Tuberous sclerosis complex and the ketogenic diet

Eric H Kossoff1, Elizabeth A Thiele, Heidi H Pfeifer

  • 1Departments of Neurology and Pediatrics, The John M. Freeman Pediatric Epilepsy Center, Johns Hopkins University, Baltimore, MD 212871000, USA. ekossoff@jhmi.edu

Epilepsia
|September 30, 2005
PubMed

Insights

The ketogenic diet effectively reduced seizures in children with Tuberous Sclerosis Complex (TSC) and epilepsy. This dietary therapy showed significant seizure reduction in most patients when medications failed.

Area of Science:

  • Neurology
  • Pediatrics
  • Dietary Therapies

Background:

  • Tuberous Sclerosis Complex (TSC) frequently causes intractable early-onset epilepsy, often presenting as infantile spasms.
  • When pharmacologic treatments fail and no clear epileptogenic tuber is identified, non-pharmacologic therapies are considered.
  • The efficacy of the ketogenic diet for epilepsy in children with TSC has not been previously documented.

Observation:

  • A retrospective chart review identified 12 pediatric patients with TSC treated with the ketogenic diet.
  • Patients ranged in age from 8 months to 18 years.
  • Treatment duration varied from 2 months to 5 years, with a mean of 2 years.

Findings:

  • 92% of children experienced over a 50% reduction in seizures at 6 months.
  • 67% of children achieved over a 90% seizure reduction.
  • Five children had a seizure-free period of at least 5 months.

Implications:

  • The ketogenic diet appears to be a generally effective treatment for intractable epilepsy in children with TSC.
  • This dietary intervention offers a viable therapeutic option for refractory epilepsy in this population.
  • Further research may explore long-term outcomes and optimal implementation of the ketogenic diet for TSC-associated epilepsy.
Abstract

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