| Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES). | |
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MedLine Citation:
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PMID: 20813015 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Rima Nabbout; Michel Mazzuca; Philippe Hubert; Sylviane Peudennier; Catherine Allaire; Vincent Flurin; Marina Aberastury; Walter Silva; Olivier Dulac |
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Publication Detail:
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Type: Comparative Study; Journal Article Date: 2010-08-31 |
Journal Detail:
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Title: Epilepsia Volume: 51 ISSN: 1528-1167 ISO Abbreviation: Epilepsia Publication Date: 2010 Oct |
Date Detail:
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Created Date: 2010-11-02 Completed Date: 2010-12-21 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 2983306R Medline TA: Epilepsia Country: United States |
Other Details:
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Languages: eng Pagination: 2033-7 Citation Subset: IM |
Copyright Information:
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Wiley Periodicals, Inc. © 2010 International League Against Epilepsy. |
Affiliation:
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Department of Neuropediatrics, Centre de Référence Épilepsies Rares, Hôpital Necker-Enfants Malades, Paris, France. rimanabbout@yahoo.com |
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| MeSH Terms | |
Descriptor/Qualifier:
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Age of Onset Anticonvulsants / therapeutic use Child Child, Preschool Cognition Disorders / diet therapy, etiology Drug Resistance Electroencephalography Encephalitis / complications Female Fever / complications* Fever of Unknown Origin / complications Humans Ketogenic Diet / methods* Male Seizures, Febrile / diet therapy Status Epilepticus / diet therapy*, etiology Treatment Outcome |
| Chemical | |
Reg. No./Substance:
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0/Anticonvulsants |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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