A 20-year-old woman with Dravet syndrome and multiple prior episodes of status epilepticus presented to our hospital in November 2018 in super-refractory status epilepticus. After 5 weeks of unsuccessful continuous treatment with anesthetics, including pentobarbital and ketamine, we sought and were granted an emergency approval by the United States Food and Drug Administration to administer fenfluramine, an investigational new drug, to this patient. One week of treatment with fenfluramine at 0.4 mg/kg/day was ineffective. The dose of fenfluramine was titrated to 0.7 mg/kg/day, and after 1 week, electrographic seizures ceased. One week later, the patient was seizure-free and off all anesthetic agents. Add-on treatment with fenfluramine was continued with no further episodes of status epilepticus and >90% reduction in tonic-clonic seizures. This case report illustrates the potential for fenfluramine to prevent reoccurrence of status epilepticus and to manage super-refractory status epilepticus in patients with Dravet syndrome.
Keywords: Dravet syndrome; Fenfluramine; Neurocognitive response; Super-refractory status epilepticus.
© 2021 The Authors.