title: Ketogenic diet therapy can improve ACTH-resistant West syndrome in Japan.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  KD was effective for WS resistant to ACTH therapy, although gastrointestinal side effects should be considered when introducing KD to milk-fed infants.
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Tokyo 162, Japan.
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Hirano
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Tokyo 162, Japan. Electronic address: hoguni@ped.twmu.ac.jp.
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Oguni
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Tokyo 162, Japan.
authors.forename: Mutuko
authors.initials: M
authors.lastname: Shiota
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Tokyo 162, Japan.
authors.forename: Aiko
authors.initials: A
authors.lastname: Nishikawa
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, Tokyo 162, Japan.
authors.forename: Makiko
authors.initials: M
authors.lastname: Osawa
mesh_terms: D002675:Child, Preschool; D055423:Diet, Ketogenic; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007564:Japan; D008297:Male; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000178:diet therapy*
keywords: ACTH; Gastrointestinal side effects; Ketogenic diet; West syndrome