Ketogenic diet in the treatment of seizures associated with hypothalamic hamartomas.

Abstract

Seizures associated with hypothalamic hamartoma (HH) are notoriously intractable to medical therapy, and while surgical resection affords most affected patients with complete or near seizure-freedom, there remains a need to identify alternative treatments. In this retrospective study, we identified six patients from a large cohort of 220 patients with HH who were treated with the ketogenic diet (KD). Four patients had a 50-90% reduction in multiple seizure types (including gelastic, partial-onset and atonic seizures), and two individuals failed to respond. In order to study possible mechanisms, we then performed microelectrode recordings of small neurons in surgically resected HH tissue slices. Exposure to ketone bodies decreased spontaneous firing in 5 of 7 small HH neurons. These preliminary results suggest that seizures associated with HH may respond favorably to the KD, and that ketone bodies might directly modulate the intrinsic epileptogenicity of HH tissue.

• Authors: Kevin E Chapman, Do-Young Kim, Jong M Rho, Yu-Tze Ng, John F Kerrigan
• Journal: Epilepsy research (Epilepsy Res) Vol. 94 Issue 3 Pg. 218-21 (May 2011) ISSN: 1872-6844 [Electronic] Netherlands
• PMID: 21377333 (Publication Type: Journal Article, Research Support, N.I.H., Extramural)
• Copyright: Copyright © 2011 Elsevier B.V. All rights reserved.

Topics

• Adolescent
• Brain Waves (drug effects)
• Child
• Child, Preschool
• Diet, Ketogenic (methods)
• Electroencephalography
• Female
• Hamartoma (complications)
• Humans
• Hypothalamic Diseases (complications)
• Male
• Retrospective Studies
• Seizures (diet therapy, etiology)