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Safe and Effective Use of the Ketogenic Diet in Children with Epilepsy and Mitochondrial Respiratory Chain Complex Defects

 Hoon-Chul Kang  ;  Young-Mock Lee  ;  Abdelhamid Slama  ;  Joon Soo Lee  ;  Heung Dong Kim 
 EPILEPSIA, Vol.48(1) : 82-88, 2007 
Journal Title
Issue Date
Child ; Child, Preschool ; Comorbidity ; Diet, Carbohydrate-Restricted ; Dietary Fats/administration & dosage ; Dietary Fats/metabolism ; Electroencephalography/statistics & numerical data ; Epilepsy/diet therapy* ; Epilepsy/epidemiology ; Epilepsy/metabolism ; Female ; Humans ; Infant ; Ketone Bodies/biosynthesis ; Ketone Bodies/metabolism ; Ketosis/metabolism* ; Landau-Kleffner Syndrome/diet therapy ; Landau-Kleffner Syndrome/metabolism ; Leigh Disease/diet therapy ; Leigh Disease/metabolism ; Male ; Mitochondrial Diseases/diet therapy* ; Mitochondrial Diseases/epidemiology ; Mitochondrial Diseases/metabolism ; Retrospective Studies ; Spasms, Infantile/diet therapy ; Spasms, Infantile/metabolism ; Treatment Outcome
PURPOSE: To evaluate the clinical efficacy and safety of the ketogenic diet (KD) for patients with intractable childhood epilepsy and mitochondrial respiratory chain (RC) complex defects. METHODS: A retrospective analysis evaluated outcomes in 14 children with intractable epilepsy and RC complex defects who were treated with the classic KD involving a 4:1 lipid to nonlipid ratio (% by weight), but without an initial fast and fluid restriction. Outcome measures included seizure frequency, electroencephalography (EEG) findings, the number of antiepileptic drugs, and adverse reactions. RESULTS: Of the 14 patients, 9 had Complex I defects, 1 had a Complex II defect, 3 had Complex IV defects, and 1 had combined Complex I and IV defects. Two patients with Complex IV defects showed clinical progress compatible with the Leigh disease. The epileptic diagnoses were as follows: 5 patients were diagnosed with infantile spasms, 4 with the Lennox-Gastaut syndrome, 1 with the Landau-Kleffner syndrome, 1 with nonspecific generalized seizure disorder, and 3 with partial seizure disorder. The study found that 7 patients became seizure-free after commencing the KD, three of whom successfully completed the diet without relapse. One patient with a greater than 90% seizure reduction, and 2 patients with seizure reductions between 50% and 90%, remained on the diet. Four patients, including two diagnosed with the Leigh disease, did not show any favorable responses to the diet or ceased the diet due to complications. CONCLUSIONS: The KD was a safe and effective therapy for seizures in children with intractable epilepsy and RC complex defects.
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1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Kang, Hoon Chul(강훈철) ORCID logo https://orcid.org/0000-0002-3659-8847
Kim, Heung Dong(김흥동) ORCID logo https://orcid.org/0000-0002-8031-7336
Lee, Young Mock(이영목) ORCID logo https://orcid.org/0000-0002-5838-249X
Lee, Joon Soo(이준수) ORCID logo https://orcid.org/0000-0001-9036-9343
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