De novo sustained refractory status epilepticus and encephalopathy: a retrospective case series

Refractory status epilepticus is a devastating persistent seizure state with a poor prognosis that requires emergency medical management. Recent studies have reported de novo, idiopathic refractory status epilepticus of unclear etiology in healthy young patients followed by severe neurologic sequelae. We present a series of 7 cases of de novo sustained refractory status epilepticus. We found that all patients were young and previously healthy and that, prior to the onset of refractory status epilepticus, all had prodromal viral-like symptoms. The onset of refractory status epilepticus was explosive and intractable, resulting in prolonged hospital stay and dependence on multiple antiepileptic medications. Clinical outcome was poor in all 7 patients. The laboratory findings suggest a possible immune activation that can have persisted in the nervous system after a nonspecific infection. We report on these patients so as to raise awareness of this unique entity to facilitate early diagnosis and treatment.