Yassuda MS1
• 1HCFMUSP; 2UFMG; 3UNICAMP; 4PROTER HCFMUSP
Objectives: Neuropsychiatric symptoms are
more prevalent in certain types of dementia.
Frontotemporal Dementia (FTD) is an umbrella term for a group of dementia syndromes that
present clinically without amnesia, especially in
the early stages. It interferes significantly in the
patient’s autonomy and it causes high physical,
34
financial and emotional tribulation to the family nucleus, leading to early institutionalization.
The objectives of this study were: to identify the
most frequent and more severe neuropsychiatric
symptoms in patients with behavioral variant
FTD compared to patients with Alzheimer disease (AD) and to evaluate which disease causes
more distress to the caregiver. Methods: Sixty-two caregivers, aged 55 or older, with at least
two years of formal education, were invited to
participate. 31 provided care for patients with
bvFTD and 31 for those with AD. bvFTD and AD
patients were matched according to the severity
of the disease, based on the Clinical Dementia
Rating (CDR). The NPI and NPI-D were applied
to the caregivers and the symptoms were analyzed with exploratory factor analysis. Results:
bvFTD caregivers reported higher frequency
and severity of neuropsychiatric symptoms, as
well as higher distress, when compared to AD
caregivers. Significant differences were found
between these groups for: delusions, agitation,
apathy/indifference, disinhibition, irritability/
lability, total score of behavioral and neuropsychiatric symptoms, and total caregiver distress.
Conclusion: The results reinforce the need for
studies regarding the neuropsychiatric manifestations in bvFTD, as they seem to be associated
with high degree of caregiver distress.
AO-088
PERFORMANCE ON THE IOWA GAMBLING TASK
OF INDIVIDUALS WITH SUBSTANCE DEPENDENCE
AND ABSENCE OF ANATOMICAL LESION IN THE
PRE-FRONTAL CORTEX
Chandretti GS1, Hohl R1, Mourão Junior CA2, Mota TR2
• 1SUPREMA; 2UFJF
The validation of psychometric tools is usually
performed associating cognitive tasks with brain
lesions. The Iowa gamblig Task (IGT) is considered as the gold standard for assessing the executive functions in decision-making. In this sense,
the IGT validation was performed on subjects
with anatomical lesions in the ventromedial
prefrontal cortex (CPV). Currently, IGT has been
used to demonstrate that individuals with substance dependence (ISD) have decision-making
impairment similarly to subjects with lesion to
the CPV. Therefore, substance dependence could
be characterized as a disease that compromises
the top-down control whose diagnosis would be
attested by the IGT performance. However, the
support of IGT as a tool for top-down dysfunction
diagnosis in non-injured subjects needs caution.
Objective: To test two IGT assumptions in ISD:
(1) ISD show homogeneous choice behavior that
characterizes the clinical population and (2) ISD
do not learn to prefer the good options over the
bad options during the IGT. Methods: The IGT
was performed by 32 men with poly-substances
dependence (alcohol, cocaine, crack, marijuana
and tobacco, 32.2±6.8 years old, 14.4±2.6 years
of school education) in a volunteer treatment facility. All subjects were assessed for DSM-IV substance dependence. In the IGT, participants are
presented with four decks of cards (A, B, C, D);
each card yields a reward and, occasionally, also
a loss. The decks A and B have high immediate
gains with long-term losses, therefore, bad choices. The decks C and D have low immediate gains
with a positive balance in the long term, therefore, good choices. Subjects with CPV damage
usually shows >50% of bad choices (i.e. A and B)
characterizing a “myopia” of future consequences. Results: The ISD preferred the good decks C
and D (mean ± sd: 36±25 and 29±17 cards respectively) in 100 choices. Twelve subjects relapsed
within two months after the IGT, but only three
ISD preferred >50% of cards A and B. Moreover,
the ISD showed idiosyncratic choice behavior.
Conclusions: This study challenges the assumption that every ISD with no history of anatomical
brain lesion suffer from a brain disease related
to severe decision-making deficits that could be
assessed by the IGT. Alternatively, the voluntary
seeking for treatment characterizes itself a major
advantageous decision of real life that may justify
the preference for choices C and D in this study.
R. Hohl received scholarship from CAPES/PNPD.
AO-089
THE CAREGIVER BURDEN AND WEAR IN
BEHAVIORAL VARIANT FRONTOTEMPORAL
DEMENTIA AND ALZHEIMER DISEASE
Silva TBL1, Bahia VS1, Carvalho VA2, Guimarães HC2,
Caramelli P2, Balthazar MLF3, Damasceno B3,
Bottino CMC4, Brucki SMD1, Nitrini R1, Yassuda MS1
• 1HCFMUSP; 2UFMG; 3UNICAMP; 4PROTER HCFMUSP
Introduction: Despite recent advances in characterizing behavioral variant frontotemporal
dementia (bvFTD), diagnosis remains challenging as less is known about its cognitive and
functional profile, compared to AD. Objectives:
1. Compare caregiver burden in bvFTD and AD.
2. Examine which factors contribute to bvFTD
and AD caregivers’ burden and wear. Methods:
Sixty-two caregivers, aged 55 or older and with
at least two years of formal education, were invited to participate. Among patients, 31 had
been previously diagnosed with bvFTD and 31
with AD. bvFTD and AD patients were matched
according to the, severity of the disease, based
on the Clinical Dementia Rating (CDR) scores.
The caregivers’ protocol included: Short Zarit
Burden Inventory; caregiver wear and patient´s
neuropsychiatric and behavioral symptoms from
the Neuropsychiatric Inventory (NPI); Disability
Assessment for Dementia (DAD) and Cornell
Depression Scale in Dementia. The patients’
protocol included: Addenbrooke‘s Cognitive
Examination-Revised (ACE-R); Executive Interview (EXIT‐25); Direct Assessment of Functional Status (DAFS-BR), and Geriatric Anxiety
Inventory (GAI). Results: Caregivers of patients
with AD and bvFTD did not significantly differ
according to age, schooling and sex. In the NPI,
bvFTD caregivers reported higher presence and
severity of neuropsychiatric symptoms, as well
as caregiver distress, when compared to the AD
group. Patients´ psychiatric symptoms correlated significantly with caregiver wear in the NPI.
In the bvFTD group only, DAD and DAFS-BR
scores were strongly correlated with caregiver
burden (ZARIT) (DAD- AD-rho=‐0.09, p<0.648;
bvDFT rho=0.63, p<0.001; DAFS-BR- AD- rho=
‐0.11 ,p<0.538; bvFTD-rho=0.55, p<0.001;). Also,
in the bvFTD group only, cognitive variables
were strongly correlated with caregiver burden (ZARIT), (ACE-R- AD rho= ‐0,12 e p<0.527;
bvFTD -rho=‐0.56, p<0.001; EXIT‐25- AD;
rho=‐0.18 e p<0.338; bvFTD rho=0.46, p<0.009).
Conclusions: bvFTD caregivers seem to experience higher levels of burden and wear than AD
caregivers. Patients’ cognitive and functional
limitations, as well as their psychiatric symptoms seem to be associated with higher burden
and wear in bvFTD caregivers. These findings are
relevant for the planning and delivery of support
for families, reinforcing the need for tailored
caregiver support that should take into account
the disease characteristics.
XXVI Congresso Brasileiro de Neurologia
AO-090
TIME PERCEPTION AND AGE
Ferreira VFM, Paiva GP, Prando N, Graça CR,
Kouyoumdjian JA
• FAMERP
Introduction: Time perception varies individually and probably there is a relationship with
aging. It is a common sense the statement that
“time goes faster when we got older”. Although
the sense that we perceive time as accelerating
as we age is very common, it is hard to prove experimentally. The neurotransmitter dopamine
is critically important to our ability to process
time; attention and memory also play a part in
it. So, to accurately measure time interval with
confidence, persons have to be able to focus and
remember a sequence of tasks or information.
Objectives: Measure the time interval of 2 minutes counted mentally in subjects of different age
groups. Method: Two-hundred and thirty-three
healthy subjects, 129 women, were studied. They
were divided into 3 age groups: G1, 15‐29 years,
n=86, mean age =22.4 years; G2, 30‐49 years,
n=76, mean age =38.9 years; G3, 50‐89 years,
n=71, mean age =59.7 years. The subjects were
asked to close their eyes and mentally count the
passing of 120 seconds; when finished up, the
examiner took the elapsed time. Results: All the
time values (seconds) from the groups had normal (Gaussian) distribution. G1, mean =114.9±35
seconds; G2, mean =96.0±34.3 seconds; G3,
mean =86.6±34.9 seconds. The ANOVA-Bonferoni multiple comparison test-showed very
significant comparison G3 versus G1 (P<0.001).
Conclusion: Mental calculation for 120 seconds
time interval was reduced in average 24.6% (28.3
seconds) in persons over 50 years of age when
compared to young persons up to 30 years of age.
In diseases, it could be related to dopamine decrease. In healthy subjects, aging are usually related to decrease interest in learning something
new, that requires sustained effort, slowing down
our internal sense of time.
Neurology of Sleep /
Polysomnography
AO-091
EXCESSIVE FRAGMENTARY MYOCLONUS: A NEW
SLEEP DISORDER IN THE CLINICAL SPECTRUM OF
MACHADO-JOSEPH DISEASE
Santos DF, Silva GMF, Pedroso JL, Braga Neto P,
Prado LBF, Cavalho LBC, Barsottini OGP, Prado GF
• UNIFESP
Introduction: Machado-Joseph disease (MJD)
is a neurodegenerative disease which usually presents several clinical findings including
cerebellar ataxia and other extracerebellar
features, such as parkinsonism, dystonia, peripheral neuropathy, and lower motor neuron
disease. Some data have demonstrated a high
frequency of sleep disorders in these patients,
including excessive daytime sleepiness (EDS),
insomnia, obstructive sleep apnea (OSA), rapid eye movement (REM) sleep behavior disorder (RBD), and restless legs syndrome (RLS).
Objective: This study aims to describe the
frequency of excessive fragmentary myoclonus (EFM) in MJD. Methods: We recruited 44
patients with MJD and 44 controls, matched
for sex and age, without MJD. All participants
underwent an all-night polysomnography
(PSG). EFM was evaluated and defined in accordance to the criteria of the American Academy of Sleep Medicine (AASM). Results: Half of
the MJD patients (n=22) had EFM diagnosed
through PSG, though no healthy control participant presented this finding (P<0.0001). In
the MJD group, older participants and men
had a higher frequency of EFM. There was no
correlation between EFM and the following
data: body mass index (BMI), apnea-hypopnea index (AHI), EDS, loss of atonia during
REM sleep, periodic limb movements during
sleep (PLMS), RLS, RBD, ataxia severity, the
number of cytosine-adenine-guanine trinucleotide (CAG) repeats, disease duration, sleep
efficiency, sleep fragmentation, and sleep stage
percentages between patients with or without
EFM. Conclusion: EFM is highly prevalent in
patients with MJD. This study demonstrates
that EFM must be included in the clinical spectrum of sleep disorders in MJD patients.
AO-092
NON-REM SLEEP PARASOMNIAS AND
MACHADO-JOSEPH DISEASE: CLINICAL AND
POLYSOMNOGRAPHIC EVALUATIONS
Silva GMF, Pedroso JL, Santos DF, Braga Neto P,
Prado LBF, Cavalho LBC, Barsottini OGP, Prado GF
• UNIVERSIDADE FEDERAL DE SÃO PAULO
Introduction: The spinocerebellar ataxias
(SCA) are a group of neurodegenerative diseases that have a marked clinical and genetic
variability. The spinocerebellar ataxia type 3
(SCA 3) or Machado-Joseph disease (MJD) is
the most common worldwide. MJD is classically characterized by progressive ataxia and variable other motor and non-motor symptoms.
Sleep disorders are common and include: REM
sleep behavior disorder (RBD), restless legs
syndrome (RLS), insomnia, excessive daytime
sleepiness, excessive fragmentary myoclonus
and sleep apnea. Objective: This study aims
to characterize and to determinate the frequency of the parasomnias, mainly non-REM
sleep parasomnias, in MJD disease compared
to a control group. Materials and Methods:
Forty-seven patients with clinical and genetic
diagnosis of MJD and 47 control subjects were
evaluated clinically and by polysomnography.
Results: MJD patients had a higher frequency of parasomnias complaints (confusional
arousal/seep terrors p=0.001, RBD p<0.001
and nightmares p<0.001), arousals from slow
wave sleep (p<0.001), REM sleep without atonia (p<0.001), periodic limb movements of
sleep-PLMS index (p<0.001), duration and percentage of N3 sleep (p<0.001) and percentage
of N1 sleep (p<0.001). Conclusion: These data
reinforce that REM sleep parasomnias and now
non-REM sleep parasomnias must be included in the spectrum of sleep disorders in MJD
patients.
Child Neurology
AO-093
A CHRONICLE ADMINISTRATION OF AGOMELATINE
REVERT BEHAVIORAL PARAMETERS OF RATS
SUBMITTED TO PRENATAL VALPROIC ACID
EXPOSURE
Rodrigues AB, Alves ASB, Staichok G, Trombetta TPZ,
Orige CB, Fortunato JJ
• UNISUL
Introduction: Agomelatine is a melatonin agonist that is being used as an antidepressant
with a distinct pharmacological profile due to
its mechanism of action. Features the ability
to resynchronize circadian cycles, influencing
the quality and continuity of sleep. Sleep is
important for the healthy development of children and previous investigations indicate that
approximately 50‐89% of autistic children have
changes in sleep patterns. Objective: Based
on these findings, the aim of this study was to
evaluate the behavioral effects of agomelatine
in an experimental model of autism. Methods:
Pregnant Wistar rats received administration
of valproic acid (VPA) (600 mg / kg) or saline
on the 12th gestational day. The male offspring
received chronic treatment of agomelatine
(10 mg / kg) or saline for 14 consecutive days
from postnatal day 22. Tests of social interaction, stereotyping and habituation to the open
field were used to assess autistic behavior of
animals with 36‐40 days of life and the effect
of agomelatine on these same parameters.
The results were analyzed as mean and standard error of the mean by the T Student test
for comparison between two groups or ANOVA for multiple comparisons between groups.
Results: The results showed that rats exposed
to prenatally to VPA exhibited autistic behavior from the reduced social approach search
and reduction of stereotypic behavior. These
parameters were reversed by treatment with
agomelatine. Conclusion: Our findings suggest that it is possible a relationship between
changes in melatonergic system and clinical
manifestations of autism and that manipulating this system, from the treatment with agomelatine, may represent a potential therapeutic target for the treatment of this disorder.
AO-094
CENTRAL ADMINISTRATION OF UFP-101 CAUSES
CHANGES IN METABOLISM ENERGY OF RATS
SUBMITTED TO THE EXPERIMENTAL MODEL OF
AUTISM
Alves ASB, Schlickmann E, Fortunato JJ, Gomes TM,
Orige CB, Soares ECC, Martins AOL, Anacleto D,
Schraiber R, Rezin GT, Rodrigues AB
• UNISUL
Introduction: Several studies have suggested the
involvement of the endogenous opioid system to
behavioral dysfunction observed in autism spectrum disorders. In particular, the nociceptin/
orphanin FQ (N/OFQ) system and its receptor
(NOP) seem to be involved in various physiological functions, including levels of anxiety,
35
Oral Presentation
depressive character, besides working on the
development of the central nervous system. An
increasing number of preclinical studies have
demonstrated that blocking the NOP receptor
leads to changes in neurotransmitter systems,
particularly the serotonergic system. Objective:
Given these findings, the aim of this study was to
investigate the effects of central administration
of UFP‐101 (NOP receptor antagonist) on behavioral and neurochemical parameters in an experimental model of autism. Methods: Pregnant
Wistar rats received administration of valproic acid (VPA) (600 mg/kg) or saline on the 12th
gestational day. The male pups (60‐65 days old)
underwent stereotactic surgery for implantation
of guide cannulae and intracerebroventricular
administration of UFP‐101 (2μL) or PBS as the
experimental group. Tests of habituation to the
open field was used to assess motor activity and,
immediately after, the brain was removed to assess the mitochondrial respiratory chain activity.
Results: The central administration of UFP‐101
in group exposed to VPA in the prenatal period
significantly reduced the number of crossing
movements, and did not change significantly the
number and the time of grooming and the number of rearing. The activity of complex IV of the
mitochondrial respiratory chain was inhibited in
the prefrontal cortex and, similarly, the activity of
creatine kinase (CK) is also reduced in the hippocampus and prefrontal cortex. Conclusion:
Taken together, these results reveal that blocking
neuropeptide NOP through antagonist UFP‐101,
may influence certain behaviors seen in autism,
however was not able to reverse the loss of energy homeostasis caused by prenatal administration of VPA.
AO-095
CLINICAL AND BIOCHEMICAL FEATURES IN
COENZYME Q10 DEFICIENCY
Arita JH1, Pedroso JL1, Barros MH2, Picosse FR1,
Ravagnani FG3, Masruha MR1, Barsottini OGP1,
Barros CCF3
• 1UNIVERSIDADE FEDERAL DE SÃO PAULO;
2
UNIVERSIDADE SÃO PAULO; 3INSTITUTO DO
CÉREBRO – INCE - HOSPITAL ISRAELITA ALBERT
EINSTEIN
Introduction: Coenzyme Q10 (CoQ10) deficiency is a mitochondrial disorder with clinical and genetic heterogeneous presentations.
Encephalomyopathy with recurrent myoglobinuria, severe infantile multisystemic disease, cerebellar ataxia, isolated myopathy and
nephrotic syndrome are the main phenotypes
described. The aim of this study is to identify
patients with suspected CoQ10 deficiency and
perform their clinical and biochemical characterization. Methods: Twenty-six suspected patients between 0‐20 years old were selected and
submitted to clinical and laboratorial investigation. Fibroblast cell lines acquired from skin biopsies were submitted to chromatografic analysis and the Ultimate 3000 High Performance
Liquid Chromatography measured CoQ10 levels. Enzyme activity from complexes I+III and
II + III were analyzed. Results: Ten patients
(38%) had low CoQ10 concentrations when
compared to controls. Five of them (50%) had
cerebellar ataxia, two (20%) had encephalomyopathy, one (10%) had infantile multiystemic
form, one (10%) with atypical presentation and
36
one (10%) with Leigh syndrome. Except for one
patient, biochemichal assays with complexes I
+ III, II + III, II and III measurements did not
show a direct correspondence with low CoQ10
patients. Discussion: results suggest that it is
possible to detect low CoQ10 levels in a large
proportion of patients with atypical ataxia, for
whom other types of ataxia were previously excluded. However, biochemical assays showed
that the majority of low CoQ10 levels patients
may have a secondary form of coenzyme deficiency. Conclusion: characterization of CoQ10
deficiencies with clinical and biochemical features may help us understand and improve diagnosis. Once it is a treatable condition, early
detection may change prognosis.
AO-096
TWIN RISKS: RELATIONSHIP BETWEEN RISK
FACTORS AT BIRTH AND MOTOR DEVELOPMENT
DELAY
Almeida M, Zonta MB, Antoniuk S, Bruck I
• UFPR
Introduction: A twin pregnancy is a condition
where different risk factors and specific diseases can impair motor development (MD).
MD is considered a predictor of health and its
monitoring is critical to early identification of
problems and referral to appropriate interventions.Objectives: To describe and analyze the
MD of twins at risk in the first two years of life,
to identify the obstetric and neonatal risk factors that were associated with motor delay and
to determine the prevalence of Cerebral Palsy
(CP) in the sample. Material and Methods: A
retrospective analysis of the medical records of
twins that were discharged from the Intensive
Care Unit and followed in the Multidisciplinary
Outpatient Clinic for High Risk Infants in the
Clinics Hospital of UFPR. It was considered
the Denver Developmental Screening Test II
applied at 6, 12 and 24 months. Results: There
were considered the medical records of 104
twin at risk; 79 (75,9%) were preterm,10 (9,7%)
term infants, and in 15 (14,4%) records there
wasn’t this information. Low birth weight was
observed in 101 (97,1%) twins, 41(39,4%) were
classified as large premature. The 5-minute Apgar score below 7 was observed in 14(13.4%),
30 (28.8%) made use of Mechanical Ventilation
(MV ),5 (4.8%) had Bronchopulmonary Dysplasia (BPD), 24 (23,1%) to Hyaline Membrane
Disease (HMD), and 18 (17.3%) Intraventricular Hemorrhage (IH). Among the 104 records
analyzed just 35 (33,3%) contained detailed information on motor development at 6, 12 and
24 months of age (Denver Test). For these twins
the motor delay was compared to risk factors.
The use of MV was related to the presence of
BPD and HMD (p=0.001). Low birth weight
was related to the presence of IH (p=0.004).
The Twin-Twin Transfusion Syndrome was observed in 9(8.7%) without association with motor delay and the CP. In this sample 14 (13.5%)
of twins were diagnosed having CP and the
remaining were considered having appropriate MD in the Denver Test at two years of age.
Conclusion: In this sample the main risk factors that were related to the delay in MD were
prematurity, low birth weight, use of VM and its
relationship with the HMD and BPD.
Interventional Neurology
AO-097
ANGIOPLASTY WITH STENT IN SYMPTOMATIC
CAROTID STENOSIS IN SUS: RETROSPECTIVE CASE
SERIES ANALYSIS
Carneiro CC1, Schulz VC2, Magalhães P2, Lovatel CC1,
Amorim FK3, Moro CHC2, Tamesawa CS1, Menegatti RS4,
Zalli M2
• 1UNIVILLE; 2HMSJ; 3CLINICA NEUROLÓGIA DA
JOINVILLE; 4HOSPITAL MOINHOS DE VENTO
Introduction: Atheromatous disease of the supra-aortic arterial trunks is responsible for about
20‐30% of isquemic cerebrovascular accidents.
Historically the NASCET and ACAS studies have
demonstrated the benefits of carotid endarterectomy in lowering the risk of stroke in patients
with moderate to severe carotid stenosis. Recently, with the publication of the CREST study and
meta-analysis comparing angioplasty with stenting versus endarterectomy, endovascular treatment of carotid stenosis has become an option
in the management of these patients, however
information about security method and morbidity rates are scarce in the Brazilian population.
Objective: To retrospectively analyze the number
of patients admitted to the São José Municipal
Hospital of Joinville, diagnosed with stroke and
TIA determined by carotid stenosis, who underwent treatment with angioplasty and stenting.
Method: We analyzed the clinical and surgical
data of all with carotid stenosis>60% in symptomatic vessel that underwent carotid angioplasty with stenting, in the period January 2012 to
December 2013. All procedures were performed
by the staff of Interventional Neuroradiology. As
outcomes, we analyzed mortality in the first 30
days, worsening neurological detected by an increase ≥3 point scale NIH and acute myocardial
infarction. Results: Of the 84 patients treated,
the average age was 65.2 years, 65 were male and
83.3% were admitted to the stroke unit. The most
prevalent risk factor was hypertension (83.3%).
The severity of the ischemic insult, as measured
by the NIH scale, was 5.2 at admission and 1.89 at
discharge. On average, patients underwent therapeutic procedure 19 days after hospital admission. Only 3 (3.75%) showed clinical outcomes
related to endovascular treatment. One patient
had cerebral hyperflow syndrome, 01 worsensed
after the procedure attributed to distal embolization of the atheromatous plaque fragments and
01 asymptomatic hemorrhagic transformation
in the area of cerebral ischemia. Conclusion:
The endovascular management of symptomatic
carotid stenosis appears to be a feasible and safe
therapeutic strategy in HMSJ Joinville. The analysis of the performance of the method provides
data for clinical decision making.
XXVI Congresso Brasileiro de Neurologia
Peripheral Neuropathy
AO-098
99M TC- DPD CARDIAC SCINTIGRAPHY IN
FAMILIAR AMYLOIDOSIS
Amaral CBMS, Nascimento OJM, Pires KL,
Coutinho IBR, Prado FM
• UNIVERSIDADE FEDERAL FLUMINENSE
Background: Familiar amyloidosis is a group of
diseases characterized by tissue deposition of
amyloid fibrils. There are three main types of familiar amyloidosis: transthyretin (TTR), apolipoprotein A1 and gelsolin. Cardiac involvement is
a leading cause of morbidity and mortality; one
new described mutation strongly related with
isolated cardiac amyloidosis is the TTR Val122Ile.
The discovery of tests that allow early diagnosis of cardiac involvement in amyloidosis, and
inferred that the etiology of the disease is of utmost importance. Objectives: In a cohort of patients with different types of familiar amyloidosis
(TTR and gelsolin), we aimed to assess the role of
99mTc‐3,3-diphosphono‐1,2-propanodicarboxylic acid (99mTc-DPD) in detecting myocardial
amyloid infiltration. Methods: We enrolled six
patients diagnosed with late familiar amyloidosis, which mutations were documented at deoxyribonucleic acid analysis: three patients with
TTR Val30Met mutation, two patients with gelsolin mutation and one patient with TTR Val122Ile mutation. Five patients were asymptomatic for
cardiac involvement and one patient (Val122Ile
mutation) had a previous diagnosis of heart
failure. Myocardial uptake of 99mTc-DPD scintigraphy was semiquantitatively and visually assessed at five minutes and three hours. Results:
The uptake of 99mTc-DPD highly demonstrated
amyloid in cardiac area in two out the three cases of TTR Val30Met and in TTR Val122Ile, and no
uptake in gelsolin cases. TTR Val122Ile case presented the highest uptake due to the exclusive
deposition of amyloid in cardiac area resulting in
severe heart failure. Conclusion: In hereditary
transthyretin-related amyloidosis, including the
mutations TTR Val30Met and Val122Ile, 99mTcDPD cardiac scintigraphy can identify infiltration even in asymptomatic individuals, allowing
an early diagnosis of cardiac compromise in this
group of diseases. As expected, there was no uptake in patients with the gelsolin mutation. We
can consider that this non-invasive test would be
a tool for the demonstration of cardiac amyloid
deposition.
AO-099
CALF HYPERTROPHY IN CHARCOT-MARIE TOOTH
DISEASE; REPORT OF SEVEN CASES
Vidal CM1, Dias JC1, Marques Junior W2, Domingues RC3,
Freitas MR1
• 1UFF; 2USP-RP; 3MULTI-IMAGEM
Introduction: Charcot-Marie-Tooth (CMT)
disease is the most prevalent hereditary polyneuropathy. Distal calf muscle atrophy often
occurs causing the classic “stork leg deformity”.
Although it is a cardinal feature of CMT there are
rare reports of calf hypertrophy. Objectives: The
aim of this study is to evaluate the clinical, electroneuromyography (ENMG), DNA findings and
MR imaging of the calves in seven patients with
CMT disease and calf hypertrophy. Patients and
methods: CMT cases under private and public
(Hospital Universitário Antonio Pedro) practice
totalize 91 patients, 33 patients were classified as
not having calf atrophy from which seven cases
with calf enlargement were selected to a complete evaluation. Results: Of the seven patients,
six were female. Their age ranged from 29 to 66
years old (median, 38). In DNA tests five are classified as CMT 1A and two were inconclusive. The
main symptoms were muscle cramps and pain,
both present in five patients. They all had absent
tendon reflexes. Other clinical findings included
scoliosis (five patients), pes cavus foot (six) and
prominent distal sensory loss in lower limbs (six).
All patients presented a demyelinating ENMG.
The average measure of the calf was 36cm (range
31‐44cm on the right leg and range 32‐43cm on
the left leg). Asymmetric hypertrophy was present in three patients. MRI of the legs was obtained in six patients and five of them showed
fatty infiltration of the muscles. All of them are
independent and have a slow progression of the
disease, although the average time of symptoms
is 28 years (range 9‐37). Discussion: Charcot-Marie-Tooth disease phenotypic manifestations are
wide, ranging from severely affected to asymptomatic at late decades. Some rare cases of CMT
1A show calf hypertrophy, which was thought to
be because of musculature enlargement. Most of
our patients presented pseudo hypertrophy due
to fatty infiltration. It seems that calf hypertrophy is associated with a more benign course of
the disease. Conclusion: MRI of the legs has an
important role in the evaluation of calf hypertrophy making difference between fatty infiltration
and muscle enlargement. There may be an association of CMT with calf hypertrophy and a more
benign course.
AO-100
CHRONIC SENSORY DEAFFERENTATION
RESULTS IN CEREBELLAR AND BASAL GANGLIA
STRUCTURAL ABNORMALITIES
Casseb RF, Paiva JLR, Campos BM, Martinez ARM,
Beltramini GC, Castellano G, Nucci A, França Junior MC
• UNICAMP
Introduction: Sensory neuronopathy (SN) is a
subgroup of peripheral neuropathies, characterized by the degeneration of the dorsal root ganglia (DRG) neurons. Although SN are considered
“purely” sensory diseases, patients have severe
motor disability due to afferent ataxia. This suggests that sensory-motor circuitry is impaired in
the disease, but little is known about the location
and extent of such damage. In this scenario voxel-based morphometry (VBM), a whole brain unbiased technique, may be useful to approach this
unsettled issue. Objective: To identify regions
with abnormal concentrations of gray and white
matter in the brain of patients with chronic SN.
Methods: Sixteen consecutive patients (8 women; 50.8±7.5 years old) and 16 control subjects (8
women; 51.0±7.3 years old) were enrolled in this
study. Patients met clinical and neurophysiological criteria for SN. We acquired T1-weighted images in a 3T scanner, with voxel size of 1x1x1mm3,
matrix size of 240 x 240, TR=7ms, TE=3.2ms and
flip angle =8°. We used the VBM8 toolbox to analyze the images. VBM is a method used to compare gray/whitte matter concentration in brain
regions by analyzing the signal intensity in the
images. Statistics were performed with SPM8
and images from both groups were compared using a two-sample t-test. Exploratory results were
obtained with no corrections for multiple comparisons, an alpha level of 0.001 and a cluster
size threshold of 25 voxels. Results: Mean disease
duration in this cohort was 15.1±8.4. Despite
comprehensive workup, 10 patients were still
considered idiopathic. In the SN group, we found
regions with both increased and decreased gray
matter volumes. There was volume increase in
(VII and VIII areas of the right cerebellum, right
putamen, right caudate, left caudate head, bilateral internal and external globus pallidus,
thalamus and superior colliculus. In contrast,
we found gray matter volume reduction in medial temporal and parahippocampal gyri. White
matter atrophy was identified in the inferior
cerebellar peduncle. We did not find areas with
increased white matter volume. Conclusions:
Chronic sensory deafferentation leads to gross
structural abnormalities in the brain, including
basal ganglia and cerebellar volumetric increase.
Such modifications are probably compensatory
phenomena to account for loss of sensory input.
Further studies are needed to evaluate the potential therapeutic usefulness of neuromodulary
approaches targeted at these structures.
AO-101
HIGH PREVALENCE OF PERIPHERAL NEUROPATHY
IN PATIENTS WITH PARKINSON´S DISEASE (PD)
AND PARKINSONISM
Araújo DF, Gondim FAA, Araujo IT, Melo Neto AP,
Horta WG, Barros IS
• UFC
Introduction: Recent studies have reported
that large-fiber polyneuropathy (PN) is common in patients with PD (Gondim, Ann Neurol
2010;68:973). Objectives: To evaluate the prevalence of large-fiber and small fiber PN in patients
with PD and parkinsonism. Method: We evaluated the presence of large & small fiber PN in 54
consecutive patients with PD or parkinsonism
in a tertiary outpatient clinic from Brazil. Initial
PN screening consisted of history/neurological
exam and skin wrinkling test -SWT (Teoh, JNNP
2008;79:835). In addition, we also performed a
NCS/EMG in all patients with PN signs/symptoms and/or abnormal SWT. This study was approved by the local IRB. We compared the groups
with descriptive statistics, t and Mann-Withney
test. Results: 38 patients with PD (10 women,
mean age: 63±2.1 years, P<0.05, mean disease
duration: 8±0.8 years) and 16 patients with other forms of parkinsonism [7 women, mean age:
50.1±3.9 years, mean disease duration: 6.9±1.1
years, 4 patients with Wilson´s disease (WD), 3
with MSA, 3 with PSP/CBGD] completed clinical
neuromuscular evaluation. SWT was performed
in 49 patients (33 PD, 16 parkinsonism). 57.6% of
the PD group (50% of all PD) had abnormal SWT
(mean grade: 1.98±0.2) while 37.5% of the patients with parkinsonism (35.3% of all parkinsonism) had abnormal SWT (mean grade: 2.3±0.3).
NCS/EMG was performed in 39 patients (26 PD
and 13 parkinsonism). 12 out of the 26 PD (34.2%
of all PD) and 4 out of the 13 parkinsonism
(23.5% of all parkinsonism) had abnormal NCS/
EMG results. There was no statistically relevant
difference between the neuropathy prevalence in
groups of PD and parkinsonism (P>0.05), neither
in NCS/EMG nor in SWT. The final neuromuscular evaluation didn’t reveal relevant differences,
with 68.4% of abnormality in the PD group and
52.9% in the parkinsonism group. There was
no significant correlation between SWT grades
37
Oral Presentation
and age, disease duration, B12 levels and use of
levodopa. Conclusions: Large fiber and small
fiber PN are common in patients with PD and
parkinsonism. SWT may be used as an useful
screening tool for evaluation of small fiber dysfunction in patients with PD. Neuropathy etiology seems to be multifactorial in PD patients.
ber dysfunction in patients with PD. Neuropathy
etiology seems to be multifactorial.
VASCULITIC NEUROPATHY: LEPROSY AS A
POSSIBLE DIAGNOSIS
Pinto TS , Antunes SL , Sousa FS , Vital RT , Gomes ML ,
1
1
1
2
Freitas ABSB2, Sano EN1, Jardim MR1
• 1FUNDAÇÃO OSWALDO CRUZ (FIOCRUZ); 2HOSPITAL
UNIVERSITÁRIO PEDRO ERNESTO (UERJ)
Introduction: Peripheral nerve (PN) vasculitis
are pathological conditions with pleomorphic
clinical features, whose diagnosis frequently depends on the histopathological study of
nerve biopsy samples. Objective: Evaluation of
histopathological features and their correlation
with clinical and neurophysiological findings in
vasculitis. Methodology: Retrospective study of
patients who underwent histopathology of nerve
samples for diagnosis of peripheral neuropathy
in a referral center for leprosy in Rio de Janeiro
/ Brazil 2001 to 2014. Twenty nerve biopsy specimens showing histological alteration consistent
with vasculitic neuropathy were selected. Patients underwent clinical, neurological, rheumatological and neurophysiological evaluation.
The samples were routinely studied by hematoxylin-eosin, Gomori‘s trichrome, Wade staining
as well as semithin sections stained by toluidine
blue. Results: Out of 20 nerve patients (mean age
55 years, 55% men) selected, 6 were diagnosed as
leprosy; 3 Wegener‘s granulomatosis; 2 systemic
lupus erythematosus; 5 isolated peripheral nervous system vasculitis; 1 rheumatoid arthritis; 1
Sjögren‘s syndrome; 1 livedoid vasculopathy and
1 monoclonal Ig A hypergammaglobulinemia.
The most common clinical presentation was
asymmetric sensory neuropathy (63% of cases)
followed by asymmetric sensory-motor neuropathy (37% of cases). Histopathology of the
nerve samples revealed alterations in all cases
consistent with axonal neuropathy. In addition,
inflammatory infiltrate surrounding vessel wall
was seen in 64% of cases; fibrinoid necrosis (4%);
acute thrombosis (4%); chronic thrombosis (8%);
hyperplasia of the muscle layer (8%); thickening
of adventitious layer (4%) and endoneurial and
perineurIal increased extracellular matrix (8%).
Conclusion: Vasculitis of the PN is a clinical entity difficult to diagnose given that laboratory tests
are not specific for vasculitis, making biopsy an
important tool to define diagnosis. Attention
should be paid in the differential diagnosis to the
possibility of leprosy as an etiology considering
the high endemicity of this disease in Brazil.
38
Rehabilitation and Neurology
Care
SEXUAL RESPONSE IN PARAPLEGIC INDIVIDUALS
Costa BT, Garanhani MR, Santos SMS, Torrecilha LA,
Souza RB
• UEL
AO-103
ACCURACY FOR FALLS PREDICTION BASED ON
BALANCE-RELATED MEASURES IN PEOPLE WITH
PARKINSON’S DISEASE
AO-102
1
AO-104
Almeida LRS1, Valença GT2, Negreiros NN1, Pinto EB3,
Oliveira Filho J2
• 1BAHIA STATE HEALTH ATTENTION CENTER FOR
THE ELDERLY–CREASI; 2FEDERAL UNIVERSITY OF
BAHIA; 3BAHIANA SCHOOL OF MEDICINE AND PUBLIC
HEALTH
Background: Recurrent falls can be considered
a disabling feature of Parkinson’s disease (PD).
Some widely used balance-related measures,
including fear of falling scales (a construct related to balance) need to be investigated further
for their potential to accurately predict falls in
people with PD. Objective: To compare the accuracy for prediction of recurrent falls of six balance-related measures. Methods: We evaluated
225 PD patients. In addition to demographic
and clinical data, patients were assessed with
the Unified Parkinson’s Disease Rating Scale,
activities of daily living and motor sections,
modified Hoehn and Yahr Scale, Berg Balance
Scale (BBS), Dynamic Gait Index (DGI), Functional Reach Test (FRT), Timed Up and Go Test
(TUG), Activities-Specific Balance Confidence
Scale (ABC) and Falls Efficacy Scale-International (FES-I). Patients were followed up for
12 months to record the incidence of falls and
those who had experienced recurrent falls (two
or more) were classified as fallers. Mann-Whitney and t test were performed. The Receiving
Operator Characteristics (ROC) curves were
constructed and the area under the curve (AUC)
and validity indices were determined. We chose
optimal cutoff points based on Youden indices. Results: The mean age was 70.6 years (6.6
SD) and eighty-four (37.3%) participants were
classified as fallers. Fallers had higher disease
severity and functional impairment than nonfallers (p<0.001). The suggested cutoff scores for
prediction of recurrent falls in a one-year period
and the corresponding validity indices were as
follows: BBS ≤49 points (AUC =79%; sensitivity
=74%; specificity =74%); DGI ≤19 points (AUC
=76%; sensitivity =73%; specificity =72%); FRT
≤17 cm (AUC =74%; sensitivity =56%; specificity
=82%); TUG >15 seconds (AUC =72%; sensitivity
=63%; specificity =71%); ABC ≤55% (AUC =73%;
sensitivity =71%; specificity =62%); FES-I>29
points (AUC =74%; sensitivity =77%; specificity
=62%). Conclusions: Our results suggest that
all selected balance-related measures have
useful predictive accuracy for recurrent falls. It
is of interest to note that either ABC or FES-I,
which assess self-perceived balance confidence
and concern about falling, respectively, reached
similar validity indices when compared to the
performance-based balance measures. The
adoption of the suggested cutoff scores may be
considered in order to improve the identification of falls risk in people with PD.
Introduction: Among the consequences of
spinal cord injury (SCI) is the change in sexual response, conditioned by physical, psychological and social factors. The degree of sexual
dysfunction can vary between paraplegics, and
from the physical aspects, is directly related to
the type of SCI. Objectives: Knowing the sexual
responses of paraplegics and correlate with the
type of SCI. Method: It is a cross-sectional study
of 28 paraplegic patients from a public hospital
ambulatory. The data were collected through the
Human Sexuality in Spinal Cord Injury Questionnaire and through a structured interview script.
The results were presented by average and standard deviation, absolute and relative frequencies, and associations between the type of SCI
and sexual responses, with a significance level
of 5%. Results: The average age was 34.82±9.38
years and 64.3% of patients have incomplete SCI.
Most patients have active sexual life (82.1%) less
than once a week (35.7%), are sexually satisfied
(85.7%) and have spontaneous erection (57.1%).
More than half (57.1%) use some resource to aid
erection, being oral medicine preferred by them
(25%) and the reason for choosing the resource
for failing to maintain an erection long enough.
Regarding the sensations during intercourse, the
increased of the heart and respiratory rate and
body temperature was reported by 35.7%. The
majority (75%) are considered sexually adjusted
in physical terms. Half of patients have orgasm
and the majority (53.6%) does not have ejaculation. When sexual responses were associated
with the type of SCI, there was statistically significant association that individuals with complete injuries do not have orgasm (p<0.01) and
are sexually adjusted in physical terms (p=0.02).
There was no statistically significant association
between the type of SCI and sexual satisfaction
(p=0.27), erection (p=0.26), ejaculation (p=0.24),
use of artificial resource (p=0.26) and use of
medication to have/maintain erection (p=0.05).
Conclusion: Despite the small sample, became
evident a difference between the paraplegic‘s
sexual response and the type of LM, complete
and incomplete, reflecting in the individual‘s
sexual practice. Search more knowledge about
this topic it becomes essential due to the contribution to these patients, by interfering at the
quality of their life, as well as actions of health
professionals.
AO-105
VALIDATION OF THE ASSESSMENT TOOL OF
COMMUNICATION ASHA FACS FOR THE APHASIC
POPULATION
NOrberto AMQ1, Okubo PCMI2, Foroni PM3,
Pontes Neto OM1, Mansur LL4, Takayanagui OM1
• 1FMRP; 2HC-FMRP; 3USP; 4FM
Introduction: The Stroke is the leading cause of
death in industrialized countries and the leading
cause of disability among adults. Engines, behavioral, swallowing, cognitive ± communication
deficits - this highlights the language and speech.
The limitation of a functional communication,
ie, the ability to understand or to send a message
independently and efficiently in response to the
demands of everyday life often appears in individuals who suffer strokes, but not all assessment
XXVI Congresso Brasileiro de Neurologia
tools available to the time can detect such
changes. The scale of the Functional Assessment
of Communication Skills - (ASHA FACS) was developed primarily to evaluate communication
in poststroke patients in the USA. In Brazil, this
instrument was translated into Portuguese and
validated only for people with Alzheimer‘s disease. This instrument has 43 questions divided
into four domains: Social Communication; Communication of Basic Needs; Reading, Writing,
and Numerical Concepts; and Daily Planning.
Objectives: To validate the Brazilian version of
the ASHA FACS for aphasic subjects as a result of
ischemic stroke in the left hemisphere. Methods:
There were 125 subjects, 50 of whom formed the
aphasia group (AG) and 75 in the control group
(CG), in addition to their family ± caregiver for
each subject. Reduced version - - and the Boston Naming Test (BNT) in each participant GA
an assessment of the implementation language
with the Boston Test for the Diagnosis of Aphasia (TBDA) was performed. The application of
the ASHA FACS was done through interviews
with family ± caregiver of all study participants.
After two weeks, a new application was made
ASHA FACS by the researcher and later, the same
instrument was administered by another pathologist. Results: The results showed that the ASHA
FACS has high intra-examiner reliability, and r
(ICC)=0.905 GCE GA r=0.657 and high inter-rater reliability r (ICC)=0.870. The results showed a
satisfactory degree of agreement and significant
between measures, with p<0.001 and 0.000 of
TNB and TBDA respectively, showing good criterion validity of the ASHA FACS. There was also a
high sensitivity 90.0% and specificity 85.1% high.
Conclusion: The ASHA FACS proved a good reproducibility intra and inter-rater instrument,
have good correlation with standardized tests
of reference and have excellent sensitivity and
specificity, suggesting to be a valid and reliable
scale for the population studied.
31st December 2013 in Organ and Tissue Procurement Service Database of HCFMUSP. Last
serum sodium prior to Brain Death determination and in the preceding 48 hours were analysed. Results Mean serum sodium level prior to
Brain Death determination was 152,78 mg/dl (±
10,86). Lowest and highest serum sodium levels
were 126 and 213 mg/dl. There was no difference of these levels compared to those within
the 48 hours preceding Brain Death determination. Mean sodium levels within 48 hours before
Brain Death determination was 153,05 mg/dl (±
10,60). Lowest and highest values were 125 and
199 mg/dl. Conclusion: Although altered serum
sodium is considered to be a major confounder
in Brain Death determination, we still need more
data relative to the detrimental role of altered
serum sodium levels in the accuracy of neurological examination in this setting. In fact, we
lack support to state that hypo or hypernatremia is a major confounder and, if so, what is the
range that should be targeted to avoid it. In one
hand, if sodium levels are a major confounder,
it should be imperative to stablish a safe range.
On the other hand, if it is not, it is not fair to stop
Brain Death protocols and postpone or even prevent organ donation. Despite that, in this analysis levels as high as 213mg/dl were considered
non-impeditive for Brain Death determination
by neurologists.
Movement Disorders
AO-107
Neuro-ICU
BRAIN CORTICAL THICKNESS AND CEREBELLAR
CORTICAL AND SUBCORTICAL VOLUMES IN
PATIENTS WITH FRIEDREICH’S ATAXIA
Karuta SCV1, Raskin S2, Carvalho Neto A1,
Gasparetto EL3, Teive HAG1
• 1UFPR; 2PONTIFICIA UNIVERSIDADE CATOLICA DO
PARANA; 3UFRJ
AO-106
SERUM SODIUM IN BRAIN DEATH DETERMINATION
Calderaro M, Moraes EL, Silva LBB, Nascimento E,
Barros JV
• HCFMUSP
Background: Brain death determination is one of
the most important attributions of neurologists.
However, major confounders must be excluded.
Brazilian Medicine Federal Council states that
hidroelectrolytic, hormonal and acid-basic disturbances and exogenous intoxication must be
excluded for Brain Death determination. However, limits are not established and there is no
consensus on what should this limits be. Serum
sodium levels are critical concerning this matter,
once cessation of brain function itself can lead to
diabetes insipidus and high serum sodium levels
may be a consequence rather than a confounder
of Brain Death. Objectives: To investigate which
serum sodium levels are considered adequate
for Brain Death determinations by neurologists.
Methods: We reviewed all 353 patients that met
Brain Death criteria from 1st January 2009 to
Background: Friedreich’s ataxia (FRDA) is the
most common hereditary ataxia caused by a trinucleotide repeat in intron 1 of the frataxin gene.
Thinning of the cervical spinal cord is a consistent observation in Magnetic resonance imaging
(MRI) of FRDA patients although neuropathological examination in FRDA reveals neuronal
loss in gray matter (GM) nuclei and degeneration
of white matter (WM) tracts in the spinal cord,
brainstem and cerebellum. Objective: To investigate possible differences in cerebral cortical
thickness and cerebellar cortical and subcortical
volumes between FRDA patients and controls.
Methods: This transversal study included 21 genetically confirmed FRDA patients and seventeen healthy controls that underwent structural
MRI of the brain. Cortical reconstruction and
volumetric segmentation were performed using
FreeSurfer version 4.0.5. Results: FRDA patients
had reduced cortical thickness in the left precentral gyrus (p<0.05), lower subcortical volume in
the left and right cerebellar WM (p<0.05) and significant differences in the left and right cerebellar
cortex. There was a strong correlation between a
higher SARA score and lower surface-based WM
volume p values, but no positive correlation between precentral cortical thickness and SARA
score. Conclusion: We have reported important brain and cerebellar neuroimaging findings
using techniques for measuring brain cortical
thickness and cerebellar cortical and subcortical
volumes in FRDA patients. These findings suggest that FRDA involves a more widespread neuronal dysfunction than previously thought.
AO-108
CIRCADIAN RHYTHM AND CRONOTYPE IN
HUNTINGTON‘S DISEASE
Godeiro Junior CO, Rosado Júnior JEB, Freire LA,
Lucena RA, Silva TKL, Araújo JF
• UFRN
Huntington´s Disease (HD) is a disorder that
causes motor and emotional control changes, impaired cognitive ability, involuntary movements
and the clinical manifestations can be predicted by some motor, behavioral anf psychological
changes. The restoration of this cycle can retard
the cognitive decline characteristic of the disease
and improve the quality of life of patients with
this genetic condition. We aimed to (1) assess the
association between circadian rhythm disorders
in patients diagnosed with HD and symptoms
of the disease, (2) identify the existence and describe the characteristics of the sleep disorder
between the study group and (3) test whether an
association exists between sleep disorders, motor symptoms, chronotype and olfaction in the
selected sample. Methods: actigraphy was used
for objective assessment of sleep and rest - activity rhythm. The cognitive state of patients with
HD was measured through experimental Brazilian version of Montreal Cognitive Assessment.
For the analysis of motor symptoms was applied
to motor part Unified Huntington‘s Disease Rating Scale Motor, These symptoms were also investigated by the International Scale Degrees of
Restless Legs Syndrome (EPI). In the subjective
assessment of sleep was used Quality Index Pittsburgh Sleep and Sleepiness Scale Epworth, the
chronotype was characterized according to the
Questionnaire for Identifying Individuals Morning and Evening. The olfaction was assessed according to the Sniffin ‘ Sticks. Results: We found
that as lower is the ability of olfactory discrimination, greater is the severity of EPI (p=0.04). The
objective evaluation of sleep and rest - activity
rhythm, actigraphy data showed that as greater
is the fragmentation of the rest - activity rhythm
(IV60), greater is the activity during the 5 hours of
lower activity (L5) (p=0.002). Correlating the tests
applied to the actigraphy data, we found no statistical significance. Conclusion: There are sleep
disorders in patients with HD, but we need more
studies to detail them. They did not correlate to
motor symptoms and neither to olfaction. There
is a correlation between resteless legs syndrome
motor symptoms and olfaction.
AO-109
DEMENTIA IN A COHORT OF BRAZILIAN
PARKINSON‘S DISEASE
Campos LS, Piovesana LG, Azevedo PC, Guimarães RP,
D‘Abreu ACF
• UNICAMP
Cognitive impairment is a common non-motor
symptom in Parkinson Disease (PD) patients,
and increasing age, older age at onset of disease,
longer disease duration, severity of parkinsonism and male gender have been correlated with
increased risk of developing dementia in these
39
Oral Presentation
patients. The rates of cognitive impairment in
Brazilian PD patients are scarce. Therefore the
aim of this study was to describe the prevalence
rates of cognitive impairment, and to evaluate
the clinical and demographics aspects that may
contribute to it. We performed a cross-sectional study in which we recruited consecutively
subjects who fulfilled the Brain Bank Criteria
for PD diagnosis from the Movement Disorders
Outpatient Clinic at UNICAMP University Hospital. All patients signed an informed consent
approved by the local ethics committee prior
to any research related procedure. Using SCOPA-COG cut off of 17 we divided these patients
in two groups ,with and without dementia . We
correlated these groups with age, gender, years of
education, disease duration, UPDRS, HY, SCOPA-PC and NMSS. We performed the analysis
using STATA version 13.1, and significance level was established at α=0.05. We fully evaluated
76 subjects, 55 (72.37%) male, age 58.81±10.00
years (33‐82), mean years of education 7.54±4.46
(1‐20), and duration of disease of 8.73±7.21 years
(0.66‐37 years). Using the SCOPA-COG cutoff of
17, the prevalence of non-demented PD patients
in our cohort was 48 (63.16%) and 28 (36.84%)
were classified demented. We performed a logistic regression based on the diagnosis of dementia as per SCOPA-COG scores, controlling
for age and gender, using years of study and
Part 3 scores as covariates. For each additional
year of schooling the odds of dementia was 27
per cent lower (OR=0.73±0.08; z=‐2.9; p=0.005;
95%CI=0.59,0.91), while for each increase in one
point in the UPDRS-Part 3 score the odds of dementia was 22 percent higher (OR=1.21±0.07;
z=3.62; p<0.01; 95%CI=1.09,1.35). This model had good calibration (Hosmer-Lemeshow
chi2(8)=2.11; p=0.9776) and very good discrimination (area under the receiver operator
curve-AUROC=0.88) Our study indicates that
higher scores in the part III of UPDRS and low
level of education are possible predictors of PD
dementia.
AO-110
DIFFERENTIAL PATTERN OF CEREBELLAR
ATROPHY IN PATIENTS WITH TREMORPREDOMINANT AND BRADIKINESIA-RIGIDITYPREDOMINAT PARKINSON’S DISEASE
Piccinin CC, Piovesana LG, Guimarães RP, Vilany LNN,
Santos MCA, Campos BM, Campos LS, Azevedo PC,
Torres FR, França Junior MC, Amato Filho AC, LopesCendes I, Cendes F, D‘Abreu ACF
• UNICAMP
Objective: To evaluate (1) the cerebellar grey
matter (GM) of patients with Parkinson’s Disease (PD), (2) the cerebellar GM of a Tremor-Predominant group of PD patients and (3) the
cerebellar GM of a Bradikinesia-Rigidity-Predominat group of PD patients. Background:
The dopamine depletion caused by the degeneration of the nigrostriatal neurons is the classical explanation for most of the motor signals
and symptoms in PD. Therefore, many authors
consider the basal ganglia-thalamo-cortical
loop as the tremor generator in PD. However,
recent studies have addressed that the cerebellum and its pathways are also modified in PD
suggesting a possiblecerebellar involvement in
genesis of the tremor in PD. Design/Methods:
Our sample contained 66 PD patients. For the
first analysis - all PD patients versus healthy
controls (HC) - we excluded six patients due
to unusual covariance in the homogeneity test.
So, in the first analysis we evaluated 60 PD
40
patients (mean age 57.78±10) and 80 HC (mean
age 57.10±9.47). Then, we divided the 66 PD
patients into two groups: 45 tremor-predominant (mean age 61.5±17.68) and 21 bradikinesia-rigidity predominant (mean age 56.5±10.6)
and compared each group to the HC group.
We acquire T1 weighted scans at a 3T scanner.
We used the SUIT tool from SPM for a more
detailed evaluation of the cerebellar GM, and
performed a VBM analysis. Statistics were done
with SPM 8/DARTEL p=0.001, uncorrected and
k=100 voxels. Results: In the global analysis we
detected GM atrophy in the left lobules Crus I
and VIIb. While in the tremor-dominant group
we found GM atrophy bilaterally in the Crus I
and in the VIIb, in the left Crus II, and in the
left VIIIa including the vermis, we did not observe any significant cerebellar GM decrease in
the bradikinesia-rigidity group. Conclusions:
We detected significant cerebellar GM atrophy
in patients with PD. An interesting finding is
the prominence of GM abnormalities in the
group with predominance of tremor, possibly
confirming the involvement of cerebellar pathways in the generation of tremor.
AO-111
DIFFUSION TENSOR IMAGING AND TRACT-BASED
SPATIAL STATISTICS ANALYSIS IN FRIEDREICH’S
ATAXIA PATIENTS
Karuta SCV1, Raskin S2, Carvalho Neto A1,
Gasparetto EL3, Teive HAG1
• 1UFPR; 2PONTIFICIA UNIVERSIDADE CATOLICA DO
PARANA; 3UFRJ
Background: Friedreich’s ataxia (FRDA) is the
most common hereditary ataxia caused by a trinucleotide repeat in intron 1 of the frataxin gene.
Thinning of the cervical spinal cord is a consistent observation in Magnetic resonance imaging
(MRI) of FRDA patients although neuropathological examination in FRDA reveals neuronal
loss in gray matter (GM) nuclei and degeneration
of white matter (WM) tracts in the spinal cord,
brainstem and cerebellum. Objective: To use
diffusion-tensor (DTI) imaging and tract-based
spatial statistics (TBSS) to test the hypothesis
that WM damage in Friedreich’s ataxia is more
extensive than previously described and probably involves normal-appearing WM. Methods:
This transversal study included 21 genetically
confirmed FRDA patients and seventeen healthy
controls that underwent structural MRI of the
brain. DTI was performed in 30 noncollinear directions. For voxelwise diffusion modeling, diffusion data were analyzed using FMRIB’s Diffusion
Toolbox in FSL 4.1. Results: The greatest decreases in FA were in the left superior cerebellar peduncle, left posterior thalamic radiation, major
forceps, left inferior fronto-occipital fasciculus
and corpus callosum and had a significance level
of p<0.01. MD and RD values were significantly
increased in the superior cerebellar peduncles
and striatum. RD was also increased in the inferior peduncles. AD was significantly decreased
in the corticospinal tracts. Conclusion: DTI and
TBSS techniques can improve our knowledge of
the pathophysiology of FRDA and allow a more
accurate analysis of cerebral and cerebellar involvement in the disease. Further studies are
needed with larger samples to correlate clinical
and genetic findings and ataxia scores.
AO-112
EFFECT OF DEEP BRAIN STIMULATION ON PAIN IN
PATIENTS WITH PARKINSON´S DISEASE
Galhardoni R, Cury RG, Ghilardi MGS, Fonoff F,
Arnout D, Fonoff ET, Myczkowski ML, Marcolin MA, BorSeng-Shu E, Barbosa ER, Teixeira MJ, Andrade DC
• HC-FMUSP
Introduction: Nonmotor symptoms (NMS) such
pain are thought to be present from the early
stages of the PD and are often more disabling and
resistant to treatment than motor symptoms.
Pain has a prevalence of 40‐85% of PD patients
and is associated with significant reduction in
health-related quality of life. Deep brain stimulation of the subthalamic nucleus (STN DBS) is
an effective treatment for the motor symptoms
of PD. It has been shown that STN DBS could
produce significant pain relief in more than 80%
of PD patients. However, the available studies focused mainly on the effects of DBS in pain intensity. So far, no study assessed the effects of DBS
on the different aspects of pain and on different
pain syndromes present in PD patients. Aim of
investigation: To evaluate the changes induced
by deep brain stimulation of the subthalamic
nucleus (STN) on pain characteristics in patients
with Parkinson’s disease and to describe the main
pain syndromes in these patients. Methods: We
have prospectively evaluated 41 patients with PD
before and one year after STN DBS. Duration of
the disease, Hoehn Yahr scale, UPDRS III, L-dopa
equivalent dose and Visual Analogic Scale were
recorded. Pain was classified into ‘‘PD-pain’’
(pain that was caused by PD) and ‘‘non-PD-pain’’
(pain related to another cause than PD). When
present, pain was classified into four subtypes:
musculoskeletal, dystonic, radicular and central.
Patients were systematically evaluated for the
presence of myofascial pain syndrome (MPS) by
a trained pain specialist blind to the DBS status
and surgery outcome. Results: The mean age
was 57±10. The prevalence of pain was 70%, and
the most common subtype was musculoskeletal
pain presented in 86.7%, followed by dystonic
pain (48.3%). The main characteristics of pain
before surgery are shown in Table 1. A year after
surgery, there was a significant improvement in
the prevalence of pain (70% to 21%; χ₂:15.814;
p<0.005). Conclusions: Pain is a common nonmotor symptom in patients with PD and responded well to STN DBS, mainly the dystonic
pain. For our knowlegment, this is the first report
to show that myofascial pain syndrome is a commom finding in PD, which can brought new insights about the treatment of these patients.
AO-113
EVALUATION OF SUBJECTS WITH PARKINSON’S
DISEASE REFERRED FOR SURGICAL TREATMENT:
INCLUSION CRITERIA AND PITFALLS – A
PRELIMINARY STUDY
Brando KCF, Pereira JS, Spitz M, Parise M, Simões EL,
Capitão C, Faria A, Cunha RL, Laurencel SM,
Ribeiro CRT, Nigri F
• UERJ
Introduction: Parkinson’s disease (PD) is a progressive neurodegenerative disorder which affects all ethnic groups and social classes, usually resulting in severe disability within 10 to
15 years of disease onset. It is associated with a
significant social and financial impact, particularly in the elderly. The high cost per year worldwide and the limitations and complications of
long term antiparkinsonian therapy overburden
this population’s therapy. The development of
XXVI Congresso Brasileiro de Neurologia
new neurosurgical techniques, with implantable neurostimulators, had a great impact on
PD treatment and resulted in better therapeutical results than in the pre-levodopa era. Motor
fluctuations, among others, represent a surgical
indication for PD, when drug adjustment is not
very efficient. Several studies have demonstrated
the benefits of PD surgery in carefully selected
patients. Objective: To analyse the clinical characteristics of PD patients referred for surgery.
Methods: Twenty six subjects with the diagnosis
of PD were referred for surgical treatment at the
Movement Disorders Sector/Neurology/HUPE/
UERJ. All were submitted to a clinical evaluation,
which included the criteria for the diagnosis of
idiopathic PD and application of a specific questionnaire. Only selected patients participated on
the follow-up protocol: extended neurological
evaluation, UPDRS (parts III and IV), MMSE,
MoCA and Beck and PDQ39 scales. The ones who
fulfilled the inclusion criteria were further evaluated by a neuropsychologist, speech therapist,
physical therapist and then referred to Neurosurgery. Results: Among the 26 patients referred for
surgery (17 male, 9 female) and submitted to the
first evaluations, the average age was 59,7 and
average disease duration 14,4 years. Tremor was
the predominant symptom, followed by rigidity
and bradykinesia, predominantly on the right.
Indications were on-off phenomenon, dyskinesias or suboptimal medical response. Among the
26 patients, 17 did not have a precise surgical indication and two required drug adjustment. Seven patients were evaluated by the neurosurgical
team: two had contraindications during surgical
risk evaluation and five underwent stereotactic
surgery: two pallidotomies, one thalamotomy
and three neurostimulators implants (DBS) – one
in a patient with prior ablation, with excellent results. Conclusion: The identification of patients
that will probably benefit from PD surgery is extremely important, representing the first step for
a successful intervention.
AO-114
MAPT H2 HAPLOTYPE AND AGE INFLUENCE
PARKINSONISM IN OLDER ADULTS
Valenca GT1,2,3, Srivastava GP4,5, White C4,5,
Oliveira Filho J6, Shulman JM7, Buchman AS8, Yu L8,
Schneider JA8, Bennett DA8, Jager PL4,5,9
• 1Federal University of Bahia; 2Broad Institute Harvard
and MIT; 3Program in Translational Neuropsychiatric
Genomics; 4Brigham and Women’s Hospital; 5Broad
Institute; 6Federal University of Bahia; 7Baylor
College of Medicine; 8Rush University Medical Center;
9
Harvard Medical School
Introduction: parkinsonian signs are common
in elderly and are associated with significant
functional impairment, increased risk of cognitive decline, dementia and mortality. In a previous study we demonstrated that susceptibility
locus for Parkinson‘s disease (PD) in the MAPT
gene is associated with mild parkinsonian signs
in patients with advanced age without clinical
diagnosis of PD. However, the biological mechanism underlying this association is unknown.
Exploring the pathophysiology of parkinsonism
in older individuals may lead us to a better understanding of motor phenotypes in age-related neurodegenerative diseases. Objective: to
avaluate the effect of the MAPT gene haplotypes
and MAPT RNA expression in age-related parkinsonism. Methods: We evaluated the clinical
(annually), neuropathological and genotypic
data of 976 subjects of two longitudinal clinico-pathological studies, Religious Orders Study
(ROS) and Memory and Ageing Project (MAP).
DNA was extracted from whole blood, lymphocytes or post-mortem brain tissue. We investigated the two major MAPT locus haplotypes by
tagging the H1 haplotype and H2 haplotype with
the major allele and minor allele of rs1052553
(MAF=0.20), respectively. The subhaplotype H1c
was tagged with the minor allele of SNP rs242557
(MAF=0.38). RNA Seq data were generated using
the postmortem brain tissue of ROS/MAP individuals. We applied linear and logistic regression
models, adjusted for age, sex, study and brain
pathologies. Results: the H2 haplotype of the
MAPT gene was associated with both, global parkinsonism at baseline (p=0.001) and with measurement at the time close to death (p=0.049).
In the analysis of specific parkinsonian signs,
we found that haplotype H2 is mainly associated
with bradykinesia in both evaluations (p=0.0002
and p=0.008); gait is only associated with H2 at
baseline (p=0.025). We also showed that macroscopic infarcts (p=0.00001) and Lewy bodies
(p=0.009) influence motor function. Similarly, we
observed that total MAPT expression (p=1.72‐14)
was associated with the H2 haplotype. Furthermore, it was found that total expression of MAPT
is strongly associated with age and decreases
linearly over time (p=0.0001). Conclusion: overall, these findings suggest that in our cohort the
association between MAPT H2 haplotype and
higher global parkinsonism and mainly bradykinesia scores may be driven by MAPT expression
levels and age-mediated tau level.
minutes/gait and balance training with multisensory cues and functional activities/attention
strategies; 3. Relaxation (5 minutes/breathing exercises and trunk control). The Group II did the
same exercises without cues. Group III receives
only orientations. The Results showed improvement after treatment, according to media score
for the Group I e II of BBS (p=0,01) Mini BESTest
(p=o,00) TUG (p=0,01) FMI (p=0,01) and presents gait balance and mobility improvement.
After 60 days there are scores maintenance only
on Group I. In Conclusion the physiotherapy
programme with MC proposed in this study is
effective and connected to medicine seems to be
effectiveness to minimize balance and gait disorders in HD and provides improvement in functional independence.
AO-115
Introduction: THAP1 mutations are associated
with idiopathic isolated dystonia (IID) in different ethnicities, but the importance of this gene
as a cause of dystonia in the Brazilian population
has not been determined. Objectives: The aim
of this study was to investigate the prevalence
of THAP1 variants in Brazilian patients with idiopathic dystonia and to describe their clinical
characteristics including non-motor symptoms.
Methods: One hundred and thirteen unrelated
patients with dystonia and their available family
members were enrolled by movement disorder
specialists in the participating centers. Individuals were selected according to recent recommended criteria for the diagnosis of IID. All probands were clinically evaluated and initially tested for TOR1A GAG deletion, three of them were
positive. One hundred and ten probands negative for this mutation were screened for THAP1
variants through genetic sequencing. Results:
We identified a total of six different THAP1 variants affecting ten families (9.0%), of which four
were novel. Variants accounted for 11.8% of the
cases with the disease onset below 40 years and
2.9% of those with late adulthood onset. Overall,
we identified 17 carriers, of which 13 were symptomatic and four were asymptomatic. The mean
age of onset was 11.9 years (range 3–46 years).
The upper limb was the most common site of the
onset, and approximately half of the patients had
dysphonia. Pain, anxiety, and sleep-onset insomnia were the most prevalent non-motor symptoms, and their prevalence was not different
from that observed in THAP1-negative patients.
Most variants lead to premature stop codons and
are located in the THAP protein domain. Two
non-synonymous variants are predicted to be
deleterious by in silico analysis. They are highly
conserved in mammals and were neither detected in 254 control chromosomes nor described in
the public databases. Conclusions: THAP1 variants are an important cause of dystonia among
individuals with an early-onset disease and a
positive family history. In Brazil, they are more
MULTISENSORY CUES COULD BE EFFECTIVE TO
IMPROVE BALANCE AND GAIT IN HUNTINGTON´S
DISEASE?
Capato TTC, Haddad MS, Piemonte MEP, Barbosa ER
• FMUSP
In Huntington´s Disease (HD) the chorea is not
the only important manifestation of the neurodegenerative disease. Many cognitive and non-motor symptoms may be more disabling than the
motor symptoms. A loss of balance and gait could
lead to functional decline and increase the risk
for falls in individuals with HD. However, there
are few evidences that a physiotherapy treatment
with Multisensory Cues (MC) improve functional
disturbances of the basal ganglia motor circuit.
The mechanisms by which improvement occur
in HD remains inexplicable. Obejctive of the
study was to check the effectiveness of a MC
physiotherapy programme to improve balance,
gait and functional mobility in HD. Methods: 30
HD were assessed by a single blind examiner before and after 10 training sessions (once in a week
during 45 min) and after 60 days of the end of the
training. Balance was assessed by Berg Balance
Scale (BBS) and Mini BESTest. Gait by Time UP
and GO (TUG), FGA and 6 minutes walk test. Mobility was evaluated by Functional Capacity (FC),
Functional Independence Measurement Scale
(FIM) and Barthel Scale. Through interviews independence to AVDS; motor performance and
cognition through UHDRS. The subjects should
have HD diagnosis genetically confirmed and
they were expected to understand tests and exercises sequences according to inclusion criteria.
During the study period there wasn´t medication
changing. The patients were divided on 3 groups
according criterial rules; aged 45.91. Group (I)
done treatment programme comprised generalized exercises with MC and was divided in three
parts: 1. Warming (10 minutes/stretching, trunk
mobility and motor control); 2. Main Part (30
AO-116
NOVEL THAP1 VARIANTS IN BRAZILIAN PATIENTS
WITH IDIOPATHIC ISOLATED DYSTONIA
Silva Júnior FP1, Santos CO2, Silva SMCA3, Barbosa ER1,
Borges V4, Ferraz HB4, Limongi JCP1, Rocha MSG5,
Aguiar PMC2
• 1DEPARTAMENTO DE NEUROLOGIA- FMUSP;
2
HOSPITAL ISRAELITA ALBERT EINSTEIN E
DEPARTAMENTO DE NEUROLOGIA E NEUROCIRUGIA
UNIFESP; 3HOSPITAL DO SERVIDOR PÚBLICO
ESTADUAL DE SÃO PAULO E DEPARTAMENTO
DE NEUROLOGIA E NEUROCIRURGIA UNIFEP;
4
DEPARTAMENTO DE NEUROLOGIA E
NEUROCIRURGIA- UNIFESP; 5HOSPITAL SANTA
MARCELINA
41
Oral Presentation
prevalent than TOR1A mutations in IID patients
(8.8% versus 2.6%, respectively). Reduced penetrance and phenotypical heterogeneity among
patients carrying similar variants show that other factors may be modulating the disease. Acknowledgments: the authors thank all patients
and their families. This study was funded by Sao
Paulo Research Foundation (FAPESP) grants #
2010/19206‐0; 2011/18202‐3; 2013/09867‐7.
AO-117
PSYCHOSIS IN MACHADO-JOSEPH DISEASE:
CLINICAL CORRELATES, PATHOPHYSIOLOGICAL
DISCUSSION AND FUNCTIONAL BRAIN IMAGING
Braga Neto P1, Pedroso JL1, Gadelha A1, Noto CS1,
Garrido GEJ2, Laureano MR1, Barsottini OGP1
• 1UNIFESP; 2UNIVERSITY OF WESTERN AUSTRALIA
Introduction: Machado-Joseph disease (MJD),
is the most common spinocerebellar ataxia
worldwide. MJD has a broad range of clinical
manifestations, but psychotic symptoms are not
well characterized. Objectives: We investigated
the psychiatric manifestations of a large cohort
of Brazilian patients with MJD in an attempt to
characterize the presence of psychotic symptoms
in our patient population. In addition, we performed a correlation between psychosis, clinical
features and brain SPECT analysis. Methods: We
evaluated 112 patients with clinical and molecular diagnosis of MJD at the Ataxia Unit of Universidade Federal de São Paulo, from February
2008 to November 2013. Patients with suspected
clinical diagnosis of psychotic symptoms were
referred to psychiatric evaluation. Results: Five
patients presented above threshold psychotic
symptoms according to psychiatric evaluation.
Patients with psychotic symptoms were older
and with a later age of disease onset (p<0.05). No
significant regional and global brain blood flow
differences were found in any psychotic symptoms compared to with psychotic symptoms
group. The autopsy of a patient with psychotic
symptoms found no relevant pathological information to better explain psychotic symptoms in
this patient, except for severe loss of neuron bodies in the dentate nucleus and substantia nigra.
Conclusions: Older patients and those with later
age of disease onset are willing to develop psychotic symptoms during the disease progression.
AO-118
REM SLEEP BEHAVIOR DISORDER IN WILSON´S
DISEASE
Tribl GG1, Trindade MC1, Bittencourt T2, Lorenzi Filho G2,
Alves RC1, Andrade DC1, Fonoff ET1, Bor-Seng-Shu E1,
Machado AA1, Teixeira MJ1, Barbosa ER1
• 1DEPARTMENT OF NEUROLOGY USP; 2SLEEP
LABORATORY, PULMONARY DIVISION, INCOR, USP
Objective: We first describe occurrence and
characteristics of REM sleep behavior disorder
(RBD) in patients with Wilson´s disease (WD).
Method: Face-to-face interviews (patients and
caregiving family members), neurological and
clinical examinations, laboratory tests, neurological (Unified WD Rating Scale [UWDRS], Global
Assessment Scale for WD [GAS]) as well as RBD
specific rating scales (RBD Questionnaire Hong
Kong [RBD-HK], RBD Screening Questionnaire
[RBDSQ], Mayo Sleep Questionnaire [MSQ]),
and Video-polysomnography (PSG, including bilateral EMG of submental, flexor digitorum superficialis and tibialis anterior muscles,
REM sleep without atonia [RWA] rated in 3-sec
miniepochs according to criteria of the World
42
Association of Sleep Medicine). Results: 41 WD
patients (m=24) had first symptoms of WD at age
17.9±6.2yrs (mean±SD) and were examined at
age 34.2±9.4yrs. All had a neurological WD (UWDRS total score 77.1±49.8, neurological subscore
57.4±41.1), with almost no hepatic manifestation
(UWDRS hepatic subscore 6.3±3.3). Five WD patients (5/41=12.2%; 4 females) fulfilled criteria of
RBD (according to American Academy of Sleep
Medicine). A further female patient had a typical history of RBD in the early phase of WD, but
had improved later. Another male patient had a
pseudo-RBD with an undiagnosed obstructive
sleep apnea (typical symptoms of RBD, RBD-HK
40, BMI 35.2kg/m2, in the first PSG apnea hypopnea index [AHI] 33.9/h and indeterminable
RWA due to respiratory events and artifacts; in
the second PSG with CPAP AHI 1.3/h and RWA
8.7%). WD+RBD and WD/non-RBD did not differ significantly in a wide range of demographic,
WD specific and sleep parameters, but WD+RBD
patients showed significantly higher values in
percentage of RWA (41.4±19.5% vs. 13.7±6.5%),
and in scores of RBD-HK (35.8±11.3 vs. 11.7±7.7),
RBDSQ (8.4±3.7 vs. 3.5±1.9), and ESS (Epworth
Sleepiness Scale, 11.2±3.4 vs. 6.7±3.5); all p<0.05.
In three WD patients, RBD had manifested before
any other symptom that could be attributed to
WD. Conclusion: These are the first quantitative
data on RBD in WD. Five of 41 patients had RBD,
three of them as initial symptom of WD. In spite
of impressive histories of dream enactment behavior, diagnosis of RBD had never been considered before. The possible manifestation of RBD
needs to be examined by a systematic diagnostic
workup, as only focused interviews, implementation of specific rating scales, and scoring of
REM sleep for RWA lead to the diagnosis.
AO-119
THE MOST COMMON SPINOCEREBELLAR ATAXIA
IN BRAZIL
Moro A1, Moscovich M1, Tensini F1, Farah M1, Arruda W1,
Raskin S2, Teive HA1
• 1HC; 2GENETIKA LABORATORY
Objective: The main purpose of this study was
to investigate the most common spinocerebellar
ataxia (SCA) in a large cohort of Brazilian families. Background: SCAs are a large and complex
heterogeneous group of autosomal dominant
degenerative disorders characterized by progressive degeneration of the cerebellum and its afferent and efferent connections. Other nervous system structures are typically affected, including
the basal ganglia, brainstem nuclei, pyramidal
tracts, posterior column and anterior horn of the
spinal cord, and peripheral nerves. Worldwide,
Machado-Joseph disease, also known as spinocerebellar ataxia type 3 (SCA3), is currently considered the most common form of SCA. Among
SCAs, the relative frequency of MJD in Brazil is
about 69‐92%. Methods: Four hundred and eighteen patients from 190 Brazilian families with
SCAs were assessed with neurological examination and molecular genetic testing for SCA types
1, 2, 3, 6, 7, 8, 10, 12, 14, 17 and DRPLA. Genetically ascertained SCAs were detected in 291 patients; the remaining 127 patients remained undiagnosed. Results: Pathological mutations were
observed in 291 (69.6%) patients. 52% of patients
were men, and 93.6% were Caucasian. The average age of patients was 43.8 (SD±12.6 years), the
mean age of onset was 34.6 (SD±10.1 years) and
the average duration of disease was 9.2 (SD±7.4
years). The most frequently identified SCA was
SCA3 (41.6%), followed by SCA10 (17.9%). Other
subtypes detected included SCA2 in 5% of patients, SCA1 in 2.6%, SCA7 in 1.7% and SCA6 in
0.7%. The remaining 30.4% of patients were left
undiagnosed genetically. Conclusions: In this
large Brazilian cohort of SCA patients, SCA3 was
the most frequent detected form, which confirms
the literature findings as the most common form
of dominant ataxia in different mo¬lecular genetic studies throughout the world. In our sample, SCA10 was the second most common ataxia,
identified in almost 18% of patients. The authors
concluded that the inci¬dence of SCA10 found
was very high and suggested that this might have
been due to the presence of a founder effect of
Amerindian ancestry.
AO-120
THREE MEN WITH VISUAL HALLUCINATIONS AND
PARKINSON‘S DISEASE: DIFFERENT CAUSES FOR
SIMILAR SYMPTOMS
Silveira JOF, Lebeis K, Cunha MF, Vilaça C, Breder R,
Leite MAA
• UNIVERSIDADE FEDERAL FLUMINENSE
Introduction: Hallucination is a “sensory perception without external stimulation of the relevant sensory organ”, according to DSM IV.1 Parkinson’s disease (PD) is one of the neurological
disorders associated with visual hallucinations
(VH).2 Simple hallucinations are defined by the
absence of form, often photopsias and geometric shapes. The complex one are clearly defined
and can be animals, objects and people.2 Sense
of presence, a vivid sensation of something or
someone being around, is common in PD.3 They
can be attributed to the disease itself, medication
treatment and other conditions associated to PD
and prevalent in the elderly.2,4,5 Many factors are
involved in their pathogenesis.6 Purpose: Report
three cases of VH in patients with PD but in different situations: drug induced, disease itself and
Charles Bonnet Syndrome (CBS). Case Report:
Case 1: A 64-years-old-man with PD has VH especially at night. He sees people with big mouth
and ears, monsters, men with guns that want to
kill him. He also complains of tactile hallucination, people touching his body. All hallucinations
disappeared when levodopa dose was reduced;
Case 2: A 62-years-old-man with PD and VH
without insight. He sees giant uniformed employees in a construction in front of his house
and a witch on a hanger in his bedroom. The
VH decreased when rivastigmina was started to
treat the cognitive disorder related to the disease.
Case 3: A 77-years-old-man with PD, glaucoma
and cataract, without psychiatric history, starts
to see flowers and animals, intermittently. The
visions occur at any time of day. He knew they
were not real and they didn’t bother him much.
The VH improved substantially after explanation
about the benign nature of them. Discussion: In
first case, the patient improved after we reduced
the dopaminergic drug. Duration, severity of the
disease and dementia are strong factors involved
in hallucinations of PD.2,5,6 In second case, VH
decreased with the cognitive disorder treatment.
There are differencial diagnoses to exclude, like
delirium, psychiatric conditions, epilepsy, CBS,
peduncular hallucinosis, hypnagogic and hypnopompic hallucinations and other neurodegenerative disease. In third case, the patient fills
diagnostic criteria for CBS, characterized by triad
of complex VH, visual impairment and preserved
cognition, with insight into unreal nature of the
visions.7,8 Hallucinations in PD could be a result
of the interaction between drug and disease-related factors.5,6
VIDEOS
Videos
XXVI Congresso Brasileiro de Neurologia
Child Neurology
Movement Disorders
Muscle Disorders
V-001
V-002
V-003
A NEW PROTOCOL OF COGNITIVE-MOTOR
ASSESSMENT FOR CHILDREN WITH SPINAL
MUSCULAR ATROPHY TYPE I
ATYPICAL PHENOTYPE IN A CASE OF MYH7
BELLY DANCER’S SYNDROME IN TEMPORAL AND
ANATOMICAL ASSOCIATION WITH ACUTE ISCHEMIC
STROKE
Polido GJ, Caromano F, Voos MC
Bönnemann C2, Laporte J3, Zanoteli E1
• USP
• 1FMUSP; 2NIH; 3IGBMC
Background: It is difficult to evaluate and follow children with spinal muscular atrophy type
I (SMA-I, Werdnig-Hoffman), who are currently living longer. Their oral communication and
trunk and limbs movements are very poor, although eye movements are preserved on most
cases. So far, studies have stated that this is a
exclusively motor disease and cognitive skills
are preserved or even optimized. However, the
lack of environmental interaction due to the severe paralysis may cause cognitive impairment.
Objective: This study aimed to (1) propose the use
of TOBII-PCEye technology and (2) investigate the
cognitive-motor performance with children with
SMA- I. Method: Four children with SMA-I were
assessed individually (3 boys aged 3, 7 and 8 yrs
and 1 girl aged 4 yrs). Each child was positioned
on his/ her adapted wheelchair, facing the notebook with TOBII-PCEye technology. Figures and
colors association tasks were proposed for all children and numbers and letters association tasks
were also proposed for the 7 and 8 year old children. The figures were displayed on the screen, in
4 x 4 cm squares. The motor response required to
select each square was 2000ms eye fixation. The
number and percentage of correct answers and
the time (limit: 480 s) were registered. Clinical
evaluation consisted of Chop Intend (CI), Pediatric Evaluation of Disability Inventory (PEDI,
subitems selfcare and social function) and Pediatric Quality of Life Inventory (PedsQL). Results:
TOBII-PCEye technology was useful to evaluate
the cognitive-motor performance of children with
SMA-I who were older than 4 yrs. The 8 yrs old
child showed 51% of correct answers; the 7 yrs old
child showed 11% of correct answers; the 4 yrs old
child, 100% of correct answers and the 3 yrs old
child, 0% of correct answers on the cognitive-motor task. On clinical evaluation, children scored
from 3 to 13% on CI, from 5 to 31% on PEDI- selfcare, from 22 to 59% on PEDI- social function and
from 29 to 55% on PedsQL. Conclusion: Despite
having low motor performance, functional independence and quality of life, children aged 4 yrs
or older could use TOBII- PCEye technology and
perform the cognitive-motor tasks. The elements
association difficulty shown by most children
suggests that the restricted environmental interaction, due to the severe paralysis and to the poor
communication, can cause cognitive impairment.
CONGENITAL MYOPATHY
Abath Neto OL1, Martins CA1, Reed UC1,
Introduction: Mutations in the MYH7 gene,
which codes for the heavy chain of the cardiac beta-myosin, a protein found in the
cardiac muscle and type 1 fibers of skeletal
muscle, are responsible for diverse phenotypes, which include familial hypertrophic
cardiomyopathy and distal myopathy of
Laing. Objectives: We here describe a patient
with an atypical presentation harboring a
MYH7 mutation. Report: An 11 year-old boy,
single child of nonconsanguineous unaffected parents, started having frequent falls after
acquiring ambulation, at one year. At age 6,
he developed progressively worsening lower
limb weakness and cervical flexor weakness.
Physical exam shows tetraparesis affecting
both proximal and distal muscle groups, and
his gait shown in the video involves both
waddle and steppage components. Muscle
MRI showed severely affected paravertebral
cervical, glutei, sartorius, tibialis anterior
and wrist extensors muscles, and moderate
involvement of triceps brachii and hamstrings, taken together representing a distinct
distribution. His serum CK level and cardiac
exams were always normal, and muscle biopsy of the biceps brachii had only mild findings, such as slight atrophy of type 1 fibers
and sparse focally disrupted intermyofibrillar
architecture. To further increase the diagnosis of this case, repeated NCS studies and
EMG had conflicting results, showing either
neurogenic or myopathic patterns. The proband and his parents had exomes sequenced,
and bioinformatics analyses demonstrated a
novel variant in exon 34 of MYH7 (c.4802T>C,
p.Leu1601Pro), which segregated in a de novo
fashion. Discussion: The novel mutation potentially expands the phenotypic spectrum
associated to the MYH7 gene. This case illustrates the challenges one can face with atypical cases of neuromuscular disorders, and the
benefits of unbiased exome sequencing to
identify genetic variants.
Afonso COM, Figueiredo MM, Godeiro Junior CO,
Correia CER, Dourado Junior MET, Brito PSM,
Campos MLS, Silva TFV, Ferreira JL, Moura FED,
Fernandes MFC
• UFRN
Introduction: “Belly dancer‘s syndrome” (BD) is
a rare condition that consists of involuntary and
repetitive movements with diaphragmatic contractions often rhythmic, in which patients may
present movements that resemble belly dancing
(diaphragmatic myoclonus or Leeuwenhoek’s disease) that do not have voluntary control and that
exacerbate on the stress. BD is not frequently diagnosed initially due to its rarity and variety of causes, including drug reactions, neurological diseases
and injuries in the central nervous system (CNS).
Many cases are still idiopathic. Objectives: To report a case of diaphragmatic myoclonus, which
occurred in temporal and anatomical association
with ischemic stroke affecting the brain stem and
cerebellum. Case report: Fifty-five years-old man,
without previous comorbidities, presented sudden
episode, after Valsalva maneuver, of intense occipital headache associated with dizziness, hiccups,
postural instability, incoordination of movements
in the left hemibody, difficulty walking and involuntary movements in the abdominal region. Cranial computed tomography showed extensive cerebellar ischemic lesion on the left (hypodense area
involving the left cerebellar hemisphere, associated
with mass effect with bulb compression). The neurological evaluation showed dysfunction of IX and
X cranial nerve on the left, no nauseous reflex on
the left, force preserved in four limbs, appendicular
hemiataxia on the left and gait ataxia. The reported
abdominal movements were irregular and involuntary with the increased frequency and amplitude by emotional stress and orthostatic position,
being characterized by abdominal myoclonus (BD)
Discussion: Cerebrovascular diseases in acute or
late stage may present with movements disorders,
despite being an unusual way. Among movement
disorders, those resulting from lesions of the basal
ganglia are the most common, with the presence of
parkinsonism, chorea or ballism. Myoclonus has
been described in the literature as a rare complication of stroke, and, so far, there are no reports of
abdominal myoclonus in this context. We reported a man who developed belly dancer’s myoclonus in strict temporal and anatomical correlation
with cerebellar acute stroke, a finding that was
not reported in the literature to date. Most studies
indicate hyperexcitability of interneurons as the
neurophysiological abnormalities underlying this
condition (BD), while other authors suggest degeneration or loss of motoneurons.
45
VIdeos
V-005
COMPLEX MOVEMENT DISORDER AND INTRINSIC
BRAIN TUMOR: A CASE REPORT.
Carrilho PEM, Santos MBM, Egewarth C, Oliveira TD,
Perez HA, Bernardi LL, Krielow TL
• HOSPITAL UNIVERSITÁRIO DO OESTE DO
PARANÁ, UNIOESTE, CASCAVEL-PR
Introduction: Movement disorders are uncommon presentation of brain tumors. How
exactly brain tumors rarely manifest clinically
as movement disorders remains unclear. We
describe a case of complex movement disorder in a patient with intrinsic brain tumor.
Case presentation: A 52 year-old right handed
man presented with a 6 month history of head
and right upper limb tremor. The tremor was
of insidious onset with progressively increasing frequency and intensity. Four previous
head CT scans were performed, the first one
2 years ago for a “suspicious stroke”. The patient is hypertensive and a smoker. He started
prolopa plus benserazide and biperiden with
no benefit. On examination he had an obvious head and right upper limb tremor at rest,
and other movements featuring ballismus and
chorea. There were some movements on the
left upper limb. Those complex movements
were present at rest and exacerbated by posture or active movement. There were no signs
of Parkinsonism. Cranial nerves were normal.
He also presented very brisk deep tendon reflexes, disorders of speech and language, and
right-sided complete hemiparesis. Magnetic
resonance imaging of the brain demonstrated
a left heterogeneously mass occupying lesion
involving mainly the frontal and temporal
lobes, the limbic lobe, the insular lobe, the
caudate nucleus, and both thalami, consistent
with intrinsic neoplasm. Discussion: This is a
rare presentation of a diffusely intrinsic brain
tumor. A case series of 225 patients with astrocytomas of the basal ganglia or thalamus
described tremor in only 12 patients, six of
whom suffering a Holmes’ tremor. Thalamomesencephalic lesions have been reported
presenting as parkinsonian or Holmes’ tremor. Other tumors that present with tremor
include those with unilateral resting tremor
and subsequently Parkinsonism which are
usually extra-axial. Posterior fossa masses can
present with intention tremor although other cerebellar signs and symptoms are usually
present. The tremor is believed to result from
disruption of the dentate–rubro–olivary and
cerebello–thalamo–cortical pathways. Lesions
within nigrostriatal pathways are postulated
to cause the resting component. It is therefore
important to consider intracranial tumors in
a patient presenting with unexplained mainly
unilateral atypical tremor.
V-006
DIAPHRAGMATIC MYOCLONUS: CLINICAL AND
PSYCHIATRIC COMORBIDITY
Soares RBV1, Santos RVSG2, Lopes JLMLJ3,
Araújo RL1, Ponte MPTR3, Sousa GG1, Sousa RNC3,
Almeida KJ4
• 1FACID DEVRY; 2UESPI; 3UFPI; 4USP
Introduction: Flutter or diaphragmatic myoclonus is a rare condition, characterized by
fast diaphragmatic contractions, involuntary
with frequency of 0,5 and 8,0 Hz that might
be present during expiration and inspiration.
Originated in the central or peripheral region,
46
irritation or compression of phrenic nerve are
peripheral mechanisms, while the main cause
of central incidence include encephalitis, tumors, stroke and idiopathic conditions. Depression might occur as a trigger or be associated with diaphragmatic flutter. Objective:
The goal of this work is to report a case of
diaphragmatic myoclonus, analysing its etiology and association to comorbid conditions.
Case report: 16-year-old patient, female, who
in the last 6 months has presented involuntary and intermittent abdominal spasms that
improve when she falls sleep and get worse
in anxiety and nervousness situations, sometimes associated with stabbing pain in thorax
basis and diffuse abdominal pain as colic. She
has lost 4 kg since the beginning of the disease, without medication. She has complained
about dyspnea at rest, dyspnea on little effort
and tachycardia associated with weariness.
She presents depression with a score of 25
in the Beck’s depression inventory. After the
abdominal frame started, there was only one
sob crisis lasted 6 hours. The exam showed no
palatal myoclonus or coordination changes.
The abdominal fluoroscopy revealed rhythmic
contration classified as bilateral diaphragmatic, frequency around 3 Hz. It has been obseved
no commitment of phrenic nerve in cervical
and thoracic CT, no hypertrophy or olivary
hyperintensity in brain MRI. Improvement
using Clonazepam 2,5 mg/ml, Fluoxetina 20
mg. Informed consent has been obtained from
pacient and her relatives for report of clinical
history. Discussion: A case of diaphragmatic
myoclonus with probable central origin was
evidenced. Depression was considered as trigger factor and psychiatric comorbidity. This
shows that serotonin dysfunction in depressive disorders can trigger and aggravate the
disorder of movement, increasing brain dysfunction. A study limitation is the absence of
confirmation of diaphragmatic contractions
through EMG. However, the method is invasive and puncture for monitoring might cause
peritonitis caused by bowel injury, or solid
organs. As the contractions were evident on
fluoroscopy, it was deemed unnecessary risk.
Key words: Diaphragmatic flutter, myoclonus,
abdominal fluoroscopy.
V-009
FUNCTIONAL MOVEMENT DISORDERS: CASE REPORT
Parolin LF, Silva MS, Tansini G, Arruda WO
• UFPR
Introduction:
Functional
(psychogenic)
movement disorders may be difficult to differentiate from organic movement disorders
and becomes a challenging for neurologists
to diagnose this patients. These disturbances are a common source of disability, suffering and significant psychological changes in
these patients. Objective: To report a case of
functional movement disorder. Case report:
A 58 years old woman, arrives at a neurology
service complaining of two years onset generalized dystonia. She related her symptoms
with an automobile accident in which she
was involved, with no serious injury. Did not
have other diseases and until the beginning
of this symptoms never made ​​continuous use
of medications. On examination, the patient
was conscious, well oriented and collaborative. Was making continuous and waving
movements involving the trunk, and whenever she was touched, she performed quick
jerky movements of the lower limbs and upper
limbs associated with hyperextension of the
neck and eye retroversion. She walked with
a walking stick in semi-flexed posture of the
trunk, legs and thighs. Her movements ceased
during sleep. She had tried using levodopa and
biperiden without success. MRI of the neuraxis showed no lesions related to the clinical. Serum copper and ceruloplasmin were normal.
Tests of hepatic, renal and thyroid functions
were normal. Electromyography within the
normal range, showed no pattern of dystonia
on the muscles studied. Patient receives the
diagnosis of functional movement disorder. It
is advised to carry out treatment with cognitive behavioral therapy. Family however refuse
diagnosis. Discussion: The diagnosis of the
functional movement disorders is challenger
because of the unusual features of some of
the organic movement disorders. Although
prognosis is poor, the treatment with cognitive strategies and physical rehabilitation can
be beneficial in some patients with functional
movement disorders.
V-012
OPSOCLONUS MYOCLONUS ATAXIA SYNDROME IN A
ADULT WITH HIV/HTLV COINFECTION AND NORMAL
COUNTS OF CD4 T-CELLS
Casagrande SCB, Melo ACP, Silva SMCA,
Berchielli LF, Nakano PT
• HOSPITAL DO SERVIDOR PÚBLICO ESTADUAL DE
SÃO PAULO
Introduction:
The
opsoclonus-myoclonus-ataxia syndrome (OMAS) is characterized
by multidirectional and rapid eye movements,
associated with generalized myoclonus and
less frequent,cerebellar ataxia and postural
tremor. The probable etiology is inflammatory (paraneoplastic majority) but also related
to infections, toxic-metabolic causes or autoimmune diseases. There are rare reports of
adults with HIV and OMAS associated. Most
of them as part of the immune reconstitution syndrome (IRIS) or seroconversion.The
relationship of concomitant HIV and HTLV
infection is common but there is no report
in the literature of OMAS in a patient with
coinfection.We describe the case of a HIV and
HTLV I/II positive and OMAS associated with
normal CD4 and undetectable viral load.The
coexistence of HTLV may promoted greater immune dysregulation causing cerebellar
symptoms´s exacerbation. Objectives: Report
an adult patient with HIV/HTLV coinfection
and OMAS.Infer that, although the HIV infection control, autoimmune complications may
occur. Case report: A 43 y/o male, HIV for 12
years,regular use of TARV,CD4>500 and undetectable viral load that developed asthenia and
headache,followed by dizziness,nausea,difficulty walking and generalized tremors.Hospitalized in another service with labyrinthitis
hypothesis.Without improvement, sought
our service. General examination was normal
and neurological show saccadic and chaotic
bilateral eye movements, generalized myoclonus and ataxia. OMAS was suspected. Blood
tests showed HIV/HTLV I/II reagents.Cranial
CT was normal and MRI nonspecific. Chest
and Abdomen CT with no significant changes. EMG of 4 members was normal. The CSF
XXVI Congresso Brasileiro de Neurologia
with pleocytosis (74 cells, 98% lymphocytes),
protein 68, glucose 76, negative cultures. Established Gammaglobulin 400mg/kg/day for 5
days in 5 monthly cycles with complete reversal frame. Discussion: The occurrence of HIV/
HTLV coinfection is common.In HTLV-infected
is rare cerebellar ataxia,tremor or nystagmus.
OMAS in HIV occurs predominantly in the initial presentation, seroconversion or IRIS.The
present case is unique because the patient
has normal CD4,viral load negative and ataxia associated with HTLV.The mechanism of
pathogenesis of OMAS is still unknown, but
evidences indicate that autoimmune basis affects brainstem., paramedian pontine reticular formation and cerebellar circuits.There are
reports that patients with HIV and normal CD4
counts are more prone to autoimmune disorders than the general population.
V-013
PHYSIOTHERAPY IN BRAIN IRON
NEURODEGENERATION
Sales VC, Freua F
of spinal cord. Etiologies, other than multiple
sclerosis, have been associated with the semiological finding of ataxic unstable hand of
Alajouanine-Akerman, all of them leading to
a severe loss of segmental awareness: tabes
dorsalis, cervical spondylosis, among others. Also, the description of this semiological
sign is found in patients with spinal cord injury without impairment of deep sensation,
causing athetosis and dystonia of the hand.
The purpose of this video is to document the
semiological finding and to disseminate their
characteristics, since it helps in topographic diagnosis and etiological reasoning, substrates of classical Neurology. Case report:
Male, 90 years, presenting gait ataxia, bilateral
pyramidal liberation, positive Romberg sign.
Presence of bilateral signal of Alajouanine-Akerman ataxic hand. Under MRI examination,
was found an extense spinal cord injury, affecting the posterior funiculus in the cervical
spine.
V-015
• FMUSP
ZOLPIDEM IN MOVEMENT DISORDER AFTER CARDIAC
ARREST
Background: Brain Iron Neurodegeneration
is a progressive disease, There is iron deposition in Basal Ganglia and Cerebelum. It is caractherized by pyramidal and extrapyramidal
symptoms, leadind to Postural Instability and
Gait disorders in the early stage of disease. Despite motor symptoms affects daily activities
and increase risk of fall, there is no paper describing conventional approaches in this case.
Methods: A 35 years old man with Brain Iron
Neurodegeneration was submmited to physiotherapy approaches twice a week during
3 months. Patient was filmed pre and post
treatment. Results: According to pre and post
filmed image there were a reduction on base
of support and an improvement on static and
dynamic balance. Conclusion: Physiotherapy
approaches were able to improve balance aspects being a great alternative to reduce disease progression.
Silva MS, Arruda WO, Teive HAG, Bertholdo DB
V-014
THE ALAJOUANINE-AKERMAN UNSTABLE ATAXIC
HAND
Merida KB1, Nascimento MTMS1, Cordellini M1,
Cavalli H1, Santos PSF1, Kowacs PA1, Cavalli H2
• 1INSTITUTO DE NEUROLOGIA DE CURITIBA;
2
INSTITUTO DE NEUROLOGIA DE CURITBA
Introduction: The Alajouanine-Akerman unstable ataxic hand was originally described in
1931 by Alajouanine, and his disciple, eminent
neurologist who worked in Brazil, Dr. Abraham Akerman. Unstability in the position of
the fingers, especially with the hand extended
- position of taking an oath - is a characteristic
. There is a worsening of this signal with the
eyes closed, suggesting an impaired proprioception. Other findings include sensory ataxia,
astereognosis and impairment of deep sensation in the affected member. This finding supports the hypothesis that protopathic sensibility loss, due to the lesion at dorsal funiculus
UFPR
Introduction: Cerebral hypoxic lesions can be
cause of neurological dysfunctions, including coma, seizures, cognitive impairments
and movement disorders. The presentation of
movement disorder is variable and the treatment is a challenge sometimes. Objective: To
present a case of movement disorder after cardiac arrest with therapeutic response to zolpidem, a drug used to insomnia. Case Report:
A 42 years old woman with intense epigastric
pain and tachypnea arrives at the Emergency
Department. Diagnosed as acute pancreatitis,
after three days, she has a clinical worsening
and a cardiac arrest. Resuscitation maneuvers are applied during 25 minutes, followed
by reversal of cardiac function. The patient is
submitted to orotracheal intubation and stays
in induced coma. After 30 days she wakes up
with a movement disorder characterized by
cervical, facial, and four members dystonia.
Therefore she can’t communicate with others. The movement disorder ceased during
sleep. She tried baclofen, buspirone, levodopa and clonazepam with no satisfactory outcomes. With 10 mg of zolpidem, the patient
had a good response at sleep induction. On an
equivocal of the relative responsible to offer
the medication, the patient received half of the
daily dose of zolpidem. After that, the family
noted that patient ceased the dystonic movements. Although she got bradykinetic and had
some difficulty to speak, she could walk with
help and take foods to the mouth. With this
outcome, the family started offering 5 mg of
zolpidem before meals. The drug effects appeared 30 minutes after the administration
and had a duration of 2 hours. Nuclear Magnetic Resonance of head showed encephalic
diffuse volumetric reduction and a bilateral T2
hyposignal at globus pallidus, indicating iron
deposition. Discussion: Although zolpidem is
currently indicated as a hypnotic drug, some
case reports had observed their beneficial effects with rigidity and bradykinesia of parkinson disease and progressive supranuclear palsy, dystonia, restless legs syndrome and type
2 spinocerebellar ataxia. A new indication of
zolpidem must be considered because their
effects observed in these cases of movement
disorders. Studies with more patients with this
conditions will be importants to a better understanding of his mechanism of action.
Neuroinfection
V-004
BENEDIKT SYNDROME AS A PRESENTATION OF
NEUROTOXOPLASMOSIS IN AIDS PATIENTS.
Carrijo Filho SL, França WCSC, Pavan P,
Martins Junior CR, Oshima M, Piovesana LG,
d‘Abreu ACF, Azevedo PC
• UNICAMP
Introduction: Holmes or rubral tremor is a
movement disorder characterized by low amplitude tremor, present at rest and worsened
in posture and action. Generally, it is caused
by a focal lesion of the midbrain. When the
midbrain tegmentum is envolved, the rubral
tremor is associated with ipsilateral complete
third nerve palsy, contralateral hemiparesis and ataxia, which comprise the Benedikt
Syndrome. Objective: To report two cases of
patients with acquired immunodeficiency
syndrome (AIDS) who have rubral tremor syndrome as part of Benedikt Syndrome, sequelae
of cerebral toxoplasmosis. Case 1: Male, 34 yo,
AIDS since 2001, on regular follow up in our
Neuroinfectious Diseases Clinic. In 2008, he
developed complete right third nerve palsy,
left hemiparesis and rubral tremor in the left
arm, leading to the diagnosis of Benedikt Syndrome due to cerebral toxoplasmosis. Case 2:
Male, 31 yo, AIDS diagnosed in 2013. At the
time of diagnosis, the patient presented complete left third nerve palsy and, within a few
weeks, epileptic seizures started, followed
by right hemiparesis and rubral tremor in
the right arm. After neuroimage, neurotoxoplasmosis was confirmed, he received proper treatment and remains in our outpatient
clinic, with the same focal deficits, featuring
another Benedikt syndrome. Both patients underwent cranial magnetic resonance imaging
in 2014 which showed sequelae injury in mesencephalic tegmentum, with hypointensity areas on T1 and hyperintensity on FLAIR. Case
1 lesion was on the right side and case 2 was
on the left. Discussion: Benedikt syndrome is
very uncommon and has great semiology. The
symptomatic treatment of rubral tremor is difficult, there are reports in the literature of response to levodopa, trihexyphenidyl, propranolol and isoniazid, however the gold standard
is the functional neurosurgery. Case 1 abandoned treatment and case 2 is currently being
47
VIdeos
treated with trihexyphenidyl and levodopa
with partial improvement of tremor, but declined surgery. According to neuroimaging
studies, concurrent functional deficits of both
the dopaminergic nigrostriatal and cerebello-thalamic pathways are capable to produce
this type of tremor, although not shown in the
cases described above. The importance of this
report is in the description of a rare brainstem
syndrome as the clinical presentation of a very
prevalent disease (toxoplasmosis) in the context of AIDS, in addition to the difficult symptoms management.
Cerebrovascular Diseases
V-007
EIGHT AND A HALF SYNDROME: IMPLICATIONS FOR
TOPOGRAPHIC DIAGNOSIS
Nascimento MTMS , Hunhevicz SC , Vosgerau RP ,
Cordellini MF1, Suzano FL1
1
1
2
• 1INSTITUTO DE NEUROLOGIA DE CURITIBA - INC;
2
CETAC
This video presents a case of “eight and a half”
syndrome (“one and a half” syndrome associated with involvement of the ipsilateral facial
nerve). Male, 49 years old, caucasian, with
Diabetes Mellitus and Chronic Hypertension
difficult to control, presented with vascular
hemorrhage in the dorsal region of the pons
(tegmentum). In the video, we can see the
failure of conjugate gaze to the right, associated with deficit of adduction of the right
eye and left eye nystagmus in left horizontal
gaze (“one and a half” syndrome). He also
presents with signs of right peripheral facial
palsy and vertical nystagmus at rest. “Eight
and a half” syndrome, as well as “one and a
half” syndrome are part of a continuum of lesions affecting the pontine tegmentum, which
can lead to various clinical manifestations,
depending on the affected structures. These
clinical findings are very important as they
help in topographic diagnosis, a key element
in neurological reasoning.
48
unusual cases of lower motor neuron disease
Rehabilitation and Neurology
Care
induced by radiation. They are generally limited to lumbar segments, presenting as weakness in lower extremities following irradiation of tumors located next to the spinal cord
(such as testicular tumors and lymphomas).
The nature of the pathogenesis is poorly understood and the etiology may vary from vas-
V-010
culopathy to viral. Objective: we report a rare
MICTURITION CYCLE
case of lower motor neuron disease following
Khouri JMN1, Khouri DMN2, Khouri JMN3, Veras R4,
Baer PJ5, Meirelles P6, Guzman G6, Cunha D7
irradiation on the cervical region, presenting
• 1UNAERP; 2UNESP BOTUCATU; 3UFRJ; 4HCV
CURITIBA; 5SANTA CASA CURITIBA; 6UFRJ
MACAÉ; 7EPRAL- PT
visible myokymical movements. Case report:
The complexity of the neurophysiology often determines the disinterest of the most students. One
example is the micturition cycle,controlled by the
nervous system, brain and spinal cord through
structures. Although this is an important point in
the academic training, we emphasize that this issue
is rarely addressed on the construction of medical
carreer, and other areas involved in the rehabilitation of the neurological patient careers. In order to
overcome the difficulties described above and enhance the training of professionals involved, we developed this video, which consists of didactic and
practical schemes which demonstrate the phases
of micturition, including all neurological mechanisms necessary for carrying out this process, since
the knowledge of the neurophysiology of urination
is essential to recognize the type of bladder dysfunction presented by the patient and to guide the
strategies used for rehabilitation.
left shoulder and arm, that have started four
as upper extremities weakness and clinically
A 35-year-old man presented with an 1-year
and 9 months history of weakness on the
weeks after radiotherapy on face and cervical
region for a nasofaringeal carcinoma, with a
medium dose radiation of 6624cGy. The weakness began on proximal muscles, gradually
progressing to hand muscles and to the right
limb, without pain, and stabilized after oneyear progression. The physical examination
revealed cervical and proximal asymmetric
superior limb weakness, worst on the left,
with atrophy and fasciculation. MRI and cerebrospinal fluid findings were unremarkable.
Myokymia was observed on both sternocleidomastoid muscles associated with lower
motor neuron features on the upper limbs.
Myokymic moviments were intermittent and
present at rest, occurring during long periods
Other Motor Neurone Disease
every day. EMG demonstrated myokymical
discharges on cervical muscles and lower motor neuron disease features on upper limbs.
Conclusion: The electromyographic features
place the likely site of the lesion at the anterior horn cell or most proximal parts of the lower motor neuron. Although the pathogenesis
V-011
is not well recognized, the development of
MYOKYMIA AND LOWER MOTOR NEURON DISEASE
AFTER CERVICAL RADIATION TREATMENT
purely lower motor neuron disease after irra-
Parmera JB, Medonça RH, Megale MZ, Zambon AA,
Castro LHM, Nitrini R
• HOSPITAL DAS CLÍNICAS DA UNIVERSIDADE DE
SÃO PAULO -FMUSP
Introduction: There are well known irradiation effects on the nervous system, as myelopathy and plexopathy, but there are also
diation demonstrates the particular vulnerability of these cells to radiation-induced damage. In this particularly case, the development
of symptoms shortly after irradiation and the
finding of myokymical discharges allow us to
input the etiology of the disease on the radiation treatment.
POsters
Posters
XXVI Congresso Brasileiro de Neurologia
P-2
Headache
A MAN WITH A MIGRAINE AND AN EPISODE OF
TRANSIENT TETRAPARESIS
Andrade RS, Vasconcelos RC, Tanaka MU,
Lourenço FHB, Ferreira KS, Loes MAG, Soares LG,
Santi Neto D, Oliveira FN
• FAMERP
P-1
INVESTIGATION OF THUNDERCLAP HEADACHE IN
A CASE OF CAVERNOUS ANGIOMA: WHEN THE MRI
MAKES THE DIFFERENCE
Souza Júnior JB, Ferreira KS, Cetlin RS, Speciali JG,
Dach F
• FMRP-USP
Introduction: Magnetic resonance imaging
(MRI) of the brain is an important test in the
investigation of thunderclap headache, after excluding the Subarachnoid Hemorrhage (SAH).
Objective: To report a case of cavernous angioma (cavernoma) in the brainstem, which clinical
presentation and computed tomography of the
brain (CCT) suggested primary Intraventricular
Hemorrhage (IVH). Case report: a 55-year-old
woman was referred to our hospital with a history of a new headache for 10 days. The patient
reported that the pain had sudden onset, pressure type, high intensity, located in the occipital
region with irradiation to the whole head. She
also had intense photophobia, nausea, diplopia
and blurred vision. Neurological examination
revealed anisocoria, bilateral low-photoreactive
pupil, complete ophthalmoplegia and eyelid ptosis at right and medial-inferior deviation of left
eye. She had a CT that showed a small amount
of blood in the third ventricle. Conventional cerebral arteriography did not show aneurysms,
arteriovenous malformation or thrombosis of
venous sinuses. At lumbar puncture, a opening
pressure of 45 cm of water was found; the CSF
analysis was normal. Brain MRI revealed an oval
lesion (10 x 10 x 06 mm) of exophytic aspect in
the interpeduncular cistern and third ventricle
compatible with brainstem cavernoma. The patient have had a partial remission of the headache with treatment for intracranial hypertension, but remained with convergent strabismus.
At the moment, she is in conservative treatment
with Neurosurgery. Discussion: we reported a
case of thunderclap headache and a small hemorrhage secondary to a midbrain cavernoma, not
previously diagnosed. The clinical manifestation
with focal motor deficits occurs in about 46%
of cavernomas and headache in 10‐90%. MRI
reveals an image in T2 sequence surrounded by
a radiolucent halo without perilesional edema.
Based on evidence of natural history, the annual
rate of bleeding for infratentorial cavernomas is
5% and 15% for rebleeding. Regarding treatment,
for cavernomas greater than 10mm and previous
history of bleeding, expectant monitoring with
regular MRI and physical activity avoidance is
an option. Therefore, this case provides evidence
of the importance of MRI in a patient manifesting thunderclap headache. Larger studies are
needed in this area to clarify how it impacts the
investigation.
Introduction: The most common form of hereditary cerebral angiopathy is CADASIL (cerebral
autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy). This
entity is characterized by dementia, migraine,
behavioral changes and recurrent subcortical
infarcts. Objectives: This Case report aims to describe a case of a man with CADASIL and family
history suggesting impairment in first-degree
relatives. Case report: A 41 year-old male driver and followed at the outpatient headache, has
10 years of weekly episodes pulsatile headache,
floating location, intense, lasting hours to days.
They are preceded by 30 minutes per scotoma bilateral or limbs paresthesias. The pain usually relief after intravenous analgesia in the emergency
room. In the last episode of migraine, tetraparesis ceased completely in less than 24 hours after
analgesia. There is a family history of headache
in two siblings and mother, who died from complications in the sixth decade from dementia.
Neurological examination is normal asymptomatic period. A MRI revealed multiple subcortical
confluent lesions, hyperintense, and areas of gliosis (T2-weighted and Flair) in anterior temporal
regions and periventricular suggestive of lacunar
infarcts. In DWI, the lesions do not change sign.
Study cardiac-vascular, serology for syphilis,
hepatitis and HIV are normal. CSF electrophoresis analysis revealed no abnormalities. Given
this context, skin biopsy was performed, using
electron microscopy. It showed focal cytoplasmic
membrane osmiofílicos deposits in arterioles of
the dermis. Thus, consistent with the diagnosis
of CADASIL. Discussion: The evidence of granular deposits osmiofílicos (GOM - granular osmiophilic materials - finding pathognomonic for the
disease) in the membranes of dermal arterioles
or kidney has a sensitivity of 45% and specificity
of 100% for CADASIL. This finding concurs with
the pathophysiology of the disease by affecting
vessels of small and medium calibers.
P-3
A PRACTICAL APPROACH OF SENTINELS
HEADACHES
Rodrigues CFA, Andrade AC, Fillus IC, Simplício LFO,
Ramos VU, Slongo E, Inkot DHC, Arce E, Beloto HG,
Aguiar MA, Alves MM, Silva MB, Rodrigues TH,
Carneiro RAT
• UNIOESTE
Introduction: Headaches are one of the most
common sign in daily medical practice. It has a
variety of different etiology. Over 90% of the general population relates history of this symptom
during life. Hence, this sign is not often treated as
important by the health professional. It is known
that a small proportion of headache cases had a
severe origin - as tumors, intracranial hypertension, and bacterial meningitis – ending up having
a relatively significant incidence in everyday life.
Headaches are classified and divided into groups
to help the doctor in the diagnosis, prognosis
and treatment. Thus, they are etiologically divided into two main groups: the primaries are those
in which the origin of headache is idiopathic and
in these cases the headache is the symptom and
the disease; the secondary headaches are those
in which its origin is related to a specific pathology. Objective: alert the medical community to
the importance of a complete anamnesis seeking
to differentiate headaches that do not bring complications of sentinels headaches, seeing that
there warning signs that can be easily checked
in a clinical exam. Method: literature review with
discussion of clinical cases of southwest Paraná.
Results: highlight the signs of sentinel headache
and their importance during clinical examination. Conclusion: headaches can often not seem
as a concern by the doctor, but there are some
signs that indicate the severity and need to be
considered. These signs can be easily identified
with a good anamnesis and physical examination. So, it is important not to forget this sign too
recurrent. Uniterms: Headache; classification;
Diagnosis.
P-4
ASSOCIATION BETWEEN CAFFEINE CONSUMPTION
AND HEADACHE: QUALITATIVE APROACH SURVEY
Moraes VCS1, Sobreira BCU2, Pontes RL1
• 1UNIG; 2UFPE
Background: Studies emphasize a link between
headache and consumption of caffeine. Ingestion of a single 150 mg (half to one cup of coffee)
was responsible headache and anxiety disorders
by endogenous adenosines ADORA 1 and ADORA 2. Investigations (Colas, 2004) emphasize
its analgesic strategies as well as its correlation
with commom analgesics (Acetaminophen).
Objectives: The aim of this study was to investigate the association between caffeine consumption and headache type and frequency in the
general adult population. Methods: Cross-sectional survey was conducted with a qualitative
approach. The following are criteria for the inclusion: a) no history of mental disease; b) headache
for more than 14 days at the last month; c) daily
consumption of caffeine. The migraineurs were
divided into two subgroups: G1, participants
with high daily intake of caffeine (>540 mg/day)
and G2, those with lower daily intake of caffeine
(0 to 240 mg/day) (p=0,007). The caffeine consumption per day was calculated by dividing the
total amount of caffeine by 30 (mg/day). The MIDAS (Migraine Disability Assessment) was used
to measure the effect migraine headaches had on
your daily function. Results: G1 was composed by
11 women and 4 men. G2 was formed by 8 women and 3 men. The mean age was 35,7 years. In
G1, the high consumption of caffeine (>540 mg/
Day) showed a strong correlation with low levels
of headache (<14 days/week). In G2, the highest
prevalence of headache (>7‐14 in days / week)
was associated with lower consumption of caffeine (0‐540 mg) (p=0.007). In relation to degrees
of desability correlated with headache, 53,3% of
G1 revealed severe disability (degree IV). In G2,
the rates of severe disability had been reported
about 63,3%. When considering the intensity of
headache, in the range of 5‐10 intensity, G1 had
the corresponding rates at 73,7%. The group G2
obtained, respectively, 72,7%. Conclusion: In our
study, the average daily caffeine intake >540 mg/
dia was linked to low frequency of headache (<14
days/month). Caffeine raised vasoconstriction in
cerebral arteries. The results may indicate that
“high caffeine consumption changes chronic
headache into infrequent headache” (HAGEN et
al., 2009).
51
Posters
P-5
ATTENTION DEFICIT HYPERACTIVITY DISORDER IN
PATIENTS WITH CHRONIC MIGRAINE
Arouca EMG, Fortini I, Torezan SW, Galvão ACR,
Peres JS, Estephan EP
• FMUSP
Introduction: patients with migraine have a
higher prevalence of attention deficit hyperactivity disorder (ADHD) than people without migraine as shown by studies in adults patients (1).
Population studies in children do not show that
Migraine or tension type headache and ADHD
are comorbid conditions, but that they are comorbid to hyperactive-impulsive behavior (2).
However, there are few data in adult patients
with chronic migraine (CM). The aim of this
study was to identify inattention and hyperactivity in patients with chronic migraine. Methods:
32 patients with CM were evaluated according to
the criteria of the International Classification of
Headache of the International Headache Society,
3rd edition, beta version (3). For all patients the
criteria for the diagnosis of ADHD in adults according to DSM V were applied (4). Results: 30
women (93.75%) and 2 men (6.25%) aged 19‐72
years with a mean age of 41.5 years were included in the study. Eighteen patients (56.25%) met
the criteria for inattention and hyperactivity/
impulsivity, 4 (12.5%) only for inattention and
4 (12.5%) only for hyperactivity/impulsivity.
Conclusion: an unexpected high proportion of
CM patients may have inattention, hyperactivity
and impulsivity, according de DSM V criteria. It is
important to identify these patients and actively
search for the symptoms of this condition because those patients rarely spontaneously have
complains concerning inattention. This information may help for better management of the
cases. 1- Fasmer OB1, Halmøy A, Oedegaard KJ,
Haavik J. Adult attention deficit hyperactivity disorder is associated with migraine headaches. Eur
Arch Psychiatry Clin Neurosci 2011; 261:595‐602.
2- Arruda MA1, Guidetti V, Galli F, Albuquerque
RC, Bigal ME. Migraine, tension-type headache,
and attention-deficit/hyperactivity disorder in
childhood: a population-based study. Postgrad
Med 2010; 122:18‐26. 3- Headache Classification
Committee of the International Headache Society (IHS). The International Classification of
Headache Disorders, 3rd edition (beta version).
Cephalalgia 2013; 33:629‐808. 4- “DSM”- Manual
de Estatística e Diagnóstico da Associação Americana de Psiquiatria, 5a edição. Editora Artmed,
2013.
P-7
BILATERAL OCCIPITALGIA FUGAX : A NEW
ULTRABRIEF PAROXYSMAL PAIN?
Jevoux CC, Krymchantowski AV
• HEADACHE CENTER OF RIO
Introduction: We report the case and bring a video of a patient with bilateral occipital headache
of short duration. Objective: Describe a new
paroxysmal pain of short duration Case report:
A 37-year-old man with no relevant medical history started suffering from repetitive attacks of
headache at age 34. The pain occurred in brief
paroxysms in the bilateral occipital region. The
attack duration ranged from 50 seconds up
to a maximum of two minutes. Pain intensity
was extremely severe (10 out of 10) and quality
was explosive. The patient reported persistent
52
discomfort between paroxysms in the bilateral
occipital region with allodynia during innocuous
stimulation of the scalp between the paroxysms.
The number of daily episodes ranged between
two and 40. Occasionally it came in bursts of up
to 30 episodes per hour. There was no presence
of accompanying symptoms such as nausea,
vomiting, photo, phono or osmofobia. No autonomic manifestation accompanied pain. The
pain attacks were not triggered by changing head
position as well as with other maneuvers. No other triggers were reported whatsoever. The attacks
had a daytime pattern and rarely woke the patient
up during the night. Physical and neurological
exams were unremarkable and there were no local trigger points. The palpation over the branches of the greater and lesser occipital nerves were
not painful. Magnetic resonance imaging of the
head and the cervical spine, cranial CT angiography, EEG and chest radiography did not show
any abnormality. Corticosteroids, topiramate,
indomethacin, verapamil, lamotrigine, codeine
plus paracetamol and sumatriptan were tested
in sufficient doses, with no improvement at all.
The pain was not relieved by bilateral anesthetic
block of the greater and lesser occipital nerves.
Despite many therapeutic trials, the symptoms
continued without remission. Discussion: Rare
primary headaches are a heterogeneous group
of headache entities with unknown underlying
pathophysiology. Therapeutic recommendations
are scarce and mostly based on Case reports
and small case series. This case resembles epicrania fugax, but with bilateral and occipital location, which was not described previously. We,
therefore, present a new entity pending better
description and classification and temporarily
name it bilateral occiptalgia fugax.
P-8
Case report: CHILD WITH TOLOSA HUNT
SYNDROME
Soares RBV1, Santos RVSG2, Leal CMB1, Lopes JLMLJ3,
Araújo RL1, Carvalho LCLS1, Silva Néto RP3, Almeida KJ4
• 1FACID DEVRY; 2UESPI; 3UFPI; 4USP
Introduction: Tolosa Hunt syndrome (THS), also
known as painful ophthalmoplegic (OD), is a
very rare disease of unknown etiopathogenesis.
THS is clinically expressed by unilateral orbital
pain associated with paresis of one or more cranial nerves, especially the third nerve, in the absence of other demonstrable intracranial lesions,
except MRI abnormalities of the affected nerve.
Objective: The aim of this study is to report a
THS case in an infant. Case report: An 8-year-old
child, male, male was acompanhado pela his aut.
She reported that the patient complained about
the third severe headache episode that had begun 3 weeks earlier. The headache was located
unilaterally at supraorbital and right frontal regions and there was phonophobia. Neither fever
nor trauma were reported. After 4 days of pain relief, it was observed ipsilateral eye ptosis and diplopia. The patient had previous migraine without
aura that occurred 2 times per month. Previous
severe headache episodes, which occurred in
January 2013 and December of the same year,
solved themselves without medical management
after 1‐2 weeks. On the other hand, current clinical picture improved significantly after the second day of a 7-day treatment with Prednisone
20mg. Clinical tests showed incomplete third
nerve palsy in the right side, with diplopia when
looking close. Fundoscopic evaluation ruled out
papilledema. Sensitivity of the face was normal,
corneal-palpebral reflex was present and visual
acuity was 20/25 bilaterally. MRI showed ipsilateral maxillary and ethmoid sinus filling by a matter with hypersinal at FLAIR-weight sequence.
CSF analysis revealed normal protein and glucose levels, 6 cells/mm3 (predominantly mononuclear). Fungus search was negative. Informed
consent was obtained from the responsible for
reporting the clinical history. Discussion: The
patient showed a clear improvement with corticosteroid therapy. According to clinical data,
MRI findings and response to steroid treatment,
THS diagnosis is suitable after analysis of the International Classification of Headache Disorders
2013 criteria. In childhood, THS is a very rare
condition. Keywords: Tolosa Hunt; Third nerve;
Corticosteroids
P-14
CHANGE IN THE FEATURES OF MIGRAINE, A
POSSIBLE PREDICTOR OF SECONDARY HEADACHE
– CASE REPORT
Henning PR, Bettin T, Schäfer AJ, Bocchi C, Iachinski RE
• FAG
Introduction: Optic Nerve Sheath Meningioma (ONSM) represents about one-third of optic
nerve tumors. Prevalence is higher among 40‐55
years, female. The characteristic symptom is the
progressive and slowly loss of vision. Objective:
Describe a case of Optic Nerve Sheath Meningioma mimicking a migraine. Case report: Female, 41 years, complaining of headache for 25
years. Left frontotemporal pulsatile pain, with
photophobia, phonophobia, nausea and vomiting in crises, which have occurred around 7x/
month. Physical examination do not present
disc edema or changes in cranial nerves. Computerized Tomography (CT) performed did not
demonstrate changes. Initiated treatment with
Topiramato and Paroxetina with improvement
in crises frequency (1‐2/month). However after
8 months of treatment, because of the change
in pain intensity, was realized cranial Magnetic
Resonance Imaging (MRI) suggestive of Optic
Nerve Sheath Meningioma. Three months after
this diagnosis, it was held surgical tumor resection. Behind six months of surgical treatment,
she is left amaurosis, with headache of migrainous pattern, and having crises around 1x/month.
At present taking Topiramato, Pregabalina e Pizotifeno. Discussion: The main indications for
neuroimaging in migraine are: abnormalities on
neurological examination, progressive increase
in the frequency of pain, history of motor or
sensitivity changes during or after the episodes
of pain, pain that wake up the patient at night,
atypical features or headache that does not
meet the definition of migraine or another type
of primary headache. In the Case reported, the
change (worsening) in the intensity and frequency of headache, despite prophylactic treatment
recommended advocated the need to perform
new neuroimaging (MRI). This suggests a tumoral lesion, like ONSM (in our Case report). In
the literature, the treatments recommended are
microsurgery or radiotherapy, but is generally
conservative. In this situation we opted for tumoral resection due to the size of the lesion. With
this treatment, the patient presents migrainous
symptoms less severe and frequent, with improvement in quality of life.
XXVI Congresso Brasileiro de Neurologia
P-15
CHRONIC MIGRAINE AND MEDICATION-OVERUSE
HEADACHE. REGARDING 64 CONSECUTIVE
PATIENTS FROM A TERTIARY CENTER
Krymchantowski AV, Jevoux CC
• HEADACHE CENTER OF RIO
Introduction: Chronic Migraine (CM) and medication overuse headache (MOH) are common in
tertiary centers. Regardless of the diagnostic difficulties until medication overuse is interrupted,
outcome may be good if the approach is efficient
and correct. Objective: To evaluate and describe
the approach in patients with CM and MOH from
a tertiary center. Methods: Every patient with the
diagnosis of CM and MOH (ICHD-III beta), firstly seen in our center from June 2013 were retrospectively studied. Headache patterns, profile of
medication overuse, treatment prescribed and
outcome were evaluated during the first consultation and after 2 and 4 months. Long-lasting
comprehensive initial consultations as well as the
requiring of filling out detailed headache diaries
were performed. Results: Sixty four consecutive
patients, ages 18‐65 years (mean 36,8) were studied. Mean headache days/week was 0,85 (4‐7), average headache history time was 21,9 years (3‐41)
and mean time with >15 headache days/month
was 4,2 years (0,5‐30). All patients were overusing
symptomatic medications (SM) and 19 (29,7%)
were using more than one pharmacological class.
The most used medications were combinations
of analgesics and caffeine (40%), triptans (39%),
Nonsteroidal antiinflammatory drugs (NSAID)
(14%) and ergotamine derivatives (10,9%). Less
than 2% of the patients were overusing opioids
or barbiturates. After 2 months, 9 (14%) didn´t
return and the mean headache frequency decreased to 0,34 (p<0.01) in the 55 patients under
treatment (adherence 86%). All patients received
prednisone during the first 5‐7 days and triptans +
NSAID for the acute attacks (maximum of 2 days/
week). Preventive treatments pescribed were
neuromodulators and combinations of tricyclic
antidepressants and/or calcium channel blockers and/or central noradrenergic modulators. After 4 months, 54 patients were under treatment
with a mean weekly headache frequency of 0,29
(adherence 84,4%). Conclusions: Although with
a difficult approach, especially due to the necessity of interrupting SM overuse, CM and MOH
patients may improve and adhere well when
conducted and treated in an effective and comprehensive way.
P-16
CLINICAL AND NEUROSURGICAL ASPECTS OF
CRANIOPHARYNGIOMA IN THE ADULT PATIENT
Lemos LEAS1, Silva IM1, Barbosa CM1, Moura AK1,
Batista CFR2, Cavalcante DC2, Ferreira PHPB3,
Araújo RL3, Rodrigues ÂB2, Macêdo LP3,
Marques Júnior MASS4, Pereira LAC4, Cardoso AC2
• 1UESPI; 2UFPI; 3FACID – DEVRY; 4UNINOVAFAPI
Introduction: Craniopharyngioma is a rare neoplasia, whose etiology is not fully understood.
With an annual incidence of 0.5 to 2 per million
cases, these tumors account for 1% to 3% of all
intracranial tumors. These neoplasia exhibits bimodal peak incidence: the first between 5 and 15
years, and the second in the fifth decade of life,
with no sex predilection. These tumors are primary of the sellar region, encapsulated, variable
consistency (solid and/or cystic, with or without
calcification) and usually have benign histological nature. In 90% of cases are located in the sellar or suprasellar area. Symptoms are insidious in
most patients, such as headache, visual and endocrine changes, besides intracranial-pressure
(ICP) and seizures. Cognitive impairment may
also be present, principally manifested by apathy
and behavior change. The therapeutic options
available are surgery and radiotherapy, besides
the application of intratumoral and radioisotope drugs, especially the antibiotic Bleomycin.
Case report: Male, 21 years old, from Teresina,
demand for emergency department with a complaint of sudden headache, nauseas and vomit.
The general physical examination reveals sexual underdevelopment, polyuria and polydipsia.
Neurological examination revealed complete
loss of visual acuity in the left eye, associated with
non-photosensitive anisocoria, besides the presence of ventriculoperitoneal shunt (VP shunt)
for correction of ICP. The patient was diagnosed
with craniopharyngioma six years ago, when he
had presented severe headache, seizures and
decreased level of consciousness. Three surgical
procedures were performed because of recurrence of the injury. In the last one, patient underwent a frontoparietal craniotomy with exposure
and drainage of the cyst formation, developing
well in the postoperative period and was discharged five days after surgery. Discussion: The
craniopharyngioma is a rare tumor, of benign
nature, with considerable recurrence rate. Often
it is related to unfavorable clinical conditions. In
this case, has a patient who is in his third surgical
approach to a recurrent tumor, and was showing
signs of endocrine and visual deficits besides VP
shunt for correction of ICP. After third surgical
approach, in which drainage of cystic content
was performed, the patient progressed well,
however, without resolution of the visual deficit.
P-17
CLUSTER HEADACHE: REFRACTORY CASE
Gomez RS, Lima DVES, Santos TM, Silva RA,
Rodrigues FLB, Pacheco JAS, Costa AAS
• HOSPITAL MADRE TERESA
Introduction: Cluster headache is classified as
unilateral primary headache characterized by
intense pain associated with ipsilateral autonomic symptoms. Typically it occurs with short
periods of crises, called clusters. It is included in
the group of trigeminal autonomic headache. It
affects 0.1 to 0.4% of the population, being more
prevalent in men in the ratio of 3:1 compared to
women. Objective: Report atypical and refractory to drug treatment table with various therapeutic attempts to neurosurgical approach of a case
of cluster headache. Clinical case CES 72 years,
hypertension, COPD, bearer of cluster headache,
with daily recurrent pain crises, initially with five
daily episodes, lasting 30‐180 minutes. Continuous intense pain on periorbital region to the left,
associated with conjunctival hyperemia, eyelid
ptosis and ipsilateral hyaline rhinorrhea. Significant pain relief with oxygen. Already in use
of Topiramate 100 mg / day, Verapamil 360mg /
day, Prednisone 20 mg / day, in addition to Acetominophen and Tramadol. Admitted for pain
control, had cardiac contraindication to increasing the dose of Verapamil due to risk of AVB. Despite the adjustment Lithium 300 to 600 mg / day
and increased to Topiramate 200 mg / day, held
7‐18 episodes per day with an average duration
of 20‐80 minutes. There was no clinical response
to a therapeutic trial with Indomethacin 150 mg
/ day. After two months of clinical treatment
without success and on the refractory and incapacitating nature of pain, we opted for neurosurgical intervention. Performed Gasserian
Trigeminal ganglion ballon compression rhizotomy of V1 and V2 roots to the left. In the immediate post-operative patient developed left facial
anesthesia without new episodes of pain. Verapamil dose to 240 mg / day topiramate suspension has been reduced and Lithium. After three
months remained with significant improvement
in pain intensity. Discussion: Chronic cluster
headache occurs in less than 10% of cases. The
rhizotomy of the trigeminal nerve compression
with Fogarty balloon consists of puncture of the
Gasserian ganglion with a needle leading the
balloon that is inflated for 60 seconds with 0.6 to
0.8 mL of iodinated contrast agent. The literature
describes a frequency of 73% relief of at least 50%
pain after the procedure. In the long term the
rate drops to about 46% of cases. Thus one can
consider the technique as an alternative treatment for refractory cases.
P-18
COMORBIDITIES: SIMILARITIES AND
DIFFERENCES BETWEEN CHRONIC MIGRAINE AND
CHRONIC TENSION TYPE HEADACHE
Fortini I, Galvão ACR, Arouca EMG, Torezan SW,
Estephan EP, Panzoldo L
• FMUSP
The study of comorbidities in patients with
chronic headaches is of fundamental importance for therapeutic decisions. Methods: 720
patients were seen in a first visit at the Headache
Clinic of Hospital das Clínicas da FMUSP from
December 2011 to July 2014. All patients were
evaluated by an expert neurologist in Headache
and Pain Medicine, seeking to characterize the
headache and abuse of symptomatic medications, according to the Classification of Headache of the International Headache Society, 3rd
edition, beta version (1), and identify comorbid
conditions presented by these patients. Results:
334 patients had chronic migraine (CM), 88.6%
women and 11.4% men, 72 patients had chronic
tension-type headache (CTTH), 61.1% women
and 35.9% men. Among CM patients, 210 (72.9%)
were overusing symptomatic medications (62.9%
women and 9% men), while among CTTH patients, 19.5% of women and 6.9% of men abused
of symptomatic medications. The most frequent
psychiatric comorbidities in patients with CM
were anxiety (43.1% women and 5.4% men),
insomnia (29.7% women and 3.3% men), depression (11.7% women and 2.5% men), bipolar
disorder (2.7% women and 0.6% men), other psychiatric disorders (2.7% women and 0.6% men),
obsessive-compulsive disorder (0.6% women
and 0% men) and substance abuse (0.3% women
and 0% men). Furthermore, fibromyalgia could
be identified in 42 cases (12.3% women and 0.3%
men). In patients with CTTH, the most frequent
psychiatric comorbidities were: insomnia in 19
patients (16.7% women and 9.7% men), anxiety
disorders 18 patients (18.1% women and 6.9%
men) and depression (9.7% women and 5.6%
men). Fibromyalgia was diagnosed in 1.4% of
women. No man had fibromyalgia. Conclusion:
a much higher proportion of patients with CM
make abusive use of symptomatic medications
compared to CTTC patients. The same could be
observed with respect to anxiety disorder and fibromyalgia. In relation to depression and insomnia no differences were observed. Ours results
are in agreement with other studies (2) that show
that the frequency of any type of anxiety disorder
was significantly higher in patients with chronic
migraine than in those with chronic tension-type
headache.
53
Posters
P-19
DEPRESSION, ANXIETY, HOPELESSNESS AND ITS
RELATION TO QUALITY OF LIFE IN PATIENTS WITH
CHRONIC MIGRAINE
Arouca EMG, Fortini I, Galvão ACR, Torezan SW,
Estephan EP, Peres JS
• FMUSP
Introduction: the aim of this study was to identify and quantify anxiety and mood disorders
as well as de degree of hopelessness and the
impairment of quality of life in patients with
Chronic Migraine (CM). Methods: 33 patients
with CM were evaluated according to the criteria
of the International Classification of Headache
of the International Headache Society, 3rd edition, beta version (1). For all patients, the Beck
Depression Inventory for depression, anxiety
and hopelessness (2) and the Headache Impact
Test- HIT 6 (3) were applied. Results: 31 women
(93.9%) and 2 men (6.1%) aged 19‐72 years with
a mean age of 41.5 years and a median of 40.8
years were evaluated. At the initial assessment,
66.6% of patients reported intense pain. Seven
patients (23.1%) had no depression or reported
minimal symptoms; 9 (27.27%) mild to moderate depression; 12 (36.36%) moderate to severe
and 5 (15:15%) severe depression. Six (18.8%)
patients had a minimum degree of anxiety, 6
(18.8%) mild, 8 (24.3%) moderate and 13 (39.4%)
severe degree of anxiety. Most patients (63.6%)
had moderate or severe anxiety and 48.48% had
concomitant moderate or severe depression
and moderate or severe anxiety. The evaluation
of hopelessness showed: minimal hopelessness
10 patients (30.3%), mild 16 patients (48.5%),
moderate 5 (15.1%) and severe: 2 (6.1%). Based
on the HIT 6 was observed that for 20 patients
(60.6%) the impact of headache in their lives is
high. Conclusion: Most patients with chronic
migraine experience some degree of depression
± anxiety, with a high proportion of patients who
concomitantly presents with moderate to severe
depression and moderate to severe anxiety. For
these patients, the impact of headache in their
lives is high.
P-20
DEPRESSIVE AND ANXIETY DISORDERS IN
MIGRAINE AND CHRONIC MIGRAINE
Borges LG1, Speciali JG2, Ruiz Júnior FB3
• 1UNICAMP; 2FMRP-USP; 3UFU
Introduction: Headache is one of the most reported symptoms to physicians and representing a significant cause of decreased quality of life.
The high co-prevalence of migraine, depression
and anxiety disorders has spared considerable
attention in the pertaining literature. Objective:
The objective of this study was to evaluate both
the presence of anxiety and depressive symptoms in patients with Migraine (M) and Chronic
Migraine (CM) by comparing them with a control group. We also examined the relationship
between age group and how long the subjetcs
have had migraine. Methods: Fifty two patients
with M and CM were examined at the Uberlandia
University School of Medicine (UFU) and diagnosed with a specific headache questionnaire
during their medical consultation, in which the
protocol used for headaches for the Headache
Clinic of the School at Ribeirão Preto (São Paulo University–USP) was followed. Patients were
diagnosed according to the IHS diagnostic criteria and were used as inclusion criteria: subjects
had to be woman between 18 and 70 years old;
have normal neurological examination; normal
54
exams (when necessary), including computed
tomography or magnetic resonance image. The
Migraineurs group was compared with a control
group of forty non-migraine women. Symptoms
of depression and anxiety were measured using the Beck Depression and Anxiety Inventory.
Results: The results of this research showed that
anxiety symptoms were significantly higher in
the CM group (58%) than in the control group
(23%) with p<0.05 and depressive symptoms
were more common in CM (42%) and M (33%)
compared to the control group (28%). Regarding the age of the subjects, in the M group there
was a tendency for the patients over 36 years old
having more symptoms of depression (47%) and
the CM group had a significantly higher percentage (83%, p=0.04) of depressive symptoms in the
younger patients (<36 years old) compared with
the control group. Conclusions: The frequency of
depression and anxiety symptoms found among
M and CM confirms the previously described relationship between migraine and psychiatric disorders. This could corroborate to the enhancement of the possible association between CM
and depression, which may be a factor for CM
perpetuation. If such a link exists, the management of depression and anxiety might be beneficial to the improvement of migraineurs quality
of life.
P-21
DIAGNOSIS AND MANAGEMENT OF MIGRAINE - A
REVIEW
Leite TT1, Torres MO1, Vasconcelos IA2, Leite DT1
• 1EBMSP; 2FUNORTE
The headache, understood as a pain in the cranial region, is one of the most common complaints
in medical practice, accounting for approximately 2% to 4% of visits to emergency services.
Migraine defined as a debilitating primary headache disorder, being one unilateral, pulsating
and recurrent headache and may or may not be
accompanied by aura. The present study, based
on theoretical and empirical works, apprehends
that in the case of migraine, estimated misdiagnosis occurs in about 50% of clinical care. Although the majority of cases diagnosed in the
emergency migraine be benign, non-observance
of warning signs may exclude severe diagnosis,
and unfolding, have serious consequences for
the patient. The treatment of migraine is a challenge, especially in the face of substantial rates of
non-response to drugs and the difficulty in predicting the individual response to a specific agent
or dose. As Objectives, the study proposes to introduce changes in the diagnosis of patients with
migraine and the changes in its management
and its treatment, based on the new guidelines
and new clinical trials. Therefore, it is a broad
review of medical journals articles contained in
electronic databases such as Medline, PubMed,
SciELO and Cochrane. It was found, therefore,
that the correct diagnosis of migraine is essential
for the establishment of specific treatment for
the disease, especially because as a multifactorial
disease, it involves distinct precipitating factors,
ranging from genetic, the quality of sleep, and
the hormonal changes, among other correlates.
Another important aspect arising from the study
is that it was recorded that there are, for the diagnosis of migraine, excessive use of imaging, a circumstance that burdens the health system and
delays the diagnosis of the patient. It was also
found that treatment for migraine is based on
the cessation of pain and decreased frequency of
episodes, and only about 11.5% of the migraine
patients are treated with specific drugs, such as
the prochlorperazine, triptans and DHE. Finally, with no conclusive result, it was found that
migraine is underdiagnosed disease that lacks
specific studies and guidelines. Its diagnosis can
be performed in the absence of complementary
tests (with some exceptions), which makes necessary a greater knowledge of health professionals about the subject. Keywords: Migraine; Headache; Migraine Disorders
P-26
EVALUATION OF THE CLINICAL AND
EPIDEMIOLOGICAL PROFILE OF PATIENTS WITH
MIGRAINE WHO LIVE IN SODRADINHO-DF
Silveira L1, Mesquita HM2
• 1HOSPITAL DE BASE DO DISTRITO FEDERAL; 2CENTRO
MEDICO MATSUMOTO
Evaluation of the clinical and epidemiological profile of patients with migraine who live in
Sodradinho-DF Introduction: It`s already known
about the regional and cultural differences in
presentation, triggers and other clinical characteristics reported by patients with migraine.
Apart from the form of different patterns of access to the treatment. Objective: To evaluate the
clinical and epidemiological profile of patients
with migraine in Sobradinho-DF, served in the
office of general neurology. Method: 200 patients
were attended in Neurology office at Matsumoto Medical Center with clinical diagnosis of
migraine with the criteria of the International
Headache Society in the period June 2013 to June
2014. All patients underwent a demographic
questionnaire that included triggers factors and
the use of medications for treatment and prophylaxis of the crisis. Results: The mean age was
33 years and 80% were women. The most important risk factors were sleep, stress, menstrual period and feeding. 23% had migraine with aura. The
average time between the onset of pain to seek
neurologist was 3.3 years. 43% of patients already
missed work or study for pain, 41% have already
been the emergence of more than once and 34%
had an imaging study, 4% needed to stay in hospital longer than 24 hours. The use of analgesics
were the main drugs used. Between those who already started prophylactic treatment, topiramate
was the most prescribed medication and the use
of triptans has been reported in only 2% of them.
Conclusion: In our study we observed a significant delay to access the speciality of neurology,
besides a large portion require attending emergency units and use of complementary tests. The
use of prophylactic drugs were few, and the most
widely used was topiramate. Triptans were rarely
prescribed. Analgesics and ergot derivatives are
most commonly used than anti-inflammatory. A
proposal to diffusion and better address of headaches in emergency and health posts could have
an impact on quality of life and reduction in the
use of units emergence and unnecessary tests.
P-27
FAMILIAL HEMIPLEGIC MIGRAINE WITH
PROLONGED HEMIPLEGIA AND MULTIPLE AURAE:
REPORT OF A FAMILY CASE AND REVIEW
Sfakianakis TE, Sant‘Ana M, Carvalho DS, Villa TR
• UNIFESP - UNIVERSIDADE FEDERAL DE SÃO PAULO
Introduction: Familial Hemiplegic migraine
(FHM) is a rare form of migraine with involvement of motor aura due to defective mutations
in neuronal and glial ion channel transporters
genes. The clinical manifestations range from
XXVI Congresso Brasileiro de Neurologia
attacks with short-duration hemiparesis to recurrent coma and prolonged hemiparesis, cerebellar ataxia, epilepsy and transient blindness.
In FHM patients, neuroimaging studies show
that propagation of cortical spreading depression during these episodes cause cortical and
brainstem dysfunction with transient hypoperfusion and hyperperfusion in the affected hemisphere, middle cerebral artery vasodilation and
augmented vasogenic leakage with cortical oedema. Objectives: To report a family with FHM
according to The International Classification of
Headache Disorders, 3rd edition (beta version)
criteria that presented multiple aura including a
prolonged hemiplegic aura. Case report: Patient
I: 13 years old girl. Her first migraine attack began at 12 years old and continued every month.
These hemiplegic attacks were preceded by typical migraine with unilateral pulsatile headache
with flashing lights or scotoma (lasting 20 minutes) and vertigo (lasting 10 minutes) accompanied by photophobia and phonophobia. In all attacks, right-sided hemiplegia and hemiparesthesia lasted 3 days. Cerebral magnetic resonance
image (MRI) was normal. The Patient‘s I father
(patient II) was 36 years old and had FHM attacks
starting at 7 years old and had 2 attacks per year.
The hemiplegic attacks were usually preceded by
typical migraine along with dysarthria and one
episode of anomic aphasia at 35 years-old. The
hemiplegic attacks could occur spontaneously
and during rest after exercise and lasted 5 minutes. Electroencephalogram (EEG) and brain
MRI were normal. The Patient‘s I brother (Patient
III) was 15 years old and had FHM attacks starting at 2 years old. The hemiplegic attacks were
preceded by typical migraine and occurred once
a year and lasted 3 days. EEG and brain MRI were
normal. Discussion: In conclusion, we report
a family who displays symptoms of prolonged
hemiplegic, cortical and basilar-type aura along
with atypical short-lasting migraine episodes. In
migraine with aura, the symptoms of neurological deficit reflecting cerebral cortex or brain stem
dysfunction rarely persists for more than few
hours and it is rarely described in the literature.
Future studies may elucidate the mechanisms
underlying prolonged aura symptoms in FHM
patients.
P-28
FREQUENCY OF HEADACHE AND THE
RELATIONSHIP WITH THE USE OF ORAL
CONTRACEPTIVES IN TELEMARKETING WORKERS
Barbosa AS1, Zocal MB1, Ros DO1, Dias AM1,
Nazario BM1, Santos LB1, Braga JF1, Agostinho VHA1,
Paula GR2, Limonte FH3, Liso E3
• 1UNILAGO; 2FACERES; 3FAMERP
Introduction: Headache is a blinking disorder that especially affects females involving in
stressful activities. Studies in female population
show that episodic migraine is the most common group of primary headache that affected
this population and has direct relation with the
use of oral contraceptives. Objective: Analyze the
frequency of headache and the use of oral contraceptives in women working in a telemarketing
company located in the state of São Paulo, Brazil.
Methods: A questionnaire on headache characteristics was applied to all employers of a telemarketing company in São José do Rio Preto. The
questionnaires were divided into two groups:
with and without reports of headaches. The
headaches were classified into four main groups
according to the criteria of the International
Classification of Headache Disorders: migraine,
tension-type headache (TTH), cluster headache
and other. In terms of reported frequency, migraine and tension-type headache group were
also classified as chronic and episodic. Data
were analyzed by chi-square and significant difference (p<0.05). Results The questionnaire was
answered by 210 workers with an average age
of 23.7 years, 120 men and 90 women. Female
workers suffered comparatively more headache
(81%,p<0.001) then man. 73 women were affected with some kind of primary headache, among
them 29 (40%) had episodic migraine, 23 (32%)
episodic TTH, 8 (11%) chronic migraine, chronic
TTH 05 (7%). The use of oral contraceptive was
associated with headache in 89% (p<0.05) of the
female workers. The employees on contraceptive
use that complained of headache, 60% of them
were affected by episodic migraine. Conclusion:
This study showed that the stressful routine of
telemarketing workers, associated with contraceptive use increased the frequency of headache
in this population. Women who use contraceptives have a higher tendency to primary headache especially migraine type. Some preventive
measures can be adopted for these professionals,
such as better working conditions, therapy and
counseling. Keywords: Woman, headache, migraine, contraception.
P-29
FREQUENCY OF PRIMARY HEADACHE AMONG
EMPLOYEES OF A TELEMARKETING COMPANY IN
THE STATE OF SÃO PAULO, BRAZIL
Paula GR1, Limonte FH2, Andrade RS2, Tanaka MU2,
Vasconcelos RC2, Esteves VB2, Liso E2
• 1FACERES; 2FAMERP
Introduction: The primary headaches usually affect young adults in their most productive
period of work. Aim: The aim of this study was
to establish the presence and frequency of primary headache among employees of a telemarketing company located in São José do Rio Preto - SP. Method: A questionnaire on headache
characteristics was applied to all employees of
a telemarketing company. The questionnaires
were divided into two groups: with and without
reports of headache. The headaches were classified into four main groups in accordance with
the criteria of the International Classification
of Headache Disorders: migraine, tension-type
headache (TTH), cluster headache and others.
In terms of the reported frequency, migraine and
TTH group were also classified as chronic and
episodic. Data were processed by chi-square test
and significant difference (p<0.05). Results: The
profile of the employees of this company consists predominantly of young individuals(n=210;
17‐30 years), most male (n=120). Headaches were
reported by 134 subjects (68%), whit 27% belonging to the TTH episodic group and 19% to the
migraine episodic group. Women suffered comparatively more headache (81%,p<0.001) specifically migraine (73%) than men. TTH was more
frequent among the men (57%, p<0.05). The
chronic migraine was identified in 9 individuals
(4.2%). Headache was a cause of absenteeism for
12.69% of the respondents to the questionnaire.
Conclusion: Primary headache was a common
problem among the employees of this telemarketing company and was an important cause of
absenteeism. Women are more affected by episodic migraine, while men are more affected by
episodic TTH.
P-30
GARCIN’S SYNDROME SECONDARY TO A
CHONDROSARCOMA OF THE SKULL BASE – A CASE
REPORT
Monteiro THOH1, Gonçalves Junior PCJ1, Santos BIS1,
Vilanova CMA1, Rodrigues ÂB1, Araújo MLB2, Vale BP3
• 1UFPI; 2HU-UFPI; 3INSTITUTO DE NEUROCIÊNCIAS
Introduction: Garcin’s Syndrome, described by
the neurologist Raymond Garcin, is a unilateral
global successive paralysis of all or most cranial
nerves on one side. The main cause usually is a
lesion affecting the skull base.1 Chondrosarcoma
is a primary tumor rarely affecting this topography.2 Objective: Describing a Garcin’s Syndrome
due to a chondrosarcoma of the skull base.
Case report: A 27-year-old woman presented
with a headache that had started two weeks ago.
She complained of pain at her left side face, beginning at the forehead radiating to the mentum.
The patitent rated the pain at 10 on a scale of 0 to
10, with 10 representing the worst pain imaginable. She had a similiar episody 8 years ago. After
the latter, she was committed by a weekly headache of lower intensity. The pain had a gradual
progression and she described it as facial burning sensation. Eventually, she reported some
risks when headache was more severe and associated with vomiting, dizziness, edema around
the left eye and itching, tearing, redness of the
left eye. Headache was followed by hypophonia
and dysphagia started 07 months ago. Furthermore, she had balance disturbance and paresthesia at left side of the face. Neurological examination showed nerves palsies of III, IV, V, VI, VII,
VIII, IX, X, XI at the left side. Magnetic Resonance
image (MRI) realized revealed a skull base tumor
at middle and left posterior fossae. She underwent surgery and histopathological specimen
showed a chondrosarcoma of the skull base. The
patient was submitted to chemotherapy since
not all the tumor was resectable. She improved
from the symptoms even thought there was residual lesion at her skull base. Discussion: Garcin’s syndrome is characterized by a progressive
ipsilateral involvement of cranial nerves, leading to paralysis of all or at least seven of them,
without sensory or motor long-tract disturbance.
Several analyses of radiographic computed tomography showed that this type of tumor does
not cause intracranial hypertension or osteoclastic involvement in the skull base.3 MRI allows
early diagnosis of skull base tumor. Nowadays it
is possible to identify Garcin‘s syndrome without
involvement of all hemibasal cranial nerves.1
According to the literature it is rare for this kind
of tumor to lead to this syndrome. Therefore, we
recommend that chondrosarcomas, despite rare,
should be remembered as differential diagnosis
of skull base tumors leading to multiple cranial
nerve palsies.
P-31
IDIOPATHIC HYPERTROPHIC CRANIAL
PACHYMENINGITIS
Jevoux CC, Krymchantowski AV
• HEADACHE CENTER OF RIO
Introduction: Hypertrophic Cranial Pachymeningitis (HCP) is a rare disease with unknown aetiology, characterized by diffuse inflammatory infiltrate in duramater. Several conditions may be
associated, but the majority of the cases are idiopathic (IHCP). Clinical symptoms may include
headache, facial pain, visual loss, cerebellar ataxia and involvement of cranial nerves. Objective:
To raise the awareness for the existence of this
55
Posters
uncommon condition thru the description of
this case. Case report: We describe a 51-year old
man with daily headache since the age of 44. The
headache was always mild to moderate, continuous, with intermittent severe exacerbations,
frontotemporal bilateral location and pressure
quality. At 48 years of age, the pain became severe
and the patient was misdiagnosed as migraineur
and treated with topiramate and flunarizine.
No improvement was observed. During the first
consultation, the neurological exam was normal.
A MRI showed thickness of the right temporoparietoocciptal dura as well as in the posterior
fossae and tentorium. Gadolinium-enhancement of these areas was also demonstrated. The
analysis of cerebrospinal fluid (CSF) revealed
high protein content (94mg/dl) and elevated
pressure (20cmH2O), which was not observed
during subsequent lumbar punctures as for pressure measures. Lab exams are normal except for
erythrocyte sedimentation rate (36 mm/h). The
patient was initially treated with methylprednisolone 1,5g/day (3 days) on a monthly basis.
Headache decreased in intensity during this
treatment, but returned soon after leading to a
daily usage of prednisone 20mg/day with total
headache remission. Azathioprine was attempted but not successful in reducing the pain. A new
MRI gadolinium enhanced was performed after
7 months with no changes. Discussion: ICHP is
a rare disease of unknown etiology, characterized
by diffuse or localized thickening of the duramater and which may be related to a newer disease
entity called IgG4 related sclerosing diseases. The
patient had a normal IgG4 (58mg/dl – normal
8‐140). Steroids may be used and for the unresponsive cases immunosuppressing agents may
be tried. The authors wish to raise awareness for
this rare condition and to the fact that it is a treatable systemic disease, which must be identified
allowing rapid treatment onset.
P-32
IDIOPATHIC INTRACRANIAL HYPERTENSION, A
CAUSE OF VISION LOSS
Souza GR, Sarto R, Schwartz DR, Immich ND,
Queiroz LP, Machado G
• UFSC
Introduction: Idiopathic intracranial hypertension (IIH) is a syndrome of increased intracranial
pressure without ventriculomegaly or expansive
lesion, with normal cerebrospinal fluid (CFS)
composition and unidentified cause. It can lead
to severe visual impairment, due to progressive
retinal nerve fiber layer loss or retinal vascular
occlusion. The annual incidence per 100,000
persons of general population is 1, being 20
times higher in young and overweight women.
Case report: A 32-year-old female, smoker, BMI
of 36 kg/m2, with a history of migraine, was admitted to the hospital complaining of severe left
hemicranial headache, nausea, vomiting, visual
blurring, bilateral tinnitus and fever (temperature was 39.5ºC). Lumbar puncture was performed, with opening pressure of 300 mmH2O,
taken out 4 mL of cerebrospinal fluid, and closed
at 190 mmH2O. CSF exam showed normal values. Cranial computed tomography (CT) Scan,
magnetic resonance imaging (MRI) and retinography findings were normal. Patient diagnosed
with IIH and acute sinusitis. Initiated treatment
with acetazolamide, 500mg every 6 hours and
antibiotic. Advised to lose weight and stop smoking. Patient returns with worsening of vision and
headache. Campimetry indicated decrease in
general sensibility with loss of tubular field in
56
both eyes. Patient was submitted to lumboperitonial shunt. No more symptoms reported after
the procedure. Discussion The diagnosis of IIH
is based on the clinical presentation and evolution of the syndrome, and mainly in laboratorial exams and image diagnostic. It is essential
to perform the differential diagnosis, since the
syndrome is a diagnosis of exclusion. The pathophysiology of IHH is not completely understood.
The most accepted mechanism is that IIH results
from decreased absorption of CSF, confirmed by
slowed circulation of CSF shown by isotope cisternography. Pharmacological treatment of IIH is
performed with acetazolamide in order to reduce
the production of CSF. When there is progressive
visual loss, even with maximum medical therapy,
it is indicated surgical treatment with optic nerve
sheath fenestration or lumboperitonial shunt.
P-34
LIFESTYLE ASSOCIATED WITH PRIMARY
HEADACHE IN EMPLOYEES FROM A
TELEMARKETING COMPANY IN THE STATE OF SÃO
PAULO
Ferreira WA1, Silva KF1, Coutinho RVC1, Freitas KA1,
Mendonça NM1, Noriega AF1, Astolfo RBF1,
Dominianni AT1, Paula GR2, Limonte FH3, Liso E3
• 1UNILAGO; 2FACERES; 3FAMERP
Introduction: The primary headaches such as
migraine, tension-type headache and cluster
headache are the most prevalent and disabling
neurological disorders. Studies showed that
some habits of life may increase headache in the
general population. Aim: To analyze the relationship between the frequency of primary headache
and lifestyle habits of employees from a telemarketing company located in São José do Rio Preto,
Brazil. Methodology: A questionnaire on headache characteristics was applied to all employers of a telemarketing company. The questionnaires were divided into two groups: with and
without reports of headaches. The headaches
were classified into four main groups according
to the criteria of the International Classification
of Headache Disorders: migraine, tension-type
headache (TTH), cluster headache and others. In
terms of reported frequency, migraine and tension-type headache group were also classified
as chronic and episodic. Data were analyzed by
chi-square and significant difference (p l<0.05).
Results: The telemarketing workers that participated in this study (n=210), 59 make use of
alcohol and 61% have some primary headache
type. In terms of the frequency of ethyl ingestion,
a less frequent headache was observed in those
who drink alcohol more than once a week (50%
of them), being preceded by sporadic ingestion
(62%). In the group of the workers who have
headache, 40% drink coffee. The group of the
ones who did not have headache coffee ingestion
is only 21%. The quantity of coffee was any difference in statistic between the groups. The smokers represent 63% of the individuals in the group
with headache; of these, 91% (p<0.001) smoke
more than five cigarettes a day. The workers who
use public transportation showed 71% (p<0.04)
more headaches compared to the users of private
one. Conclusion: The consumption of alcohol is
associated with higher frequency of headache in
these workers. Individuals with headache used to
drinking coffee, but the amount of intake coffee
was not associated with an increased frequency
of headache. Among workers who smoke more
than five cigarettes a day, the great majority
have some type of headache. The workers who
use public transportation showed to have more
headache when compared to the ones who use
private transportation.
P-35
MIGRAINE ASSESSMENT IN PATIENTS SUBMITTED
TO PERCUTANEOUS CLOSURE OF PATENT
FORAMEN OVALE
Richartz M, Rizelio V, Leal AG, Selig FA, Balbi Filho EM,
Santos PSF, Kowacs PA
• INC
Introduction: patent foramen ovale (PFO) is
present in 25% of adult population and has a
well-documented relation with paradoxical embolism and cerebrovascular events. More recently, it has been described an association with migraine, with evidence that percutaneous closure
of PFO can be beneficial to reduce symptoms.
Objective: this study aimed to assess intensity of
migraine in patients submitted to percutaneous
closure of PFO, before and after the procedure.
Methods: It was performed a retrospective study
in a hemodynamic center. The sample comprised 127 patients who had undergone PFO
percutaneous closure for the following indications: ischemic stroke/ischemic transient attack
(G1 - 83%), chronic migraine without response
to drug treatment (G2 - 13%) and other medical
conditions (G3 - 4%). The participants had been
assessed with a headache intensity scale before
(T1) and after (T2) PFO closure. The results in
these two time points were compared. Results:
General prevalence of migraine in T1 was 48.03%
(specifically 41.9% in G1 and 77.3% in G2+G3).
The mean score of migraine intensity in T1 and
T2 was 7 (ranging from 4 to 10) and 2 (ranging
from 0 to 5), respectively. The number of patients
with severe migraine (score 7 or above) went
from 37 to 2 after the procedure. Conclusions:
Percutaneous closure of PFO was beneficial to
reduce migraine intensity in this sample, corroborating current findings in the literature.
P-36
MORNING HEADACHE IN OBSTRUCTIVE SLEEP
APNOEA SYNDROME
Grassi V1, Stelzer FG2, Tomiozzo Júnior JC1,
Fornari LHT1, Eckeli A3, Barea LM2
• 1UNIVERSIDADE FEDERAL DE CIÊNCIAS DA SAÚDE
DE PORTO ALEGRE (UFCSPA); 2IRMANDADE SANTA
CASA DE MISERICÓRDIA DE PORTO ALEGRE
(ISCMPA); 3UNIVERSIDADE DE SÃO PAULO DE
RIBEIRÃO PRETO (USP-RIBEIRÃO PRETO)
Background: Headache and sleep disorders are
common in general population and often coexist in the same patient. Morning headache (MH)
is usually accepted as part of clinical findings of
obstructive sleep apnoea syndrome (OSA). However, there is controversy over the association
of MH and OSA. Aim: The aim of this study was
to estimate MH prevalence and characteristics
among OSA patients. Methods: A transversal and
prospective study including 409 adult patients
(over 18 years old) referred to full night polysomnography (PSG) between January and December
2013, in our Sleep Laboratory at Hospital São
José – Irmandade Santa Casa de Misericórdia de
Porto Alegre (ISCMPA). All individuals were interviewed by neurologists to establish headache
characteristics and to diagnose sleep disorders.
This study was approved by the research ethics
committee of the ISCMPA and all patients gave
written informed consent. Results: There were
409 patients referred to PSG in the study period
and OSA was present in 318 individuals (77.7%).
XXVI Congresso Brasileiro de Neurologia
In the OSA group, MH was reported by 91 patients (28.6%). There was no association between
MH and gender, obesity, race, caffeine use or
chronic pathologies. Headache starts later in life
in MH individuals (40.1±15.2 versus 32.0±16.8
y.o.; p<0.0001). A significantly higher number of
patients with no MH also did not experience any
other kind of headache in the last year (0 versus
30.1%, p<0.0001). MH group more frequently
complained about tiredness upon awakening
(68.1% versus 32.7%, p<0.0001). Chronic headache was more prevalent among MH group
(27.5% versus 7%, p<0.0001). There was no relationship between severity of OSA and prevalence
of MH or chronic headache. No difference was
found in PSG findings, including oxihemoglobin
levels, sleep architecture, sleep fragmentation or
efficiency. MH was not associated to restless legs
syndrome nor to chronic insomnia in this sample. Discussion: MH is a frequent complaint of
OSA patients. OSA may be part of the biological
mechanisms that lead to headache cronification. Further investigations would be important
to elucidate this subject. Disclosure: Nothing to
disclose.
P-37
MYOFASCIAL TRIGGER POINTS CAN TRIGGER
CLUSTER HEADACHE: REPORT OF TWO CASES
Cunha ALN, Ferreira K, Cetlin R, Speciali JG, Dach F
• FMRP USP
Background: Cluster Headache (CH) has well
known triggers, such as the use of antihistamines or vasodilators, alcohol intake, and eating
certain foods. It is well known that these factors
may trigger a hypothalamic sensitization, with
subsequent activation of the trigeminal vascular
system. As a result, there are release of pro-inflammatory substances, neurogenic inflammation and pain. Myofascial trigger points have
been reported, in a few articles, as precipitating
factors in CH. Objectives: this study aims to
show the association between myofascial trigger points and CH. Clinical cases: Case 1- male,
35 years old, history of Episodic CH for 10 years.
After a complete remission for one year, the new
cluster period started two months before the
consultation.The pain occurred daily, usually at
night, lasted 30 minutes and was triggered by
physical exercise, such as playing tennis. He was
treated with verapamil 80 mg 8/8 h and prednisone 40mg for 5 days without improvement. On
examination there were trigger points in trapezius, splenius capitis and splenius cervicis muscles (at right). After blocking these points with
lidocaine, there CH attacks remitted completely.
Case 2- male, 31 years old, with history of CH
for 14 years, with long periods of remission. He
has already been treated with verapamil, lithium, valproic acid, prednisone and naratriptan
successfully. In October 2013 the CH attacks
started again. On examination, a trigger point in
the right trapezius muscle was found. No other
specific medication for CH improved the pain except the blockage of that myofascial trigger point
with lidocaine plus resting the affected muscle.
Discussion: Myofascial trigger points have been
reported to contribute to CH, as Calandre et al
had already described, but studies about myofascial trigger points in CH are still scarce. In our
patients, anesthetic blockage of these myofascial
trigger points resulted in complete remission
of pain, which corroborates to our hypothesis
that these myofascial points may have a role in
the trigeminal activation. Thus, we would like to
clarify the importance of full examination of the
head and neck muscles for all patients with CH.
P-39
PARATRIGEMINAL RAEDER SYNDROME
AS MANIFESTATION OF EPIDERMOID
NASOPHARYNGEAL CARCINOMA. CASE REPORT
Bahia CMCS, Pinto TS, Araújo RBC
• HOSPITAL NORTE D‘OR
Introduction: Raeder paratrigeminal syndrome,
also known as paratrigeminal neuralgia, is a rare
condition characterized by severe, unilateral facial pain and headache in the distribution of the
ophthalmic division of the trigeminal nerve in
combination with ipsilateral oculosympathetic
palsy or Horner syndrome. There are 2 subtypes
of Raeder paratrigeminal syndrome: with and
without parasellar nerve involvement. Objective:
Report a case of a middle age man, with paratrigeminal Raeder syndrome and epidermoid nasopharyngeal carcinoma. Case report: 46 year-old
man, presented five months before admission
with left retrorbitary pain and left nasal discharge. He looked for an otorhinolaryngologist‘s
opinion and received the diagnostic of sinusitis.
Despite appropriate treatment, retrorbitary pain
continued worsening. He was evaluated for several doctors and made some exams with normal
results. He was using analgesics everyday to control the symptoms. Five moths later he noticed
he was with double vision and he decided search
for hospitalar care. On admission he had pain on
left ophthalmic branch of trigeminal nerve and
ipsilateral six nerve palsy, Horner syndrome and
nasal discharge. His initial workup with sinus tomography , brain and orbit magnetic ressonace
was considered almost normal, only with thickening of left sphenoidal and maxilar sinus, which
was initially considered as sinusitis. So, he was
treated for sinusitis again and oral corticosteroids was introduced for Tolosa Hunt syndrome.
His symptons had only partial relief. So, otorhinolaryngologist was called to perform a rhynoscopy, which demonstrate an infiltrative lesion on
maxilar sinus mucosa. The biopsy was made and
the result was consistent with nasopharyngeal
epidermoid carcinoma. Discussion: In this case
the infiltrative nature of lesion difficult the diagnosis since it was missed on initial analysis of the
image exams. However paratrigeminal Raeder
syndrome, especially with parasellar nerve involment, should always raise the hypothesis of
tumoral etiology, requiring an extensive workup. Epidermoid nasopharyngeal carcinoma is
a rare malignant tumor, with major incidence
in males with 50‐60 years old. Its diagnostic is
unfortunately made on advanced phases when
there is invasion of adjacents structures. Tolosa
Hunt syndrome is a differential diagnosis as it
can present with painfull ophtalmoparesis and
Horner syndrome in rare cases.
P-40
large load of stress generated by extensive curricular obligations. Objectives: To estimate the
prevalence of primary headache in medical students and residents, to quantify the degree of
disablement and to identify the characteristics of
headache. Methods: This was an observational
cross-sectional epidemiological study, conducted through questionnaires (ICHD-II, MIDAS and
HIT‐6). 140 medical students and 11 residents of
the Federal University of Amazonas were evaluated from September 2012 to May 2013. Results:
23 (16.43%) cases of migraine, 9 (6.43%) cases
of probable migraine and 33 (23.57%) cases of
the tensional headache were identified. Nine
subjects (6.43%) reported no headache. Other
16 (11.42%) had secondary headache (trauma,
infections and MAV). Females predominated in
all groups and represented 57.26% of the total.
This group had higher scores on the HIT‐6 test,
probably due to a higher prevalence of migraine
(OR =3.48). Most subjects reported pain consisting of a clenching or pressure sensation, bilateral
and fronto-occipital, lasting less than 4 hours.
The MIDAS and HIT‐6 scores were not related to
age, period or schedule of extracurricular activities. Migraine and probable migraine had higher scores than the other types of headache, and
therefore higher levels of disablement. There
was no relationship between work and severity
of headache. Conclusions: This study found that
the period of medical school or extracurricular
activities do not weight significantly on the degree of impact caused by headache and women
were most susceptible to headaches, particularly
migraine, which is associated with higher levels
of disablement. Keywords: Headache, Medical
Students, Migraine
P-41
PREVALENCE OF HEADACHE IN PATIENTS AFTER
CRANIOTOMY - A REVIEW
Leite TT1, Torres MO2, Leite DT1, Vasconcelos IA3
• 1EBMSP; 2EBSMP; 3FUNORTE
The analysis of literature data, from 2004 until
august 2014, focused on headache after craniotomy and its prevalence as well as risk factors
in neurosurgical patients, was performed in the
present review. It was shown that the problem of
postoperative headache in pacients after craniotomy has been underestimated for a long period of time. It is known that there are international scores for evaluate head pain in those patients,
however, that is no standardization as to its use
in worldwide centers of reference. The results
of recently studied shown that 45‐91% of the patients complain about headache after craniotomy. Untreated postoperative pain coul cause
a number of serious complications. This article
describes the epidemiollogical profile, the main
rates of prevalence and usual commorbidities in
those patients.
P-42
PREVALENCE OF HEADACHE IN MEDICAL
STUDENTS AT THE FEDERAL UNIVERSITY OF
AMAZONAS
PREVALENCE OF HEADACHE IN PATIENTS WITH
MOOD DISORDERS AND ANXIETY ACCOMPANIED
IN AMBULATORY OF REFERENCE IN SALVADOR BAHIA
Mota RSS, Lima PAMS, Stabenow R, Vieira KS,
Sousa RAP, Birmam D, Almeida CMO, Takatani M
Silva LSA1, Melo BA2, Rodrigues AL2, VilasBôas ÍGM3,
Silva EM4
• HUGV - UFAM
• 1EBMSP; 2UNIFACS; 3UFBA; 4AMBULATÓRIO
MAGALHÃES NETO, HOSPITAL DAS CLÍNICAS
Introduction: Headache is a highly prevalent
complaint and causes major impact on the patients‘ quality of life and productivity. Medical
students are particularly susceptible due to the
Introduction: Headache is a major health problem in Brazil, representing more than half of
the searches for outpatient care in neurology
57
Posters
and one of the main complaints in emergency
departments. Studies show that the prevalence
of headache in one year is 80.8% of the general
population, with 40.3% of these individuals fitting into the diagnosis of migraine. The literature
demonstrates a reciprocal and complex relationship between headache and mood, revealing high prevalence of the first in patients with
mood disorders and anxiety. Damages from the
association of these comorbidities imply disabilities and reduced academic performance ±
professional, affecting the quality of life of individuals. Objectives: To describe the prevalence
of headache in patients with mood ± anxiety as
outpatients of psychiatry at the “Hospital das
Clinicas” in Salvador. Methodology: This was
a descriptive-analytic study, transversal cutting and observational, which is comprised by
patients assisted in the psychiatry ambulatory
of the “Hospital das Clinicas” for treatment of
mood disorder ± anxiety in the period April-August 2014. To confirm the diagnosis of mental
disorder, the data records were analyzed. After selection of patients with mental disorders,
questionnaires were prepared according to the
criteria of the international classification of
headache in order to establish diagnosis for the
type of headache, if present. The sample size calculation revealed an n=74 patients. The variables
collected were stored in database built with the
SPSS 17.0 for Windows. To investigate the association between psychiatric disorders and the
presence or absence of headache, we used the
chi-square test. Results: 75.7% of patients had
headache. Among the types of headache, 37.5%
of patients were diagnosed with infrequent episodic migraine; 25% with frequent episodic migraine; 12.5% with chronic migraine; 3.57% with
tension-type headache (TTH) frequent episodic;
7.14% with infrequent episodic TTH: 5.35% with
chronic daily TTH and 5.35% with other types
of headache. Patients with isolated depressive
disorders (39.18%) had some type of headache
in 93.10% of cases; patients with isolated anxiety disorders presented headache in 59.09% of
the cases. A high correlation between mood and
headache (p=0.042) was found. Keywords: Primary Headache; Mood disorder; Anxiety Disorder; depression; Anxiety.
P-46
QUANTITATIVE STUDY OF HEADACHE FREQUENCY
AND CAUSES IN MEDICAL STUDENTS
Oliveira LTD, Lodi PR, Duarte PF, Boneli CF, Carneiro LS,
Martins TPS, Oliveira LBA, Viana EM
• UNIFENAS-BH
Headache is a pain that takes place inside the
area of the skull and it may be unilateral or bilateral, episodic or chronic, intermittent or continuous. Headache is a frequent cause of visits to
healthcare clinics and one of the most frequent
reasons patients seek healthcare. Because of the
impact this type of pain can have on the quality
of life of those who have it, a quantitative study
was carried out using a question form that included eleven closed questions, based on studies
that had been validated by the scientific community, including sixty medical students with the
purpose of assessing headache incidence, main
causes, main associated symptoms and the impact of this pain on people‘s social life and their
ability to concentrate. It was additionally found
that although this pain is a common symptom
in the population studied, the chronic headache,
which is characterized by a pain that includes
more than 15 headache episodes a month,
58
occurs in 45% of respondents. When asked what
means they seek for nursing such pain, 48% answered that they seek self-medication, therefore
confirming the trend reported in previous studies with students, where most of them, 53% reported the occurrence of headache cases in their
families. This confirmed that there is a hereditary
tendency to headache, as previously reported by
other authors, regarding first-degree relatives.
Results showed that headache was associated
with nausea, photophobia, phonophobia and
vomiting, where stress, eating habits, emotional reasons and sleep deprivation were the most
cited inducing factors. More specifically of the
study subjects, stress was an outstanding issue.
One possible explanation for the correlation between stress episodes and headache is the pain
caused by vasoconstriction of arteries and veins
that supply blood to the head area. In stressful
situations, our system responds by releasing
large doses of some hormones such as adrenaline and cortisol which, together, can significantly increase vasoconstriction. As for the impact on
social life and productivity, 73% of respondents
reported that they did not stop their social, family or entertainment activities because of their
headache, although 47% of them said they feel
they are limited with regard to their focus and
ability to study because of the pain. It was concluded that headache is a recurrent condition
among college students and their causing factors
are strongly associated to the study routine of
this population.
P-47
SELF-HYPNOSIS IN THE TREATMENT OF PRIMARY
HEADACHE: A LITERATURE REVIEW
Vilasbôas ÍGM1, Porto FB2, Lessa KP3, Araújo Filho JEO1,
Silva LSA2, Souza MP3
• 1UFBA; 2EBMSP; 3UNEB
Introduction: Headaches are a very common
complaint in clinical practice. About 95% of the
people have an episode or manifest it throughout life. Primary headaches refer to cases in
which the headache is the disease itself, but not
related to any other comorbidity, featuring potentially disabling symptoms. Self-hypnosis is
a technique recently applied to medicine that’s
being used to treat certain psychosomatic disorders and chronic pain. Among chronic pains,
there is an increasing use of this therapy in the
treatment of primary headaches. Objective: To
assess the benefits of self-hypnosis in the treatment of primary headaches. Methods: A Review
of Literature was performed based on BVS (VHL),
Pubmed and Capes Portal database, as well as on
references of the reviewed articles. Clinical trials,
cohort studies and Case reports since 1975 that
evaluated the effectiveness of self-hypnosis in
the treatment of primary headache were included. Results: All of the 11 selected articles showed
significant improvement in pain patterns. Five
studies assessed primary headaches in children
while six studies did it in adults. In terms of types
of headache, 2, 4 and 5 articles evaluated migraine, tension-type headache and other primary
headaches, respectively. These data demonstrate
direct benefits to patients in different age groups,
but reinforce that children are more responsive
to treatment. Both migraine and tension headache showed decline of the variables of frequency and intensity. Conclusion: There is evidence
of clinical improvement in patients undergoing
self-hypnosis to treat primary headache. There
is decrease of symptoms, as well as subjective
improvement in the sense of well-being. Further
studies are needed to demonstrate the effectiveness of this therapy better.
P-49
SODIUM DIVALPROATE. ALTERNATING DAILY
DOSES DOES NOT DECREASE THE WEIGHT GAIN
Krymchantowski AV, Jevoux CC
• HEADACHE CENTER OF RIO
Background and objectives: Migraine is a highly
prevalent primary headache. Preventive treatment is frequently needed. Sodium Divalproex
(DVP) is an effective neuromodulator for migraine prevention, but induces weight gain. The
objective of this study was to evaluate whether
alternating daily doses of DVP would decrease
weight gain and maintain efficacy. Methods:
Forty eight patients with migraine and headache
less than 15 days/month were retrospectively studied. All were using DVP in monotherapy
(500mg/day ER formulation), presented headache frequency reduction of higher than 50%
and gained weight after two months. During the
following two months, the patients alternated
daily doses ER of 500 and 250mg and the weight,
headache frequency and treatment adherence
were evaluated. Results: Forty three patients
(35 women, 8 men) with ages between 19 and
60 years (mean 35,8) and average frequency of
0,21 headache days per week completed the
study. Among them, 89,6% had migraine without aura and 10,4% with aura. The mean time of
headache was 19,8 years and the average weight
gain after two months was 1,7 Kg (900g‐2,8Kg).
Twenty nine (67,4%) patients maintained weight
gain (mean 1,4Kg) after two more months with
alternated doses and 16 (37,2%) presented headache frequency increase. Five female patients
with weight gain between 1,1 and 2,3Kg were
lost for follow up. Conclusions: Despite the retrospective design, alternating daily doses of DVP
doesn’t seem to reduce the weight gain and do
not increase headache frequency in most of the
patients. This study was also limited by the small
number of patients under monotherapy in our
center.
P-50
SUNCT – LIKE HEADACHE IN VERTEBRAL
DISSECTION
Amorim TA, Rocha Filho PAS, Costa Filho JTC, Sá FLC,
Lacerda KM, Holanda GN
• HUOC
Introduction: SUNCT is a rare primary headache
syndrome included in the group of trigeminal
autonomic cephalalgias, in which unilateral pain
has to be associated, by definition, with both ipsilateral conjunctival injection and lacrimation.
Although SUNCT syndrome is usually of primary
etiological nature, secondary causes, especially
injury of the pituitary gland and posterior fossa, are well known. Spontaneous cervical artery
dissection (sCAD) is a well recognised cause of
stroke, with a wide spectrum of clinical presentations. The typical clinical manifestations of
spontaneous vertebral artery dissections (sVADs)
are thought to be occipital headache, posterior
neck pain or both. SUNCT syndrome as a clinical manifestation of a vertebral dissection is
an uncommon but possible event. Objective:
Describe a case of SUNCT syndrome associated
with a vertebral artery dissection. Case report:49
years old male presenting with 20 days history
of excruciating headache lasting for seconds on
XXVI Congresso Brasileiro de Neurologia
the V1 right trigeminal territory, accompanied by
ipsilateral conjunctival hyperemia and tearing.
Patient was admitted to our service for investigation of secondary causes, before completion of
MRI and MRA, patient developed a Wallenberg
syndrome confirmed by images that showed
ischemic insult in the right dorsolateral region
of the medulla and inferior cerebellar peduncle,
as well as ipsilateral vertebral artery dissection.
Patient underwent conservative treatment and
on the time of discharge had no pain complaints
and partial improvement of other neurological
deficits. Discussion: Headache is a common
clinical manifestation of dissections with variable localization as well as other neurological
deficits. 91% of dissections presents itself with
ipsilateral pain which 61% was in frontotemporal
region. Migraine, Hemicrania, cluster headache
have all been described as resulting from vertebral dissection, however disautonomic symptoms presence is unusual. To our knowledge
there is only one other reported case of SUNCT
syndrome resulting from vertebral dissection. Ipsilateral cranial parasympathetic activation and
sympathetic hypofunction have been implicated
in the pathophysiology of autonomic symptoms
in these patients. The trigeminovascular reflex
plays an importante role in the pathophysiology
of trigeminal autonomic cephalalgias. Recognition of such rare headaches secondary to relatively common vascular events is important in
instituting the appropriate treatment.
P-51
SYNDROME OF TRANSIENT HEADACHE AND
NEUROLOGICAL DEFICITS WITH CEREBROSPINAL
FLUID LYMPHOCYTOSIS (HANDL): A CASE REPORT
Silva LG, Tinone G, Conforto AB, Calderaro M,
Yamamoto FY, Ono CR
• FMUSP
Introduction: The syndrome of transient headache and neurologic deficits with cerebrospinal
fluid lymphocytosis (HaNDL) is characterized by
episodes of migraine-like headache, associated
with neurologic deficits, most commonly hemisensory alterations and aphasia or paraparesis,
as well as lymphocytic pleocytosis and elevation in cerebrospinal fluid (CSF) protein levels.
Such episodes usually recur within 3 months.
Objective: To describe the clinical presentation
and six-month follow-up of a 33-year-old woman
who was ultimately diagnosed with HaNDL syndrome. Case report: Our patient presented with
a 2-hr episode of headache, described as oppressive, bilateral temporal pain, associated with
numbness that evolved from the right leg to the
right arm and hemiface. Neurologic examination
revealed mild hemiparesis and hemihypesthesia. Brain CT scan was normal and CSF analysis showed lymphocitic pleocytosis - white cell
count (WCC) 167/mm3 - and slight increase in
protein levels (57mg/dL). Microbiologic tests, including bacterial cultures and PCR for Herpes viruses (types 1, 2, 6 and VZV) were negative. Brain
magnetic resonance imaging and angiography
were unremarkable. The patient presented a total of five episodes of headache associated with
neurologic impairment within 16 days. Three
episodes had the same features previously described, and lasted up to 1h. After the second and
third episodes, CSF analysis revealed increasing
lymphocytic pleocytosis of 295/mm3 and 580/
mm3, on consecutive episodes, and protein levels of 96mg/dL and 134 mg/dL. The remaining
two episodes were characterized by hypesthesia
in the left side of the body with a maximum duration of 20 minutes and full recovery. Four days
after the last episode, a control CSF analysis
showed improvement in WCC and protein levels,
which dropped to 145 cells/mm3 and 78 mg/dL,
respectively. She remained asymptomatic for the
following six months and routine clinical evaluations showed progressive improvement and CSF
normalization. Discussion: HaNDL syndrome
is a diagnosis of exclusion, since its presentation could be the same of serious conditions like
stroke, vasculitides and meningitis. It is probably
under-reported. Its precise etiology is still uncertain. Some theories postulate that it is caused by
a cortical depression-like mechanism, similar
to the proposed for migraine, possibly triggered
by viral infections. We present a case that fits the
syndrome as described in literature.
P-52
TOLOSA-HUNT SYNDROME – A CASE REPORT
Costa ALC, Ferreira LS, Carneiro MRP, Rigon BGS,
Silva DS, Bueno FF
• UNICAMP
Introduction: Tolosa-Hunt Syndrome (THS) is
a rare diagnosis that consists of painful oftalmoplegia combined with a periorbital and/or
hemicranial pain ipsilateal. The etiology of this
syndrome is not yet clarified. an inflammatory
process in the region of the cavenous sinus and
superior orbital fissure is the cause of the symptoms. Neurological involvement include combinations of third, fourth, sixth and first division of
the fifth cranial nerve palsies. It is a diagnosis of
exclusion after dispose of other causative lesions
and with pain relief after corticosteroid therapy.
Objective: report a case of ths that, althouth is
rare, should prompt considerated in unilateral
headache associated with cranial nerve palsy.
Case report: a 53-year-old woman with no comorbitidies came to our hospital with a 2 weeks
history of left retrorbital pain and hemicrania
headache ipsilateral plus diplopia and left ptosis. The neurological exam showed incomplete
paralysis of the iii, iv and vi left cranial nerves associated with ipsilateral diplopia in all directions.
Pupilar reflex was preserved. mri after contrast
identified enhancement in left optical nerve in
intraorbitary portion and superior orbital fissure
ipsilateral. The blood and csf test were normal.
treatment with 1mg/kg/day of prednisone was
initiated promptly and patient presented pain
remission 48 hours after. she also had improvement on paresis in cranial nerves. MRI performed 6 months after showed no abnormalities.
Discussion: the pacient presented had fulfilling
criteria for tsh. a careful evaluation was perfomed
to rule out other etiologies. MRI findings before
and after treatment was important in diagnosis.
this patient after treatment, revealed resolution
of the contrast enhancement plus a dramatically
relief of the orbital-periorbital pain with recovery
of cranial nerve palsy after treatment. The excellent response to the beginning of corticotherapy
and exclusion of other differential diagnosis established ths diagnosis. Conclusion: although
Tolosa-Hunt Syndrome is a rare cause of unilateral headache with cranial nerve palsy, these
findings should lead to prompt consideration of
this syndrome since it causes considerable morbidity and the institution of corticosteroid therapy leads to dramatic relief of the symptoms and
may change the natural course of disease.
P-53
TOLOSA-HUNT SYNDROME: A CASE REPORT.
CLINICAL FINDINGS AND RESPONSE TO THERAPY
Mota RSS, Alves TLIA, Vieira KS, Sousa RAP, Birmam D,
Rabelo RMP, Takatani M
• HUGV - UFAM
Introduction: Tolosa-Hunt syndrome is a painful ophthalmoparesis caused by a nonspecific
granulomatous inflammation of the cavernous
sinus and superior orbital fissure. Patients often present retro-orbital or periorbital pain of
acute onset, associated with symptoms such as
diplopia, paresis of the third nerve, visual loss
and paresthesias in the face, in the territory innervetated by the first branch of the trigeminal
nerve. Tolosa-Hunt syndrome is usually unilateral, however, bilateral cases have been described.
It is a exclusion diagnosis and can only be made
when other potential causes of painful ophthalmoparesis are discarded. Satisfactory and standardized response to corticosteroids, associated
with a benign course reinforce this diagnostic
possibility. It is necessary, therefore, extensive
complementary investigation. Objectives: To
present a Case report of a patient treated at our
institution, whom had a previous diagnosis of
migraine and evolved with a changing pattern
of headache, and clinical findings suggestive of
the syndrome, having satisfactory response to
treatment with corticosteroids. Case report: A
28 year old woman reported a persistent fronto-temporal throbbing headache of strong intensity, with progressive worsening of pain intensity
for 3 weeks, presenting after this period ptosis,
loss of visual acuity and and paresis of the 3rd
right cranial nerve. Brain MRI revealed a focus
of hyperintense signal on T2 and FLAIR, isolated in the white matter of the left frontal lobe of
nonspecific appearance. The patient was treated
with corticosteroids and had a great response
to therapy. Evolved, however, with progressive
weight gain and glucose intolerance, changing
the therapy then to azathioprine, showing no
satisfactory answer. No biopsy was performed to
confirm the diadgnosis. Discussion: Tolosa-Hunt
syndrome is a painful ophthalmoparesis characterized by unilateral orbital pain and paresis of
eye movement, with a good response to therapy
with corticosteroids. Since the clinical features of
the syndrome are not specific, imaging tests and
histopathological findings are resources that can
assist the diagnosis and consequently the correct
therapy. However, it is invasive, and biopsy is not
routinely performed. Among the differential diagnoses are ophthalmoplegic sarcoidosis and
migraine. Key words: Tolosa Hunt syndrome,
cavernous sinus, painful ophthalmoparesis.
P-54
TOLOSA-HUNT SYNDROME: CASE REPORT
Almeida NDF, Sousa RD, Iwamoto KOF, Gemus GF
• UFG
Introduction: Tolosa-Hunt Syndrome (THS)
is a disease with an estimated incidence of 1:
1000000 and unknown etiology. It is characterized by stenosis of the cavernous portion of
the internal carotid artery in its intracavernous
segment, associated with involvement of one
or more cranial nerves traversing the cavernous
sinus. Is expressed clinically by unilateral orbital
pain associated with paralysis of one or more cranial nerves, particularly the oculomotor, that can
resolve spontaneously over time. The diagnosis
requires the exclusion of other known causes of
59
Posters
Painful Ophthalmoplegia (PO). Objective: To report a case of a patient with THS, featuring their
clinical, radiological shape and the response to
corticosteroids. Methodology: We used for this
study hospital patient monitoring and literature
search in the databases of Medline, PubMed and
SciELO. Case report: Patient, male, 36 years old,
started 4 months ago a clinical of intense unilateral eye pain, diplopia and right eyelid ptosis. Refers headache in periorbital region. Started doses
of prednisone, the pain persisted after 15 days,
with no improvement in eye movement and
headache. Evolved, right, with mydriasis, small
extrinsic ocular motility and paresthesia in upper
hemifacial - in addition to nausea, vomiting and
asthenia. Computed tomography (CT) showed a
filling defect in the cavernous sinus and Magnetic Resonance Imaging (MRI) showed thickening
in surveying the cavernous sinus and maxillary
sinusitis. After four weeks with corticosteroids
was clinical and imaging remission, concluding
the diagnosis of THS. Discussion: The diagnosis
of THS requires the exclusion of other causes of
PO, since the corticosteroid reverses the signs
and symptoms of both, THS as tumors, such as
lymphoma, meningioma, and tumors of the orbit, without inverting the image result. In 2004,
the International Headache Society (IHS) has
redefined the diagnostic criteria for THS, specifying that granuloma demonstrated by MRI
or biopsy is required for diagnosis. In our case,
besides the clinical fitting in all IHS criteria, the
MRI before and after treatment with corticosteroids was important in case closure. Conclusion:
THS has no biological marker, therefore, the exclusion of other causes of PO is still mandatory.
The use of the IHS criteria supports the diagnosis always when there is clinical suspicion of this
rare and still puzzling disease.
P-55
TRIGEMINAL AUTONOMIC CEPHALALGIA
RESPONSIVE TO PRE-GABALINE
Sanvito WL, Monzillo PH, Nemoto PH, Zetehaku AC,
Lima BM
• SANTA CASA DE MISERICORDIA DE SÃO PAULO
Introduction: The trigeminal autonomic cephalalgia (TACs) includes features of severe unilateral periorbital/temporal pain with parassimpathetic autonomic symptoms/signals. Cluster
headache, paroxysmal hemicrania, short lasting
unilateral neuralgiform headache represents
TACs. Cluster headache lasts 15‐180min and has
a frequency one every other day to eight times
a day. Paroxysmal hemicrania has duration between 2‐30min at least three times a day, with response to indomethacin. Short lasting unilateral
neuralgiform headache lasts for 1‐600 seconds,
occurring in stabs. Conjunctival injection and
tearing are presented in SUNCT (short lasting
neuralgiform headache attacks with conjunctival injection and tearing), while in SUNA (Short
lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms) only
one or neither of these symptoms are presented. The irregular course brings difficulty in the
management of these cephalalgias. Objective:
Case report of SUNCT that improvement with
pre-gabaline. Case report: J.A.L. 68 years old
man. He referred a severe, left, fronto- temporal
60
headache occurring as stabs for 03 days, associated to tearing, rhinorrhea and conjunctival
injection ipsilateral. Pain occurs 100x/day and
lasts for 3seconds, without improvement with
simple analgesia. In the first avaliation, he presented pain in left temporal area with autonomic
activation and periorbitary edema ipsilateral. It
was prescribed dipirone and flebocortid without
improvement. General tests (except for VHS) and
image Methods: were normal. SUNCT was hypothesized. Haldol was chosen without improvement, and then it was prescribed prednisone and
quetiapine with a little relief. Carbamazepine
was associated with poor response. Increase in
quetiapine and carbamazepine doses did not
revealed improvement. We introduced pre-gabaline. After 15 days, there was improvement of
headaches, occurring 3times/day with autonomic activation only in a few attacks. Discussion:
SUNCT is a primary headache characterized by
unilateral, moderate to severe pain, associated
to autonomic activation. The trigeminal efferent
stimulus seems to result in autonomic symptoms
by trigeminal-autonomic reflex. The functional
neuroimage reveal activation in hypothalamics
centers in pain and autonomics disturbances
origens. Lamotrigine, carbamazepine, gabapentine, lidocaine, occipital nerve block showed efficacy in SUNCT treatment. In this Case report it
was improvement of pain with pre-gabaline.
P-56
VARIABLES ASSOCIATED WITH THE ANALGESICS
OVERUSE IN PACIENTS WITH MIGRAINE
Almeida KJ1, Feitosa Neto JC2, Carvalho LCLS2,
Soares RBV2, Ponte MPTR1, Silva Neto RP1,
Santos RVSG3
• 1UFPI; 2FACID; 3UESPI
Introduction: Migraine is a frequent and disabling painful disorder among the primaries
headaches. According to Global Burden of Disease Survey (2010), migraine was ranked as the
third most prevalent disorder and seventh-highest specific cause of disability worldwide. Analgesics overuse is a frequent condition that
contributes to worsening on headache pattern.
In migraine, analgesics overuse is a risk factor
to become pain chronic, when it is diagnosed a
chronic daily headache or medication-overuse
headache. Aims: Analyse the clinics variables
associated to analgesics overuse in patients with
migraine and probable migraine. Methods: Retrospective transversal study that included patients with diagnosis of migraine and probable
migraine according to international classification of headaches disorders (third edition) evaluated in 2013 and 2014. It was accomplished a
multivariate logistic regression analysis by having as dependent variable the diagnosis of analgesics overuse and as independents variables
the epidemiological data and findings related to
pain. It was regarded the “p” value lower than 5%
to dispose of null hypothesis and a confidence
interval of 95%. Results: The sample included 266 patients with mean age of 33,31±13,10
being 21,8% of the patients female. Analgesics
overuse was diagnosed in 21,8% of the patients.
There was correlation, according the univariate
analysis, between analgesics overuse and the
following variables: Duration of pain (p<0,001)
and patients with pain five years ago or more
(p=0,021). The absence of unilateral pain associated with bilateral pain was correlated with the
absence of the overuse (p=0,022). In multivariate
analyses only the mean time of pain duration
was correlated with analgesics overuse (p=0,019).
Conclusion: The bigger mean period of pain duration was the variable associated independently
with the analgesics overuse in patients with migraine or probable migraine.
P-58
“KEEP YOUR EYES OPEN!”: A 51 YEARS OLD MALE
WITH THUNDERCLAP HEADACHE, BILATERAL
PTOSIS AND MENINGITIS
Moura AA, Rezende Filho FM, Silva CC, Ferraz ME
• UNIVERSIDADE FEDERAL DE SÃO PAULO - UNIFESP
Introduction: Although most clinicians would
attribute a thunderclap headache (TH) to subarachnoid hemorrhage, there is a wide range of
differential diagnosis to consider. Neurologists
must keep their eyes open, because details in
history and physical examination may point to
the underlying disease. Objectives: To describe a
case presenting with TH associated with unusual
signs and symptoms. Case report: A 51 years old
male presented with a 4 days history of sudden
onset headache, fever, nausea and vomiting. He
complained of drooping eyelids and said “a dark
curtain had fallen over the left side of my vision”.
His medical past was unremarkable, except for
bipolar disorder. On examination he was febrile
(38,4 Co) and tachycardic (140 beats/min). Neurological exam showed complete bilateral ptosis
with a frozen gaze, left hemianopia and nuchal
rigidity. With presumed diagnosis of meningitis,
computerized tomography (CT) was performed,
followed by lumbar puncture and full lab work.
Enlargement of the sellar region was seen on CT,
and cerebrospinal fluid (CSF) was xanthochromic, with neutrophilic pleocytosis (374 cells/
mm3; 73% neutrophils) and high protein level
(127mg/dl). As cavernous sinus thrombosis was
a concern, the patient went thru CT angiography,
which depicted no abnormalities. He received
antibiotics and steroids. Magnetic resonance
imaging of the sella turca disclosed an enlarged
pituitary gland compressing optic chiasm and
extending a both cavernous sinus with T1 and
T2 hyperintensity compatible with pituitary apoplexy. CSF cultures were sterile and blood tests
revealed pan-hypopituitarism. On discharge patient had no pain and was regaining eye movements. Discussion: Although aneurysmatic
subarachnoid hemorrhage is a frequent cause
of TH, many other conditions may display this
symptom, including central venous thrombosis,
cerebral reversible vasoconstriction syndrome
and primary thunderclap headache. Pituitary
apoplexy is a rarer cause of TH, and most often
results from hemorrhagic infarct in a previously existing macroadenoma. Symptomatology is
closely related to the structures located in the
cavernous sinus as oculomotor nerves palsies,
albeit frozen gaze was seldom reported. Vision
loss is common but meningitis is unusual and
may arise from tissue debris in the CSF, activating inflammatory pathways. Treatment is mainly
supportive.
XXVI Congresso Brasileiro de Neurologia
P-64
Cerebrovascular Diseases
ACKNOWLEDGMENT OF THE CEREBROVASCULAR
ACCIDENT BY COMMUNITY AGENTS THROUGH
QUESTIONNAIRE IN A BASIC HEALTH UNIT
Peixe AAF, Minohara SF, Dórea EL
• UNICID
P-59
A DURAL ARTERIOVENOUS FISTULA AS A
COMPLICATION OF A CEREBRAL VENOUS
THOMBOSIS
Laurentino M, Correia CER, Vasconcelos TF,
Godeiro Junior CO, Figueiredo MM, Dourado ME,
Melo CO, Morais PS, Medeiros LL, Costa MF,
Marciano LA
• UFRN
Introdução: Cerebral venous thrombosis(CVT)
is a rare condition (3‐4 / 1,000,000), seen mostly
in women before age 40. Currently, due to increase of popularity of Magnetic Ressonance, a
larger number of cases and their complications
are being diagnosed. Objective: To report a case
of dural arteriovenous fistula (DAVF) due to CVT.
Relato: female, 31 years old, previously healthy
and prolonged use of oral contraceptive, with
a history of headache and dizziness, diagnosed
with CVT 6 months earlier. In appropriate treatment with oral anticoagulants(warfarin), evolved
with persistent headache and progressive bilateral visual loss. Physical examination revealed
bilateral papilledema and audible retromastoid
murmur on the right. During the investigation,
lumbar puncture was performed (opening pressure of 34cmH20) and Magnetic Ressonance Angiography showed signs of thrombosis in superior sagittal sinus and reduced caliber with filling
defects in the left transverse sinus. Additional
Cerebral Angiography evidenced thrombosis of
the superior sagittal sinus, left lateral sinus partially repermabilized and DAVF of the torcula region. Initially, a lumbperitoneal shunt (LPS) was
performed, which was removed a week later due
to intracanial hypotension. After 10 days, the embolization of the fistula was performed with debt
reduction. The patient experienced resolution of
headache, however, the vision remained stable
without any improvement. Discussão: The CVT
has a variable clinical presentation, most commonly with headache(70‐91%), focal deficts(34
‐79%), papilledema(50%) and seizure(40%). Other symptoms are diplopia, blurred vision and
impaired consciousness. The prognosis is usually favorable when treatment is started early.
Among the complications, venous bleeding is
the most frequent, while the DAVF occurs in only
6% of cases. Fistulas are abnormal connections
between dural arteries and dural veins or sinuses
and correspond to 10‐15% of all cerebrovascular
abnormalities. The most common predisposing
factor is venous sinus thrombosis. The dural sinus involved receives arterial blood flow that
leads to mechanical obstruction and retrograde
blood drainage out of the cortical vein. Aggressive features are due to venous hypertension and
the neurological deficits are secondary to arterial
steal. Unfavorable outcome of CVT, despite adequate treatment, should alert to the presence of
DAVF.
Introduction: The cerebrovascular accident
(CVA) has important indexes of morbimortality.
The Federal Government prioritizes its combat focusing on prevention. The acknowledge
and appropriate approach regarding the CVA,
reduce the mortality rates as well as sequelae.
Objectives: Evaluate the degree of awareness
about CVA by the community health agents
(CHA) in a basic health unit (BHU). Method: A
“Yes or No” questionnaire was applied in 20 CHA
from the BHU Jardim Jacy/SP, addressing 3 paradigms about CVA: risk factors; signs/symptoms;
conduct. Results: The length of professional experience ranged from 1 to 10 years (average 5.6
years). The following risk factors related to the
CVA were recognized: hypertension (95%), sedentary nature (95%), diabetes (90%), diet (90%),
smoking (90%), alcohol or Illicit drugs (80%) and
dyslipidemia (75%). Other factors unrelated that
were mentioned as stress (90%); age over 40 years
(85%), headache (60%), traumatic brain injury
(54%) and bronchitis (5%). Among other signs
and symptoms mentioned were acknowledged:
language disorder (100%), paresthesia (95%),
facial paralysis (95%), sudden dizziness (75%),
motor deficit (95%) and visual disorder (70%).
Between the unrelated and not acknowledged as
associated: mental confusion (90%), chest pain
(65%), dyspnea (65%), vomiting (65%), swelling
of the lower limb (65%), dysuria (20%). Regarding the conduct, all affirmed that in the case of a
suspected CVA, it is mandatory to call the SAMU
(mobile emergency medical services) and 95%
would take to the closest emergency room. Other answers pointed out as positive were: take the
patient to the outpatient and medical care (40%),
give anticoagulant (30%), leave the patient to rest
(20%), give antihypertensive (20%), offer tea or
household remedy (5%). Conclusion: This CVA
study showed that CHA presented high level of
awareness regarding the main factors of risk,
signs and symptoms. But, there was a high rate
of misconceptions. In relation to the conduct, all
identified the emergency of the event, although
many showed inappropriate understanding.
Thus, this study stresses the need of a greater
qualification in the CHA so the CVA can be recognized properly.
P-65
ACUTE SUBDURAL HEMATOMA CAUSED BY
SPONTANEOUS INTRACRANIAL HYPOTENSION
Takatani M, Birman D, Mota RSS, Vieira KS, Sousa RAP,
Almeida CMO, Tenório ISAC, Sousa NAC, Galvão MLS
• HUGV - UFAM
Introduction: Spontaneous intracranial hypotension (SIH) is caused by spontaneous cerebral
spinal fluid (CSF) leakage of unknown etiology
at the level of the spine. In most cases, SIH patients have typical orthostatic headaches that
generally occur or worsen within 15 minutes of
assuming the upright position. The associated
symptoms are neck stiffness, tinnitus, hypacusia, photophobia, and nausea. The CSF pressure
is low (<60mmH2O or even negative). Magnetic
resonance imaging (MRI) findings are diffuse
pachymeningeal enhancement, subdural fluid
collections, engorgement of venous structures,
pituitary hyperemia, and sagging of the brain.
Subdural hematoma is often considered serious
complications of spontaneous intracranial hypotension. Objectives: Show a dangerous complication of spontaneous intracranial hypotension. Case report: A 41-year-old man presented
with a nontraumatic orthostatic headache. Brain
MRI with gadolinium contrast demonstrated
diffuse pachymeningeal enhancement, suggesting spontaneous intracranial hypotension (SIH).
General and neurologic examinations were normal. Conservative treatment of oral hydration
and bed rest were given initially. After oral hydration and medication, he was discharged without
a definite diagnosis. He noticed, however, that if
he maintained an upright position for more than
an hour, the headache returned. The symptoms
fluctuated thereafter. The posterior nuchal pain
was exacerbated 1 week before the patient visited
our outpatient department; even lying down did
not relieve the pain. Turbo spin echo T2-weighted MRI showed subdural fluid collection, a subdural hematoma of 0.7 cm was found in the right
hemisphere. He was not receiving anticoagulants. Laboratory results, including coagulation
profile, were normal. At a 2-month followup, MRI
documented complete resolution of the subdural collection. The patient was discharged without any neurologic deficit. Discussion: Chronic
subdural haematoma usually occurs after head
injury, and is more frequent in the elderly as
ensuing cerebral atrophy results in reduced tissue volume and hence greater movement of the
brain within the cranium1. Patients with both
spontaneous intracranial hypotension (SIH) and
subdural hematomas (SDH) are frequently undiagnosed. There is no consensus regarding the
management of SDH caused by SIH. SIH is often
considered benign and treated conservatively2,
but it can rarely manifest with serious complications requiring rapid surgical evaluation3.
P-66
ADHERENCE TO STROKE GUIDELINES ON AN
INTEGRATED CARE NETWORK: GET WITH THE
GUIDELINES STROKE PROGRAM IS THE BETTER
CHOICE?
Arec CV1, Silva DP2, Dutra LA1, Brasil IP1, Azevedo DS1,
Cirino CP1, Cunha JLM1, Albuquerque MVC1, Broner T1,
Flho RBC1, Pacheco EP1, Miranda VAD1, Gama MTD1,
Arruda MJC1, Furlan V2, Domingues RB3, Kuster GW1
• 1HOSPITAL PAULISTANO; 2HOSPITAL TOTALCOR;
3
CHRU, POLE NEUROLOGIE, UNIVERSITÉ LILLE 2
Introduction: The adherence of guidelines reduces mortality and dependence on Stroke.The
Get With The Guidelines (GWTG) is a Program
of American Heart Association to evaluate the
adherence with them. Objective: To evaluate the adherence to GWTG in the Hospitals of
PIAVEN (Integrate Program of stroke/ Amil-SP).
Methodology: The following is a list of six (6)
measures evaluated in patients diagnosed with
ischemic stroke in the Hospitals TotalCor and
Paulistano 1. IV rt-PA arrive by 2 hour, treat by
3 hour 2. Early antithrombotics: 3. VTE prophylaxis: 4. Antithrombotics: 5. Anticoagulants for
AFib/Aflutter: 6. LDL ≥100 or ND - Statin Results:
From January 2012 to June 2014 were evaluated
333 patients with ischemic stroke. Of the total of
25 patients that were eligible, 96% (24) received
IV thrombolytic therapy, considering those that
arrived within 2 hours of symptoms onset, 75%
were treated within 3 hours of symptoms onset.
Early antithrombotic were prescribed to 99% of
patients with indication, and the VTE prophylaxis to 97%. The patients diagnosed with AFib/
Aflutter 94% received anticoagulation therapy.
Antithrombotic at discharge were prescribed to
61
Posters
97% of eligible patients and 94% received statin
medication when LDL ≥100 or ND. Conclusion:
Our adherence is comparable to the hospitals included in the GWTG, getting just below the windows of anticoagulation for AFib/Aflutter and
antithrombotic at discharge. Despite achieving
goals of an American program, we believe that is
necessary a national quality stroke program with
goals compatible with the profile of the Brazilian
population.
P-67
ADULT POPULATION KNOWLEDGE ABOUT STROKE
IN MARINGÁ, BRAZIL
Rossini RC, Emerich SS, Marcelino PCO, Almeida DF,
Lenardon L, Kitayama M, Silva MP, Petta BFV,
Ferezini J, Rombaldo WL, Orathes BM
• UEM
Introduction: Stroke is a major cause of death
worldwide. Despite treatment advances, the
number of patients who have access to thrombolysis in the 4.5 h window is very limited. Population unawareness about stroke symptoms
has been an important limitation to improve
successfull thrombolysis rate. This study evaluated the population knowledge about stroke clinical manifestations, risk factors and treatment.
Methods: This was a prospective, single center
study carried out between December 2013 and
July 2014. The authors have interviewed 176 people in Public Institutions of Maringá, Brazil about
their knowledge in stroke. Results: From the
studied sample, 57,9% were males, 47,1% aged 40
to 59, 25,6% aged 18 to 39 and 27,3% aged 60 or
older. Regarding school background, 41,5% of the
sample had more than 11 years of study, and only
1.1% were illiterate. Up to 80% of the people have
already listened about stroke. Speech difficulty
(85,8%), arm or leg weakness (85,2%), unilateral
paresthesia (82,4%) and asymetric smile (78,4%)
were the most frequent reported symptoms.
Chest pain (48,3%), shortness of breath (50%),
palpitations (44,9%), faintness (46,0%), and unsteadiness (65,9%) were reported as well. The
main identified risk fator was high blood pressure (90%), followed by high cholesterol levels
(81,3%), tobacco abuse (77,3%), atrial fibrillation
(59,6%), and diabetes (55,1%). Over half of the
respondents had a relative or friend victimed by
a stroke. Only 58% of the interviewed population
would call the prehospital emergency service
(SAMU) to help a patient with stroke, although
only 37,5% know its correct phone number. More
than 30% of the respondents stated that in case
of a possible stroke in a family member or a coworker would take the victim directly to a nearby
hospital. Only 45,5% recognize the neurologist
as the stroke specialist, while 40,3% said that the
cardiologist was the most appropriate physician
to treat a stroke. Conclusion: This study was carried out in a high educational level population
with reasonable knowledge about stroke symptoms. However, some misconceptions still exist.
Of major concern is the high percentage of people that do not know the SAMU phone number
and would take a stroke patient directly to the
hospital.
62
P-69
AN ADULT ATYPICAL PRESENTATION OF PHACES
SYNDROME
Oliveira LM, Barcelos IP, Tinone G, Yamamoto F,
Santos GT, Conforto AB
• USP
Introduction: PHACES acronym refers to the
association of posterior fossa malformations,
hemangiomas, arterial cerebrovascular abnormalities, cardiac and aorta defects, eye abnormalities and sternal defects. It is rare that all
abnormalities are present in one patient. Predominant involvement of the arterial system distinguishes PHACES from other neurocutaneus
syndromes. Objective: To describe an unusual
case of PHACES syndrome in a 23-year-old man
with reticular facial hemangioma that presented
with a large right middle cerebral artery (MCA)
infarct. Case report: A 23-year-old man reported an episode of transient left facial paresis and
mild dysarthria, lasting for one hour, 20 days
prior to admission. Headache, vomiting and
fever developed for the next 6 days. After being
empirically treated with amoxycillin for 5 days,
he was submitted to a lumbar puncture that revealed mild neutrophlic pleocytosis. A cephalosporin was then given for the next 5 days. After
ten days, the patient preented mild paresis and
paresthesias in the left hand. On the day of admission to our hospital, he presented sudden left
hemiplegia. He had a facial reticular hemangioma involving the right frontal region. He had left
hemiplegia, hemianopia and neglect. Computed
tomography (CT) showed a large right MCA cerebral infarct, besides a right aracnoid temporal
cyst. Arterial neuroimaging studies revealed a
fusiform aneurismal dilatation in the supraclinoid portion of the right internal carotid artery
(ICA) with thrombosis of its distal portion, left
ICA kinking, anterior cerebral artery duplication
and fenestration of the basilar artery. Discussion:
Twenty-two individuals with PHACES and arterial ischemic stroke have been described in the literature. Arterial abnormalities occurring in this
syndrome can be categorized in four subgroups:
dysplasia, narrowing, aberrant course or origin
and persistence of embryonic anastomoses. Arterial abnormalities typically involve large and
medium-sized vessels and occur either ipsilateral to the facial hemangioma or bilaterally. Phenotypic heterogeneity accounts for underdiagnosis
of this syndrome.
P-70
ANALYSIS OF 21 CASES OF CEREBRAL VENOUS
THROMBOSIS FROM THE NEUROLOGY SERVICE OF
HC/UFPR
Parolin LF, Zetola VF, Chamma JF, Lange MC, Novak EM
• UFPR
Introduction: Cerebral venous thrombosis (CVT)
represents about 0,5% of all strokes and can
be challenging to diagnose because headache,
rather than focal neurologic symptoms, is the
proeminent feature. The major risk factors are
prothrombotic conditions, oral contraceptives,
pregnancy and the puerperium, malignancy and
infection. The onset of symptoms is classified as
acute, subacute or chronic according to the time
of clinical evolution and the majority of the cases have a delay diagnosis. Usually the prognosis
is favorable, but it may occur some permanent
disability or death. Objective: descriptive analysis of 21 cases of venous thrombosis with greater than 12 months follow-up. Methodology: We
retrospectively analyzed 23 cases of CVT who
were treated between 2002 to 2013, followed by
at least one year after diagnosis. All cases were
diagnosed by conventional angiography or magnetic resonance imaging (MRI). We excluded two
cases because lack of data. Results: Of total 21
cases, 81% (17) were female and 19% (4) were
male. The major risk factors we found were oral
contraceptives 38% (8), followed by infections
19% (4) and smoking 14% (3). No examination
was performed in the acute phase, ie in the first
24 hours of symptom onset. The majorit of the
cases 61% (13) were classified as subacute and
100% cases had headache as a main symptom,
followed by 57% (12) nausea and vomiting, 23%
(5) presented papiledema, 23% (5) complain visual problems, 19% (4) seizures, 19% (4) present
cranial nerve involvement, 14% (3) focal neurologic deficits, 14% (3) dizziness/imbalance
and 9% (2) mental disorder state. In 61,9% (13)
thrombosis envolved transverse sinus , sigmoid
sinus in 52% (11), 33% (7) superior sagittal sinus,
straight sinus, longitudinal sinus and internal
jugular vein in 9% (2) each and just 4% (1) present thrombosis of cavernous sinus. We had no
mortality or treatment-related complications, although we have permanent disability as 19% (4)
of depression/ anxiety, 14% (3) of seizures, 9% (2)
of chronic headache and 4,7% (1) of visual loss.
Only one patient 4,7% had CVT recurrence but 01
(4,7%) had pulmonary embolism and 01 (4,7%)
deep vein thrombosis. Conclusion: CVT is an
important diagnosis to keep in mind when evaluating patients with headache in the emergency
department. It is important not be overlooked
because it is a treatable condition and can avoid
morbidity and mortality.
P-74
ANEURYSMAL SUBARACHNOID HEMORRHAGE
ASSOCIATED WITH POLYCYSTIC LIVER DISEASE:
CASE REPORT
Spitz CN, Cardoso FM, Drumond MT, Rodrigues BCB,
Vaz G, Balthazar H, Freitas GR
• INSTITUTO D‘OR DE PESQUISA E ENSINO
Introduction: The polycystic liver disease (PLD)
in isolation is an autosomal dominant disorder
characterized by diffuse cystic lesion in the liver, without concomitant renal involvement. It
is a rare condition (incidence <0.01%), in which
there are increased risk of aneurysm, rupture
and dissection of the intracranianial vessels.
Aneurysmal subarachnoid hemorrhage (SAH)
is an important cause of morbity and mortality in young adults worldwide. Special attention
should be given to patients with PLD, once the
risk of cerebral aneurysm is greater than general
population. Objective and Methods: Report the
association between SAH and isolated polycystic
liver disease. Results and Discussion: female, 62
years, previous hypertension, presented sudden
headache associated with emesis. Computed
tomography scan of the brain demonstrated
SAH, with blood in the cisterns at the base and
right frontal intraparenquimatous hematoma.
Cerebral angiography showed ruptured aneurysm in right middle cerebral artery (MCA) and
unruptured aneurysm in left ophtalmic artery.
The patient was submitted to endovascular treatment of right aneurysm MCA. During hospitalization she underwent to abdominal computed
tomography scan for infection screening and
multiple polycystic images in a normal liver, the
biggest one with a diameter of 5.5cm. Although
the relation between SAH and polycystic kidney
disease, combined or not with PLD, is already
established, the detection isolated of SAH and
XXVI Congresso Brasileiro de Neurologia
PLD is rare. Conclusion: The combination SAH
and PLD is not well documented in the literature
due to its rarity. This fact can be justified by a lack
of symptoms and insidious manifestation of the
disease in gene carriers affected. Thus there is
not an useful method to exclude family members
at risk of develop cerebral aneurysm; wich makes
the report of cases of fundamental importance
for a better understanding of this association.
P-75
ANEURYSMAL SUBARACHNOID HEMORRHAGE
IN A PATIENT WITH SICKLE CELL ANEMIA: CASE
REPORT
Echegaray MVF1, Passos Neto CEB1, Fukuda TG1,
Bastos AJA2, Oliveira Filho J1, Jesus PAP1
• 1UFBA; 2HSI
Introduction: Patients with sickle cell anemia
have a higher incidence of cerebrovascular
events than the general population, and various pathophysiological mechanisms may be
involved in its occurrence. However, in the English language literature there are only 51 cases
of intracranial aneurysms in sickle cell patients
reported. Objective: To report a patient with
subarachnoid hemorrhage (SAH) aneurysm associated with sickle cell anemia and discuss its
pathogenesis. Case report: A 18 years old man,
previously diagnosed with sickle cell anemia,
presented with sudden headache and neck stiffness that evolved to opisthotonus. Tomography
scan without contrast revealed small amount of
blood in subarachnoid cisterns. A lumbar puncture was performed to collect cerebrospinal fluid, with return of hematic content. Arteriography
showed occlusion of both internal carotid arteries (ICA) associated with intensive collateral circulation and vascular proliferation (compatible
with Moyamoya); a saccular (berry) aneurysm
was observed in the left posterior inferior cerebellar artery, hence a endovascular embolization
was performed without complications. During
the hospitalization period, measures for aneurysmal SAH were maintained, in addition to
blood transfusion, hydration and analgesia. The
patient remained under surveillance for 14 days
and was discharged asymptomatic. Discussion:
The most frequent site of aneurysms in sickle cell
patients is ACI. The most accepted mechanism
for the formation of aneurysms of the anterior
circulation states that the chronic endothelial
damage leads to weakening of the vessel wall.
In patients with secondary Moyamoya pattern,
posterior circulation aneurysms can occur due
to a compensatory increased flow from the posterior circulation, leading to the appearance of
aneurysms and pseudoaneurysms in blood vessels with already weakened endothelium due to
an underlying disease. Conclusion: Aneurysmal
SAH in sickle cell disease patients, although rare,
should not be neglected because it has effective
treatment - as in the Case reported. Non-recognition or misdiagnosis (with acute sickle cell crisis)
may lead the patient to serious complications.
P-76
APOPLEXY IN PATIENT WITH NON FUNCTIONING
PITUITARY ADENOMA AFTER USE GNRH AGONIST
AS TREATMENT FOR PROSTATE CANCER
Duarte RL, Sousa MWG, Tauil CB, Sousa ACJ,
Maria PRS, Guiotti MG, Ribeiro MIB
• HOSPITAL DE BASE DO DISTRITO FEDERAL
Introduction: Androgen deprivation therapy
has shown excellent results with respect to mortality and morbidity in patients with advanced
prostate cancer. Leuprolide acetate, a synthetic
GnRH agonist commonly used for this indication, has consistent effects on androgen profile.
The increased preference for GnRH agonist therapy has revealed a rare adverse drug reaction. We
report a case of pituitary apoplexy following the
administration of Leuprolide. Objective: Present
a case of pituitary apoplexy after antiandrogen
therapy in patients with non functioning pituitary adenoma, demonstrating the importance
of screening with imaging in this population.
Case report: Man 69 years, hypertension, type
2 diabetes and prostate cancer (acinar adenocarcinoma score 6 (3 +3) Gleason) with reported
holocranial headache associated with nausea,
vomiting, diplopia and ptosis right four hours
after administration Leuprolide intramuscularly.
After five days of the event, was admitted to the
neurology unit observing full involvement of the
third cranial nerve on the right, without other
neurological signs. Initiated research with imaging being detected in conventional brain MRI a
nodular lesion in the right pituitary, measuring
about 18x16x18mm, with heterogeneous signal intensity on T2 and peripheral underling of
contrast with presence of subacute hemorrhage;
findings consistent with pituitary adenoma with
signs of apoplexy. Pituitary endocrine profile
showed decreased levels of cortisol and TSH unchanged testosterone, FSH, LH and prolactin.
We started the administration of hydrocortisone, followed by oral prednisone and levothyroxine. Patient developed an significant clinical
improvement and regression of right ptosis with
extraocular motility preserved. Discussion: The
acute presentation of this pituitary apoplexy
characterized by sudden and intense headache,
nausea, vomiting, ophthalmoplegia, and, in the
worst cases, altered consciousness. A literature
review revealed twelve previously reported cases
of pituitary apoplexy after GnRH agonist therapy
for prostate cancer. In this article we report a case
of apoplexy in non functioning pituitary adenoma after administration of GnRH agonists. The
pathophysiology of GnRH agonist-mediated pituitary apoplexy is unknown. However, multiple
factors that increase the risk or are directly associated with the cause of the bleeding have been
implicated (the size of the adenoma; Intrasellar
pressure and intrinsic pituitary vasculature.
P-78
ASSOCIATED RARE CAUSES OF STROKE IN YOUNG
ADULTS
Pereira MTR, Andrade ACA, Silva LD, Torres IPSS,
Andrade HMT, Belini LE
• PUCCAMP
Introduction: Stroke is rare in young adults, only
5–10% of acute cerebrovascular events occur in
younger than 45 years of age. By the TOAST classification, ischemic strokes are classified into
subtypes: large-artery atherosclerosis, cardioembolism, small-vessel occlusion, cryptogenic
(other determined) and undetermined etiology.
Cryptogenic stroke accounts for 30‐40% of these
subtypes, including patients with rare causes
of stroke, such as: paradoxical embolism due to
patent foramen ovale (PFO), anatomical variations and others. Goals: To relate the etiology of
stroke in young adults due to the coexistence of
PFO and artery of Percheron (AOP). Case report:
29-year-old man, previously healthy, presented
complaining of drowsiness, blurred vision, sudden impairment of memory and naming. Neurological examination: altered mental status,
aphasia; mini-mental state examination: 20/30,
unsatisfactory performance on: orientation,
recall memory, language and naming; left quadrantanopsia. The remainder of the examination was normal. Magnetic Resonance Imaging:
restricted diffusion in the bilateral paramedian
thalami and in the right occipital lobe, without
postcontrast enhancement. Transesophageal
echocardiography: PFO with shunt right-left,
visualized by infused with microbubbles during
Valsalva maneuvers. Laboratory tests: normal.
Initial screening for coagulopathies: hyperhomocysteinemia. Treatment with anticoagulation
was started. After that, the patient lost the follow
up. Discussion: Bilateral paramedian thalamic
infarcts can be caused by an uncommon anatomic variant of the paramedian thalamo-perforating arteries, called artery of Percheron, in
which a single dominant artery supplies the
bilateral medial thalami with variable contribution to the rostral midbrain. This type of infarct is
typically characterized by aphasia, mental status
and memory impairment. The AOP prevalence is
unknown and is rarely visualized with conventional angiography, the diagnosis is presumptive.
The incidence of PFO is 25% and is often related
to stroke in young adults. The stroke risk related
to the PFO was 7% per patient year. It is important to identify if the stroke is related with PFO or
if it is an incidental finding to guide treatment.
We described a rare case of the coexistence of the
AOP, PFO and hyperhomocysteinemia, resulting
in bilateral thalamic infarcts in a young adult. We
did not find similar reports in the literature.
P-79
ASSOCIATION BETWEEN BODY WEIGHT AND
CLOPIDOGREL RESISTANCE IN NEUROLOGICAL
PATIENTS
Lima PCTM1, Farias CA1, Hoppe J1, Magalhães VDS1,
Silva QM1, Maldonado IL2
• 1FTC; 2UFBA
Background and purpose: Clopidogrel is widely
used in patients intended for deployment of endovascular material (e.g. stents). However, few
studies have evaluated the prevalence of drug
resistance and the predictors of this condition
in neurological patients. In the present study,
we evaluated the body weight as a predictor of
clopidogrel resistance. Materials and methods:
This retrospective transversal study evaluated
platelet function in 182 neurological patients.
They were divided into two groups according to
body weight. Every participant received a 300mg
loading dose of clopidogrel. The VerifyNow
point-of-care assay was used to measure the percentage of platelet inhibition. Results: The group
in which the body weight was higher than 60Kg
presented with lower percentages of inhibition
(27.94±31.8%) than the group with lower body
weight (41.75±35.5%). Applying the traditional
threshold for clopidogrel resistance (platelet inhibition <40%), the prevalence of drug resistance
was observed to be 72% in the group with high
body weight versus 53% in the group with lower
body weight. Conclusion: Clopidogrel resistance
63
Posters
is a common event in cardiological and neurological patients. According to the results of the
present study, patients with a higher body weight
(more than 60kg) respond with less intensity to a
300 mg loading dose of clopidogrel and are more
frequently classified as drug-resistant.
P-80
ASSOCIATION BETWEEN INSTRUMENTAL
ACTIVITIES PRIOR AND FUNCTIONAL
PERFORMANCE AFTER STROKE
Monteiro MDC1, Pinto EB1, Maso I1, Oliveira Filho J2
• 1BAHIAN SCHOOL OF MEDICINE AND PUBLIC
HEALTH, 2STROKE CLINIC OF THE FEDERAL
UNIVERSITY OF BAHIA, BRAZIL; 3STROKE UNIT OF
THE ROBERTO SANTOS GENERAL HOSPITAL
Background: The activities performed by the
individual prior the stroke are cited as factors
that may interfere in the functionality after morbidity. According to some authors, a prior active
lifestyle can generate a protective effect after
stroke. Objective: To investigate the association
between instrumental activities prior and functional performance after stroke. Methods:This
was a prospective study realized in patients admitted at a public hospital, diagnosed with first
stroke and independent functional prior. Participants were analyzed in the period of admission,
which was applied a clinical and sociodemographic questionnaire, the Frenchay Activity Index (FAI), the National Institutes of Health Stroke
Scale (NIHSS) and Modified Barthel Index (MBI).
After 6 months was applied, by phone, MBI and
EuroQol‐5 Dimensions (EQ‐5D) for assessment
of functional capacity and quality of life respectively. After univariate analysis (Student‘s t test,
Mann Whitney, Chi-square and Fisher‘s exact
test) and considering the data in the literature,
variables were included in a multivariate logistic
regression to assess predictors of functional dependency (MBI<46) and impaired quality of life
(EQ‐5D <0.78) after 6 months. The score of the FAI
was included in two models of multivariate analysis, the first evaluating the FAI as a continuous
variable and the second considers the FAI with
a cutoff <18. Results: We analyzed 141 patients
with a mean age of 56.2±17.1 years, and 61.7%
were female. It was found that patients with
FAI<18, compared with patients with FAI≥18,
were older (p=0.039), had a lower percentage of
females (p=0.001), more severe stroke (p=0.022)
and after 6 months were more dependent, both
for basic ADLs assessed by MBI (p<0.001), and for
IADL, assessed by FAI (p<0.001). It were selected
variables: age, gender, stroke severity (NIHSS),
functional capacity of hospital admission (MBI)
and FAI as predictors of functional capacity and
quality of life after 6 months. When analyzed
in the logistic regression to predictors of functional dependency, in the model 1 only the FAI
(p=0.013)was predictiveand in the model 2, the
FAI (p=0.041)and the MBI admission(p<0.001)
remained significant. To predictor of quality of
life only gender and stroke severity (NIHSS) remained significant, in both models. Conclusion:
This study demonstrated that highest frequencies of instrumental activities prior are associated with the functional ability after stroke.
64
P-81
ATYPICAL NUROIMAGE FINDING IN POSTERIOR
REVERSIBLE ENCEFALOPATHY SYNDROME (PRES)
Rocha FA1, Maia FM1, Rocha TT2, Pitombeira MS1,
Travassos FT1, Rangel DM1, Ferreira GM1, Marinho ART1
• 1HGF; 2UNICHRISTUS
Introdutcion: PRES was first described as leukoencephalopathy, presenting only white matter lesions. In 2000, received the actual denomination
since the cortical layer was also affected. Mental
confusion, headache, blurred vision and seizures
are between the most common manifestations.
It is a condition most commonly reported in
cases of hypertensive encephalopathy. The prevalence is uncertain and there is no gender difference. Objectives: Report a case of PRES with an
atypical finding in a MRI. Case: A 39-Year-Old
man was admitted to Emergency Department
presenting tonic-clonic seizures and deterioration of consciousness. On admission, he was
tachycardic (HR 112bpm), BP 190x120mmHg
and somnolent. He had more than 5 episodes of
tonic-clonic seizures in the last 3 hours. He was
using haloperidol due to a psychotic episode of
an irregular treated schizophrenia. Initial laboratory findings demonstrated an acute renal
injury (Urea 352mg/dL; Creatinine 13,6mg/dL).
Computed tomography examination revealed
unspecific hypodense areas in high convexity.
Phenytoin was administrated and emergency dialysis was programmed. Although seizures free,
next day he still was somnolent. MRI showed
diffuse and predominantly subcortical hyperintense lesions compatible with PRES and a lobar
focal right temporal hematoma. After lowering
BP and correction of renal function, he recovered a normal consciousness status. Discussion:
PRES physiopathology is uncertain. Currently
the most acceptable hypothesis is a loss of autoregulatory mechanisms in cerebral vessels. Some
precipitants are encountered such as: high blood
pressure, immunosuppressive therapies, pre-eclampsia and AKI. A classical description in MRI
is a white matters lesions, occipitoparietal predominance. Hemorrhagic lesions, are uncommon, about 15% of patients according to some
series which are petechial in most cases. A low
percentage (<3%) presents with lobar hematoma
as showed in this case. The treatment is based in
clinical support and leading with complications
like seizures, infections and renal problems. As
in our case, most patients has a good prognosis
with few chronic neurologic disturbances.
P-82
BASILAR DOLICHOECTASIA: MACROSCOPIC
PHENOMENON OF DILATATIVE ARTERIOPATHY
Novaes MRR, Assis BDRR, Fernandes TD, Lima FD,
Braga GP, Bazan R
• UNESP
Introduction: Dilatative arteriopathy is a dilatation and stretching of thick gauge intracranial
arteries. Dolichoectasia is defined by a diameter larger than 4.5mm and lenght larger than
23,5mm. It is found in approximately 12% of
cases of stroke and is more prevalent in men,
whites and older, usually associated with hypertension, myocardial infarction and lacunar
infarcts without clinical evidence of atherosclerotic carotid disease. The pathophysiology is
still not fully understood and the treatment is a
challenge. Objectives: Clinical descriptive study
of a patient admitted to the Neurology Service
of the Botucatu Medical School, São Paulo State
University (FMB-UNESP). Case report: Man, 68
years, white, driver, admitted in the emergency
room of our service, with a history of sudden left
braquiocrural weakness that remitted before the
clinical assessment (lasting approximately 1.5
hour). He had remarkable past of atrial fibrillation, hypertension, dyslipidemia, heart failure,
benign prostatic hyperplasia. Physical examination on admission: Hypertension, irregular heart
rhythm, alert, disoriented in time and space,
without focal neurological deficits. NIHSS =02
(related to the orientation answers). Brain CT:
parietal calcifications of vessels of the anterior
and posterior circulation. Saccular basilar artery
dilatation (14 x 14 x 25 mm). Moderate microangiopathy. Discussion: The pathophysiology was
initially related to vascular changes secondary
to dyslipidemia and hypertension. The most
recently hypotheses are based on changes in
protein regulation of inflammatory activity with
degradation of collagen in the wall vessels middle layer, extravasation of metalloproteinases
to the white matter, demyelination and gliosis
(leukoariosis). Thus, not only the dilatation and
calcification of large intracranial vessels, but
mostly small vessel disease, is implicated in the
worse outcome of this condition. It is currently
considered an independent risk factor for stroke.
The arterial dilatation is an epiphenomenon of
systemic disease and treatment should be directed to its basic pathophysiology.
P-83
BATTLE OF THE SEXES ON STROKE RISK FACTORS
Pereira L, Rodrigues M
• SERVIÇO DE NEUROLOGIA DO HOSPITAL GARCIA DE
ORTA, ALMADA, PORTUGAL
Introduction: Epidemiological data on stroke
risk factors has grown in the last decade. Significant differences have been found between genders, but often not reproducible. Some may be
geographically linked, justifying studies in different populations. Objectives: Identify gender differences in a Portuguese population, concerning
stroke risk factors, presentation, severity, acute
phase treatment and disability on discharge.
Method: Retrospective observational study on
consecutive ischemic stroke patients admitted
to the Neurology ward of a Portuguese central
hospital in 2 years. Data on demographics, stroke
risk factors, symptoms, treatments and modified
Rankin scale (mRS) were collected. We present
descriptive and inferential statistics with chisquare, Mann-Whitney test and binary logistic
regression. Results: We included 462 patients
(64.5% men, median age 66). Women were 6
years older than men (median 70 vs 64, p<0.001).
Risk factor rates were hypertension 74.5%, dyslipidemia 62.3%, smoking 53.2%, alcohol intake
41.6%, diabetes 32.0% and atrial fibrillation (AF)
22.5%. Smoking (68.6% vs 20.3%, p<0.001) and
alcohol intake (68.5% vs 16.3%, p<0.001) were
more frequent in men, and AF (31.1% vs 17.8%,
p=0.01) more prevalent in women. Women presented with more severe stroke (median NIHSS
10 vs 5, p=0.003) and sought medical care earlier
albeit not significantly (mean 15.9 vs 19.5 hours,
p=0.25). Thrombolysis rates were similar (25.6
vs 18.1%, p=0.13). Women were more often on
anticoagulation prior to stroke (12.2% vs 5.0%,
p=0.02), but there were no significant differences
on its use among AF patients (women 47.1% vs
men 37.5%, p=0.43). On hospital discharge women were more disabled (mRS>2 43.5% vs 29.0%,
p=0.002) in univariate analysis, but it became
non-significant after adjusting for AF prevalence
or worse neurological deficit. There were no
XXVI Congresso Brasileiro de Neurologia
significant differences between genders on mortality. Conclusion: In agreement with previous
studies we found higher age and AF prevalence
in women. Contrary to past reports our group of
women sought hospital care no later than men
and there was no discrimination for the use of
thombolytic treatment. Female gender was a
predictor of disability, mediated by AF and worse
deficit at discharge. This wasn’t due to lower anticoagulation rates in women. It is of the uttermost
importance to recognise AF as more prevalent in
women, to achieve better secondary prevention
of stroke and to lower burden of stroke in this
gender.
P-84
BRAINSTEM INVOLVEMENT IN BEHÇET‘S DISEASE:
A CASE REPORT
Pereira VC, Assis BDRR, Braga GP, Bazan R
• FACULDADE DE MEDICINA DE BOTUCATU - UNESP
Introduction: Behçet’s disease was described by
a dermatologist named Hulushi Behçet, in 1937,
characterized by the triad of oral ulcers, uveitis
and genital ulcers. It’s been considered an inflammatory and multisystemic disturb, with
pathophysiology marked by vasculitic process.
Neurological involvement occurs in 4‐29%, with
variable clinical manifestations, often evolving
with relapsing and remmiting course. Objective:
Describe a clinical case of this relatively rare entity; Case report: M.A.S., man, 52 years old, hypertense and dyslipidemic, with previous Behçet’s
manifestations of uveitis in the right eye and
stroke since 2 months (right hemiparesy, dysarthria and urinary incontinence). He was evaluated at the neurology’s emergency room for abrupt
neurological deficit since 4 days, presented with
rotatory vertigo, motor incoordination and worsen of the dysarthria. The physical and neurological assessment showed oral ulcers, moderate
dysarthria, disproportionate right hemiparesy
(sequellar) and VI, VII, IX, X and XII left cranial nerves palsy. VHS:26, PCR: 2,8. Brain MRI
showed, in FLAIR sequence, extensive hyperintensity signal in brainstem (medulla, pons and
midbrain) and left thalamus. Cerebral angiography suggested vasculitis. CSF showed protein
level of 66mg%. Proceeded with Methylprednisolone pulse therapy for 3 days and imunossupression with cyclophosphamide. The response was
satisfactory, with good evolution in 6 months,
with remaining sequella of mild dysarthria,
disproportionate right hemiparesy, left peripheral facial palsy and left VI cranial nerve palsy .
Discussion: The neurological involvement in Behçet’s disease was firstly described in 1941, with
the brainstem and basal ganglia being the most
commonly affected sites. The described clinical
case evidentiated extensive CNS’s commitment
in neuroimaging associated with active disease
and mild neurological manifestations. The early diagnostic and aggressive activity’s control of
the disease are cardinal to assure the patient an
appropriate quality of life and preventing new
outbreaks.
P-85
CADASIL: CASE IN TWO FAMILIES
Pires KL, Silveira RC, Nohra T, Silveira JOF, Freitas MRG
• UFF
Introduction: Cadasil (cerebral autosomal
angiopathy with subcortical infarct and leukoencephalopathy) is hereditary arteriopathy
resulting mutation on chromosome 19, NOTCH
3 gene, affecting individuals between 40 and 50
years. Purpose: Report two families with Cadasil, one of these with genetic confirmation containing a known mutation. Case report: Family 1:
FCM, 34, simple partial epileptic syndrome with
secondary generalization, RMC suggestive of Cadasil, family history of two episodes of ischemic
stroke on her mother at age 61, migraine and
an episode of ischemic stroke in young brother.
The patient underwent a DNA test showing mutation in the NOTCH 3 gene. Family 2: RCC, 42
years, two episodes of sudden paresis of the left
arm and leg, migraine and memory deficit, RMC
compatible with Cadasil and family history of
ischemic stroke in young sister. Discussion: Cadasil was best known as genetic mapping in the
late twentieth century. Patients may present with
episodes of stroke, migraine and cognitive deficits caused by arterial disease caused by a mutation in the NOTCH 3 gene. Episode seizure before
the other symptoms is rare but can occur. Our
study has limitations because one of the families
can not perform confirmatory genetic test because this test isn‘t being easily available in our
country. Cadasil is a rare disease should be suspected in patients with clinical history and brain
MRI compatible. This disease has no cure, but
its symptoms can be alleviated by improving the
quality of life of patients. Because it is a genetic
disease it is important genealogical assessment.
P-86
CAN THE MOTOR COORDINATION OF LOWER
LIMB INFLUENCES THE MOBILITY AND GAIT OF
HEMIPARETIC?
Carvalho AC, Bofi TC, Barbatto LM, Silva FA, Rossi FE,
Oliveira MCS
• UNESP - PRESIDENTE PRUDENTE
Introduction: The hemiparesis is one of the
main signs after brain stroke and consists in deficit of motion, muscle weakness and tonus and
motor coordination changes which are essential
in the performance of tasks and everyday functional activities. Objective: This study aimed to
identify whether there is a correlation between
the motor coordination of lower limbs and gait
in hemiparetic. Method: In these study 18 hemiparetic goers of Extension-program project of
FCT/UNESP called “Hemiplegia project” which
uses the group therapy as a therapeutic resource
was involved. This research is filed in the FCT/
UNESP Ethics Committee in Research (CAAE:
34601014.4.0000.5402). The functional motor assessment was performed with: A) Lower Extremity Motor Coordination Test (LEMOCOT), which
quantitatively evaluates the motor coordination
of the paretic lower limb by counting hits of the
tiptoe on two targets a platform placed under
the patient‘s feet; B) Time up & go (TUG) evaluates the functional mobility and gait recording
the time taken for the patient rise from a chair,
walk 3 meters, return and sit; and C) Ten-meter
Walk Test (TMWT) evaluates the gait recording the time taken to walk 10 meters at usual
speed. The correlation between the mean values
obtained was analyzed by the Spearman correlation test. Results: The evaluation revealed a
mean and standard deviation of 21.27±9.32 hits
in LEMOCOT, 17.60±7.8 seconds on the TUG
and 0.68±0.26 m/s in TMWT. The Spearman correlation test showed a weak negative correlation
between LEMOCOT and TUG (R=‐0.27, P=0.28)
and a significant correlation between LEMOCOT
and TMWT (R=0.50, P=0.03). The analysis of the
results allows considering that the motor coordination of the paretic lower limb influenced
gait speed in TWMT, the same did not occur in
TUG. For lift and sit in the chair other skill as the
strength of the large muscle groups of the lower
limb and the coordination of trunk and upper
limbs are required. When running LEMOCOT
there is no requirement of great muscular effort, but coordination of the muscles below the
knee, which positively influences walking speed.
Conclusion: The results demonstrate that the
motor coordination of the lower limbs is correlated with the gait from the standing position,
with no correlation with the gait from the sitting
position.
P-87
CAROTID DOPPLER STUDY AND ANTERIOR
CIRCULATION ISCHEMIC STROKE
Maurya PK, Kulshreshtha D, Singh AK, Thacker AK
• DR.RMLIMS
Objective: To study the carotid doppler findings
in patients with anterior circulation ischemic
stroke. Methods: A retrospective analysis of carotid doppler findings in anterior circulation
ischemic stroke patients admitted at Dr.Ram
Manohar Lohia Institute of Medical Sciences,
Lucknow from June 2013 to July 2014 was performed. Electronic data and case record of all
clinically and neuroimaging confirmed patients
with ischemic stroke were analysed. The analysis included demography, clinical features and
risk factors. Patients with posterior circulation
stroke and rheumatic heart disease were excluded. Stroke was classified using Oxfordshire
community stroke project classification. Extracranial atherosclerosis was assessed by carotid
ultrasound and carotid intima media thickness
(CIMT), plaque presence or absence and stenosis on ipsilateral as well as contralateral side to
stroke were noted. The doppler findings were
classified as normal CIMT, Increased CIMT with
plaque and increased CIMT with stenosis of either common carotid artery (CCA) or internal carotid artery (ICA). Results: The study included 55
patients [32 (58.2%) males, 23 (41.8%) females,
mean age of 63.4 years]. Five (9.1%) patients had
total anterior circulation infarction, 34 (61.8%)
had partial anterior circulation infarction and 16
(29.15%) had lacunar infarction. Risk factor observed for atherosclerosis were hypertension in
39(70.9%), diabetes mellitus in 18 (32.7%), dyslipidaemia in 40 (72.7%), smoking in 7(12.7%),
alcoholism in 5(9.5%). Twelve patients (21.8%)
had past history of stroke. Fifty (90.9%) patients
showed abnormal doppler findings with increased CIMT in 41(74.5%).Twenty five (43.6%)
patients had increased CIMT with plaque (calcified or non-calcified) in Common Carotid Artery. Distal CCA or ICA narrowing was noted in
16/55 (29.09%) with severe narrowing >70% in 9
(16.4%) patients, while 12 (21.8%) had narrowing
ipsilateral to stroke. Presence of diabetes mellitus was significantly associated with CCA or ICA
narrowing (p=0.03). Conclusions: This study
implies a high incidence of extracranial atherosclerosis in patients with anterior circulation
ischemic stroke. The abnormalities were more
marked in diabetics. The role of carotid doppler
study as a non invasive tool in the identification
of extracranial atherosclerosis is emphasised.
65
Posters
P-88
CASE REPORT : OPHTHALMOLOGIC SYMPTOMS
IN PATIENT WITH BILATERAL SEVERE CAROTID
ARTERY STENOSIS
Flores WL, Cippiciani TM, Silva ER, Puglia Junior P,
Conforto AB
• FMUSP
Case report: Ophthalmologic symptoms in patient with bilateral severe carotid artery stenosis Male patient , 58 years old, clinical history of
smoking and untreated hypertension presents to
the emergency department at the Hospital das
Clinicas, Faculty of Medicine, University of São
Paulo. With visual loss in the past two weeks. Ocular disorder were reported as sparkes or twinles
in the right eye and the left eye, during seconds ,
frequently in bright environments. He also presented two events of sudden visual loss in both
eyes, in less than two minutes; the visual field
suggested phenomenon of loss of peripheral vision , “ saw through a glass globe”. He reported
two episodes of reversible motor deficit in the
right arm, and after a few days, in the left arm.
Cardiovascular examination revealed 75 hbpm,
elevated blood pressure (SBP 240 DBP 130) .
Cervical examination highlighted the presence
of bilateral carotid bruit . Peripheral pulses were
present . There weren’t abnormalities in the
neurological and ophthalmological examination. The patient underwent further diagnostic
investigation through study of MRI scans that
identified foci of hyperintense on diffusion in the
white matter of the right precentral gyrus and
left parietal lobe superior, consistent with recent
lacunar ischemic lesions. In the evaluation of
intracranial and cervical vessels were detected:
critical stenosis (estimated at about 90%) of the
left carotid bulb and critical stenosis (estimated
at about 95%) of the right carotid bulb . Given
the following diagnoses : acute focal cerebral
syndrome , retinal ischemic syndrome and bilateral carotid stenosis critical , the patient underwent endovascular treatment. Angioplasty with
stenting of the left carotid artery was performed
initially and after 8 days ; held the right carotid
angioplasty . Presented dysautonomia perioperatively (duration 6 hours) without evidence of
cerebral hypoperfusion . Showed resolution of
visual symptoms after endovascular intervention
, and at discharge remained without clinical evidence neurological deficits (mRS0).
P-89
CASE REPORT OF CONVERSION DISORDER IN
ISCHEMIC STROKE
Veras SRO, Vieira LC, Maia IHM, Medeiros AD,
Ferreira DB
• SERVIÇO DE NEUROLOGIA DO HOSPITAL GERAL DE
FORTALEZA
Introduction: We report a case of stroke associated with a conversion disorder as comorbidity.
Objective: To warn about psychiatric disorders
complicating acute ischemic stroke. Case report:
RHFR, 46yo, female, was admitted to the General Hospital of Fortaleza emergency room with
complete right hemiparesis and a speech disorder, with a new onset headache started on the
previous day. On the stroke unit admission, a detailed neurological examination revealed normal
attention without abulia, impaired speech with
lack of fluency and delayed latency of verbal response, whispering, normal naming, repetition
and verbal comprehension, not consistent with
aphemia, speech apraxia, akinetic mutism or
66
dysarthria. She was indifferent to her difficulty
and smiled on attempt to speaking and was able
to articulate speech without fonation (aphonia),
which made aphasia unlikely. Motor examination showed severe right hemiparesis with facial
sparing, shoulder elevation and abduction deficits and inconsistent findings on the other right
limbs muscles. In the lower limb, Hoover, Raimiste and abduction tests were consistent with
functional motor impairment, and she was unable to lower the elevated flexed right leg, despite
gravitational forces helping the limb to drop.
On distraction, she had normal spontaneous
movements in the right limbs, and even reached
to grab a cup with the right hand. There was no
motor neglect or apraxia. Brain Magnetic Resonance Imaging showed a small, round, ischemic
stroke in the left middle and anterior cerebral arteries watershed zone over the precentral gyrus.
Computed Tomography Angiography suggested
left internal carotid artery dissection with distal
subocclusive narrowing, normal left middle and
anterior cerebral artery filling through collateral
vessels. Electroencephalogram showed mild to
moderate slowing over the left cerebral hemisphere. Psychiatry evaluation revealed a history
of recent marital physical and verbal abuse and
a depression disorder. Discussion: This patient
examination was consistent with functional motor and speech disorder complicating paresis on
the right shoulder from acute ischemic stroke.
She was discharged with medical management
for carotid arterial dissection and referenced to
a psychiatric follow-up. This highlights the importance of properly investigating patients with
possible conversion disorder for an underlying
neurological condition, as both can coexist.
P-90
CEREBRAL VENOUS THROMBOSIS IN VEIN OF
LABBÉ: CASE REPORT
Silva LSA1, Kroger JS1, Paranhos DR1, Guimarães VP1,
Farias DS2
• 1EBMSP; 2HOSPITAL GERAL ROBERTO SANTOS
Introduction: Cerebral venous thrombosis (CVT)
is a rare disease which incidence is approximately 5 cases per million people / year. It is three
times more common in women than in men and
usually is responsible for one of the syndromes:
Isolated Intracranial Hypertension; Focal neurological deficits; Seizures or Decreased level of
consciousness. Depending on the extent and
location of thrombosis syndromes can occur
in an associated way. The most common sites
of involvement are: superior sagittal sinus and
transverse sinuses, being rarely affected cerebral
veins, as the vein of Labbé, which is one of the superficial cerebral veins responsible for draining
the temporal region along the occipitotemporal
sulcus. Objective: The objective of this report is
to demonstrate the evolution of a patient without risk factors for atherosclerotic disease, which
developed CVT in Vein of Labbé. Case report:
MDS, female, 36 years old, came to the rescue of
HGRS with sudden onset of headache and loss of
muscle strength in right hemibody for two days.
The story was reported by the patient’s sister, as
she was globally aphasic and sleepy, with eye
opening only with strong stimuli. Her sister denied medical history of comorbidities, hospitalizations or allergies, as well as smoking habits ±
alcoholics. Patient improved level of consciousness and speech, staying with an understanding
aphasia. Skull’ CT showed extensive ischemic
and hemorrhagic lesions on parietal area of
left hemisphere. Angiography was reported as
normal, but the attending physician identified
occlusion Vein of Labbé Left. Discussion: The
CVT is an uncommon entity, whose rapid and
accurate diagnosis should be established to
perform the appropriate treatment, and control
reversal of brain damage and reduce complications such as long-term sequelae. What the
cause of venous thrombosis remains unknown,
but studies show that localized infections (such
as sinusitis) or systemic infections may be associated, as well as the use of oral contraceptives;
pregnancy; puerperium and SAAF. The research
of prothrombotic conditions in these patients
may be useful. However, the cause is not found
in 25% of cases. Imaging tests have essential role
in detecting this disease, evaluation of possible causes, extent and consequences, as well as
monitoring of treated patients. Keywords: Cerebral Venous Thrombosis; Vein of Labbé; Clinical
symptomatology; Stroke.
P-91
CLINICAL AND EPIDEMIOLOGICAL PROFILE OF
ACUTE CARDIOEMBOLIC STROKE PATIENTS IN A
STROKE CENTER IN BELO HORIZONTE/MG
Meira FCA1, Tavares RM1, Magalhães D2, Litwinski GIS1,
Ladeia LSA1, Silva LS3
• 1HOSPITAL UNIVERSITÁRIO RISOLETA TOLENTINO
NEVES; 2UNIFENAS BH; 3UNIVERSIDADE FEDERAL
DE VIÇOSA
Introduction: Stroke is the leading cause of
death in Brazil. Cardioembolic stroke represents
15‐25% of all ischemic strokes and has a trend to
early and late recurrence which can be prevented with appropriate treatment. Cardioembolic
stroke may also result in large extension lesions when proximal vascular occlusion occurs.
Objective: To evaluate the clinical and epidemiological profile of patients admitted to Risoleta
Tolentino Neves Hospital (HRTN) with acute
cardioembolic stroke. Methodology: Clinical
files of patients with acute cardioembolic stroke
admitted between March/2012 and March/2014
were reviewed. Demographic information,
stroke’s risk factors, drugs in use, diagnostic tests
(electrocardiogram [ECG], echocardiogram),
secondary prophylaxis treatment and outcomes
were analyzed. Results: We found 1.130 patients
hospitalized for acute ischemic stroke in HRTN
between March/2012 and March/2014. Cardioembolic stroke was represented by 195 patients
(17.6%), 46.6% female, mean age 68.3 years. Most
frequently found stroke risk factors were hypertension (80.0%), cardiopathy (35.9%), previous
stroke (33.3%), diabetes mellitus (30.8%) and
atrial fibrillation (26.7%). Chagas‘ disease was
registered in 8.2% of cases. Antiplatelet drugs
were used by 36.9% and anticoagulants by 16.9%.
Atrial fibrillation (AF) was observed in the admission ECG of 80 patients (41%). In the analysis of
echocardiogram results, we found akinetic ventricular segment (24.6%), left ventricular thrombus (10.6%) and dilated cardiomyopathy (3.1%)
as the most frequent high-risk sources of cardioembolism according to TOAST‘s classification.
Anticoagulation with warfarin was the discharge
treatment for 49.2% of the patients. In-hospital
death rate was 19.6%. Higher in-hospital mortality was not associated with any cardiovascular
risk factor or any diagnostic test abnormality.
Conclusion: The proportion of cardioembolic
stroke in HRTN approximates the one described
in the literature. Hypertension was the most frequent cardiovascular risk factor found for these
patients, followed by cardiopathy and previous
stroke. AF, akinetic ventricular segment and left
XXVI Congresso Brasileiro de Neurologia
ventricular thrombus were the most common
high-risk sources of cardioembolism according
to the TOAST‘s classification.
P-92
CLINICAL AND EPIDEMIOLOGICAL PROFILE OF
ELDERLY PATIENTS WITH ISCHEMIC STROKE AND
TIA ADMITTED AT TERTIARY EMERGENCY UNIT AT
A PUBLIC ACADEMIC HOSPITAL IN BRAZIL
Rocha LJA, Martins Filho RKV, Libardi MC, Santos RSA,
Pontes Neto OM, Camilo MR, Barreira CMA, Dias FA,
Pinto PTC, Alves FFA
• FMRP-USP
Introduction: Given the delayed epidemiological transition, the incidence of stroke among patients over 80 years has increased dramaticaly in
developing countries. Stroke risk increases with
advanced age, with several factors contributing
to this context, as atrial fibrillation and hypertension. Understanding the main etiologies for
stroke among elderly patients is crucial to reduce morbidity and mortality of these patients.
Purpose: To describe the spectrum of acute cerebrovascular diseases among patients older than
80 years, identifying key cerebrovascular risk factors, the main etiologies and clinical presentation
in those patients.. Methodology: A retrospective,
observational analysis of a prospective collected stroke database including all patients aged
greater than 80 years, admitted to the emergency room of our tertiary public academic hospital
between 2011‐2014. Results: among 94 patients
included, 54 (57.4%) were female, with a mean
age of 84.2 (SD 4.0) years. The median NIHSS at
admission was 7 (IQR: 3.75 to 16.25). 81 (86.2%)
patients had known previous hypertension; 36
(38.3%) had previous ischemic stroke or TIA;
35 (37.2%) were diabetic; 32 (34%) patients had
atrial fibrillation; 26 (27.7%) had dyslipidemia;
25 (26.6%) were smokers; 21(22.6%) patients had
coronary artery disease; 14 (14.9%) were alcoholics; 7 (7.4%) patients had peripheral artery disease; and 3 (3.2%) had previous extracranial stenosis. On TOAST classification, 34 (36.2%) were
classified as cardioembolic stroke; 17 (18.1%)
as atherosclerosis of small vessels; 12 (12.8%) as
atherosclerosis of large vessels; 25 (26.6%) as undetermined; and 1 (1.1%) as other known mechanisms. Using the Bamford classification system,
36 (38.3%) were classified as PACS; 25 (26.6%)
TACS; 24 (25.5%) LACS; and 9 (9.6%) POCS. Fifty-nine (81.8%) patients had anterior circulation strokes. Seventeen (18.1%) patients died
in hospital, and two of them had symptomatic
intracranial hemorrhage. Conclusion: We identified high mortality rate among elderly patients
with stroke. Most of the deaths were nor directly
atributted to stroke itself, but to pre-existing or
acquired comorbidities and complications. We
also found a high prevalence of cardioembolism
as the leading etiologic mechanism for the index
and recurrent stroke / TIA. Further studies are
needed to improve our understanding the complex clinical profile of elderly patients with stroke
in Brazil.
P-93
COCAINE USE ASSOCIATED TO CEREBRAL
VASCULITIS
Correia DSA1, Rebello LC1, Valencia CEU1, Dias EC2,
Vizioli CR2, Carvalho IFS2
• 1HOSPITAL DE BASE DO DISTRITO FEDERAL;
2
HOSPITAL BRASÍLIA
Background: Cocaine use is associated to a significant number of strokes, especially in young
patients. This Case report describes a severe case
of cerebral vasculitis developed in a cocaine user,
who was treated with corticoids and cyclophosphamide. Objective: The main Objectives: are: to
discuss the management of vasculitis associated
to cocaine use, to describe the image findings
before and after pharmacological treatment,
and to describe the clinical response observed.
Case report: Male patient with twenty three
years old, referring history of smoking and cocaine use, with complain of headache during
fifteen days and sudden focal deficit. The clinical examination showed a right arm monoplegia and right homonymous hemianopsia. The
magnetic resonance image showed hyperacute
vascular lesions in both cerebral hemispheres
(left frontal and occipital, and parietal lobes bilaterally). The patient developed decreased level
of consciousness, left hemiplegia and worsening
of the visual deficit. The magnetic resonance angiography showed acute ischemic lesions, associated to irregularities and multifocal narrowing
of the intracranial arteries. Rheumatologic tests
and CSF were normal. The patient showed a clinical improvement after a short course of methylprednisolone 1g daily for five days. A new magnetic resonance angiography demonstrated a
reduction in the narrowing areas detected in the
previous exam. In the following days, a pulse of
cyclophosphamide was administered, with subsequent clinical and radiological improvement.
Discussion: Cocaine use is associated to stroke.
Cerebral vasculitis related to cocaine use is a rare
and recurrent entity, due to lesion of the tunica
media of the vessels. The principal mechanisms
are: vasospasm, increase in the platelet aggregation, and loss of the cerebral flow autoregulation.
The diffuse cerebral involvement and the narrowing of the terminal vessels found in the magnetic resonance angiographies were suggestive
of vasculitis. High-dose corticoids has been the
treatment of choice in cocaine-related vasculitis.
In this case, the improvement in motor and visual functions, and even in the vessel narrowing,
was evident. The use of cyclophosphamide in
cocaine-related vasculitis has few descriptions in
literature. Nevertheless, in our opinion, the use
of cyclophosphamide was important to consolidate the clinical improve in this patient.
P-94
COGNITIVE FUNCTION AS A RISK FACTOR FOR
FALLS IN STROKE PATIENTS
Sasaki AC1, Mendel T1, Souza MCM1, Pinto EB2,
D‘Oliveira Junior A1, Oliveira Filho J1
• 1UNIVERSIDADE FEDERAL DA BAHIA; 2ESCOLA
BAHIANA DE MEDICINA E SAÚDE PÚBLICA
Introduction: Recently, studies have shown
interaction between cognitive function and
motor control. Stroke patients present high frequency of cognitive impairments, which could
affect postural control and predispose to falls.
Objective: to identify the association between
cognitive function and the occurrence of falls in
individuals after stroke recruited from a stroke
outpatient. Method: crossectional study with
155 post community dwelling stroke individuals
with independent gait and ability to understand
instructions of the following tests/scales: NIHSS,
Barthel, MMSE, Verbal Fluency, Timed Up & Go
Test (TUG), TUG with cognitive task (TUGcog).
The occurrence of falls in the previous 12 months
was described. A theoretical determination model with four hierarchical blocks was built to analyse factors associated with falls. Variables were
adjusted among each other within each block;
those with P≤0.20 were included in the logistic regression model and adjusted to the immediately
higher level, considering P≤0.05. Results: Mean
age of 56.6±13.7 years; 91(58.7%); females. Median NIHSS score of 2 (0‐9) and Barthel: 49(32‐50);
15(1‐183) months since stroke, Verbal Fluency
test: 10(2‐22); MMSE: 24(12‐30); TUG: 13(6‐54) e
TUGcog: 17(8‐68). Forty four pacientes (28,4%)
suffered at least one fall over the past 12 months.
After multivariate analysis only cognitive function remained significant(P<0,01). Conclusion:
cognitive function is associated with ocurrence
of falls in stroke individuals with independent
gait. So it is important to identify people with
cognitive impairment who are prone to fall, and
estabilish adequate prevention strategies.
P-95
COMPARATIVE AVALIATION OF FORTALEZA’S
POPULATION ABOUT GENERAL KNOWLEDGE OF
STROKE IN TWO DIFFERENT SIDES OF THE CITY
Monteiro EMR, Costa IA, Albuquerque ES, Araujo DF,
Oliveira ISC, Chaves Filho AJM, Cavalcante RO,
Oliveira SA, Barbosa PS, Rodrigues GC
• UFC
Introduction: The knowledge of general concepts and risk factors of cerebrovascular accident (CVA) is very important in preventing and
reduction of cases of the disease, since most of
these risk factors are modifiable. Objectives: To
evaluate and compare the level of knowledge of
stroke between a sample population frequents of
a public square in the center of Fortaleza and a
shopping mall in the prime area of the city, combining this knowledge with the disparate level of
schooling found in both samples. Method: We
applied a structured questionnaire about basic
knowledge and risk factors for cerebrovascular
disease in question to citizens of a square from
the center of Fortaleza and a shopping center in
prime area of the city. Results: 70 questionnaires
were answered, 30 were completed by the Public
Square (Pa) and 40 by the group at the mall (Sa)
group. Of respondents in the square, 27% had
incomplete primary education and 13% completed high school; at the mall, the percentages
were respectively 2.5% and 45%. A sample of the
square had smaller amount of people who knew
what was stroke (Pa: 76%; Sa: 85%), and fewer
people who knew 1‐3 risk factors for stroke compared with the sample from shopping (PA: 6.7%;
Sa: 35%). Despite these data, the sample of the
square had more people who have had or knew
someone who suffered stroke (Pa: 87%; Sa: 67%).
Conclusion: It was observed that people with
less education had poorer knowledge of stroke
compared to people with higher education.
However, it was observed that the prevalence of
occurrence of stroke is higher in people of the
social sector with lower education. Campaigns
to increase knowledge of stroke can have a major impact on public health, especially among
groups at highest risk for this condition, as the
population with lower education. Keywords:
stroke, public knowledge, lay knowledge.
67
Posters
P-96
CRYPTOGENIC CEREBRAL ISCHEMIC STROKE
ASSOCIATED WITH PATENT FORAMEN OVALE
Faria DD, Santos DH, Melo ACP, Gois L, Diniz J
• IAMSPE-HOSPITAL DO SERVIDOR PUBLICO
ESTADUAL
Introduction: Patent foramen ovale (PFO) is
present in up to 25% of the general population
and is a frequent association with cerebrovascular accident (CVA) and ischemic (I) vascular
cryptogenic that can match between 25 and
40% of all stroke cases. The PFO is developed
by a failure of the septum primum to fuse with
the septum secundum allowing communication
between the atria. The diagnosis is given with
transesophageal echocardiographies (Ecote),
which by injecting saline into the venous system
are seen bubbles in the left atrium in 3‐5 cardiac
cycles. Objective: Describe the case of a patient
with cryptogenic ischemic stroke and PFO documented by echocardiography, which had deep
venous thrombosis (DVT) in the left femoral
vein and popliteal vein. Case report: Patient of
58 years, diabetes, hypertension, history of DVT
in the left leg in irregular use of Rivaroxaban was
admitted to the emergency room of the Hospital
do Servidor Público Estadual de São Paulo with
loss of strength in the left hemisphere and imbalance. Research conducted for stroke and showed
ischemic insults bilateral in the nuclear capsuloligamentous on CT. Resonance angiography and
cranial vessels presenting ischemic focus capsular-thalamic nuclear to acute / subacute right
path with vessels, caliber and signal flow preserved. The Doppler showed left lower limb DVT
in the left femoral and popliteal vein and Ecote
with regular rhythm with right-left shunt at PFO,
left atrial 42mm normal right atrium and was
not visualized abnormal echoes consistent with
thrombus, and normal ventricular cavities with
fraction ejection of 49%. AAS progressed to full
recovery of motor deficit and balance, receiving
anticoagulation with warfarin and referred to the
clinic for anticoagulation control. Discussion:
The relationship between PFO and ischemic
stroke is not well determined but the possibility
of a thrombus in transit through a PFO causing
a paradoxical embolism is a plausible cause of
ischemic stroke in patients with PFO.
P-97
DEJERINE`S SYNDROME: A CASE REPORT
Rigoni L, Alessi R
• FMABC
Introduction: The medulla is the lower portion
of the brainstem, which is responsible for conducting nerve impulses and contains nucleus of
the lower cranial nerves. The main clinical signs
of involvement of the medulla are misuse of
language (occurs by involvement of the cortical
bulbar fibers, the core of the issue or the hypoglossal nerve) and the possibility of incomplete
and alternating hemiparesis. Due to the multiple perfusion bulb, four vascular syndromes
are described. Average Medullary Syndrome or
Syndrome Déjerine is the rarest, described today
in less than thirty patients. Objective: Describe
a case of this unusual syndrome and review the
semiological etiology and anatomical concepts.
Case report: MST, female, 67 years, w/ hypertension and DM II comes to the clinic complainining
about winding tongue 2 years ago, sudden onset,
w/ gradual and slight improvement. Neurological
examination patient has medullary dysarthria,
68
paresis of the tongue to the left, w/ deviation
of protrusion on the same side (the slow utterance of the sound “C” and “T” alternating movements). The remainder of the neurological examination showed signs of left soft palate, decreased
gag reflex and ipsilateral hemiparesis alternating
(degree of muscle strength and IV pyramidal in
right hemisphere, without further amendment).
Examination MRI demonstrated small lesion in
the left medial medullary high and MR angiography of the cervical vessels and cranial without
abnormalities, suggesting lacunar infarction for
the etiology. Discussion: Dejerine syndrome is
a rare clinical entity, usually produced by occlusion of the distal vertebral artery or the superior
portion of the anterior spinal artery dissection.
Semiology of tongue movement is neglected and
considered a poor signal or distinction between
upper and lower motor neurons. A careful examination and use of diagnostic Methods: allow the
inference of cause of injury. Patient sought explanations for two years in many consultations
and diagnostic procedures without satisfactory
response, and the lack of basic neurological examination the main cause of the delay.
P-98
DELAYED DIAGNOSIS OF HYPERTENSION IN
INDIVIDUALS ATTENDING AN OUTPATIENT
REFERRAL OF STROKE IN SALVADOR, BAHIA,
BRAZIL
Echegaray MVF, Passos Neto CEB, Matias LG,
Silva BCG, Moreira HLC, Fukuda TG, Jesus PAP
• UFBA
Introduction: Hypertension affects a significant
portion of the world population (20‐30%). Among
the conditions which hypertension predisposes, stroke stands as the second leading cause of
death in Brazil. Early diagnosis and treatment of
hypertension, a modifiable cardiovascular risk
factor, delay the progress of lesions in target organs and prevent sequelae and elevated costs,
thereby should be priority goals of the health
system. Objectives: To describe the frequency of
delayed diagnosis of hypertension in an outpatient referral for secondary prevention of stroke
in Salvador, Bahia, Brazil. Methods: This study
was a cross-sectional study of 127 patients attending an outpatient referral of stroke, assessed
from April 2014 to August 2014. It is an analytical
study, with data collected from medical records
and through pre-defined interview. Measures of
central tendency and frequency were used for
description. For univariate analysis, chi-square
tests (categorical variables) and Mann-Whitney
test (numerical variables) were made; logistic
regression by backward conditional method was
used for multivariate analysis. The data were analyzed using the software SPSS v21. Results: The
sample included 127 hypertensive patients. 68
(53.5%) were women and 59 (49.4%) were men.
The average age was 59.19 years old. 19 (14.9%)
patientes were white, 46 (36.2%) were black and
55 (43.3%) were coloured. 104 (81.9%) subjects
had dyslipidemia, 48 (37.8%) had type 2 diabetes
mellitus, 18 (14.2%) had heart failure, 14 (11%)
had atrial fibrillation and 13 (10.2%) had arterial
coronary disease. 106 (83.4%) of the subjects experienced ischemic stroke; and 10 (7.87%) experienced hemorrhagic stroke, 2 being of hypertensive etiology. 32 (25.2%) individuals were diagnosed during hospitalization of stroke. In univariate analysis, there was a statistically significant
association between delayed diagnosis and sex
(p<0.03), age (p<0.03) and smoking (p<0.139).
There was no association between variables in
the multivariate analysis. Conclusions: Hypertension is considered a major cause of reduced
life quality and sequelae in patients. Delayed diagnosis impairs the patient and the public health
system, because treatment of lesions in target
organs is expensive. The rate of 25.2% of delayed
diagnosis in this study points to the deficit in
the hypertension primary prevention goals and
reinforces the need to improve health education
strategies.
P-99
DO YOU KNOW HOW MANY PATIENTS ARE
FUNCTIONALLY DEPENDENT ONE YEAR AFTER
STROKE? THE JOINVASC STROKE REGISTRY
Campos LM1, Franco SC1, Muller M1, Oliveira LIM1,
Nagel V2, Cabral NL1
• 1UNIVILLE; 2REGISTRO DE AVC DE JOINVILLE
Background: Global burden of stroke measured
by disability-adjusted life years lost have been
rising worldwide. Very few population-based
studies measured the functional dependence in
low or middle-income countries. Aim: We aim
to know the proportion of dependent patient after first-ever stroke in Joinville, Brazil. Methods:
We extracted from Joinville Stroke Registry, an
ongoing population-based stroke databank established in Joinville since 2005, all consecutive
patients who had had a first ever stroke from
2009‐13 in Joinville. By telephone, we measured
the functional dependence (mRankin 3‐5), in 30
days, 6 and 12 months after hospital discharge.
To predict functional dependence we analyzed
previous demographical and clinical variables
among the 285 survivors at 12 months. Results:
Over 4 years, we registered 400 stroke survivors
and 115 deaths during the first year. The 285
remaining were predominantly males (58.2%),
mean age 63.1±16,2 years, with low educational
(77.3%) and social (72.3%) level. Over 61.5% had
low admission NIHSS score, 39.4% had two pre
morbid risk factors and 7.4% had recurrence. At
30 days, 23.1% (72/312; 95% CI, 18.4% to 27.8%)
of survivors were functionally dependent, at 6
months 16.6% (49/295; 95%CI, 12.4% to 20.8%)
and at one year 15.8% (45/285; 95%CI, 11.6% to
19.9%). Increasing age was a significant predictor
of functional dependence at 12 months among
stroke survivors (p<0,001). Conclusions: These
findings point to a favorable functional dependence evolution of post-stroke patients during
12 months of follow up. Nevertheless, the high
prevalence of functional dependence observed
among stroke survivors makes it a public health
problem. These prevalence data are useful for
planning health rehabilitation policies.
P-100
DOCUMENTED PARADOXAL EMBOLISM TO THE
BRAIN
Fróes ALR1, Meira FCA2, Xavier RMB2, Silva RW1,
Vieira AARM1, Antunes AG1
• 1UFMG; 2HOSPITAL RISOLETA TOLENTINO NEVES
Introduction: Stroke is the main cause of disability in the world. Approximately 20% of all
strokes have a cardioembolic etiology. Young patients deserve special attention in the etiology of
stroke, since there may be present less prevalent
mechanisms. Appropriate secondary prevention
can prevent the recurrence of this disease at this
age has a significant impact on psychological,
social and economic aspects. Objective To report
the case of a young patient, admitted to Risoleta Tolentino Neves Hospital with cardioembolic
XXVI Congresso Brasileiro de Neurologia
stroke associated to documented paradoxical
embolism. Case report CCN, 48 years old, male,
presented acute hemiparesis and left homonymous hemianopia. The weakness resolved on
the way to the hospital (with no more time for
intravenous thrombolysis), however, the visual
deficit remained. The head computed tomography (CCT) at admission did not show acute abnormality. On the first day at the Stroke Unit, the
patient complained of pain and swelling on the
right calf. With a venous duplex scan of the lower
limbs, deep vein thrombosis was diagnosed. Anticoagulation was then initiated. The carotid duplex scan showed no alterations. A control head
CT performed on the second day showed the
definition of the acute ischemic area on the right
occipital lobe. On the third day of hospitalization, the patient presented sudden upper right
limb weakness and motor aphasia. An emergency head CT was performed and showed signs of
acute ischemia of the left frontal region. During
hospitalization, the patient was submitted to a
transesophagic echocardiogram, which identified right ventricular failure and pulmonary hypertension (PH) (42mmHg), the presence of ostium secundum interatrial communication (IAC).
Due to the patient´s complaints of dyspnea and
the PH finding on the echocardiogram, a thoracic angiotomography was performed, which
identified the presence of pulmonary thromboembolism (PTE). Thus, it was established and
documented that CCN presented deep vein
thrombosis, with PTE and multiple paradoxical
embolism (through IAC) to the brain, resulting
in an ischemic stroke. Discussion Although the
classic risk factors, such as arterial hypertension
and dyslipidemia are present on the young population diagnosed with stroke, assessment with
Methods: that clarify the etiologic definition of
these stroke patients is essential. The cardioembolic etiology is dominant in such patients, suggesting that transesophageal echocardiogram
should be more widely used.
P-101
DOES GENDER INFLUENCE THE OUTCOME
OF ISCHEMIC STROKE PATIENTS AFTER
INTRAVENOUS THROMBOLYSIS?
Camilo MR, Pinto PTC, Barreira CMA, Alves FFA, Dias FA,
Rocha LJA, Santos RSA, Martins Filho RKV, Libardi MC,
Pontelli TS, Abud DG, Pontes Neto OM
• FMRP-USP
Background: It has been reported that women
with stroke have a worse outcome than men.
Intravenous thrombolysis is the main treatment
strategy to improve outcome of stroke patients.
However, the influence of the gender on the efficacy and safety of intravenous tPA for stroke
remains controversial. Objectives: The aim of
this study was to evaluate whether gender was
associated to in-hospital mortality and occurrence of symptomatic intracranial hemorrhage
(SIH) among acute stroke patients treated with
intravenous tPA. Methods: Consecutive stroke
patients were retrospectively selected from a single-center, hospital-based prospective registry of
acute stroke patients admitted between January
2001 and July 2014. Demographic, clinical and
radiological data were collected. Results: A total
of 307 patients with ischemic stroke were treated
with intravenous TPA. Among those, 139 (45.3%)
were females and 168 (54.7%) males. Women had
a more often history of atrial fibrillation, heart
failure, dyslipidemia and use of antiplatelet than
men. Systolic and diastolic blood pressure levels
on admission were significantly lower in women.
Men were more likely to be current smokers. No
gender difference was found in the age (p=0.29),
baseline NHISS (p=0.43), time to thrombolysis from symptom onset (p=0.41) or mortality
in-hospital (p=0.32). In multivariable analysis,
atrial fibrillation (OR 2.5; 95% CI 1.34 to 4.70) and
diastolic blood pressure levels (OR 0.98; 95% CI
0.96‐0.99) were associated with female gender. A
nonsignificant trend toward a lower risk of SIH
(OR 0.41; 95% 0.16‐1.09) was observed for women. Conclusions: Our results suggest no gender
difference on early mortality among patients
treated with intravenous tPA, and a trend toward
a lower rate of SIH among women.
P-102
DOES THE SIDE OF MIDDLE CEREBRAL ARTERY
COMPROMISE MATTERS IN THE MORTALITY AFTER
THROMBOLYSIS IN ISCHEMIC STROKE?
Ducci RD1, Lange MC1, Moro CH2, Harger R1, Longo AL2,
Germiniani FMB1, Cabral NL3, Nóvak EM1, Zetola VF1
• 1DEPARTAMENTO DE NEUROLOGIA, HOSPITAL DE
CLÍNICAS - UFPR; 2DEPARTAMENTO DE NEUROLOGIA
DO HOSPITAL MUNICIPAL SÃO JOSÉ
Objectives: The impact of the side in middle cerebral artery (MCA) ischemic stroke (IS) is not
well established. Our aim was to analyze the
differences between right and middle cerebral
artery ischemic stroke in patients submitted to
intravenous thrombolysis and the influence of
the affected side in the patient‘s mortality after 3 months. Methods: Patients with MCA IS
who were submitted to intravenous thrombolysis from March 2010 to December 2011 at two
Brazilian Stroke Centers (Curitiba and Joinville)
were included in the current study. Differences
between patients with right and left middle cerebral artery ischemic stroke were identified by
univariate analysis. Results: Forty-five patients
with right middle cerebral artery ischemic stroke
and 67 with left middle cerebral artery ischemic
stroke were analyzed. Patients with left middle
cerebral artery ischemic stroke had a higher incidence of atrial fibrillation (p=0.031), although
patients with right cerebral artery ischemic
stroke more often had a history of a previous
ischemic stroke (p=0,034). The mortality over
3 months was similar for either side (OR=1.20;
0.37‐4.29, p=0.772). Conclusion: The side of the
middle cerebral artery ischemic stroke did not
influence on the patients mortality in the present
study.
P-103
DRUG INTERACTIONS IN A STROKE UNIT
Possamai KS, Schmidt L, Fernandes FM
• HOSPITAL MUNICIPAL SÃO JOSÉ
Introduction: Stroke Unit (U-stroke) of the Municipal Hospital São José - Joinville / SC is comprised of an interdisciplinary team, this being
reinforced by the implementation of multidisciplinary home health, covering the professions
of pharmacist, physiotherapist, nutritionist and
psychologist. Drug interactions occur when the
effects ± toxicity of a drug are affected by the
presence of another. They are usually unpredictable and undesirable. Can be physical-chemical, pharmacodynamic or pharmacokinetic.
The phenomenon of drug interactions is today
one of the most important topics of pharmacology for the clinical practice of health professionals. Objectives: To identify and quantify
the major interactions between prescription
drugs to the U-stroke patients. Methods: Between the months from April to June 2014, the
requirements of U-stroke were analyzed by pharmacist in order to identify and quantify drug interactions classified as serious. Bases Medscape
Drugs and data were used to evaluate drug interactions. The data were tabulated according
to the combinations of drugs prescribed within
24 hours. Results: Analyzed 477 prescriptions
for 115 patients, 299 classified as serious drug
interactions have been identified. 38 different
potential drug interactions, five of them were
more frequent. These drug interactions occurred
between acetylsalicylic acid and heparin (13%),
amlodipine and simvastatin (18%), enoxaparin,
and warfarin (14%) fluoxetine and clopidogrel
(7%) and omeprazole and clopidogrel (6%). Interventions were made whenever arose potential
drug interactions, and evaluated by the medical
staff and conducted management, taking into
consideration the risk benefit of the therapy to
the patient. Conclusion: The presence of drug
interactions is a permanent risk in hospitals and
the number of drugs / day is a high positive predictive value for these. Therefore, awareness of
the team of professionals involved in prescribing, dispensing and administration of medicines
become clearly important. A infortatized data
system is being implemented as a means to identify drug interactions when prescribing.
P-104
DYSPHAGIA‘S CHARACTERIZATION AFTER STROKE
IN PATIENTS AT EMERGENCY ROOM AND THE
RELATIONSHIP WITH LESION‘S TOPODIAGNOSIS
Corder HM, Oliveira Neto IC, Saconato M, Gonçalves MIR
• UNIFESP
Objective: To characterize dysphagia after cerebral cortex stroke according to cerebral hemisphere involved. Method: A retrospective study
was made with the analyses of speech-language
pathology evaluation protocols of patients with
unilateral cerebral stroke at ER in and University Hospital at São Paulo city, between March of
2013 and March of 2014. Results: 21 protocols
were analyzed; 57% patients were female and
43% were male; mean age was 66.5 years. Of
the total sample, 43% presented right cerebral
cortical lesion (RCCL) and 57% had left cerebral cortical lesion (LCCL). During swallowing
evaluation, it was observed that 55.5% of RCCL
patients presented severe oropharyngeal dysphagia, 22.2% moderate oropharyngeal dysphagia and 22.2% normal swallow. Regarding LCCL
patients, 25% presented severe oropharyngeal
dysphagia, 16.7% moderate oropharyngeal dysphagia and 58.3% normal swallow. Considering
swallowing oral stage alterations, 44.4% of patients with RCCL and 25% with LCCL presented
it. Conclusions: RLLC patients presented more
severe and moderate oropharyngeal dysphagia, and higher number of swallowing oral stage
alterations.
P-105
EARLY VERSUS LATE CAROTID ARTERY STENTING
FOR SYMPTOMATIC CAROTID STENOSIS: A
SINGLE-CENTER RETROSPECTIVE STUDY
Castro-Afonso LH, Nakiri GS, Monsignore LM,
Santos AC, Leite JP, Fabio SRC, Pinto PTC, Camilo MR,
Pontes-Neto OM, Abud DG
• HCFMRP-USP
Background and purpose: Early carotid revascularization (≤14 days) is recommended for
symptomatic carotid stenosis. Carotid artery
stenting (CAS) has become an alternative to
69
Posters
carotid endarterectomy (CEA); however, safety data on early CAS is controversial. The study
aims to compare early versus late CAS, when
CAS is performed as a first intention revascularization strategy. Methods: A retrospective analysis of all symptomatic patients admitted to our
stroke unit who underwent CAS was conducted.
Patients were divided between two groups: patients who had undergone CAS within 14 days
after symptoms and those who had undergone
CAS later. Primary endpoints were ipsilateral ischemic stroke or ipsilateral parenchymal
hemorrhage (iPH) at 30 days. The secondary
endpoints were major adverse cardiac and cerebrovascular events (MACCE) at the 30-day and at
the 12-month follow-up. Results: One hundred
twenty-seven consecutive patients were evaluated. Primary endpoints obtained in the early and
late CAS groups were, respectively, ipsilateral
stroke (2.0% vs. 2.6%, p=1.00) and iPH (2.0% vs.
0.0%, p=0.40). The rates of MACCE between the
early and the late CAS groups were, respectively,
(7.8% vs. 2.6%, p=0.21) at the 30-day follow up,
and (12.2% vs. 10.5%, p=0.77) at the 12-month
follow-up. Conclusions: In this retrospective
study, CAS seems to be as safe as CEA when used
as first-intention revascularization treatment
within 14 days of symptoms. Our results reinforce
the need for randomized trials comparing CAS to
CEA within the first two-weeks of symptoms.
P-106
EMERGENCY CARE FOR VICTIMS OF ACUTE
STROKE: ASSESSMENT OF THE INTERVALS OF
TIME BEFORE AND AFTER PUBLICATION OF THE
OFICIAL PROTOCOLS OF THE BRAZILIAN MINISTRY
OF HEALTH (DECREES 664 AND 665 OF 12 APRIL,
2012)
Farias CA1, Paranhos DR2, Guimarães VP2, Ferreira THS2,
Barreto ES2, Brito FS2, Casal YR2, Almeida ERP2,
Maldonado IL3
• 1FACULDADE DE TECNOLOGIA E CIÊNCIAS; 2EBMSP;
3
UFBA
Introduction: Stroke is one of the most frequent
causes of acquired morbidity and mortality in
the world. This fact highlights the need to pursue
actions that speed up assistance for the victims.
In this context, the Brazilian Ministry of Health
published two official protocols (number 664
and 665 of April 12, 2012) that strengthened the
importance of patient care in the acute phase.
Objective: To determine the time interval in
which stroke patients obtain a first medical attention in referral center before and after publication of the official protocols. Methods: This
was a cross-sectional study with retrospective
and observational design. The sample consisted of 88 patients admitted in the years 2011 and
2013 in the emergency department of a referral
center of Salvador-Bahia. The following variables were recorded: time interval from the onset
symptoms to the hospital admission and from
the onset of symptoms to the first medical evaluation. Results: There was an increase in the total
number of adults that searched for medical care
because of suspected stroke: from 1095 to 1230. A
statistically significant increase in the proportion
victims who were admitted in the first 24 hours
after onset was observed: from 48% to 58.4%
(p=0.022), and a tendency (non-significant) of
increase of the number of patients admitted in
the therapeutic window for intravenous thrombolysis (<4h30min). Conclusion: According to
the results of the present study, there was an
increase in the number of emergency appointments for stroke victims in 2013 in comparison
70
with 2011 (approximately 12.9%). A greater proportion of victims arrived to the emergency department within 24 hours of onset of symptoms.
P-107
ENDOVASCULAR TREATMENT OF CRITICAL
BASILAR STENOSIS: A RELATE OF TWO CASES
Santos RVSG1, Rodrigues AB2, Macedo LP3,
Gonçalves Junior PCJ2, Vale BP4
• 1UESPI; 2UFPI; 3FACID; 4INSTITUTO DE
NEUROCIÊNCIAS DO PIAUÍ
Introduction: Vertebrobasilar arterial territory
nourishes one-quarter of human brain. It constitutes some vital and strategic parts of the central
nervous system. The basilar artery stenosis (BAS)
is an uncommon condition that occurs when
there is any plaque or blood clot partially or
completely blocking the passage of blood in this
artery. It is observed more in men than women,
the sixth and seventh decade of life. This study
aims to relate two cases of BAS with intracranial
angioplasty and stent placement. Case 01: OTD,
80, male, history of previous left brain stroke,
neurological deficits on the right, with apathy,
dizziness, fainting episodes. Physical and neurological examination revealed bilateral cervical
and supraclavicular murmur. Resonance angiography revealed lesions in the left internal carotid and vertebral arteries. MRI demonstrated
left ischemic stroke and cerebral angiography
showed lesions and critical stenosis of the left
vertebral artery and occlusion of the left internal carotid artery. We conducted a basilar artery
angioplasty with implantation of drug-eluting
stents (3,5x20). In follow-up, five months after,
through Doppler Carotid and vertebral arteries, showed good flow in the region. Abnormal
behavior, apathy, agitation, walking with assistance, Poststroke sequelae grading with ranking
rating 3 (moderate disability). Case 02: JBS, 67,
male, Glasgow 3. Family related symptoms of
vertebrobasilar syndrome. Cerebral angiography
showed occlusion of the basilar artery along its
entire length. Was held up microcatheterization
basilar artery infusion of thrombolytic enzyme
and stenting 3,0x12. In control angiography
showed satisfactory anatomical result. Reduced
visual acuity and left homonymous hemianopia, with rankin rating 1 (no significant disability
despite symptoms). Discussion: Endovascular
treatment of intracranial atherosclerotic disease
is controversial. Acute ischemic stroke (AIS) due
to thrombosis of an in situ stenosis has undergone little investigation. Infarction from such
disease in the posterior circulation causes pronounced morbidity. The combination of initial
angioplasty followed by delayed endoluminal
stent placement may reduce the neurological
morbidity associated with endovascular treatment of long, high-grade stenotic lesions.
P-109
EVALUATION OF OXIDATIVE STRESS,
INFLAMMATORY, CELL DEATH BIOMARKERS AND
ALA16VAL–MNSOD2 GENE POLYMORPHISM IN
PATIENTS WITH CHRONIC STROKE
Fighera MR, Flores AE, Duarte MF, Almeida C, Kegler A,
Pascotini E, Prado ALC, Santana D
• UFSM
Introduction: The superoxide dismutase manganese dependent (MnSOD2) catalyzes O2 in
H2O2 into mitochondria and is encoded by a
single gene that presents a common polymorphism that results in the replacement of alanine
(A) with a valine (V) in the 16 codon, who has
been implicated in a decreased efficiency of
SOD2 transport into targeted mitochondria in V
allele carriers. Previous studies described an association between VV genotype and neurological
diseases, including stroke. However, the causal
mechanisms to explain this association need to
be more elucidated. Objective: Was be investigate if have a relationship in the oxidative stress
and inflammation biomarkers with the polymorphism in the patients with chronic stroke (CS).
Methods: Cross-sectional study with comparison group (n=40) CS patients and 40 without any
disease (control group). The study population
consists of patients with CS (>6 months), regularly accompanying on the Neurologic Clinic of
the Hospital of Federal University of Santa Maria - Brazil (HUSM) and subjects without any
disease. A questionnaire was applied to baseline
characteristics and clinical and after laboratory
for identification of serum glucose (GLU), glycated hemoglobin (HBA1C), cholesterol (CHO),
urea, phosphatase (PHO), acetylcholinesterase
(ACHE), dihidrodiclorofluorescein (DCFH), tumoral necrosis factor (TNF-α), Caspase 3 and
PicoGreen (PG). All analyses were carried out
using the SPSS statistical software, version 18.0
(SPSS Inc., Chicago, IL). Ala16Val–MnSOD results
were presented in mean (%). For comparison of
serum l we used t test analysis. Significance was
set at 5%. Results: Statistical analyses showed
that patients with CS presented GLU (t=3.58 mg/
dL), HBA1C (t=2.6%), CHO (t=3.89 mg/dL), LDL
(t=3.09 mg/dL). In addition, we observed that
patients with CS presented Urea (t=6.37 mg/
dL), PHO (t=5.7 UL/ml) and ACHE (t=6.7 UL/L)
levels higher than control group. Futhermore,
statistical analysis showed that patients with CS
presented higher DCFH (t=2.9% of DCF), TNF-α
(t=8.8 pg/ml), Caspase 3 (t=6.8 Fl/min/ml) and
PG (t=7.7 pg/ml) levels than control group. We
observed that CS group presented higher percentage of VV genotype (40%) as compared control group (10%). Conclusion: Our results suggest
that the polymorphism could influence the oxidative stress and inflammatory response in the
patients with CS.
P-110
EVALUATION OF THE DEATH RISK FROM
PNEUMONIA IN PATIENTS WITH STROKE:
CORRELATION BETWEEN NATIONAL INSTITUTE OF
HEALTH STROKE SCALE (NIHSS) AND MODIFIED
CLINICAL PNEUMONIA INFECTION SCORE (CPIS)
Souza AP, Reges DS, Oliveira FTM, Pagiola IC,
Zetehaku AC, Fortes ALVS, Luca NC, Melges NS,
Gagliardi VDB, Massaia I, Gagliardi RJ
• IRMANDADE SANTA CASA DE SÃO PAULO
Objective: To correlate the scores of the National
Institute of Health Stroke Scale (NIHSS) and the
modified Clinical Pneumonia Infection Score
(CPIS) with the risk of death from pneumonia in
patients with stroke. To date, there are no studies
on the correlation between the NIHSS and the
modified CPIS in patients with stroke at risk of
death from pneumonia. Studies correlating the
CPIS score are scarce and with small samples.
The present study aims to contribute in this
scenario. Methods: Prospective observational
study with patients with hemorrhagic stroke and
who underwent mechanical ventilation (MV)
for more than 48 hours. Patients with epilepsy
or convulsive episodes and patients who used
beta-blockers in the last 60 days were excluded.
On admission assessment, the values of NIHSS
and the modified CPIS were noted; considered
as pneumonia associated with MV (PAV) patients
XXVI Congresso Brasileiro de Neurologia
with CPIS>6. The sample consisted of 128 patients; 71 were male (55.5%), and patients were
divided into two groups: G1: patients with PAV
(n=88) and G2: patients without PAV (n=40).
Results: The correlation of admission NIHSS
vs final NIHSS between groups resulted in G1:
19.36±4.17 vs. 21.30±4.18 (P=0.035) and in G2:
20.35±3.09 vs. 19.30±5.23 (P=0.001). The analysis
of the mean NIHSSadmission vs CPIS during the
first episode of PAV were 20.35±3.09 vs 6.75±0.35
points (P=0.017); Mortality in the sample G1 and
G2 were respectively of 38 (43.2%) and 10 (25.0%)
patients (P=0.012). Conclusion: The presence of
correlation between NIHSS and modified CPIS
may be indicative of mortality from PAV.
P-111
EXCESS FACTOR VIII AS A CAUSE OF
CEREBROVASCULAR DISEASE
Piedade GS, Rizelio V, Scheffer BE, Merida KLB,
Takeshita BT, Kowacs PA
• INSTITUTO DE NEUROLOGIA DE CURITIBA, UFPR
Background: Elevation of coagulation factor
VIII has been described in patients with venous
thromboembolism, and, since then, its high
levels were proved to be associated with both
arterial and venous thrombotic events. The relationship between factor VIII and cerebrovascular
disease has been investigated, and factor VIII has
shown to be an independent risk factor related
to the severity, early outcomes and recurrence
risk of events such as acute ischemic stroke and
cerebral venous thrombosis. Objective: To report
and review five cases of acute ischemic stroke
and one case of cerebral venous thrombosis, all
of them with elevated factor VIII as an important
risk factor. Method: Factor VIII levels were examined in patients with thrombotic cerebrovascular
events presenting to our hospital between 2011
and 2014. Laboratory tests were performed in
young patients, in case of recurrence and when
there was no conventional risk factors. We identified six patients from overall 479 cases. Results:
Mean level of factor VIII among all the patients
was 184%, exceeding the laboratory reference
range (<150%), all confirmed with second exam.
The group had a few risk factors, with one smoker and only two patients with dyslipidemia.
Mean age in stroke group was 45,6 years. One
young patient had patent foramen oval (previous
undergone percutaneous close). One young patient, had ischemic stroke with no identified risk
factor, and previous postoperative deep venous
thrombosis history, not currently in antithrombotic therapy. High factor VIII level accompanied
C protein elevation in one stroke case, and in the
case of cerebral venous thrombosis. One patient
had pulmonary embolism some months after
stroke. Conclusion: Excess factor VIII can be a
cause of cerebrovascular disease when there is
no other or few identified risk factors, mainly in
young patients. It is associated with a heightened
risk for ischemic stroke and cerebral venous
thrombosis, and elevated factor VIII levels could
predict a patient in risk of recurrent systemic
thrombotic events.
P-112
FIFTEEN CASES OF CEREBRAL AUTOSSOMAL
DOMINANT ARTERIOPATHY WITH SUBCORTICAL
INFARCTS AND LEUKOENCEPHALOPATHY
(CADASIL) IN A FAMILY: CASE REPORT
Carneiro FG, Siqueira PB, Feitosa AFC, Assunção CDM,
Witt MCZ, Truppel YM
• HCV-PR
Introduction: Cadasil is a generalised small
vessels disease, ocurring because of a mutation
on the Notch 3 gene, situated on the chromossome 19 (19p13.1), causing ischemic stroke in
Young adults, vascular dementia, migraine with
aura and mood disorders. The most common
manifestation is the subcortical lacunar stroke.
On most cases, there are none of the frequent
cardiovascular risk factors. As the second most
common manifestation, we have cognitive deficit. Based on clinical manifestation and magnetic ressonance imaging (MRI) and family history,
one can estabilish the Cadasil diagnosis. Studies
of chromossome should confirm the mutation of
Notch3. Objective: To report a case of Cadasil in
a specific patient with other fifteen cases on her
Family, and to compare it with available bibliography. Case report: Female, 60 years old, complaining of migraine with over 30 years of onset,
combined with memory loss starting two years
ago. She denied previous neurological deficits.
Family inquiry reveals fifteen family members
with clinical manifestation suggestive of Cadasil,
of which nine have MRI findings correlated with
the disease and six of them refused to investigate.
On physical examination, the patient presented
with generalised symmetrical hyperreflexia, no
clonus. Strength, tonus, coordination and sensibility were preserved. Her MRI revealed multiple
alteration of signal captation on White matter of
both cerebral hemispheres, also compromising
the tip of both temporal lobes and pons, with
small lacunar previous lesions bilaterally on the
White matter. These findings are suggestive with
chronic ischemia that might be related to central nervous system vasculitis, compatible with
the clinical hypothesis of Cadasil. Currently, the
patient is using 100mg of acetylsalicylic acid
per day. Discussion: The most common cause
of stroke and dementia in adults is Cadasil, and
frequently around 49 years of age. Hence, this diagnostic should be of high clinical suspicion and
investigated, with special attention for patients
with migraine with aura and Family history of
Young adults suffering strokes. Unfortunately,
making the diagnosis will not change the disease
progression of dementia and repeated lacunar
strokes. There is no specific treatment available.
P-113
FUNCTIONAL OUTCOME IN STROKE UNIT: A
COMPARATIVE STUDY BETWEEN PATIENTS WITH
AND WITHOUT ATRIAL FIBRILLATION
Maso I1, Jesus PAP1, Monteiro M2, Silva FL2, Mendel T2,
Muniz L1, Almeida J1, Gois JA1, Cruz J1, Pinto EB2,3
• 1STROKE UNIT OF THE ROBERTO SANTOS GENERAL
HOSPITAL; 2BAHIAN SCHOOL OF MEDICINE AND
PUBLIC HEALTH; 3STROKE CLINIC OF THE FEDERAL
UNIVERSITY OF BAHIA
Background: Atrial fibrillation (AF) is an important risk factor for the development of ischemic
stroke and the presence of this comorbidity
may affect the individual‘s recovery. The association between AF and functional outcome after
stroke as reported in some studies is still unclear.
Objectives: To assess the association between the
presence of atrial fibrillation and the short-term
functional outcome after stroke. Methods: This
is a retrospective study with secondary data
from patients hospitalized in stroke unit in the
city of Salvador, Bahia. Demographic and clinical data were collected and the following scales
were applied: National Institute of Health stroke
Scale (NIHSS) at admission to identify the severity of stroke and modified Rankin Scale (mRS) at
discharge to assess the functional outcome. Patients with AF were compared to those without
AF. Initially was performed univariate analysis
(t Student, Mann-Whitney and Chi square test),
then multivariate analysis was carried out using
a logistic regression model in order to identify
predictors of outcome functional at discharge,
and mRS ≤2 was defined as good functional
outcome. Results: Between May 2013 and April
2014, 530 patients were admitted in the unit, 179
were excluded from this study because they had
no confirmed diagnosis of stroke or had incomplete data. The average age of study participants
was 64 years (±15), 52% were female, 93% had
ischemic stroke and 13% were thrombolized.
The median of length of stay was 8 days (range
5‐11), of mRS at discharge was 4 (range 2‐5) and
stroke severity measured by NIHSS was 11 points
(range 7‐17). Hypertension was present in 66% of
patients, 27% had diabetes mellitus, 8% had AF
and 17% other heart disease. Observed smoking
history in 9% of patients and alcohol consumption by 5%. When comparing the two groups
was observed that patients with AF had higher
age (p=0.018), higher NIHSS score at admission
(p=0.001) and higher mRS at discharge (p=0.017).
However, there were no significant differences
between groups when considering the number
of patients with mRS ≤2 at discharge (p=0,065).
A multiple linear regression analysis showed that
only age (p=0.008) and NIHSS (p<0.001) emerged
as predictors of functional outcome. Conclusion:
Despite the presence of AF is associated with
more severe stroke, expressed by worse NIHSS
score on admission, this condition alone does
not seem to interfere with short-term functional
outcome in patients after stroke.
P-114
FUSIFORM COMPLEX ANEURYSM OF INTERNAL
CAROTID ARTERY SECONDARY TO ATRIAL MYXOMA
Assis RM, Ruschel LG, Suzano FL, Meneses MS,
Rizelio V, Kowacs PA
• INC
Background: Patients with atrial myxoma have
a variety of neurological syndromes, even in the
absence of cardiac symptoms and may occur
even after complete and successful surgical removal of the tumor. Cardioembolic stroke is well
described, however other neurological complications, such as brain aneurysms, are unusual,
with about 50 cases reported in the literature
since the 1960s. These aneurysms are multiple,
and predominantly fusiform but also saccular
aneurysms. Objective: To describe a case of complex fusiform aneurysm of left middle cerebral
artery with a late diagnosis in a patient with a
history of cardiac myxoma and ischemic stroke.
Case report: Female patient, 25 years old, with a
history of ischemic stroke in the left middle cerebral artery (MCA) caused by left atrial myxoma,
surgically removed after three months. Brain
Magnetic Resonance Imaging (MRI) showed ectasia of distal left internal carotid artery (ICA),
A1 segment of anterior cerebral artery (ACA) and
M1 segment of middle cerebral artery (MCA) at
left. After 5 years a new MRI showed fusiform
aneurysm of choroidal segment of left ICA, A1
71
Posters
segment of ACA, M1 segment of MCA and at origin of left posterior communicating artery, and
also a saccular aneurysm of left MCA. All finds
were confirmed by digital subtraction angiography. In a further MRI, after 7 years, there was a
small growth of the saccular aneurysm (5.5mm
dome, 3mm deep). The patient was not submitted to surgical or interventional treatment.
Discussion: Patients with atrial myxoma may
present late fusiform aneurysms in brain vessels.
Previous studies hypothesize that tumor microemboli infiltrate the vascular wall, causing their
fragility and the development of fusiform and
saccular aneurysms. Due to high complexity, the
surgical treatment or intervention of those aneurysms are at high risk for complications.
P-115
GET WITH THE GUIDELINES STROKE
PERFORMANCE INDICATORS IN PATIENTS WITH
TRANSIENT ISCHEMIC ATTACK
Massaud RM1, Miranda RCAN1, Vaccari AMH1,
Figueiredo MM2, Cendoroglo Neto M1, Silva GS1
• 1HIAE; 2UFRGN
Introduction: Get With The Guidelines Stroke
(GWTG-S) is an in-hospital program for improving stroke care by promoting adherence to scientific guidelines. Patients with transient ischemic
attack (TIA) have a high risk of stroke, with around
15% of patients having a stroke within 3 months.
Therefore adherence to guidelines is crucial for
patients with TIA as well. The goal of our study
was to evaluate adherence to stroke treatment
quality indicators in patients with TIA. Methods:
We evaluated a database of consecutive patients
admitted with acute ischemic stroke (AIS) or TIA
from Aug 2008 to Dec 2013 to a Brazilian tertiary
hospital. We compared the GWTG-S treatment
quality indicators applicable to both AIS and
TIA: antithrombotic medication use within 48h
of admission; discharge use of antithrombotics
and of anticoagulation for atrial fibrillation (AF);
treatment for LDL 100 mg/dL, and counseling for
smoking cessation. Results: A total of 357 consecutive patients with TIA and 787 patients with
AIS stroke were evaluated. The median ABCD2
score in patients with TIA was 3 [2,4]. Patients
with TIA were younger (66.1±15.7 vs 71.8±16.1
yo, p<0.01), and had a lower frequency of previous stroke (16.9% vs 25.8%, p<0.01) than patients
with AIS. Antithrombotic medication use within
48 h of admission (94.4% in patients with TIA vs
92.7% in patients with AIS, p=0.1) and discharge
use of antithrombotics (95% in patients with
TIA vs 97.9% in patients with AIS, p=0.05) were
similar between groups. Patients with TIA were
more frequently treated with anticoagulation
for AF (79.5% versus 75%, p<0.01). Conversely,
lipid lowering treatment (57.7% in patients with
TIA versus 63.7% in patients with AIS, p<0.01)
and counseling for smoking cessation (57.9% in
patients with TIA versus 81.6% in patients with
AIS, p=0.04) were more frequently performed
in patients with AIS. Conclusions: Adherence
rates to the GWTG stroke quality indicators lipid
lowering treatment and counseling for smoking
cessation were worse in patients with TIA than in
patients with AIS, therefore measures should be
undertaken to reinforce the importance of such
quality indicators in patients with TIA.
72
P-116
GIANT CELL ARTERITIS WITH CORONARY
COMPLICATIONS
Carneiro MAD, Diniz DS, Ribeiro TAGJ, Teixeira KS,
Monteiro PPB, Ferreira GC, Divino NP, Fraga DS
• HC
Introduction: Giant cell arteritis (GCA) is a systemic immune-mediated chronic granulomatous vasculitis affecting arteries of large and
medium caliber. The most affected population
include the north european females over 50
years. The main symptom is headache, usually
temporal, but may have other locations and temporal artery biopsy remains the gold standard for
diagnosis. Vision loss for anterior ischemic optic
neuropathy, strokes and aortic aneurisms are the
most commons complications. The GCA related
deaths are rare, often attributed to insufficient
treatment. Objective: To report a case of giant cell
arteritis with coronary artery involvement likely
that even after the pulse scheme evolved into
sudden death. Case report: A 81 year-old male
patient, natural of Cardoso/MG, former telegraph operator sought the neurology service of
HC/UFG for headache and behavioral changes.
One year ago started with recurrent frontotemporal headaches of moderate or strong intensity accompanied by verbal aggression, change
in the pattern of family life, loss of appetite and
worsened after 9 months with visual hallucinations. One month ago presented with progressive
vision loss and bilateral temporal artery tenderness to palpation. Evidence of altered inflammatory activity was present (VHS =78 mm/h) and
cranial MRI showed thickening of the extracranial arteries. Suspicion of GCA, the temporal artery
biopsy was performed and pulse therapy with
methylprednisolone prescribed for 5 days, with
a significant improvement of cognition mainly,
but not full of visual symptoms of the patient. After two days of finished pulsetherapy, the patient
evolved to cardiopulmonary arrest and sudden
death. The body was sent to necropsy, which
proved a coronary infart. The artery biopsy confirmed the diagnose of GCA. Discussion: The diagnostic criteria of GCA supported by American
College of Rheumatology are: 1) Age >50 years
2) New onset cephalalgia 3) Temporal artery abnormalities 4) VHS >50 mm/h 5) Altered arterial
biopsy. The patient presented with all criteria for
the disease and realized treatment in accordance
with the current practice, but evoveld to death.
Although coronary is not a commonly affected
artery, there are reported cases of acute myocardial infarction by GCA, even under pulse therapy.
treated conservatively with antiplatelet therapy.
Case report: A man, now aged 58, has had four
episodes of acute ischemic vascular injury, all
in the same territory, the right cerebral hemisphere, the first one in 1989. During etiological
research magnetic resonance angiography of
intracranial and neck vessels revealed a expanding tumor measuring 3.6 x 2.6cm located in the
jugular fossa. The arteriography confirmed it to
be a pseudoaneurysm of the right carotid artery
of unknown cause. The patient was treated with
antiplatelet therapy since 2006 and no longer appealed symptoms presenting minimal disability
(modified Rankim scale 1). Discussion: The surgical and endovascular approach is traditionally
recommended for the treatment of pseudoaneurysms. We describe a successful case, with long
follow-up period, in which we employed noninvasive conservative therapy with antiplatelet
therapy.
P-118
HEAD POSITION IN STROKE TRIAL (HEADPOST)
Background: There is insufficient evidence to
recommend a specific head position in patients
with either acute ischemic stroke (AIS) or acute
intracerebral hemorrhage (ICH). Observational data indicates potential beneficial effects of
the lying flat position in AIS, and conversely the
sitting up head position in patients with ICH.
Any potential benefits may be offset by an increased risk of aspiration pneumonia, cardiac
failure or increased intracranial pressure. Aims:
To compare the effects of lying flat (0°) head position with sitting up (≥30°) applied in the first
24 hours of admission, for patients presenting
with AIS, on poor outcome (death or disability)
at 90 days. Key secondary: To determine whether
lying flat is superior to sitting up on poor outcome at 7 days in AIS and whether sitting up is
superior to lying flat on these outcomes in acute
ICH. Methods: and patients: A multicentre, prospective, cluster randomised, crossover, blinded outcome assessment study through a global
network of investigators in Australia, Europe and
South America. Eligibility criteria will evaluate
the treatment effect in a broad range of patients
with AIS and ICH. Conclusions: Given the uncertainty over the balance of benefits/risks, and
variability regarding the ideal head position for
these patients around the world, reliable randomized evidence is required to standardize
clinical practice recommendations.
P-119
P-117
GIANT PSEUDOANEURYSM OF THE EXTRACRANIAL
CAROTID ARTERY CONSERVATIVELY TREATED.
CASE REPORT OF A BENIGN OUTCOME AT 25 YEAR
FOLLOW-UP
HEMODYNAMIC CEREBRAL STROKE IN A FOUR
YEAR OLD CHILD WITH MOYAMOYA DISEASE CASE REPORT
Limonte FH1, Borges APP1, Andrade RS1, Tanaka MU1,
Vasconcelos RC1, Paula GR2, Ferreira KS1, Borges MA1
Pitta IJR, Marangon LM, Miranda IRP, Oliveira LC,
Pereira CA, Henriques KMC, Lannes EA, Freitas GR
• 1FAMERP; 2FACERES
• UFF
Introduction: Moyamoya disease (MMD) is a
progressive vessel-occlusive disorder of unknown etiology. The angiographic findings are
characterized by progressive bilateral stenosis
of the terminal portions of the internal carotid
arteries, associated with the proliferation of collateral vessels at the base of the skull in smoke
format (Moyamoya vessels). A critical stenosis of
the carotid arteries may be associated with hemodynamic cerebral stroke at nearby cerebral
vascular territories areas also called watershed
areas. Objective: This study reports the case of a
Brazilian, Hispanic, not a descendant of Japanese
Introduction: The carotid artery pseudoaneurysm is an uncommon entity that mainly occurs
after traumatic or spontaneous dissections. Due
to the morbidity and mortality attributed to rupture of a pseudoaneurysm of extracranial carotid
and its embolic potential, surgical or endovascular intervention is traditionally advocated,
with little evidence for conservative treatment.
Objective: We report the case of a giant pseudoaneurysm of the extracranial carotid artery,
with 25 years of follow-up and a benign outcome
XXVI Congresso Brasileiro de Neurologia
children with rapidly progressive cerebral vascular disease, and late diagnosis of Moyamoya
Disease. Case report: SFL, 4 years old, Hispanic, born and raised in Fernadopolis-SP, Brazil,
admitted in Hospital de Base, São José do Rio
Preto in April 2012. The mother relates that the
child started a week ago insidious symptoms of
weakness in the right arm and right leg, noticed
when the child picked up objects and walking. It
progressed with increasing weakness of the right
side of the body, malaise, difficulty swallowing
and aphasia in the next day. Previously history:
2 months ago the child had limb weakness lasting 2 minutes with spontaneous resolution and
events described as repeated periods of reduced
attention. On neurologic examination, the child
had aphasia, dysphagia for liquids and disproportionate right hemiparesis (grade II MSD and
grade III MID). Magnetic resonance images (MR)
was performed in emergency showed many areas of acute ischemia in watershed areas of both
hemispheres. Brain vassel image study with MR
and conventional cerebral angiography showed
a pattern of Moyamoya. Among the laboratory
tests ordered on admission, it identified rheumatoid factor in low titers and absence hematologic disease. EEG pathological presenting
asymmetrical, disorganized diffuse bilateral
slowing, predominantly on the left and right
attenuation. Without identifying any known pathology associated with Moyamoya syndrome,
the patient was diagnosed with Moyamoya disease. Discussion: Once the frequency of DMM in
our country is low, it is necessary to high suspicion of this disease in children with symptoms of
transient brain ischemia in order to make a quick
diagnosis. In addition to MR, the role of conventional cerebral angiography has important in the
diagnosis of DMM.
P-120
HYPERTENSIVE EMERGENCY: WOULD YOU THINK
IT TO BE A CEREBRAL AMILOID ANGIOPATHYRELATED INFLAMMATION?
Martinello TD1, Betz RL1, Boehringer AK2, Barbosa JS2,
Mousquer AL1, Tiellet NL1, Quessada AF1, Fiquene JC1,
Siquineli F1, Oliveira JF1
• HOSPITAL SANTA ISABEL DE BLUMENAU; FURB
1
2
Background: Cerebral amiloid angiopathy
(CAA) is defined by the progressive deposition
of β-amiloid protein, most commonly within the
small and medium cortical and leptomeningeal
vessels. The main clinical presentations are intracranial hemorrhages, micro bleedings, cerebral
ischemia and cognitive decline. More often, in
CAA-related inflammation, the patient presents
with cognitive decline, seizures, headache and
stroke like symptoms which resolves with corticosteroid therapy. Objective: Single Case report
of a CAA-related Inflammation presented as a
hypertensive emergency. Case report: A 67 years
old male was admitted at the Emergency Room
with headache, hypertension and progression to
disorientation in space and time. A cranial computadorized tomography scan documented a
right parietal lesion associated to vasogenic edema that confirmed a diagnosis of CAA-related Inflammation by a brain Magnetic Ressonance Imaging and which responded successfully to corticosteroid treatment. Conclusion: Appropriate
identification, evaluation, and treatment of the
hypertensive emergency are the mainstay in the
emergency department to prevent progression of
organ damage and death. Therefore, the neurological examination and imaging was crucial to
the correct diagnosis and successful treatment
in this case which presented with features that
raised de suspicion of a neurological disorder.
P-121
HYPOGLOSSAL NERVE PALSY AFTER RIGHT
INTERNAL CAROTID ENDARTERECTOMY
Corrêa TD, Martins WRM, Castro JPS, Franco IA,
Vale TC, Pires LA
• UFJF
Introduction: Carotid endarterectomy has been
established as the standard of care for stroke
prevention in patients with severe carotid stenosis in an average risk population. Hypoglossal
nerve injury is the most frequent postoperative
complication. The nerve crosses over the internal carotid artery (ICA), where it is deeply seated
beneath and together with the internal jugular
vein. It then passes forward between the vein
and artery and crosses the carotid bifurcation.
Studies demonstrate different incidence of nerve
injuries, being the hypoglossal nerve the most
frequently injured (1 to 17%, due to the proximity of the bifurcation) followed by the recurrent
laryngeal nerve (1 to 8%). Several predictors
are associated with the increased risk of neural
injury in carotid endarterectomy, such as emergency procedures, re-exploration during surgery
and re-operation, especially in cases of trauma.
However, none of them are specific to the hypoglossal nerve. Objectives: Report a case of hypoglossal neuropraxia after endarterectomy of the
right ICA. Case report: An 80-year-old male patient, hypertensive, with a history of a transient
ischemic attack (TIA) characterized by left-sided hemiparesis lasting 20 minutes, underwent
carotid endarterectomy due to severe stenosis
(above 90%) of the right ICA. In the immediate
postoperative period, the patient developed
tongue deviation to the right and could not properly move the bolus within the oral cavity. Neurologic examination identified deviation of tongue
on its protrusion, preserved pharyngeal reflex
bilaterally and neither deviation of the uvula nor
facial changes. Physiotherapy and speech therapy were started and the patient recovered completely in an eight-month follow-up consultation. Discussion: The majority hypoglossal nerve
lesions are benign and transient, with recovery of
function occurring between one and six months.
Direct injury or nerve section is rare, and an average of 0.7% of patients have persistent lesions.
Dysfunction is due to edema generated by trauma on account of retraction and dissection of the
nerve. Manipulation and cauterization of small
vessels around may also contribute to the lesion.
P-122
IDENTIFYING COPYNUMBERVARIATIONS WHICH
ARE RELATED TO PATHOGENESIS IN PATIENTS
WITH HEMORRHAGIC STROKE
Donatti A1, Secolin R1, Ferreira LE2, Torres FR1,
França PH2, Nagel V2, Cabral NL2, Lopes-Cendes I1
• 1UNICAMP; 2UNIVILLE
Introduction: : Stroke is the third most common
cause of death or disability worldwide. Hemorrhagic stroke (HS) occurs in 15%‐20% of patients
with stroke and it is characterized by the rupture
in cerebral blood vessels. There is evidence that
genetic factors play a role in determining HS.
However, this is the first study to systematically
search for copy number variations (CNVs) as the
basis for the genetic predisposition to HS. CNVs
are one of the most common types of mutation
occurring in the human genome and they have
being shown to cause significant changes in
gene expression due to variation in the number
of gene copies present or disruption in gene(s)
sequences. Objective: To analyze the presence
of CNVs in patients with HS. Methodology: We
analyzed DNA sample obtained from peripheral blood of 23 patients with HS, as well as 23
controls (individuals without stroke). Patients
were further classified based on the location
of ruptured blood vessels within the brain in
4 lobar, 18 nonlobar and 1 without classification. All samples were obtained from the Joinvile Stroke Biobank stablished at the UNIVILLE
University as part of a population-based epidemiologic study. CNVs were analyzed using the
Genome-Wide Human SNP 6.0 DNA chips (Affymetrix Inc.), which exams CNVs present in the
entire human genome. CNV data were estimated by Bayesian Robust Linear Model using Mahalanobis(BRLMM) andCanaryalgorithms in the
Genotype Console®Software(Affymetrix Inc.). In
addition, a principal component analysis (PCA)
was performed using the R software in order to
evaluate population stratification in the sample.
To evaluate possible interaction among genes
found to contain CNVs, a gene network was built
using METACORE TM software. Results: We identified a total of 162 CNVs among patientswith HS,
including 76 losses and 86 gains of copies. Within
the CNVs found we identified 43 genes which are
located within the deleted regionsand 63 genes
within the duplicated regions. According to
gene network analysis, three of these genes were
foundto be functionally related to blood pressure regulation, DEFB103B, DEFB4Band PRODH
genes. In addition, PCA analysis showedthat the
total sample did not present population stratification, which allows for unbiased comparisons
between patients and controls. Conclusion: Our
study identified CNVs in genes which are functionally related to regulation in blood pressure in
seven patients, representing 30% of patients with
HS studied.
P-124
INITIAL CARE AND ADVANCED SUPPORT TO
HEMORRHAGIC STROKE (HS)
Franco AC, Nogueira AC, Almeida CHR, Martins G,
Teles LO, Fernandes Junior WS, Almeida Filho CAA,
Pereira DS
• UNIC
Introduction: The HS is among the three leading
causes of death in industrialized countries, being
responsible for high rates of morbidity. Initial
care is essential to decrease morbidity / mortality in patients with HS. Objectives: Describe
and evaluate the management of the initial care
of HS. Case report: Patient, 50 years, chronic
hypertensive, admitted to the emergency department with epistaxis, vomiting and intense
headache. On physical examination the patient
was hypertensive (BP: 190x90 mmHg), drowsy,
decreased level of consciousness, Glasgow 13,
with left hemiplegia, labial deviation to the left
and dysphasia. Computed Tomography (CT)
image showed capsular hemorrhagic core at
right, associated with edema and hypodense
halo and hematic content within the ventricular
system. Volume of 8,7ml with midline deviation
of 0.55mm. The patient was transferred to the
ICU where he received supportive treatment by
proper monitoring of mean arterial pressure;
glycemic control, temperature, hidroelectrolytes,
CVP, head elevation of 30 degrees, water balance
and diuresis. After 5 days, an external ventricular
73
Posters
drainage (EVD) was installed in order to drain
the hematoma, and the intracranial pressure
(ICP) was monitored (<20 mm Hg), maintaining the cerebral perfusion pressure (CPP) higher than 60 mmHg. The EVD was removed after 9
days, due decreased cerebrospinal fluid drainage
and hemorrhagic volume (about 2ml). During
the hospitalization the patient developed severe
sepsis, renal disfunction, requiring hemodialysis. After 83 days of hospitalization, the patient
was discharged from the ICU, hemodynamically
stable due of antihypertensive drugs. The patient
remained hemiplegic on the left side, dysarthric,
but with improved communication. Discussion:
The diagnosis of HS should be quick and the
assistance aggressive. Initially, an advanced life
support must be instituted (MOV, ABDC), neurological assessment is required by using recognized scales (GLASGOW, NIHSS), neuro-check
every 30 minutes, MBP (100‐130mmHg), ICP
(<20mmHg) , CPP(>60 mmHg); in case of ICH is
allow to use mannitol (1‐2 g / kg) at 20% or a 7%
hypertonic saline solution (3‐4ml / kg, 1 hour); CT
up to 25 minutes from the entrance. Analysis of
CT by a radiologist or a experienced specialist in
the field of neurology, neuroimaging of stroke in
45 minutes. ICP monitoring when necessary, surgical removal of clot and located craniectomy in
selected cases.
P-125
IODINATED CONTRAST-INDUCED
ENCEPHALOPATHY: CASE REPORT
Ribas GC, Germiniani FMB, Tansini G, Ikuta CS,
Roginski A, Zetola VF, Lange MC, Figueiredo LR
• UFPR
Introduction: Cardiac catheterization has an
extremely importance on the evaluation of ischemic coronary disease, but it is not risk-free.
Complication rates are low and may vary according to the applied technique. Major complication
include cardiac arrhythmia, arterial punction related complications, myocardial infarction, coronary dissection or other vases, hemodynamic
collapse, stroke/transient ischemic attack, intramyocardial contrast injection and anaphylaxis. Neurologic complications are less common,
but have the potential to lead to great morbidity
and mortality. Contrast neurotoxicity is an even
rarer neurologic complication, whose clinical
presentation includes encephalopathy, seizures,
cortical blindness and focal neurological deficits
such as ophthalmoplegia. Objective: To report
the case of a possible iodinated contrast-induced
toxic encephalopathy. Case report: A 90 years old
female patient with past history of arterial hypertension, diabetes mellitus type II, chronic kidney
disease and chronic atrial fibrillation, presented
with chest pain suggestive of acute myocardial infarction, which prompted an emergency
cardiac catheterization. The procedure was in
itself uneventful, without complications. However, 15 minutes after the end of the procedure
the patient suddenly became comatose and had
to orotracheal intubation and mechanical ventilation. A brain MRI was normal, without any
signs of acute ischemia. An emergency cerebral
angiography was also normal, with no signs of
intracranial vascular occlusion. The patient had
a progressive and spontaneous improvement
of consciousness and could be safely extubated
within 6 hours. Neurological examination was
unremarkable, with no focal signs that could
suggest an underlying stroke. One week later the
patient had a probable episode of post-syncope
seizure caused by arrhythmia. A new brain MRI
74
was once again normal and an EEG demonstrated rare signs of epileptiform activity over the left
anterior temporal region, but was considered to
have low epileptogenicity. Discussion: Neurological complications of cardiac catheterization
are rare, but potentially severe. Neurotoxicity induced by the iodinated contrast, especially in elderly patients with renal dysfunction, is an even
rarer complication. Diagnosis is made based on
clinical findings and after other causes have been
excluded, such as stroke.
P-126
IS THROMBOLYSIS SAFE IN SPONTANEOUS
INTRACRANIAL DISSECTION?
Kuster GW, Brasil IP, Toso FF, Pacheco EP, Miranda VA,
Pieruccetti MA, Volcov C, Dutra LA
• 1HOSPITAL PAULISTANO
Introduction: Spontaneous intracranial dissection (SID) is a rare and often unrecognized
cause of stroke and subarachnoid haemorrhage
(SAH), especially in young adults. IAD represents
10% of spontaneous cervicocephalic artery dissections and 3% to 5% of SAHs. Oral contraceptives, migraine, hypertension, recent infection,
Ehler-Danlos syndrome and fibromuscular displasia are considered as predisposing factors.
Management of IAD is controversial because
randomized controlled trials are lacking, especially on thrombolysis safety. Objective: To report five cases of IAD from Hospital Paulistano
Stroke Center Results: Mean age at presentation
was 47,7±21,89 years; mean NIH at presentation
was 9,8±7,80; two patients presented hypertension, one had dislipidemia. Four out of five patients were male and two had asian ancestry.
The only female patient presented migraine, was
receiveing oral contraceptives and presented
dissection of the posterior cerebral artery. The
remaining patients presented basilar dissections.
Most common involved regions were pons, cerebellum, mesencephalon, tallamus and occipital cotex. One patient presented SAH unrelated
to thrombolysis. Three patients (60%) received
thrombolysis, two intravenous and one intrarterial thrombolysis. All patients that received
thrombolysis were discharged with mRs ≤3 and
none of them presented bleeding complications.
One patient died, three were discharged home
with mRs =1. Most patients were managed with
antiagregation. NIH at discharge was 7,6±9,39.
Conclusion: This is the first Brazilian IAD series.
We found that the posterior vascular territory
was the most common affected, data in line with
previous studies. In our population, at least one
of the predisposing factors were found: asian
ancestry, oral contraceptives, migraine and hypertension. We provide evidence that thrombolysis in IAD is safe and patients present favorable
functional outcome.
P-127
ISCHEMIC STROKE IN A PATIENT WITH DIFFUSE
SYSTEMIC SCLEROSIS: CASE REPORT
Gois LCP, Melo ACP, Saba RA, Marques AC,
Marques LVD
• HOSPITAL DO SERVIDOR PÚBLICO ESTADUAL DE SÃO
PAULO
Introduction: Systemic sclerosis (SS) is an autoimmune disease characterized by widespread
microvascular, vascular lesions and increased
deposition of connective tissue in the skin and
internal organs. However, the central nervous
system is rarely affected. Recent evidence has
demonstrated increased atherosclerosis, calcification in cerebral arteries and high prevalence
of coronary artery disease, suggesting increased
overall cardiovascular risk. Thus, SS appears as
an independent risk factor for ischemic events,
and indirectly by increasing the prevalence of
associated factors such as hypertension and
atherosclerosis. Objectives: To conduct a brief
review of SNC manifestations in SS and report a
case of ischemic stroke in a patient with diffuse
systemic sclerosis. Case report: Woman, 63, diagnosed with diffuse systemic sclerosis, showing
sudden change in consciousness levels and loss
of strength in the left hemisphere. During a neurological examination we observed complete left
hemiplegia, with pyramidal liberation signs and
disarthria. During the investigation, an angiography was performed by MRI which showed occlusion of the M1 middle cerebral artery. The image
is compatible with ischemic vascular accident in
the right capsular core region, with hemorrhagic
transformation. After etiological investigation,
cardioembolic causes and arterioarteriais embolisms were discarded. She evolved with partial
improvement of motor deficit during hospitalization. Discussion: The mechanisms involved
in the pathophysiology of neurological manifestations of systemic sclerosis are associated with
chronic inflammation, structural and functional
changes of vascular beds due to fibrosis and atherosclerosis more pronounced in these patients.
Another important change is the calcification
of small vessels, which can be seen on imaging.
Thus, recognizing the systemic sclerosis as an
independent risk factor for ischemic cerebral
events is of paramount importance, since it
makes the control of modifiable risk factors in
this population even more necessary.
P-128
ISCHEMIC STROKE IN A PATIENT WITH PATENT
FORAMEN OVALE ASSOCIATED WITH TAMOXIFEN
TREATMENT: CASE REPORT
Gois LCP, Melo ACP, Silva SMCA, Silva GRO, Nakano PT
• HOSPITAL DO SERVIDOR PÚBLICO ESTADUAL DE SÃO
PAULO
Introduction: Patients with cryptogenic arterial
ischemic stroke have a high prevalence of a patent foramen ovale (PFO). However, the simple
existence of a right-to-left shunt (RLS) does not
appear sufficient to explain the increased risk
of ischemic stroke, and other significant factors
must be required. A hypercoagulable state (the
uses of Tamoxifeno and cancer, for example)
could be one of these factors, because it increases thrombogenic mechanisms, which is related
to paradoxical embolism. Objective: This report
aims to present a case of ischemic stroke associated with PFO in a patient with breast cancer
that uses tamoxifen , both risk factors for thrombosis. Case: A 57-years-old woman was admitted
to the emergency unit of Hospital do Servidor
Público Estadual de São Paulo with left hemiparesis, dysarthria, abnormal balance, vertigo,
nausea and vomiting of 9 hours’duration. She
had an invasive ductal carcinoma with lung metastasis, using tamoxifen (40mg daily) for 4 years.
No cardiovascular risk factor was present.The
Magnetic Resonance Imaging of head showed
subacute ischemic lesions in the left cerebellar
hemisphere and CT angiography of the head and
neck showed no abnormalities. The transesophageal echocardiogram showed a patent foramen
ovale and RLS. The tamoxifen was suspended
and oral anticoagulant therapy established. She
developed improvement of symptoms in 48
XXVI Congresso Brasileiro de Neurologia
hours, remaining a slight change of balance. She
was discharged with surgical PFO programming
approach. Discussion: Paradoxical embolism
through a PFO should be strongly considered
in young patients with cryptogenic stroke but
a hypercoagulable state must be investigated.
Tamoxifen may increase ischemic stroke risk by
promoting thrombosis, particularly in women
who may already be hypercoagulable because
of breast cancer. As a result of its prothrombotic properties, tamoxifen may be associated
with paradoxical embolism and cerebral venous
thrombosis. Paradoxical embolism via a patent
foramen ovale in the setting of venous thrombosis could explain some of the cases of ischemic
stroke with tamoxifen. We emphasize that this
increase in risk of stroke does not mean that use
of tamoxifen should be reduced, given the drug‘s
considerable benefits. Rather, it means that
women should be screened for possible stroke
risk before the drug is prescribed.
P-129
ISCHEMIC STROKE IN YOUNG PATIENTS:
ETIOLOGICAL INVESTIGATION
Santos LTL1, Souza MES1, Sousa GG1, Torres LA1,
Barbosa MU1, Lopes BRC1, Santos IPRN1, Magalhães IN2
• 1FACID - DEVRY; 2HUT
Introduction: Strokes are among the leading
causes of mortality in Brazil. About 85% of strokes
are ischemic. Its incidence increases exponentially with age, therefore is lower in young adults,
whose causes are more diverse than in the older
population, requiring further evaluation. Contemporary neuroimaging and specific tests for
diagnosing other rare causes of stroke in this age
group has allowed the identification of the most
frequent causes. Objective: To report a case of
strokes in young adults highlighting its evolution
and diagnostic investigation. Case report: Male
patient, 34 years old, history of sudden onset
left sided weakness, dysarthria and deviation of
the contralateral labial commissure. Admitted in
referral hospital about 9 hours after onset, with
elevated blood pressure, disproportionate hemiparesis ipsilateral Babinski sign and dysarthria
with 6 points in NIHSS. Admitted to ICU about
50 hours after the stroke, with GCS 15, disproportionate left hemiparesis, without aphasia, moderate dysarthria and left sided tactile extinction
with 5 points in NIHSS 5, perfoned conservative
aproach. Remained four days in ICU with slight
improvements on limb strength and extinction.
Forwarded to the ward with NIHSS4. Discharged
presenting left upper limb paresis and mild dysarthria with2 points in NIHSS, with prescription
for aspirin,and simvastatin. Requested etiology
of stroke with CT angiography of cervical and
intracranial arteries, transthoracic echocardiography, ECG, and research for vasculitis, thrombophilia, syphilis, Chagas disease and HIV. The
transesophageal echocardiography showed an
atrial septal defect (ASD) of 6 mm and microbubble constrat-enhanced TCD with identified
the curtain effect. From these findings anticoagulation was initiated. Discussion: Currently, on
the treatment of a young adult with suspected
stroke, the diagnostic challenge is to identify its
cause. In the case described, the presence of the
ASD is directly related to the ischemic episode,
therefore its etiology is cardioembolism. Despite improvements in diagnosis and treatment,
stroke in this age group remains a catastrophic
event, its course can cause death, disability, and
hamper the quality of life of patients.
P-130
ISQUEMIC STROKE IN CHILDHOOD
Silva RR1, Oliveira AB2, Hamad APA1, Oliveira RP1,
Funayama CAR1, Cruz MN1, Pontes Neto OM1
• 1USP RP; 2HOSPITAL FEDERAL DA LAGOA
Introduction: The incidence of childhood stroke
is 2‐13/100.000 according to European and North
American data. Causes, symptoms and outcome of stroke in children are different from
those in adults, and, furthermore, since prenatally to 18 years of age. Objectives: To identify
main causes, analyze associated features, clinical presentation, follow-up and disclosures.
Material and Methods: A retrospective study
based on identification of children and adolescents, with age ranging from 2 months to 17
years, with cerebral infarction confirmed by
brain image, admitted at our service, from 1996
to 1999. Charts of these patients were reviewed.
Children with incomplete or inconclusive data
records or undefined neuroimaging, as well cases
of pre-or perinatal injury were excluded. Results:
23 patients, 15 (65.3%) female were included.
Mean age was 3.53 years: 11 (47.82%) patients
aged between 2 months and 2 years, 7 (30.43%)
from 2 to 6 years, 1 (4.34%) from 7 to 10, and 4
(17, 39%) from 10 to 17. Etiology could be defined in 8 cases (34.7%): cardiopathy in 3 (13%),
hemoglobinopathy in 1 (4.34%), autoimmune
inflammatory disease in 2 (8,69%) and trauma in
2 (8,69%). In the remaining 15 patients (65.3%),
9 (39,1%) had infectious disease 3 months prior
or during the installation of event. 11 children
(47.8%) showed no focal deficit as early clinical
presentation, including 8 (34,7%) with seizures,
1 (4.34%) with decreased level of consciousness and 2 (8,69%) with febrile syndrome. 16
(69,5%) patients were hospitalized in the acute
phase with average time of hospitalization of
31 days. Mean follow-up was 7,5 years (ranged
from 10 days to 17 years). At last evaluation 19
patients (82.6%) still presented mild/moderate
focal deficits. Mortality rate was 4/23 (17.3%).
Conclusions: Stroke in children is characterized
by a wide variety of etiologies, requiring extensive investigation. Even though, etiology remains
unclear in majority of cases, as seen in literature.
Concerning clinical presentation, seizures even
in absence of paresis is an important onset stroke
symptom in pediatric population. Another finding is the association of vascular and infectious
diseases. The mortality rate and late sequelae
were expressive and should be better analyzed in
a larger sample.
P-131
KLEPTOMANIA SYMPTOMS CAUSED BY LESION
IN HYPOXIC-ISCHEMIC BILATERAL CAUDATE
NUCLEUS: CASE REPORT
Guebert M, Santos W, Lucia EMS, Rocha SFB, Rizelio V,
Kowacs PA
• INSTITUTO DE NEUROLOGIA DE CURITIBA
Lesions in the caudate nucleus cause numerous
neuropsychiatric symptoms, behavioral and cognitive, among which memory deficits, language,
disinhibition, disorientation, executive dysfunction, apathy, atypicalaphasia, psychosis, personality disorders and predisposition to delirium.
Objective: Describe the clinical case of a patient
with atypical psychiatric condition secondary to
hypoxic-ischemic encephalopathy (HIE), with
bilateral lesion in caudate nucleus. Case report:
Female, 40 years old, with no psychiatric diseases in the past, submitted to elective surgery,
presented drowsiness, apathy, disorientation
and memory deficits in the immediate postoperative period.Neurological evaluation after 11
days identified HIE.Magnetic resonance imaging (MRI) of the brain showed ischemic lesions
in caudate nucleusbilaterally, globuspallidus
and putamen. Three months after the event a
neuropsychological examination found symptoms of apathy, decline of memory and temporo-spatialdisorientation. Retrograde amnesia of
lacunar profile, evasivespeaking, increased emotionality and change of traits of personality were
also found. Apathyshowed an improvement,
andlately,psychiatric symptoms (kleptomania)
emerged with a transitory character. The perfusion scintigraphy demonstrated cerebral relative
hypoperfusion mainly in the frontal lobes, the
anterior portion of the cingulate gyrus, in basal
ganglia, with emphasis on the right, and the cerebella, with heterogeneous pattern. Discussion:
Considering the hypothesis that the dysfunction
of circuits involving the caudate nucleus may be
directly related to psychiatric disorders withthe
symptom of kleptomania.In this patient kleptomania can be understood in the light of the
failure of inhibitory control exercised by caudate
nucleus in limbic circuits.
P-132
KNOWLEDGE OF THE PEOPLE FROM TERESINA-PI
ABOUT CEREBROVASCULAR ACCIDENT
Coelho RFS1, Moreira FJS1, Morais JS1, Gonçalves GP1,
Reis KF1, Nascimento LC1, Almeida KJ2
• 1UESPI; 2USP
Introduction: A cerebrovascular accident (CVA),
also known as stroke, is a disease with high prevalence, morbidity and mortality. The knowledge
of risk factors, pathophysiology, symptoms, signs
and appropriate conduct is an important weapon against these unfavorable rates. Objective:
Evaluate the knowledge of the population
about basic information about CVA. Method:
Cross-sectional population-based study by the
application of a simple medical case followed by
a semi-structured questionnaire in several places of the city of Teresina-PI. 105 individuals participated in the survey, whose ages were greater
than or equal to 18 years; who agreed to participate, thus signed the Statement of Informed
Consent. Data were organized and analyzed in
the software EpiInfo version 7.1.2.0. A Confidence Interval (CI) of 95% was used. Results: In
the surveyed population, 55(52.3%) were female
and 50(47.6%) were male and the mean age was
35.1 years. When asked about the possible diagnostic for the case shown the main answers given were CVA 34(32,3%,CI 23,5%‐42,2%), Cancer
6(5,7%,CI 2,1%‐12,0%), “Derrame” 30(28,5%,CI
20,1%‐38,2%) and 17(16,1%,CI 8,3%‐20,9%), said
that did not know the asnwer. In this study the
population showed some knowledge about the
5 major risk factors for CVA: Hypertension, cited
by 34 people (32.4%,CI 23,5%‐42,2%); Smoking
by 21(20.0%,CI 12,8%‐28,9%); Diabetes and Sedentary Lifestyle by 14(13.3%,CI 7,4%‐21,3%); and
Obesity by 10(9.5%,IC 4,6%‐16,8%). Regarding the
conduct, among those who answered that they
would call the emergency service 28(26.6%,CI
12,3%‐31,3%), only 13(46.4%) reported the correct number of the Medical Service (SAMU). With
regard to treatment, 76 individuals (72.4%,CI
62,8%‐80,6%) reported that there were treatment
and, for this purpose, physiotherapy was cited by
45.2%(CI 31,7‐52,6%) of the sample. In relation
to rehabilitation for CVA patients, 12(11.4%,CI
6%‐19,1%) said there were no rehabilitation
and 93(88.6%,CI 80,8%‐93,9%) said yes, there is
75
Posters
some kind of rehabilitation and physiotherapy was also the most cited response 38.1%(CI
31,7%‐52,6%). Conclusion: From the analysis of
the data obtained it was found a deficiency of information about the subject, reflecting the need
for investments in educational campaigns for the
prevention and adequate treatment of CVA, both
emergency and long-term.
P-134
MALIGNANT MIDDLE CEREBRAL ARTERY
INFARCTION AND ACUTE ARTERY OCCLUSION AS
CARDIOEMBOLISM MANIFESTATION
Souza MES1, Sousa GG1, Torres LA1, Lopes BRC1,
Martins LS1, Barbosa MU1, Magalhães IN2, Tavares RM1
• 1FACID-DEVRY; 2HUT
Introduction: Malignant middle cerebral artery
(MCA) infarction is one of the most devastating
forms of ischemic stroke, with a mortality of up
to 80% in untreated patients. About 20% are of
cardiogenic origin. Early diagnosis and clinical
follow-up are essential in these cases. Objective:
To report a case of concomitant malignant MCA
infarction with acute arterial occlusion (AAO) of
cardioembolic etiology. Case report: The patient,
female, 46 years old, admitted to a referral hospital in Teresina-PI, with mild dyspnea, decreased
level of consciousness and left hemiplegia. Within 48 hours developed cyanosis, hypothermia
and absence of radial pulse in right upper limb
(RUL) and worsening of respiratory pattern, being referred to the intensive care unit (ICU). New
CT scan showed malignant cerebral infarction in
the territory of right MCA with midline shift and
underwent decompressive craniectomy. Arterial
Doppler ultrasonography performed with RUL,
with AAO distal third of the brachial, ulnar and
radial arteres. Performed amputation of the distal third section of RUL. Performed an echocardiogram showing septal and inferior hypokinesia
of the left ventricle. ICU discharge after 8 days
presenting left hemiplegia, ipsilateral sensory
loss and partial palsys of conjugate lateral gaze
with 12 in NIHSS. After 15 and 21days from onset,
new CT scans performed aiming anticoagulation, this is not indicated for social reasons. Hospital discharge with significant improvement,
recovery of sensorial loss, left hemiplegia and a
slight deviation deviation of the contralateral labial commissure presenting respectively 15, 10
and 4 points inGlasgow Coma Scale, NIHSS and
modified Rankin scale and amputation of distal
two-thirds of the RUL. Not capable of working.
She was discharged to outpatient follow-up and
performed physical therapy and speech therapy.
Conclusion: The cardioembolism is one of th e
main causes for ischemic stroke without early diagnosis, proper treatment and satisfactory
follow-up, there is considerable increase in morbidity and mortality, as identified in the case. The
trivialization of treatment outside the hospital
is an obvious problem in our country because it
slows the reintegration of patients to their daily
activities and impairs family life, requiring further intervention.
76
P-135
MECHANICAL THROMBOLYSIS IN BASILAR ARTERY
STROKE: A SUCCESSFUL CASE
Takeshita BT, Rizelio V, Merida KLB, Cavalli H,
Meneses MS, Kowacs PA
• INSTITUTO DE NEUROLOGIA DE CURITIBA
Background: Posterior circulation stroke is responsible for approximately 27% of the cases.
Mortality is elevated, reaching 85%, and good
functional outcomes can be achieved in a quarter of patients underwent thrombolysis. The
main mechanisms for basilar artery occlusion
are thrombosis and embolism. Objective: To
report the first mechanical thrombectomy for
basilar artery stroke in a neurological center.
Case report: Female patient, 60 years old, with
risk factors arterial hypertension and dyslipidemia, was evaluated in Emergency Room due
to sudden left hemiplegia lasting four hours.
Blood pressure was 172x107 mmHg, and National Institutes of Health Stroke Scale (NIHSS) was
7 (left facial central palsy, mild dysarthria, left
hemiparesis). Stroke team decided to perform
the stroke protocol for intrarterial thrombolysis.
Brain magnetic resonance image (MRI) was performed, depicting acute stroke at the right pons,
and basilar artery occlusion. Digital subtraction
angiography confirmed a basilar flow interruption. Thrombectomy was performed with Solitaire® device in 6 hours of ictus, restoring the
blood flow and posterior circulation reperfusion.
At 24 hours follow-up, the patient NIHSS was 6
and brain MRI showed delimited image related
to the previous MRI. Craniocervical angiotomography showed pervious basilar artery, absence of
stenosis or occlusion of the intracranial vessels.
At discharge, NIHSS was 2, and at 30 days follow
up NIHSS was 0, Modified Rankin Scale was 0.
Discussion: Solitaire® mechanical thrombectomy is well described as device that restores blood
flow in obstructed vessels, but evidence-based
guidelines recommend alteplase intravenous
thrombolysis as first option. Even though, in
large vessels occlusion, as basilar artery, this
therapy should be considered for patients not
elective for alteplase use or in extended time
windows, selected by neuroimage studies.
P-136
MORTALITY RATE IN ACUTE STROKE PATIENTS IS
MUCH HIGHER IN SECONDARY HOSPITALS THAN
IN STROKE UNITS
Diniz BB, Tamietti MF, Queiroz BMA, Maia FM
• UNIFOR
Introduction: Stroke remains the major cause
of mortality in our country and a percentage of
these patients die in the acute phase or in the
first months after stroke. One of the major factors influencing mortality rates is the quality of
care offered in the inhospital period, but, due to
health system overload, many stablishments are
not prepare to treat these patients. Objective:
To compare mortality rates in patients admitted
with acute stroke regarding their inhospital care:
stroke unit, secondary hospital or emergency department. Methods: This is a prospective study,
in which 102 patients were randomly selected
as they were admitted to the emergency department of the Hospital Geral de Fortaleza. Patients
were evaluated in regard to their age, gender, NIH
score, glucose level, blood pressure admission
measures. Patients were not followed during the
inhospital period: they were transferred to three
major destinations, depending on availability of
hospital beds, to three major locations: stroke
unit, other secondary hospitals or remained in
the emergency department. After 3 months, patients or family were contacted again through
telephone and data regarding their destination after the first evaluation and occurrence of
death were recorded. Results: 80 patients were
contacted after the 3 month period. Mean age
was 68 years old, 57,1% male, hypertension was
present in 81% and previous stroke was reffered
by 35,7%. Mean glucose level on admission was
143mg/dL. 26,2% were transferred to the Stroke
Unit, 33,3% to secondary hospitals and 16% remained on the emergency department. No major diferences were detected between groups,
regarding NIH score, age, blood pressure. Stroke
Unit mortality rate was 15,4% (p=0,03 IC: 0,0443
– 1,8676), while patients in secondary hospitals
had a much higher rate of 57,1% (p=0,05 IC: 1,88
– 25,9). Conclusion: Although transfering stroke
patients to secondary hospitals is necessary to
the management of our public health system, it
may be a risk factor to increase mortality in these
patients.
P-137
MOYAMOYA DISEASE: CASE REPORT
Santos TB1, Lack ATF2, Ary CC1, Ferreira IBM1,
Ribeiro EML3, Cristino Filho G2, Ponte KF2, Ribeiro FLF1,
Ribeiro VD2, Silva RR1
• 1UFC; 2SCMS; 3SANTA CASA DE MISERICÓRDIA DE
SOBRAL - SCMS
Introduction: Moyamoya disease (MMD) is a
progressive cerebral vascular disease characterized by chronic bilateral stenosis or occlusion of
the arteries around the circle of Willis, with emphasis on the resulting collateral arterial circulation. Patients displaying the angiographic pattern of Moyamoya and without known risk factors are considered as having MMD, while those
with secondary causes are classified as suffering
from Moyamoya Syndrome (MMS). Objective:
To report the case of a Brazilian woman, with no
japanese ancestry, white, with history of multiple
ischemic strokes (ischemic stroke). Case report:
CLDC, female, 46 years old, born and raised in
Ipueiras - Ce. She presented with sudden left
hemibody motor deficit, right central facial palsy, dysarthria, difficulty walking, and decreased
level of consciousness and was sent to Santa
Casa de Misericórdia de Sobral. The cranial CT
and MRI with contrast showed cranial stenotic
blood vessels, collateral circulation areas, as well
as cerebral infarction on the right frontoparietotemporal topography. A cerebral arteriogram
revealed stenotic intracranial arteriopathy, a
characteristic pattern of MMD. During hospitalization, symptoms improved and the patient was
discharged for outpatient monitoring and she is
now waiting for cerebral revascularization surgery. Discussion: Abnormalities of nonatherosclerotic cerebral vasculature predisposes one
to ischemic stroke at all ages, but especially in
children and young adults. These abnormalities
lead to a severe stenosis or occlusion of blood
vessels as observed in MMD, with decreased
cerebral blood perfusion, which can lead to
cell death. MMD is one of the leading non-atherosclerotic vascular disorders associated with
ischemic stroke. The patient did not show any
secondary condition linked to MMS, so was classified as MMD. Furthermore, it is reported that
hemorrhagic strokes are more common in adults
with MMD. However, in this case, there were
a large number of recurrent ischemic strokes.
Asian studies conflict with the European studies regarding epidemiology, making it important to identify MMD patients in Brazil because
XXVI Congresso Brasileiro de Neurologia
the deficiency of epidemiological studies in the
country hinders the diagnosis and treatment of
the victims of this disease.
P-138
MULTIDISCIPLINARY INTERVENTION OF A PATIENT
WHO SUFFERED A ISCHEMIC STROKE
Mian JA, Melo CB, Correia TA, Moreira CTGA,
Medeiros S, Diccini S, Stanich P, Gonçalves MIR
• UNIFESP
Introduction: Strokes are the most responsible
causes for functional disability in the western
world. After the acute stage of the injury, chronic
dysfunction responsible for functional dependency are found. It is a function of the multidisciplinary team to evaluate the needs of the patient and his family, and to provide the necessary
resources to the implementation of care to the
patient, looking for results that demonstrate full
health assistance. Therefrom, actions that help
the patient return to his functional and psychosocial activities are needed, still in the unit of intensive care. Objective: To describe the effects of
hospital care multidisciplinary interventions in a
patient that suffered a ischemic stroke. Method:
It is a case-study, held in the Neurology Unit of
Intensive Care of Hospital São Paulo, based in the
patient’s medical record and is assistance offered
on april and may/2014. Case-study: Male J.B.A.,
62 years-old, retired, incomplete higher education, married, from Sao Paulo/SP. He was diagnosed with a stroke in the left middle cerebral artery and occlusion of the left internal carotid artery. In the unit of intensive care, the patient was
evaluated by a multidisciplinary team, including
nurse, phisiotherapist, speech therapist and psychologist. Debate: This patient had right side
hemiplegia, no control of the trunk, dependency
for all functional activities and disability for deambulation, moderate oropharyngeal dysphagia
and global aphasia. He counted on family support. The nursery assistance had the goal to prevent deleterious effects and rehabilitation, using
techniques and interdisciplinary actions with
an active presence of the caregiver. The physiotherapist work aimed the acquisition of independence, focusing on the functional training, muscular strengthening, sensitive stimulation, with
the use of orthosis and positioning. The intervention of the speech therapist consisted in myofunctional activities and language stimulation,
besides strategies to promote communication.
In psychology the work favored better cognitive
and psychological adaptation of the patient, as
well as better confrontation and resistance of
the family. Conclusion: It was demonstrated that
the interventions enabled the reduction of risks
coming out of a stroke, stimulation of the locomotory and cognitive harmed functions, and the
patient’s social reintegration.
P-139
MULTIPLE PONTINE STROKES CAUSED BY
VARICELLA-ZOSTER VASCULOPATHY
Sampaio PHMA, Barbosa HRR, Pontes Neto OM
• HCFMRP-USP
Introduction: Varicella-Zoster virus (VZV) vasculopathy is an established etiology for stroke.
Primary VZV infection causes varicella, after
which the virus become latent. Later, VZV can
reactivate to cause zoster and may be followed
by vasculopathy. The spectrum of VZV vasculopathy includes stroke and TIA but its diagnosis
requires high degree of clinical suspicion and can
be challenging, as illustrated in this Case report.
Case report: An immunocompetent 42-yo man
developed a zoster in right maxillary and mandibular trigeminal branches and right C2 dermatome. Three weeks later, he developed mild
left hemiparesis, dysarthria, bilateral cerebellar
ataxia and hyperreflexia. Non-contrast brain CT
was normal. MRI showed signs of recent stroke
on right pons and left pontomedullary transition,
post-contrast enhancement of right trigeminal
nerve and normal angiogram. Lumbar puncture
revealed mild mononuclear pleocytosis, normal
protein, elevated red blood cell count, and discreetly reduced glucose. VZV DNA detection was
negative. CSF anti-VZV IgG was 1.661 (serum:
1.320). Diagnostic workup for other causes of
stroke was negative. Small vessel VZV vasculopathy was diagnosed. He was started on aspirin and
treated with IV acyclovir, with substantial clinical
recovery. Discussion: After reactivation from peripheral ganglia, VZV can migrate transaxonally
to infect cerebral arteries, resulting in small and
large vessel focal or multifocal vasculopathy.
VZV vasculopathy is suspected in the patient
with new onset of neurological symptoms after
an episode of zoster. However, vasculopathy can
occur from weeks to several months after zoster,
and in some cases a rash is not present. Virtually
all patients have abnormal brain imaging findings, including unifocal or multifocal ischemic
strokes and involvement of small and large vessels. Lesions characteristically affect the graywhite matter junction and may also enhance in
post-gadolinium MRI. Lumbar puncture usually
shows a mild mononuclear pleocytosis (<100),
elevated red blood cells and protein, and normal
glucose. However, absence of pleocytosis does
not exclude the diagnosis. Presence of oligoclonal bands is common. Detection of anti-VZV
IgG has higher sensitivity than VZV DNA detection through PCR. IV acyclovir with or without
concomitant steroids is the treatment of choice.
However, the optimal dose and duration of antiviral treatment, and benefit of steroid therapy has yet to be determined and awaits further
studies.
P-140
MULTIPLE STROKES SECONDARY TO ATRIAL
MYXOMA: A CASE REPORT
França C, Godoy LFS, Studart Neto A, Tinone G,
Yamamoto F, Conforto AB
• HOSPITAL DAS CLÍNICAS DA FACULDADE DE
MEDICINA DA USP
Introduction: Atrial myxoma is a rare cardiac
tumor, with estimated incidence of 0,5/million/
year, most commonly in 50 year old women.
Eighty percent are in the left atrium and 30% can
present with neurological symptoms or signs,
often caused by ischemic stroke. Objective: Description of a case of cerebral infarcts secondary
to atrial myxoma. Case report: A 65 year-old
Brazilian man presented sudden, short-duration
episodes of vertigo and wide based gait 9 months
prior to admission. On March, 2014, he reported a similar episode, with a duration longer (2h)
than usual. His neurological examination was
normal. This patient had prior history of rheumatic fever in childhood, weight loss (20kg in 2
years) and depression. Results: The first brain
MRI showed recent and old ischemic areas in
both cerebellar hemispheres and in the frontoparietal white matter. The echocardiogram
showed a 5.5cm sessile and heterogeneous mass
inside the left atrium that projected to the left
ventricle during diastole and was most likely an
atrial myxoma. Cardiac MRI confirmed the findings. Blood work-up revealed an increased C-Reactive Protein (135 mg/L) and Erythrocyte Sedimentation Rate (67 mm). Patient was submitted
to cardiac surgery 9 days after admission and was
discharged with a mild dysarthria and left central
facial palsy. Conclusions: This case illustrates an
uncommon cardiac tumor as a cause of cerebral
infarcts. It reinforces the importance of the cardiac imaging in the etiological investigation of every case of focal acute syndrome (stroke or transient ischemic attack). Fast evaluation and treatment can prevent recurrent ischemic lesions and
long-lasting disability.
P-141
MYELOPROLIFERATIVE SYNDROME WITH
MUTATION JAK 2 V617F AND ITS RELATION TO
ISCHEMIC THROMBOTIC SYNDROMES - CASE
REPORT
Martins SNS, Furtado VJ, Lima L, Saraiva SB,
Nogueira MACK, Carvalho GS
• HNMD
Introduction: Thrombotic events represent
high impact in morbidity and mortality in patients with myeloproliferative neoplasms (MN).
The discovery of the mutation JAK 2 V617F in
the pathogenesis of these diseases, and the fact
that its positivity can be related with platelet
activation and, consequently, with thrombotic
events has been the subject of many research.
Objectives: To report a case of a patient that
had two strokes and an acute coronary event
associated with myeloproliferative neoplasm,
and a positive test for the mutation JAK 2 V617F.
Case report: Male patient, 62 years, had an ischemic stroke with hemorrhagic transformation in
July of 2013. Five months later, the patient had an
acute coronary syndrome when a tri vascular lesion was diagnosed in catheterization with pharmacological stent placement. Increased platelets
had been shown in both events. In April 2014,
the patient had a new ischemic stroke evolving
with large hemorrhagic transformation. A research by hematology showed search BCR/ABL
chromosome Philadelfia= negative; search of
the gene mutation JAK 2 V617F= positive; bone
marrow biopsy compatible with chronic myeloproliferative neoplasm favoring primary myelofibrosis and presence of mild myelofibrosis.
Discussion: Myeloproliferative neoplasms Philadelphia chromosome negative are considered
acquired prothrombotic states in which thrombotic events are generally the factor that leads
to diagnosis. In these events, the presence of the
mutation JAK2 V617F may increase morbidity
and mortality among the patients, because its
presence has been implicated as additional risk
for both thrombosis arterial and venous. Events
of great arteries in MN are commonplace such
as stroke, acute myocardial infarction, unstable
angina, transient ischemic attack, and others. Although the research as etiology in patients with
ischemic stroke and myocardial was not statistically significant, in those who have the mutation,
the probability increases chances of thrombotic
events. The multifactorial analysis in patients
with thrombotic ischemic events is required, but
myeloproliferative neoplasms with the mutation
JAK2 V617F should be considered in a context of
recurrent events and hematological alterations,
because its control has impact in the following of
these patients.
77
Posters
P-142
NEUROCYSTICERCOSIS PRESENTING WITH
RECURRENT TRANSIENT ISCHEMIC ATTACK
Cardoso TAMO, Avelar WM, Carneiro MRP, Ferreira LS,
Rigon BGS
• UNICAMP
Introduction: Cysticercosis is the most common parasitic disease in central nervous system with variated clinical manifestations wich
are correlated with the number, type, size and
topography of lesions and the host immune response to the parasite. Cerebrovascular disease
is a relatively common but underrecognised
complication of neurocysicercosis (NCC), estimated between 4% and 12%. Objective: report
an unusual and underdiagnosed presentation of
ncc. Case report: a 41 year old man, with multiple and recurrents paroxysmal episodes of motor
deficit and alteration in speech with full recovery
of the deficit few minutes after. during episodes,
it was noted right hemiparesis with fasciobrachial predominance and dysarthria. the neurological examination between episodes showed
no abnormalities. CT scan on admission showed
multiple diffuse parenchymal calcifications and
a left frontotemporal hypodense area with an inespecific hyperdense halo. a later performed mri
revealed racemose cysts in brain cisterns and a
greater inflamatory cyst, with mass effect near
the proximal left middle cerebral artery (mca)
segment, with an associated local stenosis of this
vascular segment. CT and MRI angyography performed later also demonstrated multiple areas
of stenosis and leptomeningeal enhancement
that are related to signs of vasculitis. Discussion:
cerebrovascular complications in neurocysticercosis are closely related to the distribuition of
cysticerci and the accompanying arteritis, by inflamatory occlusion of the arteries at the base of
the brain secondary to cysticercotic arachnoiditis. In this situation, the clinical syndrome will
range from asymptomatic infarct and cerebral
infarction with transient symptoms to a classic
lacunar syndrome. transient focal neurological
symptoms in ncc have been reported and a vascular origin has been commonly attributed. in
addition, immune inflammatory response was
recently described as a cause of neurological dysfunction that resembles trasient ischemic attack
(TIA). The clinical picture of this patient probably can be explained by multiple mechanisms
such as acm extrinsic compression, imunne inflamatory response combined with vasculitis.
After high doses of corticosteroids there was no
recurence of deficits. Conclusion: neurocysticercosis must be considered in the differential diagnosis of tia and other cerebrovascular complications in endemic areas.
P-143
NEUROFUNCTIONAL DIAGNOSIS OF PUSHER
SYNDROME IN A SPECIALIZED SERVICE OF
REHABILITATION
Brito CSGF1, Araújo AVL2, Batista FLA2, Lima ALS2,
Franco CIF2, Araújo DP2, Galdino GS2
• 1UFPB; 2UEPB
Introduction: Stroke is a public health problem
and a significant cause of disability in the world.
The contralateral hemiplegia is a common
symptom, although others also disabling and
influence the functional capacity. Among those
78
symptoms observed Pusher Syndrome (PS),
clinical condition in which there is alteration of
postural control with body and severe imbalance
leaning opposite the brain damage, the action of
pushing the paralyzed side with the non-affected hemisphere and resistance to passive correction in sitting and standing positions side. It
is estimated that PS affects approximately 10%
of individuals with stroke and give an underdiagnosed condition. A method of diagnosis of PS
is the observation of the presence of the criteria
proposed by Davies (1996). Objectives: Diagnosing PS in hemiplegic or hemiparetic, characterize the sample and identify the effectiveness of
the criteria proposed by Davies. METHOD The
study is characterized in descriptive, exploratory, transversal and quantitative with a sample
of individuals of both sexes, older than 18 years,
with neurological sequelae resulting from ischemic stroke ± bleeding. To identify the PS used
a Profile of Neurological Assessment and the criteria proposed by Davies (1996). The data were
documented in spreadsheets and analyzed using
statistical correlation. Results: The sample consisted of thirty-two individuals with neurological
sequelae resulting from one or more ischemic
± hemorrhagic stroke, with a prevalence of 53%
males, mean age 55.78±13.79, 53.2% of onset between the ages of 41‐60 and with hemiplegia or
hemiparesis right in 62.5% of cases. The PS was
diagnosed in 6.25% of the sample, with total for
males and for the involvement of the left hemisphere and aged between 39‐71. Conclusion: In
the present study it was possible to identify individuals affected by PS, obtain diagnostic efficacy
through the criteria proposed by Davies and perform characterization of the total sample. When
approaching 10% the incidence of diagnosed
cases of PS in the present study, the concordance
with current literature about the topic.
P-144
NO INCREASE OF STROKE IN YOUNG: INCIDENCE
TRENDS IN JOINVILLE, BRAZIL OVER THE LAST 9
YEARS
Santos N1, Carneiro C2, Moro C1, Longo A1, Okamoto VT2,
Lovatel C2, Cabral N2
• 1HMSJ; 2UNIVILLE
Background: Recent data have suggested that
stroke incidence in young people may be rising.
In this population-based study, we aimed to determine wheter the incidence and proportion of
stroke in people aged <55 years- old had changed
over the last nine years. Methods: All cases of
first-ever event of ischaemic and hemorrhagic
stroke bellow 55 years old occurring in Joinville
from 2005 to 2013 were prospectively ascertained
from a population-based registry. Incidence rates
were calculated and temporal trends were analysed by age groups and stroke subtypes using a
poisson regression to estimate incidence rate ratios (IRR). The rates were age-adjusted to World
population in 2000. Results: Over the last 9-years
we recorded 3288 cases of ischaemic stroke (IS)
and 341 cases of hemorrhagic stroke (HS). The
proportion of patients with ischaemic stroke
bellow 55 years – old ranged from 18% (94 /515
; 95% CI,0.15 – 0.21) in 2005 to 23% (124 /536 ;
95% CI,0.20‐0.26) in 2013. The proportion of hemorrhagic stroke bellow 55 years-old ranged from
32% (26/80; 95% CI, 0.22‐0.43) in 2005 to 31%
(18/58; 95% CI, 0.2‐0.4) in 2013. The age-adjusted incidence <55 years-old among IS in 2005 was
20 per 100,000 inhabitants and 19 per 100,000 in
2013 (IRR:0.99; 95% CI, 0.66‐1.41) . The incidence
<55 years-old among HS was 5.1 per 100,000 in
2005 and 3.0 per 100.000 in 2013 (IRR: 0.55; 95%
CI, 0.23 - 1.08.). Conclusions: The incidences
and proportions of IS in people bellow 55 years
old are stable over the last 9 years in Joinville. We
observed a boderline decrease of HS in the same
age-strata. The diverse socio-economic and epidemiological transitions in Brazil might explain
the diferences in our rates comparing to high income countries.
P-145
OLFACTION LOSS IN MILD HEAD TRAUMA
Sales FT1, Fraga GPBLOD1, Guerreiro MM1, Lees AJ2,
Warner TT2, Silveira-Moriyama L1, Lila R1
• 1UNICAMP, CAMPINAS, BRAZIL; 2UCL INSTITUTE OF
NEUROLOGY, LONDON, UK
Introduction: The association of olfaction loss
and head trauma has been repeatedly demonstrated in cases of severe trauma. However, mild
traumas are much more frequent and there are
few studies of olfaction in these cases. The existing studies show conflicting results. Objectives:
To compare groups of patients with mild head
trauma (Glasgow 13‐15 and normal cranial computed tomography - CT) with control groups of
the same gender, age and socio-economic conditions, using a standardized smell test (Sniffin
Sticks – SS16), with a translation into Portuguese
already validated, to study olfactory function.
Methods: 30 patients with mild head trauma
(trauma patients who had a score between 13‐15
at admission and who had a normal CT scan)
were selected from a database of trauma patients admitted during 2011. Patients and controls were recruited under informed consent and
underwent a brief neurological examination,
cognitive screening using the Mini Mental State
Examination and smell identification testing using the SS16. Patients and controls were paired
by sex, smoking, self-reported rhinitis and family income. Pair by age was considered as having less than 2 years (y) difference between the
age of each case and control. Results: The test
were applied to 60 subjects in total, with a mean
age of 38.93 years (range 16y‐77y, SD=16.57y), of
which 36 (60%) were male and 24 (40%) female.
Results are summarized by group as mean [SD]:
range. Control group: age=39.1y [16.9y]: range
17y‐77y; Mini Mental State Examination (MMSE)
score=28.9 [1.5]: range 24‐30; SS‐16 score=13.4
[1.6]: range 10‐16. Mild head trauma group:
age=38.8y [16.6y]: range 16y‐77y; MMSE score
=28.3 [1.8]: range 24‐30; SS16 score=12.5 [1.9]:
range 8‐16. A multiple linear regression using
the score in the SS‐16 as the outcome variable
and age, gender, smoking, self-reported rhinitis,
family income, MMSE score and group (control
or trauma) demonstrated that gender (p<0.001),
family income (p=0.001) and group (p=0.03) independently influenced the results of the SS‐16.
The 95% confidence interval for beta for the
group factor was ‐1.652 to ‐0.089, indicating a mild
but significant effect of mild trauma over olfaction. Assumptions were checked by visual inspection of the residuals. Conclusion: Our study
demonstrates an effect of mild head trauma on
smell identification. We suggest that further
studies investigating olfaction should include a
brief questionnaire about mild head trauma.
XXVI Congresso Brasileiro de Neurologia
P-146
ONE-AND-A-HALF SYNDROME CAUSED BY
MIDBRAIN INFARCTION: CASE REPORT
Harger R1, Lange MC1, Parolin LF2
• 1HOSPITAL DE CLÍNICAS - UFPR; 2NEUROLOGIA DO
HOSPITAL DE CLÍNICAS - UFPR
Introduction: One-and-a-half syndrome is a
gaze abnormality characterized by a conjugate
horizontal gaze palsy in one direction plus an
internuclear ophtalmoplegia in the other. In
the fully developed syndrome, one eye is in the
midline and fails to move horizontally while the
other can only abduct. Objective: Report a case
of One-and-a-half syndrome caused by midbrain
infarction. Case report: A 81-year-old woman
presented with an acute onset of dysarthria,
trunk ataxia and divergent strabismus with binocular diplopia. She had a past history of hypertension. On examination, she had complete horizontal gaze palsy to the left eye, with a contralateral internuclear ophtalmoplegia. MRI revealed
left paramedian midbrain diffusion restriction.
She had a normal electrocardiogram, extra and
intracranial Doppler-sonography, but the transthoracic echocardiogram revealed akinesia of
apical segment. She received the diagnosis of
cardioembolic midbrain stroke. Discussion:
One-and-a-half syndrome is usually caused by
pontine injury with unilateral lesion of the paramedian pontine reticular formation or the abducens nucleus, and interruption of fibers of the
medial longitudinal fasciculus, originated in the
contralateral abducens nucleus. We described a
patient with the same clinical features, but with a
different topography of injury, the midbrain.
P-147
OPALSKI SYNDROME: AN UNUSUAL VARIANT OF
LATERAL MEDULLARY INFARCTION
Bouza CB, Echegaray MVF, Monteiro JMC, Lyra VMOB,
Passos Neto CEB, Martin DLD, Fukuda TG, Jesus PAPD
• UFBA
Introduction: Opalski syndrome is an uncommon variant of lateral medullary infarction
(Wallenberg syndrome), whichis related to intracranial vertebral artery occlusionimmediately
below the decussation of medullary pyramids. It
is marked by hemiparesisipsilateral to the stroke
site. The aim of this paper is toalertto the syndromedetection and the diagnosis formulation.
Case report: A 37 years old Caucasian woman,
without previously diagnosed diseases, woke up
with headache in the right temporal region, dizziness, nausea, vomitsand imbalance with tendency to fallto the right side. At the admission,
the patient presented with hypertension and
elevated blood glucose levels, gait ataxia, right
hemiparesis and hyperreflexia, associated with
left dysesthesia, partial ptosis and right miosis,
as well asloss of the nasolabial fold. The brain
computed tomography showed no abnormalities. Magnetic resonance imaging (MRI) showed
a lesionbelow the decussation of medullary pyramids at the right side. During the hospital investigation, electrocardiogram, transthoracic echocardiography, carotid and vertebral Doppler, and
magnetic resonance angiography of intraand
extracranial vessels did not show any abnormalities. Laboratory tests confirmed the elevated
blood glucose levels as well as a high lipid profile,
hence the patient was diagnosed with metabolic
syndrome. She was discharged after 45 days of
hospitalization to outpatient care, with improvement of symptoms, however maintaining dysesthesia in the left hemibody. The main suspicion
was arterial dissection, but it couldnot be proven.Discussion:This syndrome was described in
1946 and is considered rare. The main etiologiesare vertebral artery dissection or occlusion, with
involvement of the penetrating medullary arteries and subsequent vascular infarction. Cranial
MRI with diffusion weighted imaging is a sensitive method to detectmedullary infarction.Conclusion: Therefore, Opalski syndromeis a Wallenberg syndromevariant that involvesinfarction of
an area below the medulla, which presents with
ipsilateral hemiparesis to the stroke site and may
have other signs and clinical symptoms suggestive of posterior circulation insult, its recognition
requires proper understanding of neuroanatomy
and neurological exam for suspicion.
P-148
ORAL ANGIOEDEMA AFTER RTPA FOR ISCHEMIC
STROKE. TWO CASES REPORTS AND LITERATURE
REVIEW
Silva MS, Lange MC, Zetola VF, Ducci RDP,
Rosário Filho NA
• UFPR
Introduction: Angioedema occurs in 1.3% to
5.8% of patients with acute ischemic stroke submitted to thrombolysis with alteplase (rtPA).
Objective: To present two cases of angioedema
during rtPA infusion and review the literature
regarding the risk factors, the management and
the outcome to these patients. Methodology:
Two cases of orolingual angioedema (OA) related to rtPA in patients admitted to a Stroke Unit
are presented. The literature was reviewed, based
on the PUBMED databank. It was considered all
Case reports and case series of OA during rtPA in
acute ischemic stroke patients until May 2014.
The variables analyzed were previous angiotensin-converting enzyme inhibitors (ACEI), insular
stroke, relation between side of stroke and side of
angioedema, management of the OA, advanced
airway support and death. Results: The two cases
presented are female patients previously hypertension and using ACEI. Both had insular stroke
contralateral to OA and they were undergone to
treatment with hydrocortisone with good outcome. 47 cases were found on PUBMED database. Literature review suggest an association
between OA with insular or anterior frontal cortex stroke and previously use of ACEI. In 61% of
the cases the stroke side was contralateral to OA.
89.8% of patients were treated with corticoids
or anti histamine drugs. Eight patients needed
ventilator support and no death was observed in
those patients. Conclusion: Despite uncommon,
the OA is a possible adverse effect to be considered in patients on ACEI use and insular or anterior frontal cortex stroke. Its occurrence should
be treated with caution, given the potential risk
of progression for ICU support.
P-149
OUTCOME DETERMINANTS OF STROKE PATIENTS
IN A TERTIARY HOSPITAL IN SÃO PAULO
Kuster GW1,2, Dutra LA1,2, Azevedo DS1,2, Cirino CP1,2,
Cunha JLM1,2, Albuquerque MVC1, Broner T1,2,
Cerqueira Filho RB1,2, Brasil IP1,2, Pacheco EP1,2,
Miranda VA1,2, Gama MTD1,2, Arruda MA1, Volcov C1,
Domingues RB3
• 1HOSPITAL PAULISTANO; 2PIAVEN AMIL SÃO PAULO;
3
CHRU, POLE NEUROLOGIE, UNIVERSITÉ LILLE 2,
LILLE, FRANCE
Background: The mortality related to stroke in
Brazil is one of the highest among Western countries; however, the stroke outcome determinants
are still poorly known in this country. Objectives:
To assess functional outcome and in-hospital
mortality determinants in a São Paulo tertiary
hospital. Methods: We prospectively evaluated
the data of all stroke patients assisted between
2012 and 2014 in the “Hospital Paulistano, São
Paulo”. Clinical and treatment data were recorded. Modified Rankin scale (mRs≤2 and mRS>2),
in-hospital mortality, and NIH score at discharge
were used to assess outcome. Students’ t test was
used for the mean comparisons of continuous
data. Categorical data were compared using chisquare analyses. The correlation analyses were
performed with Pearson test. Binary logistic regression analysis was performed with in-hospital
mortality and discharge Rankin modified score
as dependent variables. Results: Three hundred
forty-one patients were included in the study,
being 52.2% male with mean SD age of 66.8 15.7
years. The most common stroke type was ischemic stroke (IS) (59.2%); 35% of IS were of cardioembolic etiology, and 13.4% of IS were treated
with thrombolytic therapy. Intracerebral hemorrhage (ICH) accounted for 11.1% of the patients
and ICH was associated with greater severity and
poorer outcome than IS. After adjusted analysis,
the significant functional outcome determinants
for the whole group and for IS were higher NIHSS (P<0.001 for the whole group and IS), lower
Glasgow score (P=0.008 and 0.021, respectively),
and lower oxygen saturation level (P=0.026 and
0.016, respectively). The only significant mortality determinant for the whole group and for
IS was the presence of visual symptoms at admission (P=0.018 for the whole group and IS).
Conclusions: The characteristics of the studied
population, including the proportion of ICH and
IS were similar to other previous studies. The rate
of thrombolysis and other validated treatments
were according to international recommendations. The functional outcome determinants
found here, including lower NIHSS, lower level
and consciousness, and lower oxygen saturation
were already found in previous studies carried
out in other countries. Visual symptom was the
only predictor of in-mortality probably reflecting
the greater severity of strokes involving posterior
circulation vessels.
P-151
PARINAUD SYNDROME AFTER THALAMIC
HEMORRHAGE: CASE REPORT
Spitz CN, Cardoso FM, Rodrigues BCB, Drumond MT,
Vaz G, Balthazar H, Freitas GR
• INSTITUTO D‘OR DE PESQUISA E ENSINO
Introduction: Parinaud syndrome (PS) is caused
by lesions of dorsal midbrain and it is characterized by light near dissociation of the pupils, lid
retraction (Collier sign‘s) and convergence retraction nystagmus. Objective: Relate a case of
PS resulting from injury not located in the midbrain. Methods: Describe clinical and radiological aspects of a complete PS due to hemorrhagic
injury in thalamus and ventricular inundation.
Results and Discussion: A 48-old male patient
had sudden loss of consciousness followed by
nausea, vomit and binocular vertical diplopia.
The neurological examination showed full PS,
besides hypoesthesia in left leg and left central facial palsy. Computed tomography scan
revealed right thalamic and intraventricular
hemorrhage, confirmed by magnetic resonance
imaging. Magnetic resonance angiography was
normal. PS is the result of involvement of the
dorsal portion of the midbrain. Full syndrome
after thalamic lesions is rare. Dissociation of the
79
Posters
pupillay light reflex – accomodation isolated is
described in thalamus-tectum lesions, probably
due to involvement of pathways located between
cortical centers convergence and neurons in
oculomotor nerve situated in the tectum. This
may also explain isolated vertical gaze palsy.
PS in thalamic lesions can be attributed to increased pressure in the pretectal and tectal area
or narrowing of communication inducing hydrocephalus secondary to aqueductal compression.
Conclusion: Although PS is usually due to midbrain injury, thlamic damage may also result in
ocular symptoms and should be investigated in
patientes with PS.
P-152
PATENT FORAMEN OVALE: VILLAIN OR SPECTATOR
Gagliardi RJ, Cerqueira LG, Pagiola IC, Zetehaku AC,
Oliveira FTM, Fortes ALVS, Luca NC, Melges NS,
Gagliardi VDB, Reges DS, Sanvito WL
• SANTA CASA DE SÃO PAULO
Introduction: The importance of patent foramen
oval (PFO) in ischemic stroke has been widely
studied, especially in young patients. The association of the PFO, and other abnormalities of the
interatrial septum has been linked with several
other disorders of the central nervous system
such as cryptogenic stroke, temporary global
amnesia, disorder of cerebral decompression in
divers. The etiology of stroke remains undetermined even after exhaustive investigation, and
only the presence of PFO may be linked to the
ischemic event. However, this finding is common
in general population which may be a confounding factor. Objectives: Assess the prevalence of
PFO in patients with cryptogenic stroke from the
ambulatory of vascular neurology of Santa Casa
in Sao Paulo. Methods: It was selected patients
who are being followed at the ambulatory of
vascular neurology, rated as cryptogenic stroke
and performed transcranial doppler and microbubble test for the presence of left and right
shunt. Results: It was selected 22 patients with
cryptogenic stroke. Among the 22 patients investigated, 11 had positive transcranial doppler with
microbubbles of moderate to high conductance.
This value corresponds to 50% of patients studied. In literature the average prevalence in the
general population varies FOP from 20 to 30%.
Conclusion: There are few prospective epidemiological studies linking the occurrence of ischemic stroke in patients with PFO. There is one
meta-analysis that divided the study into three
main groups: the first relates the occurrence of
PFO, ASA or both in ischemic stroke compared
with the control group without ischemic stroke;
the second compared the presence of PFO, ASA
or both in cryptogenic ischemic stroke and the
control group with patients with known etiology
of ischemic stroke; and a third group, the occurrence of atrial abnormalities described above in
cryptogenic ischemic stroke compared with controls without ischemic stroke. This meta-analysis
found little statistical significance because the
confidence intervals were very large. The PFO
closure was introduced in 1992, and after some
meta-analysis of observational studies of these
patients brought inconclusive results, because
despite the closure may be associated with reduced risk of stroke recurrence, there was increased risk of developing atrial fibrillation, necessary to perform anticoagulation even after PFO
closure. Data are preliminary because the work
is ongoing.
80
P-153
PERCHERON ARTERY INFARCTION: A CASE
REPORT
Menezes JR1, Brando KCF1, Nassif DV1, Pereira TR1,
Schmidt F1, Terrana D1, Reis MM2, Bagdadi LV1
• 1UERJ; 2UFRR
Introduction: The thalamus is supplied by small
vessels originating from the posterior communicating artery and the P1 and P2 segments of the
posterior cerebral artery. The paramedian infarction bithalamic occurs because an anatomical variation of the paramedian arteries known
as the artery of Percheron (AOP), and presents
some patterns of ischemia. Objective: To report
a case of bilateral paramedian thalamic infarction, showing clinical manifestations and report
patterns of AOP. Highlight the importance of AOP
infarction as a differential diagnosis of coma hyperacute installation. Case report: A patient 65
years, female, previously hypertensive and left
amaurosis was admitted to the neurology department of the University Hospital Pedro Ernesto due to decreased level of consciousness, with
drowsiness and bradypsychism, 10 days of evolution with acute onset. During the first 12 hours
of installation, the patient showed response only
to painful stimuli and at times indicating ventilatory support due Glasgow 8, according to reports
from the local hospital. At admission the patient
presented with apathy and memory impairment
with confabulation. Presented paralysis of vertical gaze, with correction to the vestibule-ocular
reflex. The NIHSS on admission was 4, the patient
lost 1 point in orientation and 4 point guidance
in visual fields. Pre RANK was 0 and admission
was 4. To research were performed USG DOPPLER carotid and vertebral unchanged; Normal
CT angiography with hypoplastic posterior communicating arteries, but no change in flux; echocardiography and Holter unchanged and MRI of
the brain with lacunar infarcts in the thalamus
bilaterally, more evident on the left. Discussion:
The classic clinical infarct AP is composed of altered mental status, memory impairment and
palsy of vertical gaze. This infarction may present four distinct patterns of involvement: bilateral paramedian thalamic with rostral midbrain;
bilateral paramedian thalamus; paramedian and
bilateral anterior thalamus to rostral midbrain;
bilateral paramedian and anterior thalamus. The
patient described had bilateral paramedian thalamic infarction without midbrain involvement.
Conclusion: The patient can not present the
complete triad of AP infarction and not always
the palsy of vertical gaze necessarily implies the
involvement of the midbrain.
P-155
PREDICTIVE FACTORS OF FUNCTIONAL
INDEPENDENCE MEASURE AFTER STROKE IN
ELDERLY PATIENTS
Santos-Pontelli TE, Favoretto DB, Braga MC,
Barbosa MRSMSB, Mello LB, Bem FJD, Rimoli BP,
Norberto AMQ, Weber KT, Silva LAT, Figueiredo CS,
Pileggi CSM, Pacheco AC, Mazin SC, Cougo-Pinto PT,
Baggio JAO, Pontes-Neto OM
• FMRP-USP
Background: The incidence of stroke increases with age and functional independence after
stroke is highly related to the quality of life in elderly patients. The identification of factors that
determine the functional outcome after stroke is
important to acute stroke treatment, discharge
and rehabilitation planning. To the best of our
knowledge, this is the first study that assessed
the predictive factors of the Functional Independence Measure (FIM) scale in a sample of Brazilian stroke elderly patients. Objective: To analyze
the determinants of FIM scores at 3 months after
stroke in elderly patients. Methods: we conducted a retrospective analysis of patients aged 65
years or more, selected from the hospital-based
prospective stroke cohort (REAVER) at Ribeirao
Preto Medical School. The primary outcome
was the FIM score at 3 months after the stroke.
A Backward method was used to select the final linear regression model. Results: Between
January and August 2014, 38 patients (55.26%
female), mean age 74.58±7.66 years, mean National Institute of Health Stroke Scale (NIHSS) of
10.72±9.27 (CI95%: 7.59 – 13.86), 86,84% patients
with ischemic strokes, 44.74% of the patients
had previous stroke, mean FIM of 88.89±37.97.
The only independent predictor of the FIM at 3
months was the NIHSS at admission (p<0.001).
Conclusions: The functional independence in elderly patients after stroke has strong association
with the stroke severity at admission. Aggressive
medical treatment directed to reverse the initial
deficit should be encouraged for elderly stroke
patients.
P-156
PRIMARY PREVENTION OF STROKE IN PRIMARY
HEALTH CARE: REVIEW OF LITERATURE
Leal LCC, Seixas LF, Alves GM
• UNIRG
Introduction: In 2012, cardiovascular disease
(CVD) victimized 100 751 people in Brazil, accounting for 8.89% of hospital admissions.
Among the modifiable risk factors, some stand
out for their magnitude, transcendence and vulnerability: high blood pressure (hypertension),
diabetes mellitus, dyslipidemia, smoking, obesity, and physical inactivity with potential for effective treatment in primary health care. The primary care in Brazil, plays a key role in controlling
and reducing morbidity and mortality related to
stroke / TIA. Objective: To review the most recent
aspects of literature related to primary prevention of ischemic stroke with emphasis on use in
primary health care. Methods: We carried out a
survey of material published in the period 2008
to 2014 using the Medline and Lilacs databases
with the key words in English and Portuguese:
“stroke”, “primary prevention” AND “primary
health care”. Were found 20 articles, of which six
were selected because they are texts that are suited more to the proposal from the search. Results:
1. Physical Activity - Physical inactivity is an important modifiable risk factor. Observational
studies show an association between physical inactivity and increased risk of stroke (RR =1.82). 2.
Control of obesity – USA cohort studies have verified association between BMI and incidence of
stroke. BMI>32 reveals an increase of 2.37 times
(RR 2.37) when compared w / BMI <21. 3. Reduction of smoking - A meta-analysis and systematic
review of observational studies have examined
the association between smoking and stroke. The
consumption of>20 cigarettes / day was related
to an increased rate of 2.52 times in total stroke
(RR 2.52). 4. Control DM - Studies reveal that
there is a RR =2.82 for individuals with blood glucose>140 mg / dL. 5. Control of hypertension Hypertension is a major risk factor for stroke. The
incidence of stroke increases directly in relation
to the degree of blood pressure elevation. The
increase of stroke in hypertensive patients is 4
times (RR 4.0) second review of recent studies. 6.
Dyslipidemia -Also constitutes an important risk
XXVI Congresso Brasileiro de Neurologia
factor. The use of statins to lower cholesterol levels reduces the risk of stroke by 21% (RRR 21%).
Conclusion: Primary care should aim to reduce
the risk of ischemic stroke in individuals who are
asymptomatic and can do this by reducing the
major known risk factors such as hypertension,
type 2 diabetes, dyslipidemia, and smoking.
P-157
PROFILE OF ASSISTANCE OF PATIENTS RECEIVING
IV-TPA AT CLINICS HOSPITAL OF UNIVERSITY OF
SÃO PAULO MEDICAL SCHOOL IN 2013
Calderaro M, Mendonça RH, Barros JV
• HCFMUSP
Background Prognosis of acute stroke patients is
related to time to recanalization of the occluded
artery. Intravenous tissue plasminogen activator (IV-tpa) enhances the probability of artery
recanalization. As long as the shorter the time
to recanalization the better the prognosis, it is
imperative to monitor the quality of stroke assistance. Objectives: Authors present the profile
of stroke assistance in Acute Stroke patients receiving IV-tpa at Clinics Hospital of University of
São Paulo Medical School (HC-FMUSP) in 2013.
These data integrate a more comprehensive database which is under development and intend
to monitor and improve Stroke assistance in this
medical facility. Methods: Pharmacy records
of patients that received IV-tpa at HC-FMUSP
in 2013 were analysed and ischemic stroke patients were selected to compose a database. Retrospective analysis of medical records of these
patients was performed. Data was analysed
in software Excel 2011. Results: From January
1st to December 31st 2013 twenty seven acute
stroke patients received IV-tpa at HC-FMUSP.
Most patients were male (62,96%) and mean age
was 65,86±14,56. Twelve patients (44,44%) were
admitted to an intensive care unit (ICU), where
they stayed on average 9,42 days. Patients admitted to an ICU were older (mean age 74,08±9,00
vs 56±14,05) and had higher NIH Stroke Scores
(mean NIHSS 14,08±6,24 vs 11,15±5,55). In hospital mortality was 18,52%, although prognosis
from 29,63% of patients could not be recovered
from electronic medical records. Mean door-toneedle time in these patients was 58±27 minutes,
but this information could be recovered in only
14 (51,85%) patients. Conclusion: Our data show
that it is possible to target door-to-needle time to
less than 60 minutes for acute stroke patiens. The
majority of patients are not admitted to an ICU,
probably a reflex of the limited amount of ICU
beds in our facility. More effort should be done to
improve the quality of the information provided,
so that measures in order to reduce time to artery
recanalization can be monitored.
P-158
PROFILE OF ASSISTANCE OF PATIENTS RECEIVING
IV-TPA IN BRAZILIAN MINISTERY OF HEALTH
CERTIFIED STROKE CENTERS IN 2013
Calderaro M, Barros JV, Dias RD
• HCFMUSP
Background: Intravenous tissue plasminogen
activator (IV-tpa), stroke units and stroke centers
have been shown to improve prognosis of stroke
patients. In 2012 the Ministery of Health of Brazil fomented a program that certified centers in
Brazil to provide comprehensive Stroke treatment. Objectives: Authors present the profile of
stroke assistance in Stroke Centers in Brazil in
2013. Methods: All ischemic stroke patients that
received IV-tpa in 2013 in Stroke Centers certified by the Ministery of Health of Brazil were
analysed. Data was extracted from Hospital Admission System (SIH/SUS). Descriptive analysis
was performed in software Excel 2013. Hospitals
were classified as academic or not according to
Ministery of Health information. Data concerning neurology residency programs was obtained
in the Brazilian Academy of Neurology Secretary
and Website (www.abneuro.org). Results Nineteen Ministery of Health certified Stroke centers
administered IV-tpa in 341 acute stroke patients
in 2013. Most (70,38%) were treated in Rio Grande
do Sul. Others were treated in Paraná (13,78%),
São Paulo (12,02%), Bahia (2,05%), Minas Gerais
(0,88%) and Pernambuco (0,88%). Concerning
Hospital Size, 69,21% thrombolysis were performed in extra centers (more than 500 beds),
24,05% in big centers (150 to 500 beds) and 6,74%
in medium centers (50 to 150 beds). The majority of thrombolysis was performed in hospitals
with neurology residency (67,4%). Hospitals with
others residency programs (not neurology) performed 17,3% of thrombolysis, while 15,2% were
performed in non-teaching hospitals. Mean age
of patients was 64,7±13,8 years and 46,3% were
male. Patients stayed in hospital for 10,6±9,1
days, and 27,9% of them were admitted to an
ICU, where they stayed on average 4±4,5 days. In
hospital mortality was 13,8%. Conclusion: Governmental initiatives to improve stroke manegement are needed and welcome. However, stroke
assistance Nationwide is quite heterogenous and
the majority of IV-tpa is still performed in more
developed States and in Hospitals with neurology residency programs. Only a small fraction of
patiens are admitted to an ICU, probably a reflex
of the small number of Critical Care Unit Beds.
More effort and investment are needed in order
to provide adequate assistance to stroke patients
in Brazil.
P-159
PROSPECTIVE NEUROPSYCHOLOGICAL
EVALUATION OF EXECUTIVE FUNCTIONS ON
HEMORRHAGIC STROKE PATIENTS
Camargo APA1, Tabaquim MLM2, Pontes Neto OM1,
Alves FFA1, Flores FA3, Zanetti AC3, Alves TO3
• 1FMRP; 2USP; 3ANHANGUERA
Background: Among stroke subtypes, intracerebral hemorrhage (ICH) is commonly associated
with unfavourable functional outcome, cognitive impairment and high incidence of depressive disorders. Elevated morbidity and mortality
rates in those patients compromise an evaluation of the neuropsychological domains intrinsically affected by the ICH. Objectives: To assess
neuropsychological executive functions (EF)
after ICH and its relation with depressive disorders after stroke, cognitive impairment, and site
of stroke lesion. Study methods: we performed
a prospective evaluation of neuropsychological
EF on patients diagnosed with ICH, admitted by
the Neurosurgery Department at a public tertiary
academic hospital in Brazil. Patients with recurrent stroke or lost on follow-up were excluded.
The neuropsychological evaluation (NPE) was
planned at admission, and after 30-days and
12-months, and consisted of: Wisconsin Card
Sorting Test; WAIS Digit Span subtest; Corsi
Block Tapping Test; Trail Making Test; Stroop
Test; and verbal fluency tasks. Stroke severity was
scored by the National Institutes of Health Stroke
Scale, Glasgow Coma Scale, and the ICH score.
Functional outcome was defined by the Barthel Index, and depressive symptoms by the Beck
Depression Inventory and the DSM-IV-SCID-IV
structured interview. Site of lesion was determined through brain computed tomography
analysis at admission. Results: During 2012, 12
patients were included in this study with primary spontaneous ICH, and evaluated upon admission and 30-day after onset, the median age was
58,5 years. At the 12 months follow-up, 7 of 12
patients were reevaluated. The median time-after-onset of NPE were 50,4 hours at admission,
36 days at 30-days, and 12 months. NPE detected
EF impairment (91,6% and 71%), impaired functional outcome (75% and 57%), and depressive
symptoms (66% and 71%) on both 30-days and
12-months, respectively. The EF impairment on
30-days and 12-months was found more preeminent on patients with subcortical ICH. There was
an improvement on physical functional outcome
on 71% of the patients, but depressive disorder
and EF impairment were still present on 60% and
66% of the subcortical ICH patients, respectively,
after 12 months. Conclusions: Even though there
was an improvement on physical functional outcome 12 months after stroke, the prospective
evaluation of patients with ICH showed high frequency of vulnerable executive functions, cognitive impairment and depressive symptoms.
P-160
REALITY OF STROKE AND ACUTE MYOCARDIAL
INFARCTION (AMI) AT PUBLIC HOSPITALS IN
SALVADOR: AN ECOLOGICAL AND ECONOMIC
ANALYSIS
Santos Junior JR, Santos ÍGS, Oliveira JVVC, Leal MP,
Oliveira LF, Lessa KP, Braga JC, Santos MRM,
Di Girolamo LM, Sampaio GS, Cardoso ES
• UNIVERSIDADE DO ESTADO DA BAHIA
Introduction: Cardiovascular and cerebrovascular diseases are the leading causes of morbidity,
disability and death in Brazil. The costs of hospitalization by SUS are increasing paired with
mortality growth. It is essential to monitor the
quality of patient care, with the prospect of the
creation and adoption of care protocols for these
harms. Objectives: Compare the impacts on
public health in Salvador caused by myocardial
infarction and stroke through the analysis of data
on hospitalizations, deaths, daily rates, mortality
and total spending on public hospital network.
Method: An ecological study with individuals of
both sexes and all ages admitted at public hospitals in Salvador-BA between July/2013 and
June/2014. Data about hospitalizations, deaths,
daily rates and total amount of hospital expenses
related to AMI (ICD‐10 I‐21), Transient Ischemic
stroke (ICD‐10 G‐45), Cerebral Infarction (ICD‐10
I‐63) and stroke not specified as hemorrhage or
infarction (ICD‐10 I‐64) were obtained from DATASUS. Cerebral Infarction, Stroke and Transient
Ischemic stroke not specified as hemorrhage or
infarction were included in a single group to be
confronted with the AMI. Results: The public
hospitals network in Salvador had 1126 hospitalizations and 12,794 days of hospitalization related to AMI, while stroke data correspond to 2542
internalizations and 31,010 days of hospital stay.
The AMI is responsible for 116 deaths, and has a
mortality rate of 10.3%. Stroke caused 318 deaths,
with a mortality rate of 12.5%. The AMI generated R$3,328,724.91 in expenses, the average expenditure per hospitalization is R$2,956.24. The
AVC demanded resources about R$4,443,150.52,
with an average cost of R$1,747.90 for relocation.
Conclusion: The stroke has a higher incidence,
mortality rate relative and absolute, demands
greater trading volume and hospital services
in comparison to AMI. The primary prevention
81
Posters
strategies of these two conditions are similar, but
the data collected shows that are required greater
efforts to adopt secondary and tertiary strategies
prevention of stroke. Investing in job training,
implement the recommended guidelines and restructure the hospitals emergencies is the most
appropriate path to minimize socioeconomic
impacts on the public health system.
P-163
REVERSIBLE CEREBRAL VASOCONSTRICTION
SYNDROME (CALL-FLEMING SYNDROME): REPORT
OF SEVEN CASES
Guerra DV, Veloso VN, Schmid MF, Baeta AM,
Bezerra MLE, Baccin CE, Piske RL
• BENEFICÊNCIA PORTUGUESA DE SÂO PAULO
Introduction: The Call-Fleming Syndrome (CFS)
is a benign angiopathy characterized by segmental cerebral vasoconstriction demonstrated
by angiography, angiotomography or angioresonance, with reversal documented in a control
study after twelve weeks. Clinical manifestations
include sudden headache, focal neurological
deficits related to ischemic and or haemorrhagic injuries, posterior reversible encephalopathy
syndrome and brain oedema. Objective: To describe clinical and angiographic features, treatment and outcome of seven patients with CFS.
Method: Clinical and angiographic study in seven female middle-aged patients, who presented
with sudden neurological symptoms and had
neuroimaging exams showing signs of stroke,
lobar haemorrhages and or subarachnoid haemorrhage, as well as compatible angiographic
findings. All patients underwent CSF analysis
and extensive laboratory screening for systemic
diseases, especially collagen disorders. Results:
All patients had multiple sites of segmental vasoconstriction in intracranial vessels. Most patients
had history of chronic use of dual antidepressants
and serotonin reuptake inhibitors. After treatment with calcium channel antagonists and oral
corticosteroids, complete reversal was observed
in angiographic control study after twelve weeks.
Residual mild to moderate neurological deficits
were observed only in patients whose clinical
presentation was ischemic stroke. No patient
had significant CSF or other laboratory findings.
Conclusion: The Call-Fleming syndrome should
be considered in the diagnostic hypothesis in patients with focal neurological deficits of sudden
onset. Diagnosis requires demonstration of focal
stenosis in intracranial arteries, as well as reversal in a control study in twelve weeks. Its incidence is probably underestimated due to lack of
awareness about the syndrome. In this context,
the clinical panel presented by this series provides detailed information that can assist in the
diagnosis and treatment of this syndrome.
P-164
REVERSIBLE CEREBRAL VASOCONSTRICTION
SYNDROME IN A PATIENT WITH BACTERIAL
MENINGITIS: A CASE REPORT
Marangon LM, Dias JC, Pitta IJR, Oliveira PV, Silveira RC,
Corade LR, Oliveira LC, Freitas GR
• HQD
Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by multifocal areas of narrowing in cerebral arteries,
which by definition disappear in 12 weeks. It
typically presents with severe headache, acute
onset, with or without focal neurological signs
82
and symptoms. It can be caused by many factors,
among the most important ones are pregnancy,
puerperium and exposure to sympathomimetic drugs. Bacterial meningitis may present with
cerebrovascular complications, such as vasculitis, vasospasm and arterial and venous thrombosis. However, there are no reports in the literature
of its association with RCVS. Objective: To report
a case of a patient with bacterial meningoencephalitis that developed RCVS after use of sympathomimetic drugs. Case report: An 53 years
old man, had severe headache of sudden onset,
with migrainous characteristics associated with
fever. Initially diagnosed with bacterial sinusitis
was treated with oral antibiotic and a combination of antihistamine, analgesics, and systemic
vasoconstrictor drugs. He persisted with severe
headache and after 3 days envolved with diplopia and blurred vision, and then he was admitted
to our hospital. He had paresis of the left lateral
rectus muscle and neck rigidity. Brain magnetic resonance imaging(MRI) disclosed cortical
subarachnoid hemorrhage, cortical infarcts and
pus in the lateral ventricles. AngioMRI showed
multifocal arterial narrowing consistent with
RCVS. A lumbar puncture showed neutrophilic
pleocytosis (650 cells), and the patient was treated with ceftriaxone for 14 days for bacterial meningoencephalitis. A control AngioMRI after 10
days showed no vasoconstriction. Discussion:
We describe a case of RCVS in the presence of
an infectious process of the central nervous
system associated with the use of systemic vasoconstrictor drugs. Although still remain uncertain real pathophysiology of cerebrovascular
events in bacterial meningitis, it is reasonable to
think that such acute neurological insult is able,
through the release of inflammatory cytokines,
to increase cerebral vasoreactivity, making it
more susceptible to vasoconstriction. Patients
with bacterial meningoencephalitis may develop
brain infarcts due to vasculitis. Maybe in some of
these patients RCVS may play a role in the etiology of infarcts.
P-165
REVERSIBLE CEREBRAL VASOSPASM SYNDROME
(RCVS) DUE HYPERCALCEMIA: AN UNCOMMON
CAUSE OF STROKE
Rocha FA1, Pitombeira MS1, Travassos FT1, Rangel DM1,
Veras SRO1, Carvalho JJF1, Rocha TT2, Ferreira DB1
• 1HGF; 2UNICHRISTUS
Introduction: RCVS is an underdiagnosed entity,
but the formulation of diagnostic criteria in 2007
has been responsible for the emergence of more
cases. It is more prevalent in women and the typical age of presentation is around 40y. The typical
RCVS presentation is a Thunderclap Headache
(TCH). Objective: The objective is to report a
common electrolyte disturbance presenting as
an atypical manifestation. A unusual stroke because of hypercalcemia. Case: A 45-Year-Old female presented to the Emergency Department
with typical TCH. Two weeks before admission,
she had 3 similar episodes with 5 minutes duration that resolved partly with oral NSAID. She
had been previously well, took no regular medication, was a non-smoker and had a history of
episodic migraine. She practiced regular physical
activities and used injectable vitamin D and E for
cosmetic purpose. On presentation general and
neurologic examination was normal. An initial
CT Brain showed a temporoparietal hematoma
and an initial MR Angiogram revealed no aneurysm and a multifocal widespread narrowing of medium and small size cerebral arteries.
On evaluation: Total Calcium=13,8 (NR 8,5‐10,5);
Vitamin D>150 (NR 30‐100); PTH=6,6 (NR 10‐55).
The provisional diagnosis was RCVS. Electrolyte
was corrected and nimodopine was initiated for
headaches. After 2 weeks she was free-headaches
and a new CT Angiogram showed complete vasospasm resolution. Discussion: RCVS has been associated with various clinical conditions, including vasoactive drugs (e.g., triptans), post-partum
period, some tumors (e.g., pheocromocytoma)
and hypercalcemia. There are few reports of intoxication with high Vitamin D doses used by
athletes causing a calcium increase that promotes vasoconstriction. The Physiopathology is
uncertain. Apparently the Raphe Nucleus sends
stimulus for perivascular nerves and then occurs
a increase in Noradrenaline and Serotonin levels
promoting vasoconstriction. It is believed too
that hypercalcemia promotes vasoconstriction
because of endothelial dysfunction Despite it
isn’t a common cause of stroke, up to on third of
RCVS patients has a vascular complication such
as SAH, ischemic/hemorrhagic stroke. The treatment consists of supportive care and correction
of precipitating factor. Nimodipine is used for
preventing new crisis of headaches. The prognostic is favorable and only 5% patients have any
chronic deficits.
P-166
SICKLE-CELL DISEASE AND MOYAMOYA-LIKE
CEREBROVASCULAR DISEASE: REVIEW BY
ILLUSTRATIVE CLINICAL SITUATIONS
Paranhos DR1, Farias CA2, Guimarães VP1, Tosta NR1,
Brito FS1, Barreto ES1, Almeida ERP1, Maldonado IL3
• 1EBMSP; 2FTC; 3UFBA
Introduction: A relatively common cause of
stroke in children is sickle cell anemia. Although
this syndrome is well characterized in the literature, the treatment of occlusive ‘moyamoya-like‘
cerebrovascular disease needs further clarification. Objective: To review in the light of current
medical literature the diagnosis and management of occlusive cerebrovascular disease associated with sickle cell disease, illustrating the
Discussion with clinical vignettes. Methods: We
reviewed scientific articles obtained in systematic searches performed on NCBI-PubMed that
addressed the management of sickle cell disease
and cerebrovascular disease simultaneously. The
information was then organized to discuss illustrative clinical situations, stressing the following elements: therapeutic options, indications,
angiographic results, clinical outcomes and
complications. Results: The search conducted
in April 2014 resulted in 65 articles. Since 1990,
chronic transfusion therapy has been the standard for the care of children who present with
AF and evident cerebrovascular damage, being the main recommendation of the American
‘National Institute of Health‘. With a lower level
of evidence, the success of surgical treatment
through
encephalo-duro-arterio-synangiosis
in case series has encouraged studies regarding
the use of this technique in the context of AF.
Conclusion: The occlusive cerebrovascular disease is an important cause of early morbidity in
patients with sickle cell disease in children. The
hypothesis of vascular disease with intracranial
stenosis should be considered with a high degree
of suspicion and Methods: of prophylaxis such
chronic transfusion should be applied when
necessary. The results of this review also suggest
that selected patients with sickle cell disease and
cerebrovascular ‘moyamoya-like‘ may benefit
from a neurosurgical opinion.
XXVI Congresso Brasileiro de Neurologia
P-168
SNEDDON’S SYNDROME ASSOCIATED WITH
LIBMAN-SACKS ENDOCARDITIS: CASE REPORT
Melo BA1, Silva LSA2, Rodrigues AL1, Vilasbôas ÍGM3,
Almeida CF2, Rosário MS4, Branco RCC4, Farias DS4,
Pereira DF4
• 1UNIFACS; 2EBMSP; 3UFBA; 4HOSPITAL SANTA
IZABEL
Introduction: Sneddon, in 1965, described 6 cases with the association of ischemic stroke and
Livendo Reticularis (LR). Hemiparesis, hemianopia, seizure may be part of the syndrome,
depending of the affected area. The skin alterations result from circulatory disorders, and can
precede or follow the neurologic presentation.
Thorough medical history and complementary
exams such as anti-phospholipid antibodies;
Cranial Computed Tomography (CCT), Magnetic
Resonance Imaging (MRI) and Cerebral Arteriogram (CA) are the basis of diagnosis. Objetive:
Present a case of a patient with suggestive manifestation of Sneddon Syndrome. Case report:
JSF, 35-year-old, female, right handed, living in
Salvador. Admitted in the emergency with paresthesia and paresis of the left upper limb (LUL)
and paresis in left hemi-face few hours before. A
year ago noticed alteration of skin characterized
by light violet spots distributed in upper and lower limbs and trunk. In the period, had numbness
in face and LUL, however without weakness. The
physical exam presented irregular violet lesions,
interspersed by health skin in thighs, extensor
surface of arms and bilateral proximal plantar region of the hallux. Neurological exam: dysarthria
and slight deviation of labial commissure to the
right, pain and tactile hypoesthesia in left hemiface and LUL. Pronation in LUL in maneuver of
extended arms. Brain MRI showed restricted on
diffusion weighted images on right parietal lobe
as well as subcortical diffuse microangiopathy;
cerebral AngioMRI without alterations. There
was small images on atrial face of both cusps
suggestive of vegetation; atrial sept with transseptal flux suggestive of patent foramen ovale seen in transesophageal echocardiography.
Discussion: Sneddon Syndrome is rare, with an
incidence yet unsure, having an estimate of four
cases in one million/ year. There is a clear association between this illness and the Anti-phospholipid Syndrome. Aseptic cardiac valve vegetation
has been reported previously associated with
this syndrome. Keywords: Sneddon Syndrome;
Stroke, Livedo Reticularis; Anti-phospholipid antibodies; Liebmann-Sacks endocarditis.
P-169
SODIUM NITROPRUSSIDE, A LOW PRICE AND SAFE
DRUG TO CONTROL BLOOD PRESSURE DURING
THROMBOLYSIS IN ACUTE ISCHEMIC STROKE. A
CASE-CONTROL STUDY
Koslyk JLA, Ducci RDP, Nóvak EM, Zetola VF,
Lange MC
• UFPR
Background: The blood pressure (BP) control is
an important issue in the management of acute
ischemic stroke (AIS) patients submitted to intravenous thrombolysis. Current guidelines suggest that BP must be maintained bellow 180/105
mmHg when recombinant tissue plasminogen
activator (rtPA) is used. Sodium nitroprusside
(SN) is an option to reduce BP, but it could be
related to rebound effect and BP control liability that could influence prognosis of patients
submitted to intravenous rtPA. The aim of the
study is to analyze if the use of SN to BP control
is safe in patients with AIS submitted to intravenous rtPA. Methods: A case-control study where
60 patients composed the sample, all them had
ischemic stroke (IS) and were submitted to intravenous rtPA. Patients were prospectively followed but retrospectively selected and pared by
age, gender and admission NIHSS in two groups:
Thirty patients in the Control Group (CG) with
BP levels below 180/105mmHg, without requiring intravenous antihypertensive drug to control
the BP previous or during the rtPA infusion. Thirty patients in SN Group (SNG) with BP levels over
180/105 mmHg requiring SN to control the BP.
The BP considered was the high value before rtPA
infusion. It was analyzed the presence of symptoms related to BP variability in both groups in
the first 24 hours of admission. Outcome variables were: 1) the presence of any hemorrhagic
transformation (HT) between 24 and 36 hours
after rtPA infusion; 2) the presence of symptomatic HT (SHT) between 24 and 36 hours after rtPA
infusion; 3) NIHSS after 24 hours of treatment;
4) the independence on discharge, considering
modified Rankin score (mRS)≤2; 5) Death until
three months after stroke onset. Results: There
was no statistical difference between groups
comparing risk factors, except by hypercholesterolemia that was more frequent in CG (86.7%
vs. 60%, p=0.020) and admission systolic and diastolic BP (in mmHg), both were higher in SNG
(systolic BP: 191.6±15.7 vs. 149.4±20.5, p<0.001;
diastolic BP: 111.9±17.9 vs. 87.6±10.1, p<0.001).
The presence of symptoms (headache, confusion, nauseas, vomiting) related to BP variability
in the first 24 hours was observed in three (10%)
patients from CG and in eight (26.67%) patients
from SNG, p=0.181. There were no statistical differences between both groups to any of the outcome variables analyzed. Conclusion: The SN is
safe in the BP control during intravenous thrombolysis to AIS.
P-170
of the soft palate. Regarding swallowing, 100% of
the sample was diagnosed with oropharyngeal
dysphagia with important involvement of the
voluntary phase of swallowing. Conclusion: It
was concluded that 100% of the population studied had speech-language disorders with greater
prevalence in changes in mobility of the soft palate, dysphonia and dysphagia.
P-171
SPEECH-LANGUAGE DISORDERS IN WALLENBERG
SYNDROME
Gomes NAS, Abbas RTB, Saconato M, Gonçalves MIR
• UNIVERSIDADE FEDERAL DE SÃO PAULO - UNIFESP
Introduction: The syndrome of Wallenberg, also
called posterior inferior cerebellar artery syndrome, has corresponding clinical pathology
infarction dorsolateral portion of the bulb. This
region may be associated with speech-language
disorders involving severe dysphagia and dysphonia (injury to the ambiguous nucleus - affects
functions of the vagus nerve X and glossopharyngeal nerve IX), vestibular syndrome (lesion
in the vestibular nuclei) and alteration of facial
sensitivity (injury to the spinal tract and nucleus
trigeminal V). Objective: verify speech-language
disorders in patients with Wallenberg Syndrome.
Method: This was a retrospective study conducted in the Integrated Service of Speech Pathology,
Hospital São Paulo. Records of 13 patients were
analyzed, 9 (69.2%) men and 4 (30.7%) women with a mean age of 54 years. Results: It was
observed that of the total sample nine patients
(69.25%) were dysphagic, 4 (30.75%) had normal swallowing and 6 patients (46.15%) were
identified with dysphonia. Conclusion: It was
observed, in most of patients studied, dysphonia
and mild and severe dysphagia.
P-172
SPEECH-LANGUAGE DISORDERS IN POSTERIOR
FOSSA LESIONS
SPONTANEOUS CERVICO-CRANIAL ARTERIAL
DISSECTION: CASE REPORT AND REVIEW OF THE
LITERATURE
Abbas RTB, Gomes NAS, Saconato M, Gonçalves MIR
Araújo BAF, Castro GD, Morales RR
• UNIVERSIDADE FEDERAL DE SÃO PAULO - UNIFESP
• UFU
Title: Speech-language disorders in posterior
fossa lesions Introduction: The base of the skull
is formed by three fossae, the anterior fossa, middle and posterior. The posterior fossa is located
in the posterior region of the skull and through
its foramens there is the passage of the following
cranial nerves: VII, VIII, IX, X, XI and XII. In this
region we also observe the passage of the internal
jugular vein, bulb, meninges, cerebrospinal fluid,
vertebral arteries and spinal roots. Since this is
a common area for the passage of many cranial nerves, speech-language disorders involving
dysphagia, dysarthrophonia, dysphonia, facial
paralysis may be present in patients with tumor
or vascular lesions in posterior fossa. Objective:
verify speech-language disorders in patients with
lesions in posterior fossa region. Method: This
was a retrospective study conducted in the Integrated Service of Speech Pathology, Hospital São
Paulo. Records of 14 patients were analyzed: 10
men and 4 women with a mean age of 52 years.
Of the whole sample, 64.2% patients were diagnosed with posterior fossa tumor and 35.8% with
vascular injury in this territory. Results: It was
observed that 85.8% had dysphonia during perceptual evaluation, with roughness and loudness
diminished, 14.3% had facial paralysis of central
type and 57.2% had the peripheral type. From
the total sample, 71.5% had abnormal mobility
Introduction: Spontaneous cervical and cerebral arterial dissections are uncommon causes
of cerebrovascular ischemic disease, being more
likely to affect young adults. Early diagnostic and
treatment have major impact on morbidity and
mortality. Objective: To describe a case of recurrent ischemic cerebrovascular disease secondary
to spontaneous cervico – cranial arterial dissection, and review the literature. Case report: We
describe a 39-year-old male with previous moderate arterial hypertension and dyslipidemia,
who presented sudden cervical pain 10 months
before. Fifteen days after, he experienced vertigo and left hemiparesis, and was admitted at the
emergency department. Brain magnetic resonance imaging showed no abnormalities. Ultrasound with Doppler and magnetic resonance
angiography showed segmental arterial stenosis
of the right carotid. Secondary prophylaxis with
acetylsalicylic acid and clopidogrel was prescribed, and maintained ambulatorial care. After
8 months, he had recurrence of the ischemic episode with the same symptoms, plus numbness
and epileptic seizure. Brain imaging showed
right parietal ischemic area. After the episode,
he developed “restless legs syndrome” on the left
hemibody. He was attended at the Neurology Department of the Federal University of Uberlândia, and submitted to cerebral angiography, that
83
Posters
showed segmental arterial stenosis, or “string
sign”, at the proximal third of the cervical segment of the right internal carotid artery, which
is a characteristic sign of arterial dissection.
Discussion: Spontaneous cervical and cerebral
arterial dissection is a rare cause of ischemic
cerebral disease, but can be encountered at up
to 20% of the cases that occur under 50-yearsold patients. It is usually preceded by dissection
symptoms and signs, and can be associated to
cardiovascular risk factors. The present case has
shown to be clinically similar to the traditional
description. However, recurrence of the cerebral
ischemic episode after use of the antithrombotic therapy is rare according to the literature.
There is no consensus about the best treatment
strategy. Most studies agree that conservative
management is the first approach to be tried
(antithrombotic therapy or anticoagulation). Endovascular management is not considered firstline treatment for spontaneous cervical cerebral
artery dissections, and should be considered in
selected cases.
P-173
STROKE AFTER LEFT VENTRICULAR ASSIST
DEVICE FOR LONG-TERM IMPLANTATION: A CASE
REPORT
Polin MAM, Braga GP, Luvizutto GJ, Fusco DR,
Cicchetto LAF, Hueb JC, Bazan SGZ, Bazan R
• FMB-UNESP
Introduction: The left ventricular assist device
(LVAD) for long-term is implantable, magnetically accentuated axial flow pump designed to
support the left ventricle for adults with endstage heart failure until heart transplant. Aim: to
describe the stroke with end-stage cardiac failure
who were supported with the LVAD. Case report:
J.A.S., male, 53 years, ex-tobacco smoker, hypertensive, dyslipidemic, Acute Myocardial Infarction (AMI) previous, with cardiac failure (CF),
having opted for anticoagulation. After 5 years
evolved with a new AMI and cardiogenic shock.
After one month, he presented unstable angina,
evolving with CF functional class IV refractory
to inotropic medication therapy. It required an
aortic balloon, with subsequent implant of LVAD
(Berlin Heart INCOR®). After 30 days he presented with lacunar ischemic stroke of the right thalamus. Patient prescribed double antiaggregation
and anticoagulation, receives hospital discharge
with mRS 2. At 1 month after hospital discharge
he indicated dependent ventilatory thoracic pain
and difficulty in grasping objects with the right
hand, evolving with NIHSS 19; and CT showed
extensive intraparenchymatous hematoma in
the left hemisphere. He underwent anticoagulation reversion, hematoma drainage and decompressive craniectomy. Conclusion: Ischemic
cardiovascular disease and ischemic stroke are
frequently associated. In this patient, there is
probable compromise of cerebral microcirculation characterized by disease of small vessels,
resulting in cerebral ischemia, and subsequent
severe intraparenchymatous hemorrhage. Although LVAD can alter the cerebral hemodynamic, its true role in stroke genesis remains unclear.
Given the high prevalence of these comorbidities, screening for diseases of the small vessels
must be suggested prior to LVAD.
84
P-174
STROKE AND PEDIATRIC HIV VERTICALLY
ACCQUIRED INFECCTION. CASE REPORT AND
REVIEW OF THE LITERATURE
Zetola VF, Chamma J, Lucca G, Lange MC, Germiniani F,
Almeida SM
• UFPR
Introdution: The epidemiology consideration of
pediatric HIV infection must take into account
HIV-infected women and children together because the worlwide increase in the number of
children with vertically accquired HIV-infection.
Over 90% of HIV positive children accquired their
infection perinatally from their mothers before,
during the birth or from breastfeeding. Although
stroke is an uncommom complication in pediatric patients with AIDS, some co-infection make
it more frequently. Objective: report a stroke in
a pediatric HIV vertically accquired infecction
Case report: we report the case of a 12-year-old
boy with a known history of AIDS who presented
at emergency room with right hemiparesis, nauseas and vomiting. During the preceding days
the adoptive mother related mental status (confusional state) and reduced conscious level. CD4
T lymphocytes was 216 µl The imaging studies
MRI showed a subcortical left temporal posterior
isquemia and a discrete white matters lesions.
The cerebrospinal fluid (CSF) was examined
without any any greater suspicion (glucose CSF
67 mg/dl (blood glucose 155 mg dl), white cells
2,8 mm3 and proteins 69 mg/dl, Ziehl-Neelsen
(ZN), SDA and culture for M. tuberculosis resulted negative. CSF cultures for bacteria and fungi
were also negative. Latex agglutination test for
Cryptococcus resulted negative both on CSF
and blood. Tuberculose opportunistic infection
of the central nervous system was detectable by
PCR and a treatment was begun. The The workup
for other additional etiologies of stroke was negative. Comments: The co-infection tuberculosis
(TB) and acquired immunodeficiency syndrome
(AIDS) has been causing worsening of the morbidity and mortality for both diseases in the last
two decades. TB and multiple underling pathologies including HIV infection, as well as other risk
factors can lead to a greater risk of stroke.The different prognoses associated with the etiologies
of stroke in these patient population mandate a
careful and thorough evaluation.
P-175
STROKE EPIDEMIOLOGY FROM A PRIMARY
STROKE CENTER IN SÃO PAULO-BRAZIL:
IMPORTANCE OF RECORD AND MANAGEMENT
PROTOCOLS
Areco CV1, Brasil IP1, Dutra LA1, Azevedo DS1,
Cirino CP1, Cunha JLM1, Albuquerque MVC1, Broner T1,
Cerqueira Filho RB1, Pacheco EP1, Miranda VAD1,
Gama MTD1, Arruda MJC1, Domingues RB2, Kuster GW1
• 1HOSPITAL PAULISTANO; 2CHRU, POLE NEUROLOGIE,
UNIVERSITÉ LILLE 2, LILLE, FRANCE
Background: Stroke is the leading cause of mortality in Brazil. Nevertheless, few brazilian papers evaluated epidemiological data related to
the disease and the impact of record and management protocols for better clinical outcome.
Objective: Describe the epidemiological profile of patients affected by stroke in a primary
stroke center (Joint Commission International
certified), evaluating the treatment used and its
outcome. Methods: Protocols were instituted for
patients with acute cerebrovascular disease in
a private hospital in São Paulo, Brazil. The data
of all patients consecutively admitted between
January 1, 2012 and July 31, 2014 were prospectively collected. Results: During this period, 401
patients were followed up (mean age, 65.24 years
old; 52.11% males). One-hundred and twenty
(29.41%) patients arrived at the hospital within
4.5 h after the symptom onset. Mean NIHSS score
was 5.7 (range 1‐38) at admission. The average
time from hospital admission to neuroimaging
was 23.61 minutes among the group of patients
who arrived before 24 hours. Ischemic stroke
occurred in 247 (58.11%) cases; subarachnoid
hemorrhage, in 91 (21.41%); intraparenchimal
hemorrhage, in 53 (12.47%); and transient ischemic attack (TIA), in 34 (8%). Thirty six (14.5% of
the patients with ischemic stroke) were treated
with thrombolytic therapy, either intravenously
(n=23; at a median door-to-needle time of 68.13
minutes) or by intra-arterial techniques (n=13;
median door-to-needle time of 143.37 minutes).
At hospital discharge, the mean NIHSS score
among patients with ischemic stroke was 4.87,
and 207 (66.13%) patients had scores <2 on the
modified Rankin Scale. The mean length of stay
was 9.46 days, and the overall in-hospital mortality rate was 8.7% (n=35). Conclusion: The record
and management protocols represent an useful
tool for stroke patients evaluation. The obtained
data allow inform that the hospital has shown a
care profile that is similar to stroke units in developed countries. Besides the door to needle time
must be improved.
P-176
STROKE ETIOLOGY AND DIAGNOSTIC EVALUATION:
ARE WE PAYING ENOUGH ATTENTION TO WOMEN?
(YES, WE ARE.)
Pereira L, Rodrigues M
• SERVIÇO DE NEUROLOGIA DO HOSPITAL GARCIA DE
ORTA, ALMADA, PORTUGAL
Introduction: New guidelines on stroke in women highlight the importance of gender differences. Questions have been raised on equal access
to diagnostic tools. Objectives: Identify gender
differences on diagnostic evaluation and stroke
etiology in a Portuguese population. Method:
Retrospective observational study on ischemic
stroke patients admitted to a Portuguese Neurology department in 2 years. Demographic and
clinical data, diagnostic tools, and stroke subtype
(ASCO classification) were collected. We present
descriptive and inferential statistics with chisquare and binary logistic regression. Results:
We included 462 patients (64.5% men, median age 66 years). Women were older (p<0.001),
had more atrial fibrillation (AF) (p=0.001) and
smoked less (p<0.001). Diagnostic evaluation
comprised cranial CT/MRI imaging in 99.6%,
carotid study 93.5%, EKG/Holter 99.6%, transthoracic echocardiogram (TTE) 90.5%, and lipid
profile 96.3%. A complete study was achieved in
83.8%. We did not detect significant gender differences in usage of these tools, nor in complete
evaluations. However, older (>65years) women
with cardioembolic stroke had fewer carotid
evaluations compared to older men (76.6% vs
91.8%, p=0.04). There was a trend towards fewer
completed evaluations in older females (79.3%
vs 90.6%, p=0.07). Stroke etiology (ASCO grades
1+2) was cardiac source in 28.1%, atherosclerosis 23.8%, small vessel disease 18.6%, and other
etiology 5.0%. 32.9% had undetermined etiology.
Cardiac sources were more prevalent on women
(34.8% vs 24.5%, p=0.019), but after adjustment
for unbalanced risk factors the difference loses
significance, being AF the only predictor. Small
vessel disease was associated with male gender
XXVI Congresso Brasileiro de Neurologia
(21.5% vs 13.4%, p=0.033), even after adjustment
for lacunar stroke risk factors. The remaining
etiologies carried no significant gender differences. Undetermined etiology was equally found
in men and women (p=0.54), but was associated
with incomplete evaluation, because of fewer
TTE (p<0.001). The major cause for incomplete
evaluation was mild stroke (NIHSS≤5 63.3% vs
44.3%, p=0,005). Conclusion: Contrary to previous reports there was no gender discrimination
on the diagnostic evaluation or its completeness.
However, attention must be paid to older women with diagnosed AF who aren’t getting further
investigation because of causality attributed to
AF. Less disabled subjects were frequently discharged without complete evaluation or established etiology.
P-177
STROKE KNOWLEDGE IN THE POPULATION OF
SÃO BERNARDO BASIC HEALTH UNIT (BELO
HORIZONTE, BRAZIL)
Magalhães D1, Silva LS2, Machado FAS1, Monteiro CS1,
Chagas ATC1, Oliveira LG1, Andres L1, Silva ACF1,
Meira FCA1
• 1UNIFENAS; 2UFV
Introduction: cerebrovascular disease represents
1/3 of all deaths attributed to the circulatory system in Brazil. Recent data sets stroke as the leading cause of death in the country (accounting
for 9.2% of the total death causes). Knowledge
on stroke‘s clinical manifestations and risk factors are very poor. Objectives: To assess stroke
knowledge (risk factors, clinical manifestations
and management of stroke) of the population
assisted by a basic health unit (BHU) in Belo
Horizonte. Methodology: individuals assisted
by the Sao Bernardo BHU (8,343 inhabitants,
2,756 residences), in Belo Horizonte-MG, were
interviewed between February and July/2014.
After demographic data collection, they were
asked to watch a video that consisted of a person presenting stroke signals (facial asymmetry,
superior limb weakness and dysartria) and they
were asked to answer what was the condition
shown on the video. Afterwards, they answered
a semi-structured questionnaire to evaluate their
stroke knowledge. Results: 703 people were interviewed (62.1% female, mean age 46.7 years,
47.8 with less than 8 years of study). The recognition of a person having a stroke on the presented video was achieved by 56.1% of the subjects
(34.4% male and 65.6% female [p=0.029]). Family
history of stroke also increased the odds for correctly identifying the condition showed on the
video (p<0.001). The most commonly mentioned
clinical manifestations of stroke were weakness
(34.7%), disturbed speech (31.6%) and facial
asymmetry (23.6%). The risk factors for stroke
that were most named were “unbalanced diet”
(42.3%), hypertension (33.7%), lack of physical
activity (28.3%) and emotional stress (24.0%).
The majority of the subjects would call the emergency medical system (EMS) (66.9%), 17.6%
would go to a hospital and 6.9% would seek help
in other levels of the health care system. The
correct EMS telephone number was mentioned
by 46.6%, whereas 27.6% did not know which
phone number to call. Conclusion: female sex
and family history of stroke increased the odds
of recognizing the condition shown on video as a
stroke. The clinical manifestations most cited are
the most meaningful, but they were mentioned
by only a small part of the sample. The majority
of the studied population would have the best
attitude in the presence of someone having a
stroke, but knowledge of the EMS telephone
number was low.
P-178
STROKE MIMICS IN A BRAZILIAN STROKE CENTER.
CLINICAL ASPECTS AND PROGNOSIS
Nones DP, Ducci RDP, Novak EM, Zetola VF, Lange MC
• UFPR
Introduction: Stroke mimics is diagnosed in
less than 2% of patients submitted to intravenous thrombolysis (IVT), and previous studies
demonstrated a reduced risk of complication
associated to these patients. Authors present the
clinical aspects and prognosis of stroke mimics
patients admitted in a Stroke Unit in the IVT
time window. Methods:Retrospective analyses
of all patients admitted in the Stroke Unit of the
HC-UFPR between October 2012 and September 2013 as acute stroke with less than 4 hours
of symptoms onset. Stroke mimics was defined
as other disease than stroke (ischemic, hemorrhagic or TIA) after complete evaluation, including brain MRI. Results:In the study period, 253
patients were admitted to the Stroke Unit of the
HC-UFPR, from these, 140 (55%) cases arrived
in less than four hours from symptoms-onset.
Ten (7.1%) of these patients had a final diagnosis of stroke mimics. Six (60%) were functional,
one (10%) had Todd paresis, one (10%) had exogenous intoxication, one (10%) had diabetic ketoacidosis and one (10%) had clinically isolated
syndrome. Six (60%) were female, mean age of
43.1±16.2 years-old. Hypertension and dyslipidemia were presented in 4 (40%),diabetes mellitus in 2 (20%) and previous seizure in one (1%).
Motor symptoms were presented in 9 (90%) of
patients; dysarthria and sensitive symptoms in
6 (60%); cortical symptom (negligence) and gaze
paresis in one (10%). The median admission, 24
hours and seven days NIHSS was 7.5, 2.5 and 0.5.
From all patients, six (60%) were submitted to
IVT without any complication. On discharge, all
10 patients presented modified Rankin score less
than 3, and 5 (50%) were mRS =0. In functional
patients, only 20% were mRS zero. Conclusion:
7.1%(10) of the patients admitted to the stroke
unit of the HC-UFPR with less than 4 hours had a
final diagnoses of stroke mimics, between them,
most of the cases were functional (60%). The female gender was more affected (60%) and the
mean age of the symptoms onset was about 43.1
years old. The associated disease most found
between those patients was diabetes mellitus
(40%) and most of them had motor (90%) and
sensitive (60%) symptoms. The IVT did not bring
complications and all patients had rankin score
modifications on discharge. Discussion: more
research is needed to improve the diagnosis and
the treatment of Stroke mimics. Also, is necessary
to understand further complications.
P-179
STROKE OF POSTERIOR CIRCULATION: WHAT IS
THE THERAPEUTIC WINDOW TIME? - CASE REPORT
Ferreira MFS, Aguiar MS, Rebello LC, Sousa MWG,
Guiotti MG, Olveira AJB, Leão PAL
• HOSPITAL DE BASE DO DISTRITO FEDERAL
Introduction: Cerebrovascular accident (CVA)
of posterior circulation corresponds to 20% of
ischemic strokes being thrombosis and occlusion of the basilar artery accounted for only 3%
of them.The mortality of the lesion by basilar artery occlusion is greater than 75%.Since started
the treatment of acute ischemic stroke there is a
question if the therapeutic window for lesion of
circularion posterior would be the same set up of
the anterior circulation:4.5h.This report presents
a case of posterior circulation ischemic stroke,
which was treated with mechanical thrombectomy. Objective: Report a case of a basilar artery ischemic stroke submitted to a treatment
with mechanical thrombectomy with the Solitaire. Case: Male,70y,admitted to the emergency
with a history of having been found by his wife
fallen out of his bedone hour before admission
to.He had been seen for the last time at 23 pm
the previous day.On admission the patient was
drowsy,miotic pupils and double hemiparesis.
TC showed hyperintesity of the basilar artery.
He was intubated due to progressive neurological worsening(NIHSS26),sent to MRI of the
brain which showed isquemic injury in bridge
in sequence spread with preservation in FLAIR
sequente.The patient was refered to hemodynamic service where the occlusion of basilar
artery was confirmed and being made intraarterial infusion of rtPA associated with mechanical
thrombectomy with Solitaire.He was observed
on the fifth day of hospitalization clinical improvement with spontaneous eye opening and
active mobilization of the four members. He
persisted as soon as he go away of the hospital
with mild ataxia and dysarthria getting NIHSS3
and Rankin2. After seven months of the event,
in ambulatorial folclore up, patient present
complete resolution of neurological symptoms:
NIHSS:0,Rankin:0. Discussion: The time is the
main modifiable factor in a stroke,and the interaction with a favorable outcome was studied by
the researchers of the major clinical trials with
alteplase(NINDS,ECASS
I,II,ATLANTIS),concluding that the result is directly related to the
precocity ir the treatment.However,have been
questioned the real therapeutic window for the
different types of stroke. For this,there is a growing tendency to analyze each case individually,
prioritizing criteria of image for better therapeutic approach.The case presented here shows a
case of complete neurologic outcome for a patient with a potentially high mortality rate which
was treated after 13h of onset of condition neurological begin.
P-180
STRUCTURED TELEPHONE FOLLOW-UP OF
PATIENTS DISCHARGED AFTER STROKE: A LONGTERM QUALITY IMPROVEMENT PROGRAM
Escobosa DM, Bizutti CCG, Marques FRB,
Miranda RCAN, Vaccari AMH, Massaud RM,
Cendoroglo Neto M, Silva GS
• HIAE
Stroke is the most important cause of death
in Brazil a major cause of disability all over the
world. The follow up of patients with Stroke is
important in order to detect recurrence, as well
as to measure the impact of acute treatments in
the long-term follow up. Objective: Our objective
was to describe the implementation of a program
of long term follow up (“célula de desfecho”) of
patients admitted with acute stroke in a private
tertiaty center in Brazil, as well as the difficulties in implementing this follow up. Methods: A
group of nurses were trained in application of the
modified Rankin Scale using telephone contact
as well as in the delivery of a structured questionnaire containing questions regarding stroke
recurrence and use of medications. We describe
the results of the follow up of patients admitted
to our hospital from May 2011 to June 2014. A
telephone interview was planned 30, 90, 180 days
85
Posters
and one year after hospital discharge. Results: A
total of 345 patients were discharged home after
a stroke in this time period. Only 211 patients
(61%) responded to the first telephone interview (30 days after discharge), 190 (55%) in 90
days, 169 (48.9%) in six months and 106 (30.7%)
in one year. The main reasons for loss of follow
up were: lack of answer to telephone contact and
hospital readmissions at the time of the contact.
Conclusion: A structured follow up of patients
discharged after a stroke is feasible, however alternative Methods: of personal contact such as
email and protected digital social networking
should be further evaluated in order to improve
adhesion to the program.
P-181
SUBARACHNOID HEMORRHAGE ASSOCIATED WITH
MYCOTIC ANEURYSM IN A PATIENT AFFECTED BY
BACTERIAL ENDOCARDITIS
Albino PHC, Bresciani AP, Aranha FG, Sillero RO
• SOS CARDIO
Introduction: Mycotic aneurysm is a rare complication of septic emboli and can be a cause of
subarachnoid or parenchymal hemorrhage. It’s
associated with significant morbidity and mortality. Objective: To report a case of subarachnoid
hemorrhage associated with mycotic aneurysm
in a patient affected by bacterial endocarditis.
Case report: A 56 years old male, diabetic, admitted with signs of malaise, asthenia, weight loss
and fever. He had underwent dental procedure
four weeks before admission, but not performed
prophylaxis properly. Exams showed bacterial endocarditis with vegetations on the mitral
valve and moderate regurgitation. Blood culture
confirmed Streptococcus gordonii. Ten days later, already in use of ceftriaxone and garamycin,
he developed headache and paresis of the right
lower limb (RLL). Computed tomography (CT)
and transcranial doppler showed no abnormalities. Magnetic resonance imaging (MRI) of the
brain showed an acute ischemic lesion in the left
parasagittal region of the anterior cerebral artery
associated with moderate subarachnoid hemorrhage (SAH). Cerebral arteriography demonstrated infectious aneurysm in pericalosis left distal
artery without possibility of endovascular approach. Then underwent mitral and aortic valve
replacement after two weeks, progressing to
worsening of RLL paresis and mental confusion.
New CT confirmed signs of bleeding on the trunk
of the corpus callosum associated with right intraventricular bleeding and posterior horns of
both lateral ventricles, and subacute ischemic lesion in the left cingulate gyrus and reduced HSA
in middle cranial fossa. The patient continued
antibiotic therapy for six weeks, with a good evolution, still presenting paresis in RLL and mild
cognitive impairment. Discussion: Mycotic aneurysms designates an aneurysm caused by a localized bacterial or fungal inflammation of an artery, related to a septic emboli adhered to a vessel wall, generally in a distal location. The usual
pathogenic sequence is an embolic occlusion of
a small artery, for later rupture of the weakened
vessel wall, causing hemorrhage. However, this
patient presented clinical manifestation of ischemic stroke (RLL paresis) with SAH at the same
time. A consensus regarding the treatment has
not been established. Besides antibiotic therapy
for at least six weeks, in 30% of cases is still appropriate approach for arteriography.
86
P-182
SUCCESSFUL ENDOVASCULAR TREATMENT OF
DEEP CEREBRAL VENOUS THROMBOSIS
Uehara MK, Alves MAM, Resende HAC, Duarte LN,
Bezerra RP, Jorge FAV, Nalli DR, Bobotis VC,
Ribeiro PTM, Silva GS
• UNIVERSIDADE FEDERAL DE SÃO PAULO - UNIFESP
Introduction: Cerebral Venous Thrombosis
(CVT) is a serious disorder despite uncommon.
Treatment should be introduced as soon as the
diagnosis is established. Study objective: To Report a case of a successful endovascular treatment of CVT and the transcranial Doppler findings before and after treatment. Case report: A
31 years old woman was admitted to our service
complaining of progressive and severe headache,
associated with nausea and vomiting. A head CT
showed spontaneous hyperdensity in the straight
and superior sagittal sinuses, suggesting CVT.
She underwent immediate anticoagulation with
unfractionated heparin. Seventy-two hours after
admission, she presented a decreased level of
consciousness. In the neurological exam she was
somnolent, had eye opening to painful stimuli
and no verbal response. Her Glasgow Coma score
was 5 A Magnetic Resonance Imaging of the brain
showed hyperintensities on T2 and FLAIR in the
periventricular deep white matter, dorsolateral
portions of the midbrain, internal capsule and
basal ganglia, with symmetrical involvement,
as well as foci of low signal intensity on gradient
echo sequence in both thalami. TCD showed reduction of the cerebral blood flow velocities and
increased pulsatility index in both posterior cerebral arteries. Due to her clinical deterioration,
endovascular treatment was performed. She underwent mechanical thrombectomy and thrombus aspiration (Penumbra System), followed by
chemical thrombolysis with intra-sinuses rt-PA
(total 30mg), and the inflation of a balloon in
the left transverse and straight sinuses. Partial
recanalization of the left transverse sinus and
the deep venous system was observed after the
procedure. Forty-eight hours after the treatment,
the patient woke up. She was discharged home
with a mild right hemiparesis(modified Rankin
scale 2), using warfarin. A follow up TCD examination showed normalization of the blood flow
velocities and pulsatility index in the posterior
cerebral arteries. Discussion: Patients with CVT
and important clinical deterioration despite anticoagulation might benefit from endovascular
procedures.TCD showing increased pulsatility
index restricted to the posterior circulation that
improved after sinus recanalization suggests the
presence of a compartmentalized intracranial
hypertension. Further studies are warranted to
evaluate the role of endovascular procedures in
the initial treatment of patients with CVT and
very severe clinical presentation.
P-183
TACROLIMUS RELATED NEUROTOXICITY: BEYOND
THE ARTERIAL WALL CHANGES
Mousquer AL1, Tiellet NL1, Godoy MS1, Drago CP1,
Bonfanti V1, Barbosa JS2, Fiquene JC1, Oliveira JF1,
Siquineli F1
• 1HOSPITAL SANTA ISABEL; 2FURB
Introduction: Calcineurin inhibitor-induced encephalopathy is a possible secondary complication of immunosuppressive therapy used in solid
organ transplantation, whose main function is
to prevent graft versus host disease (GVHD). The
toxicity related to the use of tacrolimus may occur early or late and neurologic manifestations
are widely variable. Objective: To show the importance of neurological complications with
immunosuppressive therapy with calcineurin
inhibitors after kidney and liver transplantation.
Methods: report of four cases of neurological
complications secondary to the use of tacrolimus in a solid organ transplants unit from a tertiary
care hospital. First case: male patient with initial
frame of akinetic mutism; second case: male
patient with cerebellar tremor, medullary dysfunction and simple partial seizures; third case:
male patient who developed akinetic mutism
and complex partial seizures; fourth case: male
patient with cerebellar ataxia involving the trunk
(caudal vermis syndrome). Discussion: Severe
central nervous system toxicity from calcineurin
inhibitors has been rarely reported in solid organ
transplantation and is often misdiagnosed. Risk
factors and clinical outcome of remain largely
unknown. The reduction or a change in immunosuppressive regimen is the only means of clinical management. Every effort should be focused
on early detection to prevent the patient from
this life-threatening event, which is often associated with poor life quality.
P-184
TACROLIMUS-ASSOCIATED CEREBRAL
MICROBLEEDS – A NOVEL VASCULOPATHY
PATTERN AFTER LIVER TRANSPLANTATION
Tiellet NL, Mousquer AL, Godoy MS, Drago CP,
Fiquene JC, Oliveira JF, Siquineli F, Tiellet NL
• HOSPITAL SANTA ISABEL BLUMENAU
Introduction: Tacrolimus (FK‐506) is an immunosuppressive drug that is used mainly after allogeneic organ transplant to reduce the activity
of the host’s immune system and reduce the risk
of organ rejection. Side effects can be severe and
include viral infections, cardiac damage, hypertension, blurred vision, liver and kidney toxicity,
hyperkalemia, hypomagnesemia, hyperglycemia, diabetes mellitus, itching, lung damage and
various neuropsychiatric problems such as loss
of appetite, insomnia, Posterior reversible encephalopathy syndrome, confusion, weakness,
depression, cramps, neuropathy, headache, seizures, tremors, and catatonia. Objectives: Introduce the concept of a new pattern of vasculopathy associated with the use of tacrolimus as an
immunosuppressant after liver transplantation,
characterized by intraparenchymal bleeding and
signs of brain microbleeds in brain magnetic resonance imaging (MRI). Case report: White male
66 years old, with a history of diabetes mellitus
type 2, who underwent a liver transplantation for
cryptogenic cirrhosis and subsequent liver failure. There were postoperative complications by
portal vein thrombosis that led to moderate abdominal hemorrhage with the need for massive
transfusion. About 30 days after, he had a mild
headache vertigo, gait ataxia, right dysmetria
in the finger-to-nose test. A brain MRI showed
right cerebellar hemorrhage (dentate nucleus)
and multiple punctate foci of T2 hypointense
signal corresponding to hemosiderin deposits.
Discussion: Several authors have reported the
occurrence of acute and subacute neurovascular changes resulting from the use of tacrolimus
and other calcineurin inhibitors after solid organ transplantation. Reversible cerebral vasoconstriction syndromes (RCVS) are commonly
reported and often result in ischemic damage
to the brain with intracerebral hemorrhage can
occur in some circumstances. Some recent evidence suggests an immunogenic mechanism for
altered cerebral vasoregulation, based on toxicity
XXVI Congresso Brasileiro de Neurologia
and endothelial activation, vasoconstriction and
leukocyte trafficking, alone or in combination.
However, the pattern of cerebral microbleeds is
rarely reported as a vascular complication associated with the use of calcineurin inhibitors. We
believe that patients who use these agents should
be subject to adequate neuroimaging studies
and neuropsychological evaluation if clinical
signs that may indicate neurotoxicity occur.
P-185
TAKAYASU ARTERITIS: UNUSUAL CAUSE OF
STROKE IN YOUNG PATIENTS
Castro JPS, Paula JM, Pires LA
• UFJF
Intrduction: Takayasu arteritis (TA) is a relatively rare chronic systemic vasculitis that involves
aorta, its main branches and pulmonary arteries.
TA affects predominantly young individuals and
consists of an idiopathic granulomatous inflammation resulting in stenosis, occlusion, dilation
or aneurysm formation. Clinical features, such
as limb claudication, decreased or absent peripheral pulses, renal hypertension and coronary
artery disease reflect reduced blood flow and
ischemia. Neurological symptoms as dizziness,
visual disturbance, headache, seizures, transient
ischemic attacks (TIA) and stroke may occur.
Objective: Report a case of stroke in a patient
with TA presenting arterial stenosis and ascending aortic aneurysm. Case report: A 45-year-old
female patient, hypertensive, with aortic and
mitral regurgitation presented upper limbs claudication and recurrent chest pain for the last 30
years. After 19 years, she evolved with dizziness,
syncope and left hemiparesis. Physical examination showed no pulse in the left upper limb,
systolic murmur in right carotid artery, left disproportionate hemiparesis with brachial facial
predominance, hyperreflexia, left Babinski sign
and painful hyperesthesia in the left upper limb.
CT angiography of the cervical and thoracic vessels showed ascending aortic aneurysm and atherosclerotic calcification in the thoracic aorta;
dominant right vertebral artery in comparison
with the contralateral; left and right common
carotid arteries occlusions; retrograde flow in the
left vertebral artery. Brain CT demonstrated right
frontotemporoparietal hypodense lesion. Stroke
and TA were diagnosed according to criteria of
American College of Rheumatology. Secondary
prevention of cerebrovascular disease was started. Discussion: 10‐20% of patients with TA will
have a TIA or stroke. Some studies have revealed
premature atherosclerosis in TA patients, what
may lead to brain infarcts. Diagnosis in the early
phase can be difficult, resulting in chronic ischemic changes in the affected vascular territories.
Albeit uncommon in TA patients, stroke morbidity and mortality are significant.
P-186
THE COGNITIVE PROFILE OF SEVENTEEN
PATIENTS IN ONE STROKE UNIT OF FORTALEZA
Rocha FA, Frota NAF, Braga Neto P, Pitombeira MS,
Travassos FT, Rangel DM, Maia FM, Carvalho JJF,
Vieira LC
• HOSPITAL GERAL DE FORTALEZA
Introduction: Stroke is among top four causes
of death worldwide and it is the first one in Brazil. It is responsible for long term disability in
adults. The possible mechanisms are embolic,
atherothrombotic and lacunar. The latter one are
defined as cerebral infarcts with diameters up to
15mm. They are commonly located in the basal
ganglia, thalamus, internal capsule and brainstem. Vascular Cognitive Impairment (VCI) may
be due to large infarcts, strategic infarcts, multiple
infarcts and diffuse white matter disease. Classically, VCI have cognitive profile in which memory is preserved and executive function impaired.
Objectives: Assess cognitive changes in patients
with lacunar/subcortical and strategic ischemic
stroke. Methods: The Patients were evaluated at
Hospital Geral de Fortaleza’s Stroke Unit between
May and July 2014. The patients were submitted
to a careful neurological evaluation, including
the application of NIH Stroke Scale (NIHSS), and
a Magnetic Resonance Imaging. A brief cognitive battery was made with Addenbrook Revised
(ACE-R) scale and the memory subtest of figures
from the Brief Cognitive Screening Battery. Verbal Fluency was also performed. Results: The
average age was 45,58±17,24. All patients had
studied at least 4 years during life. Nine (52,9%)
had lacunar stroke: 4 in posterior circulation and
5 in anterior circulation. Ten (58,8%) had BAMFORD lacunar syndromes. The average of NIHSS,
MMSE, ACE-R was 1,52±2,18, 25,64±3,55 and
71,94±13,95 respectively. One patient had prior
Vascular Dementia another 4 (23,52%) had VCI.
Verbal Fluency was the evaluated domain more
committed, followed by Memory. We observed
a significant correlation between NIHSS and
ACE-R (r:‐0,527) and NIHSS and MMSE (r:‐0,504).
Conclusion: The vascular cognitive impairment
occurs even in patients with lacunar strokes with
variable intensity, mainly characterized by executive dysfunction. There is a correlation between
the NIHSS and cogniti ve performance.
P-187
THE USE OF ASTRAL SCORE TO ASSESS ISCHEMIC
STROKE OUTCOME IN A BRAZILIAN INSTITUTION
Miranda VAD, Kuster GW, Dutra LA, Cirino CP,
Cunha JLM, Azevedo DS, Albuquerque MVC, Broner T,
Cerqueira Filho RB, Brasil IP, Gama MTG, Volcov C,
Pacheco EP, Domingues RB
• HOSPITAL PAULISTANO
Background: The prediction of stroke outcome
may guide clinicians in clinical decisions. Several
ischemic stroke (IS) prognostic scales, including
ASTRAL score, have been proposed but none of
them have been previously evaluated in a Brazilian population. Objectives: To assess ASTRAL efficacy to predict in-hospital functional outcome
and mortality in São Paulo tertiary hospital population. Methods: We prospectively evaluated the
data of all IS patients assisted between 2012 and
2014 in the “Hospital Paulistano” in São Paulo
city. The ASTRAL score was calculated according
to its original version: [(A) age‐1 point for every 5
years+(S) severity-NIH score+(T) time of delay
being two points if >3h+(R) range of visual field
being two points if abnormality was present+(A)
acute glucose 1 point if lower than 3.7 mmol/L or
higher than 7.3 mmol/L+(L) level of consciousness 3 points if reduced]. The cut-off was set at
31. Modified Rankin scale (mRs≤2 and mRS>2) at
discharge and in-hospital mortality used to assess outcome. The statistical significance of ASTRAL to predict the two outcome measures was
assessed with chi-square test. We calculated sensitivity, specificity, positive and negative predictive value (PPV and NPV) of ASTRAL>31 to predict statistically significant outcomes. Results:
Three hundred and three IS patients were included in the study, being 52.1% male with mean
SD age of 66.7 15.6 years. ASTRAL did not reach
statistical significance for mortality (P=0.073).
ASTRAL was statistically significant to predict
mRS>2 (P<0.001). The sensitivity, specificity, PPV
and NPV of SP‐100 for mRS>2 were 36.1%, 97.3%,
84%, and 79.56%, respectively. Conclusions: ASTRAL was previously tested in Europe; however,
its reproducibility has not yet been sufficiently
tested, especially in a Brazilian population. The
only significant association of ASTRAL was found
with mRS>2, suggesting that this index worked
well as a predictor of functional outcome but
not mortality. An alternative explanation is that
the low in-hospital mortality rate (7.9%) has precluded the demonstration of any significant association of ASTRAL and mortality. ASTRAL>31
showed high specificity and high PPV to predict
mRS>2 indicating a very low percentage of false
positives. Therefore, our data suggest that ASTRAL>31 is good predictor of poor outcome in IS
Brazilian patients.
P-188
THE USE OF SPAN 100 INDEX TO ASSESS
ISCHEMIC STROKE OUTCOME IN A BRAZILIAN
INSTITUTION
Azevedo DS1, Kuster GW1, Dutra LA1, Cirino CP1,
Cunha JLM1, Albuquerque MVC1, Broner T1,
Cerqueira Filho RB1, Brasil IP1, Pacheco EP1,
Miranda VAD1, Gama MTD1, Arruda MJC1, Areco CV1,
Domingues RB2
• 1HOSPITAL PAULISTANO/PIAVEN (Programa
Integrado de Acidente Vascular Encefálico/ Amil);
2
UNIVERSITÉ LILLE 2, FRANCE
Backrgound: The prediction of stroke outcome
may help clinicians in clinical decisions. Several
ischemic stroke (IS) prognostic scales have been
proposed to predict outcome but they have not
yet been previously evaluated in a Brazilian population. Objectives: To assess SPAN‐100 (SP‐100)
efficacy to predict in-hospital functional outcome and mortality in São Paulo tertiary hospital
population. Methods: We prospectively evaluated the data of all IS patients assisted between
2012 and 2014 in the “Hospital Paulistano” in São
Paulo city, Brazil. The SP‐100 was calculated according to its original version (SP‐100=NIH score
+ age in years) and the cut-off was set at 100.
Modified Rankin scale (mRs≤2 and mRS>2) at
discharge and in-hospital mortality were used to
assess outcome. The statistical significance was
assessed with chi-square analyses. We calculated
sensitivity, specificity, positive and negative predictive value (PPV and NPV) of SP‐100>100 to predict statistically significant outcomes. Results:
Three hundred and three IS patients were included in the study, being 52.1% male with mean SD
age of 66.7 15.6 years. SP‐100 did not reach statistical significance for mortality (P=0.455). SP‐100
was statistically significant to predict mRS>2
(P<0.001). The sensitivity, specificity, PPV and
NPV of SP‐100 for mRS>2 were 18.97%, 99.32%,
91.67%, and 75.77%, respectively. Conclusions:
SP‐100 was previously used in North America. It
is a simple index and can be quickly performed;
however, its reproducibility has not yet been sufficiently tested, especially in a Brazilian population. SP‐100 showed statistically significance to
predict functional outcome but not to be predict
in-hospital mortality. It is possible that the low
in-hospital mortality rate (7.9%) has precluded
the demonstration of any significant association
of SP‐100 and mortality. SP‐100>100 showed high
specificity and high PPV to predict mRS>2 indicating a very low percentage of false positives.
Therefore, our data suggest that SP‐100>100 is
good predictor of poor outcome in IS Brazilian
patients.
87
Posters
P-189
THREE-MONTH POST STROKE EVALUATION
OF PATIENTS SUBMITTED TO THROMBOLYTIC
TREATMENT WITH ALTEPLASE AT HOSPITAL DE
BASE DO DISTRITO FEDERAL
Aguiar MS, Ferreira MFS, Sousa ACJ, Rebello LC
• HOSPITAL DE BASE DO DISTRITO FEDERAL
Introduction: Stroke is a major public health issue and the leading cause of death and disability in Brazil. Thrombolytic treatment with rtPA
has evidence level IA in reducing disability and
death among patients undergoing treatment.
The modified rankin scale (mRS) is a widely used
functional outcome scale that measures the degree of disability or dependence of patients who
suffered a stroke. Objective: To compare mRS
scores of patients submitted to thrombolytic
treatment with alteplase at hospital discharge
and at 3 month follow up. Methods: Sixty nine
acute stroke patients submitted to thrombolytic
treatment at Hospital de Base do Distrito Federal
from October 2013 to April 2014 were included in
the study. We compared the mRS score at hospital discharge and 3 month follow up. The patients
were classified into groups based on their scores:
No disability/mild disability (scores 0, 1 and 2),
moderate disability (score 3), severe disability
(scores 4 and 5), death (score 6). Results: Of sixty
nine patients initially included, nine lost follow
up and nine died during hospital stay, because
of brain hemorrhage after the treatment with
alteplase. Fifty one patients were evaluated at 3
month follow up. The mean age was 59,4 (31 – 89
years) and 28 patients (54,9%) were female. The
comparison of mRS scores at discharge and at
follow up showed statistical significant difference (p<0.05) with an increase number of patients with no or mild disability at 3 month follow
up (52,9% vs 58,8%) and a decrease number of
patients with severe disability (33,3% vs 25,5%).
Two patients (3,9%) died of infectious complications after hospital discharge. Conclusion: Although there was no control group in our study,
the majority of patients submitted to thrombolytic treatment had good functional outcomes
at hospital discharge and at 3 months follow up.
Future analysis should be made with a control
group in order to reaffirm the data findings in
thrombolysis related trials.
P-190
THROMBOLYTIC THERAPY FOR ACUTE STROKE AT
THE HOSPITAL DE BASE DO DISTRITO FEDERAL:
CASE SERIES
Valencia CEU, Rebello LC, Guiotti MG, Sousa ACJ,
Grippe TC, Maria PRS, Ferreira MFS, Dias RM,
Garbero RF
• HBDF
Background: Thrombolytic therapy in acute
stroke with intravenous recombinant tissue
plasminogen activator (rtPA) in the setting of a
Vascular Unit, was implemented in our center
in 2012. Since then, the number of patients for
stroke evaluation and consequently submitted
to IV thrombolytic treatment has been rising.
Methods: We retrospectively assessed the medical records of all patients admitted to our acute
stroke unit from the period of June 2012 to July
2014. We collected epidemiological and clinical
data to describe the profile and outcomes of the
patients submitted to intravenous thrombolysis. Finally, data from each year were compared
in order to describe the evolution of pre-hospital care. Results: One hundred and nineteen
88
patients were submitted to rtPA, of whom sixty one (51.3%) were males. The mean age was
59.5±1.4 years, the mean onset-to-treatment
time was 194.1±4.9 minutes, and the mean
door-to-needle time was 56.9±2.6 minutes. The
mean NIHSS score at admission aaas 13.1±0.6
(median 12). The most prevalent comorbidities
were hypertension (66.4%), tabagism (21.8%)
and diabetes (18.5%). Twenty five (21%) events
of haemorragic transformation (HT) were detected, but just fifteen (12.6%) were considered
symptomatic (HT-s). Decompressive craniectomy was performed in 7 patients (5.9% of the
sample). Seventy five patients (63%) were discharged to a Low-complexity unit after rtPA infusion. Our early-mortality rate was 6,7% (8 cases).
In fourteen cases (11,8%), the final outcome was
death. The percentage of HT-s dramatically reduced from 2012 (46.2%) to 2014 (7.3%), as well
as the mortality rate (25% to 8.3%). Mean age,
onset-to-treatment, onset-to-door, and door-toneedle time were not different among the years
analyzed (p’s>0,155). Conclusion: A considerable
number of patients were treated with rtPA during
the study period. The clinical profile is consistent
with other samples of brazilian population. The
HT-s rate in the three-year period was above the
expected. Our early and final mortality rate are
below expected. The onset-to-treatment, onset-to-door and door-to-needle time are within
expected. Overall, our data show the increasing
number of thrombolysis and the challenge it
imposes. They also suggest that, although the
pre-hospital care did not improve over the years,
the cumulative expertise with the thrombolysis
procedure has led to better outcomes.
P-191
THROMBOLYTIC THERAPY FOR ACUTE STROKE AT
THE HOSPITAL DE BASE DO DISTRITO FEDERAL:
VARIABLES ASSOCIATED TO BETTER OUTCOMES
Valencia CEU, Rebello LC, Guiotti MG, Sousa ACJ,
Aguiar MS, Sousa MWG, Duarte RL, Ferreira AGF,
Garbero RF
• HBDF
Background: Thrombolytic therapy for acute
stroke with intravenous recombinant tissue
plasminogen activator (rtPA) was implemented in our Vascular Unit in 2012. The cumulative
data of those years allowed us to investigate, in
a case-control analysis fashion, which variables
were associated to favorable outcomes. This
information will help us to improve the quality
of patient care. Methods: We retrospectively assessed the medical records of all patients admitted in our acute stroke unit from June 2012 to July
2014. We aimed to determine which variables
were associated (OR [IC95%]) to symptomatic
hemorrhagic transformations (HT-s), the need
of high-complexity (HC) care (ICU, surgery),
and fatal outcomes. Results: Twenty five (21%)
events of hemorrhagic transformation were detected, but just fifteen (12.6%) were considered
symptomatic. Twenty one patients (23.5%) were
transferred to HC facilities after rtPA infusion.
In fourteen cases (11,8%), the final outcome was
death. HT-s were more likely to occur in 2012
(46.2%) than in 2014 (7.3%) (OR 10.9 [IC95%
2.9‐40.8]). Patients that needed HC care were older (65.8±2.8 vs 55.7±1.6 yo; p=0.004). HC care was
more likely in the elderly (>60 yo) (OR 2,9 [IC95%
1.1‐7.7]), when the admission NIHSS score were
Severe-Very severe (OR 2.8 [IC95% 1.1‐7.4]), and
when HT-s occurred (OR 15.4 [IC95% 4.6‐51.5]).
The mean age was different between fatal outcomes and survivors (67.4±3.6 vs 55.7±1.7;
p=0.007). Fatal outcomes were more likely to occur in the elderly (OR 3.7 [IC95% 1.1‐12.2]), when
the patient had history of previous strokes (OR
4.1 [IC95% 1.3‐13.0]), and when HT-s occurred
(OR 18.5 [IC95% 5.2‐66.1]). Onset-to-treatment
time was not associated to the primary outcomes
analyzed. Conclusions: The global rate of HT-s
was higher than expected. However, there was a
remarkable improvement over the years. Nowadays, our rate of HT-s is near to the accepted rate
of 6%. The study design did not allow us to infer
causality between clinical variables and primary outcomes. Nevertheless, in this case-control
design, we could identify some variables associated to better outcomes in our sample. Those
variables were also identified in other case series. These results will enhance bedside decision
making, both prior and after IV rtPA.
P-192
THROMBOSIS OF THE TRANSVERSE AND SIGMOID
SINUS AS A MANIFESTATION OF PRIMARY
SJOGREN‘S SYNDROME
Silva JP1, Prudente DL1, Menezes BQ1, Braga TKK1,
Jucá Neto FOM1, Yoshikawa GT2, Fujihara S1
• 1UEPA; 2UFPA
Introduction: Sjogren‘s syndrome is an autoimmune disease that alters the physiology of the
exocrine glands. The most common symptoms
are dry eyes and mouth and both central and
peripheral neurological systems may possibly
have complications. Cerebral venous thrombosis
is a rare vascular disorder, representing only 1%
of strokes and has diverse clinical consequences. Objective: Report a case of a patient with
thrombosis of the transverse and right sigmoid
sinus as a manifestation of primary Sjogren‘s
syndrome. Case report: Female patient, 37 years
old, was admitted to the hospital referring headache similar to migraine with worsening of the
intensity and frequency in recent 30 days.. The
neurological examination was normal. In the investigation, the head MRI showed recent thrombosis of the transverse and right sigmoid sinus.
At that time, was started full anticoagulation
with low molecular weight heparin (enoxaparin). The only risk factor identified was the use
of oral contraceptives. Reported smoking in adolescence. Denied any infections before the onset
of described symptoms. Moreover, as personal
history, reported xerophthalmia and xerostomia,
and also that the ophthalmologist confirmed
dry eye condition by clinical examination. Given
this information the following laboratory tests
were performed: blood count with mild leukopenia (3,700); Standard PCR; Positive rheumatoid factor in high titers (128 IU / mL); negative
anti-DNA; Positive ANA 1/640 fine speckled
nuclear pattern; positive anti-Ro (240 U / ml);
negative anti-La; normal complement, protein
S decreased (44.1%); Determination of Protein
C: normal; negative anti-DNA; anti-SM; negative
lupus anticoagulant; electrophoresis of proteins:
gamma globulin peak (22.4%). Positive Schirmer test, compatible with mild to moderate dry
eye in both eyes. Currently, the patient has clinical improvement and warfarin 5 mg / day and
hydroxychloroquine 400 mg / day. Discussion:
The prevalence of neurological involvement
in Sjögren syndrome ranges between 8% and
70%. The central nervous system is involved in
1.5%–36%. CNS involvement may be represented by myelitis, vasculitis of the microcirculation,
associated with white matter lesions, aseptic
meningitis, and encephalopathy. Arterial stroke
has also been reported Cerebral vein thrombosis
XXVI Congresso Brasileiro de Neurologia
associated with Sjogren‘s syndrome is a rare condition and so far there are only two descriptions
in the literature.
P-194
P-193
Azevedo DS1, Kuster GW1, Dutra LA1, Cirino CP1,
Cunha JLM1, Albuquerque MVC1, Broner T1,
Cerqueira Filho RB1, Brasil IP1, Pacheco EP1,
Miranda VAD1, Gama MTD1, Arruda MJC1, Areco CV1,
Domingues RB2
TIME ON EFFECTIVE ANTICOAGULATION WITH
WARFARIN AT AN OUTPATIENT STROKE CLINIC
OF A PUBLIC TERTIARY ACADEMIC HOSPITAL IN
BRAZIL
Barreira CMA, Alves FFA, Rocha LJA, Libardi MC,
Santos RSA, Martins Filho RKV, Camilo MR,
Pontes Neto OM
• FMRP-USP
Introduction: warfarin is a vitamin K antagonist
widely used since the 1950’s both for prophylaxis
and treatment for thromboembolic conditions.
Its clinical use for stroke patients is supported
by several studies. Its dose regimen and efficacy
is influenced by many factors, such as vitamin
K intake, multi-drug interactions, liver metabolism and dysfunction. Therefore, the patient on
warfarin is frequently exposed to two extreme
situations: uneffective anticoagulation (with
possible thrombotic event) or hemorrhagic complications. The new oral anticoagulants (NOACs)
seem to have better efficacy and safer profile,
but their use is still not covered by the public
Brazilian health care system (SUS). Objectives:
to evaluate the percentage of time on effective
anticoagulation with warfarin among patients
at an outpatient stroke clinic; and to identify
the factors independently associated with poor
anticoagulation control. Methods: we reviewed
all consecutive consults to the anticoagulation
clinic of our Stroke Service during a 6-month
period, including patient’s demographics, comorbidities, reason for anticoagulation, warfarin weekly dose and International Normalized
Ratio (INR). Results: 183 patients were evaluated during that period with overall 881 appointments. 48,7% were men. The median absence
rate was 0,84±0,97 per pacient. The main indication for warfarin therapy was cardioembolic conditions (74,1%); stroke of undetermined etiology
accounted for 5,5% and cerebral venous thrombosis for 6,1%. The median weekly dose of warfarin was 33,8±18,2mg. 63,9% of patients hadt
wo consecutive INR measurements within the
therapeutic range goal. Only 31,3% achieved the
therapeutic goal within three consecutive INRs.
A cardioembolic etiology was independently associated with bad INR control (p=0,04). During
the study period, 6 patients switched from warfarin to NOACs. Conclusions: Warfarin oral anticoagulation is very labile and the optimal INR
range is achieved in only around 60% of the time
in a public tertiary stroke clinic in Brazil. The
identification of factors associated with poor anticoagulation status, such as lack of compliance
to treatment, dietary issues, and multi-drug interactions are critically important to optimize
chronic warfarin treatment success. A throughout Discussion about these aspects, including
cost effectiveness analysis of warfarin therapy is
required, as NOACs incorporation is being considered by the public health care system in Brazil.
TOTALED HEALTH RISKS IN VASCULAR EVENTS
(THRIVE) SCORE TO ASSESS ISCHEMIC STROKE
OUTCOME IN A BRAZILIAN INSTITUTION
• 1HOSPITAL PAULISTANO/PIAVEN (Programa
Integrado de Acidente Vascular Encefálico/ Amil);
2
UNIVERSITÉ LILLE 2, FRANCE
Backrgound: Previous studies suggested that
THRIVE is a simple tool to predict ischemic
stroke (IS) outcome, potentially guiding clinicians in clinical decisions. However, this score
has not yet been previously evaluated in a Brazilian population. Objectives: To assess THRIVE
efficacy to predict in-hospital functional outcome and mortality in São Paulo tertiary hospital
population. Methods: We prospectively evaluated the data of all IS patients assisted between
2012 and 2014 in the “Hospital Paulistano” in
São Paulo city. The THRIVE score was calculated
by assigning 1 point for an age of 60‐79 years, 2
points for an age ≥80 years, 2 points for an NIHSS score of 11 to 20, 4 points for an NIHSS
score ≥21, and 1 point each for hypertension, diabetes mellitus, and atrial fibrillation. Thus, the
THRIVE score could range from 0 to 9. According to THRIVE results patients were divided into
three groups: THRIVE 0‐2, THRIVE 3‐5, THRIVE>5.
Modified Rankin scale (mRs≤2 and mRS>2) at
discharge and in-hospital mortality were used
to assess outcome. The statistical significances
were assessed with chi-square test. We calculated sensitivity, specificity, positive and negative
predictive value (PPV and NPV) of THRIVE>31
to predict statistically significant outcomes.
Results: Three hundred and three IS patients
were included in the study, being 52.1% male
with mean SD age of 66.7 15.6 years. THRIVE did
not reach statistical significance for mortality
(P=0.142). THRIVE was statistically significant to
predict mRS>2 (P<0.001). The sensitivity, specificity, PPV and NPV of THRIVE>5 for mRS>2 were
17.24%, 99.32%, 90.91%, and 75.38%, respectively. Conclusions: THRIVE was previously tested
in China; however, its reproducibility has not yet
been sufficiently tested, especially in a Brazilian
population. The only significant association of
THRIVE was found with mRS>2, suggesting that
this score predicts functional outcome but not
in-hospital mortality. It is also possible that the
low in-hospital mortality rate did not allow the
demonstration of any significant association between ASTRAL and mortality. THRIVE>5 showed
good specificity and positive predictive value for
predicting mRS>2, indicating a very low rate of
false positives with THRIVE>5. These data suggest that THRIVE>5 is a good predictor of poor
outcome in a Brazilian IS population.
P-195
TRANSIENT GLOBAL AMNÉSIA DOCUMENTED BY
MRI : A CASE REPORT
Casagrande SCB1, Melo ACP1, Casagrande TC2,
Torrico VLO2, Galliano CT1
• 1HOSPITAL SERVIDOR PÚBLICO ESTADUAL DE SÃO
PAULO; 2HOSPITAL SERVIDOR PÚBLICO DE SÃO
PAULO
Introduction: Pathological impairment of memory is termed amnesia and occurs in association
with a variety of neurological conditions that
disrupt the brain in particular the hipoccampus
(medial temporal lobe). Syndrome of transient
amnesia presents a diagnostic challenge to
physician. The underlying mechanisms have
long been a mystery. Here we describe a case of
Transient Global Amnesia (TGA) documented
by neuroimaging. Objectives: To describe the
clinical case of a patient with transient global
amnesia, documented by magnetic resonance
imaging (MRI) of cranial , through research of
etiology and risk factors. Case report: A 62 y /o
female was seen in the emergency room with
signs of behavioral change and repetitive phrases. Patient does not remember how he returned
to her home and, due to delay their arrival and
apparent confusion, relatives took her to our service. The examination showed retrograde amnesia. CT of cranial and EEG was normal, Echocardiography showed mitral prolapse. Cranial MRI
done early showed focus of restriction of water
molecules‘s diffusion in the left medial temporal region (hippocampus). Patient improved the
symptoms and MRI was repeated in a few days
presenting disappearance of the signal previously seen. The patient was discharged with normal
neurological examination. Discussion: The TGA
is characterized by a sudden onset of transient
profound amnesia for a variable period of time,
but lasting less than 24h, generally occurring
over than 50 years without significant cardiac or
cerebrovascular disease. These episodes usually disappear completely, leaving rarely a partial
amnesia and sometimes a short period of permanent retrograde amnesia. There are transient
imaging findings usually involving the region of
hippocampus. The underlying cause of transient
global amnesia is unknown. There appears to be
a link between TGA and a history of migraines.
Etiologic factors are vascular, migraine, epilepsy
and psychogenic disorder. In patients with transient amnesia, careful assessment of the amnesic
deficit is mandatory to indentify treatable conditions. For diagnosis of exclusion, Methods: such
as MRI and EEG are used. Most authors consider
TGA a benign condition with a low risk of recurrence and of subsequent vascular events and
mortality. Diagnosis presence of such syndromes
can be challenging and their causes have been
debated for years and there are many differential
diagnoses that should be depart.
P-197
TUMOR NECROSIS FACTOR BETA NCOI
POLYMORPHISM (RS909253) IS ASSOCIATED WITH
INFLAMMATORY AND METABOLIC MARKERS IN
ACUTE ISCHEMIC STROKE
Parreira JS, Kallaur AP, Lehmann MF, Oliveira SR,
Frizon DA, Delongui F, Araujo MCM, Rossato C,
Almeida JT, Bragatto EF, Morimoto HK, Simão ANC,
Kaimen-Maciel DR, Reiche EMV
• UNIVERSIDADE ESTADUAL DE LONDRINA
Introduction: Polymorphisms in genes coding for pro-inflammatory molecules represent
important factors for the pathogenesis and
outcome of stroke. Objectives: The aim of this
study was to evaluate the relationship between
the TNFB NcoI (rs909253) polymorphism with
inflammatory and metabolic markers in acute
ischemic stroke. Methods: Ninety-three patients
and 134 controls were included. The TNFB polymorphism was determined using PCR-RFLP
with NcoI restriction enzyme. Stroke subtypes
and neurological deficit score were evaluated.
White blood cell counts, erythrocyte sedimentation rate (ESR), plasma levels of IL‐6 and TNFA,
serum high sensitive reactive C protein (hsCRP),
serum lipid profile, plasma levels of glucose and
insulin, and homeostatic model assessment of
insulin resistance (HOMA-IR) were determined.
89
Posters
Results: Stroke patients presented higher white
blood cell counts, hsCRP, ESR, glucose, insulin,
and HOMA-IR, and lower HDL cholesterol than
controls (p<0.01). There was no difference in
genotypic and allelic frequency of TNFB NcoI
polymorphism among patients and controls
(p>0.05). However, stroke patients carrying the
TNFB2/B2 genotype presented higher levels of
TNFA, white blood cell counts, total cholesterol,
LDL cholesterol, glucose, insulin, and HOMA-IR
than those with other genotypes (p<0.05). White
blood cells, IL‐6, hsCRP, and ESR were positively
correlated with the neurological deficit of the
patients (p<0.05). Conclusions: TNFB NcoI polymorphism, by itself, was not associated with increased susceptibility for stroke development.
However, the homozygous genotype for the allele
TNFB2 was associated with higher expression of
classical inflammatory and metabolic markers of
development and outcome of stroke than other
genotypes.
P-198
VASCULAR DEPRESSION POST-STROKE (VDPS):
CASE REPORT
Sousa RD, Iwamoto KOF, Almeida NDF, Caixeta LF
• UFG
Introduction: The term Vascular Depression
(VD) describes the depression associated with
cerebrovascular disease, accompanied by cognitive impairment, psychomotor slowing, executive dysfunction and lack of insight. In addition
to the risk factors for vascular disease and diffuse or multifocal cerebrovascular lesions in the
Neuroimaging exam. The pathogenetic mechanism of the disease results from a neuronal injury, especially in the frontal subcortical region
- affecting prefrontal cortico-subcortical circuits.
Among the therapeutic possibilities are tricyclic
antidepressants and selective reuptake inhibitors. Objective: To report a case of VDPS offering
an overview of the neuropsychological aspects
by means of Computed Tomography (CT), in addition to the therapeutic options. Methods: Ambulatory Monitoring of the patient, patient chart
review and literature search in the databases of
MEDLINE, PubMed and SciELO. Case report:
Patient, female, 61 years old, left dominance,
has hallucinations and amnestic syndrome following surgical clipping of cerebral aneurysm.
Refers Hemorrhagic Cerebral Vascular Accident
(CVA) in 2001 after pressure peak, and episodes
of epistaxis and hemiparesis in right hemisphere.
Evolved with retrograde amnesia after postoperative, working memory demoted, prosopagnosia, anhedonia, emotional lability, insomnia,
aphasia, lack of insight and social isolation. The
CT showed signs of temporal craniotomy on the
left, producing surgical clip artifact, and subcortical low attenuation area in the left temporal aspect sequelae. Showed improvement after
therapy with tricyclic antidepressants and selective serotonin reuptake inhibitors. Discussion:
The characteristics of VDPS resemble other depressive episodes and appear in three distinct
areas: affective, cognitive and somatic, which
are closely related to left frontal region and basal
ganglia. The clinical of the patient is compatible
with VDPS besides neuroimaging sequelae present in the left frontotemporal subcortical region.
Treatment with tricyclic antidepressants and selective serotonin reuptake inhibitors closes the
triad for diagnosis of VD, satisfactory treatment
in this case. Conclusion: VDPS is very common
and underdiagnosed. Knowledge about the
90
factors involved can assist in improving the therapeutic intervention and increase the diagnostic
accuracy.
Muscle Disorders
P-199
VERTEBROBASILAR STROKE DUE TO BLUNT
CEREBROVASCULAR INJURY:CASE REPORT
Laurentino M, Correia CER, Vasconcelos TF,
Godeiro Junior CO, Figueiredo MM, Dourado ME,
Melo CO, Morais PS, Costa MF, Marciano LA,
Medeiros LL
• UFRN
Although underdiagnosed, blunt cerebrovascular injury (BCVI) is potentially dangerous condition related to cervical trauma. Motor vehicle
crashes account for more than half of cases. The
anterior and posterior circulation can be both involved. BCVI is usually the result of a significant
force that twists or stretches the vessels, ending
in most cases with intimal tear. Objectives: To report a case of cerebellar and occipital ischemic
stroke followed by cervical trauma. Methods:
Male, 76 years old, victim of an automobile accident. Hours after injury, started presenting neck
pain and difficulty walking. Physical examination
revealed ataxic gait, and abnormal coordination
on the right side with dysmetria and decomposition of movement, most evident in lower limb.
The rest of the neurological examination was
unchanged. Cervical spine computed tomography (CT) was performed, showing fracture of
the lateral mass of C1 / C2 with bone fragment in
the right vertebral artery’s foramen. Brain Magnetic Ressonance (MR) showed ischemia in the
territory of posterior inferior cerebelIar artery
at right and occipital lobe at left. Cervical Magnetic Ressonance Angiography (MRA) revealed
irregularity in the contour of the right vertebral
artery, with intimal tear. Was opted for conservative treatment through the use of antiplatelet
(aspirin) and cervical immobilization. Currently,
the patient improved the symptoms and shows
a normal neurological examination. Discussion:
Neurologic complications of undiagnosed BCVI
remain problematic in trauma’s rotine. Approximately 1% patients hospitalized for trauma show
BCVI affecting carotid or vertebral vessels, which
the majority of them are diagnosed just after the
development of symptoms secondary to central nervous system ischemia, with a resultant
neurologic morbidity of up to 80%. Therefore, it
is mandatory to suspect of a BCVI after trauma
with involvement of the cervical spine. The main
risk factors associated are: diffuse axonal injury,
Glasgow ≤6, LeFort II/III or petrous bone fracture and cervical spine (CS) fracture. However,
the routine evaluation for BCVI is only indicated for a high-risk CS injury pattern: subluxation,
fracture extension into the transverse foramen,
and fractures within the upper CS (C1–C3). The
screening tests of choice are MRA or CT angiography and the treatment may be clinical with
antiplatelet agents or anticoagulation, or surgical
with endovascular approach, vascular anastomosis or thrombectomy.
P-200
A NOVEL ACTA1 MUTATION IN A PATIENT WITH
NEMALINE MYOPATHY
Martins CA, Abath Neto OL, Reed UC, Zanoteli E
• USP
Introduction: Skeletal muscle alpha-actin is the
major protein component of the skeletal muscle
thin filament, encoded by the ACTA1 gene. Mutations in ACTA1 are known to cause different
types of muscle disease, the most conspicuous of
which, nemaline myopathy (NM), is a congenital
myopathy characterized by protein aggregates
in the form of intracytoplasmatic, subsarcolemmal or intranuclear rods in the muscle biopsy.
More than 200 mutations have been described
in ACTA1, mostly inherited in a de novo fashion in sporadic patients. Objectives: We here
report a case of NM in which we found a novel
mutation in the ACTA1 gene. Case report: A 10
year old male, single child of unaffected nonconsanguineous parents, was hypotonic in the first
year of life and displayed delay to achieve motor
milestones, along with weakness of proximal
muscles. However, he steadily and progressively
improved his muscle function with rehabilitation, eventually developing all milestones and
being able to walk independently. Actually he
can climb stairs without support, stand up from
the floor but complain of frequent falls also can
not run or jump. He never had trouble to chew
or swallow but has difficulty in weight gain. He
started to present signs of respiratory disfunction, which has been demonstrated to be due to
restrictive ventilatory dysfunction (CVF=56%)
but has no frequent pulmorary infections. No
cardiomyopathy was detected. He has no contractures but is developing kyphoscoliosis. Cervical and distal limb muscle bulk and strength
are preserved. Sanger sequencing of the entire
length of the ACTA1 gene showed a heterozygous missense mutation in exon 4 (c.611C>T,
p.Thr204Ile), which is expected to be pathogenic according to multiple prediction tools, such
as PolyPhen‐2 and SIFT, and sits in a conserved
position. Subsequent sequencing of exon 4 in
the parents showed that the mutation is de novo,
fitting the family segregation. Discussion: This
novel mutation expands the spectrum of genetic
alterations that can be found in the ACTA1 gene
and confirms the relative intolerance of this gene
to all kinds of mutations.
P-201
ACTIVITY OF KREBS CYCLE ENZYMES IN MDX MICE
Hoepers A1, Ventura L1, Freiberger V1, Mina F2,
Dominguini D2, Scaini G2, Vainzof M3, Streck EL2,
Quevedo J2, Comim CM2
• 1UNISUL; 2UNESC; 3USP
Introduction: Duchenne muscular dystrophy
(DMD) is a degenerative disease of the skeletal
muscle caused by defects in the dystrophin gene,
which leads to respiratory or cardiac muscle failure. Most recently it was also demonstrated the
Central Nervous System involvement, where
XXVI Congresso Brasileiro de Neurologia
mitochondrial dysfunction and oxidative stress
may underlie the pathophysiology of DMD and
the Krebs cycle is the central point of oxidative
metabolism, providing carbon for biosynthesisand reducing agents for generation of adenosine
triphosphate (ATP). Objective: The present study
aims to evaluate the Krebs cycle enzymes activity
in the cortex, diaphragm and quadriceps of mdx
mice. Methods: To this aim, cortex, diaphragm
and quadriceps tissues from male dystrophic
mdx and control mice were used. Results: We
observed an increase of Malate dehydrogenase
activity in the cortex; an increase in the activity
of Malate dehydrogenase and Succinate dehydrogenase in the diaphragm; and increase in the
activity of Citrate synthase, Isocitrate dehydrogenase and Malate dehydrogenase in the quadriceps of mdx mice. Conclusion: In conclusion,
the present study shows the increased activity
of KC enzymes in cortex, quadriceps and diaphragm in the mdx mice. Thus, we suggest an
interaction between KC enzymes’ increase and
mitochondrial dysfunction in the mdx mice.
P-203
AGOMELATINE INCREASES MUSCLE
STRENGTH AND REDUCES THE EXPRESSION OF
INFLAMMATORY CYTOKINES IN MDX DYSTROPHIC
MICE
Delgado PO, Tondato VA, Lamnhuk LM, Gomiero FP,
Petri G, Alves B, Fonseca FLA, Carvalho AAS, Feder D
• FMABC
Objective: Based on the antioxidant and anti-inflammatory properties of melatonin in DMD patients we decided to study agomelatine action in
mdx mouse. Background: The new antidepressant agomelatine is an agonist of melatonergic
MT₁/ MT₂ receptors as well as an antagonist of serotonergic 5-HT2C receptors. Among the pathogenic mechanisms proposed for DMD, oxidative
stress and inflammation are directly involved in
the dystrophic process. Design/Methods: The
study was conducted in male mdx with life 69.5
days (average of 31‐114 days). The animals were
maintained with food and water ad libitum. They
were divided into two groups: Control(n=22), agomelatine (n=22). The first group received saline
by gavage and the second one received agomelatine 30 mg /kg / day by gavage. The animals
underwent treadmill exercise motorized 5 times
/ week, 18 cm / s for 10 min. Muscle strength
was measured weekly. After five weeks, the diaphragm muscle was removed. Gene expression of TGF beta 1, TNF, and osteopontin were
performed in muscle by real time PCR. Results:
Agomelatine’s group had a significant increase
in muscle strength after 5 weeks treatment. The
values of the measured cytokines were as follows:TGF beta1: Control (n=16)/Agomelatine
(n=15):1,42±0,12/ 1,32±0,17, p=0,05; TNF alpha:
Control (n=16)/ Agomelatine(n=15): 1,46±0,11;
1,36±0,16,p=0,04; Osteopontine: Control (n=16)/
Agomelatine
(n=15):
1,36±0,11/1,26±0,12,
p=0.02. Conclusions: The anti-inflammatory action of melatonin is accompanied by a reduction
of proinflammatory cytokines such as IL‐1b, IL‐6,
TNF-a and interferon (INF)-c in reducing oxidative stress and also increases IGF-I preventing
muscle atrophy in mdx mice castrated. The study
demonstrated that agomelatine reduce the expression of inflammatory cytokines and increase
muscle strength in mdx mice, which could be an
alternative option for the treatment of muscular
dystrophy.
P-204
ATYPICAL PRESENTATIONS OF LATE-ONSET
POMPE DISEASE: A DIAGNOSTIC CHALLENGE
Tavares Júnior JWL, Gondim FAA, Morais AA,
Melo Neto AP, Holanda MA
• UNIVERSIDADE FEDERAL DO CEARÁ
Introduction: Pompe disease, also known as
glycogen storage disease type II, is an autossomal recessive disorder, caused by deficiency
of acid α‐1,4-glucosidase, leading to intracellular glycogen accumulation, mainly in muscles.
Objectives: To describe 2 patients with atypical manifestations of the late-onset variant of
Pompe disease diagnosed at the Universidade
Federal do Ceará. Case report: Case 1: A 60 yearold man had complaints of exercise-induced
dyspnea since young adulthood. His condition
deteriorated over time and he developed pneumonia in January of 2012 followed by respiratory
failyre. He failed to wean mechanical ventilation,
spent several months in an Intensive Care Unit,
underwent tracheostomy and subsequently was
discharged home with marked diaphragmatic
weakness. Neuromuscular evaluation was requested for evaluation of possible diaphragmatic
paralysis. Neuropathy of the critical care patient
was suspected and electromyography was performed. EMG revealed generalized sensorimotor
neuropathy and myopathy. Persistent dyspnea
with myopathic gait led to suspicion of Pompe
disease, that was confirmed by blood and genetic
testing. Case 2: A 54 year-old man with history of
slowly progressive lower extremity weakness (especially proximal) since age 40 underwent EMG
testing that revealed myopathy and some neuropathic features. Marked anserine gait similar
to patient 1 and associated with forced forward
abdominal projection and chest retraction led to
suspicion of Pompe disease. Blood tests revealed
low alpha-glucosidase levels and a genetic tests
confirmed Pompe disease. Discussion: Late-onset variant of Pompe disease is a rare disease
with a wide variety of manifestations leading to
significant diagnostic challenges. In patient 1,
long-term ICU admission delayed diagnostic
suspicion. The possibility of treatment with enzyme replacement reinforces the importance of
an early diagnosis and need for high levels of suspicion in older patients with limb-girdle myopathy pattern and significant respiratory distress.
P-205
BILATERAL FACIAL PARALYSIS: A DIAGNOSTIC
CHALLENGE
Padovani ED, Twardowschy CA, Filla L, Tensini F,
Boschetti G, Barcellos I, Machado THS
• PUCPR
Introduction: Peripheral facial paralysis is a clinical entity with several possible etiologies; when
the etiology is unknown, the condition is known
as Bell’s palsy. It is characterized by the inability
to close one eye, disappearance of the ipsilateral
nasolabial fold, and deviation of the rima oris.
Most cases are unilateral and idiopathic, but bilateral cases tend to be secondary to neurological disorders, infections, traumas, neoplasms, or
metabolic disorders. The etiology is undefined
in 23% of the cases. Objectives: The differential
diagnosis of bilateral facial palsy is broad, and
the physician should be aware of the various
outcomes, considering that some of them are
fatal. Case report: A 35-year-old male was admitted to the emergency unit with paralysis of
the right side of the face. He had hypertension,
diabetes, and dyslipidemia; was under treatment
for depression; and had a history of gastroplasty
14 months previously because of morbid obesity
and had lost 72 kg since then. One month prior
to admission, he had consulted a general practitioner because of peripheral facial palsy of the
left side of the face. On admission, he presented
with bilateral peripheral facial paralysis, Bell’s
sign, slow speech, salivation, and bilateral facial
hypoesthesia. He remained hospitalized for 8
days, received acyclovir and prednisone, underwent speech therapy, and received eye care. Despite extensive investigation, it was not possible
to identify a precipitating factor for the symptoms, corroborating the fact that the etiological
diagnosis of bilateral peripheral palsy is often
challenging and complex. Discussion: Bell’s palsy is relatively common, with an incidence of
13–34 cases per 100,000, and the incidence rate
of the bilateral type varies between 0.3% and 2%.
In the latter, the symptoms are generally secondary to other diseases, including Guillain–Barré
syndrome, sarcoidosis, meningitis (infectious
or neoplastic), Lyme disease, idiopathic multiple cranial neuropathy, benign intracranial
hypertension, diabetes mellitus, syphilis, HIV
infection, mononucleosis, Melkersson–Rosenthal syndrome, Moebius syndrome, bilateral
neurofibromas, leukemia, and pre- or intrapontine tumors. Treatment includes addressing the
underlying diseases, and similar to the management of unilateral paralysis, corticotherapy with
acyclovir is indicated in most situations.
P-206
BIOPHOTOMETRY AND MANUAL GONIOMETRY IN
THE PHENOTYPIC EVALUATION OF MYOPATHIES
WITH NUCLEAR CENTRALIZATION
Rosa TS1, Iwabe-Marchese C1, Deloroso FT2, Jr MCF1,
Nucci A1
• 1UNICAMP; 2UFU
Introduction: Myopathies with nuclear centralization (CNM) are rare and heterogeneous diseases in clinical and genetic characteristics, and
were defined by muscle histopathology revealing
central nucleus in most muscle fibers. Objective.
To describe a refinement of phenotypic evaluation of patients with CNM, emphasizing postural
changes of the vertebral column, muscle retraction and hyperextension, expressed by angular
changes in joints. Methods: The gold standard
for patients‘ diagnosis was muscle biopsy. The
study enrolled 13 patients. We used: 1) Manual goniometry (n=13) techniques and reference
values according to Kapandji et al (2000). 2) Biophotometry (n=12): photos in anterior, posterior and lateral position of patient was take, after
demarcation of reflective points that were established in protocol; distance between the camera
and the patient was 3 meters. Photos were processed using specific software that measured
several selected angles. 3) Descriptive statistics.
Results: Postural abnormalities of the vertebral
column were observed in 12/12 patients, with a
prevalence of scoliosis (11/12) associated with
misalignment of shoulders (11/12); accentuation
of lumbar lordosis (9/12); valgus knee (8/12). Goniometry showed a reduction in amplitude for
cervical flexion, extension and lateral rotation in
13/13, most severe for rotations (8/13). In relation to shoulders, the largest angular reduction
occurred for abduction, adduction and extension (8/13), and the internal and external rotation showed slightly impaired of articular amplitude. Slight and symmetric retraction of the
elbow occurred in 8/13. In contrast, 5/13 showed
elbow hyperextension. Increased symmetric
91
Posters
wrist extension was noted in 3/13 patients. Two
patients had severe decrease of external rotation of the hip. Hyperextension of the knees was
seen in 3. Severe and symmetric decrease in ankle dorsiflexion occurred in 3 patients and was
mild and moderate in 9, indicating contraction
of plantar flexors muscles. There was only one
patient with normal amplitude of ankle dorsiflexion bilaterally. Flat feet was observed in 13/13
patients. Conclusion: The vertebral column was
affected at different severities by muscle disease
in all patients. Amplitude of cervical rotation for
both sides was limited. Retraction of posterior
muscles of the legs occurred in 100%.
P-207
BOTULISM: THE IMPORTANCE OF EARLY
DIAGNOSIS
Modenesi CA1, Megale MZ2, Leite JM1, Oliveira KA1,
Souza LC1, Adoni T2, Heliópolis H0, Bahia VS2,
Heliópolis H0
• 1HOSPITAL HELIÓPOLIS; 2FMUSP
Introduction: Botulism is a severe neuroparalytic desease, with sudden onset, caused by
bacteria Clostridium botulinum. Although it is
a rare disease(Brazil:5.2cases/year) has a high
mortality rate in the country. Goal: Documenting
the importance of early recognition of signs and
symptoms of Botulism and electrodiagnostic
study. Case report: Mrs.JSA,53,black,married,housewife,presented in emergency room from
Heliopolis Hospital complaining 1 week of mild
visual blurring, diplopia and eyelid parcial ptosis
in the left eye; on the 3rd day evolved into complete bilateral ptosis, hypophonia, dysarthria
and progressive dysphagia with mild dyspnea,
and proximal muscle weakness of the upper
limbs.On the 5th day of evolution:progressive
worsening of symptoms with disability to walk
by proximal weakness of the lower limbs.Concurrently, Mr.ASA, son of JSA, 31, black, single, lived
with his mother, began six days ago with diplopia, blurred vision, gradually evolving with dysphagia,aphony, proximal weakness of the upper
limbs and severe dyspnea with intolerance to lie
down. Exam:JSA- Bedridden,vigil,normal temporo-spatial orientation, dysarthric speech with
moderate hypophonia, proximal weakness of
the upper and lower limbs(grade 2),normal deep
reflexes,cranial nerves:complete bilateral ptosis,
mydriatic pupils, unilateral VI nerve palsy and
severe dysphagia.Preserved coordination and
sensitivity/ASA- Vigil, bradypnoea,normal gait,
temporo-spatial orientation,proximal weakness
of the upper limbs(grade 2),normal reflexes,bilateral parcial ptosis, mydriatic pupils,VI nerve
palsy and severe dysphagia with aphonia. Electromyography(FMUSP):Abnormal and early fatigability (myasthenic phenomenon) associated
with compound muscle action potential increment on repetitive stimulation in upper limbs,
reduction in motor amplitudes and preserved
sensory nerve conduction: consistent with a
pre-synaptic defect on neuromuscular junction.
Discussion: Considering patients who share the
same epidemiological history, with similar evolution of acute symmetrical descending paralysis,
afebrile, affecting cranial nerves, fixed pupillary
mydriasis and the absence of sensory symptoms;
should immediately consider the possibility of
botulismo.The diagnosis is confirmed by electrophysiological testing, identification of toxin in
serum and stool.The EDX study in patients with
suspected botulism provides a rapid and readily available method in patients whom bioassay
studies for botulinum toxin are pending.
92
P-208
CARDIOLOGIC FINDINGS IN NEUROMUSCULAR
DISORDERS
Chaves ÂV1, Miranda APP1, Mota VVDL1, Markman M2,
Markman Filho B2, Santiago GS2, Moser L3
• 1AACD - PE; 2PROCARDIO; 3CISAM
Introduction: Neuromuscular diseases are frequently associated with cardiovascular involvement and cardiac abnormalities and contribute
to a significant morbidity and mortality in these
patients. Objectives: To analyze the cardiac findings in a series of patients with neuromuscular
disorders from the out patient clinic of cardiology of AACD, Pernambuco. Methods: Analysis
of 103 patients treated in the clinic of cardiology
of AACD between April 2013 and May 2014, regarding clinical, electrophysiological and echocardiographic findings. Results: Between April
2013 and May 2014, in the out patient clinic of
cardiology of AACD-PE, 103 patients were attended suffering neuromuscular diseases. All the
patients were submitted to anamnesis, physical
examination and electrocardiograms. An echocardiogram was asked for all patients, as well as a
Holter for most of them. The age varied between
0 and 60 years (an average of 18 years), and 63%
were male. In this out patient clinic of cardiology, the prevalence of diseases in descending order is: Duchenne Muscular Dystrophy (24,5%),
Steinert’s Disease (Myotonic Muscular Dystrophy) (8%), Becker Muscular Dystrophy (4%),
Limb-Girdle Muscular Dystrophy (4%), Pompe
Disease (4%), Friedreich Ataxia (4%). From a
cardiovascular point of view, 28% of patients
are symptomatic, the most common symptom
being palpitations (15%). Regarding the electrocardiogram, 50% presented some form of alteration, from which the most frequent was sinus
tachycardia (34%), followed by left ventricular
hypertrophy (12%), and left anterior hemiblock
and first-degree atrioventricular block (12%).
Only 47% of patients returned with the echocardiogram, of which 35% showed some alteration.
The most common was the systolic dysfunction (14%), followed by ventricular hypertrophy
(12%). The results from Holter were inexpressive
since most patients did not succeed in having
the test taken. Conclusion: The cardiovascular
involvement is frequent in patients with neuromuscular disorders. These patients require early
cardiologic investigations and close follow-ups.
P-209
CENTRAL PAIN IN LONGITUDINALLY EXTENSIVE
TRANSVERSE MYELITIS WITH NO BRAIN
LESIONS: CLINICAL CHARACTERIZATION OF PAIN
SYNDROMES
Ciampi D, Silva FV, Pereira SA, Callegaro D,
Rodrigues ALL, Fernandes DTRM, Teixeira MJ
• HC-FMUSP
Aim of investigation: Pain in longitudinally extensive transverse myelitis (LETM) is frequent
but has not been clinically characterized. Also,
it is an appropriate model to assess pain mechanisms after spinal cord inflammatory lesions
since there are no brain lesions that could bias
pain assessment, cognition and mood assessment. It has been suggested that the prevalence
and severity of pain syndromes among patients
with neuromyelitis optica (NMO) and LETM
is greater when compared to those with multiple sclerosis, leading to a greater impact on
the health-related quality of life (HRQOL) and
in daily activities. It is not clear whether higher
scores in EDSS (expanded disability status scale)
could influence pain and HRQCOL scores. The
present study was aimed at assessing the correlation between disability, neuropathic pain
and quality of life in this population. Methods:
patients with spinal cord-restricted demyelinating disease (last relapse at least 12 months prior
to the evaluation) and complaint of pain underwent full physical examination (e.g. spasticity,
sensory level) and filled out the DN‐4 questionnaire (DN‐4), Brief Pain Inventory (BPI), Neuropathic Pain Symptom Inventory (NPSI) and SF‐12
Health Survey. Patients were classified as “at-level”, “above-level” and “bellow-level” pain. The
most severe pain was assessed. Results: Twenty
patients were included (15 female, 45.3±9.8 y.o.).
Twelve (60%) had the diagnosis of clinically defined NMO, while the remaining had exclusively
LETM. “At-level” pain was present in nine (45%),
and bellow-level in ten (50%). Thirteen (65%)
patients had more than one pain syndrome. In
the following analyses only the main pain was
considered.
P-210
CLINICAL ANATOMICAL AND EPIDEMIOLOGIC
STUDY OF 12 PATIENTS WITH MCARDLE’S DISEASE
Koch ME, Delgado PO, Fonseca FLA, Feder D,
Carvalho AAS
• FACULDADE DE MEDICINA DO ABC
The objective was to analyze clinically and
epidemiologically 12 patients with McArdle.
Background: Mc Ardle’s disease, also known as
Glycogenosis Type V was described for the first
time in 1951 by McArdle, It is an autossomic recessive disease, although it prevails in male sex.
It is the most frequent glycogenosis. It characterizes by the absence of the enzyme miofosforilase b in the muscle. The incidence of McArdle
is 1:100.000. It characterizes for the exercise intolerance, myalgia and painful cramping since
childhood/adolescence triggered by physical activity. Exercise may produce dark urine or even
a rhabdomyolysis. Methods: We have studied 11
patients with Mc Ardle’s disease from clinical,
laboratorial and anatomical-pathological view.
Results: 12 patients, 8 men and 4 women. 100%:
Symptoms since childhood/adolescence; intolerance to physical exercise: 100%: High CPK in
rest: 100%, from 4 to 22 times the reference value (less than 180); average age of diagnosis: 35,5
years; race: 88% white; ancestry: portuguese: 33%
and brazilian: 66%; cramps: 66%; myalgia: 50% ;
fadigue:100%, dark urine: 83,3%, muscle’s biopsy: 75%, absent of miofosforilase:100%; presence
of fosfofrutoquinase:100%. Conclusions: The
importance of the study refers to the fact that
McArdle is under diagnosis due to the genetic
and clinical heterogeneity. A significant number of McArdle’s patients are only diagnosed in
adult life, even though their symptoms/signals
have been present since childhood/adolescence.
This means the diagnosis passes by unnoticed in
the majority, leaving the patient subject to fatal
consequences.
P-211
CLINICAL AND HISTOLOGICAL FEATURES OF
BRAZILIAN PATIENTS WITH NEMALINE MYOPATHY
Martins CA1, Abath Neto OL1, Carvalho M1, Oliveira ASB2,
Fireman M2, Reed UC1, Zanoteli E1
• 1USP; 2UNIFESP
Introduction: Nemaline Myopathy (NM) is the
most common congenital myopathy, defined
by the presence of rods at the muscle biopsy. It
XXVI Congresso Brasileiro de Neurologia
shows a wide variability of clinical presentations,
ranging from lethal neonatal to mild adult onset
forms. The typical form manifests with neonatal hypotonia, motor developmental delay and
proximal muscle weakness. Other clinical features, such as distal and cervical muscle dysfunction, respiratory and cardiac involvement, as
well as skeletal deformities can invariably occur.
Moreover, the localization and distribution of
rods in the muscle biopsy is also heterogeneous.
Objectives: To describe clinical and histological
aspects of 14 Brazilian patients with the typical
congenital form of NM. Methods: After reviewing
muscle biopsy reports from two biopsy banks of
Sao Paulo, Brazil, we recruited patients to perform a full clinical assessment. Results: Fourteen
patients were selected by the presence of rods in
the muscle biopsy. They are regularly followed at
the muscle diseases outpatient clinics of FMUSP
and UNIFESP. Ten patients are male and 4 female, with ages ranging from 3 to 40 years old. All
had neonatal onset, mild to moderate proximal
weakness, hypotonia, facial paresis and normal
serum CK levels, matching classical descriptions
in the literature. Of note, however, all patients
had mild to moderate cervical flexor weakness,
and there was an enrichment of 10 patients in the
cohort with moderate to severe distal weakness,
characterized by impairment of feet dorsiflexion
and wrist extension. Seven patients had trouble
chewing, and 9 already have restrictive ventilatory dysfunction, 2 of which requiring intermittent noninvasive ventilatory support (BIPAP),
even if still ambulatory. No patient had cardiac
involvement. Skeletal deformities were detected
in 12 patients, most frequently kyphoscoliosis
and thorax wall collapse. Conclusions: This cohort follows classical descriptions of NM. It is
noteworthy that cervical flexors weakness, chewing difficulties, respiratory and skeletal involvement were early manifestations in this group of
patients. Awareness and vigilance to recognize
these aspects in advance helps active prevention
and improvement of quality of life in NM.
P-212
CLINICAL AND HISTOLOGICAL FINDINGS IN 12
PATIENTS WITH SPORADIC INCLUSION BODY
MYOSITIS (IBM)
Camargo LV, Estephan EP, Carvalho MS, Shinjo SK,
Zanoteli E
• FMUSP
Sporadic inclusion body myositis (IBM) is the
most common acquired muscle disease after 50
years of age. Although included in the inflammatory myopathies group, is a very distinct condition with a particular mechanism of muscle
degeneration characterized by aberrant protein
accumulation in muscle fibers. The disease is
clinically characterized by slowly progressive
asymmetric weakness of the finger flexors and
quadriceps muscles, and classically resistant to
immunosuppressive drugs. Objective: To present
clinical and histopathological findings in 12 IBM
patients. Methods: We analyzed clinical, laboratory and histopathological aspects of IBM patients using different diagnostic criteria. Muscle
analysis (15 muscle biopsies) included histological and histochemical reactions for all of them,
and Congo-red and immunohistochemical staining for lymphocytes (CD4, CD8), macrophages
(CD68), MHC-I, p62, alpha-synuclein, TDP‐43
and LC3B, for some of them. Results: Twelve
patients with IBM were included (male:female
ratio of 3:1), with medium age of 54,1±9,8 years.
Twenty five percent developed symptoms before
50 years of age and 66% before the 6th decade of
life. The medium time between initial symptoms
and the IBM diagnosis was 7,8±8,4 years. Nine
patients were previously diagnosed as polymyositis, but with pour clinical response. Dysphagia was present in 67% of cases. CPK levels were
between 1,35 and 8,62 times the upper limit of
normal value. The muscle biopsies showed in all
cases increased endomysial inflammatory infiltrate, rimmed-vacuoles and myopathic changes, such as increased connective tissue, nuclear
centralization, acid phosphatase reaction, and
fiber size variability. Only 2 biopsies presented
with mitochondrial abnormalities. Immunohistochemical study showed increase in tissue
pattern of CD68 and CD8, and variable increase
of MHC-I expression. All muscle samples tested
with anti-p62 (n=6), anti-α-synuclein (n=6) and
anti-TDP‐43 (n=3) showed positive reaction. Four
samples were tested with anti-LC3B, with 75% of
positivity. All samples submitted to Congo-red
stain showed β-amyloid deposits. Conclusion:
Our patients have similar clinical aspects described in literature with a strong positivity for
p62, alpha-synuclein, TDP‐43, reinforcing the degenerative theory in IBM pathophysiology.
P-213
DEVELOPMENT OF A FUNCTIONAL EVALUATION
SCALE FOR DUCHENNE MUSCULAR DYSTROPHY
AND A SOFTWARE FOR ITS APPLICATION
Martini J, Hukuda ME, Carvalho EV, Goya PSA, Voos MC,
Caromano FA
• UNIVERSIDADE DE SÃO PAULO
Objective: To develop a Functional Evaluation
Scale for Duchenne Muscular Dystrophy (FESDMD) and a software for its application (FESDMD-DATA). Method: The development of the
scale occurred in stages: observation and characterization of functional movements in 30 health
children and in 120 children with DMD; elaboration of the first version of the scale and its manual
and submission to 10 experts; readjustments and
presentation of the final version of the scale; inter and intrarater reliability analysis. After that, a
software for the application of FES-DMD was developed. The software offered score calculation
and reporting and organized database of reports
and films. Fifteen physiotherapists with expertise
in clinical practice with DMD analysed its usability. Results: Very good to excellent repeatability
and reproducibility (ICCs ranging from from 0.84
to 1.00) were demonstrated. FES-DMD analyzes
seven activities: sitting on the ground, standing
from the ground, sitting on a chair, standing from
a chair, climbing up steps, climbing down steps
and walking for 10 meters. The scale counts and
scores the compensatory movements. Higher
scores mean the child needs a higher number of
compensatory movements. The technical evaluation of the software contributed to improve its
layout and usability. Ten from fifteen physiotherapists strongly agreed that the software was usable in clinical practice, three agreed, while one
disagreed and one strongly disagreed. Regarding
the time needed for application, the software
made it faster than the paper and pencil former
version (p<0.001). Conclusion: FES-DMD is a
reliable instrument that allows the evaluation of
many functional activities in children with DMD.
FES-DMD-DATA is usable and requires less time
than the paper and pencil version, stores images and data securely, generates reports, and
may help physical therapists in clinical practice.
Descriptors: Physical therapy; Muscular dystrophies; Disability evaluation; Software validation.
P-216
EVALUATION OF BDNF, ENOLASE AND S100B IN
SERUM OF STEINERT MYOTONIC DYSTROPHY
PATIENTS
Comim CM1, Muradas TB1, Dominguini D2,
Mendonça BP2, Jeremias GC2, Camargo-Fagundes ALS2,
Gomes KM2, Cassol-Junior OJ1, Silva NC2, Quevedo J2,
Rosa MI2
• 1UNISUL; 2UNESC
Introduction: Steinert Myotonic Dystrophy
(SMD) is the most common form of adult muscular dystrophy and it is a highly pleiotropic
autosomal-dominant disease involving skeletal muscles, heart, lungs, gastrointestinal tract,
bone, skin, and central and peripheral nervous
system. The SMD are caused by mutations in
DMPK gene. Some studies show that SMD patients presented cognitive impairment, however, the mechanisms are still not clear. Objective:
Thus, the aim of this study was to evaluate the
levels of serum BDNF, Enolase and S100b, considered as biomarkers of brain injury, in SMD
patients. Methods: Participated in this study 11
patients from ASCADIM with diagnostic of SMD
and 11 control subjects pared by age and gender without neurological and neuromuscular
disease. Peripheral blood was collected to assess levels of BDNF, Enolase and S100b. Results:
SMD patients presented decrease levels of BDNF
when compared with control subjects. There
weren’t alterations in the Levels of Enolase and
S100b. Conclusion: The BDNF is responsible for
diverse functions in the adult brain as a regulator
of neuronal survival, fast synaptic transmission
and activity dependent synaptic plasticity. This
research showed the first evidence of BDNF involvement in the SMD patients.
P-217
EVALUATION OF CENTRAL NERVOUS SYSTEM
INVOLVEMENT IN THE ANIMAL MODEL OF
CONGENITAL MUSCULAR DYSTROPHY 1D
Silva ALS1, Ventura L1, Freiberger V1, Palmas D1,
Mendonça BP2, Dominguini D2, Steckert AV2, Scaini G2,
Vainzof M3, Streck EL2, Dal-Pizzol F2, Quevedo J2,
Comim CM1
• 1UNISUL; 2UNESC; 3USP
Introduction: Congenital muscular dystrophies
present mutated gene in the LARGE mice model
and it is characterized by an abnormal glycosylation of α-dystroglycan (α-DG), strongly implicated as having a causative role in the development
of central nervous system abnormalities such as
cognitive impairment seen in patients. However,
the pathophysiology of the brain involvement
remains unclear. Objective:Therefore, the objective of this study is to evaluate the oxidative
damage and energetic metabolism in the brain
tissue in the LARGE(myd) mice model of muscular dystrophy. Methods:With this aim, we used
adult homozygous, heterozygous, and wild-type
mice.Results: In summary, it was observed that
homozygous mice presented increased lipid peroxidation in the prefrontal cortex, hippocampus,
striatum, and cerebellum; an increase of protein
peroxidation in the prefrontal cortex, hippocampus, striatum, cerebellum, and cortex; a decrease
of complex I activity in the prefrontal cortex and
cerebellum; a decrease of complex II activity in
the prefrontal cortex and cerebellum; a decrease
of complex IV activity in the prefrontal cortex and
cerebellum; an increase in the cortex; and an increase of creatine kinase activity in the striatum
and cerebellum. Conclusion: This study shows
the first evidence that abnormal glycosylation
93
Posters
of α-DG may be affecting oxidative particles and
energetic metabolism thus contributing to the
memory storage and restoring process.
P-219
HIGH PERFORMANCE AT SPORTS: A NEW
PHENOTYPE FOR DYSFERLINOPATHY
Dourado Júnior MET, Santos HA, Calife ER, Galvão JS,
Silva TFV, Nascimento PRP, Jerônimo. SMB
• UFRN
Introduction: The regular practice of sports is
not commom in the history of patients diagnosed
with muscular dystrophies before the onset of
symptoms. Objectives: etermine the frequency
of sports praticing before the onset of symptoms
in a cohort study of 17 subjects, born and resident in Ouro Branco municipality, a city located
250 km from Natal, in the Eastern Seridó region,
suffering from Dysferlinopathy. Methods: a
questionnaire with yes / no answers was applied,
beyond the neuromuscular examination and
family history. The diagnosis of dysferlinopathy
wasmade based on phenotypic analysis and dysferlin expression in peripheral blood monocytes
by Western blot. Results: When analyzing family
pedigree we identified 45 individuals affected by
Muscular Dystrophy in 5 families. 17 patients out
of the 45 were clinically evaluated (ten females
and seven males). All patients examined revealed
consanguineous marriage of parents or grandparents. The mean age at onset of symptoms was
19.35±3,952 years old; The current mean age was
44±11.10 years old . Miyoshi phenotype was observed in 9 cases (52.94%) and a mixed pattern,
Miyoshi and waists, in 8 (47.05%). Eleven (64.7%)
reported sports activity before the onset of symptoms (football practice, voleybol, swimming,
running, rodeo). The patients diagnosed with
dysferlinopathy had no band corresponding to
dysferlin by western blot. Conclusion: More than
half of our patients (64.5%) reported sports activity before the onset of symptoms. This indicates
that they were not just normal muscle strength
but had excellent muscle strength before the onset of symptoms. This observation contradicts
the clinical course of other muscular dystrophies. It is believed that the presence of excessive
turnover of satellite cells early in the disease is
responsible for this phenomenon.
P-220
HYPERTHYROIDISM WITH NEUROLOGIC
APRESENTATION
Rangel DM, Travassos FT, Pitombeira MS, Rocha FA,
Maia FM, Frota NAF, Rodrigues CL, Borges ALP
• ESPCE
Introduction: Thyroid hormones play an important role in the skeletal muscle metabolism, being
associated to high levels of metabolism, protein
breakdown, and weight loss. Many neurological
diseases, as chronic or acute myopathy, periodic
paralysis, ophthalmoplegia and even myasthenia gravis, can be secondary to hyperthyroidism. Objective: Report the case of a patient with
hyperthyroidism and muscular presentation.
Report of the Case: Diabetic patient would have
presented, on 2013 february, pain in the right
upper limb, for which, after 6 months, made him
look for an orthopedist without improvement.
One month later, he evolved with migration to
the left upper limb, which lasted for 4 months,
evolving later with weakness in the 4 limbs.
Refers yet loss of 20kg in the last year, without
94
hyporexia, and for the last 4 months, dysphagia
to solids. Due to the weight loss, he was admitted
for investigation. On the exam, there were proximal tetraparesis with associated atrophy, with
normal reflexes and without other abnormalities. During the hospitalization, paraneoplastic
research was negative, CSF normal, CPK: 44,
eletroneuromiography with signs of motor recruitment, chronic neurogenic type on the proximal and scapula territory, with signs of sparse
denervation, however with atypical findings like
excess of short polyphasics. On an thyroid ultrasound, this one was larger, with the migration of
the inferior pole to the superior mediastinal, heterogeneous, like parenchimal disease. The thyroid hormones were TSH <0,004, T4L >6, and the
antibodies antiTPO >1000, antiTGB >3000, TRAB
>16. After methimazole, the patient improved
his strength. Discussion: 67% of the patient with
hyperthyroidism have muscular complaints. Although several studies emphasize the weakness
in the hyperthyroidism like myopathy, in DUYFF
et al, a prospective study, it was seen 52% of the
patients with symmetrical proximal weakness,
even though there were only equivocal signs
of myopathy on the electrophysiology test and
normal CPK. As has already been described, our
patient also presents severe and rapid weakness,
with improvement after hormonal adjustment.
P-221
IMPACT ON PRODUCTIVE CAPACITY IN ONES
AFFECTED BY MYASTHENIA GRAVIS
Barbosa FMB1, Seguti L1, Mateus SRM2, Reis MJF1,
Gava M3, Venâncio FNC4
• 1HOSPITAL UNIVERSITÁRIO DE BRASÍLIA;
2
UNIVERSIDADE DE BRASÍLIA - UNB; 3INSTITUTO
NACIONAL DO SEGURO SOCIAL; 4HOSPITAL DE BASE
DO DISTRITO FEDERAL
Introduction: Myasthenia Gravis (MG) is a disease of unknown etiology, with a prevalence of
1 to 4 in 100 thousand individuals. It is related
to gender and age, with two peaks: the first one
between second and third decades – affecting
predominantly women, and the other, between
sixth and seventh decades – affecting more frequently men. The core symptom is fluctuating
fatigue, which varies in intensity and location.
Diagnosis of MG, which is a chronic condition,
generally implies prolonged treatments and/
or surgical intervention, with clear impact on
productive capacity. Objective: To describe the
profile of individuals diagnosed with MG who receive benefits from National Institute For Social
Insurance (“Instituto Nacional de Seguro Social”
- INSS). Methods: We selected all welfare benefits that involved MG and other neuromuscular
diseases (CID G70 to G70.9), cataloged between
1999 and 2013. Information was collected by
reviewing medical reports from INSS (Distrito
Federal section), available in information systems of analogical (PRISMA) and digital (SUIBE
and SABI) origins. Variables analysed were: gender, age, occupation, prevalent symptoms and
type of benefit granted. Results: We selected 106
reports concerning CID of neuromuscular disease. Nearly 20% (20 from 106 individuals) were
included in CID G70.0, and had clinical and laboratorial description compatible with MG. The
majority were females (12 of 20). The age ranged
from 15 to 59 years for women, and from 33 to
71 years, for men. The age range more frequently
observed was the one between 40 and 59 years
(six patients). There was a predominance of
manual workers (janitors and general services
assistants). The most prevalent symptom was
muscle weakness (present in all males insured,
and in half of the females insured), followed
by: diplopia, palpebral ptosis and respiratory
impairment. There was a recommendation of
retirement for invalidity in 60% (12 of 20). Five
individuals were considered disabled and were
included in the continued disability benefits
(Benefício de Prestação Continuada - BPC). The
remaining (3) received critical illness insurance
for a long time. Conclusion:MG is a disabling
disease with individuals insured being predominantly females and manual workers. The absence in work for a long time, in early ages, and
the recommendation of retirement for invalidity
in productive age, show the negative impact on
quality of life for the individual affected, and the
elevated cost for society.
P-222
INFATILE POMPE DISEASE: ATYPICAL VARIANT
Rodrigues T1, Marrone CD2
• 1UFRGS; 2CLÍNICA MARRONE
Pompe disease (glycogen storage disease type II,
glycogenosis II, or acid maltase deficiency) is a lysosomal storage disorder in which an acid a-glucosidase (GAA) deficiency causes intralysosomal
accumulation of glycogen in all tissues, notably
skeletal muscles. Clinically, presents a wide spectrum of phenotypes, ranging from the severe and
rapidly progressive infantile onset form which
incorporates patients who display symptoms
before one year of age and cardiomyopathy . The
heterogeneous and more slowly progressive late
onset form, which develops symptoms after one
year of age and include the childhood, juvenile
and adult onset groups. This type typically presents respiratory insufficiency and no cardiac
manifestations. The infantile onset is classified
in classic infantile (presence of cardiomyopathy)
and atypical or muscular variant (without cardiomyopathy).We report a case of a boy who started
with respiratory insufficiency at birth, but the
diagnosis of Pompe disease was made only at 1
year and 5 months of age. He had oropharyngeal
dysphagia and multiple respiratory infections. At
1 year and 3 months he was transferred at UTI
of HMIPV because of complication of respiratory
infection and necessity of mechanic ventilation.
Neurological examination showed global hypotonia and arreflexia. He way submitted to an electromyography that showed a myopathic pattern,
a muscular biopsy with glycogen accumulation
and vacuolated fibers containing acid phosphatase positive material. DBS and DNA evaluation
confirmed the diagnosis. and an echocardiography with no cardiomyopathy, which distinct the
classic infantile form from the atypical infantile
form and late onset form. He started treatment
with Enzyme replace treatment (ERT) with 1 year
and 9 months and presented partial clinical enhanced. This case illustrate an atypical pattern
of infantile onset form in a child with symptoms
at birth and partial response to ERT. Early diagnosis and early treatment are both important
for patients with infantile onset form, especially in patients with atypical form, and ERT has
been associated with improved motor capability
and stabilized pulmonary function. Therefore,
Pompe disease is the first neuromuscular disorder with an FDA-approved treatment. The early
respiratory involvement and the characteristic
laboratory abnormalities in a myopathic patient
should include GAA deficiency in the differential
diagnosis.
XXVI Congresso Brasileiro de Neurologia
P-223
LATE-ONSET POMPE DISEASE: DIAPHRAGMATIC
PARESIS WITHOUT WAISTLINES INVOLVEMENT
Martinello TD1, Barbosa JS2, Boehringer AK2,
Betz RL1, Mousquer AL1, Tiellet NL1, Gonçalves MVM3,
Tomaseli P4, Fiquene JC1, Siquineli F1, Oliveira JF1
• 1HOSPITAL SANTA ISABEL DE BLUMENAU; 2FURB;
3
HOSPITAL REGIONAL HANS DIETER DE JOINVILLE;
4
HOSPITAL DAS CLÍNICAS DA FACULDADE DE
MEDICINA DE RIBEIRÃO PRETO
Introduction: Pompe disease is a rare lysossomal disorder of the glycogen muscle metabolism caused by deficiency of alpha-glicosidase.
Objective: single Case report of a patient with
late onset Pompe Disease. Case report: men, 49
years old, with low back pain for 4 years caused
by liposubstitution of paravertebral muscle as
documented by lumbar spine MRI. Progressed
with respiratory symptoms, dyspnea on exertion
and severe obstructive sleep apnea-hypopnea
syndrome (OSAHS) associated with mild disphagya and resumed to acute respiratory failure
with intubation and mechanic ventilation due
to respiratory muscle weakness. Electromyography, anti-acetylcholine receptor antibodies
and anti-musk were normal and finally a result
of alpha-glucosidase activity of 1.0 micromoles
/ liter / hour and two heterozygous mutations
(c.‐32‐13T> G and c.1064T> C p.L355P), confirmed
the diagnosis of Pompe disease. The patient is in
enzyme replacement, however keeping the pattern of diaphragmatic weakness and assisted by
tracheostomy for OSAHS. Discussion: Pompe
disease is an autosomal recessive inheritance
disorder characterized by accumulation of glycogen in muscle tissue that leads to progressive
weakness and loss of function and that presents
with a wide spectrum of clinical phenotypes, severity and progression. A common clinical manifestation is progressive neuromuscular disorder
in which respiratory symptoms can be one of
the earliest manifestations, which differs from
the other neuromuscular diseases. Other manifestations include proximal muscle weakness,
hypotonia and hypertrophic cardiomyopathy.
Enzyme replacement therapy is available and
has proven effectiveness in reducing the intensity and progression of the condition.
P-224
LENNOX-GASTAUT SYNDROME: SWALLOWING
INTERVENTION – CASE REPORT
Beneplacito F, Sato PH, Melo CB, Saconato M, Bittar A,
Gonçalves MIR
• UNIFESP
Introdution: The “Lennox-Gastaut Syndrome”
(LGS) is characterized by multiple types of seizures (tonic, atonic and atypical absence) and
cognitive and behavioral abnormalities. Includes
1% to 10% of childhood epilepsies, incidence of
1: 50.000 to 1: 100.000 children, and boy prevalence. Around 70‐80% of patients presents brain
damage, poorly brain formation, hypoxemia,
encephalopathy, meningoencephalopathy, neurocutaneous syndromes and rarely metabolic
syndromes. Children with SLG present mortality between 5 and 17% of cases between 12 and
21 years. Objective: To characterize swallowing
alterations in a patient with SLG. Case report:
RB, 30 years, male, diagnosed with of SLG, was
admitted with fever, cough and pulmonary sepsis. During the speech pathology assessment,
it were observed reduction of tone, mobility,
coordination and sensitivity of the lips, tongue
and cheeks, salivary stasis in oral cavity, with
risk of pulmonary aspiration and impairment
of cognitive function. Functional assessment of
swallowing was realized, offering to the patient
homogeneous paste consistency and it were observed: voluntary and not effective oral uptake,
increased oral transit time, inneficient managing
of the bolus, and inefficient and delayed triggering of swallow. These findings characterized a
moderate oropharyngeal dysphagia with Functional Oral Intake Scale Scale (FOIS 1). The initial
rehabilitation swallowing sessions used strategies and tools to promote oral motor control
and to reduce the delay of swallowing triggering.
Impaired cognitive function limited the choice of
techniques to improve strength and tone of the
speech organs. Indirect therapy was performed
with thermal tactile and gustatory stimulation;
direct therapy with volume control and clearance
maneuvers as multiple swallows helped patient
swallowing. The patient was discharged with exclusive oral feeding of paste and fine liquids diet
(final FOIS 5). Discussion: The patients in this
study had moderate oropharyngeal dysphagia,
with significant risk of pulmonary aspiration.
Despite the therapeutic limitations, management of swallowing, with consistency restrictions, volume controls and clearance maneuvers
contributed to a safe swallowing, with possibilitty of oral feeding.
P-226
MYASTHENIA GRAVIS, ACUTE FULMINANT FORM:
A CASE REPORT
Viana DCR, Araujo RV, Pinto TVL, Ferreira RC,
Christo PP, Santos MCV, Gomes Neto AP
• SANTA CASA DE BELO HORIZONTE
Introduction: Myasthenia Gravis (MG) is characterized by a change in nerve-muscle transmission, resulting from a deficiency, blockage
and destruction of acetylcholine receptors at the
neuromuscular junction. Usually it has quick
and effective response to cholinesterase inhibitor drugs, and the use of corticosteroids is directed to chronic immunotherapy. We present a case
of acute fulminating MG with quick response to
oral corticosteroid therapy. Objective: To report
a case myasthenia gravis, acute fulminant presentation (Osserman grade III), which showed
rapid response to treatment with high doses of
corticosteroids. Case report: E.A.R.A. male, 33
years old, previously healthy, presented in April
/ 2014 an episode of muscle weakness in the lower limbs that evolved in 24 hours, to tetraplegia,
involvement of the face and difficulty breathing.
Admitted to the ICU, the patient was treated with
oral corticosteroids at high doses, with fast response (about 48 hours) and partial recovery, but
significant muscle strength. Although respiratory
distress,mechanical ventilation was not necessary. Chest CT showed mediastinal mass suggestive of thymoma. Anti-acetylcholine antibodies
were negative, but the sample collection was
performed after patient‘s exposure to corticosteroids. Introduced pyridostigmine due to the diagnosis of “acute fulminant presentation” of Mistenia Gravis, while the progressive withdrawal of
corticosteroid was performed, with continued
clinical improvement. Programmed thymoma
resection. Discussion: Acute fulminant presentation of myasthenia gravis (Osserman grade III)
is rare, and frequently associated with thymoma.
The use of cholinesterase inhibitor drugs are the
rule, but in this case, the use of corticosteroids,
commonly used in chronic immunosuppression,
proved very useful, being responsible for the rapid improvement of fulminant presentation.
P-227
MYOPATHY AND EXERCISE INTOLERANCE
ASSOCIATED WITH THE M.3460G>A MTDNA
MUTATION: EXPANDING THE PHENOTYPE
PRESENTATION
Carvalho EHT, Brum D, Marques Junior W, Barreira AA,
Sobreira C
• FACULDADE DE MEDICINA DE RIBEIRÃO PRETO-USP
Introduction: Exercise intolerance is a frequent
symptom of patients with mitochondrial diseases and mutations in mtDNA ND2 and ND5 complex I protein genes. The m.3460G>A mutation in
the ND1 gene is usually associated with LHON.
Patients and Methods: We describe the clinical
and laboratorial findings of two related patients,
mother and daughter, followed at the neuromuscular outpatient clinic of a university hospital.
Further studies included muscle biopsy with
histochemistry, respiratory chain enzyme assays
in muscle homogenates, molecular analysis of
mtDNA. Results: Patient 1: a 47-year-old woman
presented with slowly progressive exercise intolerance since age 7 years, associated with hand
postural tremor, depression, chronic diarrhea
and a mild proximal muscle weakness. There
was no visual impairment although there was
a family history of male maternal relatives with
blindness. Laboratorial results disclosed normal
serum CK and elevated serum lactate after effort
(4,5mmol/l). Radioisotopic exam showed moderated delay of gastric emptying. Brain MRI showed
mild cerebral atrophy. Patient 2: a 21-year-old
young woman presented with slowly progressive exercise intolerance, associated with hand
postural tremor, mild muscle proximal weakness
and intestinal chronic constipation with vomits
and abdominal distention. Laboratorial results
disclosed normal serum CK and increased serum lactate after effort (4,0mmol/l). Pulmonary
vital capacity was reduced (77% of normal limit).
Radioisotopic exam showed moderated delay of
gastric emptying. Muscle biopsy from patient 1
showed signs of mitochondrial dysfunction in
rare fibers (rare COX+ ragged red fibers and rare
COX – fibers). Respiratory chain enzyme assays
disclosed complex I deficiency. Because of their
familial history of maternal relatives with visual
deficit, they were tested for the usual mitochondrial DNA mutations associated with LHON and
we found a homoplasmic m.3460G>A mutation
in the ND1 gene. Conclusion: the m3460G>A
mutation can manifest in woman with a phenotype where exercise intolerance is the main
symptom and should be tested in patients with
exercise intolerance, specially in woman with a
family history of visual loss.
P-228
NECROTIZING MYOPATHY ASSOCIATED TO HIV:
CASE REPORT
Ducci RD, Magalhães FB, Gomes-da-Silva MM,
Kay CSK, Lorenzoni PJ, Scola RH, Werneck LC
• UFPR
Introduction: Necrotizing myopathy could be an
acute or subacute event associated with myalgia,
proximal and symmetric muscle weakness, elevated levels of creatine kinase (CK), myopathic
pattern on electromyography and histopathologic analysis shows necrosis on muscular fibers
and regenerating fibers. This pathology can occurs after use of toxic agents, due to autoimmune
mechanisms or neoplasia. The aim of this report
is to demonstrate the acute onset of HIV with
necrotizing myopathy. Case report: A 20-yearold man had been well until 30 days before
95
Posters
admission when proximal progressive muscle
weakness, intense and diffuse myalgia and loss
of weight developed. Seven days after the onset
of these symptoms he came up with fever and
lymphadenopathy. On examination, there were
temperature of 37,8°C, lymphadenopathy, diffuse edema on limbs, specially proximally and in
upper limbs. On neurologic examination he presented with bilateral peripheral face palsy, nasal
voice, impaired palate elevation, proximal flaccid
tetraparesis (grade 3). Laboratory shows creatine
kinase (CK) 26,167 U/L (VR: 30‐200 U/L), LDH
1,534 U/L (VR: 125‐220 U/L), CD4 count: 300/UL
(33%), viral load: 50,027 copies (4,7 log). Other
serologies were negative. CSF analysis was unaltered. Electromyography performed on brachial
biceps revealed a myopathic pattern. The first
biopsy demonstrated denervation and active
myopathy. A MRI was performed to guide a new
biopsy which was typical of necrotizing myopathy. With the diagnostic of AIDS and necrotizing
myopathy, we started the treatment. Antiretroviral therapy, immunosuppressive therapy with
prednisone 1mg/kg/day and immunoglobuline
400mg/kg/day. The patient improved and after
one month presented undetectable viral load,
CD4 count 1,179/UL, CK 139 U/L. On examination a minor proximal muscular weakness (grade
4+). Discussion: Myalgia and weakness are common symptoms in HIV and are commonly associated to Zidovudine. Also, direct toxic effects
from the virus can damage peripheral and central nervous system. Generally those patients reports myalgia, fatigue, proximal muscular weakness, elevated CK levels and a myopathic pattern
on electromyography. There are no randomized
controlled trials to guide therapy on these cases,
but necrotizing myopathy and HIV myopathy
should responds to immunossupressive therapy.
There is a lack of information about the importance of HIV therapy for those patients and prognosis is uncertain.
P-229
NESPRIN-2 (SYNE2) GENE MUTATION CAUSING
AN EMERY-DREIFUSS MUSCULAR DYSTROPHY
PHENOTYPE
Abath Neto O1, Estephan E1, Martins CA1, Reed UC1,
Oliveira ASB2, Laporte J3, Zanoteli E1
• 1FMUSP; 2UNIFESP; 3UNIVERSITY OF STRASBURG,
FRANCE
Introduction: Emery-Dreifuss muscular dystrophy (EDMD) is a skeletal and heart muscle
disease characterized by multiple contractures,
muscle weakness in a scapuloperoneal distribution, and arrhythmias. It is most commonly
inherited in an X-linked pattern, in association
with the EMD gene, but there are autosomal
dominant and recessive forms as well, in which
the genes LMNA, FHL1, SYNE1 or SYNE2 have
been implicated. Objectives: We here show a
sporadic case with an EDMD-like phenotype
with an atypical muscle biopsy suggestive of centronuclear myopathy, in which we found a mutation in the SYNE2 gene (nesprin‐2), which had
previously been reported only once in EDMD.
Case report: A 22-year old male with unaffected
nonconsanguineous parents, born at term out
of an uneventful pregnancy, had motor developmental delay, having walked only after 2 and
a half years of age. His motor function then progressively improved, but he never achieved the
96
same level of skills of his peers, not being able to
run or ride a bicycle. At age 15, he started having
trouble to climb stairs, which improved after regular rehabilitation. On physical exam, he has hypotonia, hypotrophy and weakness in a limb-girdle and biceps-triceps distribution with distal
upper limb sparing, associated totoe walking due
to heel contractures. He also has prominent knee
and elbow contractures, but no facial or cervical
involvement. His serum creatine kinase level
was normal, as well as his heart exams. He had
a muscle biopsy sampled at age 17 in the deltoid
muscle, which showed a myopathic pattern with
slight increase in connective tissue, mild fiber
size variability, and numerous prominent centralized and internalized nuclei in the majority of
muscle fibers. In oxidative reactions, there were
both areas with increased activity and focal lack
of mitochondria. Exome sequencing identified a
known pathogenic mutation in the SYNE2 gene
(c.18632C>T, p.Thr6211Met). Further Sanger sequencing of parents demonstrated the mutation
to be de novo. Discussion: The mutation has
been reported only once, in association with an
autosomal dominant family with a phenotype of
EDMD, but with severe heart involvement. This
sporadic case with an EDMD-like phenotype and
no heart involvement may suggest the mutation
has variable expressivity, but the patient will be
nevertheless actively monitored with cardiac
exams. Of note, this is the only mutation so far
described in the SYNE2 gene in association with
EDMD.
P-230
PAIN IN FACIOSCAPULOHUMERAL MUSCULAR
DISTROPHY: SEVERITY AND INTERFERENCE WITH
DAILY ACTIVITIES
Cruz CA1, Toledo FO2, Santos ACJ1, Marques Junior W1,
Sobreira CFR1
• 1HC FMRP-USP; 2FMUSP
Introduction: Facioscapulohumeral muscular
dystrophy (FSHD) is an autosomal-dominant
disorder characterized by muscle weakness and
wasting in a rather specific distribution. The
muscles of facial expression, stabilization of the
scapula and proximal muscles of the upper limbs
are the most affected. In descriptions of FSHD,
pain is rarely mentioned as an important clinical
feature of the condition. Bushby et al., in 1998,
emphasized that pain was an under-reported but
significant symptom of FSHD; however, pain in
FSHD was the focus of only a few scientific studies since then. Purpose: Characterize the pattern
of muscle pain in patients affected by this disorder in terms of pain severity and interference
with daily activities. Patients and Methods: Sixteen adult patients with FSHD, whose diagnoses
were based on the presence of a muscular dystrophy with weakness of FSH distribution and
autosomal dominant inheritance, were recruited
in the neuromuscular disorders outpatient clinic
of our university hospital. A version of the Brief
Pain Inventory adapted to the Portuguese language spoken in Brazil (BPI-B) was applied, with
a focus on pain severity (4 items with maximum
possible score of 40) and pain interference in
daily life (7 items with maximum possible score
of 70). Higher scores indicate stronger pain and
higher interference in daily life. Correlation of
BPI-B scores with age, functional capacity (Vignos and Brooke scales) and response to pain
treatment were analyzed using the Spearman
correlation coefficient. Results: All 16 patients
reported pain as part of the clinical picture. Pain
severity scores ranged from 12 to 29 (mean of 21;
standard deviation [SD] of 5). Interference scores
ranged from 10 to 63 (mean of 37; SD of 16). No
correlation was found between age, BPI-B scores
and functional capacity. Pain treatment was unsatisfactory for the majority of patients; only 5
patients reported >60% improvement of pain.
Better responses to treatment seem to occur in
patients with a better functional capacity (r=‐0.53;
p=0.06). Conclusions: Pain is a frequent symptom in FSHD and it is probably undertreated. In
patients with slowly progressive neuromuscular
diseases, such as FSHD, chronic pain can increase the burden of the disease. Therefore, it is
important to standardize a therapeutic approach
to the treatment of pain in those patients.
P-231
POMPE DISEASE: DISTAL MYOPATHY IN A
PREVIOUSLY UNREPORTED HETEROZYGOUS
VARIANT
Amaral CBMS, Pires KL, Coutinho IBR, Farinhas JGD,
Pupe CCB, Nascimento OJM, Davidovich ER
• UFF
Objective: To report a previously unreported
heterozygous variant in exon 8 of the GAA gene
(c.1198G>A p.V400I) associated to a distal myopathy presentation. Background: Pompe disease
(PD) is an autosomal recessive, rare disease,
caused by a deficiency on lysosomal enzyme
alpha-glucosidase (GAA). It causes progressive
muscular weakness and may lead to respiratory
impairment. Presentation may occur as soon as
in newborns (early-onset) or later in children, adolescents or adults (late-onset). There are genotypic and phenotypic variations on disease. We
describe two cases (mother and daughter) with
previously unreported mutations and clinical
heterogeneity. Methods: Patient 1 is a 48-year-old
female presented with a 5-year disease progression that started with distal limb weakness, with
foot dorsiflexion impairment. She has recently
started to present dyspnea. Electroneuromyography (EMG) showed normal neuroconduction
and an unequivocal distal myopathy pattern.
Patient 2, her daughter (23 y.o.) presented dyspnea and no evident weakness at neurological
examination; a pericardic effusion was observed
at ultrasound examination. Parents are consanguineous. EMG findings suggested a proximal
myopathy. Both patients were tested genetically
for PD (CENTOGENE®). Results: Genotype for
both patients are presented: Patient 1 - Tandem
MS alpha-glucosidase (Pompe) - 1,7 μmol/l/h
(reference:>3 μmol/l/h). Gene sequencing GAA
- heterozygous variant (c.1198G>A p.V400I). Tandem MS sphingomyelinase (control) - 1,9 μmol/
l/h (reference:≥2 μmol/l/h). Patient 2 - Tandem
MS alpha-glucosidase (Pompe) - 2,7 μmol/l/h
(reference:>3 μmol/l/h) Gene sequencing GAA
- heterozygous variant (c.1198G>A p.V400I).
Conclusion: Our patients present the phenotypic heterogeneity of PD under the same genotypic
variation in the same family, being ours the first
distal myopathy Case reported. We also detected
a previously unreported heterozygous variant
in exon 8 of the GAA gene (c.1198G>A p.V400I).
PD is a rare, heterogeneous condition that may
mimic many neuromuscular conditions.
XXVI Congresso Brasileiro de Neurologia
P-232
PROFILE OF ISCHEMIC MODIFIED EXERCISE TEST
IN PATIENTS WITH MCARDLE‘S DISEASE AND
PATIENTS WITH IDIOPATHIC ELEVATED CREATINE
PHOSPHOKINASE
Delgado PO, Koch ME, Fonseca FLA, Pagura JR, Feder D,
Carvalho AAS
• FMABC
Introduction: McArdle‘s disease (Glycogen V) is
an autosomal recessive disorder. It is the most
prevalent glycogenosis. It is characterized by
the absence of the enzyme b myophosphorylase
in muscle causing exercise intolerance, myalgia and cramps since childhood / adolescence.
The ischemic exercise test was first described
to detect McArdle disease (DM), however, variants of this test has been performed (modified
ischemic exercise test) for patients with other
metabolic myopathies. The classic test of the
forearm requires the dosage levels of ammonia
and lactate in venous blood at rest and after 10
minutes of sustained contraction of the hand.
The majority of patients show a slight increase or
no increase in lactate and a normal or abnormal
increase of ammonia depending on the pathology. Objectives: Evaluation of the variation of the
modified ischemic test profile in patients with
DM and patients with idiopathic elevated CPK.
Materials and Methods: 2 groups were selected: 1: DM patients confirmed by muscle biopsy
(absence of myophosphorylase); 2-patients with
CPK≥150U / L with nonspecific muscle biopsy
and absence of myophosphorylase. The patients
who underwent the modified ischemic exercise
test in which there were four blood collections:
the first at home, one immediately after exercise
60 seconds, one minute and five minutes after, for lactate and ammonia. Results: Group 1:
12 patients with DM; 8 men and 4 women with
a mean age of 39.6 years. Group 2: 9 patients, 5
men and 4 women with a mean age of 41.4 years.
The increase in lactate was mild in group 1 (1.3 to
1,4mmol / L) and in group 2, we observed a significant increase in lactate levels (1.5 to 2.2 mmol
/ L) after exercise. Discussion: As expected, patients with DM did not show an increase in lactate after exercise, because due to the absence of
myophosphorylase, muscle glycogen cannot be
cleaved into glucose, preventing lactic fermentation. The second group showed no changes in
any specific condition, however we can rule out
the possibility of most muscle glycogenosis.
P-235
RARE PRESENTATION OF MITOCHONDRIAL
DISEASE WITH OPHTHALMOPLEGIA, DYSARTHRIA
/ DYSPHAGIA AND SENSORY ATAXIA: REPORT OF
TWO CASES
Silva LD1, Andrade ACA2, Pereira MTR2, Costa MCM1,
Andrade HMT2
• 1UNIVERSIDADE FEDERAL DE SÃO PAULO;
2
PONTIFÍCIA UNIVERSIDADE CATÓLICA DE CAMPINAS
Introduction: There are few cases describing
the association of ophthalmoplegia, dysarthria/
dysphagia and sensory ataxia in mitochondriopathies. These clinical features are linked to some
nuclear DNA (nDNA) mutations found on some
modalities of these syndromes. Objectives: To
report two rare cases of mitochondriopathy.
Case report 1: A 46 years old woman who had
a 16-year history of progressive altered balance
followed by bilateral ptosis. Five years ago she
experienced loss of gait, dysarthria and dysphagia. She had one sister with clinical similarities.
During clinical exam she presented dysarthria;
bilateral palate weakness; bilateral ptosis; motor
strength: grade (gd) 5 on proximal upper limbs
(UL), gd 4 on distal upper limbs and gd 4 in the
lower limbs (LL); global areflexia, abasia and
pseudoathetosis in the four limbs. Complementary investigation with muscle enzymes, vitamin
B12, folate, thyroid function and rheumatologic
markers and brain MRI were all normal. Electroneuromyography study showed severe sensory
impairment suggestive of ganglionopathy with
no myopathic findings. Muscle biopsy revealed
the presence of Ragged red fibers and rare negative cox fibers. Case 2: A 59-years-old woman
with a 10-year history of progressive bilateral
ptosis. One year ago, she started with dysphagia,
neuropathic pain in legs and gait disturbance.
She had chronic coronary heart disease and
hypothyroidism. Several family members had
palpebral ptosis. Clinical exam revealed bilateral ptosis and bilateral facial weakness; motor
strength: gd 5 in UL, gd 3 in the LL, gd 4 in leg
extension; bilateral foot drop, areflexia in the UL
, patellar hyporeflexia and areflexia in both achilles, hipopallestesia and hypoesthesia in LL and
ataxic gait . Complementary investigation with
muscle enzymes, vitamin B12, folate, thyroid
function and rheumatologic markers were all
normal. Electroneuromyography study revealed
myopathy feature with a severe axonal sensory
neuropathy. Muscle biopsy showed Ragged red
fibers. Discussion: Mutations in mitochondriopathies can be located in the mitochondrial DNA
(mtDNA) and nDNA, which includes changes
in POLG 1/2 genes, encoding the gamma-polymerase, which is responsible for replication and
repair of mtDNA. In POLG-related disorders,
there is the Sensory Ataxic Neuropathy, Dysarthria and Ophthalmoparesis (SANDO) Syndrome
(OMIM: #607459), and we consider these two
cases described as examples of this rare presentation of mitochondriopathy.
P-236
RELATION BETWEEN TIMED MOTOR
PERFORMANCE AND COMPENSATORY
MOVEMENTS IN CHILDREN WITH DUCHENNE
MUSCULAR DYSTROPHY DURING FUNCTIONAL
AMBULATION ACTIVITIES
Martini J, Caromano FA, Simões M, Voos MC
• UNIVERSIDADE DE SÃO PAULO
Background: The timed performance and,
more recently, the compensatory movements
have been used in the functional assessment
of children with Duchenne muscular dystrophy (DMD). However, the existing literature
on these measures does not bring enough data
for a further comprehension of their meaning,
making the evaluation of DMD progression
difficult. Objective: This study aimed to (1) describe the timed performance and the compensatory movements in functional ambulation
activities needed by children with DMD in a
one-year period and to (2) investigate possible
relationships between these variables. Method:
Thirty-two boys were filmed, mean age 10 yrs,
performing the tasks 10-m walking, climbing up
and climbing down four steps. Each child was
filmed in three moments: initial, after 6 months
and after 12 months. The timed performance
of each activity was registered in seconds. The
functional evaluation scale for DMD scored the
compensatory movements. Analysis of variance
compared the three assessments (alfa<0.05).
Spearman correlation tests investigated the relationships between the timed performance and
the compensatory movements. Results: Distinct
motor strategies have been observed (parameters: timed performance and compensatory
movements score) on functional ambulation
activities. On walking, timed performance and
compensatory movements increased every sixmonths. On climbing up steps compensatory
movements increased, followed by timed performance. On climbing down steps, both parameters increased at first and, on the last assessment,
only timed performance increased (p<0,05). The
correlation coefficients between the timed performance and the compensatory movements
were classified as moderate to strong on walking
(r=0,67), climbing up (r=0,55) and climbing down
steps (r=0,76). The coefficients increased on every reassessment. Conclusion: Timed performance and compensatory movements increased
in twice in a year evaluation carried out. During
functional ambulation, the timed performance
and the compensatory movements are related.
However, climbing up and down steps showed
dissociation between these parameters, because
one of them, increased visibly more than the other in different intervals of six months. Keywords:
Duchenne muscular dystrophy; measures; assessment; motor activity; function.
P-237
RELATIONSHIP BETWEEN COGNITIVE AND MOTOR
FUNCTIONS IN PATIENTS WITH DUCHENNE
MUSCULAR DYSTROPHY
Favero FM1, Voos MC2, Artilheiro MC2, Leite IPA2,
Caromano FA2
• 1UNIFESP; 2FMUSP
Background: Duchenne muscular dystrophy
(DMD) is a severe and relatively common disease, which progresses with muscle weakness
and functional weakness. A high percentage of
patients also show cognitive impairment. However, it is not known whether cognitive deficits
are related to more severe motor outcomes.
Objective: The present study aimed to investigate possible relationships between cognitive and motor function in patients with DMD.
Method: Seventeen patients diagnosed with
DMD (aged 12‐21 yrs, classified from 3 to 8 on
Vignos Scale, with formal education 2‐12 yrs)
were submitted to neuropsicological assessment
with 1. Mini Mental State Examination (MMSE),
2. Clock drawing test, 3. Verbal fluency (animals
and words with p), 4. Digits direct and reverse order. Motor function was evaluated with the Motor Function Measure (MFM), domains 1 (walking and transfering), 2 (proximal function) and 3
(distal function). Spearman correlation tests investigated the relationships between motor and
cognitive scores (alfa<0.05). Results: Domains 1
and 2 of MFM were poorly related to all cognitive scores. However, domain 3 of MFM, which
evaluated mainly upper limbs distal functions,
was strongly correlated to the scores on MMSE
(r=0.76), clock drawing test (r=0.79), digits (direct
and reverse order) (r=0.80 and r=0.70, respectively) and moderately related to the verbal fluency (words with p) (r=0.64) (p<0.05 for all correlations). Conclusion: Cognitive performance
seems to be significantly related to upper limb
distal function in DMD.
97
Posters
P-238
RELIABILITY OF ADAPTED JEBSEN-TAYLOR
TEST TO EVALUATE UPPER LIMB FUNCTION IN
MUSCULAR DYSTROPHIES
Artilheiro MC1, Favero FM1, Caromano FA2, Voos MC2,
Oliveira ASB1, Sá CSC1
• 1UNIFESP; 2USP
Introduction: The Jebsen-Taylor Test (TJT) was
indicated as a sensitive method to measure the
upper limb (UL) function in Duchenne Muscular
Dystrophy (DMD), but not in other forms of muscular dystrophies (MD). The specific adaptation
and reliability analysis for the translated Portuguese version can represent an accurate assessment of upper limb function in DM. Objective:
To adapt and to analyze the intra and interrater
reliability of TJT for patients with DM. Methods:
The study was approved by the Federal University of São Paulo (132193) and was conducted
in two stages. Firstly, DMD patients (n=3), Myotonic Dystrophy Type I (DM1) (n=1) and Fascioscapulohumeral Dystrophy (FSH) (n=1) (21±8.1
years) were filmed performing two attempts
with both upper limbs in seven subtests of the
TJT. Two raters analyzed the films, measured the
time and identified the need to exclude the subtest named “picking up heavy objects” and the
possibility of inclusion of two subtests that minimized the TJT limitations, previously pointed out
in the literature: the absence of a task involving
bringing the hand the mouth and of bimanual
activities. At the second stage, 25 patients with
DMD, Becker Muscular Dystrophy, Limb Girdle
Muscular Distrophy, FSH and DM1 (21.1±7.9
years old) performed the adapted TJT with the
original subtests “writing”, “turning over cards”,
“picking up small common objects”, “simulate
feeding”, “stacking checkers”, “picking up large
light objects” and with the inserted new subtests
“bringing the food to the mouth” and “picking up
light objects with both hands”. The analysis of inter and intra rater reliability to adapted TJT was
performed by intraclass correlation coefficient
(ICC) in SPSS (SPSS Inc, Chicago, USA) version
17.0. Results: Good intra rater (ICC 0.95 to 0.96)
and inter rater (ICC 0.97 to 1.00) coefficients were
obtained to adapted TJT. Conclusion: The adapted TJT was a reliable method to evaluate the upper limb function in DM.
P-240
ROLE OF NEURAMINIDASE-1 IN SKELETAL
MUSCLE REGENERATION
Neves JC, Rizzato VR, Fappi A, Chadi G, Zanoteli E
• FMUSP
Introduction: Neuraminidase‐1 (Neu1) participates in sialoglycoconjugates catabolism in lysosomes. Congenital Neu1 deficiency is the basis of
sialidosis, a severe neurosomatic disorder associated with osteoskeletal deformities, hypotonia
and muscle weakness. Mice with Neu1 deficiency
(Neu1-/-) develop an atypical form of muscle degeneration characterized by abnormal fibroblast
proliferation and expanded extracellular matrix
(ECM), invasion of muscle fibers by fibroblast,
cytosolic fragmentation, vacuolar formation and
muscle atrophy. Despite muscle degeneration is
well characterized in these animals, myogenesis
has not been studied so far. Objective: The aim
of this study was to evaluate the involvement of
Neu1 in muscle regeneration process after cardiotoxin (CTX) injection in Neu1-/- mice and
normal controls. Methodology: CTX was applied
98
in the right tibialis anterior muscle and the mice
were euthanized by cervical dislocation in different periods after injury. The muscles were
analyzed by histology; cross-sectional area of
regenerative muscle fibers; quantification of
proliferative cells (with BrdU positive nuclei);
immunohistochemistry for neutrophils, macrophages, embryonic myosin and collagen III;
gene and protein expression of muscle transcription factors. The data were compared and
variances considered statistically significant in
case p≤0.05. Results: In animals with Neu1 deficiency, both inflammatory process (mainly macrophagic response) and proliferative potential
were increased in the initial stages, accompanied
by overexpression of Pax7. We observed delay in
muscle maturation characterized by higher expression of embryonic myosin later in muscle
regeneration. MyoD and MyoG genes were overexpressed from 5 to 10 days after injury, though
the expression of these proteins was reduced. At
the end of muscle regeneration, reticulin deposition in ECM was increased, indicating fibrotic
process. Conclusion: Neu1 seems to participate
in all stages of muscle regeneration, since acute
injury phase through the control of cell proliferation, towards muscle maturation, and at the final stages when it would regulate the deposition
of ECM componentes. (FAPESP 2009/02937‐4 e
2011/03853‐9).
P-241
SCHWARTZ-JAMPEL SYNDROME (SJS) – CASE
REPORT
Prado MR, Gomes ACD, Acchar MC, Oliveira NP,
Penque GMA, Duro LAA, Bastos ICC
• UFRJ
Introduction: The Schwartz-Jampel syndrome
(SJS) is characterized as an autosomal recessive
disease whose clinical features are continuous
myotonic dystrophy, short stature, typical facial
appearance and multiple skeletal abnormalities.
Objective: Describe the dysmorphic findings of
a typical case of SJS. Case report: Patient afro
descendant female, incomplete university, from
Rio de Janeiro, 40 years-old. She was admitted to
Instituto de Neurologia Deolindo Couto (INDC)
of UFRJ at 07 years old, because of the gait impairment and sit down. The first symptoms were
observed at 7 months of age with progressive
evolution. At 2 years old, she showed these symptoms: a muscle stiffness, joint and bone deformities, dwarfism, sardonic facies, micrognathia,
cleft palate, nasal voice, epicanthus, limitation
of mouth opening, permanent contracture of
the forehead, chin and perioral region, stiffness
in the abdominal muscles, bilateral genu valgus,
universal limiting, more severe in the hip joints,
knee and left ankle, flat feet and limited abduction joint movements. Psychomotor development without changes. The family history is negative for neuromuscular diseases and the parents are non consanguineous. She has a healthy
brother. Neurological exam: anserine gait;
restriction of joint movements. Bilateral myotonic phenomenon to percussion of the thenar
region. Electrophysiological findings: electrical
activity of muscle fibers, with a tendency to myogenic pattern in both the light and maximum effort. Muscle biopsy: primary muscular atrophy.
Discussion: SJS is a rare muscular disease. It is associated to mutations in the gene HSPG2, encoding the Perlecan. Perlecan is a large heparin sulfate proteoglycan, that is found in the basement
membrane of the extracellular matrix and that is
essential in maintaining cartilage integrity and
in regulating muscle excitability. The diagnoses
was based on dysmorphic features, both facial
and skeletal, and on electromyographic changes
showing continuous electrical activity of muscle
fibers. This syndrome has three defined types
according to age of onset of symptoms. Type 1A
is recognized in infancy and presents moderate
bone dysplasia, type 1B is detected at birth, with
more pronounced bone dysplasia and type 2 is
a severe neonatal form, with high rates of infant
mortality. The reported case is the type 1B. There
was a good response to carbamazepine, with improvement of myotonia and activities of daily life
as usually expected.
P-243
SLOW-CHANNEL CONGENITAL MYASTHENIC
SYNDROME: CASE REPORT
Prando N, Morita MPA, Coelho EN, Pozo MM, Paiva GP,
Kouyoumdjian JA
• FAMERP
Introduction: Slow-channel congenital myasthenic syndrome is a rare disorder caused by
postsynaptic neuromuscular junction defect.
Mutations in genes that encode acetylcholine receptors subunits alter its affinity to the receptor
and the closing time of the ion channel. Object:
To report a Slow-Channel congenital myastenic
syndrome case. Case report: Female, 26 years,
onset of symptoms at 10 years of age with bilateral and symmetric weakness for fingers and
wrist extension, arms raising, walking and climbing stairs. These symptoms worsened in extreme
temperatures (very hot and very cold), emotional
and physical stress and also during the course
of a regular day. She evolved with bilateral ptosis, visual impairment - blurring and occasional
diplopia - and worsening upper limbs weakness.
Neurological examination: upper limbs weakness with hyporreflexia, mainly for finger and
wrist extensors, slight eyelid ptosis on the right
side, bilateral lateral rectus paresis more marked
on the left side and mild bilateral facial paresis.
Fatigability was observed in the upper limbs.
The father had similar symptoms and died from
hemorrhagic stroke. There was no consanguinity.
Ck: 70 IU / l; muscle biopsy with histochemical/
immunohistochemical studies showed nonspecific findings as type 1 fiber predominance;
nerve conduction studies (NCS) showed repetitive discharges after a single stimulus in median, ulnar and common peroneal motor nerves
(compound muscular action potential - CMAP second wave); repetitive nerve stimulation (RNS)
of median (3 and 30 Hz) and common peroneal nerves (3 Hz) showed abnormal decrement.
Treatment with pyridostigmine led to slight and
temporary improvement. There was improvement in fatigability with fluoxetine 30 mg daily.
Discussion: The slow-channel congenital myasthenic syndrome is a rare autosomal dominant
disorder (paternal inheritance) that can manifest
at any age with weakness and fatigability mainly
in the neck muscles, shoulder girdle and distal
muscles of the upper limbs. There is no response
to immunomodulatory. The response to anticholinesterase drugs is variable. Diagnosis is based
on clinical picture, family history, NCS showing
CMAP with two or more components and RNS
with decrement response to low rate (2‐3 Hz). The
differential diagnosis should be done especially
with acquired autoimmune myasthenia gravis
and congenital myasthenic syndrome due to
acetylcholinesterase deficiency.
XXVI Congresso Brasileiro de Neurologia
P-244
SOCIAL ASPECTS OF DUCHENNE MUSCULAR
DYSTROPHY
Grippe TC1, Barbosa FMB2, Martins TAC1, Gava M3,
Segut L2
• 1HOSPITAL DE BASE DO DISTRITO FEDERAL;
2
HOSPITAL UNIVERSITÁRIO DE BRASÍLIA; 3INSTITUTO
NACIONAL DO SEGURO SOCIAL
Introduction: Progressive muscular Duchenne
dystrophy (DMP) is the most frequent primary
muscle disorder; it is characterized by a degenerative and hereditary behavior. It is already known
that most parents of DMP patients have a psychological maladjustment. There are few studies regarding the social security aspects related to the
DMP. The main objective of this paper is to describe the profile of the patient and his relatives
involved in the use of the resources provided by
the “Continuation provision benefit” (BPC). The
BPC is a benefit of a monthly minimum salary
paid to people with disabilities that made them
unabled for working and for independent living.
To receive this benefit it is necessary to have a familiar monthly income lower than proportion of
25% of the minimal salary per person. Methods:
A descriptive study – survey of 20 patients with
DMP, registered under the International disease
classification (CID) number G71.0, who were
enrolled for the BPC benefit in the social security agency (INSS). Results: 20 applicants were
identified with CID G71.0 in the BPC system.
The legal representative was the mother in 85%
(17 of 20). All legal representatives reported being unemployed. The average age of the mother
at time of child‘s birth was 25 years, only0 24%
(4 of 17) of the mothers reported the existence of
husband. The average age of the child, when he
was asked what benefit was 8.7 years. The average time of perceived benefit was 5.5 years, and
in one family, two patients received BPC. The
origin of the child was mainly from surrounding
the Federal District (19 of 20) region, standing,
with 45% of the total, the regions of Ceilândia (5)
and Samambaia (4). Conclusions: The DMP has
an important socio-economic impact, not only
regarding the granting of long-time benefit for
patients with the disease, which overloads the
pension system, but also about the reflections on
family life style, especially the mother who is the
primary caregiver. All the legal guardians in their
productive age were unemployed and almost
80% of mothers had no husband, demonstrating,
in a way, the resilience and ability to develop effective coping strategies.
P-245
SPEECH THERAPY PERFORMANCE IN
MYASTHENIA GRAVIS: CASE REPORT
Melo CB, Beneplacito F, Saconato M, Gonçalves MIR
• UNIFESP
Introduction: Myasthenia gravis (MG) is a disease manifested by progressive muscle weakness
and fatigue. It affects between 5 and 15 individuals per 100,000. Are common the following
manifestations: dysarthrophonia, dysphagia and
dyspnoea, with a view to respiratory insufficiency resulting from a myasthenic crisis. Objective:
To describe the speech therapy performance in
a patient with MG, suffering from a severe dysphagia and dependent on invasive mechanical
ventilation. Case report: A female, 42 years old
patient suffering from MG in clinical assessment
had to use a nasogastric tube (NG) and tracheostomy, since she was dependent on mechanical
ventilation in pressure support mode. During
the structural evaluation was realized a change
of tongue strength with salivary stasis in the
oral cavity. During the adaptation of the speaking valve in the mechanical ventilation, it was
possible to see the vocal was wet and constant
accumulation of mucus in the throat during
deglutition of saliva. The patient was diagnosed
with severe oropharyngeal dysphagia, requiring
the maintenance of an exclusive NG - scale FOIS
1. The speech rehabilitation aimed the returning of the oral diet and the aid in weaning from
mechanical ventilation. Taking into account the
counterindication to anticholinergic medications, it was started indirect speech therapy with
the use of speaking valve in mechanical ventilation, aiming to increase the frequency of the deglutition of saliva. It was started a training with
soft food by mouth and, because of the delay to
trigger the swallowing reflex, it was proposed exercises to increase strength of the tongue base,
increasing contact between the tongue base with
posterior pharyngeal wall and maneuver of neck
flexion. After 17 sessions, the patient was no longer dependent on the mechanical ventilation
and started eating soft heterogeneous food (FOIS
6). Discussion: The counterindication to anticholinergic medication, showed up as a barrier
for the speech rehabilitation of the deglutition of
saliva. The indirect therapy using taste thermal
tactile stimulation proved to be very effective in
increasing the frequency of deglutition. Speaking
valve proved to be an important therapeutic tool
in this case, since the possibility of communication and the improvement in deglutition provided by its use have contributed positively to return
the feed to oral feeding and the weaning.
P-246
STIFF PERSON SYNDROME IN A PATIENT WITH
DIABETES MELLITUS- A CASE REPORT
Campos LO1, Gotardelo DR2, Costa JHSM1, Ribeiro FML1
• 1HOSPITAL MARCIO CUNHA; 2FACULDADE DE
MEDICINA DO VALE DO AÇO
Background: Stiff Person Syndrome (SPS) is
the most prominent disease in a group of neurological disorders associated with the presence of anti-glutamic acid decarboxylase (anti-GAD) antibodies, an enzyme largely found
in central nervous system gabaergic neurons.
The hallmark of this syndrome is progressive
muscle stiffness with episodes of superimposing muscle spasms accompanied by pain.
Comorbidity with other autoimmune diseases
such as type 1 diabetes mellitus (DM), thyroiditis, and myasthenia gravis, is common.
Less frequently, SPS may present as a paraneoplastic syndrome. Treatment is based on
gabaergic potentiation with drugs such as
diazepam, vigabatrin, and baclofen, which
provide symptom relief. The role of immunomodulators such as plasmapheresis and immune-globulins is still unclear, but they seem
to offer substantial improvement. Aim: To report a case of SPS in a patient with a diagnosis
of type 1 DM. Case report: A 54-year-old male
patient, with hypertension and insulin-dependent DM, presented with a 3-year history
of painful muscle spasms that were refractory
to optimized analgesia. The symptoms worsened in intensity and frequency, with associated gait alteration, rigidity, and slight anterior flexion of the trunk. On neurological examination, muscle strength was normal in the
four limbs, as were reflexes and cranial nerves.
The patient had been extensively investigated
over the course of multiple hospitalizations.
During the last hospital stay, the patient
progressed with loss of consciousness in the
presence of pain for likely vasovagal response.
No alterations were detected on brain, cervical spine, and dorsal spine MRI scans. Lab
tests were normal, including blood chemistry,
thyroid function, viral serological tests, ionogram, CSF analysis, and tumor markers. Electromyography was typical of the disease. No
nutritional deficiencies were found. ANA titers were positive at 1:640; anti-GAD antibody
titers were 119 IU/mL; positive anti-islet cell
antibodies; C-peptide level was 0.57 ng/mL,
and CPK level was 613 IU/L. Muscle biopsy
excluded myopathy. Full remission of symptoms was achieved with 20 mg diazepam and
30 mg baclofen once daily, which allowed ambulatory follow-up. Discussion: The diagnosis
of SPS should be considered in patients with
nonspecific muscle manifestations of uncertain or obscure etiology, especially when associated with autoimmune diseases.
P-247
STUDY OF PULMONARY FUNCTION IN
MYASTHENIA GRAVIS’S PATIENTS
Santos NS, Salomão RPA, Cunha MCB, Carvalho AAS,
Feder D
• FMABC
Introduction: Myasthenia Gravis (MG) is an
autoimmune disease characterized by fatigability and fluctuant weakness of skeletal
musculature. Respiratory involvement is directly is related to the severity of the disease.
An early evaluation of respiratory function is
important as this measure could prevent serious complications such as atelectasis and
bronchopneumonia, which are related to high
mortality risk. Objective: This study examines
the role of pulmonary function in Myasthenia
Gravis’ patients and analyzes the clinical test
applicability as a prognostic factor. Methods:
33 patients were selected from an outpatient
clinic of Neuromuscular Disorders with confirmed diagnosis of MG. The inclusion criteria for the diagnosis: electroneuromyography
with decrement ≥10% and/or antireceptor
of acetylcholine or antimusk antibodies.
Exclusion criteria: Respiratory pathologies.
The patients performed the pulmonary function test in Vitalograph’s spirometer to determinate the FVC (forced expirtory vital capacity), FEV1 (forced expiratory volume in 1 second) and the correlation between both values.
Patients with decreased FVC and FEV1/FVC
>78% were classified as restrictive respiratory
insufficiency (RRI). Results: The mean age of
patients was 44,8 years (6‐ 79 years old). 72, 7%
(n=24) were female; 87, 9% (n=29) were under
medication (corticosteroids, azathioprine,
pyridostigmine); 30, 3% (n=10) of patients
showed RRI, being 40% (4) a mild myasthenia
form (I and IIa) and 6 had a moderate to severe form (IIb). In relation to symptomatology, 40% (4) had a generalized form, whereas
30% (3) had an ocular form and 30% (3) exhibited a bulbar form. In comparison, patients
with normal pulmonary function (n=23),
presented a mild myasthenia form (I and IIa)
in 70% (16) cases and a moderate to severe
form in 30% (7) cases. From them, 52,2% (12)
showed generalized symptoms, 8,7% (2) had
only bulbar involvement and 39,1% (9) had
an ocular form. Conclusion: 30,3% of patients
presented a decreased pulmonary function,
which characterizes a restrictive respiratory
99
Posters
insufficiency. It was not possible to associate
the altered pulmonary function with neither
the disease’s severity nor the symptomatology
in the studied sample. Even though, the pulmonary function test can be a useful parameter to evaluate and follow up these patients.
P-248
SWEET’S SYNDROME IN A PATIENT WITH
MYASTHENIA GRAVIS
Hussein MA1, Iachinski RE1, Oliveira Filho JMR1,
Oliveira TFT1, Nascimento MTMS2
• 1FACULDADE ASSIS GURGACZ; 2INSTITUTO DE
NEUROLOGIA DE CURITIBA
Introduction: Myasthenia Gravis is an autoimmune disease, with a prevalence of 150 per
million populations, where about one million
people worldwide have the disease diagnosis.
The mortality incidence, before the proposed
treatments, reached 50% in 10 years of the illness
course. The treatment is divided into two phases:
the acute phase (myasthenic crisis) and the
chronic phase. Generally, the treatment is based
on the use of acetylcholinesterase inhibitors,
corticosteroids, immunosuppressors/immunomodulators, plasmapheresis and immunoglobulin. In some cases, the use of immunosuppressive drugs such as azathioprine may cause the
Sweet’s Syndrome, which happens in patients
with hypersensitivity to the drug. Objective: To
describe a case of Sweet’s Syndrome in a patient
after using azathioprine. Case report: Female, 43
years, clinically and electromyographically diagnosed with Myasthenia Gravis, started its treatment with prednisone 60mg/day and azathioprine, increased until a 150mg/day dosage. The
patient complained of measured fever (>38,3oC)
after one week of treatment with azathioprine,
which started 06 hours after its use. Concomitant
with the increasing of the medication, cutaneous
injuries and chills started to affect the patient
in the same interval of the drug administration.
From this, a time change of the medication was
made with no success and the patient kept being stricken with the same symptoms at the
same 06 hours interval from azathioprine use.
Conclusion: Sweet’s Syndrome was initially described in 1964 and it’s characterized by fever,
neutrophilic leukocytosis and painful papules
commonly located at the lower and upper members, nape, face and dorsal body. The clinical presentation starts in 2 or 3 weeks after the immunosuppressor’s use and its treatment is usually
done with corticosteroids, colchicina and potassium iodide, with good results. Cases of Sweet’s
Syndrome in patients previously diagnosed with
Myasthenia Gravis and in use of azathioprine are
extremely rare even on PUBMED, which has only
3 published cases. In this patient, the reaction to
azathioprine was suspected duo to the temporal
relationship between drug administration and
the onset of symptoms. The patient’s improvement after removing the immunosuppressor,
confirmed the diagnosis. Despite of believing in
a type III hypersensitivity reaction, the pathophysiology is still unknown.
100
P-249
P-251
SYSTEMIC SARCOIDOSIS PRESENTING WITH
MYALGIA AND WEAKNESS
THE DIFFICULTIES IN EPIDEMIOLOGICAL SURVEY
OF MYOPATHIES IN FEDERAL DISTRICT
Scalco RS1, Holton J2, Brady S2, Gomes I1, Staub H1,
Becker J1
Tosta ED1, Pontes M2, Moura M3, Menezes SR4,
Schappo A5, Fernandez RM4, Cavalcante MV5,
Grippe TC2, Sahdo AM2, Bretones LA2, Almeida S2,
Gava M6, Seguti L4
• 1PUCRS; 2UCL
Introduction: Sarcoidosis is a non-caseosum
granulomatous inflammatory condition which
phatophysiological mechanism is still unknown.
Although rare myalgia and weakness may be the
main clinical presentation of systemic sarcoidosis
resembling other causes of neuromuscular disorders. Methods and Results: Here we describe a
patient with systemic sarcoidosis initially misdiagnosed as polymyosistis. Muscle biopsy was the
key diagnostic tool. Although abnormal muscle
findings raised the suspicious of a granulomatous
disorder, few sections of the same muscle were normal, illustrating the patchy pathology of this condition. Conclusion: Sarcoid myositis (SM) may be the
first manifestation of systemic sarcoidosis. Muscle
pathology, which is the key diagnostic tool for SM,
may reveal normal muscle pathology highlighting
the need for extra levels / sections to be performed
when SM is suspected.
P-250
THE ACQUISITION OF BREATH-HOLD ABILITY
DURING IMMERSION IS DISSOCIATED FROM
THE RESPIRATORY AND MOTOR FUNCTION
PROGRESSIVE LOSS IN LIMB-GIRDLE AND
DUCHENNE MUSCULAR DYSTROPHY
Voos MC1, Caromano FA1, Leal B2, Favero FM3,
Albuquerque P4
• 1USP; 2AACD; 3UNIFESP; 4FMUSP
Background: Limb-girdle and Duchenne muscular
dystrophy (LGMD, DMD) are characterized by the
progressive loss of motor and respiratory functions.
Although aquatic therapy is currently indicated for
patients with muscular dystrophy, no study in the
literature has described the acquisition and evolution of the underwater breath-hold time in patients
treated with aquatic therapy. This ability is critical
to provide independence in the aquatic environment, which ensures a better mobility and quality
of life for muscular dystrophy patients. Objective:
To compare the evolution of the respiratory and
motor functions and the evolution of underwater
breath-holding time in LGMD and DMD patients
treated with aquatic therapy in a period of one year.
Method: Sixteen patients with LGMD and forty-one
with DMD (11‐29 yrs, with Vignos classification from
2 to 8) were evaluated twice, with the interval of
one year between the assessments. The ability to
hold the breath in immersion was measured with
a chronometer and registered, in seconds. The motor function was assessed with the Motor Function
Measure (MFM) and the forced vital capacity (FVC)
and the forced expiratory volume in 1 s (FEV) were
measured by spirometry. ANOVAs compared the assessments and groups. Results: The groups did not
differ in MFM, but the second assessment showed
motor function decrease for both (p<0.001). The
groups did not differ in underwater breath-holding time, but the second assessment showed time
increase, mainly for LGMD (p=0.049). For the respiratory parameters, FVC and FEV significantly decreased for both groups (p<0.001 for both comparisons). Conclusion: In LGMD and DMD patients,
although motor and respiratory parameters reflected the loss of function and the progression of the
muscular dystrophies, the underwater breath-hold
time increased, mainly in the LGMD group, showing
a higher adaptability to the aquatic environment in
patients treated with aquatic therapy for one year.
• 1ACADEMIA BRASILEIRA DE NEUROLOGIA; 2HOSPITAL
DE BASE DO DISTRITO FEDERAL; 3HOSPITAL
REGIONAL DA ASA NORTE; 4UNIVERSIDADE DE
BRASÍLIA; 5HOSPITAL DA CRIANÇA DE BRASÍLIA;
6
INSS
Introduction: Epidemiological survey require
costly and require a huge time and persistence
of researchers, especially when studying rare
diseases. The decision to perform this kind of
survey must take into consideration not only the
basic characteristics of a good research question
(innovation, relevance, originality), but essentially the cost-benefit. Objectives: To present
the difficulties and limitations of conducting an
epidemiological study of myopathies in the Federal District. Methods: The authors describe the
entire trajectory, from first contact between the
researches, in January 2011, choice and evaluation of the social impact and importance of the
subject until the detection of cases of genetically determined myopathies such as muscular
dystrophy and myotonic dystrophy. Results:
The choice of the theme held on rare conditions
with genetic transmission, which resulted in low
numbers of subjects and an early withdrawal
of the majority of professionals involved in the
project. Among the obstacles encountered, we
must consider: the limitations of the assessment
method with consisted of evaluation of signs and
symptoms. In addition, the low reliability of data
obtained from the hospital records system, the
disbelief of professionals involved in research,
the scarce data obtained and the resistance of
the hospital’s managers in DF for the project implementation. In three years of survey, 54 cases
were detected, the most frequent was Duchenne
muscular dystrophy, followed by the Limb-girdle muscular dystrophy. The obtained average
of age was 29 years, with the majority (%) male.
Conclusion: In three years of study, there were
only a few dozen cases, among hundreds expected, stamping the enormous difficulty of making
a clinical epidemiological study within the spectrum of neuromuscular diseases.
P-252
THE EFFECTS OF OMEGA-3 SUPPLEMENTATION
ON DEXAMETHASONE-INDUCED MUSCLE
ATROPHY
Fappi A, Neves JC, Rizzato VR, Godoy TS, Chadi G,
Zanoteli E
• FMUSP
Introduction: Many conditions are associated
with muscle atrophy including inactivity, aging,
sepsis, diabetes, cancer and steroid therapy. All
these conditions produce muscle atrophy due to
increase of protein degradation and/or reduction
of protein synthesis involving at least five systems: lysosomal, calpains, caspases, metalloproteinase, and ubiquitin-proteasome. The steroids
produce atrophy acting in some of these systems.
Considering that steroids have been commonly
used in the medical practice, the identification of
drugs or nutritional supplements that are able to
alleviate its side effects under the skeletal muscle
would be very important. Objective: The objective of this study was to assess the effect of the
n‐3 fatty acid supplementation (EPA/DHA) on
the prevention of the muscle atrophy induced
XXVI Congresso Brasileiro de Neurologia
by the steroid Dexamethasone (DX). Methods:
Treated and non treated rats with n‐3 were subjected to the dexamethasone administration,
and their muscles were analyzed by Imunohistochemical and RT-PCR. Results: DEXA administration produced a reduction of 25% on cross
sectional area of type IIB muscle fibers, as well
as on Omega‐3 group. On type I an IIA fibers the
n‐3 group shows the larger atrophy, not seen in
other groups. Real time showed elevated ATROGIN‐1 activation and Myogenin inhibition in n‐3,
higher than DX group. Concomitant administration of n‐3 with DX was not effective to alleviate
the muscle atrophy and increase of atrogenes expression, instead causing larger muscle atrophy
and atrogene expression. Conclusion: while the
Omega‐3 is known to be effective in attenuating
the muscle atrophy induced by sepsis and cancer, its concomitance with glucocorticoid can
aggravate its side effects to skeletal muscle, as
muscle atrophy. The identification of nutritional
supplements able to alleviate the side effects of
steroids on skeletal muscle, i.e. muscle atrophy,
and the potential molecular pathways involved
in this process, would be very important in the
medical practice. (FAPESP 2011/03862‐8).
other two were retired for invalidity. Conclusion:
Myopathy considerably interferes with productive capacity, even when considering clinical
variability. The prevalence of BPC as the major
benefit granted (80%) evidences not only the disabled status of the individual (condition required
for inclusion) and the absence of employee relationship, but also the low purchasing power, considering that the individual insured has to prove
a per capita monthly income lower than ¼ of
minimum wage.
Pain
P-256
P-254
WELFARE BENEFITS GRANTED TO ONES
AFFECTED BY MYOPATHY
Galhardoni R, Cury RG, Fonoff ET, Ghilardi MGS,
Fonoff F, Arnaut D, Myczkowski ML, Marcolin MA, BorSeng-Shu E, Barbosa ER, Teixeira MJ, Andrade DC
Barbosa FMB1, Reis MJF1, Mateus SRM2, Gava M3,
Azevedo LGC4, Seguti L1
• HC-FMUSP
• HOSPITAL UNIVERSITÁRIO DE BRASÍLIA;
2
UNIVERSIDADE DE BRASÍLIA - UNB; 3INSTITUTO
NACIONAL DO SEGURO SOCIAL; 4HOSPITAL DE BASE
DO DISTRITO FEDERAL
Introduction: Nonmotor symptoms (NMS) such
as pain are present in a large majority of PD patients, and their importance has been increasingly recognized. DBS has gained general use in the
management of motor symptoms in PD patients.
However, the impact of DBS on NMS has been
less often investigated. Aim of investigation: To
assess pain changes after long-term subthalamic nucleus (STN) deep brain stimulation (DBS)
and to explore its relationship between changes in motor and quality of life in PD patients.
Methods: We have prospectively evaluated 41
patients with PD before and one year after STN
DBS. Hoehn&Yahr scale, UPDRS III, L-dopa
equivalent dose were recorded. A second examiner blind to the motor outcome of DBS assessed NMS with the following tools: McGill Pain
Questionnaire (MPQ); Brief Pain Inventory (BPI);
Visual Analogic Scale (VAS); Neuropathic Pain
Symptom Inventory (NPSI); Hospital Anxiety and
Depression Scale (HADS) and Non-Motor Symptoms Scale (NMSS). Pain was also correlated with
changes in the motor symptoms and quality of
life (SF‐36 Quality of Life Questionnaire). Results:
The mean age was 57±10 and Hoehn&Yahr
off-medication score was 2.80±0.64. UPDRS-III
scores were 19.7±8.2 and 43.5±12.5 in on and off
medication conditions, respectively. The prevalence of pain was 70%, and the most common
subtype was musculoskeletal pain presented in
86.7%. A year after surgery, there was a significant improvement in the prevalence and intensity of pain (VAS=5.96±2.84 to 1.64±2.48, p=0.001).
There was significantly improvent in pain scores,
HADS, NMSS and SF‐36 after DBS (tables 1). There
was a strong correlation between the change in
pain intensity and the improvement in quality
of life (r=0.708; p<0.005). There was no correlation between pain improvement and response to
levodopa or stimulation (rho=0.247,p=0.197 and
rho=0.249,p=0.193, respectively). Conclusions:
Pain is a common nonmotor symptom in patients with PD. Patients experience various types
of pain, and accumulated evidence, as shown in
our study, indicates that STN DBS improves pain
Introduction: Myopathies constitute a heterogeneous group of diseases, whose manifestation
presents with a wide range of clinical variability
(severe forms, early onset, more benign forms
and slow progression forms). Such conditions
might interfere with productive capacity of the
individual, which allows him the right to appeal
to National Institute For Social Insurance (Instituto Nacional de Seguro Social - INSS) for help.
Among the benefits, they might be eligible for:
critical illness insurance, professional rehabilitation, review in 2 years, continued disability benefits (Benefício de Prestação Continuada - BPC)
and retirement for invalidity (Aposentadoria por
Invalidez - LI). Objective: Introduce the type
of benefits granted in INSS to ones affected by
myopathy. Methods: We selected 215 registries
of benefits with CID G71 to G71.9; G72 to G72.9
and G73.4 to G73.7, completed between 1999
and 2013 in INSS – Executive Management of
Distrito Federal. Reports from each registry cataloged with CID compatible with myopathy were
analysed separately and compared with clinical
history, side exams and diagnosis. The variables
considered were: gender, type of benefit and type
of myopathy. Results: From 215 registries, only
84 truly corresponded to CID of myopathy. There
was a predominance of males. Almost half (41)
presented with clinical and laboratorial status
compatible with CID G71.0 (muscular dystrophy); 27 were cataloged as CID G71.8 (primary
muscle disorder) and the remaining 16, as CID
G72 (other myopathies). Concerning benefits
grated by INSS, from 41 individuals affected by
muscular dystrophy, 29 (70%) didn’t have insured
status and received BPC; seven were retired using
LI. In relation to ones affected by primary muscle
disorder, 17 (63%) received BPC, eight were in IL
and two received critical illness insurance. From
16 allocated in other types of myopathies, 9 (56%)
received BPC, four, critical illness insurance and
P-257
DORSAL ROOT GANGLION BLOCK: AN IMPORTANT
TOOL IN THE TREATMENT OF NEUROPATHIC PAIN
Veloso VN, Pereira ECV, Bezerra MLE, Guerra DV,
Schmid MF, Baeta AM
• BENEFICÊNCIA PORTUGUESA DE SÃO PAULO
CORRELATION BETWEEN PAIN, MOTOR
SYMPTOMS AND QUALITY OF LIFE IN PATIENTS
WITH PARKINSON´S DISEASE AFTER DEEP BRAIN
STIMULATION
1
in PD while also improving motor symptoms and
quality of life. Thus the potential benefit of this
procedure on pain in PD should be considered
and discussed with patients.
Objective: To demonstrate the benefit of dorsal
root ganglion block in patients with acute or
chronic radicular pain. Methods: This retrospective study included patients who underwent dorsal root ganglion block, between 2010 and 2013.
In total, 92 patients underwent the procedure,
64% of which were female and 36% of which were
male. Patients were between 19 and 95-yearsold, and the majority of patients that underwent
the procedure were in the 51- to 70-year-old age
group. Among them, seven patients underwent
the procedure more than once, and one patient
underwent five dorsal root ganglion blocks. Periradicular injection (bupivacaine, triamcinolone,
and xylocaine) was performed through a lateral
foraminal approach under computed tomography guidance after the injection of a contrast
agent. Results: All patients had refractory radicular pain, causing them to rely heavily on medications for neuropathic pain. In most cases,
radicular syndrome by entrapment was due to
disc herniation and postoperative spine surgery
that evolved with periradicular fibrosis. After the
procedure, all patients demonstrated significant
improvement of their symptoms, significant reduction in the doses of their medications, and
an overall improvement in their quality of life.
Conclusions: The dorsal root ganglion block is
an important tool in the treatment of acute or
chronic radicular pain that is either cervical or
lumbar in nature. This procedure should be considered regardless of the age of the patient, since
it leads to remission of major pain, consequently
reducing antalgic drug dose.
P-258
EPIDERMAL CYST OF SKULL - CASE REPORT
Rodrigues CFA1, Fillus IC1, Conte T2
• 1UNIVERSIDADE ESTADUAL DO OESTE DO PARANÁ;
2
UNIVERSIDADE DO OESTE DE SANTA CATARINA
Introduction: Epidermoid cysts of the central
nervous system are uncommon conditions, often
located in the cerebellopontine angle and around
the bridge. Account for only 1% of all intracranial
mass lesions. Are usually originate from defects
associated with surface elements of the ectoderm of the nervous system during the closure
of the neural groove or formation of secondary
brain vesicles. Have growth reading, being the
same due to the progressive accumulation of
keratin and cholesterol, produced by sloughing
of epithelium surrounding the cyst. Objective: To
report a case of epidermal cyst of the skull associated with neurological symptoms and surprising dimensions that differ from classical lesion
described in the literature, considered routinely
as low expansive potential. Case report: Patient
female, white, 82 years, reports feeling a ‘hardening skull “over 1 year ago and show signs of”
loss of strength “muscle on the left side about
20 days. The patient also had shown changes
in level of consciousness (Glasgow 10). The left
hemiplegia and the difference in bone density
101
Posters
were described due to an intracranial epidermal
cyst with extension of the lesion into the bone of
the skull. On preoperative computed tomography of the patient (TC), we observed large lesion
dimensions (13x12x15 cm), including entering
the bone. The patient underwent surgery for a
craniotomy, uneventful, with the horse shoe type
incision right asterio-pterional. After surgery
the symptoms mentioned in the interview were
mitigated, already completing 60 days in which
the patient is stable and under monitoring.
Discussion: The epidermal cysts are referred to
as structures that grow slowly and stop growing
when they reach between 1 and 5 cm in diameter.
And it is the surprising extent of that injury patient reached when compared with that reported in other cases, it was the first motivation for
you to write this article. These cysts total about
0.3% to 1.8% of tumors of the central nervous
system and have a yet unknown etiology. The
most accepted theory is that the intracranial
EC come from incomplete cleavage of the neural ectoderm and skin between the 3rd and 5th
embryonic week. Conclusion: Although limited
and benign epidermal cyst of the skull may reach
considerable size and should receive appropriate
treatment to avoid undesirable complications as
possible, but rare malignancy.
P-260
FIBROMYALGIA AND DNA METHYLATION- A PILOT
STUDY
Galhardoni R, Vieira HC, Chile T, Dalle CS, Pagano RL,
Gouveia GR, Brentani HP, Kaziyama HH, Teixeira MJ,
Andrade DC
• HC-FMUSP
FM is a complex disorder. Different lines of evidence point to genes, epigenetic mechanisms
and gene-environmental interactions contributing to the risk of FM. One of the mechanisms of
epigenetic control of the genome is DNA methylation. DNA Methylation is one of the most important mechanisms to modulate gene expression. We performed a whole-genome methylation analysis in FM patients and healthy controls
to investigate differentially methylated genes in
these two groups. Method: Blood from 25 FM
patients and 25 paired healthy subjects (HS) was
collected. FM patients underwent cortical excitability evaluation with rTMS and filled out the
McGill and Fibromyalgia Impact Questionnaire
and the Brief Pain Inventory. Extraction of DNA
was performed by the saltingout method. Methylation pattern of the genome was made by the
Illumina Infinium HD assay (480K). Data was analyzed by Genome Studio Data Analysis Software
and the IMA-package, with t-Student test and
Benjamini-Hochberg (BH) for false discovery ratio (FDR <=0.05).We used two different software
DAVID and WebGestalt (hipergeometric test and
FDR <=0.05) searching for hyper represented
pathways. We found 24 genes differently methylated in FM patients. The hyper represented
pathways included cytokine-cytokine receptor
interaction-(CXCL5, MPL, TNF-RSF19), Adherence junction-(NLK,CTNNA2), MAPK signaling
(NLK,PRKCG,MECOM), Arrhythmogenic right
ventricular cardiomyopathy-(ARVC), Leukocyte
Transendothelial Migration-(PRKCG,CTNNA2),
Cancer pathways-(PRKCG,CTNNA2), vascular
smooth muscle contraction-(PRKCG,CALD2),
tightjunction-(PRKCG,CTNNA2), Calcium-Signaling pathways-(RYR2,PRKCG). These data
102
suggest that different methylation patterns exist
in genes related to immunity and inflammatory
response in FM. It remains to determined whether these findings would have a classification use
or provide insight into its mechanisms of disease.
P-261
GIANT CELL TUMOR OF THE SPINE: A RARE CASE
Lemos LEAS1, Silva IM1, Rocha Neto MP1, Feitosa AGCS1,
Sousa WGS1, Ferreira PHPB2, Cavalcante DC3,
Rodrigues AB3, Macedo LP4, Marques Júnior MASS5,
Santos RVSG1, Sousa CM4, Ribeiro RG3, Sousa ACB6
• 1UESPI; 2FACID-DEVRY; 3UFPI; 4FACID; 5UNINOVAFAPI;
6
INSTITUTO DE NEUROCIENCIAS
Introduction: Giant cell tumor (GCT) is a benign and aggressive bone neoplasia that exhibits uncertain behavior. Histologically consists of
multinucleated giant cells scattered throughout
the tumor tissue. These tumors are average incidence of 3.9% of all bone tumors and are often
located in long bones (femur and tibia), sacrum.
Vertebral involvement is rare, being described in
only 2.9% of cases of GCT. In this case, it manifests as a mass lesion with bone destruction and
impairment of nerve roots, showing early signs
of neurological involvement at the level of injury and pain later.Currently the approach in GCT
of the spine is the ample resection of the lesion
(neurosurgery). This procedure significantly reduces local recurrence, resulting in cancer cure
with satisfactory functional result. Case report:
Female, 13 years old, sought treatment in the
urgencies hospital in Teresina with complaints
of back pain and progressive paraparesis, started a few months ago. Neurological exam: grade
I muscle strength in the lower limbs, absence of
deep and superficial sensibility and Babinski`s
sign present. The patient was submitted to surgery, which was performed resection of the lesion associated with thoraco-lumbar arthrodesis. Patient progressed well in the postoperative
period, showing improvement of paraparesis
with grade II muscle strength in the lower limbs
and partial return of deep and surface sensitivity
and six days after surgery. Discussion: The relevance of this case is to report a rare bone neoplasia (2.9% of bone tumors) in an unusual location
(vertebra) that developed neurological symptoms in order to disseminate knowledge about
this, to be mentioned as a differential diagnosis
in spinal pathologies.
P-262
PAROXYSMAL EXTREME PAIN DISORDER
(PREVIOUSLY CALLED FAMILIAL RECTAL PAIN)
Barros BCC, Leão AVR, Almeida Neto JG, Carlos KCL,
Lima KM, Amancio KAL, Alúcio KT, Bomfim RC,
Gameleira FT
• UFAL
Introduction: Paroxysmal extreme pain disorder
(PEPD) is a rare autosomal dominant disease
with varying degrees of penetrance. It is characterized by paroxysms of severe burning pain in
the rectal, ocular, or submandibular region and
flushing. Begins in childhood and progresses
over the years. 1,2 Objectives: The study presents
the clinical, electrophysiological and neuropsychological assessment of a patient with extreme
pain disorder, and aims to help understanding
of it and its mechanisms. Case report: Since the
23 years has episodes of extreme pain to painful
stimuli in the lower limbs or unexpected in the
anorectal region (for example, the pain of a difficult defecation or rectal touch). The last time he
had this crisis was 3 years ago. The pain starts by
one or both legs or the site of the trauma (for example, the thigh). The pain is in intense burning
and has swift rise to the ipsilateral upper limb. If
the pain is in the left lower limb, it also causes
an intense dysautonomia, with bilateral facial
plethora. The total duration of pain is 3‐5 minutes.
In the post-ictal period has intense malaise and
allodynia. Never fainted in one of those crises.
In interictal period and prodrome has no pain;
in the post-ictal period has intense malaise and
allodynia in the same area of ictal pain, which
last around 4am. The interictal EEG and MRI
of the skull were normal. His daughter and her
mother have epilepsy and PEPD. No medication
at this time. Normal neurological examination.
Discussion: The PEPD‘s main manifestations of
excruciating rectal pain are caused by defecation
or perineal stimulation, usually followed by submandibular or ocular pain and redness. 1,3,4 Is
related to the gain of function mutations in SCN9A encoding mostly sodium channel Nav1.7
dependent voltage and causes a hyperexcitability in ganglia dorsal root. 1 The diagnosis is based
on clinical investigation of the patient. There is
a possibility to have a mechanism epilepsy and
carbamazepine has been used in the relief of
symptoms. 1,3.
P-263
PERCUTANEOUS VERTEBROPLASTY FOR
TREATMENT OF OSTEOPOROTIC FRACTURES IN
PATIENTS WITH CHRONIC PAIN
Schmid MF, Veloso VN, Guerra DV, Bezerra MLE,
Pereira ECV, Baeta AM
• BENEFICÊNCIA DE SÃO PAULO
Objective: To assess the efficacy of percutaneous vertebroplasty (PVP) in pain management
in 31 patients with acute osteoporotic vertebral
fractures (AOVF). Methods: From June 2000 to
December 2010, 31 patients from the Neurology
Clinic with intense, refractory thoracic or lumbossacral pain associated with AOVF were referred to the Interventional Radiology Center for
PVP. Patients underwent unilateral or bilateral
transpedicular injection of poly-methyl methacrylate. Non-invasive pain management was overseen by neurologists before and after the procedure. Results:Fifty-eight PVP were performed in
31 patients with pain associated with AOVF. The
patients were predominantly elderly females: 22
women, 9 men, with age from 38 to 95 (median:
72 years old). Younger patients were related with
chronic corticosteroid use. The affected vertebrae ranged from T8 to L5, the most affected
being L2 (10 patients) and the less affected, T8
(1 patient). Regardless of age, corticosteroid use
and affected vertebrae, all patients presented
improvement in both functional deficit and pain
perception. Conclusions:Percutaneous vertebroplasty significantly improved pain scores in
the presented series. Improvement in quality of
life, status performance and marked decreased
in pain medication consumption were also noted. This method provides efficient symptomatic
relief, and constitutes important therapeutic alternative for this condition, especially in elderly
patients.
XXVI Congresso Brasileiro de Neurologia
P-265
TRIGEMINAL NEURALGIA ASSOCIATED WITH
PERSISTENT PRIMITIVE TRIGEMINAL ARTERY:
REPORT OF 3 CASES
Borges R, Valença MM, Holanda AC, Costa RG,
Asfora CA, Soares MC, Batista LL
• UFPE
Background: Despite being frequent, trigeminal neuralgia’s pathophysiology is poorly
understood. Most of the cases are considered
idiopathic. Around 1% of patients show a
structural lesion displaced during the neuroimaging evaluation. In such cases, either a tumor or a vascular compression over the nerve
is considered the cause. The most common
cause of the idiopathic trigeminal neuralgia
are tortuous arteries in the prepontine space
compressing the trigeminal nerve. Purpose:
To present three cases of patients with a persistent primitive trigeminal artery, but with
bilateral or contralateral trigeminal neuralgia.
Case report: We will cite the cases of three
patients (2 women), 2 of whom presented,
atypically, in their early age, bilateral trigeminal neuralgia associated with a persistent
primitive trigeminal artery; in the other patient, the pain was located on the contralateral side of the arterial abnormality, suggesting that the persistent trigeminal artery may
cause hemodynamic changes which, in turn,
may affect the trigeminal systems bilaterally. Conclusion: Although a rare association,
trigeminal neuralgia may be caused by a persistent primitive trigeminal artery.
Epilepsy
P-266
“PACHI-BAND”- A RARE CONDITION CASE REPORT
Coelho VCM, Morita ME, Coan AC, Alvim MKM,
Bueno FF, Cendes F
• UNICAMP
Introduction: Malformations of cortical development are abnormalities that interfere with
a complex organized process of cortical development. Subcortical band heterotopia (SBH),
which is rare in males, shows lack of lamination
and cortical organization, resulting in two cortical bands: the first in normal localization, and
the second under the subcortical white matter
giving the aspect of double cortex. Sometimes,
the SBH can appear anteriorly with pachygyric cortex, known as “pachy-band”. Aim: To describe a patient with SBH/“pachy-band”, a rare
condition that affects cortical development in
males. Case report: A right-handed 27-year-old
man presented a history of seizures that began
at the age of 12. His seizures were described as
generalized tonic-clonic without aura. Family
also reported episodes of aggressive behavior
intercalated with periods of lack of emotional reactivity, that required psychiatric admission. He
had school delay and mild-moderate cognitive
impairment. The magnetic resonance imaging
(MRI) showed pachygyria in the frontal lobes
and SBH sparing the posterior brain regions.
Discussion: SBH is a neuronal migration disorder that results in a double cortex. The subcortical laminar heterotopia usually affects the
entire brain with variable thickness. The thicker
the band heterotopia the worse is the gyral abnormality in the overlying cortex and the clinical
manifestations. These patients often show epilepsy and variable degrees of mental retardation.
Most patients are female and it rarely affects
males. Patients with mutations in doublecortin
gene have more severe clinical phenotype. Other
patterns of SBH have been described, including
pachygyria-SBH pattern, found only in males.
The diagnosis can be aided by imaging, mainly
MRI that shows heterotopic band isointense with
the cortex in all sequences, with variable extension and thickness. In addition, the gyri show
simplified patterns of small gyri and shallow
sulci. These findings can be best viewed using
curvilinear reconstruction, assisting the structural imaging and providing good anatomical
perspective.
P-268
ADHERENCE MEASURES PATIENTS WITH
MEDICALLY REFRACTORY EPILEPSY
Settervall CHC1, Ferrari CMM2, Castro LHM3,
Sousa RMC1
• 1ESCOLA DE ENFERMAGEM DA UNIVERSIDADE DE
SÃO PAULO; 2CENTRO UNIVERSITÁRIO SÃO CAMILO;
3
FACULDADE DE MEDICINA DA UNIVERSIDADE DE
SÃO PAULO
Introduction: Objective and subjective measures
of treatment adherence present limitations.
Goal: To describe and to evaluate the correlation between different adherence measures of
patients with refractory epilepsy. Method: A prospective, transversal and observational study was
performed with refractory epilepsy patients in
an outpatient setting. Adherence measurements
included serum antiepileptic drug (AED) levels,
seizure frequency and self-reported adherence
were collected immediately after a medical appointment. Results: We studied 91 patients with
refractory epilepsy. There was no gender predominance. Average age was 37.8±12.1 years. Average
education was 9.8±3.1 years. About half of patients were black, had no marital bond, and were
not inserted in the labor market. AED treatment
duration was on average 20.7±12.9 years, the
Epilepsy Medication and Treatment Complexity Index (EMTCI) average was 18.8±9.8 points,
polytherapy (68.3%) and symptomatic focal seizures (75.6%) were predominant. At baseline,
59.4% of patients regarded their seizures as not
adequately controlled. The sample reported an
average of 4.9±13.0 seizures the previous month.
Indication of low and intermediate adherence by
Morisky-Green test was an inclusion criterion in
the study. 84.6% of participants showed average
adherence before interventions. Considering the
initial AED serum levels, only 42% of participants
had subtherapeutic AED levels. We found no correlation between adherence measures. there was
no significant association of Morisky-Green categories and seizure occurrence (r= ‐0.032; p>0.5)
and subtherapeutic serum AED levels (r=0.089;
p>0.5). Seizure frequency was also independent
of subtherapeutic AED levels (r=0,025; p>0,5).
Conclusion: Adherence measurements were not
converged, indicating that these measurements
should be used with caution to evaluate adherence in patients with refractory epilepsy.
P-269
ANTIEPILEPTIC DRUGS: EFFECTS ON
CARDIOVASCULAR RISK MARKERS
Urquiza PAC, Pereira MP, Fonte MHC, Valença LPAA
• UFPE
Introduction: Enzyme inducers antiepileptic
drugs (AEDs) (carbamazepine, phenytoin and
phenobarbital) have been associated with dyslipidemia, while inhibitory enzymatic AEDs (valproic acid) to weight gain, insulin resistance and
elevated uric acid levels (1,2) . These metabolic
alterations may increase the risk of cardiovascular events since epileptic patients need to use
these drugs in a long-term basis (1‐3). Objective:
This study aimed to evaluate the influence of
AEDs on lipid profile, ESR, uric acid and BMI in
patients with epilepsy. Methods: A cross-sectional case-control study was conducted in patients
with epilepsy AED users (n=80) and control subjects (n=32) at the University Hospital Oswaldo
Cruz-UPE, between February and November
2013. Individuals with personal or family history of early cardiovascular events, chronic renal
disease, liver disease, smokers, drinkers and patients in use of drugs to treat dyslipidemia were
excluded. Results: The mean age of patients and
control subjects was, respectively, 26.7 years
± 11,09 and 30±12.95 years (Mann Whitney,
p=0,08). As far as drug therapy is concerned, 48
(58.7%) were in a monotherapy regimen: carbamazepine (63.8%), the most used AED, followed
by valproic acid (19.1%), phenobarbital (10.6%),
topiramate (4.2%) and hidantal (2.1%). We observed significant differences (p<0.05) in Total
and LDL cholesterol in patients taking phenobarbital monotherapy compared to the control
group (Table 1). We found no significant correlation between Total cholesterol, HDL, LDL,
VLDL, triglycerides, uric acid and glucose levels
in the other groups compared to the control one.
The uricemia of patients taking valproic acid on
monotherapy was higher than the control group,
p<0.05, Mann Whitney. Patients on polytherapy
had a high ESR compared to control subjects
(p<0.05, Mann Whitney). Conclusion: On the
contrary to expectations, there was no significant
increase of dyslipidemia in patients using carbamazepine. The findings of dyslipidemia with usage of phenobarbital and increased uric acid in
patients using valproic acid are consistent with
findings of related literature.
P-270
ASSOCIATION OF HYPOTHALAMIC HAMARTOMA
AND GELASTIC SEIZURES: CASE REPORT
Coutinho AFAR1, Sarmento SA1, Melo GPF1,
Guedes GACR1, Melo APF1, Cunha AMR1, Jordão LJP1,
Barbosa DRC2, Santana MMF1, Ribeiro MP1, Nóbrega N3
• 1FAMENE; 2UPE; 3FMABC
Introduction: The hypothalamic hamartoma
(HH) is a rare congenital malformation of the
tuber cinereum. It may manifest as seizures,
especially gelastic, developmental delay, behavioral disorders and early puberty. Gelastic
seizures are a rare manifestation of HH and
generally unrecognized, although can be associated to lesions in other topographies. The
ictal laughter is not associated to feelings, and
does not last long. Due to electroencephalogram (EEG) findings in gelastic seizures being
diverse and nonspecific, the discovery of epilepsy and HH is postponed. Objective: Report
a case of gelastic seizures caused by hypothalamic hamartoma. Case report: Patient of 22
years, in Endocrinology service since 2006,
103
Posters
accompanied by microprolactinoma and obesity, presented report of early puberty, with menarche at 18 months, and use of GnRH analogue
until 9-years-old. Tomography of the pituitary, in
1986, showed no disorders. Proceeded with seizures, either tonic-clonic or other episodes difficult to define, associated with behavioral change.
Made use of anticonvulsants in mono and polytherapy in optimal considered doses, but still
without control of seizures. Monitoring by EEG,
in 2008, revealed psychomotor agitation crises,
unrelated to matching electroencephalographic activity. In 2013, there was a deterioration of
the crisis and obesity status, and a new Magnetic
Resonance showed an oval nodule, located posterior to the pituitary stalk, near the mammillary
body, measuring 0.5 x 0.5 cm, compatible with
HH. On the second EEG, gelastic seizures were
evidenced. Patient was submitted to a pterional
craniotomy for a microsurgery and hamartoma
resection. After surgery, recovered without neurologic deficits, presented remission of seizures,
and intact pituitary function. Discussion: The
diagnosis of HH as a cause of epilepsy is usually
delayed, because it has atypical seizure signs in
EEG, with nonspecific and difficult visualization
of the lesion on Resonance examinations. The
hypothalamic hamartoma’s research, despite
the small dimension, caused a clinical picture
of early puberty and gelastic seizures. Patients
with gelastic seizures refractory to medical treatment are candidates for surgery. If the resection
is complete, there is seizure control, improved
behavior, motor skills, language and cognitive
skills. The lesionectomy through microsurgery
or radiosurgery, is the most effective treatment.
When neurosurgery is not possible, is considered
intractable epilepsy.
P-271
AVALIAÇÃO DA MMP-9 EM EPILEPSIAS E GRUPO
CONTROLE
Santos MCV1, Christo PP1, Sandrim VC2, Fernandes KSS2
• 1SANTA CASA DE BELO HORIZONTE; 2INSTITUTO
DE ENSINO E PESQUISA DA SANTA CASA DE BELO
HORIZONTE Introduction: Epilepsy is a prevalent neurological disorder that affects approximately 2%
of brazilian population. Only 70% of these patients, on average, have the disease under medical control and this finding per se, reveals the
high percentage of individuals found refractory
to conventional treatment Recently, some scientific reports shows the role of matrix metalloproteinase 9 (MMP‐9) in epileptogenesis.
Objectives and Methods: Considering the importance of the molecular mechanisms involved
in this disease, in this study there were analysed
MMP‐9 plasma levels between epileptic individuals aiming the correlation between plasma levels
of this biomarker and clinical variables, namely:
age of epilepsy onset, generalized or focal epilepsy syndrome well controlled and drug refractory focal epilepsy syndrome, well controlled and
drug refractory generalized epilepsy syndrome.
To undertake such data, therewere studied a
group of 82 individuals diagnosed with epilepsy
and followed at the Santa Casa de Belo Horizonte
Neurologic Clinic. This patient group were compared with a control group, consisting of 26 asymptomatic individuals. Results: There weren`t
observed any differences in MMP‐9 plasma levels
between groups. However, the stratified analysis
of the refractory generalized epilepsy patients
showed a trend to higher MMP‐9 plasma levels.
104
Conclusion: This study reveals the need for further research with more robust samples or analysis of the MMP‐9 expression in other tissues.
P-272
Case report: RASMUSSEN’S ENCEPHALITIS
Gajo RFM, Pereira Neto AG, Christo PP, Santos MCV,
Xavier MT, Barbosa AVS, Costa BS, Pinto TVL
• SANTA CASA DE BELO HORIZONTE
Introduction: Rasmussen’s encephalitis is a
chronic inflammatory disease of unknown origin, usually affecting one brain hemisphere, associated with progressive neurological dysfunction and intractable seizures. Objective: To report
a case of Rasmussen’s encephalitis Case report:
S.M.S., 4 years-old, female, with normal neuropsychomotor development until 3 years and 6
months of age, when she developed partial epileptic seizures in her left side and infrequent generalized tonic-clonic seizures, which worsened
in time and became refractory to medical treatment. Brain CT and blood tests were uneventful.
Electroencephalogram showed right frontotemporoparietal sharp waves and spike-and-wave
discharges. The patient was started on phenytoin, phenobarbital, clobazam, sodium valproate
and carbamazepine, without remission. Brain
MRI showed T2 and FLAIR hypertintensity in the
right cerebral hemisphere. The patient then received pulse theraphy with methylprednisolone
and IV immunoglobulin due to the suspicion of
Rasmussen’s encephalitis. Antiepileptic drugs
were adjusted and the patient was kept on use of
topiramate, carbamazepine and clobazam, with
partial response. As the seizures persisted, she
was transferred to Santa Casa de Belo Horizonte for further investigation and treatment. She
was admitted with continuous simple seizures
in her left side, preserved cognition, left sided
dysmetria and unable to walk due to left-sided
hemiparesis, besides left ankle clonus, extensor
plantar response in her left foot and left central
facial palsy. Subsequent brain MRI showed T2
and FLAIR hypertintensity in the right cerebral
hemisphere and ipsilateral cerebral hemiatrophy. Further therapy with corticoids and IV immunoglobulin was ineffective. Patient was submitted to functional hemispherectomy of the
right hemisphere, which resulted in a decrease
in seizures. Tractography showed remnant of a
connection in a small part of the posterior corpus callosum. Patient received new surgical procedure for complete disconnection, resulting in
complete remission of seizures. Afterwards, the
child was kept on carbamazepine alone, with
no further seizures, good cognition and walking autonomy despite left-sided hemiparesis.
Discussion: Rasmussen’s encephalitis is a severe
form of epilepsy that can lead to intractable seizures, hemiparesis and cognitive loss. Epilepsy
surgery can play an important role in the improvement of these patients.
P-273
CHAGAS DISEASE: EXOTIC RISK FACTOR FOR
EPILEPSY IN BRAZIL
Borges APP1, Limonte FH1, Tanaka MU1, Andrade RS1,
Esteves VB1, Vasconcelos RC1, Paula GR2, Prando N1,
Borges MA1
• 1FAMERP; 2FACERES
Background: Chagas disease is an endemic disease in Latin America, mainly in southeastern
Brazil, caused by Trypanosoma cruzi. Chronic
manifestations affect the heart, esophagus and
colon, and acutely may cause myocarditis or encephalitis. Case reported: FCG, 41 year, female,
born and raised in São José do Rio Preto hospitalized due to a persistent and progressive headache in 02/08/2011. She had a aphasic seizure
progressing with right head deviation seizure
followed by secondary generalization - tonic /
clonic type. After that patient remained comatose and with right hemiplegic. CSF showed mild
lymphomonocytic standard with slight increase
in protein. The image of MRI was suggestive of
tumor however the brain biopsy showed necrotic
lesions with numerous amastigotes of Trypanosoma cruzi parasite. Previous history: In 1998,
the patient had several partial left focal motor
seizures evolving to generalized tonic-clonic
seizure. Chagas myocarditis was diagnosed with
mural thrombi, and small area of right frontal encephalomalacia (stroke). We controlled the seizures with carbamazepine. Due to heart failure
secondary to dilated cardiomyopathy underwent
cardiac transplantation on 28/04/2010 immunosuppressants have been used. Discussion: The
study of Chagas infection in the CNS has gained
increased interest in immunocompromised individuals such as patients with cancer, and those
undergoing transplants in acquired immunodeficiency syndrome (AIDS). This case shows the
etiologic agent trypanosome cruzi in reactivated
inflammatory form with different pathophysiology behaving as a risk factor leading to two independent types of seizures in the same person.
The first form (1998) was due to embolic stroke
caused by mural thrombus myocarditis and second form (2011) was due to cerebral inflammatory (pseudo-tumor) that is rare and exotic, but
that needs to be considered in immunocompromised patients with Chagas disease living in Latin America. Keywords: epilepsy, the risk factor,
chagoma, Latin America.
P-275
EARLY VERSUS LATE ANTIEPILEPTIC DRUG
WITHDRAWAL FOR PEOPLE WITH EPILEPSY IN
REMISSION
Strozzi I1, Nolan SJ2
• 1UNIVERSIDADE FEDERAL DO PARANÁ; 2UNIVERSITY
OF LIVERPOOL
Introduction: Antiepileptic drugs (AED) are the
main interventions used to prevent seizures and
control epilepsy. Although very effective, AED’s
are related to long-term cognitive and behavioral
adverse effects. Thus, when epilepsy is in remission, it may be in the individual’s best interest to
discontinue medication. Objectives: To compare
risk of seizure relapse, status epilepticus and
mortality after early and late AED discontinuation in adult and pediatric epilepsy patients; to
assess which variables modify the risk of seizure
relapse; and to define a subpopulation in which
early AED discontinuation is safe. Methods: We
searched for randomized controlled trials that
evaluate withdrawal of AEDs after varying periods of seizure remission in adults and children
with epilepsy. We used the Cochrane Epilepsy
Group Specialised Register, CENTRAL, MEDLINE, CINAHL, SCOPUS, ClinicalTrials.gov and
WHO ICTRP. Two authors independently extracted data and assessed trial quality. Relative risks
(RR) with 95% confidence intervals (CI) were calculated for each trial, and summary RRs were calculated using a fixed-effects model. Results Five
trials were included, representing 924 randomized children with epilepsy, all under 16 years of
age at randomization, with a median follow-up
of 5.6 years. No eligible trial evaluated adults nor
XXVI Congresso Brasileiro de Neurologia
assessed mortality or status epilepticus as outcomes. The pooled RR for seizure relapse after
early AED withdrawal (less than 2 seizure-free
years) was 1.34 (95% CI 1.13 to 1.59, P=0.0007).
Early discontinuation was associated with greater pooled RR in people with partial seizures (RR
1.51 95% CI 0.97 to 2.35, P=0.07). Absence type
epilepsy showed a lower risk of relapse. Variables
associated with higher seizure relapse were abnormal EEG findings (RR 1.44, 95% CI 1.13 to
1.83, P=0.003), especially epileptiform activity
(RR 2.58 95% CI 2.03 to 3.28, P<0.0001), epilepsy
onset before 2 years or after 10 years of age, history of status epilepticus, intellectual disability
(IQ <70) and high seizure frequency before and
during treatment. Conclusions: There is evidence to support waiting for at least two seizure
free years before discontinuing AEDs in children,
particularly if they have an abnormal EEG and/
or partial seizures. There is insufficient evidence
to establish when to withdraw AEDs in children
with generalized seizures. There is no evidence to
guide the timing of withdrawal of AEDs in seizure
free adults.
P-277
EPIDEMIOLOGICAL ASPECTS OF EPILEPSY IN
BRAZIL AND IN PARANÁ STATE FROM 2008 TO 2013
Oliveira MAA1, Souza FL1, Rocha CD2, Oliveira Júnior JA2,
Naves WN3, Cavalcante JES3
• 1UFPR; 2UFG; 3HC UFG
Background: Epilepsy is the commonest neurological condition affecting people of all ages,
race and social class. There are about 50 million
people with epilepsy in the world, 40 million
in developing countries with little or no access
to treatment. There are few studies on epidemiology of epilepsy in Brazil, despite being an
important public health problem. Objective:
To describe the profile of patients hospitalized
due to epilepsy in Brazil and in Paraná state, according to sex and age, in the period from 2008
to 2013, comparing to literature. Material and
Methods: Data from hospital admissions in Brazil and in Paraná state, in the period from 2008
to 2013, registered by Brazilian Health System
based on database (DATASUS). Results: The total number of hospitalization due to epilepsy in
Brazil from 2008 to 2013 was 284507, of which
166144 (58,4%) in men and 118363 (41,6%) in
women. Of the total admissions, 95924 (33,7%)
occurred from 0 to 9 years, 35124 (12,3%) from
10 to 19 years, 25700 (9,0%) from 20 to 29 years,
29772 (10,5%) from 30 to 39 years, 32849 (11,5%)
from 40 to 49 years, 25510 (9,0%) from to 50 to
59 years, 17840 (6,3%) from 60 to 69 years, 13542
(4,8%) from 70 to 79 years, and 8246 (2,9%) above
80 years. In the state of Paraná there were 25229
hospital admissions due to epilepsy in the same
period, of which 14201 (56,3%) in men and 11028
(43,7%) in women. Of the total admissions, 8443
(33,5%) occurred from 0 to 9 years, 3492 (13,8%)
from 10 to 19, 2305 (9,1%) from 20 to 29 years,
2542 (10,0%) from 30 to 39 years, 2898 (11,6%)
from 40 to 49 years, 2133 (8,4%) from 50 to 59
years, 1612 (6,4%) from 60 to 69 years, 1207
(4,8%) from 70 to 79 years, and 597 (2,4%) above
80 years. Conclusion: The incidence of epilepsy
in the world varies from 40‐70 per 100.000/year
in developed countries to 100‐190 per 100,000/
year in developing countries, as Brazil. It is more
common in males than females but this difference is rarely significant. The highest incidence
is in early childhood, with a decrease in adolescence, in both scenarios. In Brazil and in the state
of Paraná, the incidence is lower among elderly,
different from world, where the incidence above
80 years is significant.
P-280
EPILEPSY AND DEPRESSION: A DOUBLE STIGMA
Amaral RM, Andrade Filho AS, Britto RM, França KS,
Lopes GVDO
• ESCOLA BAHIANA DE MEDICINA E SAÚDE PÚBLICA
E FUNDAÇÃO DE NEUROLOGIA E NEUROCIRURGIA INSTITUTO DO CÉREBRO Introduction: Epilepsy is the most prevalent
serious neurological condition. Historically
suffered social stigma, with laws that excluded people with epilepsy from public places and
sterilization policy, which existed until 1970.
Depression has prevalence between 29 and 60%
in these patients. Studies indicate that this relation is linked not only to psychosocial factors,
but also to pathophysiological mechanisms.
The underdiagnosis can reach 60%. Objectives:
To estimate the prevalence and underdiagnosis
of depression in patients with epilepsy, identifying possible relation between the type, etiology
and medications. Methodology: Cross-sectional
study by primary data collection, using the Beck
Depression Inventory (BDI) as a collection tool.
Considering the prevalence of 45%, the sample
calculation for a confidence level of 95%, with
difference acceptable of 10% was 96. Categorization of intensity of the BDI was based on
the BDI-II Manual and Steer and Kendall et al.
(1987), the currently most acceptable. Results:
The average age was 38 years (SD =14, min of 13
and max of 73). 55% were male. 59% of seizures
were generalized tonic-clonic type; 8% of typical absences and 7% tonic. 12% of the seizures
were simple and 83% generalized. The etiology
were idiopathic in 52% of the cases, 17% were
post-traumatic and 9% were caused by infections. The medications most commonly used
were carbamazepine (57%), phenobarbital (22%)
and valproic acid (19%). The overall median BDI
score was 14 (SD =11 min. 0 and max. 46). It was
observed that 47% of the patients had minimal
or no depression, 14% were mild, 24% moderate
and 12% severe depression. 36% of patients had
major depression. Phenobarbital had statistically significant increase in the indices of the BDI
(p=0.004) and in the major depression (p=0.02).
Based on the relative risk, these patients were 3
times more susceptible to depression. There was
no difference in BDI score compared to benzodiazepines, etiology or type of crisis. Conclusion:
epilepsy has a higher prevalence of depressive
symptoms compared to the general population
(8%), and to other conditions such as heart disease (15%), indicating a possible pathophysiological association and psychosocial factors. Its
prevalence was 36%, with 97% underdiagnosis.
It was not found a relation between seizure type
and etiology. Phenobarbital showed three times
more chance of presenting depression.
P-281
EPILEPTIC SEIZURES RECOGNITION BY
EMERGENCY CARE SERVICES‘ PHYSICIANS IN
BLUMENAU
Mendonça GS1, Barth JÁ2, Siquineli F3, Machado JNP2,
Ricardo Júnior E2
• 1HOSPITAL DAS CLÍNICAS FMRP; 2FURB; 3HOSPITAL
SANTA ISABEL
Background and objectives: Epilepsy is one of
the most important neurologic disease. It’s know
there are some difficulties to secure a correct
diagnosis of the epileptic seizures due to the
huge variability in their clinical presentation.
This study assessed the ability of emergency
service physicians’ to differentiate and classify
epileptic seizures, and psychogenic nonepileptic seizures based solely in semiologic features.
Methods: Videos from non-epileptic seizures
and different types of epileptic seizures was
shown to 45 volunteer emergency service physicians’. Each participant watched 5 randomly
chosen videos, obtained from a pool of fifteen
video’s electroencephalograms presenting epileptic seizures (generalized, simple partial,
complex partial, secundarily generalized), and
psychogenic nonepileptic seizures. All the participants watched one nonepileptic seizure’s video plus four epileptic seizure’s video (one of each
category). After every video the physicians had
to mark in a closed questionnaire their classification choice. Results: The results showed that
simple partial seizures have high rates of accuracy whereas other epileptic seizures and psychogenic nonepileptic seizures had less correct diagnosis. Physicians whose time span since graduation is shorter and those who have less experience in emergency services were able to classify
seizures more accurately than their older peers.
Conclusion: Both psychogenic and epileptics
seizures have difficulty in their diagnosis. Less
experienced physicians had higher correct diagnosis than their more experienced colleagues
probably due to a recent contact with this matter. The authors believe that the knowledge of
epileptic seizures (and its various types), as if the
psychogenic nonepilpetic seizures should have
improvements in emergency services’ to provide
the right management of these patients.
P-282
EPILEPTOGENESIS INDUCED BY PERIPHERAL
INJURY: SCORPION STING
Almeida Neto JG, Leão AVR, Barros BCC, Carlos KCL,
Lima KM, Amancio KAL, Alúcio KT, Bomfim RC,
Gameleira FT
• UFAL
Introduction: Peripheral lesions can cause epileptogenesis, by inducing plastic changes in the
sensorimotor cortex, leading to cortical hyperexcitability and epilepsy, with seizures initially focal in the same region of the peripheral lesion [1].
The scorpion venom is known as neurotoxic, epileptogenic, but we don’t know previously reports
of these causing focal epilepsis with the mechanism above described. Objectives: Describe a
case in which a previously epileptic patient, after
a scorpion sting, modified his pattern of crisis
and increased its frequency. Case report: Male,
36 years. The crisis have an aura of feeling that
a woman is cherishing his scrotum, with erotic
connotation, lasting three minutes, followed by
a ascending epigastric discomfort, “like going
down a roller coaster”, followed by disconnection
with environment, mucocutaneous pallor and
oro-facial, masticatory, bimanual automatisms,
lasting 5‐10 minutes. Have post-ictal sleepness.
He has about 10 partial complex crisis with aura
per month and numerous isolated scrotal auras. Had an isolated Generalized Tonic-Clonic
Seizure, 5 years ago. At eight, a scorpion stung
his arm and scrotum. Had the first crisis at nine
years old, started presenting aura like “look at the
belly and see particles swimming” and paresthesias like “swallowing testicle”. The neurologic examination is normal. The EEG reveals a left temporal anterobasal irritative activity. Neuropsychological assessment shows a deficit of verbal
105
Posters
memory. A CT scan of the skull is normal. The
interictal SPECT shows a clear deficit of the left
temporo-parietal perfusion. Discussion: It‘s long
known that sensory stimuli or peripheral lesions
may interfere with seizures [1‐2]. Scorpio’s stings
cause intense local pain, paresthesias and local
decrease in strength. It can also induce drowsiness, tremors, confusion and seizures. Understanding the mechanisms by which peripheral
lesions can be epileptogenic can help establish
more effective treatments for seizures.
P-285
EVALUATION OF THE DEGREE OF DRUG
TREATMENT ADHERENCE IN PATIENTS WITH
EPILEPSY IN USE OF ANTIEPILEPTIC DRUGS
Fonte MHC, Segundo AFSM, Urquiza PAC, Valença LPAA
• UFPE
Introduction: There is a relative paucity of data
on adherence to drug treatment in patients with
epilepsy. The low adherence reduces the effectiveness of treatment and also increases the
predisposition to status epilepticus, physical
injury, hospitalizations, in addition to the psychosocial effects associated with uncontrolled
seizures. Objective: To assess the degree of drug
treatment adherence to antiepileptic drugs as
well as the factors associated with medication
adherence. Method: A cross-sectional study was
conducted in 135 patients with epilepsy at HCUFPE between October-December/2013. The
evaluation of drug adherence was made by the
Morisky-Green test (MGT) and Brief Medication
Questionnaire (BMQ). Clinical variables (seizure
frequency, age of onset and duration of epilepsy,
treatment regimen and the perception to adverse
events by Liverpool Adverse Event Profile - LAEP)
and socio-demographic variables (gender, age,
marital status, education level family income,
employment and supply of medication) were
compared between MGT≤2 and MGT≥3 groups
(Mann Whitney test or Fisher‘s exact test). The
scores of LAEP and MGT (Spearman test) were
also correlated. Results: 51.1% of patients were
women (69/135), the mean age was 36.73±14.53
years. The duration of epilepsy was 20.16±14.10
years and the age of onset of epilepsy was
16.26±15.46 years. 58.5% (79/135) at MGT (0‐2)
and 72% (77/107) BMQ regimen were considered
poorly adherent to therapy. The agreement between MGT and BMQ in the nonadherent group
was 84% and in the adherent group was 42.8%.
No influence of the different clinical variables
was noticed on drug adherence, except for LAEP
which average score was 41.84±11.84. 60.74%
(82/135) of patients showed scores considered
high (≥45). A significant difference in LAEP score
between the low adherence (44.77±11.55) and
high adhesion (37.7±11.09) groups was noticed
(p=0.0002, Mann-Whitney). There was an inverse
correlation between the LAEP score and the
MGT score (r= ‐0.2658 95% CI {‐0.4202 to ‐0.09638},
p=0.0018, Spearman). Conclusion: We found a
high frequency of poor adherent patients. There
was good agreement between MGT and BMQ in
identifying nonadherent individuals. We found
no clinical, demographic or socioeconomic factors associated with poor adherence, except for
LAEP. The lower the degree of drug adherence by
MGT the higher the score in LAEP.
106
P-287
FACTORS ASSOCIATED WHIT QUALITY OF LIFE IN
PATIENTS WITH EPILEPSY IN BRAZIL
Ferrari CMM1, Settervall CC2, Castro LHM2, Sousa RMC2
• 1CENTRO UNIVERSITÁRIO SÃO CAMILO;
2
ENFERMAGEM DA UNIVERSIDADE DE SÃO PAULO
Introduction: Quality of life is an important indicator of the individuals adaptation to a disease.
Currently, physical, psychological, emotional,
social, educational and vocational aspects of
individuals with epilepsy are assed by quality of life instruments, designed for this specific
population. Goal: The aim of this study was to
describe the quality of life of epilepsy patients,
showing associations with patient characteristics, disease, therapy and social support.
Methods: Prospective, transversal study of individuals with epilepsy in outpatient care. 385
patients, aged 18 years or older, literate, independent in daily living activities, without active
psychiatric disease. Quality of life was assessed
with the Quality of Life in Epilepsy Inventory‐31
(QOLIE‐31). Association tests were performed for
QOLIE‐31 and independent variables, followed
by multiple linear regression for quality of life.
Antiepileptic drug (AED) treatment complexity
and adherence were measured by the Epilepsy
Medication Treatment Complexity Index (EMTCI) and Morisky-Green test. Results: Average age
was 39.7±12.6 yrs, 53.5% were women, 79.5% had
symptomatic focal epilepsy, 71.1% were on multiple AEDs, 79% presented at least one seizure in
the preceding six months and treatment duration was 21.5±13.2 yrs. The EMTCI ranged from
2.0 to 44 (average 14.7±8.0). 60.5% of participants
had medium, and 5.7%, low adherence. Mean
QOLIE‐31 was 64.2±18.2, with lowest averages
seen in seizure worry (53.2±31.4), and medication effect (61.3±32.6). Highest scores were on
social function (69.8±25.7). Better quality of life
was associated with perception of seizure control and employment status. Patients with worse
quality of life received more support from family
or friends and were more often of African ancestry. Conclusion: The QOLIE‐31 scores indicated
that epilepsy had a negative impact on quality
of life. Patients of white and yellow race, those
inserted in the labor market and individuals
with controlled seizure had better quality of life.
Surprisingly, family or friends support showed
a negative impact on quality of life, possibly by
overprotection and restrictions in daily activities,
which may have hampered adaptation to illness,
interfering in quality of life. Keywords: Epilepsy; Quality of life; Ambulatory care; Outpatient
Clinics
P-288
GENETIC ASSOCIATION STUDY OF CLASS II
HLA DRB1, DQA1 AND DQB1 IN PATIENTS WITH
TEMPORAL LOBE EPILEPSY ASSOCIATED WITH
MESIAL HIPPOCAMPAL SCLEROSIS
Horta WG1, Leon SVA1, Paradela E1, Figueiredo A1,
Meira ID2, Pereira VCSR2, Moura P2, Rego C2,
Souza JPBM2, Paiva CLA1
• 1UNIRIO; 2UFRJ
Introduction: Temporal Lobe Epilepsy (TLE) is
the most common focal epilepsy in adults. The
neuroinflammatory mechanisms of epilepsy
have been described as an important factor in
the genesis of seizures and in refractory epilepsy, especially those with progressive behavior,
as occurs in temporal lobe epilepsy associated
with mesial hippocampal sclerosis (TLE-HS).
Objective: To study the profile of genetic susceptibility of patients diagnosed with TLE-HS investigating possible association between TLE-HS
and HLA class II system. Materials and Methods:
Peripheral blood samples of 42 patients with TLEHS and peripheral blood samples of 89 healthy
controls were collected. Analysis of the expression and typing of HLA class II DRB1, DQA1 and
DQB1 was performed using the method of Polymerase Chain Reaction (PCR) and identified by
sequencing. Statistical analyzes of the relative
frequencies of the alleles were obtained through
excell spreadsheet and the value of p, the relative
risks (RR) and odds ratios (OR) were calculated using Epi Info 6.0 software. The p value was
considered significant when it was <0.05 after
Bonferroni correction. Results: The HLA-DRB1 *
13 : 02 allele was the only one statistically significant (P=0.01) when comparing their frequency
among patients and controls. However, this value did’t resist the Bonferroni correction (P=0.44).
The other alleles of HLA-DRB1, HLA-DQB1 and
HLA-DQA1 regions showed no significant association. Conclusion: We found the trend as a
susceptibility factor association with HLA DRB1
* 13: 02 allele in TLE-HS.
P-289
ICTAL CONTRALATERAL INVOLVEMENT IN
UNILATERAL MESIAL TEMPORAL SCLEROSIS IS
ASSOCIATED WITH MEMORY PERFORMANCE: A
WADA TEST STUDY
Moreira GP, Lima Filho HC, Passarelli V, Pinto LF,
Jorge CL, Valerio RMF, Castro LHM
• HC-FMUSP
Objective: To evaluate the effect of contralateral
electrographic involvement on memory (measured by Wada memory asymmetry) in patients
with unilateral mesial temporal sclerosis (MTS)
associated epilepsy. Methods: We studied fifty
patients (27 women) with drug resistant epilepsy
and unilateral MTS (29 with left MTS) submitted
to prolonged noninvasive video-EEG monitoring
and bilateral Wada test. Patients were classified
in two groups: Contralateral Ictal Involvement
(IIC+): one or more seizures evolving with rhythmic activity in the contralateral temporal region.
Absent contralateral ictal involvement (IIC-): all
seizures with ictal EEG activity in the ipsilateral
temporal region and absent contralateral ictal
activity or not fulfilling criteria for IIC +. A twelveitem recognition paradigm was used for Wada
memory testing. Wada memory asymmetry
(MemAsy) score was calculated for each patient
subtracting the number of recalled items after
ipsilateral injection (to the lesion) from the number of recalled items after contralateral injection.
Expected asymmetry (EA) was considered when
MemAsy >0, and reversed asymmetry (RA) when
MemAsy ≤0. Results: The two groups did not
differ in demographic, clinic and videomonitoring variables. The IIC+ group presented higher
frequency of RA (57.2% vs 27%; OR: 3.55, 95 CI
1.07‐11.08; p:0.03). Conclusion: Ictal contralateral
involvement in unilateral MTS is associated with
decreased memory performance evaluated with
the Wada test.
XXVI Congresso Brasileiro de Neurologia
P-290
INFLAMMATORY AND APOPTOTIC BIOMARKERS
ASSESSMENT IN EPILEPSY PATIENTS
Fighera MR, Kegler A, Arend J, Palma R, Arceno P,
Bertazzo T
• UFSM
Epilepsy is a neurological disease, characterized by a brain tissue disfunction, whose consequences affect different areas, as example,
neurocognitive area. Recently researches are
linking inflammatory processes with the phatophisiology of epilepsy, establishing cytokines
could be seizures mediators. Such as recent researches are connecting inflammation with possible seizures developments, the objective of the
study is to quantify and compare inflammation
biomarkers in epilepsy patients with healthy
people. A total of 83 volunteers participated of
the study (42 diagnosed epilepsy patients from
Santa Maria Hospital University and 41 healthy
control). Interleukin‐1, Interleukin‐6, Interferon,
Tumor Necrosis Factor, Caspase 1, Caspase 3 and
Caspase 8 were measured and analyzed. The results demonstrated inflammatory and apoptotic
markers increased, when compared with control group. Interleukin‐1 (t=5.310), Interleukin‐6
(t=12.20), Interferon (t=10.38), Tumor Necrosis
Factor (t=9.100) and apoptotic markers Caspase1
(t=20.59), Caspase3 (t=24.90) and Caspase8
(t=20.84). P<0.0001 was considered significantly.
According to the results, epilepsy patients indicated higher leves both inflammatory and apoptotic markers, suggesting a relation between
pathophysiology of epilepsy with increased levels of inflammation and cell death markers.
P-291
LATE ONSET TEMPORAL LOBE EPILEPSY DUE TO
CEREBRAL HYPOPERFUSION: CASE REPORT
França WCSC, Alvim MKM, Pavan P, Morita ME,
Coan AC, Cendes F
• UNICAMP
Introduction: Temporal lobe epilepsy(TLE) is
the most common form of refractory focal epilepsy in adults. Generally, it is secondary to hippocampal atrophy (HA) and other MRI signs of
hippocampal sclerosis (HS), which has been
associated with prolonged febrile and recurrent
seizures, but the relationship between repeated seizures and HS is not wellknown. Aim: To
describe a late onset TLE patient with a hippocampal atrophy due to probable ischemic etiology. Case report: Male, 65 years old, with arterial
hypertension and schistosomiasis associated
with portal hypertension. Underwent to an Azigo-portal disconnection in September 2007.
During surgery, there was bleeding in large quantity. After surgery he had an episode of nonspecific malaise followed by loss of consciousness
and automatism in the right hand during seconds without postictal symptoms. Since then, he
had such episodes about seven times a month. In
May 2013, there was an increase in the frequency
of episodes and he started treatment. In November 2013, he reported medication side effects and
no significant improvement in seizure frequency.
Neurologic examination was normal. He had no
other risk factors for epilepsy, with the exception of the surgery described above. MRI showed
bilateral HA, more pronounced on left. EEG
showed infrequent epileptiform activity and frequent non epileptiform activity in the left temporal region. Treatment was optimized. The patient
has been followed in our clinic. Discussion: This
Case report presents well-established temporal
relationship between an abdominal major surgery with cerebral hypoperfusion and epilepsy.
In a study in rats, the model of four-vessels occlusion causing cerebral ischemia led to seizure
in most animals during the first hours of reperfusion. Similar mechanism may be assigned to
our patient. Hippocampal injury can lead to
increased levels of neurogenesis that appear to
be beneficial in acute hippocampal dysfunction.
In the chronic phase of injury, abnormal neurogenesis and synaptogenesis that arise in this area
may lead to aberrant reorganization of synapses
and progressive loss of GABAergic inhibition,
generating decline in neurogenesis and inflammation, which is potentially epileptogenic. The
literature shows that stem cells of the nervous
system used in the early injured hippocampus in
an animal model with minor injuries may have
therapeutic value in preventing the development
of HS and epilepsy.
P-292
MEASUREMENT OF DISPLACEMENT IN ABSTRACT
FIGURE RECALL
Rocha SFB1, Santos W1, Guebert M1, Meneses MS1,
Kowacs PA1, Ferraz AC2
• 1INSTITUTO DE NEUROLOGIA DE CURITIBA; 2UFPR
Background/aims: Temporal lobe epilepsy has
been associated with verbal memory deficit in
left temporal lobe epilepsy and nonverbal memory deficit in right temporal lobe epilepsy (visual
and spatial memory). Putative mechanisms to
explain spatial memory include the spatial mapping theory, which is based on the ability to learn
positional relationships. Objective: The objective of this study was to compare the results of
the Spatial Position Test with other visuospatial
tests applied to right temporal lobe epilepsy patients, left temporal lobe epilepsy patients and
healthy controls. Methods: Twenty-four subjects
matched for age and level of education were
selected (10 with right temporal lobe epilepsy,
seven with left temporal lobe epilepsy and seven
healthy controls). The assessment also included
WAIS subtests, the Logical Memory Test and the
Rey-Osterrieth Complex Figure Test. Pharmacological bias was screened with the Adverse Events
Profile questionnaire. Our results showed the
spatial memory task to be superior in detecting
visuospatial abnormalities (in the delayed spatial location subtest) in the right temporal lobe
epilepsy group compared with the left temporal
lobe epilepsy group (p<0.04) and control group
(p<0.002). In the other components of visuospatial memory testing (visual learning, recognition
and delayed visual recall), the performance of all
groups was similar. Additionally, the right and left
temporal lobe epilepsy groups performed poorly
in the Rey-Osterrieth Complex Figure test compared with the control group. Discussion: These
results support the hypothesis that positional
memory (delayed spatial memory) is the most
sensitive spatial memory subcomponent among
those used to detect memory asymmetries in
right vs. left temporal lobe patients. Conclusion:
More sensitive tasks to elucidate spatial memory
are essentials in the epilepsy surgery programs
to elucidate underestimated function deficits involving nondominant hyppocampal system.
P-293
MUSICOGENIC EPILEPSY - A CASE REPORT
Vasconcelos RC, Andrade RS, Marques LHN,
Limonte FH, Borges APP, Ferreira KS
• FAMERP
Introduction: The musicogenic epilepsy corresponds to the occurrence of seizures triggered
by musical stimuli. Does not constitute an epileptic syndrome, it is why should speak of reflex
seizures triggered by music at an epilepsy syndrome. It is a rare disease (1 in 10 million individuals), which generally occurs after the age of
20. Objective: To report the case of a patient with
reflex seizures triggered by a particular song in a
focal epileptic syndrome. Case report: A woman,
34 years old, reports that in 2005 she started episodes characterized by stop behavioral and swallowing movements, which usually last less than
1 minute. She describes perception of onset of
seizures with intense fear, “butterflies”, déjà vu,
lasting 5‐10 minutes. Also refers strong post-ictal
headache. Seizures are triggered by menstrual
period, when watching the movie “City of Angels” and when listening to the theme song “Angel - Sarah Mclachlan,” which was played at the
entrance of her wedding. At the beginning, the
rate of the attacks was every three months, but
a year and a half ago, the same period that extend her workload, there was an increase in seizure’s frequency, which became fortnightly. Now
she is in polytherapy with lamotrigine, valproic
acid and topiramate. The patient was admitted
for prolonged video-EEG monitoring (PVEM),
staying for four days asymptomatic, without
response to photostimulation and hyperventilation. On the 5th day of hospitalization, after
watching the film reported, she presented a focal
seizure without impairment of consciousness
and after listening to the song “Angel” there was a
discognitive focal seizure that progressed to generalized tonic-clonic seizure, bilateral seizure.
Both clinical events were concomitant with electrographic crisis with predominant epileptiform
activity in anterior temporal hemispheres. The
brain MRI was normal. Studies of brain SPECT
(baseline and ictal) were performed and concluded high probability of epileptogenic zone in
the left temporal lobe. Discussion: Musicogenic
epilepsy appears to represent a peculiar form of
complex reflex epilepsy, with a strong correlation
to the temporal lobe. It appears to depend on certain emotional reaction mediated through limbic
mesial temporal lobe structures. Clinical studies
suggest that the processing of music within the
human brain involves numerous cortical areas,
working within connected networks, that could
be recruited during a seizure manifesting as musical phenomena.
P-294
PERCEIVED CHANGES IN MEMORY IN PATIENTS
UNDERGOING EPILEPSY SURGERY
Guebert M, Santos W, Lucia EMS, Laroca HNHS,
Rocha SFB, Kowacs PA, Meneses MS
• INSTITUTO DE NEUROLOGIA DE CURITIBA
Background/Aims: Epilepsy is a neurological disorder that affects 0.5 to 1% of the world‘s population. About 30% of cases do not achieve epileptic
seizure control. The epilepsy surgery, in particular the temporal lobectomy, can lead to the control of the crises and to the decrease of the medication.Despite the improvement in crises, the
perception that patients have of their cognitive
performance post-surgery may not correspond
to this clinical improvement. Objective: Evaluate
107
Posters
the subjective perception of memory of a patient
who underwent epilepsy surgery. Methods: Fifty-one patients from 163 subjects operated on
answered the questionnaire for self-assessment
of processes of verbal and visual-spatial memory related to pre- and post-operative period.
Surgeries consisted of seven lesionectomies,
22 amygdalohippocampectomies, 15 temporal
lobectomies, one parieto-temporal ablation,
one corticectomy, one resection of meningioma, four implants of vagus nerve stimulation.
Results and Discussion: improvement in memory was reported by 35% of the patients after
surgical procedure, 35% reported no change and
30% perceived a decline of mnemonic processes.
From these cases 91% believed that the change
in memory had an impact on daily life. Thirteen
patients amygdalohippocampectomy with lesion in the left side: 54% reported improvement
in memory, 15% showed no change and 31%
reported worsening. Of these, 69% reported impact on quality of life. Nine patients had surgery
on the right side: 11% reported improvement in
memory and 89% no changes. Conclusion: The
data suggest that the memory is considered an
important factor in quality of life and that patients with lesions at the left side refer more
changes in memory than patients with surgery in
the non-dominant hemisphere. Subjective perception is related with patient’s expectations and
may differ from Objectives: results of the surgery,
the pharmacological treatments and the change
in the control of seizures. Comparison with the
cognitive Objectives: data can elucidate these
discrepancies.
P-298
PROFILE OF EPILEPSY INCIDENCE CONSIDERING
SEX AND AGE IN 2004-2012 LONGITUDINAL STUDY
BASED ON MEDICAL FILES IN SAO JOSE DO RIO
PRETO-BRAZIL
Borges MA, Gasparini CS, Morais TLB, Borges APP,
Lopes LC, Min LL, Valêncio CR, Scarpelini Neto P,
Zanetta DMT
• FAMERP
Background: Epilepsy remains a public health
problem. The goal of this study was to determine
the profile of the incidence of epilepsy, considering the age and sex in the city of São José do Rio
Preto (424.466 hab. in 2012) situated in the state
of São Paulo / Brazil in the period 2004 - 2012.
Methods: A longitudinal epidemiological study
of epilepsy based on computerized patient and
hospital records was performed, from 1/1/2004
until 31/12/2012. Pearson x2 tests, “Two-Sample
T-Test” was used and for a confidence interval of
95% (95% CI) for analysis of the results and considered significant for p-value <0.05. Results: A
total of 6589 people with epilepsy were identified
with 2308 being treated by the lead researcher.
The average age-adjusted incidence of epilepsy
is higher in the first four years (774,6.105 hab. /
year in males and 507.5 for females), stabilizes at
a level of 50 / 105hab. in both sexes, rising again
after 70 years when it reaches 290 / 105hab.,
especially in men (p=0.0001). Conclusion: The
overall mean incidence was high in the first decade of the second millennium similar to the
incidence in under-developed countries. The
overall prevalence of epilepsy is high, similar to
rates in under-developed countries. The main
risk factors were stroke, perinatal causes, mesial
temporal sclerosis, head trauma, meningitis, alcoholism and cysticercosis.
108
P-300
QUANTITATIVE NEUROIMAGING OF IDIOPHATIC
GENERALIZED EPILEPSY: A STUDY OF THE
CINGULATE CORTEX
Braga AMS, Verdade RC, Paschoalato RP,
Paschoalato RP, Yamashita S, Betting LE
• UNESP
Introduction: Studies using techniques of
quantitative neuroimaging have showed subtle abnormalities in patients with idiopathic
generalized epilepsy (IGE). These findings have
several locations but the midline parasagital
structures are more commonly implicated. The
cingulate cortex is related and may be involved.
Objective: The objective of the current investigation was to perform a comprehensive analysis of
the cingulate cortex using multiple quantitative
structural neuroimaging techniques. Method:
Thirty-two patients (19 women, 29±9 years) and
36 controls (18 women, 32±11 years) were submitted to 3T MRI. The volumetric 3D sequence
was acquired and used for investigation of the
cingulate cortex. Images were submitted to automatic processing using the FreeSurfer routines
and recommendations. Structural parameters
extracted were area, curvature index, volume,
folding index, Gaussian curvature, mean curvature and thickness. A voxel-based region of
interest analysis comparing the cingulate cortex of the two groups also was conducted using
SPM8 and VBM8 softwares. Shape analysis was
finally conducted on the anterior rostral, anterior caudal, posterior and isthmus cingulate using
SPHARM-PDM. Results: Twenty-five (78%) of
the patients presented abnormalities in at least
one of the structural parameters evaluated. VBM
analysis showed differences mainly in the anterior cingulate (656mm3, p=0.001). Shape analysis
also demonstrated a predominance of anterior
cingulate abnormalities specially observed in the
distance map. Conclusion: Current investigation
suggests that some patients with IGE have structural abnormalities in the cingulate gyrus mainly
localized at the anterior portion. This finding is
not constant and may variate among patients.
P-301
REFLEX EPILEPSY: EPILEPSY GRAPHOGENIC
Rossi ACA, Santos MCV, Chagas Filho CAQ, Christo PP,
Xavier MT, Gomes Neto AP
• HOSPITAL SANTA CASA DE BELO HORIZONTE
Introduction: Reflex Epilepsy encompasses a
spectrum of crises evoked by sensory stimulation determined. The graphogenic type is a
rare form of epilepsy induced that also includes
language-induced epilepsy and speech reading. Objective: To report a case of seizure induced by the act of writing with his right hand.
Case report: APN, 33, male, married, native of
Ant (MG) started the occurrence of seizures at
4 years of age that persist to the present day,
in varying frequency in free intervals of four
months of crisis and similar. The crisis is characterized by abrupt abduction and extension of
the right upper limb, quickly followed by asymmetric stiffness in 4 limbs, progressing to clonia
right hemifacial version of the head to the right
tonic-clonic movements and widespread. The
patient declare that crises are precipitated particularly by the act of writing with his right hand,
but also other movements can, like shaving, fine
motor and perform acts in his sleep phase inicial.
Was made Video-EEG performed presenting EEG
record crisis with character epileptogenic. Also
underwent MRI scans with an extensive lesion
in the left superior frontal gyrus with the appearance of low grade glioma. Discussion: The reflex
epilepsy are rarely found in the population. The
graphogenic form of epilepsy is a reflex epilepsy, epilepsy induced as part of the language. The
graphogenic epilepsy is much more common
associated with induced language and reading.
As main differential diagnosis, in literature and
in the case above , has the myoclonic epilepsy,
making it very important EEG monitoring with
video, for correct identification and subsequent
treatment this type of crisis.
P-302
REFRACTORY EPILEPSY IN A PATIENT WITH A
SCHIZOAFFECTIVE DISORDER AND INTERICTAL
PSYCHOSIS
Maia IHM1, Melo MCA2, Lobo FVP2, Silva Júnior JMS2,
Andrade JBC1
• 1SERVIÇO DE NEUROLOGIA DO HOSPITAL GERAL
DE FORTALEZA; 2HOSPITAL DE SAÚDE MENTAL
PROFESSOR FROTA PINTO
Introduction: The association between epilepsy and schizophrenia is known in the literature,
however only a few studies treat the relation
between epilepsy and schizoaffective disorder.
Objectives: systematize the current knowledge about psychiatric symptoms in epileptic
patients, using as an example the association
between the schizoaffective disorder and the
refractory epilepsy, providing a source of additional study and a better way of dealing with
such diseases by neurologists and psychiatrists.
Case report: R.L.S., female, 51 years old, began
her simultaneous epileptic seizures and auditory and visual hallucinations by the age of 12. She
used to listen to voices demanding her to commit suicide and “saw dead stabbed people”. The
epileptic seizures happened among psychotic
crisis. They were simple partial seizures and,
most of the time, they generalized, ending with
loss of conscience. By the age of 16, she started
to show symptoms of anhedonia, hypobulia, asthenia, depression and easy crying, besides loss
of hope. She tried suicide three times, always associated with psychotic symptoms. Her depression symptoms have lasted for 8 years, when she
demonstrated disproportionate rage, accelerated
thoughts, logorrhea and aggressiveness with her
children. She felt like “breaking objects, taking
off her clothes and run about”. She cleaned her
house over and over and washed her clothes several times. She went throught situations in which
she wanted to be rich and made a lot of debts,
purchasing clothes exaggeratedly. She was treated irregularly by the psychiatrist and neurologist
for 34 years. The hallucinations never stopped.
EEG revealed an epileptiform activity at the left
frontotemporal region. The CT of the skull did
not show any alteration. Neurological exam was
normal. She is currently in a semi-intensive unity
of interneship, making use of valproic acid 1g/
day, carbamazepine 600 mg/day, olanzapine
10mg/day, diazepam 40mg/day and carbolitium
600mg/day. The epileptcal and psychoptical
crisis have not stopped. Discussion: This case
illustrates the need of broadening the studies
about psychiatric comorbidities on patients with
epilepsy, so that we can better understand its influence and neurological mechanisms. The sum
of the related experiences can be used as material for new studies, besides better guiding the
attending physician on his therapeutic decisions.
XXVI Congresso Brasileiro de Neurologia
P-303
SECONDARY TO LIMBIC ENCEPHALITIS EPILEPSY:
ELETROCLINIC AND NEUROPSYCHOLOGICAL
STUDY
Leão AVR1, Almeida Neto JG1, Santos CJA1, Costa RT1,
Carlos KCL1, Barros BCC1, Lima KM1, Amancio KAL1,
Alúcio KT1, Bomfim RC2, Gameleira FT1
• 1UFAL; 2HMAR
Introduction: Limbic Encephalitis is a inflammatory disorder that involves regions of the
hippocampus, amygdala and insula, and the mesial temporal lobe epilepsy patients in a typical
manifestation. Objectives: This study reports the
clinical, electrophysiological and neuropsychological evaluation of two patients who developed temporal lobe epilepsy secondary to mesial
limbic encephalitis, since it constitutes a rare
disease with a broad differential diagnosis. Case
1:JQRS,42,male,presented focal epileptic aura
type of dizziness, followed by disconnection with
the environment, “staring” and cephalic version
to the right. The duration of this episode was several minutes, with suggestive facial expression
of intense pain,and masticatory and bimanual
automatisms. Evolved immediately with a status epilepticus non-convulsive(SENC),expressed
by a post-ictal coma for five days,with complex
partial seizures subcontinuos.The EEG during
the SENC,a zone revealed onset of ictal and a
mesial temporal left zone symptomatogenic.
Magnetic resonance imaging showed a left hippocampal sclerosis.The liquor showed a protein
concentration and a lymphocytic pleocytosis.
Makes use of Oxcarbazepine and have 3‐4 complex partial seizures/month. Absence of generalized tonic-clonic of seven years.Neurological
examination is normal and neuropsychological
assessment shows a significant deficit in recent
memory. Case 2: A.M.S,female,28 years old, presented aura-type malaise,followed by disconnection with the environment and unconscious myoclonus in the left limbs. Also tonic-clonic generalized seizure with mental confusion post-ictal
for several hours and new CCTCG interspersed
during 03 days without regaining consciousness
for over 10 days. After wake of this crisis, now
has behavior auto/hetero-aggressive, besides
expressing a lush hypersexuality verbal. Evolved
over the following months, with complete regression of hypersexuality.The ictal EEG showed a
left temporal epileptogenic activity electroclinical.CT scan of the skull was normal and MRI
revealed an extensive hyperintense on FLAIR in
the left latero-basal temporal region. Discussion:
Patients with limbic encephalitis usually present
with subacute progressive impairment of shortterm memory, seizures, mental confusion, sleep
disorders and psychological disorders, such as
personality or behavior changed.1,2,3The diagnosis is based on established and the recognition
of this disease is important for treatment and
prognosis.3,5
P-304
SOCIAL AND OCCUPATIONAL FUNCTIONING SCALE
FOR EPILEPSY (SOFSE) – TRADUÇÃO E ADAPTAÇÃO
PARA O PORTUGUÊS – BRASIL
Fernandes PS, Meiga C, Pinto LM, Nickel R, Silvado C
• UFPR
Introduction: Epilepsy can cause significant
restrictions in activities of various areas of life,
compromising quality of life and functional and
psychosocial status. The Social and Occupational Functioning Scale for Epilepsy (SOFSE) was
developed by Wei-HanWang and collaborators
(Epilepsia, 2013) with the objective of evaluating
the social and occupational functioning of people with epilepsy. It contains 30 items, divided
in six dimensions: interpersonal relations, communication, social activities, leisure activities,
instrumental living skills and occupation. Its
score varies from 0 to 100, with higher values
reflecting better functional status. Objective: To
translate and adapt the Social andOccupationalFunctioningScale for Epilepsy (SOFSE) to the
language and culture of Brazil;to verify the subjects’ understanding in relation to the translated
version of the scale; and to use it to assess the social and occupational functioning of people with
epilepsy in Brazil. Methods: The process of the
translation and cultural adaptation of the SOFSE
followed guidelines widely used in this type of
study. The application of the translated version
of the SOFSE has been undertaken in patients
with temporal lobe epilepsy, who are literate, of
both sexes, without any other associated health
conditions considered capable of compromising
the participant’s performance, and who were being attended in the Difficult-to-Control Epilepsy
Outpatient Center at the Federal University of
Paraná Teaching Hospital. Results: In the analysis of the first 43 patients, we observed that 13.9%
of them evidenced severe functional compromise (<50), 58.1% showed moderate functional
compromise (50 – 75) and 23.25% obtained a
score above 75 points, demonstrating mildfunctional compromise (>75). The areas of life affected most were: occupation, followed by leisure
activities, social activities and communication.
None of the participants reported difficulty in
relation to understanding the questions or terms
of the Portuguese version of the questionnaire.
Conclusion: The translated version of the SOFSE
was considered easy to understand and valid for
analyzing the social and occupational functioning of the subjects evaluated. This study’s results
will serve for ascertaining which areas of the
lives of subjects with epilepsy are compromised
most, for collecting data on the progression of
their condition, and also as a measurement of
outcomes in clinical and interventional studies.
P-305
SOURCE INVESTIGATION OF INTERICTAL
EPILEPTIFORM DISCHARGES IN MESIAL
TEMPORAL LOBE EPILEPSY
Peixoto DEB, Betting LEGG, Oliveira LAB, Yamashita S
• UNESP
Objectives: Hippocampal sclerosis (HS) is the
pathological substrate behind mesial temporal
lobe epilepsy (mTLE). Epileptiform discharges
probably arise from the mesial structures but
neocortex also may play a role in their generation. Investigations also show that patients with
left or right HS may have different clinical and
neurophysiological profiles. The objective of this
investigation was to explore neurophysiological
features in patients with right and left interictal
spikes. Methods: Thirty-one patients with mTLE
with typical interictal discharges were selected.
EEG exams were recorded with 10‐20 system of
electrode placement using Nihon-Kohden system. Source localization was conducted using
BESA software. Spikes were selected, centered
and averaged. Equivalent dipole and Classical
LORETA Analysis Recursively Applied (CLARA)
algorithms were applied for source localization.
Statistical analysis was conducted using t test
comparing the CLARA maps of right and left discharges. Statistical level selected was a corrected
p<0.05. All patients and a control group of 35 individuals (18 women, mean age 32±7 years) were
also submitted to 3T MRI. Volumetric sequence
was used for structural analysis. All hippocampi
were automatically segmented with FSL. Results:
1562 discharges were evaluated. 15 patients had
left discharges, 12 had right and 4 had bilateral.
HS was observed in 20/31(64%) patients. Mean
hippocampal volumes were 3202±569 mm3 for
the right and 3211±591 for the left hippocampus.
Of the 11 patients without HS, 4 had right and 7
left discharges. Source analysis showed similar
findings for the right and left discharges. A total
of 78 sources were computed. The 3 main structures involved were: claustrum (21/78, 10 right
and 11 left), inferior frontal gyrus (15/78, 8 right
and 7 left) and superior temporal gyrus (10/78,
4 right and 6 left). Mean images produced by
CLARA and the overlap of all right and left analysis disclosed involvement of the posterior portion of the corpus callosum and cingulate gyrus
in the right discharges. Slightly posterior distribution and contralateral medial temporal lobe
involvement was observed in the left discharges. Conclusions: EEG source analysis showed
similar findings to neuroimage investigations
demonstrating extra-hippocampal involvement.
It was also disclosed a different neuroanatomical
involvement in patients with right or left HS.
P-306
STURGE-WEBER SYNDROME WITHOUT FACIAL
NEVUS
Machado DF, Pinto TVL, Santos MCV, Christo PP,
Gomes Neto AP
• SANTA CASA DE BELO HORIZONTE
Introduction: The Sturge-Weber Syndrome
(SWS) is a rare, congenital disease, characterized
by cutaneous and leptomeningeal capillary malformation. The classic clinical triad consists of
seizures, mental retardation and a “port-wine”
facial nevus. Objective: To describe a Case report
of Sturge Weber Syndrome without the typical
port-wine stain. Case report: W.E.R., 39-y-old
woman, with an initially difficult management
Epilepsy, since she was 4 y of age. She developed
permanent left hemiparesis grade 4/5, since adolescence. The seizures were focal with secondary generalization and the postictal phase was
marked by the hemiparesis impairment, which
persisted for several weeks. Report of delayed
motor development and mild learning difficulties. From adolescence on, alcoholism was significant, associated with uncontrolled epilepsy.
Cranial Tomography performed in June of 2014
showed the giriforme calcification in the right
parietal-occipital region. Cranial Magnetic Resonance imaging revealed parietal-occipital angiomatosis, ipsilateral choroid plexus enlargement
and right cerebral hemiatrophy. The EEG showed
mild, but diffuse disorganized background activity, without identifiable epileptic foci. Discussion:
SSW or Encephalotrigeminal Angiomatosis is the
most frequent neurocutaneous disorder (1 per
50,000 live births). The pathophysiology involves
the abnormal venous drainage of the cortex.
There may be deep veins recruitment, leading
to increased volume and vascularity of the choroid plexus. Chronic cerebral ischemia leads to
cortical atrophy with dystrophic giru calcification. The diagnosis clue is a nevus affecting the
V1 distribution, with or without involvement of
V2 or V3. However, in rare cases, there is no flammeus nevus. Leptomeningeal angiomatosis is
the defining condition of the disease. Ipsilateral
glaucoma may occur. The post-ictal, transient,
109
Posters
neurologic deficits are described as extended,
which typyfies the AVE-like episodes, that may
last weeks. Cognition ranges from normality to
severe deficits. There may be other neurological
deficits depending on the leptomeningeal angioma area. Behavioral, mood disorders and migraine are frequent.
P-307
SUBJECTIVE AND Objective PARAMETERS OF
SLEEP IN REFRACTORY MESIAL TEMPORAL LOBE
EPILEPSY
Lima AVS, Watanabe N, Walz R, Sukys-Claudino L, Lin K
• UFSC
Introduction: Sleep complaints are common
among patients with epilepsy (PWE). Objectives:
To evaluate the sleep of patients with TLE with
hippocampal sclerosis (HS) through standardized sleep scales and polysomnography (PSG).
Methods: Fifty-six consecutive adult patients
(over 18 years of age) with drug-refractory mesial
temporal lobe epilepsy (MTLE), who were considered candidates for epilepsy surgery, were interviewed for clinical sleep assessment through
Epworth Sleepiness Scale (ESS), Pittsburgh Sleep
Quality Index (PSQI), Stanford Sleepiness Scale
(SSS) and Fletcher & Luckett Questionnaire
(FLAQ) and they were submitted to one night
PSG evaluation and approximately five days of
video-EEG. Results: Thirty-eight patients (67.9%)
answered to the ESS, SSS, PSQI and FLAQ. Nine
(16.1%) patients had clinically significant excessive daytime sleepiness (EDS) (ESS>10), 4 patients
(7.1%) had SSS ≥3, 15 (26.8%) had PSQI >5 and 8
(14.3%) had FLAQ scores >1. In the PSG study, the
mean sleep efficiency was 86.1±9.5%(mean±SD),
sleep latency (28.1±25.2min.), REM latency
(104.1±60.2min.), WASO (17.4±15.6%), N1 sleep
(7.5±4.6%), N2 sleep (49.0±10.6%), N3 sleep
(26.6±11.8%), REM sleep (16.7±6.6) and awake
index (11.59±6.6min.). Ten (17.9%) PWE showed
REM without atonia, and 2 (3.6%) Sleep Onset
REM period (SOREMP). Forty-one (73.2%) PWE
showed alpha delta sleep pattern, and 4 (7.1%)
had seizure during the PSG. Conclusion: We
evaluated sleep quality based on standardized
scales and objective parameters in a homogeneous group of patients with drug-refractory
MTLE. We observed sleep fragmentation, increased WASO, reduced REM, higher N3 sleep,
and a significant number of alpha delta pattern.
These findings may be caused by the presence
of discharges during sleep, use of antiepileptic
drugs or the occurrence of other sleep disorders.
It is important to recognize these factors in order
to improve their quality of life.
P-308
THE VALUE OF CONTINUOS VEEG RECORDINGS
FOR THE DEFINITION OF ICTAL ONSET ZONE IN
THE PRE-SURGICAL EVALUATION OF MEDICALLYREFRACTORY EPILEPSIES
Volkmann AF, Paola L, Crippa AC, Hoepker C, Terra V,
Germiniani F, Silvado C
• UFPR
Introduction: Recording a sufficient number of
typical seizures (szs) for adequate localization of
seizure onset and to evaluate for a possible surgical treatment can be laborious. Also, if VEEG
recording are performed only during daytime,
several seizures can be missed. Objective: To
evaluate the role of VEEG for the localization of
the zone of ictal onset; as well as determining
the minimal duration for adequate sampling of
110
typical epileptic seizures, the period with higher seizure incidence, to evaluate potential risks
and to compare these findings among different
groups of epilepsy. Methods: We performed a
retrospective study of VEEGs performed as part
of the investigation protocol for epilepsy surgery in our centre. Technical notes, VEEG reports
and patients’ files of the 1044 VEEG recordings
performed were reviewed and we included
those with a continuous duration of 24 hours or
more. Incomplete data, registries of exclusively Non-Epileptic Psychogenic Szs, intracranial
monitoring and those with more than 10 szs per
exam were excluded. VEEG recordings were classified according to localization into five groups:
frontal, temporal, posterior quadrant, generalized or undetermined. Results: We included
655 VEEGs, 57% of them of male patients. Mean
duration of VEEG recordings was 56.7±46.5 hr
for generalized onset seizures and 99.9±16.4 for
focal onset; with a mean duration of 102.5±62.5
hr for temporal onset (524 VEEGs). Antiepileptic
drugs were tampered down in 50.6% of temporal, in 48.6% of frontal szs, 33.3% of posterior
quadrant szs and in 50% of generalized onset szs.
Complications were more frequent in the temporal group (unrecorded szs in 16% and either a
prolonged sz or status in 1.7% of VEEGs). Mean
duration of VEEG recording until the first ideal sz
was 56.6 (± 52.1) hr for frontal szs; 53.2 (± 48.5) hr
for temporal szs; 39.7 (±32.4) for posterior quadrant szs. The zone of ictal onset was adequately localized in 67.7% of temporal szs, in 63% of
posterior quadrant szs, in 40% of frontal szs and
in 4% of generalized szs. For the group of focal
epilepsies, 24.4% of szs occurred in the morning,
33.7% in the afternoon and 41.9% at night. 70.1%
of szs occurred during arousal. Conclusions: The
minimum recording time of VEEG for determining the zone of ictal onset should range from 53
hr (1st ideal sz) to 79 hr (last recorded sz). VEEGs
recordings should include all circadian periods,
as much as 42% of all szs occurred only during
nighttime.
P-309
THERAPEUTIC DRUG MONITORING OF
TOPIRAMATE AND ASSOCIATIONS IN SALIVA
SAMPLES BY LC-MS/MS
Amorim JC1, Cerqueira LB1, Trzaskos R1, Smach T2,
Silva PR2, Silvado CES2, Piovesan EJ2, Pontarolo R1,
Campos FR1
• 1UFPR; 2HC-UFPR
Introduction: Epilepsy is a disorder characterized by seizures and the treatment was performed with drugs called anticonvulsants. Topiramate is an anticonvulsant of new generation
which has its use generally associated with other
already well established anticonvulsant. Thus,
there is a need to develop Methods: to monitor
the concentration of topiramate and their associations in the plasma circulation of each patient
allowing the design of individualized treatment
protocols and reduce side effects. The saliva appears as optimal for this type of array analysis,
as it allows a simple, easy and well accepted way
too collect samples from the patients. Objetive:
The aim of this work was develop and validate
a method for LC-MS/MS able to monitor the
concentrations of topiramate, phenytoin and
phenobarbital in plasma and saliva matrices.
Methods: For the method development several
analysis conditions was tested. The best results
were acquired using an aliquot of 30 µL of plasma and saliva followed by the extraction process
by protein precipitation solution. Subsequently, 20 µL of the supernatant was injected into a
LC-MS/MS System. For the quantification was
used prednisone as internal standard and the
experiment of Multiple Reaction Monitoring
(MRM). Samples analyzed in the present study
were obtained from 13 adults with epilepsy using
different doses of topiramate, who attended the
Department of Neurology, Hospital de Clinicas
of Paraná. Results: The method was validated
for plasma and saliva matrices, with excellent results of precision, accuracy and linearity (r>0.99).
Moreover, it was possible to maintain the same
methodology for quantification of pharmaceuticals for both matrices, which makes it possible to
state the actual correlation between plasma and
salivary concentration for associations of topiramate, phenytoin and phenobarbital. It was observed that three hours after the administration
of topiramate the drug concentration in plasma
and saliva were extremely close, indicating that
after this period the relationship between the
concentration of saliva / plasma is close to 1.
Conclusion: Thus was provided a reliable, simple, rapid and low cost method by LC-MS/MS
for the analysis of plasma and saliva samples
from patients treated with topiramate and some
association. The results indicates that the use of
saliva for topiramate drug monitoring is reliable
and brings many benefits for the welfare of the
patients.
P-310
TRANSIENT GLOBAL AMNESIA X TRANSIENT
EPILEPTIC AMNESIA: A CASE REPORT
Silveira JOF, Amaral CBMS, Dib JG, Cal HSR, Pupe CCB
• UFF
Introduction: Memory is the capacity to store
and retrieve information. Impairment of this
ability is called amnesia and occurs in association with a variety of neurological disorders. An
acute amnesic syndrome can be caused by an
ischaemic insult, hypoglycaemia, adverse drug
effects, psychiactric conditions, head trauma,
transient global amnesia (TGA) and epilepsy.
Purpose: report a case of an acute-onset transient
amnesia in a woman with break contact episodes
with automatisms, suggesting the occurrence
of a TGA in an epileptic patient. Case report: A
63-years-old woman, with diabetes and hypertension, experiented an episod of amnesia. She
began asking repeatedly where she was and the
date, without loss of consciousness or personal
identity, weakness or paresthesia. In the emergency room she was found to be amnesic with
normal neurologic exam and brain computed tomography. Eletroencephalography (EEG), done
one day later, shows intermitent sharp waves in
the anterior temporal region at left. The amnesia
resolved over about 14h. Magnetic Ressonance
(MRI) of the brain 2 days later was normal. Her
husband reports 2 episodes of break contact with
oral automatisms that happened briefly 8 months
before. She was discharged 4 days later with oxcarbazepine 300mg twice a day. Discussion: TGA
is defined by a global amnesic episode that lasts
up to 24h, without focal neurological signs, loss
of consciousness or the personal identity and no
recent histoty of head trauma. Mild vegetative
symptoms might be present. It can be preceded
by intense emotional stress. On diffusion and T2
weighted MRI can occur highly focal lesions in
the CA1 field of the hippocampus some days after the episode. This alterations disappear within
two weeks. The recurrence is rare. Transient epileptic amnesia (TEA) is defined by recurrent episodes of transient amnesia lasting until one hour,
epileptiform abnormalities on the EEG, clinical
XXVI Congresso Brasileiro de Neurologia
signs of epilepsy (orofacial automatisms and
olfactory hallucinations) and response to antiepileptic treatment. The attacks often occur on
awakening. Our patient, although the history of
two episodes of break contact and automatisms
suggestive of temporal lobe epilepsy corroborated by the EEG, presented a classic acute-onset
transient amnesic syndrome that fills the diagnosis criteria for TGA. Thus we believe that it‘s a
case of TGA on an epileptic patient.
P-311
USO DO MONTREAL COGNITIVE ASSESSMENT
(MOCA) NA AVALIAÇÃO DA FUNÇÃO COGNITIVA DE
PACIENTES COM EPILEPSIA DO LOBO TEMPORAL
Fernandes PS, Meiga C, Pinto LM, Nickel R, Silvado C
• UFPR
Introduction: Epilepsy interferes in the social,
psychological, conjugal, work and cognitive
conditions.The cognition of patients with epilepsy may be compromised by the antiepileptic
medications, by the seizures, or by the structural alterations which cause the epilepsy, with
relevant repercussions in routine activities. The
MoCA is widely used for evaluating the cognitive
functions of patients with situations of dementia. Objectives: To describe the cognitive profile
of the subject with temporal lobe epilepsy using the Montreal Cognitive Assessment (MoCA).
Methods: This is a descriptive transversal study
with patients with temporal lobe epilepsy, literate, of both sexes, without severe psychopathology or any other associated health condition considered able to compromise the performance of
those patients who are receiving treatment in the
Difficult-to-Control Epilepsy Outpatient Center
of the Federal University of Paraná Teaching Hospital. Results: The results of the first 43 patients
included until now indicate that the participants’
mean age was 39 years old. Of these, 25.6% had
concluded senior high school; 2.3% had completed university level education and 7% had
reached postgraduate level. 46.5% of the subjects
have right-sided temporal lobe epilepsy (R-TLE);
46.5% of the subjects have left-sided temporal
lobe epilepsy (L-TLE); and 7% have bitemporal
epilepsy. Those who have left-sided temporal
lobe epilepsy presented lower results (12% lower)
than those with the right-sided temporal lobe epilepsy. It was the case that the cognitive functions
of memory, naming abilityand language were
the most compromised in subjects with L-TLE
and attentionwas compromised most in subjects
with R-TLE. The subjects with bitemporal epilepsy presented moderate compromise (10‐19);
70% of the subjects with L-TLE had moderate
compromise (10‐19), 25% had mildcompromise
(20‐30) and 5% had severe compromise (0‐9); 65%
of the subjects with R-TLE had mild compromise
(20‐30), 30% had moderate compromise (10‐19)
and 5% had severe compromise (0‐9). Only 6.98%
of the subjects evaluated did not evidence cognitive compromise. Conclusions: The Montreal
Cognitive Assessment (MoCA) was shown to be
a sensitive, standardized and easy-to-apply instrument for evaluating cognitive deficits and indicating which cognitive functions are compromised in patients with temporal lobe epilepsy.
P-312
VALPROIC ACID ASSOCIATED WITH PERIPHERAL
EDEMA - REPORT OF 4 CASES
Alvim MKM, Morita ME, Coan AC, Cendes F,
França WCSC, Guerreiro CAM
• UNICAMP
Introduction: Peripheral edema (PE) secondary
to the use of valproic acid (VPA) is described in
the literature, but is rare and frequently underdiagnosed. Objective We describe four patients
with PE using VPA and conduct a brief review.
Case 1 A 65 year old woman, with temporal lobe
epilepsy presented lower limbs edema after
using VPA (1250 mg/day) for 5 months. Lab exams revealed hyponatremia (treated), and TSH
11mUI / L and T4L 0.9 ng / dl. She developed
cellulite in right lower limb. PE improved after
reduction of VPA. Case 2 A woman with partial
seizures and myoclonus was using VPA for 8
years. She presented with generalized edema
when reached the dosage of 1500 mg/day. Exams
revealed subclinical hypothyroidism. Remission
of edema occurred after the VPA dosage was reduced to 1000 mg/day. Case 3 A man with Unverrich-Lundborg Disease (ULD), after 5 years in
use of VPA (2000 mg/day) presented lower limbs
edema. TSH 10.5 mIU/L and FT4 1.02 ng/ dl.
Treatment for hypothyroidism and reduction of
VPA dosages lead to edema improvement. Case
4 A 37 years old sister of case 3 with ULD, with
VPA 2000 mg/day presented edema in the lower
limbs and developed ulcer and osteomyelitis. It
was not possible to reduce VPA due to worsening of seizures. Her hypothyroidism was treated
without edema improvement. Discussion Patients developed PE secondary to the use of VPA.
VPA could cause PE due to the possible influence
in the modulation of capillary resistance via peripheral GABA receptors. There are reports suggesting that long-term use and high doses of VPA
can induce a higher cytochrome p450 oxidation,
increasing the chances of idiosyncratic reaction.
Our patients presented abnormal thyroid function; three of them with subclinical hypothyroidism. In the first three cases PE improved with reduction of VPA, and in case 3 the reduction was
concomitant to the use of thyroid hormones. In
case 4 it was not possible to reduce VPA and there
was no improvement of the PE, even with hypothyroidism treatment. Therefore, in some cases,
even in the presence of thyroid abnormalities
(which may also cause PE), reduction of VPA dosage might be enough to improve PE. GABA acts
directly on the pituitary and on hypothalamic
factors related to TSH, thus VPA could also be responsible for the thyroid abnormality. Concluding, VPA might be related to PE. The reduction of
VPA dosage, when possible, might reverse the PE
and avoid secondary cutaneous lesions.
P-313
VIGABATRINE FOR TREATMENT OF PERIODIC
SPASMS IN CHILDHOOD
Rodrigues LO, Seraphim EA, Corso JT, Naves PVF,
Guaranha MSB, Yacubian EMT
• UNIFESP
Introduction: Epileptic spasms (ES) are considered non classifiable seizures. In 1987, Gobbi et
al. first reported peculiar seizures characterized
by series of periodic bilateral spasms in patients
with focal or multifocal epilepsy, and categorized
these seizures as periodic spasms (PS). The ictal
EEG showed an atypical pattern without hypsarrhythmia. They suggested that PS were focal
seizures with secondary generalization, which
could be triggered by abnormal interactions between the cortex and brainstem. The possible
predictive value of the phenomenon regarding
the presence of a malformative etiology and a
related unfavorable outcome was postulated
early in literature. Objective: Report Vigabatrine (VGB) treatment for PS and focal epilepsy
in childhood. Case report: An eight-month-old
boy started presenting cluster of symmetric ES.
He was treated effectively with Oxcarbazepine
(OXC) for three years. With the withdrawal of
OXC, there was return of ES and cognitive decline. Brain MRI showed left temporal pole hyperintense signal with blurring of the grey/white
matter junction and left temporal lobe atrophy.
Ictal PET demonstrated left frontal hypermetabolism. The reIntroduction: of antiepileptic
drugs (PB, VPA, CBZ, LTG, OXC) was ineffective.
A video-electroencephalography (V-EEG) study
revealed centrotemporal sharp waves in the left
hemisphere. The ictal recording showed periodic complexes of high voltage slow wave with
superimposed fast activity that are described in
patients with tonic spasms (that occurred as PS
in this case). Deltoid EMG recording revealed a
typical diamond-shape aspect ranging from 0.5‐2
seconds in duration. VGB was introduced (dose
50 mg/kg/day, increased to 100 mg/kg/day) with
remission of seizures and cognitive gains. The
V-EEG after VGB maintained the interictal activity and there were no records of PS. Discussion:
VGB is a first line GABAergic antiepileptic drug
for treating West syndrome, especially when it is
related to tuberous sclerosis. It may also be effective in patients with focal epilepsy associated
with PS as described in this case.
P-314
WORK AND EPILEPSY - THE INFLUENCE OF THE
EPILEPTIC SEIZURES ON THE WORK OF PERSONS
WITH EPILEPSY
Pinto LM1, Fernandes P2, Esteves L1, Nickel R1,
Silvado C2
• 1UFPR; 2HC-UFPR
Introduction: Persons with epilepsy are exposed
to different restrictions in the activities of daily living, such as, for example, in work. Hence,
identifying the restrictions which they have at
work is relevant for establishing strategies allowing the patients to achieve their potential.
Objective: To describe the characteristics of people with epilepsy who undertake work activity,
in accordance with the treatment undertaken,
as well as the control or not of the epileptic seizures. Methods: A descriptive, transversal study
undertaken in March 2012 – August 2013, in
the Epilepsy Service of the Federal University of
Paraná Teaching Hospital. A total of 120 patients
was selected who were receiving treatment for
epilepsy who had already undertaken some form
of regular work with records kept in their work
documentation throughout their lives, and who
had no other associated health condition which
could compromise their health, divided into four
groups of 30 patients each, depending on the
mode of treatment (surgical v. medication) and
epileptic seizures (controlled v. uncontrolled).
One control group of 30 persons without epilepsy was used for comparison with this condition.
Results: Patients whose seizures were controlled
had the highest rates of maintaining regular employment, particularly those being treated with
medication and whose seizures were controlled
(MED-CT), with 77% of the patients in regular
employment. In the group receiving surgical
treatment, with seizures controlled (SUR-CT),
111
Posters
73% were in regular employment, a rate above
that observed in the control group (63%). The
highest rates of unemployment are found in patients whose seizures are not controlled, being
greater (37%) in those receiving surgical treatment and whose seizures are not controlled
(SUR-NC). The group receiving treatment with
medications without control of the seizures
(MED-NC) had an unemployment rate of 47%.
Considering the total number of patients with
epilepsy evaluated, 60% undertook regular work
and 40% did not. In the comparison between the
groups, statistically significant differences were
only observed in the comparison of the MED CT
v SUR NC Groups (0.0022) and SUR NC v SUR CT
(0.0089). Conclusion: The control of the seizures
was shown to be determinant for the condition
of employability of the patients with epilepsy,
but other factors which may promote or compromise work for the person with epilepsy must
be identified.
Amyotrophic Lateral Sclerosis
peripheral neuropathies, getting negative result.
We made the diagnosis of ALS as demyelinating
neuropathy associated with acquired asymmetric due to HIV infection. Discussion: Motor Neuron disorders and peripheral neuropathy may
occur in association with HIV infection, however,
a causal relationship remains uncertain. The initial clinical presentation of our case was marked
by cramps and fasciculations with posterior
distal paresis and atrophy in the left arm. EMG
tracings, characteristic of suffering from anterior horn of the spinal cord and peripheral nerves,
were found. At the same time the patient discovered to be HIV positive, with high viral load. It
has undergone antiretroviral therapy, with load
control, however the above did not show clinical
remission.
P-317
BRANCHED-CHAIN AMINO ACID USE IN
AMYOTROPHIC LATERAL SCLEROSIS: PROTEIN
SUPPLEMENT OR NEUROTOXIC SUBSTANCE?
Milagres EAN1, Loureiro MPDL1, Silva AC2, Matos AC2,
Ramalho A2
• 1INDC; 2INJC
P-315
ALS-LIKE AND DEMYELINATING NEUROPATHY
ASSOCIATION IN HIV INFECTION: CASE REPORT
Orsini M1, Freitas MR2, Nogueira CB2, Dias JC2,
Barbosa MD2, Souza JA2, Leite MAA2, Nascimento OJ2,
Oliveira AB3
• 1UNISUAM; 2UFF; 3UNIFESP
Introduction: In recent years, several reports
of Amyotrophic Lateral Sclerosis (ALS-Motor
Neuron Diseases) and peripheral neuropathies
are associated with human immunodeficiency
vírus (HIV) infection. Despite the widespread
success of combination antiretroviral therapy in
minimizing morbidity and reducing mortality in
HIV infection, but HIV-related peripheral and
central nervous system disorders remains prevalent. Objective: To report a case of an association
between Motor Neuron Disease and Acquired
Demyelinating neuropathy in HIV infection.
Case report: Male, 56 years old, refers during
the last four years muscle cramps and myofasciculations, initially located in the distal portion
of the left arm, with subsequent progression to
the entire arm followed by atrophy and paresis
of the hand. At the same time, he was diagnosed
as HIV positive, with a high viral load. Antiretroviral treatment was initiated. Despite the reduction in viral load reported clinical worsening
evolving with speech difficulties. Electromyography(EMG) showed no sign of involvement single cell acute anterior horn of the cervical and
lumbosacral cords at various levels, but diffuse,
mixed pattern of sensorimotor involvement of
peripheral nerves (axonal and demyelinative) in
all four limbs as well. In the inflammatory process was to be found upon nerve biopsy. Endo
and epineurial vessels showed thickening of the
walls, with a pattern suggesting remyelination
/ demyelination. Masson‘s trichrome staining
showed loss of nerve cells. The biopsy showed
also a loss of nerve cells with thinning of the
remaining fibers. There was no axonal degeneration or regeneration. Research was conducted
for other causes of motor neuron diseases and
112
Epidemiological studies have shown increased
risk of amyotrophic lateral sclerosis (ALS) in
American and Italian football players when compared with the rest of the population. Several
hypotheses have been raised to explain this fact,
among them, the excessive and chronic intake of
branched-chain amino acids (BCAAs) which has
contributed to the worsening of the disease progression. This probably occurred because aminoacids are glutamate precursors and their overconsumption stimulates the glutamate. Aim: To
assess existing scientific evidence about the use
of BCAAs by patients with ALS . Methodology:
The study was carried out based on a search
for scientific literature related to the intake of
BCAAs. Literature survey was performed by accessing the electronic databases of the Scientific Electronic Library Online (SciELO), Virtual
Health Library (BIREME) and Cochrane Library,
CAPES, databases; publications within the 1988
to 2013 period were taken into consideration. Articles in Portuguese and English were included. It
was performed by using some descriptors, such
as the terms branched-chain amino acids, glutamate, amyotrophic lateral sclerosis, neurotoxicity, and exitotoxicity. Results: Six articles related
to the intake of BCAA amino acids and ALS were
selected. Among them it was possible to observe
that 50% showed a close relationship between
the excessive and chronic BCAA intake, with the
worsening of the disease. It is important to know
that such studies were developed by using above
the recommended BCAAs. Other 50% evidenced
the beneficial effects of the BCAAs, mainly regarding the weight gain. Conclusion: The BCAAs
supplementation should not be indicated due
to a possible harmful effect. Patients may be encouraged to consume protein food, nutrient-rich
in BCAAs, as long as they respect the suggested
recommendation. However, more studies should
be conducted as there are scarce studies in this
area. Keywords: Branched-chain amino acids,
glutamate, amyotrophic lateral sclerosis, neurotoxicity, exitotoxicity.
P-318
CLINICAL PROFILE OF PATIENTS WITH
AMYOTROPHIC LATERAL SCLEROSIS FOLLOWED
AT REFERRAL CENTERS IN BELO HORIZONTE, MG:
PRELIMINARY Results
Prado LGR1, Santos IBC1, Resende MVL2, Gomez RS1,
Souza LC1, Teixeira AL1
• 1HOSPITAL DAS CLÍNICAS; 2HOSPITAL JULIA
KUBITSCHEK
Introduction: Despite its severity, there are few
clinical-epidemiological studies of amyotrophic
lateral sclerosis (ALS) in Brazil. Objective: To
describe the clinical profile of patients with ALS
followed at two referral centers. Methods: This
is a descriptive and transversal study of patients
diagnosed with probable or definite ALS according to Awaji’s criteria followed at Hospital das
Clínicas - UFMG and at Hospital Julia Kubitschek
- FHEMIG. Results: To date, 44 patients were
studied, 39 with sporadic (88.6%) and 5 with the
familial form (11.4%). Twenty-nine patients were
male. The average age of onset of the symptoms
was 53.9 years and the mean age at diagnosis was
55 years. The first symptom was paresis of the
upper limbs in 18 patients, paresis of the lower
limbs in 14, impaired speech in 5, dyspnea in 2.
Five reported other symptoms like cramps and
weight loss. As potential factors related to ALS,
24 reported amateur sports practice, 22 exposure
to toxic products, 23 tobacco use. The average
duration of the disease until the date of evaluation was 4.5 years. Bag-valve-mask device and
BIPAP have been used by 20 and 13 patients, respectively. Two patients have tracheostomy and
7 have gastrostomy. Conclusion: The profile of
this sample is similar to other national series,
and this knowledge has allowed the organization of services and the definition of health care
priorities.
P-319
CONCURRENT MULTIPLE SCLEROSIS AND
AMYOTROPHIC LATERAL SCLEROSIS
Lourenço PM, Rocha MMV, Papassidero PC,
Marques VD, Marques WJ, Barreira AA
• USP
Introduction: The concurrence of multiple sclerosis and amyotrophic lateral sclerosis (MS-ALS)
is extremely uncommon. The prevalence of this
association would be 3 cases for 10 billion people. Objective: To present a multiple sclerosis
(MS) patient that had concomitant amyotrophic
lateral sclerosis (ALS). Case report: A 61 years
age female patient had a sudden muscle weakness in right limbs and blurred vision 4 years ago.
She was initially treated as having had a stroke
without any improvement. Eleven months later,
she presented a subacute paraplegia and anesthesia below T3. MS diagnosis was made based
in MacDonald’s criteria and she was treated with
intravenous corticosteroids, with noteworthy
improvement. The patient started using interferon beta 1b but developed progressive asymmetrical tetraparesis and after a few months,
she was confined to bed with mild dyspnea and
difficulty to swallow. Neurological examination
showed a mini-mental state examination 20/30,
left homonymous hemianopsia, dysphagia, dysphonia, atrophy and tongue fasciculations. She
had vibration sense loss on both lower limbs up
to the knees and spastic quadriparesis, hyperreflexia and global atrophy and fasciculation in
4 members. A magnetic resonance image study
of brain and spine fulfilled the Barkof criteria.
Focal spine lesions were seen from T1 to T3.
XXVI Congresso Brasileiro de Neurologia
After an electrophysiological study the diagnosis of clinically definite ALS was made based on
the Awaji and the Revised El Escorial criteria.
No improvement of the paresis were obtained
after methyl-prednisolone pulse followed 7
days after by plasmapheresis. A fast progressive
worsening of weakness, dysphagia and dyspnea
is occurring and the patient is being mechanically ventilated and fed through a gastrostomy.
Comments and Conclusion: The coexistence of
MS and ALS is extremely rare and it is unclear
whether there is a causal link between the two
conditions or if it would occurs at random. Recent studies showed hexanucleotide repeat expansion in the C9ORF72 gene in 80% of patients
presenting with MS and ALS. We don’t have yet
this data from our patient. Other hypotheses
looking for a link between ALS and MS have
also been suggested, and include contact with
Epstein-BarrVirus, vitamin D deficiency and involvement of pro-inflammatory mechanisms in
both diseases. The follow-up time of our patient
and her accumulated deficits since the ALS beginning is in the range of the majority our ALS
patients progression of deficits: 2 to 4 years.
P-320
EPIDEMIOLOGICAL AND CLINICAL FACTORS
IMPACT ON THE BENEFIT OF RILUZOLE ON
SURVIVAL IN ALS
severals specialists on mental health. Methods:
Have been researched on Medline/Pubmed with
the keywords “ALS”, “depression” and “anxiety”. It
was used limits of meta-analysis, trials, Case report, reviews, english language and published on
the last 5 years, and have been found 40 articles.
Results: The most patients with ALS reported on
the research archived to relieve depression and
anxiety, as well as others mental health disorders,
from interventions performed to improve their
quality of life. Physiotherapy and occupational
therapy helped with mobility and activities of
daily living. Interventions from phonoaudiology
optimize the nutrition and communication. The
use of botulinum toxin type-A improve symptom
of salivary disorder. In addition, many symptoms like pseudo-bulbar affection, constipation,
spasms and colic can be effectively treated with
drugs. Conclusion: Although there is no treatment that can stop or revert this progressive condition, the control of some symptoms can contribute to the regression of depression and anxiety. This may happened because of some reports
that collaborate with social stigma of the illness.
After relived the symptoms, there are a higher social integration and easier rehabilitation.
P-322
Favero FM1, Voos MC2, Caromano FA2, Fontes SV1,
Oliveira ASB1
MULTIDISCIPLINARY INTERVENTION IN
AMYOTROPHIC LATERAL SCLEROSIS (ALS) WITH
PREDOMINANCE OF BULBAR INVOLVEMENT: A
CASE STUDY
• 1UNIFESP; 2FMUSP
Baltezan RL, Ortiz LDN
Objective: To investigate the impact of epidemiological and clinical factors on the benefit of riluzole in Amyotrophic Lateral Sclerosis (ALS) patients. Method: Patients with ALS (n=578) from
the Division of Clinical Investigation on Neuromuscular Diseases (1999‐2011) were followed.
Descriptive statistics and Kaplan-Meier curves
showed the survival. Results: The median survival was 19 months and patients were divided into
shorter and longer this time (S19MS and L19MS)
in Kaplan Meier curves. In S19MS, riluzole increased the survival of patients with lower limbs
onset and diagnosed after the first appointment.
In L19MS, patients with bulbar onset and diagnosed on the first or after the first appointment
showed longer survival. Males lived longer than
females. Conclusion: Clinical and epidemiological differences between S19MS and L19MS were
found.
P-321
INTERVENTIONS TO IMPROVE DEPRESSION AND
ANXIETY SYMPTOMS IN AMYOTROPHIC LATERAL
SCLEROSIS
Salgado ABMA, Maia BBV, Endres DC, Assis CHPC,
Morais ALG, Araujo MTF
• UNIRG
Introduction: Amyotrophic Lateral Sclerosis
(ALS) it‘s a neurodegenerative disorder from a
unknown etiology, that can be chronic and lethal.
Classified from CID 10 as illness of motor neuron,
it is characterized by progressive atrophy from all
muscles of the body. A psychological care of theses patients leeds to appears depression and anxiety symptoms, probably caused by the lost of the
functional independence. Objectives: Evaluate
if depression and anxiety symptom, developed
during the ALS, can relieve with interventions of
• ARELA RS
Introduction: The Amyotrophic Lateral Sclerosis
(ALS) is a progressive degenerative disease that
leads to diffuse muscle weakness. In 30% of ALS
patients, the first symptoms are bulbar, including dysphagia and they cause a respiratory impairment. Dysphagia, treated by a speech therapist, is characterized by impaired oral phase of
swallowing with significant impact on the pharyngeal phase. Its changes intensify the risk of
respiratory complications. Physiotherapy works
seeking and maintaining the quality of pulmonary function through the realization of some
respiratory therapeutic procedures. The work of
these professionals is important in ALS, because
there is a high risk of bronchial aspiration during
swallowing due to the loss of muscle strength,
especially exhalation, that normally results in an
ineffectiveness of the patient coughs. Objective:
Report the experience of integrated work among
professionals of physical and speech therapy in
the treatment of ALS patients with bulbar symptoms, to prevent the risk of bronchial aspiration.
Case report: A man, 62, with the first symptoms
of ALS in 2011 (dysarthria), with early diagnosis
and care of physical and speech therapy in 2012.
Physiotherapy assessment of pulmonary function (lung auscultation, nocturnal oximetry, oxygen saturation was performed during sessions of
chest radiography) and underwent 2 sessions per
week at the first year and 1 day per week since
the second year. The speech evaluation consisted of: assessment of oral motor and swallowing,
with the indication of one speech therapy once
a week. Discussion: After the speech evaluation,
by the team, it was nominated a gastrostomy,
however the patient was orally with supervision
to functional stimulation. Although there was
disease progression and worsening of swallowing, the patient remained without radiological
pulmonary changes, decrease of oxygen saturation and dyspnea. The only visible injury was
the increase of drooling, and because of that
the use of medication, therapy and oropharyngeal aspiration were necessary. In patients with
bulbar ALS symptoms bronchial aspiration can
occur. The physical therapy and speech therapy
must act prophylactically since the beginning,
adapting itself according to the evolution of the
disease, providing, in this way, bigger safety and
quality of life for the patient.
P-323
NUTRITIONAL CARE AND ITS RELATIONSHIP
BETWEEN ANTHROPOMETRIC VARIABLES LIKE
WEIGHT AND WEIGHT LOSS SERIOUSNESS
IN PATIENTS WITH AMYOTROPHIC LATERAL
SCLEROSIS
Milagres EAN1, Silva AC2, Pernes ML1, Matos AC2,
Machado SN2, Ramalho A3
• 1INDC; 2UFRJ; 3INJC
Introduction: Amyotrophic Lateral Sclerosis
(ALS) is a progressive neurodegenerative disease
characterized by a progressive loss of motor neurons; malnutrition is frequent in these patients
and affected by it and associated with the worsening of clinical symptoms and interfering negatively on prognosis. Weight gain is associated
with a better quality of life and survival increase
in ALS patients. Such aspects make the evaluation and weight maintenance important allies for
favorable clinical conditions in these patients.
Aim: Evaluate the effectiveness of nutritional
care in weight control. Methodology: A study
carried out with ALS patients diagnosed according to the El Escorial revised criteria and cared
for at the nutritional ambulatory of the Instituto
de Neuorologia Deolindo Couto - Universidade
Federal do Rio de Janeiro (Institute of Neurology Deolindo Couto, Federal University of Rio de
Janeiro). For the nutritional assessment, weight,
height and body mass index (BMI) calculation
were used, with the latter being classified according to WHO (1995, 1998) for adults and Lipschitz
(1994) for elderly (≥60 years). Anthropometric
variables were obtained in the first nutritional assessment (T0) and six months after (T1). Weight
loss seriousness assessment was performed by
using the formula (usual weight-current weight)
x100/usual weight (Blackburn, 1977). Weight loss
exceeding 10% over six months was considered
significant. Result: 30 patients were assessed:
53,3% (n=16) male and 46,7% (n=14) female.
The average age was of 55,6+10,8 and the average of number of nutritional consultations was
4,77+1,8. The average in weight T0 was 60,6
kg+14,2 and BMI was 22,5+4,5Kg/m2, while T1
was 59,0 kg +14,2 and BMI was 21,8+4,3Kg/m2.
At the end of the study period, it was verified that
26,7% (n=8) had weight gain and 73,3% (n=22)
weight loss. Among those who showed weight
loss, 81,8% (n=18) were not considered serious,
whereas 18,2% (n=4) were classified as having severe weight loss. Significant difference between
the mean weight and BMI at T1 and severity of
weight loss was found (p=0.005 and p=0.03).
A significant positive correlation between the
number of nutritional consultations and BMI at
T1 (r=0,3, p=0,089) was observed. Conclusion:
Nutritional care should begin soon after the
disease diagnosis, being an integral part of the
ongoing care of the patient so as to combat the
deleterious effects of malnutrition.
113
Posters
P-324
P-325
P-326
SUFFERERS FROM AMYOTROPHIC LATERAL
TEXTURE ANALYSIS OF MRI REVEALS BASAL
THE IMPORTANCE OF THE MONITORING OF
SCLEROSIS AND SWALLOWING
GANGLIA DAMAGE IN PATIENTS WITH
THE FORCED VITAL CAPACITY AND FATIGUE
Paim ÉD1, Jarces M1, Zart P1, Varela DL2
AMYOTROPHIC LATERAL SCLEROSIS (ALS)
IN PATIENTS WITH AMYOTROPHIC LATERAL
• UNIVERSIDADE DE PASSO FUNDO; SERVIÇO DE
Albuquerque M, Anjos L, Andrade HMT, Oliveira MS,
SCLEROSIS: CASE REPORT
Castellano G, França Junior MC, Nucci A
Labronici RHDD1, Labronici E2, Holsapfel S1,
1
2
NEUROLOGIA E NEUROCIRURGIA DE PASSO FUNDO
Introduction: Amyotrophic Lateral Sclerosis
(ALS) is a progressive degenerative neuromuscular disease with significant reduction of the
• UNICAMP
Cunha MCB1, Chieia MA1, Oliveira ASB1
• 1UNIFESP; 2ABRELA; 3FMABC; 4NATU SAUDE
Introduction: Motor neuron damage is the
hallmark of Amyotrophic Lateral Sclerosis
Introduction: Amyotrophic Lateral Sclerosis
(ALS), but there is growing evidence that oth-
(ALS) is a disease that affects motor neurons,
er cerebral regions are involved in the disease
leading to muscle weakness, fatigue and de-
as well. Texture analysis of MRI is a technique
creased forced vital capacity (FVC), interfering
based on the evaluation of gray levels between
with the ability of patients to continue with
complications. Objective: The objective of the
pixels in a given region of interest. It has been
their motor activities and daily life activities.
research was to analyze the characteristics of
found to be useful in several neurodegenera-
the swallowing of ALS patients by videofluo-
tive disorders, through the identification of
roscopy. Method: This study was approved by
cerebral abnormalities even before true atro-
the Ethics Committee in Research of the Passo
phy appears. Objective: To study basal gan-
Fundo University, in opinion: 447.792. Twenty
glia damage in patients with ALS by texture
patients aged between 43 and 75 years were se-
analysis. Methods: We acquired volumetric
lected, diagnosed with ALS without other un-
T1-weighted images in axial, coronal and sag-
derlying disease, which did not use alternative
ittal planes with 1mm cuts in a 3T MRI (Philips
routes to tracheostomy and feeding. The clini-
Achieva) of 32 ALS patients (19 men, mean
cal history was taken and after an examination
age: 56.5) and 32 healthy controls (20 men,
functions of the limbs, trunk, pharyngeal and
respiratory muscles. Among the symptoms,
dysphagia is one of the most important problems faced in ALS and is considered one of
the leading causes of death due to respiratory
of videofluoroscopy. For this, foods with contrast (Bariogel®) in liquid, pasty and solid consistencies were offered to patients, three offers
of 5 ml and a portion of 5g of bread. Results:
In the data analysis it was found that for liquid consistency, the most significant change
was the presence of residues in the vallecula
in eleven patients. For pasty, twelve patients
mean age: 53.5). The segmentation of structures of interest (cerebral peduncle, putamen,
caudate and thalamus) and texture analysis
were made blindly using the Mazda software.
We calculated 11 texture-based parameters
(Angular Second Moment (ASM), Contrast
(CT), Correlation (CO), Sum of Squares (SS),
Objective: To evaluate fatigue and accompany
the CVF on a patient, who performs the aquatic
physiotherapy. Case report: N.M., with 60 years
of age, diagnosis ALS (UNIFESP-ABRELA), for
18 years, presenting clinical picture of spastic
paralysis, with muscular strength in MMSS
(grade II/prox and distal/I) and MMLL (grade
III/prox and distal/I), no complaint of fatigue,
well nourished, with CVF: 30.7%, 0.78 l/s (predicted 2.54 l/s), with use of noninvasive ventilation for 15 p/day. Is stable with this painting
since 2008by performing motor physiotherapy
(1 x per week, plus daily stretches) and acupuncture (once a week). Their chief complaint
was motor activities and improve their quality
of life. Aquatic physiotherapy service was held,
during 2 years ( June 2012 to June 2104), once
a week, for 45 minutes in a pool heated to 33°
showed reduced laryngeal elevation, other
Inverse Difference Moment (IDM), Sum Aver-
eleven people showed residues in the region
ages (SA), Sum Variances (SV ), Sum Entropy
of the tongue base and twelve patients Esoph-
(SE), Entropy (E), Difference Variance (DV )
agus, only one patient aspirated. Already solid
and Difference Entropy (DE)) and five distanc-
in consistency, in oral transit time presented it-
es (d) for each structure. Statistical analysis
self increased to 7, the tongue movements were
was performed using the Mann-Whitney test,
reduced to 10 and the presence of residue in
adopting uncorrected p values <0.01. Results:
the oral cavity in 10 pacients. A total of twenty
one year increased from 30.7% to 37%, 0.93 l/s
Significant changes were found in the right
patients eleven had mild dysphagia, five had
(2,51l/s predicted). As the patient reported fa-
caudate and thalamus bilaterally. The dis-
moderate / mild and one had moderate dys-
tigue, during and after the sessions, the thera-
tances and texture parameters more mean-
pies have evolved with exercises in seated and
ingful were d=1 (0.4368 x 0.3972,p=0.0350),d=2
standing with 50% to 70% of his body ‘ in im-
(0.1527 x 0.0895,p=0.0075) and d=3 (0.0434 x
mersion, with the interference of hydrostatic
‐0.0152,p=0.0032) for CO parameter in the right
pressure, and FVC was 37% to 39.7%, 0, 99l/s
caudate and d=1 (0.5877 x 0.5405,p=0.0111) for
(2,51l/s predicted). In the literature the hydro-
CO, d=4 (0.1886 x 0.2072,p=0.0107) for IDM, d=5
static pressure for patients with muscle weak-
(0.1659 x 0.1908,p=0.0064) for IDM in the left
ness, with degenerative and FVC less than 50%,
thalamus and d=4 (‐0.0310 x 0.0008,p=0.0168)
it would be contraindicated, the pros couldn‘t
solid. It was also observed that those who had
for CO, d=5 (‐0.1411 x ‐0.0984,p=0.0111) for CO,
use aquatic physical therapy for these patients.
speech therapy, showed a slower progression
d=5 (0.1870x0.2081,p=0.0150) for IDM in the
We conclude that the monitoring of a multi-
in relation to the degree of dysphagia, allowing
right thalamus. Conclusion: Texture analysis
professional team and evaluations of FVC, can
point that speech therapy is effective and may
identified basal ganglia damage in ALS. Fur-
give patients with her, greater safety in aquatic
prolong the oral feeding, thus improving the
ther studies are needed to investigate the clini-
physiotherapy. Key-words: ALS, forced vital ca-
quality of life of patients ALS.
cal correlates of such damage.
pacity and aquatic physiotherapy.
phagia. Conclusion: It was concluded that all
participating had dysphagia, in different degrees, and level 5, discrete, for most subjects.
The pharyngeal phase was the most committed to the pasty and liquid, with a significant
presence of residue in the vallecula and Esophagus, followed by oral, with a time of increased
oral transit and tongue movements reduced to
114
C, using the BIPAP (Time: 10‘ , before and after
the sessions, outside of the pool). Discussion:
The therapies were carried out initially with
stretches and exercises active-assisted, supine,
without the interference of hydrostatic pressure, with accompaniment of FVC, where after
XXVI Congresso Brasileiro de Neurologia
P-330
History of Neurology
ACADEMIC LEAGUES AND NEUROLOGY: A NEW
SCENERY IN MEDICAL EDUCATION
Ferreira ACAF, Maia LFH, Santos CF, Faria ACO,
Dantas Neto L
• UNIRIO
P-328
A HISTORICAL REVIEW OF WILSON‘S DISEASE
Germiniani FMB, Scheffer BE, Teive HAG, Arruda WO
• UFPR
Introduction: Wilson’s Disease (WD) is one of the
most important neurological disorders due to
metal deposition in the central nervous system,
with a rich history from discovery until successful treatment. Objective: To make a brief analysis of the historical hallmarks of WD worldwide,
focusing on the Brazilian contributions. Results:
First described as “pseudosclerosis” by Westphal
in 1883, when he reported 2 patients with tremors, and by Gowers in 1888, who coined the term
tetanoid chorea, WD would eventually become
notorious following the description by Samuel
Alexander Kinnier Wilson. Wilson’s monograph
on “progressive lenticular degeneration” became
the longest single article published on the medical journal Brain, on March 1912, with 214 pages.
Shorter versions were published in The Lancet
and Revue Neurologique. His series was composed of 4 original patients, 2 additional cases
from the registry of London’s national hospital
and 6 other previously published cases. The clinical and anatomical descriptions would remain
essentially unaltered even 102 years after his
report. Wilson performed all the anatomopathological studies himself and drew all the figures
of his monograph. Prior to Wilson’s monograph,
Kayser and Fleischer independently reported the
finding of a pigmented ring around the cornea in
patients with suspected multiple sclerosis, which
would later become one of the hallmarks in the
clinical diagnosis of WD. As to the evolution of
therapy, the use of copper chelating agents remained unsuccessful until the Introduction:
by Walshe in 1956 of Penicilamine, providing a
means of long-term treatment. In Brazil the first
reported case of WD was made by Austregésilo
Filho in 1944, who highlighted tortion spasms
as the main neurological manifestation of his
patient, suggesting it was a dystonic form of WD.
Cerebral abnormalities and hepatic cirrhosis on
autopsy would confirm the clinical diagnosis. In
the sixties Horacio Martins Canelas contributed
with laboratory techniques and specific tools for
measuring the metabolism and values of copper
in different organic fluids and participated in
clinical studies that helped understand the progression of the disease. We also highlight the recent contributions of Egberto Reis Barbosa with
his analysis of a large cohort of Brazilian patients
in 2006. Conclusion: WD is one of the main neurological disorders due to abnormal metal deposition. We attempted to highlight some of its historical hallmarks.
Introduction: The progressive amount of information has been overloading medical course
curriculum, therefore, some contents, as neurology, have been minimally approached. Academic
leagues have arisen to support students to improve their knowledge about an area of interest in
Medicine, stimulating their critical and reflexive
thinking, and fomenting the study of additional
topics. Thus, academic leagues may contribute
to develop general practitioners with ethics and
social responsibility sense. Objective: Ilustrate
the impact of academic leagues on medical education and also their contribution to Brazilian
neurology. Method: Seek, in websites for search
(Google ®) and social networking (Facebook®),
all medical neurology leagues that have ever
been registered in Brazil on the internet. The Key
words used were “liga” and “neurologia” or “neurociência”. Results: The survey in research site
found 77 medical leagues, 36 from Southeast and
30 from public universities. At least 6 of the total
involve more than 1 institution (4 cases in Northeast). 15 of 77 offer neurology contents from their
own authorship, besides scientific overviews.
23 of 77 also display a page on networking site.
Some leagues are showed only on networking
site: 35, being 19 from southeast and 10 from
public universities. The facility to post on this
kind of site may help justifying why 25 of all these
leagues have updates from the actual year, which
happens only on 12 conventional websites. Except one league, all of them were created after
2000. The neurology leagues demonstrate the
effort to succed at least in the domain of education, promoting symposiums, inviting professors
and specialists to give classes. The extension also
seems to be practised, specially activities directed to stroke prevention campaings. The internet,
for its turn, has propitiated regional and national
meetings between leagues, as documented in the
last national neurology congress. Conclusion:
Academic leagues have been arisen to supplement medical curriculum. They have a unique
role in which students may improve theoretical
knowledge in association to medical practice,
which includes extracurricular activities in academic education, scientific research and community assistance projects. However, the leagues
are relatively new in the academic scene and the
internet has proved to be very useful in registering their activities, showing their production and
making the connection between students from
many places.
P-333
DYSTONIA IN THE LIGHTS FROM THE NORTH
EXHIBITION OF GERMAN RENAISSANCE
DRAWINGS AND PRINTS FROM THE MUSÉE DU
LOUVRE
Germiniani FMB1, Zorzetto FP1, Moro A1, Becker N1,
Nickel R1, Munhoz RP2, Tensini F1, Arruda WO1,
Teive HAG1
• 1HOSPITAL DE CLÍNICAS - UFPR; 2MOVEMENT
DISORDERS CENTRE, TORONTO WESTERN HOSPITAL
Introduction: Medicine and pictorial art often
intermingle. Although traditional portraits of
some medical conditions are somewhat common, the representation of movement disorders
in Medieval prints is somewhat unexpected.
Objective: To describe two medieval depictions
of dystonia in the Baron Edmond de Rothschild‘s
Collection from the Musée du Louvre. Method:
By careful analysis of two prints of the Lights
from the North Exhibition (German Renaissance
Drawings and Prints in Baron Edmond de Rothschild‘s Collection from the Musée de Louvre),
the authors propose that characters were depicted with different forms of dy stonia and make
some considerations with the historical context.
Results: In the Lights from the North Exhibition
(German Renaissance Drawings and Prints in
Baron Edmond de Rothschild‘s Collection from
the Musée du Louvre), presented at the MASP
(Museu de Arte de São Paulo), two prints by Jean
de Cologne, depicted characters with what could
be described as different forms of dystonia. In
“Christ Arrested”, one of the peasants in the foreground appears to have a left lower limb dystonia, while in “Ecce Homo or Christ presented to
the people”, one character engaged in conversation seems to have a more complex, generalized
form of dystonia. We discuss briefly the historical context and compare it with the traditional
historical association with Saint Vitu‘s Dance.
Conclusions: We briefly described two depictions of dystonia in medieval prints of the German Renaissance series of drawings and prints in
Baron Edmond de Rothschild‘s Collection from
the Musée du Louvre and made some comments
about the historical setting.
P-334
FEAR AND PREJUDICE: THE EXPERIENCE OF
EPILEPSY IN LETTERS OF MACHADO OF ASSIS
Amorim GS
• UFMS
Introduction: Regarded as one of the greatest
figures in world literature, Machado de Assis was
diagnosed with epilepsy since childhood. Little
is know about his medical, personal and family
history but the theme of epilepsy has a prominent place in his correspondence, making clear
the contrast between the bright public face, recognized by that society as great intellectual and
suffering in his private life with an incurable
and highly stigmatized disease. This dark side of
his life that he tried to hide at all costs marked
his history and had great influence on his work.
Objective: Analyze the personal correspondence
of Machado, describing the impact of epilepsy in
his work and how the author lived with this condition for carrying a stigmatized disease by their
own society. Methodology: From January to April
2014, 271 personal correspondence, sent and
received, were analyzed, in addition to several
private notes of the author and correlated with
biographical data available. Results: The analysis
of this correspondence between the writer and
close friends has high confessional character of
testimony, revealing that Machado sought comfort in their letters and support to endure the suffering that carried all his life. The name of the disease is never written, and the reference to it is always made of veiled way,always uses other words
such as “radically sick”, “my illness”. The analysis
of the epistolary writings highlights the constant
fear that round:fear to have a crisis in public,of
mental degeneration,of becoming criminal. In
the last decade of life, crises have become more
frequent, and the writer began to suffer from
severe depression. In his last letter, one month
before his death, Machado said he had read the
biography of Flaubert, and have identified with
it, in the sense loneliness, sadness and sickness
that both possessed. Conclusion: The greatest
115
Posters
consequence of Machado‘s epilepsy was his psychological suffering due to the prejudice of the
times. Fear of the disease was so obvious that
perhaps this was the reason why Carolina and
Machado have not had children for fear that they
were born with the same disease his father. From
the analysis of his writings, it is possible to infer
that have a lot of autobiographical, so maybe
can assume a hidden, confessional and allusive
meaning to epilepsy, the famous final phrase of
The Posthumous Memoirs of Brás Cubas: “I did
not have children, do not transmit to any creature the legacy of our misery “.
P-335
FIRST LIVER TRANSPLANTATION IN BRAZIL,
FOURTH IN WORLD FOR FAMILIAL AMYLOIDOTIC
POLYNEUROPATHY TREATMENT
Prado FM, Kosac V, Pupe C, Dornas R, Cal H, Vianna F,
Matta A
• UFF
Introduction: Familial amyloidotic polyneuropathy (FAP) is an autosomal, dominant, debilitating and fatal condition caused by a point mutation in transthyretin (TTR). It affects the peripheral and autonomous nervous system as well as
the heart, the kidneys and the intestinum. Liver
is the main source of the circulating mutant form
of TTR and orthotopic liver transplantation (OLT)
was introduced in 1990 as a radical and hopefully
curative treatment for FAP. In Brazil, the first liver
transplantation in FAP was performed in 1993,
the fourth in the world. Objective: To describe
the first liver transplantation performed in FAP
in Brazil. Case report: A.N.C., 25y.o, presented
three years ago with an important disautonomia
associated with motor and sensory deficit in lower limbs. Some members of his family also have
the same symptoms. DNA analysis performed by
Prof. Maria Joao Saraiva (MJS), from Porto, Portugal, and a sural nerve biopsy were diagnostic for
FAP. Prof Osvaldo JM Nascimento (OJMN), from
UFF, neurologist of Mr. ANC, proposed a liver
transplantation to minimize the disease progression. Eight months later, Prof. Silvano Raia, from
USP - Liver Institute was convinced by OJMN to
performed OLT. Surgery was successful resulting
in TTR Met30 concentration drop in serum. This
OLT was referred in a very known newspaper
journal (“Jornal do Brasil”) opening the interest
of FAP patients for this procedure in our country. The ratification for this OLT came also from
MJS during her visit, at that occasion, to our university hospital, HUAP-UFF, Rio de Janeiro. The
patient had slow progression of neuropathy dying almost twenty years later. Discussion: More
than 95% of circulating TTR is produced in the
liver, the remainder in the choroid plexus and in
the retina. OLT abolishes the hepatic source of
amyloid in TTR variants and it remains the only
potentially palliative treatment until now. In the
last 20 years, all liver transplantations should
be included to the FAP World Transplant Registry (http://www.fapwtr.org). Until now, almost
2000 liver transplants were reported to the registry. This case was the first liver transplantation
in Brazil, and fourth in world for FAP treatment.
Survival statistics are better for patients transplanted earlier in their disease course and for
those <50 years of age. Improvement in autonomic and peripheral nerve symptoms were reported mainly for transplant recipients with the
Val30Met mutation
116
P-338
MY BRAIN DRAWS WELL: THE USE OF
ELECTROENCEPHALOGRAM AS ARTISTIC TOOL
Bruscky IS
• HOSPITAL GERAL DE AREIAS
Objective: Describe the use of electroencephalogram as artistic tool in northeastern Brazil in
1976. Methods: Historical description. Results:
In 1976, the Brazilian artist, Paulo Bruscky, with
support from the neurologist Gilson Edmar
Gonçalves e Silva, performs a performance in a
device that uses electroencephalography to capture the graphics generated by your thoughts.
Bruscky viewed this machine able to produce
designs without the use of hands, a direct design of the brain, in which the artist abdicates
manual technical mastery. A psychographics of
thoughts made with electroencephalography, as
a score of signs, a mental calligraphy that places
the resulting graphs in the category of new visual poetry, performance. Thus, the spheres of art
and science are not set apart and do not respect
watertight boundaries. The artist, like the scientist, invents, speculates, and intervenes, proposing despite the technical impossibilities of time.
However, the artist fit the dream, diversion and
provocation, free of the pressures. It was a job
that gave fruit to a graphic universe of the human mind when it is being analyzed and translated by a machine. Currently, the works are at
the Bronx Museum and the Museum of Modern
Art (MoMA), both in New York. Conclusion: The
work of art is seen as detached proposition, sent
a message to the world without specific sender,
that one day will come to a concrete destination,
are poetic proposals. My brain draws well puts
the relationship between science and mind, an
aesthetic sui generis that would make the delights of both Freud and Goethe.
P-339
NEUROLOGICAL EXAMINATION VIRTUAL
PLATFORM
Paiva BBM, Rodrigues GT, Silva TR, Camargos ST,
Couto N
• UFMG
Introduction: The Neurologic Examination Virtual Platform project consists of detailed semiological technic of the exam on video, showing
the normal exam along with the abnormal one,
with further theoretical explanation accompanied by anatomical and clinical illustration. In
case the viewer has any doubts, the exam is subdivided, so he will be redirected to a video with
the semiological technic The Virtual Platform is
available online for the academic community
through CETES (Centro de Tecnologia e Saúde).
Objectives: To spread ,through the academic
community , the techniques of neurologic examination and the demonstration of abnormal
exam findings. Methods: The videos were filmed
at 1440x1080px 30fps , and exported to the best
possible format for compatibility with browsers
and mobile devices. Filming of the abnormal
neurologic examination started after the patient’s
appropriate signing of an informed consent ,
which authorized academic viewing of the content with concealing his/her identity. Video edition was made using software such as Adobe Premiere®, in order to make the files compliant with
the web’s most compatible formats. Creation of a
website , using PHP , javascript and mySQL , to
allow continuous maintenance without the need
of a programmer. Creation anatomical drawings
of structures closely related to the neurologic examination , overlapping with the video actors , in
order to generate clinical correlations. Results:
A creation of a comprehensive website containing videos from a normal neurological exam and
from patients with certain semiological signs as
well as anatomic and clinical correlation images
and a didactic material were entirely produced
by students and professors at the School of Medicine (Federal University of Minas Gerais) and
hosted at proper servers from the institution.
The platform can be freely accessed by the hotsite from the University: http://medicina.ufmg.
br/neuroexame Conclusion: In a context of increasing amount of information and academic
knowledge, especially in medicine, the need to
optimize the teaching has arisen. This virtual
platform promotes a interactive semiological
course, and thus, a more efficient and friendly
way of presentation to the student.
P-342
THE ESSENTIAL ROLE OF PIERRE MARIE TO
NEUROLOGICAL SCIENCES
Almeida GM, Teive HAG, Germiniani FMB
• UFPR
Introduction: During the ninetieth and twentieth centuries clinicians, anatomists and pathologists performed a considerable work in the field
of Neurology. A special credit should be given
to Pierre Marie, an eminent French neurologist
of the early twentieth century. Objectives: The
purpose of the present work is to highlight the
role of Pierre Marie to the build-up of Neurology.
Results: Pierre Marie (1853‐1940) was a prominent member of the French neurological world
of the early twentieth century and a brilliant
student of Charcot. At the end of his residency
he defended his M.D. thesis on Graves‘s disease
and became chief resident under the direction
of Charcot. Together, they described a condition
characterized by progressive muscular atrophy,
later known as Charcot-Marie-Tooth disease.
Along with Édouard Brissaud, he founded the
journal Revue Neurologique in 1893, and the Société de Neurologie de Paris in 1899, of which he
was the first General Secretary. He was appointed
interne des hôpitaux de Paris in 1878 and associate professor at the Bicêtre Hospital and later
at the Salpêtrière Hospital, in 1895 and 1911 respectively. Marie was also a professor of pathology in 1907 and a member of the Académie de
Medicine. He described acromegaly, cerebellar
tonsil herniation, hereditary cerebellar ataxia,
hypertrophic pulmonary osteo-arthropathy, hereditary cleidocranial dysostosis and rhizomelic
spondylosis, as well as contributing to the study
of bone and joint pathology. His work on aphasia
was opposed to that of Paul Broca and Karl Wernicke, which resulted in several famous and intense debates with Dejerine in 1908. Pierre Marie
published various works dedicated to the neurological sequels due to World War I injuries in
collaboration with Charles Foix (1882‐1927) and
Henry Meige (1866‐1940). At the age of 64, he took
over from Dejerine - whom he hated most implacably – as the chairman of the Clinic of Nervous
Diseases, which he held for six years, from 1917
until his retirement in 1923. In the context of his
time, Pierre Marie displayed in all his work his
scientific skills, performed with rigour and exactness. Conclusion: Pierre Marie‘s researches
contributed to the elucidation and description of
several neurological pathologies, such as hereditary cerebellar ataxia and Charcot-Marie-Tooth
XXVI Congresso Brasileiro de Neurologia
disease. His works led to an incontestable growth
of the medical sciences that transcended his
time.
P-344
WHO WAS CHARCOT‘S FAVOURITE PUPIL?
Germiniani FMB1, Teive HAG1, Munhoz RP2
• 1HC-UFPR; 2TORONTO WESTERN HOSPITAL, CENTRE
FOR MOVEMENT DISORDERS
Introduction: Professor Charcot had several
pupils in his famous neurological service at the
Slapêtriere Hospital in Paris. Some of them would
later achieve prominent roles as Neurologists in
Paris and in other centres. Objective: To review
the achievements of some of Charcot‘s many
stellar pupils and propose who among them
was his favourite. Method: The authors make a
historical review of the achievements of some of
Charcot‘s most prominent pupils and try to establish who his favourite was. Results: Professor
Charcot had several pupils in his famous neurological service at the Salpêtrière hospital. Among
them, Pierre Marie, Joseph Babinski, Gilles de la
Tourette and Edouard Brissaud were ranked as
Charcot’s most favoured disciples. Conclusion:
Historical evidence suggests that probably Brissaud was Charcot’s most favourite pupil.
adjacent to the anterior commissure regions. It is
also noted mild hyperintensity on T2 on the mesial regions. Progressed with episodes of seizures,
controlled with phenobarbital. One stereotactic
biopsy of the brain lesion which showed grade
II astrocytoma and opted for treatment with radiotherapy (RT) was performed. Currently, the
patient is in good general condition, ambulating
and outpatient. Discussion: The case is based
on the differential diagnosis of brain stem lesions. The clinical history and neurological examination suggest the location of stem tumor,
but the imaging study with spectroscopy plays
an important role in demonstrating the clinical
behavior of the lesion. The differential diagnosis
consists of primary or secondary brain tumors,
because the patient had a history of histiocytosis
X in childhood and use of chemotherapy. In the
above case the biopsy was had a key role in clarifying the etiology to guide treatment.
P-347
CEREBRAL AMYLOID ANGIOPATHY-RELATED
INFLAMMATION: AN UNCOMMON PRESENTATION
OF CEREBRAL AMYLOID ANGIOPATHY WITH
UNIQUE NEUROIMAGING FINDINGS
Oliveira LM, Santos DC, Simabukuro MM, Pinto LF,
Trindade BMC, Silva FEF, Lucato LT, Castro LHM,
Nitrini R
• HCFMUSP
Neuroimaging
P-346
ASTROCYTOMA BRAINSTEM IN PATIENT WITH
HISTIOCYTOSIS X
Ferreira JL, Godeiro Junior CO, Dourado Júnior MET,
Moreira Neto M, Correira CER, Figueiredo MM,
Afonso COM, Campos MLS, Brito PSM, Silva TFV
• UFRN
Introduction: Diffuse astrocytomas are lowgrade malignant brain tumors of slow growth,
but with heterogeneous clinical behavior. The
incidence is 8‐15% of all primary brain tumors
in adults. Objective: To present an unusual case
of low grade astrocytomas in a patient with Histiocytosis X. Clinical case: A female patient, 26
years old, with a history of dizziness and imbalance 1 year ago. The patient started presenting
gait ataxia and dysarthria. During the following
3 months, the patient showed gradual worsening of imbalance and dizziness, dysphagia for
solids and liquids, weakness in right hemibody
and frequent falls. Past Histiocytosis X in childhood, trated with Radiotherapy and Chemotherapy. The pacient was conscious, oriented,
with ataxia, dysarthria and preserved trofismos
tone, strength of upper limbs preserved, dysmetria, Babinski sign on the right, normal reflexes
in upper limbs and high reflexes in lower limbs.
Presence of horizontal and vertical nystagmus.
Superficial and deep sensitivity preserved. Cranial MRI performed spectroscopy which showed
hyperintense T2 lesions affecting the brainstem,
with expansion to bridge involving cerebellar
peduncle depth of the cerebellar hemispheres,
associated with foci of hyperintensity on T2
without expansive effect, the depth of the cerebral hemispheres, nucleocapsulares in and
Introduction: Cerebral amyloid angiopathy-related inflammation (CAA-I) has been recently
recognized as an uncommon presentation of
cerebral amyloid angiopathy. It usually presents with acute or subacute onset of headaches,
cognitive and behavioral changes, seizures and
focal neurological deficits. Magnetic resonance
imaging (MRI) shows characteristic findings.
Objective: To report a case of cerebral amyloid
angiopathy-related inflammation emphasizing
its clinical presentation and distinctive neuroimaging findings. Case report: A 64-year-old man
reported sudden onset of altered sensation and
weakness of the left arm. He presented to our
hospital with mild left arm weakness. Cognitive
examination showed mild executive dysfunction. Soon after admission, he developed focal
status epilepticus, characterized by left sided
clonic jerks, successfully treated with intravenous phenytoin. MRI showed a right frontoparietal intraparenchymal hemorrhage and typical
imaging findings of CAA-I: FLAIR/T2 white matter hyperintensities in the frontal lobes and in
the right temporal lobe suggestive of vasogenic
edema, associated with multiple scattered cortical/subcortical microbleeds evidenced in susceptibility weighted imaging. CSF was normal
except for mildly elevated protein; EEG showed
no epileptiform activity. Infectious and neoplastic causes were ruled out. Based on clinical presentation and characteristic neuroimaging findings, the patient fulfilled criteria for ‘probable
CAA-I’. We decided against a brain biopsy. CSF
anti-amyloid β antibody assay was ordered and
intravenous methylprednisolone and cyclophosphamide were started. Discussion: Clinicians
should strongly consider the diagnosis of CAA-I
when MRI reveals microbleeding associated with
multiple white matter lesions suggestive of vasogenic edema. Treatment includes high-dose
corticosteroids and immunosuppressive agents,
primarily cyclophosphamide. Most patients
show at least a partial response in the first three
weeks. Optimal treatment duration remains
uncertain. Radiological response usually parallels clinical improvement and should help to
guide therapy duration. This case highlights the
importance of early recognition of the clinical
symptoms and typical radiologic features of this
potentially treatable disease.
P-348
CEREBROTENDINEOUS XANTHOMATOSIS –
REPORT OF TWO SISTERS
Oliveira NP1, Prado MR1, Acchar MC1, Lima MA2
• 1INDC; 2UFRJ
Introduction: Cerebrotendineous xanthomatosis (CTX) is a rare autosomal recessive disease
characterized by deficiency of 27-hydroxylase,
leading to lipid accumulation in various tissues.
Objective: Describe the clinical, radiological and
laboratory findings of CTX in two sisters. Case
reports Patient 1: 34 year-old woman with a previous diagnosis of,schizophrenia presented with
gait impairment due to spasticity and cramps.
Medical history revealed perinatal anoxia as
well as congenital cataracts and chronic diarrhea since childhood. One sister (patient 2) has
a similar clinical picture. Clinical examination
disclosed xanthomas in the ankles , an ataxic
gait with positive Romberg sign, spasticity and
brisk reflexes in the four limbs and palatal tremor. Patient 2: 35 year-old woman. with history
of seizures and gait impairment. Neurological
examination showed universal hypotonia and
hyperreflexia, bilateral intention tremor. The
electrophisiologic finding of patient 1 shows a
sensory-motor polyneuropathy with with axonal features restricted to the lower limbs. Brain
Magnetic resonance imaging (MRI): hyperintense signal in T2 and Flair in the white matter
, symmetric leukomalacia and hyperintense
signal in the pons, cerebelar dentate nuclei and
peduncles. Cerebellum atrophy. Hyperintense
signal in the medula oblongata olive’s. Serum
Cholestanol was 20,58mcg/ml. The MRI of patient 2 shows hyperintense signal in T2 and Flair
in the cerebelar dentate nuclei. Discussion: CTX
is a autosomal recessive disease characterized
by the accumulation of lipids due to mutations
in the gene responsible for the enzyme sterol
27-hydroxylase. Clinical manifestations include
chronic diarrhea, cataracts and xanthomas. Neurological involvement is heteregeneous. Pyramidal signs, peripheral neuropathy, ataxia, seizures
and cognitive decline can be obseved. The diagnosis can be confirmed by the dosage of colestanol. Electrophysiological findings show axonal
changes described as mixed neuropathy. MRI
usually demonstrates hyperintense lesions in
T2 and Flair located in periventricular area, basal ganglia and dentate nuclei of the cerebellum
with cerebellar atrophy. Chenodeoxycholic acid
replacement therapy is used in order to prevent
the colestanol deposit in the tissues. Early recognition and treatment can prevent neurological
injury.
P-349
CORTICAL LAMINAR NECROSIS AFTER PROMPT
HYPONATREMIA CORRECTION
Grativvol RS, Grativvol RS, Pereira Filho JCG,
Cavalcante WC, Studart Neto A, Castro LHM
• HCFMUSP
Introduction: Hyperintense cortical lesions in
T1 weighted brain MRI radiologically define cortical laminar necrosis. This finding is generally
atributed to severe neuronal damage following
hypoxia, but other less common causes such as
fast hyponatremia correction should be remembered as differential diagnosis. The present paper
117
Posters
is a report of cortical laminar necrosis after rapid
sodium replacement presenting as a demential
syndrome. Objectives: Reassure about the relevance of careful sodium correction, as well as
demonstrate an uncommon image finding attributable to inadequate hyponetremia treatment.
Case report: A 65-year-old female, with a past
medical history of hypertension, was admitted to
a local hospital with an abrupt onset of abdominal pain, vomiting, diarrhea and altered mental
status. A diagnosis of diverticulitis was made and
she was started on intravenous antibiotics. Laboratory tests revealed a severe hiponatremia with
a sodium level of 109mg/dl, which was immediately treated with hypertonic saline. Seven hours
following the beginning of electrolyte replacement a new sodium value of 139mg/dl, and the
patient was intubated due to worsened level of
conscience. After a few days in the intensive care
unit, mechanical ventilation was withdrawn after
improvement in the clinical scenario and mental
status. Nonetheless the patient showed severe
signs of cognitive disturbance (descrever melhor
que tipo) not appreciated before, which led to
extensive investigation during hospitalization.
Brain MRI revealed diffuse cerebral cortical signal changes associated with hypersignal seen on
T1 weighted sequence, without restricted diffusion or enhancement after contrast injection. All
the other several additional tests were unremarkable. The patient was discharged several days
later with little clinical improvement of the cognitive syndrome. Discussion: Cortical laminar
necrosis, characterized by severe damage of the
central nervous system, is a rare complication of
inadequate management of hyponatremia. This
condition should be remembered as a cause of
neurological disorder following rapid correction
of low sodium levels with hypertonic saline.
P-350
DEVELOPMENT OF EDUCATIONAL MATERIAL FROM
MAGNETIC RESONANCE IMAGES IN AXIAL COURT
FOR THE STUDY OF NEUROANATOMY
Oliveira KLC, Raeder MTL, Reis F
• UNIVERSIDADE ESTADUAL DE CAMPINAS - UNICAMP
The diagnostic neuroimaging technologies, such
as magnetic resonance imaging (MRI), may also
contribute to the development of learning tools
for the study of neuroanatomy by medical students, allowing a direct contact with these images that have revolutionized the knowledge and
the treatment of neurological disorders. Thus,
the aim of this study was to develop an atlas with
MRI images of the supra and infratentorial regions that can be used as an instrument for the
study of neuroanatomy. MRI images in T1 and
T2 weighted and 3D-CISS were selected, with the
identification of the anatomical elements in levels of court in axial planes highlighting structures
in the following observed areas: bulb, bridge,
midbrain, cerebellum, cranial nerves, diencefalic
structures, basal ganglia, telencephalon, vessels
and the ventricular system. The recognition of
anatomical sites involved in pathologies of the
central nervous system (CNS) contributes to the
learning process of neuroanatomy and is quite
useful for radiological practice. Our pictorial essay is useful for undergraduates in medicine who
are studying neuroanatomy and radiologists,
as it provides developed material for study and
consultation.
118
P-351
DEVELOPMENT OF EDUCATIONAL MATERIAL FROM
MAGNETIC RESONANCE IMAGES IN SAGITTAL
AND CORONAL PLANES FOR THE STUDY OF
NEUROANATOMY
Raeder MTL, Oliveira KLC, Reis F
• UNICAMP
Introduction: The diagnostic neuroimaging
technologies, such as magnetic resonance imaging (MRI), may also contribute to the development of learning tools for the study of neuroanatomy by medical students, allowing a direct contact with these images that have revolutionized
the knowledge and the treatment of neurological
disorders. Objective:The aim of this study was to
develop an atlas with MRI images in sagittal and
coronal planes of the supra and infratentorial regions that can be used as an instrument for the
study of neuroanatomy. Methods: MRI images
in T1 and T2 weighted and 3D-CISS were selected, with the identification of the anatomical elements in levels of court in sagittal and coronal
planes highlighting structures in the following
observed areas: bulb, bridge, midbrain, cerebellum, cranial nerves, diencefalic structures,
basal ganglia, telencephalon, vessels and the
ventricular system. Discussion: The recognition
of anatomical sites involved in pathologies of the
central nervous system (CNS) contributes to the
learning process of neuroanatomy and is quite
useful for radiological practice. Conclusion: Our
pictorial essay is useful for undergraduates in
medicine who are studying neuroanatomy and
radiologists, as it provides developed material for
study and consultation.
P-352
DIFFUSION- WEIGHTED IMAGING IN ACUTE
HYPERAMMONEMIC ENCEPHALOPATHY
Narimatsu K, Toller VB, Pandullo FL, Morgulis RF
• HOSPITAL ISRAELITA ALBERT EINSTEIN - HIAE
Introduction: Hyperammonemic Encephalopathy (HE) has been described as alteration in brain
function manifesting as an attentional disorder,
progressive drowsiness, coma and death due to
high level of blood ammonia. Often, the clinical
evalution is unsatisfactory and imaging may provide early clues to the underlying disorder. Abnormal signal intensity an diffusion restriction
patterns on MRI may provide important clues
as to the diagnosis. Objetive: We present one
case with characteristic DWI features of acute
HE. Case: A 36-year-old male patient was transferred to hospital with episodes of confusion that
progressed to lethargy over 5 days. He had been
taking excessive amounts of acetaminophen for
migrainous headaches and manipulation medication for exercises. Neurological examination
hampered by intubation and sedation. Laboratory testing demonstrated acute liver failure with
ALT 213 U/L, AST 168 U/L, RNI 1,76 and ammonia 67 micromol/L. The others exams was normal included screening infection, hepatitis panel, paraneoplastic antibodies, autoantibodies
and cerebrospinal fluid. Electroencephalogram
was inespecific. The MRI/ DWI showed restricted
diffusion bilateral and symmetric involvement
of the frontal, insular cortex and cingulate gyrus.
Day 2, the MRI/ DWI showed additional involvement of parietal and temporal lobes. The MR
spectroscopy reveals elevation in glutamine/glutamate followed by decrease in myoinositol and
choline peaks. After treatment of encephalopathy with lactulose, dietary protein restriction,
propofol sedation, L- ornitin and antibioticoterapy for reduction ammonia, he was subsequently
extubed on hospital day 4. He survived with mild
intellectual impairment. Discussion: The radiologic findings of acute HE are less reconized in
the adult literature. In the pediatric population,
acute HE and its imaging findings have been well
described as a result of inborn errors of metabolism. In this adults, this condition is more commonly encoutered in drugs use (acetaminophen,
sodium valproate, chemotherapy). Bilateral
symmetric involvement of the insular cortex and
cingulate gyrus is fairly consistent. The involved
regions are restricted on DWI. Moreover, it is very
plausible from the striking similarity among our
case and others report in the literature, that the
imaging pattern described is directly related to
HE.
P-354
GLIOMATOSE CEREBRAL COM DISTÚRBIO
COMPORTAMENTAL COMO MANIFESTAÇÃO INICIAL
Moreira FH, Corrêa LM, Cruzeiro MM
• UFJF
Cerebral gliomatosis: Comportamental Changes as Initial Manifestation The Gliomatosis
cerebri (GC) is a rare and diffusely infiltrating
glial neoplasm of the central nervous system,
involving at least three adjacent brain lobes. Its
clinical presentation is highly variable, whose
course is poorly defined and fatal. Described initially by NEVIN in 1938 and defined by the World
Health Organization (WHO) has dark prognosis
with median survival of 50% in 1 year and 25%
in 3 years. There are few cases reported to date.
The objective of this study is to report the case
of a man with GC whose initial manifestations
were comportamental changes. 49-years-old
man, in psychiatric supervision, progressed with
parcial seizures in a few months, iniciating corticosteroids and antiepileptic drugs. The skull
magnetic resonance imaging (MRI) showed an
extensive area of poorly defined margins and
heterogeneous intensity signal, predominantly
hypointense on T1 and hyperntense on T2 and at
FLAIR sequence, not contrast captured, showing
perfusion diffusely increased affecting the frontal lobes, especially the white matter subcortical
and fronto-temporal lobes, thalamus and right
lentiform nucleus, anterior portion of the corpus
collosum, causing effacement of the front horns
and anterior portion of the lateral ventricles,
adjacente cortical sulcus and sylvian fissures.
Currently he has periods of hallucination, labile
and erratic behavior; diabetic microangiopathy;
hyperphagia; urinary and fecal incontinence,
global muscle atrophy, weakness and hypotonia.
The GC grade III diagnosis was defined by brain
biopsy; being instituted radiotherapy with clinical iprovement and stabilization of the tumor
growth. It was followed by endocrine-metabolic
disorders (hyperglicemia, hypertriglyceridemia
and hypercholesterolemia). The symptoms described in literature are variable, with reports of
seizures, focal signs, headache, cognitive changes, cranial neuropathies, intracranial hypertension, spinocerebellar disorder and progressive
hemiparesis. Infiltrative MRI findings were highly suggestive and the biopsy was defining. The
extent of the injury prevents resection and radiotherapy was the most appropriate treatment
option. We concluded that performing imaging
studies in cases of behavior changes is important, because, despite the rarity, GC can be the
cause, variable is the evolution, and may have
considerably extended survival.
XXVI Congresso Brasileiro de Neurologia
P-355
GLUTARIC ACIDURIA TYPE 1 (GLUTARIL-COADEHYDROGENASE DEFICIENCE) WITHOUT
MACROCEPHALY: THE IMPORTANCE OF MAGNETIC
RESONANCE IMAGING TO DIAGNOSIS
Medeiros LL, Souza Filho MCM, Leandro Junior JE,
Melo ÁN
• DEPARTAMENTO DE PEDIATRIA
Introduction: Glutaric Aciduria type I (GA-I) is a
rare autosomal recessive disorder resulting from
glutaryl-CoA dehydrogenase deficiency, its prevalence is 1 in 56000. Approximately 90% of these
patients will develop neurological disease during
a finite period of brain development (age 3‐36
months) following an acute encephalopathic crisis. It is characterized by macrocephaly at birth
(75% of patients during infancy); dystonia is the
dominant symptom, usually superimposed on
axial hypotonia. Neuroimaging serves as a usefull
tool, many times providing the first clue to the
diagnosis. Objective: Our purpose is to illustrate
the value of brain magnetic resonance imaging
(MRI) in the diagnosis of GA-I even without macrocephaly. Case report: An 18-month-old male,
born from no consanguineous parents, was referred to our hospital after acute encephalopathic crisis. His motor development was normal.
His neurological examination showed: dystonia,
which was the dominant extrapyramidal symptom, superimposed on axial hypotonia, opisthotonos and normal head circumference. Complete
blood picture, serum electrolytes, blood glucose,
liver function test, and CSF study were normal.
MRI revealed frontotemporal atrophy, dilated
sylvian fissures with open opercula (bat-wing appearance) with hyperintense lesions in bilateral
basal ganglia suggestive of glutaricaciduria type
1. The diagnosis was confirmed by urinary organic acid analysis by TMS (tandem mass spectrometry) which revealed a marked excretion of
glutaric acid. Discussion: Neuroimaging plays a
key role in the investigation of children with AGI. Early detection and institution of a therapy is
important and may result lessening the neurologic sequelae. MRI is the imaging modality of
choice. The early features is frontal atrophy and
basal ganglia abnormalities and are encountered
in over 90% of patients. The incomplete opercularization of the insular cortex, widening of the
sylvian fissures, and CSF spaces are also characteristic of GA‐1. The diagnosis of GA‐1 rests on the
demonstration of urinary excretion of glutaric
acid, 3-hydroxyglutaric acid, and glutaconic acid
by TMS. GA‐1 is an important neurometabolic
disorder in children that is sometimes misdiagnosed, but it can be diagnosed easily based on
high index of suspicion on typical clinical-neuroimaging. This report shows that diagnosis may
also be suggested by characteristic finding MRI
in patient with acute encephalopathic crisis
without macrocephaly.
P-356
HYPERTROPHIC OLIVARY DEGENERATION
SECUNDARY TO CENTRAL TEGMENTAL TRACT
INJURY: A CASE REPORT
Raeder MTL, Araújo NAA, Oliveira KLC, Reis F
• UNICAMP
Introduction: The Hypertrophic Olivary Degeneration (HOD) represents a rare transsynaptic degeneration that occurs due to an injury involving
the dentato-rubro-olivary tract and results in the
disconnection of afferent axons to the inferior olivary nucleus. Objective: The aim of the present
study is not only to familiarize the radiologist
with this diagnosis, once it is uncommon in everyday practice and it is characterized by a peculiar clinical presentation, but also to avoid misdiagnosis. Case report: A 25 year old male patient
with a history of illicit drugs abuse developed
daily crises of roundabout episodic dizziness
and after thirty days a sudden loss of balance
followed by drop and fleeting blindness with
visual recovery and subsequent blurred vision,
diplopia, left facial paresthesia and buccal angle
deviation to the right. After beeing submitted
to a Magnetic Ressonance Imaging (MRI) of the
cranium, he was diagnosed with a bulbar hemorrhagic stroke. The patient was referred to the
neurosurgery ambulatory for follow-up, where
an expectant approach was adopted. About six
months later, it was revealed by MRI a hyperintensity and an increased volume of the left cerebellar olive, featuring the diagnosis of HOD. His
last MRI shows hyperintensity in the left cerebellar olive, but in involution when compared
to previous exams. Discussion: The inferior olivary nucleus is part of the dentato-rubro-olivary
pathway, also called Guillain-Mollaret triangle,
and it designs its efferent fibers through the superior cerebellar peduncle until the contralateral
red nucleus. Fibers from the red nucleus are sent
to the ipsilateral inferior olivary nucleus via central tegmental tract. The inferior olivary nucleus
in turn connects with the contralateral dentate
nucleus through efferent fibers via inferior cerebellar peduncle thus forming a triangle. Insults
to any of these components may result in an
axon interruption to the inferior olivary nucleus leading to its degeneration. When the lesions
are restricted to central tegmental tract, HOD is
ipsilateral. When the dentate nucleus or the superior cerebellar peduncle are affected, HOD is
contralateral. If both central tegmental tracts are
injured or lesions involve the superior cerebellar
peduncle and the contralateral central tegmental
tract simultaneously, HOD is bilateral. A T2 hyperintense image observed in MRI and located in
the anterolateral portion of the bulb that shows
no uptake of contrast is suggestive of HOD.
P-357
IGG4-RELATED HYPERTROPHIC
PACHYMENINGITIS
Sousa ACJ, Correia DSA, Martins TAC, Taranto AG,
Tauil CB
• HOSPITAL DE BASE DO DISTRITO FEDERAL
A 58-year-old asymptomatic woman was referred
to our neurologic clinic for investigation of pachymeninigitis. The patient had a previous history of systemic arterial hypertension that was well
controlled with anlodipine. Neurological history was positive for a previous episode diplopia
which lasted about a month with spontaneous
remission seven years prior to admission. There
were no signs of systemic disease. The patient
denied fever of chronic cough. Neurological examination was unremarkable except for hypoacusia in the right ear. Brain MRI showed hyperintense dural thickening predominantly at the tentorium cerebelli (Figures 1 and 2). Routine blood
work was normal. Serum IgG was 2470mg/dl
(normal range 700‐1600) and IgG4 was 3670mg/L
(normal range 69‐888 mg/L). Cerebral spinal fluid
(CSF) analysis revealed no abnormalities except
the presence of oligoclonal bands. CSF was negative for bacteria, fungi, tuberculosis or neoplastic
cells. CSF levels adenosine deaminase were also
normal. Infectious workup including HIV, VDRL,
PPD was negative. Thyroid hormone levels
within normal range, but anti-TPO and anti-peroxidade were elevated. Serum angiotensin converting enzyme was also normal. Computerized
tomography of the chest and abdomen showed
no abnormalities. The patient awaits meningeal
biopsy to confirm the diagnosis of IgG4-related
hypertrophic pachymeningits and to rule out
other possible treatable conditions. Discussion:
IgG4-related disease is a rare fibroinflammatory condition that was recently recognized to
involve many different organs. The diagnosis is
confirmed by histopathological analysis of the
biopsy material. Classic findings include dense
lymphoplasmacytic infiltrate, mild to moderate
eosinophil infiltrated and sclerosis of the affected region. There are only a few Case reports
of IgG4-related hypertrophic pachymeningits.
Approximately 70 to 90% of patients with this
condition have elevated serum IgG 4 levels. CSF
studies generally reveal clear fluid, variable degree of lymphocytic pleocytosis, normal glucose
concentrations and normal or mildly increase
protein levels. Since our patient has not been
submitted to menigeal biopsy yet, the diagnosis
could not be confirmed, although it is probable.
In conclusion, it is important to emphasize that
even though IgG4-related disease is a rare condition, it should be considered in the differential
diagnosis of hyperthrophic pachymeningitis.
P-358
IN SEARCH OF A BETTER METHOD TO ASSESS
PERIVASCULAR SPACES IN VASCULAR
PARKINSONISM
Corrêa TD, Martins WRM, Castro JPS, Marques HC,
Paula JM, Vale TC, Pires LA
• UFJF
Introduction: Perivascular spaces (PVS) are extensions of subpial spaces and interstitial fluid
filled channels that house penetrating arteries
and emerging veins. Once enlarged, PVS may be
seen on MRI along the path of penetrating vessels
into basal ganglia, centrum semiovale and pontomesencephalic junction, being isointense with
cerebral spinal fluid (CSF) on all pulse sequences. Differentiation between enlarged PVS can be
challenging for neurologists and radiologists,
mainly when it comes to diagnoses such as vascular parkinsonism (VP). This secondary form of
parkinsonian syndrome results from cerebrovascular disease (CVD) and leads to strategic infarcts
in the basal ganglia and diffuse ischemic lesions
of the subcortical white matter; its diagnosis is
based on clinical and imaging findings. SPACE
MRI sequence is a 3D high resolution turbo spinecho reconstruction, with variable angulation,
chosen to better demonstrate subcortical lacunar lesions and perivascular spaces. Objective:
Evaluate if the SPACE sequence is able to differentiate lacunar lesions from perivascular spaces
in VP. Case report: An 85-year-old male patient,
hypertensive, with chronic atrial fibrillation,
chronic kidney disease stage III, started a year
ago symptoms compatible with VP. Neurological
examination showed parkinsonism with lower-limb predominance, pyramidal signs, urinary
incontinence and dementia (Mini Mental State
Examination =13/30, with 4 years of schooling).
Levodopa was gradually increased up to 1 g/day
without response. Brain MRI demonstrated diffuse cortical atrophy with ischemic lacunes and
diffuse white matter lesions. Using the SPACE
sequence, it was possible to see with greater
detail hyperintense lesions in the basal ganglia,
thalamus and internal capsule bilaterally, but
not allowing accurate differentiation between
119
Posters
enlarged PVS and lacunar infarcts. Discussion:
Some criteria to distinguish enlarged PVS from
lacunar infarcts have been reported, such as
size, shape, intensity and location. It consists of a
challenging task and some authors consider the
absence of any hyperintense rim surrounding
large PVS detected on FLAIR images to differentiate them from lacunar infarctions. In our case,
SPACE sequence, even with excellent contrast
between CSF and other structures, was not useful to distinguish PVS from lacunar infarcts.
P-360
MAGNETIC RESSONANCE DIFFUSION IMAGING IN
THE DIAGNOSIS OF CENTRAL NERVOUS SYSTEM
DISEASES: A LITERATURE REVIEW
Ratzkob GF, Gariba MA, Aguiar LR
• PUC
Introduction: Magnetic resonance diffusion
tensor imaging (DTI) allows the visualization of
bundles (tracts) of nerve fibers in vivo, as well
as their orientation and integrity, by quantifying
the diffusion of water molecules in the neural
fibers. The key parameters used to evaluate the
diffusion in white matter are Fractional Anisotropy (FA) and the Mean Diffusivity (MD). High
structural integrity of white fibers leads to high
values of FA and low values of MD, and variations in these parameters indicate an impairment of the fibers. This technique has been used
in the investigation of central nervous system
diseases. Variations in the affected regions and
their FA and MD values were found. Objective:
To identify and classify the applications of the
MRI diffusion technique in the diagnosis of
central nervous system diseases. Method: The
research consisted of a systematic review of the
literature, between August 2013 and June 2014,
using the terms “DTI” and “Systematic Review”
in PubMed and SciELO bases. A total of 59 out of
the 180 articles found were selected, since they
quantified FA and MD. Results: It was found
that this technique has been employed to study
neuropsychiatric diseases and in neurosurgery.
The most common were: Parkinson‘s disease
(3%), Alzheimer‘s disease (10%), Multiple Sclerosis (3%), Amyotrophic Lateral Sclerosis (3%),
Epilepsy (5%), Stroke (5%), Concussion/Brain
Trauma (10%), Brain Tumor (10%), Schizophrenia (5%), Bipolar Disorder (5%) and depression
(7%). In the majority of them, the findings were
a decreased FA value and an increased MD value,
indicating degenerative and/or harmful processes in diffuse areas of the brain, such as in Alzheimer‘s disease, or in specific tracts, depending on
the disease analyzed. Conclusion: This quantitative imaging technique proved to be useful in the
research of certain diseases of the central nervous system. Furthermore, it has applications in
surgical planning for resections or biopsies, early
diagnosis of asymptomatic neoplasia or degenerative diseases of the nervous system. It can be
used to evaluate the extent of diseases, monitor
its progress, the response to treatment and prognosis. The main limitations of the technique occur in regions where there are crossing fibers, the
fibers branch in more than one direction, there is
a lack of homogeneity in the magnetic field, there
is head movement during acquisition or by the
presence of artefacts.
120
P-361
NEUROANATOMY OF LEADERSHIP:
UNDERSTANDING THE LEADER`S BRAIN
Mesquita HM, Furtado TR
• FUNDAÇÃO GETULIO VARGAS
Neuroanatomy of Leadership: understanding
the leader`s brain Introduction: The main characteristic of a leader and the ability to build social
relationships that inspire and motivate others to
do their best. Recents advances in neuroscience,
using tools such as qEEG and functional magnetic resonance imaging, allow us to understand
the internal mechanisms that cause some people
are more efficient than others in leading people.
Objectives: Conduct a literature review to describe what the brain mechanisms are related to
effective leadership and ineffective leadership.
Methods: Review of studies published from 2009
to 2014 using the Pubmed and Lilacs platforms.
Key words adopted: leader, leadership, neuroscience, cognition, social intelligence, EEQ, functional magnetic resonance imaging. Results: It
was observed activation of 14 regions of interest
in the brain of the effective leaders while only 06
brain regions were activated and 11 were inactivated in the dissonant leaders (chief ) . The activated regions in the brain of the effective leader
were: Attention activation area (area of the anterior cingulate), social networks pattern (right
inferior frontal gyrus), Mirror Systems (parietal
Right lower lobe) and relationship (putamen
and insula bilateral). The dissonant leader inactives areas related to social relations and mirror
system, and activate areas related to directed
attention (bilateral cingulate cortex) and those
related with less compassion (left posterior cingulate cortex) and negative emotions (posterior
inferior frontal gyrus and). The cellular standpoint highlights the importance of mirror neurons, neurons and neurons in oscillatory zone.
Conclusion: The brain mechanisms associated
with effective leader allows him to have the ability to understand the emotional reactions of their
subordinates, as well as their own control. This
evolutionary advantage allows direct their gestures, actions and oratory activate the emotional
circuits of their subordinates inspiring them and
motivating them to do their best.Currently we
live in a leadership crisis, where 75% of leaders
fail to take the proposed office, the knowledge
of these brain mechanisms can be an important
tool in the future, in selecting a leader for a specific situation, as well as guide your training.
P-362
NEUROVASCULAR CONFLICT - A CAUSE OF
UNUSUAL PARESTHESIA IN FACE - CASE REPORT
Schäfer AJ, Iachisnki RE, Bocchi C, Henning PR,
Bettin TE
• FAG
Introduction: The presence of neurovascular
conflict between superior cerebellar artery and
the trigeminal nerve on magnetic resonance
imaging (MRI) usually diagnostic of trigeminal
neuralgia. This is characterized by severely paroxysm al pain triggered by chewing or talking,
and that is usually not followed by hyposensitivity or paresthesia in the face. Touch sensitive
face symptoms (paresthesia, hyposensitivity)
alone do not suggest the diagnosis of neurovascular conflict. Objective: Report the case of
a patient with left facial paresthesia path V1, V2
and V3 and that presents neurovascular conflict
in RM. Case report: Woman, 36 years, refers left
facial paresthesia progressively initiated 3 years
ago. Continuous symptom not associated with
pain and not triggered by external factors or by
chewing movements. No other symptoms. On
neurological examination presents tactile hyposensitivity left facial (path V1, V2 and V3), with remainder of the neurological examination of cranial nerve sand no particularities. Brain MRI was
performed with neurovascular conflict research,
which demonstrated the presence of vascular
loop between the left superior cerebellar artery
and the left trigeminal nerve. Discussion: The
presence of paresthesia in the face, especially in
young people, proposes diagnosis of demyelinating, infectious, psychiatric, metabolic and oncological diseases. On the basis of PUBMED were
not found publications on neurovascular conflict
and facial paresthesia. The reported case expresses the importance of investigating, through
MRI research vascular loop, patients complaining of abnormal sensitivity in the face, especially
those in which the main causes were excluded.
P-363
PHASE CONTRAST X-RAY IMAGING OF HUMAN
PERIPHERAL NERVES
Scopel JF1, Queiroz LS2, O’Dowd FP3, França Júnior MC2,
Nucci A2, Hönnicke MG4
• 1UNIVERSIDADE FEDERAL DE GOIÁS; 2UNICAMP;
3
LNLS; 4UNILA
Purpose: Diagnostic imaging techniques play an
important role in assessing the exact location,
cause, and extent of a nerve lesion, thus allowing clinicians to diagnose and manage more
effectively a variety of pathological conditions,
such as entrapment syndromes, traumatic injuries, and space-occupying lesions. Ultrasound
and nuclear magnetic resonance imaging are
becoming useful Methods: for this purpose, but
they still lack spatial resolution. In this regard,
recent phase contrast x-ray imaging experiments
of peripheral nerve allowed the visualization of
each nerve fiber surrounded by its myelin sheath
as clearly as optical microscopy. Methods: In
the present study, we attempted to produce
high-resolution x-ray phase contrast images of a
human sciatic nerve by using synchrotron radiation propagation-based imaging (PBI). Results:
The PBIs showed high contrast and high spatial
resolution, allowing clear identification of each
fascicle structure and surrounding connective
tissue. The outstanding result is the detection of
such structures by phase contrast x-ray tomography of a thick human sciatic nerve section. This
may further enable the identification of diverse
pathological patterns, such as Wallerian degeneration, inflammatory infiltration and amyloid
deposits. Conclusion: To the best of our knowledge, this is the first successful phase contrast
x-ray imaging experiment of a human peripheral
nerve sample. Our long term goal is to develop
peripheral nerve imaging Methods: that could
supersede biopsy procedures.
P-364
POST-TBI CHRONIC ENCAPSULATED
INTRACEREBRAL HAEMATOMA ASSOCIATED WITH
GIANT CAVERNOMA
Shinosaki JSM, Santos MBC, Silva ACAL, Souza LP,
Tunala T, Faria LF
• UNIVERSIDADE FEDERAL DE UBERLÂNDIA
Introduction: Cavernomas represent 5‐13% of
cerebral vascular malformations. The giant ones
are very rare. Chronic encapsulated intracerebral
haematomas(CEIH) are also rare; its association
XXVI Congresso Brasileiro de Neurologia
with arteriovenous malformations is well known,
but not with cavernomas. We present a case seen
as post-TBI CEIH that has proven to be a giant
cavernoma. Objectives: To highlight the need to
pursue differential diagnoses of CEIHs even having an apparent cause, especially given an unusual clinical and radiological evolution; emphasize the importance of MRI in the search for diagnostic clues, since cavernomas are angiographically occult. Report: man, 39y, TBI in April/2012.
CT showed a hyperdense temporal lesion, considered hemorrhagic contusion conservatively
managed. Soon he developed headaches, and
after six months started presenting epileptic
seizures controlled with carbamazepine. MRI
in April/13 showed the same right temporal lesion, 4,7x4,8x3,1cm, with heterogeneous signal,
partially hematic content at different stages of
hemoglobin degradation, exacerbation of magnetic susceptibility phenomenon in T2*, with hypointense halo, without significant mass effect.
After two months, he developed symptomatic
worsening that improved partially due to drug
adjustment. Cerebral arteriography was normal.
MRI on November/13 showed some mass effect.
The intraoperative procedure on March/2014
evidenced an encapsulated haematoma and a
lesion which microscopy showed to be an extensive hemorrhagic area with hemosiderin and hematoidin deposits, indicating previous hemorrhage. At the periphery of the lesion, there were
blood vessels of varying caliber, sometimes with
discontinuous internal elastic lamina, consistent
with vascular malformation. There was epilepsy
remission and improvement of headache afterwards. Discussion: Approximately 50% of cavernomas are diagnosed incidentally. In this case,
the TBI may have triggered the formation of the
hematoma or its previously asymptomatic expansion, with consequent development of symptoms. It is important to notice the MRI showing
hemoglobin in different stages of degradation
(the greatest diagnostic clue) without neglecting
the possibility of variations in the case of giant
cavernomas, such as mass effect, besides maintaining a high degree of suspicion for underlying
pathologies in post-TBI haematomas that are not
resolved at the usual time. In this case, only the
surgical approach allowed the diagnosis and epilepsy remission.
P-365
POSTERIOR REVERSIBLE ENCEPHALOPATHY
SYNDROME AFTER RITUXIMAB IN A PATIENT WITH
SYSTEMIC LUPUS ERITHEMATOSUS
Prado FM, Amitrano D, Cal H, Kosac V, Nogueira CB,
Freitas MR
• UFF
Introduction: Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder
first described by Hinchey et al, in 1996 that normally presents with headaches, seizures, altered
sensorium, and loss of vision. Additionally, PRES
is also accompanied by characteristic, potentially reversible, computadorized tomography (CT)
and magnetic resonance image (MRI) findings.
Typically it reveals edema predominantly affecting white matter within the parietooccipital
regions of the brain. Objective: To report a case
of a patient with lupus nephritis, hypertension and poorly controlled chronic renal failure
who developed PRES after a week of using rituximab Case report: A 27-year-old Caucasian
woman, diagnosed six years prior with SLE who
developed severe lupus nephritis, for which hydroxychloroquine, prednisone, azathioprine
and cyclophosphamide had all been prescribed
without improvement. Pulse therapy of 1000 mg
rituximab in association with mycophenolate
mofetil maintenance was tried, and showed excellent clinical response. Seven days after second
rituximab dose the patient developed disorientation, seizures, nausea, tetraparesis, hypereflexia,
and blurred vision. A cranial CT scan revealed
bilateral occipital hypodensity , while brain MRI
using flair-weighted imaging showed bilateral
hyperintensity at the same location . A diagnosis of PRES was made and treatment started with
clinical support, rigorous blood pressure control
and rituximab discontinuation. The patient had
early clinical improvement after 10 days and
complete resolution after 14 days. Discussion:
Several drugs, some of which are immunosuppressants, have been implicated in causing this
syndrome. Reports of rituximab causing PRES
are still rare and poorly described. This is the fifth
PRES Case reported occurring after rituximab infusion and the second case after rituximab on a
hypertensive and uremic SLE patient. Know the
adverse effects of new drugs, such as rituximab,
or any medical conditions that can be PRES related is of great importance. The typical clinical presentation of acute multifocal neurologic
deficit may erroneous lead physicians to request
unnecessary tests as PRES prognosis is benign in
most cases. Predisposing factors identification is
also relevant, since treatment includes removal
of PRES triggers, if possible. Furthermore, although recurrence is rare2, clinicians may want
to avoid medication previously associated with
PRES in a particular patient.
P-366
POSTERIOR REVERSIBLE ENCEPHALOPATHY
SYNDROME: A CASE REPORT
Meira FCA, Gomez RS, Pacheco JAS, Costa AAS,
Silva RA, Rodrigues FLB, Santos TM, - DVESL
• HOSPITAL MADRE TERESA
Introduction: Posterior reversible encephalopathy syndrome (PRES) consists of clinical and radiological alterations that are usually reversible.
Most common causes include exposure to toxic
agents and hypertension. Pathogenesis is controversial. Clinical presentation varies from seizures
to visual alterations. Magnetic resonance imaging (MRI) is essential to diagnosis. Objectives: To
report a case of a patient admitted with PRES at
Madre Teresa Hospital (HMT) and discuss differential diagnosis. Case report MCCL, 76 years-old
female, previously diagnosed with hypertension and glaucoma, was admitted at HMT with
48 hours of diplopia and labile blood pressure.
Neurologic examination showed pupil asymmetry (3+/2+), diplopia at horizontal gaze and mild
left ptosis. No alterations were found in the head
computed tomography, hence, the patient was
submitted to brain MRI, which evidenced T2 hyperintense signal at the periaqueductal region,
as well as at the dorsal mesencephalon. On the
following morning, the patient presented severe
tachypnea, miosis and ptosis. She was intubated
and admitted to the ICU. The patient presented
fever, seizures, depressed level of consciousness
and loss of brainstem reflexes in a rostrocaudal
manner. The possibility of rhombencephalitis
of undefined etiology in a patient with a neurologically severe condition was considered. Methylpredinisolone and a wide antibiotic therapy
(antivirus and antibiotics) were started. Cerebral
spinal fluid analysis (including several viral PCRs)
showed no alterations. EEG showed bursts of
rhythmic delta waves. Control brain MRI showed
T2 hyperintense lesion on the dorsal portion of
the brainstem (mesencephalon to medulla), and
acute bilateral ischemia in the occipital region.
After 37 days in the ICU, the patient started neurological recovery. She was discharged from the
hospital with right homonymous hemianopia,
bilateral ptosis, left peripheral facial palsy and
sensorineural hearing loss. Consciousness and
cognition were preserved. Discussion: Clinical
and radiological diversity increases the challenge that represents diagnosing PRES. Several
conditions may mimic this syndrome, such as
rhombencephalitis and stroke. Diagnosis must
be based on history, physical examination and
neuroimaging. Lumbar puncture, specially when
central nervous system infection is suspected,
and EEG must be performed. The diversity of the
differential diagnosis must be considered for the
management of PRES.
P-367
RADIOLOGICAL BRAIN CHANGES ASSOCIATED
WITH VITAMIN B12 DEFICIENCY
Reges DS, Sanvito WL, Oliveira FTM, Pagiola IC,
Zetehaku AC, Fortes ALVS, Luca NC, Melges NS,
Gagliardi VDB, Figueiredo JFV, Mendes MF
• IRMANDADE SANTA CASA DE SÃO PAULO
Introduction: Vitamin B12 deficiency can produce neurological, psychiatric and hematologic
disorders. With regard to the nervous system,
the disease mainly affects the myelin sheath,
with diffuse demyelination of posterior and lateral spinal columns. Lesions with same characteristics can also be highlighted in the brain.
Objectives: To describe two cases of vitamin B12
deficiency with radiological changes in the brain.
Case report: MJGM, 38 years old, female, with
migrane type headache associated with mental
confusion and right motor deficit that remitted
spontaneously within a week, staying with persistent daily headache. MJF, 33 years old, female,
with progressive installation of crural paraparesia over one year and with worsening in one
month pior to admission at the Neurology service, requiring a wheelchair for mobility. Associated with the motor deficit, the patient presented
with urinary retention, hypoesthesia with sensorial level in T4 and memory deficit. In both cases,
research was conducted for collagen diseases,
infectious diseases and demyelinating diseases
like multiple sclerosis, which were excluded. CSF
analysis of both were without changes. Dosage
of vitamin B12 in these patients was below the
reference value and high digestive endoscopy
showed atrophic gastritis. Brain MRI of the patients concerned revealed signal changes in the
periventricular white matter and semiovais centres with confluent aspect with characteristics of
metabolic impairment of hemispherical white
substance and cerebellar cortex. Discussion: in
the early stages of the disease occurs swelling
of the myelin sheath, usually beginning in the
mid-thoracic level. With the course of the deficiency there is degeneration of axons of both ascendant (posterior bundle) and pyramidal tracts.
Intracranial lesions in the topography of the
pyramid bundles are explained by the phenomenon of retrograde axonal degeneration related
to the demyelinating lesion, resembling multiple sclerosis lesions and being clinically related
to the associated neuro-psychiatric syndrome.
Conclusion: the intracranial lesions of B12 hypovitaminosis may enter in the differential diagnosis of neuropsychiatric disorders associated with
hyperintensity on T2 signal in the white matter.
121
Posters
P-368
REGIONAL ACTIVATION-DEACTIVATION PATTERNS
IN FMRI LANGUAGE TASKS
Castro LHM1, Almeida JP1, Castro BPSM1, Sato JR2,
Jorge CL1, Valerio RMF1, Listik C1, Chaim KT1,
Arantes PR1, Jr EA1
• 1FMUSP; 2UNIVERSIDADE FEDERAL DO ABC
Background: Language subsystems are organized in neural networks. fMRI language paradigms should evaluate individual hemisphere
contribution in each task. We employed three
fMRI language tasks to determine regional activation and lateralization patterns. Methods: 22
right-handed healthy volunteers, native Portuguese speakers, aged between 19‐52 years, ≥8 education years, IQ>70, without neuropsychiatric
disease, underwent three language fMRI paradigms: word generation (WG), visual confrontation naming (VCN) and reading responsive
naming (RRN). Individual activation maps were
obtained with general linear model, nonparametric inference p<0,05, fMRI Expert Analysis
to generate z statistic images (cluster cut-offs
z>2.3 and cluster significance p=0.05). Nine language ROIs were defined for each hemisphere on
aparc.a2009: inferior frontal(IFG), middle frontal(MFG), superior frontal(SFG), inferior temporal(ITG), middle temporal(MTG), superior temporal(STG), angular(AG), supramarginal(SMG],
and occipital-temporal lateral/fusiform(OTG)
gyri. We assessed language lateralization index
(LI) in each ROI with: LI=(left-right activated voxels/(left+right activated voxels). To explore each
hemisphere contribution to LI, we evaluated Percent Signal Changes (PSC) from baseline in each
ROI using FSL featquery tool. LI and PSC values
were compared in the nine ROIs, with non-parametric analysis (p=0.05 level). Results: Mean LI
values were above 0.2, for in all ROIs and paradigms, indicating left (L) lateralization. For VCN,
greater activations were noted in the LIFG, LITG
and bilateral (B) OTG, with deactivations in all
right (R) FG and RSTG, RMTG, BAG, BSMG, and
the LSTG. For WG, greater activations were seen
in the LIFG, LMFG, LSFG, LITG, LAG, BTOG and
RMFG, and deactivations in RMTG and RSMG.
For RRN, greater activations were observed in all
LFG and LTG, LAG, and BTOG, and deactivations
in all RFG, RMTG and RSTG, RSMG and RAG.
Conclusion: Although all paradigms presented strong left lateralization, we found distinct
regional activation-deactivation patterns for
different tasks: WG showed prominent bifrontal
and left temporal-parietal activation with right
temporal-parietal deactivation, RRN showed
extensive left hemisphere activation and right
hemisphere deactivation, while CN showed predominately posterior activation with extensive
right hemisphere deactivation. Disease states
affecting language laterality and reorganization
may affect differently the studied networks.
P-371
SUPERFICIAL SIDEROSIS SECONDARY TO
VENOUS THROMBOSIS OF SIGMOID SINUS IN
ASYMPTOMATIC PATIENT: IMAGING FINDINGS
Souza TS, Carvalho AGG, Castro LM, Martins WRM,
Santos RHA, Pires LA, Marques HC
• UFJF
Introduction: Superficial Siderosis (SS) is a rare
disease caused by deposition of free iron and hemosiderin in the superficial and subpial layers,
122
resulting in a destruction and demyelination of
Central Nervous System (CNS). The average age
is between fifties and sixties and predominates in
males (2:1). Most cases are idiopathic and may be
secondary to chronic subarachnoid hemorrhage,
CNS tumors, arteriovenous malformations and
trauma. The progression of symptoms is slow
from years to decades after the initial bleeding.
It is characterized by the classic triad: progressive sensorineural deafness, cerebellar ataxia
and myelopathy, occasionally diagnosed by
routine findings of magnetic resonance imaging
(MRI) in asymptomatic patients. Objective: to
report a case of subclinical SS through specific
imaging finding. Case report: female, 83-year
old, hypertense and diabetic, using valsartan,
amlodipine, chlorthalidone, atenolol and metformin, she came to us complaining of pain
in the left hemibody. MRI of the brain revealed
hyposignal at leptomeningeal of left perisylvian
region on T2-weighted and gradient-echo with
loss of the usual flow-void the left sigmoid sinus.
Discussion: SS being a progressive neurological
condition should be recognized early. Due to the
possibility of progression to clinical manifestation, in the case of the patient we opted for an
outpatient. Even in subclinical cases, brain of
MRI is the main screening exam to confirm the
diagnosis of SS. Hyposignal of leptomeningeal
due to hemosiderin deposition are pathognomonic findings seen in a sequence gradient-echo
T2-weighted. In clinical practice, it is emphasized the importance to suspect of SS of CNS,
avoiding diagnosis and treatment delays, when
it is possible.
P-372
USE OF IMAGING Methods: IN DIAGNOSIS OF
DEMENTIA: SYSTEMATIC REVIEW
Kroger JS, Silva LSA, Paranhos DR, Guimarães VP,
Lopes RN, Coimbra LL
• EBMSP
Introduction: As a result to the increasing global
population aging, prevalence of neurodegenerative diseases is becoming higher every year. Dementias are neurodegenerative diseases of high
prevalence in old population, what cause many
nuisances for these people. However, there are
still no drugs able to prevent disease progression, and there are few biomarkers in imaging
Methods: that can detect early signs of neurodegenaration and differentiate between types
of dementia. Both things would be essencial
for early diagnosis and revertion of the cognitive impairment. Thus, as important as investing in studies for developing modifying drugs,
is investing on image methods, which could
detect mild cognitive impairment. Objective:
Review current literature about the use of imaging Methods: in the diagnosis of dementia.
Methods: Electronic databases were consulted
(Medline, IBECS, LILACS), from the year 2010,
whith the following keywords: “dementia”, “diagnosis” and “imaging”. Where selected articles in English, Spanish and Portuguese whose
subject was dementia and clinical aspect the
diagnosis. Results: Imaging Methods: currently
does not contribute too much for the diagnosis
and differentiation between dementia types.
That’s because changes on imaging recognized
as biomarkers (such as hippocampal atrophy on
Alzheimer disease and gray matter hyperintensities in cerebrovascular dementia) occur only
in advanced stages of neurodegeneration. Furthermore, these Methods: are not very specific,
then the diagnosis of dementias types depends
on pathological examination. It is known that
neurodegeneration precedes in years to clinical
onset of dementia. Studies have been performed
showing relationship between findings in several
imaging methods, including RNMf, PET, SPECT,
with mild cognitive impairment and early neurodegeneration. However, it’s necessary to validate
those Methods: and its protocols as biomarkers
for cognitive impairment. Conclusion: Early diagnosis for the various types of dementia could
result in treatment from an early stage of the disease, retarding its advance and preventing cognitive decline. If there where modifying drugs for
dementia, imaging Methods: capable to detect
early biomarkers would be critical in the diagnosis and treatment of these diseases, promoting
better prognosis for patients. Keywords: dementia, diagnosis, imaging methods.
P-374
WHITE MATTER HYPERINTENSITIES IN
FRONTOTEMPORAL DEMENTIA-AMYOTROPHIC
LATERAL SCLEROSIS
Heringer ÉM, Lacerda PE, Teixeira FHS, Martins WRM,
Vale TC, Marques HC, Pires LA
• UFJF
Introduction: Frontotemporal dementia (FTD)
is a complex disease characterized by a wide
range of clinical and pathological manifestations, hence the relationship with amyotrophic
lateral sclerosis (ALS), increasingly recognized
today. In FTD-ALS complex, brain magnetic resonance imaging (MRI) shows degeneration of
corticospinal tracts, inferior longitudinal fasciculus and anterior corpus callosum, as well as atrophy of frontal and temporal lobes. Objectives:
To report a peculiar neuroimaging finding in a
FTD-ALS case. Case report: A 56-year-old male
started a three-year history of progressive behavioral changes with psychomotor agitation,
disinhibition, impulsivity, craving for food, exploratory behavior and echolalia that impaired
daily functional activities. Mini-Mental State
Examination was of 17/30 (four years of schooling). After two years, he presented with fasciculations in the upper limbs, tongue and chest.
Physical examination showed muscle atrophy,
asymmetric brachial paraparesis, global hyperreflexia with Babinski signs and bilateral grasping.
Electromyography (EMG) was abnormal, with
findings suggestive of anterior horn of the spinal
cord involvement. Brain MRI showed frontotemporal atrophy and did not detect degeneration
of corticospinal tracts in the T1-weighted spinecho/magnetization contrast-enhanced transfer (MTC) sequence. However, this sequence
showed frontal and temporal hyperintensities.
Discussion: Brain MRI has great utility in FTD by
demonstrating asymmetric frontal and temporal atrophy. In FTD-ALS complex, brain MRI can
assist by using the MTC sequence to show signal
hyperintensity of the corticospinal tract. Our patient presented a clinical diagnosis of FTD-ALS
complex and underwent brain MRI with MTC
sequence aiming to detect corticospinal tract degeneration. However, MTC sequence showed significant change in the white matter of the frontal
and temporal lobes, a finding not yet described
in the literature that needs further confirmation
and that might help physicians in the diagnosis
of FTD-ALS complex cases.
XXVI Congresso Brasileiro de Neurologia
Interventional Neurology
P-375
CEREBELLAR HEMANGIOBLASTOMA AND
LYMPHOID ACUTE LEUKEMIA: A CASUAL
ASSOCIATION OR GENETICALLY DETERMINED?
Pessoa BL, Araujo LM, Landeiro JA, Andrade MC,
Nascimento OJM, Dornas RM
• UFF
Introduction: Hemangioblastomas are benign
tumors with histopathological features well defined, which arise from the disorderly growth of
stromal and vascular cells. Despite some controversy, are described by the WHO as being of meningeal origin. Represent 1.5‐3% of CNS tumors
and can occur sporadically or in association with
von Hippel Lindau disease. The hemangioblasts
are precursor cells that can originate to hematopoietic and endothelial cells. Objective: Reporting of a rare combination of an isolated cerebellar hemangioblastoma and acute lymphoblastic
leukemia (ALL) and literature review. Casuistry
and methods: 16 years old patient with cerebellar expansive process, submitted to tumor resection with histopathological diagnosis of hemangioblastoma. Noteworthy, in the past medical
history, was the reporting of ALL in the second
year of life. We conducted a comprehensive research of the PubMed and MEDLINE databases
to evaluate this association. Results: Despite
some articles correlate hematologic disorders
with vascular origin tumors, to the best of our
knowledge no cases of ALL and hemangioblastoma was found so far. Conclusions: Hemangioblastomas are rare CNS tumors and association
with ALL was first described. Hypothetically, the
association between the two neoplasms can be
justified by the origin in the same precursor cell the hemangioblast.
P-376
EARLY CALCIFIED EPIDURAL HEMATOMA IN A
YOUNG MAN (CASE REPORT)
Oliveira Júnior JP1, Cruz PP2, Silveira RLP3,
Almeida JMD1, Santos Júnior JM4, Santana PMR5
• 1UFBA; 2UNEB; 3FTC; 4EBMSP; 5HGRS
Introduction: Epidural hematoma (EDH) is considered a rare event, especially when calcified.
The HED is considered an acute neurosurgical
emergency between 6 to 12 hours after trauma.
However, some cases of late outcome have been
reported. Case report: Boy,17 years old, black,
resident in Bahia, victim of fall at high speed in
motorcycle accident with right frontal cranial
injury. Not wearing safety items (helmet). Previously healthy, he denies seizures, smoking, illicit
drugs use or previous brain trauma injury. After
the accident, he was drowsiness, but he does not
have signs of disorientation or decrease in the
level of consciousness. Holocranial headache
immediately after the trauma, which remained
until the surgery. The patient was admitted on
the hospital emergency in good general condition, GCS 15, isochoric pupils and light reaction,
without neurological deficits, despite holocranial headache. Head Computed Tomography of
the skull without venous contrast, which demonstrated massive right frontal epidural collection
with signs of convex calcification in brain without midline deviation and it does not effacement of cortical sulcus or cisterna. Preoperative
laboratory tests were ordered: phosphorus 3.9
mg/dL; total calcium 10.2 mg/dL; total protein
8.3 g/L; albumin 5 g/L; prothrombin time 86%;
RNI 1.07; APTT 31.6 seconds; Hb 14.7 g/dL; Leukocytes 6820 cells/mm3; Platelets 236.000 cells/
mm3. After eight days of the accident, the patient
underwent the drainage procedure of hematoma
and frontotemporal craniotomy with removal
the convex calcification and bone replacement.
The histopathologic study demonstrated hemosiderin intercalated with islands of calcification.
Therefore, the suspected of meningioma was
ruled out. The patient remained asymptomatic
in the ward and was discharged with instructions
to use phenytoin at home and ambulatory monitoring. Conclusion: There is not consensus in the
literature about the temporal definition of EDH.
Some authors consider those with chronic EDH
with over 48h of evolution, while others authors
consider those with more than 13 days of evolution. The chronic EDH affects young people under 40 years. However, there are few Case reports
of people so young and with rapid calcification
of EDH in literature, mainly with the total serum
calcium was on the limit of the reference value
and normal serum phosphate.
P-377
ENDOVASCULAR TREATMENT OF SYMPTOMATIC
INTRACRANIAL ARTERIAL STENOSIS
Araújo MLB1, Vale BP2, Rodrigues AB1,
Gonçalves Júnior PCJ1
• 1UNIVERSIDADE FEDERAL DO PIAUÍ; 2INSTITUTO DE
NEUROCIÊNCIAS, TERESINA
Introduction: Intracranial stenosis (ICS) is one
of the major causes of ischaemic stroke worldwilde. Almost 100.000 new cases of stroke in
United States each year are due to ICS and the
risk of reccurrence in these patients is almost
20%. The best option for treatment remains uncertan. Endovascular therapy with angioplasty
and stenting has emerged as an important option of treatment in the last years, specially in
refractory cases to optimized clinical treatment.
Objectives: Investigate the long-term outcome
in patients undergoing to endovascular therapy
of symptomatic ICS and correlate the presence of
ICS with risk factors for cerebrovascular disease.
Methods: Retrospective selection of consecutive
patients undergoing to endovascular treatment
of symptomatic ICS. Digital cerebral angiography was performed in all patients. The clinical
outcome was evaluated by a Neurologist applying the Rankin modified scale (Rms) and the
presence of a new cerebrovascular event (stroke
or transient ischaemic attack). Medical records
were reviewed for investigation of risk factors.
Results: Seven patients were enrolled for this
study, all male. Of these, one was excluded due
to loss of follow-up. Good clinical outcome (Rms
0 - 2) was observed in 83% of patients. Arterial
hypertension and dyslipidemia were the risk factors for cerebrovascular disease most prevalent,
observed in all patients. Smoking was present in
only two patients. Conclusion: Intracranial atherosclerosis with stenosis is an important cause
of recurrent stroke. Aggressive treatment of risk
factors (specially arterial hypertension and dyslipidemia) is strongly indicated. Endovascular
treatment appears as an importante and promissal option in this cases.
P-378
HEMIPARESIS IPSILATERAL ISCHEMIC EVENT DUE
TO NOT DECUSSATION PYRAMID
Gagliardi RJ, Simis M, Cerqueira LG, Fortes ALVS,
Oliveira FTM, Luca NC, Gagliardi VDB, Melges NS,
Reges DS, Zetehaku AC, Pagiola IC, Sanvito WL
• IRMANDADE DA SANTA CASA DE SP
Introduction: The occurrence of ipsilateral
hemiparesis in an ischemic event is rare. It is
known that 70‐90% of the fibers of the corticospinal tract decussate before reaching the cord
to innervate the contralateral side. There are
rare Case reports that suggest the existence of
non-crossed tracts. This finding may be nonspecific or related to malformations. A review of
2013 analyzed 10 cases of ipsilateral hemiparesis
secondary to vascular event, the most common
cause ipsilateral projection of the primary motor
cortex. Objective: To discuss a case of ipsilateral
hemiparesis after ischemic event. Case report:
Female patient, 43 years without comorbidities,
sought PS-ISCMSP on 07/08/2014 with acute
dysarthria and left hemiparesis complete disproportionate predominantly crural- NIHSS
=5, associated with headache and nausea. Held
tomography that showed an acute ischemic lesion on the left and angiography of intracranial
vessels showed no areas with significant stenoses. Normal electrocardiogram, laboratory tests
without biochemical changes, HIV, hepatitis B
and C and VDRL negative, and normal rheumatologic tests. MRI of the brain with diffusion
in the acute phase showed changes suggestive
sign of subacute ischemic lesion in the territory
of the left middle cerebral artery, and tractography study showed that subacute ischemic injury
was affecting cortico-spinal tract to the left, but
it had no decussation, or the level of the medullary pyramid. Echocardiogram with ejection
fraction 63% and absence of intracardiac shunts
on microbubble test were also performed; and
Doppler ultrasonography of the cervical arteries
without changes. Discussion: Our case reflects
one of the rare occasions when there was ipsilateral hemiparesis in vascular injury. Data from the
literature review suggest that ipsilateral hemiparesis is associated with three distinct situations,
which should be investigated in these patients.
Congenital malformations (such as malformations of the posterior fossa, occipital encephalocele, Dandy-Walker malformation, Joubert
From Sd, or malformations extensive trunk like
Mobius sd); cortical reorganization (especially in
patients with previous vascular insults); and no
decussation of the cortico-spinal tract pure, as
the case presented.
P-380
MECHANICAL THROMBECTOMY: ACUTE ISCHEMIC
STROKE IN RIVAROXABAN USER
Narimatsu K, Araújo MF, Pedroso JL, Protti GG,
Morgulis RF
• HOSPITAL ISRAELITA ALBERT EISNTEIN - HIAE
Introduction: The novel oral anticoagulant
(NOAC) agents have been approved for prevention of stroke in patients with atrial fibrillation.
Clinical trials of the NOACs suggest that, each
year, approximately 1.0%-2.0% of individuals
with AF who are receiving one of these agents
can be expected to experience an acute ischemic
stroke (AIS). However, laboratory testing traditional for coagulation are not adequate to assess the coagulation status and risk of bleeding.
Currently, endovascular therapy encompasses
a range of approaches, including endovascular
123
Posters
pharmacological thrombolysis, mechanical and
aspiration thrombectomy, are alternative strategies. Objetive: We report a patient who was
treated by endovascular thrombectomy who
suffered from acute ischemic stroke under treatment with NOAC. Case: A 80-year-old man had
a medical history with atrial fibrillation and was
then administered 10 mg of rivaroxaban daily.
Although she took rivaroxaban in the morning,
ischemic stroke occurred at 10 pm of that day.
Soon after transferring to our hospital at 4h and
NIHSS: 16. His coagulation parameters were normal. We confirmed right middle cerebral artery
(MCA) occlusion in the patient and endovascular thrombectomy was performed. The patient
showed recanalization of the MCA neurological improvement, NIHSS: 5 and minimal motor
weakness. Discussion: Patients who experience
AIS should be considered for urgent thrombolytic therapy to restore perfusion and function
of the ischemic brain. However, effective anticoagulation present at the time of reperfusion is
a contraindication for thrombolysis because of
the possibility of increased risk of symptomatic
hemorrhage. However, international normalized
ratio or prothrombin time are not adequate to
assess the coagulation status and risk of bleeding in patients who are on a NOAC. Thrombolysis
should only be initiated in patients receiving one
of the NOACs if the clinical history and a laboratory test reliably suggest the absence of an anticoagulant effect, or until at least two half-lives
have elapsed since the most recent dose of the
NOAC (in patients with normal renal function).
This case, mechanical thrombectomy alone was
the treatment of choice because rivaroxaban use.
Alternative therapy was successful reperfusion
and endovascular thrombectomy might be safe
for patients treated with the new anticoagulant
rivaroxaban.
P-381
SPONTANEOUS DISSECTION OF INTERNAL
CAROTID ARTERY ASSOCIATED WITH TRANSIENT
ISCHEMIC ATTACK: CASE REPORT AND
THERAPEUTIC ASPECTS
Rocha MMSD1, Rodrigues ÂB1, Marques Júnior MASS2,
Sousa CM3, Gonçalves Júnior PCJ1, Vale BP4
• 1UNIVERSIDADE FEDERAL DO PIAUÍ; 2UNINOVAFAPI;
3
FACID; 4INSTITUTO DE NEUROCIÊNCIAS
Introduction: Arterial dissections are ruptures of
the vessel wall which frequently attack the medium layer. The dissections of the carotid artery(a.)
may be considered as a possible etiology of brain
stroke and Transient Ischemic Attack(TIA) in
young and middle-aged patients and generally
have a good prognosis. The Computerized Tomography-Angiography is an alternative or complementary diagnostic technique used for evaluation of dissection. Goals: To report a case of dissection in the right carotid artery associated with
TIA episode. Case report: Male patient, 51 years
old, was admitted in the emergency department
reporting a TIA history, in the presence of hemiplegia to the left with duration of 10 minutes, 4
hours before. During the TIA, the patient was
treated with Acetylsalicylic Acid and Clopidogrel.
At neurological admission, he was lucid, conscious, with no motor impairment, with cervical
murmur to the right, NIH Stroke Scale (NIHSS)=0
and Glasgow coma scale(GCS)=15. The patient
was submitted to Computerized Tomography
and Computerized Tomography-Angiography.
After 8 hours, his condition worsened with the
resurgence of hemiplegia to the left, NIHSS=16
and (GCS)=9. As treatment, the Angioplasty was
124
adopted for the implantation of two stents in the
dissected segment of the right internal carotid a.
After the procedure, the patient presented a satisfactory progress, with reversal of the lowering
of the level of consciousness and hemiplegia,
with muscle strength grade III after 7 hours of
the procedure. After 2 days, it was performed the
Transcranial Doppler, with the results: stenosis
of the middle cerebral a. to the right; absence of
pathological findings in distal right cervical internal carotid a.; and absence of vasospasm or
intracranial hypertension. Discussion: There is
no consensus on the treatment of dissections. In
most cases, the dissected arteries(aa.) re-channel
and heal completely few months after the initial
event. However, in some patients, the rechanneling does not happen and the aa. present with severe stenosis. In these cases, the treatment with
anticoagulant prevents the occurrence of new
thromboembolic events, but it does not prevent
hemodynamic events associated with reduced
cerebral blood flow, such as TIA. Considering the
advantages as well as an increase of the risks associated with the open surgery, the angioplasty
that was mentioned above associated with stent
implantation was the method chosen to treat the
patient.
P-382
THE CRANIOPLASTY AS A DRIVER OF IMPROVED
SELF-ESTEEM, COGNITION AND QUALITY OF LIFE
IN PATIENTS WITH CEREBRAL TRAUMA SKULL
Andrade AC, Martins HO, Matos CS, Matos HMC,
Silva MCB