Sturge-Weber disease--neurophysiological evaluation of a case with secondary epileptogenesis, successfully treated...

A case of Sturge-Weber's disease with a generalized seizure at 7 months of age is described, followed 7 months later by the development of very frequent and therapy resistent myoclonic astatic fits. The neurophysiological evaluation indicated a primary lesion in the right occipital area followed by signs of secondary epileptogenesis. The patient was cured by a right-sided occipital lobe-ectomy including an area of leptomeningeal angiomatosis.