Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES)

摘要: SUMMARY Purpose: Fever induced refractory epileptic encephalopathy in school age children (FIRES) is a devastating condition initiated by prolonged perisylvian status epilepticus (SE) triggered fever of unknown cause. SE may last more than 1 month, and this evolve into pharmacoresistant epilepsy associated with severe cognitive impairment. We aimed to report the effect ketogenic diet (KD) condition. Methods: Over 12 years we collected data nine patients FIRES who received 4:1 ratio fat combined protein carbohydrate KD. They presented SErefractorytoconventionalantiepileptictreatment. Results: In seven patients, KD was efficacious within 2‐4 days (mean 2 days) following onset ketonuria 4‐6 4.8 diet. one responder, early disruption followed relapse intractable SE, patient died. Epilepsy affected other six responders few months. Discussion: be an alternative therapy for might proposed types childhood.