Nonconvulsive status epilepticus in a child with congenital bilateral perisylvian syndrome

A 9-year-old male with congenital bilateral perisylvian syndrome is describ ed. He had pseudobulbar palsy, mental retardation, and intractable epilepsy . Computed tomography and magnetic resonance images of the brain demonstrat ed bilateral perisylvian malformations and a diffuse pachygyric appearance. At 8 years of age, he had episodes of excessive drooling, fluctuating impa irment of consciousness, unsteady sitting, and frequent head drop that last ed several days, The electroencephalogram demonstrated continuous diffuse s low spike and waves. These findings suggested atypical absence status epile pticus. Intravenous administration of diazepam resulted in transient improv ement of clinical and electroencephalographic findings. Status epilepticus recurred within several minutes after diazepam administration. Although no patient has been reported to have a history of status epilepticus among tho se affected by this syndrome, it seems that atypical absence status can occ ur more frequently than expected, as seen in Lennox-Gastaut syndrome. After recognition and confirmation of nonconvulsive status epilepticus, immediat e treatment must be attempted. (C) 1999 by Elsevier Science Inc. All rights reserved.