title: Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES).
abstract: OBJECTIVE
  Fever induced refractory epileptic encephalopathy in school age children (FIRES) is a devastating condition initiated by prolonged perisylvian refractory status epilepticus (SE) triggered by fever of unknown cause. SE may last more than 1 month, and this condition may evolve into pharmacoresistant epilepsy associated with severe cognitive impairment. We aimed to report the effect of ketogenic diet (KD) in this condition.
  
  
  METHODS
  Over the last 12 years we collected data of nine patients with FIRES who received a 4:1 ratio of fat to combined protein and carbohydrate KD. They presented with SE refractory to conventional antiepileptic treatment.
  
  
  RESULTS
  In seven patients, KD was efficacious within 2-4 days (mean 2 days) following the onset of ketonuria and 4-6 days (mean 4.8 days) following the onset of the diet. In one responder, early disruption of the diet was followed by relapse of intractable SE, and the patient died. Epilepsy affected the other six responders within a few months.
  
  
  CONCLUSIONS
  KD may be an alternative therapy for refractory SE in FIRES and might be proposed in other types of refractory SE in childhood.
authors.affiliation: Department of Neuropediatrics, Centre de Référence Épilepsies Rares, Hôpital Necker-Enfants Malades, Paris, France. rimanabbout@yahoo.com
authors.forename: Rima
authors.initials: R
authors.lastname: Nabbout
authors.forename: Michel
authors.initials: M
authors.lastname: Mazzuca
authors.forename: Philippe
authors.initials: P
authors.lastname: Hubert
authors.forename: Sylviane
authors.initials: S
authors.lastname: Peudennier
authors.forename: Catherine
authors.initials: C
authors.lastname: Allaire
authors.forename: Vincent
authors.initials: V
authors.lastname: Flurin
authors.forename: Marina
authors.initials: M
authors.lastname: Aberastury
authors.forename: Walter
authors.initials: W
authors.lastname: Silva
authors.forename: Olivier
authors.initials: O
authors.lastname: Dulac
mesh_terms: D017668:Age of Onset; D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000178:diet therapy / Q000209:etiology; D055423:Diet, Ketogenic / Q000379:methods*; D004351:Drug Resistance; D004569:Electroencephalography; D004660:Encephalitis / Q000150:complications; D005260:Female; D005334:Fever / Q000150:complications*; D005335:Fever of Unknown Origin / Q000150:complications; D006801:Humans; D008297:Male; D003294:Seizures, Febrile / Q000178:diet therapy; D013226:Status Epilepticus / Q000178:diet therapy* / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants