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The Causes of Epilepsy The Causes of Epilepsy
Common and Uncommon Causes in Adults and Children
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Chapter 26 - Sturge–Weber syndrome

from Section 3 - Symptomatic epilepsy

Published online by Cambridge University Press:  05 March 2012

Simon D. Shorvon
Affiliation:
National Hospital for Neurology and Neurosurgery, London
Frederick Andermann
Affiliation:
Montreal Neurological Hospital and Institute
Renzo Guerrini
Affiliation:
Child Neurology Unit, Meyer Pediatric Hospital, Florence
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Summary

This chapter presents the clinical features, etiology, and treatment of Sturge-Weber syndrome (SWS). SWS is a rare, sporadic, congenital disorder arising from an early developmental lesion affecting the facial skin, the eye, and the central nervous system. The association of neurological and developmental deterioration and the onset of seizures means that patients with SWS often suffer considerable disability. The effect of choroidal or ciliary body hemangioma in SWS interferes with the angle of the eye, causing elevated episcleral venous pressure or hypersecretion of fluid. Epileptic seizures are the predominant symptom of SWS and occur in about 80% of cases presenting port-wine stain (PWS) and leptomeningeal angioma. Magnetic resonance imaging (MRI) should be performed at a distance of a seizure event to avoid false interpretation of gadolinium enhancement, due to contrast leakage because of alteration of the blood-brain barrier.
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The Causes of Epilepsy
Common and Uncommon Causes in Adults and Children
, pp. 189 - 195
Publisher: Cambridge University Press
Print publication year: 2011

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