De novo status epilepticus due to multiple cerebral hamartomas.

An 18-year-old woman with no history of seizures experienced complex partial status epilepticus after a brief febrile illness. MRI of the brain was normal, and CSF obtained by lumbar puncture did not show infection. Postmortem examination revealed multiple cortical hamartomas. Dysplastic cortical lesions caused de novo status epilepticus (SE) and a syndrome resembling viral encephalitis. The patient was in good health until 2 weeks before admission, when she experienced fever, vomiting, and headache. Her physician prescribed promethazine. Three days before admission she had a generalized tonic-clonic seizure. There was no history of closed head injury, febrile convulsions, or encephalitis/meningitis. An aunt and an uncle had experienced seizures. She was admitted to another hospital after a second generalized tonic-clonic seizure. Over the following 2 days, seizures continued, with staring, blinking, and left facial twitching, despite carbamazepine monotherapy. She became progressively confused and aphasic. Valproic acid was added without effect. CT of the head and MRI of the brain were normal. EEG reportedly did not show …