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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Academia Brasileira de Neurologia - ABNEURO
2007
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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 |
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Cardoso,Juliana Lange,Marcos C. Lorenzoni,Paulo J. Scola,Rosana H. Werneck,Lineu C. |
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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 |
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http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2007000100037 |
work_keys_str_mv |
AT cardosojuliana dandywalkersyndromeinadultmimickingmyastheniagravis AT langemarcosc dandywalkersyndromeinadultmimickingmyastheniagravis AT lorenzonipauloj dandywalkersyndromeinadultmimickingmyastheniagravis AT scolarosanah dandywalkersyndromeinadultmimickingmyastheniagravis AT wernecklineuc dandywalkersyndromeinadultmimickingmyastheniagravis |
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1756374363391655936 |