Sturge-Weber syndrome: a favourable surgical outcome in a case with contralateral seizure onset and myoclonic-astatic seizures

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• 作者：Premysl Jiruska (1) (2)
  Petr Marusic (3) (5)
  John G. R. Jefferys (1)
  Pavel Krsek (2)
  Roman Cmejla (4)
  Vera Sebronova (2)
  Vladimir Komarek (2)
  
• 关键词：Sturge ; Weber syndrome ; myoclonic ; astatic seizures ; contralateral seizure onset ; epilepsy surgery
• 刊名：Epileptic Disorders
• 出版年：2011
• 出版时间：March 2011
• 年：2011
• 卷：13
• 期：1
• 页码：76-81
• 全文大小：492KB
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• 作者单位：Premysl Jiruska (1) (2)
  Petr Marusic (3) (5)
  John G. R. Jefferys (1)
  Pavel Krsek (2)
  Roman Cmejla (4)
  Vera Sebronova (2)
  Vladimir Komarek (2)
  
  1. Neuronal Networks Group, Neuropharmacology and Neurobiology, School of Clinical and Experimental Medicine, University of Birmingham, Birmingham, UK
  2. Department of Pediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague, University Hospital Motol, Prague, Czechoslovakia
  3. Department of Neurology, 2nd Faculty of Medicine, Charles University in Prague, University Hospital Motol, Prague, Czechoslovakia
  5. Department of Neurology, Charles University in Prague, 2nd Faculty of Medicine, University Hospital Motol, V Uvalu 84, Praha 5, 150 06, Czech Republic
  4. Department of Circuit Theory, Faculty of Electrical Engineering, Czech Technical University, Prague, Czech Republic
  
• ISSN：1950-6945

文摘

Sturge-Weber syndrome is a neurocutaneous disorder classically characterized by the presence of facial port-wine stain and ipsilateral leptomeningeal angiomatosis. It is often associated with refractory epilepsy which requires surgical treatment. We present a case of a patient who initially presented with partial seizures of temporo-occipital origin, ipsilateral to the pial angiomatosis. During the course of the disease, the patient developed medically refractory epilepsy with partial seizures originating predominantly from the contralateral temporo-occipital area as well as myoclonic and myoclonicastatic seizures. Resection of the occipital and temporal lobe affected by the pial angioma resulted in favourable outcome. Bilateral dysfunction observed in Sturge-Weber syndrome may result in an increased capability of focal discharges to generate synchronous epileptiform activity leading to an increased incidence of generalised seizures, most probably via a mechanism of secondary bilateral synchrony.