Sturge Weber Syndrome--unusual presentation. .

نویسنده

  • N S Neki
چکیده

Sturge Weber Syndrome or encephalo-trigeminal angiomatosis is non-hereditary, congenital and rare disorder of unknown aetiology. It is characterised by vascular malformation with capillary venous angiomas involving face, eye and leptomeninges resulting in neurological and orbital manifestations. A case of 23 years old female presented with history of tonic-clonic convulsions, evidence of Port wine stain on face since birth, characteristic CT findings diagnosed as a case of Sturge Weber Syndrome is reported here for its rarity.

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Background: Sturge-Weber syndrome is one of the rare neurocutaneous disorders with frequency of approximately 1 per 50,000. Sturge-Weber syndrome consists of a constellation of symptoms and signs including a facial nevus (port wine stain), seizure and hemiparesis. In many cases it may associate with mental retardation. Case presentation: A 7- year old girl with mental retardation and a large fa...

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عنوان ژورنال:
  • Journal of Ayub Medical College, Abbottabad : JAMC

دوره 26 1  شماره 

صفحات  -

تاریخ انتشار 2014