Safe and effective use of the ketogenic diet in children with epilepsy and mitochondrial respiratory chain complex defects.
OBJECTIVE
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.
Kang
Hoon-Chul
HC
Department of Pediatrics and Epilepsy Center, Sanggye Paik Hospital, Inje University College of Medicine, Seoul, Korea.
Lee
Young-Mock
YM
Kim
Heung Dong
HD
Lee
Joon Soo
JS
Slama
Abdelhamid
A
D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D050528:Diet, Carbohydrate-Restricted; D004041:Dietary Fats / Q000008:administration & dosage / Q000378:metabolism; D004569:Electroencephalography / Q000706:statistics & numerical data; D004827:Epilepsy / Q000178:diet therapy* / Q000453:epidemiology / Q000378:metabolism; D005260:Female; D006801:Humans; D007223:Infant; D007657:Ketone Bodies / Q000096:biosynthesis / Q000378:metabolism; D007662:Ketosis / Q000378:metabolism*; D018887:Landau-Kleffner Syndrome / Q000178:diet therapy / Q000378:metabolism; D007888:Leigh Disease / Q000178:diet therapy / Q000378:metabolism; D008297:Male; D028361:Mitochondrial Diseases / Q000178:diet therapy* / Q000453:epidemiology / Q000378:metabolism; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000178:diet therapy / Q000378:metabolism; D016896:Treatment Outcome
D004041:Dietary Fats; D007657:Ketone Bodies