title: Low-dose ACTH therapy for West syndrome: initial effects and long-term outcome.
abstract: BACKGROUND
  Most Japanese pediatric neurologists attempt other treatments before using adrenocorticotropic hormone (ACTH) therapy for West syndrome (WS), and even then, they use only a low-dose synthetic ACTH to avoid serious adverse effects. In this multi-institutional study, the authors analyzed the initial effects, adverse effects, and long-term outcome in patients treated with low-dose synthetic ACTH in Japan.
  
  
  METHODS
  The medical records of 138 patients with WS, who were treated with low-dose synthetic ACTH therapy for the first time at the authors' institutions between 1989 and 1998, were analyzed.
  
  
  RESULTS
  At the end of ACTH therapy, excellent effect on seizures was noted in 106 of 138 (76%) patients, good effect in 23 (17%), and poor effect in 9 (7%). Initial effects on EEG were excellent in 53 of 138 (38%) patients, good in 76 (55%), and poor in 9 (7%). As for seizure prognosis at the time of follow-up, 51 of 99 (52%) patients were seizure-free, whereas 48 (48%) patients had seizures. Mental outcome was normal in 6 of 98 (6%) patients, mild mental retardation in 16 (16%), moderate mental retardation in 26 (27%), and severe mental retardation in 50 (51%). The initial effects of ACTH on seizures and long-term outcome were not dose dependent (daily dosage 0.005 to 0.032 mg/kg, 0.2 to 1.28 IU/kg; total dosage 0.1 to 0.87 mg/kg, 4 to 34.8 IU/kg). The severity of adverse effects correlated with total dosage of ACTH, and the severity of brain volume loss due to ACTH correlated well with the daily dosage and total dosage of ACTH.
  
  
  CONCLUSIONS
  Low-dose synthetic ACTH therapy is as effective for the treatment of WS as the higher doses used in previous studies. The dosage of synthetic ACTH used in the treatment of WS can be decreased as much as possible to avoid serious adverse effects.
authors.affiliation: Department of Pediatrics, Shiga Medical Center for Children, Moriyama, Japan. ito-mccs@mx.biwa.ne.jp
authors.forename: M
authors.initials: M
authors.lastname: Ito
authors.forename: H
authors.initials: H
authors.lastname: Aiba
authors.forename: K
authors.initials: K
authors.lastname: Hashimoto
authors.forename: S
authors.initials: S
authors.lastname: Kuroki
authors.forename: K
authors.initials: K
authors.lastname: Tomiwa
authors.forename: T
authors.initials: T
authors.lastname: Okuno
authors.forename: H
authors.initials: H
authors.lastname: Hattori
authors.forename: T
authors.initials: T
authors.lastname: Go
authors.forename: H
authors.initials: H
authors.lastname: Sejima
authors.forename: S
authors.initials: S
authors.lastname: Dejima
authors.forename: H
authors.initials: H
authors.lastname: Ikeda
authors.forename: M
authors.initials: M
authors.lastname: Yoshioka
authors.forename: O
authors.initials: O
authors.lastname: Kanazawa
authors.forename: T
authors.initials: T
authors.lastname: Kawamitsu
authors.forename: J
authors.initials: J
authors.lastname: Ochi
authors.forename: N
authors.initials: N
authors.lastname: Miki
authors.forename: H
authors.initials: H
authors.lastname: Noma
authors.forename: K
authors.initials: K
authors.lastname: Oguro
authors.forename: N
authors.initials: N
authors.lastname: Ozaki
authors.forename: A
authors.initials: A
authors.lastname: Tamamoto
authors.forename: T
authors.initials: T
authors.lastname: Matsubara
authors.forename: T
authors.initials: T
authors.lastname: Miyajima
authors.forename: T
authors.initials: T
authors.lastname: Fujii
authors.forename: Y
authors.initials: Y
authors.lastname: Konishi
authors.forename: T
authors.initials: T
authors.lastname: Okuno
authors.forename: H
authors.initials: H
authors.lastname: Hojo
mesh_terms: D001921:Brain / Q000187:drug effects; D003366:Cosyntropin / Q000008:administration & dosage* / Q000009:adverse effects; D004569:Electroencephalography / Q000187:drug effects; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000209:etiology; D008297:Male; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology
chemical_list: D003366:Cosyntropin