Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES).
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.
Nabbout
Rima
R
Department of Neuropediatrics, Centre de Référence Épilepsies Rares, Hôpital Necker-Enfants Malades, Paris, France. rimanabbout@yahoo.com
Mazzuca
Michel
M
Hubert
Philippe
P
Peudennier
Sylviane
S
Allaire
Catherine
C
Flurin
Vincent
V
Aberastury
Marina
M
Silva
Walter
W
Dulac
Olivier
O
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
D000927:Anticonvulsants