Dandy-Walker syndrome in adult mimicking myasthenia gravis
Autor(a) principal: | |
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Data de Publicação: | 2007 |
Outros Autores: | , , , |
Tipo de documento: | Relatório |
Idioma: | eng |
Título da fonte: | Arquivos de neuro-psiquiatria (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2007000100037 |
Resumo: | The Dandy-Walker syndrome (DWS) is a rare posterior fossa malformation. The DWS can occur associated with other brain or systemic malformations, but ocular abnormalities in this disease are rare and clinical findings mimicking myasthenia gravis have not been described to date. We report a 23-year-old woman who presented mild limitation of the ocular movements with progressive palpebral ptosis, which changed in intensity during the day. The investigation showed negative anti-acetylcholine receptor antibody, repetitive nerve stimulation and "Tensilon test", but the brain magnetic resonance image reveals DWS with hydrocephalus associated with calosal dysgenesis. The characteristic of disease, clinical manifestations and pathologic features, specially the clinical evaluation of ocular abnormalities in suspicion of DWS, including the MG in differential diagnosis are discussed. |
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Dandy-Walker syndrome in adult mimicking myasthenia gravisDandy-Walker syndromehydrocephalusmyasthenia gravispalpebral ptosisThe Dandy-Walker syndrome (DWS) is a rare posterior fossa malformation. The DWS can occur associated with other brain or systemic malformations, but ocular abnormalities in this disease are rare and clinical findings mimicking myasthenia gravis have not been described to date. We report a 23-year-old woman who presented mild limitation of the ocular movements with progressive palpebral ptosis, which changed in intensity during the day. The investigation showed negative anti-acetylcholine receptor antibody, repetitive nerve stimulation and "Tensilon test", but the brain magnetic resonance image reveals DWS with hydrocephalus associated with calosal dysgenesis. The characteristic of disease, clinical manifestations and pathologic features, specially the clinical evaluation of ocular abnormalities in suspicion of DWS, including the MG in differential diagnosis are discussed.Academia Brasileira de Neurologia - ABNEURO2007-03-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2007000100037Arquivos de Neuro-Psiquiatria v.65 n.1 2007reponame:Arquivos de neuro-psiquiatria (Online)instname:Academia Brasileira de Neurologiainstacron:ABNEURO10.1590/S0004-282X2007000100037info:eu-repo/semantics/openAccessCardoso,JulianaLange,Marcos C.Lorenzoni,Paulo J.Scola,Rosana H.Werneck,Lineu C.eng2007-03-23T00:00:00Zoai:scielo:S0004-282X2007000100037Revistahttp://www.scielo.br/anphttps://old.scielo.br/oai/scielo-oai.php||revista.arquivos@abneuro.org1678-42270004-282Xopendoar:2007-03-23T00:00Arquivos de neuro-psiquiatria (Online) - Academia Brasileira de Neurologiafalse |
dc.title.none.fl_str_mv |
Dandy-Walker syndrome in adult mimicking myasthenia gravis |
title |
Dandy-Walker syndrome in adult mimicking myasthenia gravis |
spellingShingle |
Dandy-Walker syndrome in adult mimicking myasthenia gravis Cardoso,Juliana Dandy-Walker syndrome hydrocephalus myasthenia gravis palpebral ptosis |
title_short |
Dandy-Walker syndrome in adult mimicking myasthenia gravis |
title_full |
Dandy-Walker syndrome in adult mimicking myasthenia gravis |
title_fullStr |
Dandy-Walker syndrome in adult mimicking myasthenia gravis |
title_full_unstemmed |
Dandy-Walker syndrome in adult mimicking myasthenia gravis |
title_sort |
Dandy-Walker syndrome in adult mimicking myasthenia gravis |
author |
Cardoso,Juliana |
author_facet |
Cardoso,Juliana Lange,Marcos C. Lorenzoni,Paulo J. Scola,Rosana H. Werneck,Lineu C. |
author_role |
author |
author2 |
Lange,Marcos C. Lorenzoni,Paulo J. Scola,Rosana H. Werneck,Lineu C. |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Cardoso,Juliana Lange,Marcos C. Lorenzoni,Paulo J. Scola,Rosana H. Werneck,Lineu C. |
dc.subject.por.fl_str_mv |
Dandy-Walker syndrome hydrocephalus myasthenia gravis palpebral ptosis |
topic |
Dandy-Walker syndrome hydrocephalus myasthenia gravis palpebral ptosis |
description |
The Dandy-Walker syndrome (DWS) is a rare posterior fossa malformation. The DWS can occur associated with other brain or systemic malformations, but ocular abnormalities in this disease are rare and clinical findings mimicking myasthenia gravis have not been described to date. We report a 23-year-old woman who presented mild limitation of the ocular movements with progressive palpebral ptosis, which changed in intensity during the day. The investigation showed negative anti-acetylcholine receptor antibody, repetitive nerve stimulation and "Tensilon test", but the brain magnetic resonance image reveals DWS with hydrocephalus associated with calosal dysgenesis. The characteristic of disease, clinical manifestations and pathologic features, specially the clinical evaluation of ocular abnormalities in suspicion of DWS, including the MG in differential diagnosis are discussed. |
publishDate |
2007 |
dc.date.none.fl_str_mv |
2007-03-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/report |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
report |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2007000100037 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2007000100037 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/S0004-282X2007000100037 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Academia Brasileira de Neurologia - ABNEURO |
publisher.none.fl_str_mv |
Academia Brasileira de Neurologia - ABNEURO |
dc.source.none.fl_str_mv |
Arquivos de Neuro-Psiquiatria v.65 n.1 2007 reponame:Arquivos de neuro-psiquiatria (Online) instname:Academia Brasileira de Neurologia instacron:ABNEURO |
instname_str |
Academia Brasileira de Neurologia |
instacron_str |
ABNEURO |
institution |
ABNEURO |
reponame_str |
Arquivos de neuro-psiquiatria (Online) |
collection |
Arquivos de neuro-psiquiatria (Online) |
repository.name.fl_str_mv |
Arquivos de neuro-psiquiatria (Online) - Academia Brasileira de Neurologia |
repository.mail.fl_str_mv |
||revista.arquivos@abneuro.org |
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1754212761388187648 |