Presurgical evaluation and cognitive functional reorganization in Fishman syndrome
- 作者：Donaire ; Antonio ; Carreno ; Mar ; Bargall脙脙 ; Nuria ; Setoa脙脙n ; Xavier ; Agudo ; Rolando ; Mart脙脙n ; Gisela ; et. al.
- 关键词：Cognitive reorganization ; Epilepsy ; Fishman syndrome ; Neurocutaneous lipomatosis
- 刊名：Epilepsy and Behavior
- 出版年：2005
- 期刊代码：76_15255050
- 类别：neu
- 出版时间：May, 2005
- 卷：6
- 期：3
- 页码：440-443
- 文件大小：461 K
摘要
Fishman syndrome, also known as encephalocraniocutaneous lipomatosis (ECCL), is a rare, congenital neurocutaneous syndrome characterized by unilateral skin, eye, and brain abnormalities. Epileptic seizures and developmental delay are usually present. We report the clinical, radiological, and, for the first time, neurophysiological findings in a 24-year-old woman diagnosed with ECCL who was evaluated for epilepsy surgery. Functional magnetic resonance imaging revealed transfer of memory and language functions to the nonaffected hemisphere, providing evidence that functional reorganization and restoration of cognitive functions may occur in the context of extensive malformations, such as neurocutaneous syndromes.