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Brain Dev. 2015 Jan;37(1):18-22. doi: 10.1016/j.braindev.2014.01.015. Epub 2014 Feb 18.

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

Author information

1
Department of Pediatrics, Tokyo Women's Medical University, Tokyo 162, Japan.
2
Department of Pediatrics, Tokyo Women's Medical University, Tokyo 162, Japan. Electronic address: hoguni@ped.twmu.ac.jp.

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.

KEYWORDS:

ACTH; Gastrointestinal side effects; Ketogenic diet; West syndrome

PMID:
24559837
DOI:
10.1016/j.braindev.2014.01.015
[Indexed for MEDLINE]

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