A female infant with classical Sturge-Weber syndrome, hemifacial hemangioma, hemiplegia and intracranial calification, was followed up since her birth. At one and half months old, she developed an episode of prolonged generalized hemiconvulsion, which prompted EEG examination revealing asymmetrical lazy activities and anticonvulsant medication. At four months of life, a series of typical massive myoclonic seizures of flexion type developed suddenly after bout of crying. EEG examination at that time revealed diffuse and asymmetric irregular high voltage slow waves with randomly scattered spike discharge, which could be called as hypsarhthmia with asymmetry. Desynchronized fast activities were observed synchronously with clinical seizures, which occurred incidentally during the EEG recording and was witnessed by one of the authors as a typical attack of infantile spasms. The attack has been promptly eradicated by ACTH therapy and remained seizurefree till now (15months).
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