Short-duration ACTH therapy for cryptogenic West syndrome with better outcome.
In this study, seven patients with cryptogenic West syndrome were subjected to short-duration adrenocorticotrophic hormone (ACTH) therapy. ACTH was administered daily for 7 to 12 days (mean 10 days), and then was withdrawn. Daily single dose was 0.022 to 0.027 mg/kg/day (0.024 mg/kg/day, 0.96 IU/kg), total dose was 0.17 to 0.28 mg/kg (0.23 mg/kg, 9.0 IU/kg), and treatment lag was 12 to 105 days (median 14 days). ACTH therapy controlled tonic spasms with no serious side effects in all patients, but two patients still had electroencephalographic abnormality. The intelligence quotients or developmental quotients of six patients with short treatment lag were 79 to 110 at the age of 2 to 6 years; only one patient with long treatment lag had a developmental quotient of 60. This new short-duration ACTH therapy could yield better cognitive outcomes for cryptogenic West syndrome.
Hattori
Ayako
A
Department of Pediatrics, Neonatology and Congenital Disorders, Nagoya City University, Graduate School of Medical Sciences, Kawasumi, Nagoya, Japan. aykhat@med.nagoya-cu.ac.jp
Ando
Naoki
N
Hamaguchi
Kiyo
K
Hussein
Mohamed Hamed
MH
Fujimoto
Shinji
S
Ishikawa
Tatsuya
T
Togari
Hajime
H
D000324:Adrenocorticotropic Hormone / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D003071:Cognition / Q000187:drug effects; D004569:Electroencephalography; D005500:Follow-Up Studies; D006728:Hormones / Q000008:administration & dosage*; D006801:Humans; D007223:Infant; D007361:Intelligence Tests; D013036:Spasms, Infantile / Q000188:drug therapy*; D016896:Treatment Outcome
D006728:Hormones; D000324:Adrenocorticotropic Hormone