Epilepsy in the setting of neurocutaneous syndromes.
The neurocutaneous syndromes are characterized by congenital dysplastic abnormalities involving the skin and nervous system. The commonest neurocutaneous syndromes manifesting epilepsy are tuberous sclerosis and the Sturge-Weber syndrome. Neurofibromatosis and other lesser-known entities, such as epidermal nevus syndrome, are also known to be accompanied by epilepsy. These syndromes are not related to one another. This article reviews what has been learned about the epileptic syndromes in these disorders.
Kotagal
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Section of Pediatric Epilepsy & Neuropharmacology, Cleveland Clinic Foundation, Ohio 44195.
Rothner
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D000293:Adolescent; D000328:Adult; D000818:Animals; D001921:Brain / Q000002:abnormalities; D002642:Chick Embryo; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D017253:Neurofibromatoses / Q000175:diagnosis*; D009506:Nevus / Q000151:congenital; D013341:Sturge-Weber Syndrome / Q000175:diagnosis*; D014402:Tuberous Sclerosis / Q000175:diagnosis*