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

Summary Purpose: 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 p...

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Bibliographic Details
Published in:	Epilepsia (Copenhagen) Vol. 51; no. 10; pp. 2033 - 2037
Main Authors:	Nabbout, Rima, Mazzuca, Michel, Hubert, Philippe, Peudennier, Sylviane, Allaire, Catherine, Flurin, Vincent, Aberastury, Marina, Silva, Walter, Dulac, Olivier
Format:	Journal Article
Language:	English
Published:	Oxford, UK Blackwell Publishing Ltd 01-10-2010 Wiley-Blackwell
Subjects:	Age of Onset Anticonvulsants - therapeutic use Biological and medical sciences Child Child, Preschool Cognition Disorders - diet therapy Cognition Disorders - etiology Devastating epileptic encephalopathy in school age children Diet, Ketogenic - methods Diseases of the nervous system Drug Resistance Electroencephalography Encephalitis Encephalitis - complications Female Fever - complications Fever induced refractory epileptic encephalopathy in school age children Fever of Unknown Origin - complications Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy Humans Ketogenic diet Male Medical sciences Nervous system (semeiology, syndromes) Neurology Radiotherapy. Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, orthophony, crenotherapy. Diet therapy and various other treatments (general aspects) Seizures, Febrile - diet therapy Status epilepticus Status Epilepticus - diet therapy Status Epilepticus - etiology Treatment Outcome Human Encephalitis Nervous system diseases Devastating epileptic encephalopathy in school age children Epilepsy Fever induced refractory epileptic encephalopathy in school age children Fever Ketogenic diet Cerebral disorder Refractory Subintrant crisis Encephalopathy Central nervous system disease Child Status epilepticus
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Summary:	Summary Purpose: 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. Discussion: KD may be an alternative therapy for refractory SE in FIRES and might be proposed in other types of refractory SE in childhood.
Bibliography:	ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-1 content type line 23 ObjectType-Article-1 ObjectType-Feature-2
ISSN:	0013-9580 1528-1167
DOI:	10.1111/j.1528-1167.2010.02703.x