An unfortunate challenge: Ketogenic diet for the treatment of Lennox-Gastaut syndrome in tyrosinemia type 1

Eur J Paediatr Neurol. 2016 Jul;20(4):674-7. doi: 10.1016/j.ejpn.2016.02.015. Epub 2016 Mar 16.

Abstract

The ketogenic diet is an evidence-based treatment for resistant epilepsy including Lennox-Gastaut syndrome. This diet is based on low carbohydrate-high fat intakes. Dietary treatment is also therapeutic for inborn errors of metabolism such as aminoacdiopathies. We report a child with both Lennox-Gastaut syndrome and tyrosinemia type 1. This epilepsy syndrome resulted form a porencephalic cyst secondary to brain abscesses that occurred during the management of malnutrition due to untreated tyrosinemia type 1. We used a ketogenic diet as treatment for Lennox-Gastaut syndrome taking into account dietary requirements for tyrosinemia type 1. The patient was transiently responder during a 6-month period. This report illustrates that ketogenic diet remains a therapeutic option even when additional dietary requirements are needed.

Keywords: Antiepileptic drugs; Epilepsy; Ketogenic diet; Lennox–Gastaut syndrome; Tyrosinemia type 1.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Diet, Ketogenic / methods*
  • Diet, Protein-Restricted / methods*
  • Female
  • Humans
  • Infant
  • Lennox Gastaut Syndrome / complications
  • Lennox Gastaut Syndrome / diet therapy*
  • Tyrosinemias / complications
  • Tyrosinemias / diet therapy*