Efficacy of the ketogenic diet in Lennox-Gastaut syndrome: a retrospective review of one institution's experience and summary of the literature

Dev Med Child Neurol. 2012 May;54(5):464-8. doi: 10.1111/j.1469-8749.2012.04233.x. Epub 2012 Mar 22.

Abstract

Aim: To determine the efficacy of the ketogenic diet for children with Lennox-Gastaut syndrome (LGS) at our institution and in the literature.

Method: The records of children with LGS initiated on the ketogenic diet at our institution from 1994 to 2010 were reviewed. Inclusion criteria included the presence of ≤2.5Hz spike-and-wave complexes on electroencephalogram, multiple seizure types including tonic, atonic, or atypical absence, developmental delay, and age under 1 year. We additionally reviewed the literature for cases of LGS treated with the ketogenic diet and their outcomes.

Results: Seventy-one children (41 males, 30 females, median age 3y 6mo, range 18mo-18y), with LGS were initiated on the ketogenic diet. Using an intent-to-treat analysis, after 6 months, 36 (51%) achieved more than 50% seizure reduction, 16 (23%) experienced more than 90% seizure reduction, and 1 (1%) achieved seizure freedom. Results were similar after 12 months. Age, sex, side effects, valproate use, and history of infantile spasms were not predictive of more than 90% seizure reduction. In the literature, 88 of 189 (47%) children with LGS had more than 50% seizure reduction after 3 to 36 months of ketogenic diet treatment.

Interpretation: The ketogenic diet is efficacious in the treatment of LGS, with approximately one-half of children responding at 12 months.

MeSH terms

  • Adolescent
  • Anticonvulsants / therapeutic use
  • Brain / physiopathology
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Diet, Ketogenic*
  • Electroencephalography
  • Epilepsy, Absence / diagnosis
  • Epilepsy, Absence / diet therapy
  • Epilepsy, Absence / physiopathology
  • Epilepsy, Generalized / diagnosis
  • Epilepsy, Generalized / diet therapy
  • Epilepsy, Generalized / physiopathology
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Intellectual Disability / diagnosis
  • Intellectual Disability / diet therapy*
  • Intellectual Disability / physiopathology
  • Intention to Treat Analysis
  • Lennox Gastaut Syndrome
  • Male
  • Retrospective Studies
  • Spasms, Infantile / diagnosis
  • Spasms, Infantile / diet therapy*
  • Spasms, Infantile / physiopathology

Substances

  • Anticonvulsants

Supplementary concepts

  • Epileptic encephalopathy, Lennox-Gastaut type