title: [Study of the efficacy of low-dose synthetic ACTH therapy without tapering to treat West syndrome].
abstract: OBJECTIVE
  We evaluated the effectiveness of synthetic adrenocorticotropic hormone (ACTH) therapy without tapering in treating patients with West syndrome.
  
  
  METHODS
  Forty-four patients with cryptogenic (n = 7) or symptomatic (n = 37) West syndrome were treated with synthetic ACTH therapy between 2003 and 2012. The synthetic ACTH dosage was 0.0125 mg/kg/day administered daily for 2 weeks and then stopped without a tapering period. The initial effectiveness, long-term seizure outcome, and adverse effects were examined.
  
  
  RESULTS
  During synthetic ACTH therapy, epileptic spasms disappeared in 37 of 44 patients (84.1%) and hypsarrhythmia on electroencephalography disappeared in 42 of 44 patients (95.5%). The average number of synthetic ACTH injections needed to achieve spasm control in these 37 patients was 5.8. Long-term seizure outcomes were assessed in 31 patients followed up for longer than half a year after synthetic ACTH therapy. Nine (29.0%) of these patients experienced recurrence of epileptic spasms, with a mean interval to recurrence of 2.4 months. Overall, 12 patients (38.7%) experienced various types of seizures other than spasms with a mean interval to recurrence of 8.0 months. Although adverse effects such as hypertension, infection, and mild brain shrinkage were noted in 13 patients (29.5%), no severe adverse effects were observed.
  
  
  CONCLUSIONS
  These results are comparable to those of other reports on the initial effectiveness and long-term seizure control following synthetic ACTH therapy, and suggest that administration without tapering is reasonable to treat patients with West syndrome.
authors.forename: Kazuo
authors.initials: K
authors.lastname: Kodama
authors.forename: Taku
authors.initials: T
authors.lastname: Omata
authors.forename: Hidee
authors.initials: H
authors.lastname: Arai
authors.forename: Yuzo
authors.initials: Y
authors.lastname: Tanabe
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000008:administration & dosage* / Q000009:adverse effects; D000927:Anticonvulsants / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D007223:Infant; D007555:Isoxazoles / Q000008:administration & dosage; D008297:Male; D012008:Recurrence; D013036:Spasms, Infantile / Q000188:drug therapy*; D013997:Time Factors; D016896:Treatment Outcome; D000078305:Zonisamide
chemical_list: D000927:Anticonvulsants; D007555:Isoxazoles; D000078305:Zonisamide; D000324:Adrenocorticotropic Hormone