Dandy-Walker syndrome in adult mimicking myasthenia gravis

Dandy-Walker syndrome in adult mimicking myasthenia gravis

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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Main Authors: Cardoso,Juliana, Lange,Marcos C., Lorenzoni,Paulo J., Scola,Rosana H., Werneck,Lineu C.
Format: Digital revista
Language:English
Published: Academia Brasileira de Neurologia - ABNEURO 2007
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2007000100037
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spelling oai:scielo:S0004-282X20070001000372007-03-23Dandy-Walker syndrome in adult mimicking myasthenia gravisCardoso,JulianaLange,Marcos C.Lorenzoni,Paulo J.Scola,Rosana H.Werneck,Lineu C. Dandy-Walker syndrome hydrocephalus myasthenia gravis palpebral ptosis 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.info:eu-repo/semantics/openAccessAcademia Brasileira de Neurologia - ABNEUROArquivos de Neuro-Psiquiatria v.65 n.1 20072007-03-01info:eu-repo/semantics/reporttext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2007000100037en10.1590/S0004-282X2007000100037
institution SCIELO
collection OJS
country Brasil
countrycode BR
component Revista
access En linea
databasecode rev-scielo-br
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region America del Sur
libraryname SciELO
language English
format Digital
author Cardoso,Juliana
Lange,Marcos C.
Lorenzoni,Paulo J.
Scola,Rosana H.
Werneck,Lineu C.
spellingShingle Cardoso,Juliana
Lange,Marcos C.
Lorenzoni,Paulo J.
Scola,Rosana H.
Werneck,Lineu C.
Dandy-Walker syndrome in adult mimicking myasthenia gravis
author_facet Cardoso,Juliana
Lange,Marcos C.
Lorenzoni,Paulo J.
Scola,Rosana H.
Werneck,Lineu C.
author_sort Cardoso,Juliana
title Dandy-Walker syndrome in adult mimicking myasthenia gravis
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
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.
publisher Academia Brasileira de Neurologia - ABNEURO
publishDate 2007
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2007000100037
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AT lorenzonipauloj dandywalkersyndromeinadultmimickingmyastheniagravis
AT scolarosanah dandywalkersyndromeinadultmimickingmyastheniagravis
AT wernecklineuc dandywalkersyndromeinadultmimickingmyastheniagravis
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