Safe and effective use of the ketogenic diet in children with epilepsy and mitochondrial respiratory chain complex defects

Epilepsia. 2007 Jan;48(1):82-8. doi: 10.1111/j.1528-1167.2006.00906.x.


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.

Publication types

  • Comparative Study

MeSH terms

  • 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


  • Dietary Fats
  • Ketone Bodies