Ketogenic diet therapy can improve ACTH-resistant West syndrome in Japan.

Abstract

PURPOSE:

Ketogenic diet therapy (KD) has been used to treat children with refractory generalized epilepsy. We herein reported the efficacy of KD for West syndrome (WS) resistant to ACTH therapy.

SUBJECTS:

SUBJECTS, consisting of 6 patients (3 boys, 3 girls) with WS who continued to have epileptic spasms (ES) and hypsarrhythmia, received KD because other treatments including ACTH therapy failed to control WS.

METHODS:

We retrospectively studied the clinical details of these patients and the efficacy of KD.

RESULTS:

The mean age at the onset of epilepsy was 4 months (0-15 months). The underlying etiology consisted of lissencephaly, Down's syndrome, and focal cortical dysplasia. Hypsarrhythmia disappeared 1 month after the introduction of KD in 5 patients. The disappearance of ES was achieved in 2 patients, the frequency of ES episodes was 80% less in 3, and no change was observed in 1. Psychomotor development was promoted in 5 patients, along with improvements in ES and EEG. Gastrointestinal complications and lethargy, presumably caused by rapid ketosis, were reported as side effects in 3 patients during the first week of KD. Side effects including lethargy, anorexia, and unfavorable weight gain continued thereafter in these patients in spite of tolerance to KD.

CONCLUSION:

KD was effective for WS resistant to ACTH therapy, although gastrointestinal side effects should be considered when introducing KD to milk-fed infants.

• Authors: Yoshiko Hirano, Hirokazu Oguni, Mutuko Shiota, Aiko Nishikawa, Makiko Osawa
• Journal: Brain & development (Brain Dev) Vol. 37 Issue 1 Pg. 18-22 (Jan 2015) ISSN: 1872-7131 [Electronic] Netherlands
• PMID: 24559837 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

Topics

• Child, Preschool
• Diet, Ketogenic
• Female
• Humans
• Infant
• Infant, Newborn
• Japan
• Male
• Retrospective Studies
• Spasms, Infantile (diet therapy)