Therapeutic efficacy and adverse effects of adrenocorticotropic hormone therapy in west syndrome: differences in dosage of adrenocorticotropic hormone, onset of age, and cause.
OBJECTIVE
To determine the dosage and factors influencing efficacy of adrenocorticotropic hormone (ACTH) for West syndrome.


METHODS
A retrospective study of 135 patients receiving ACTH therapy with a synthetic analogue for initial effect, seizure outcome 1 year after therapy, and adverse effects. Efficacy and adverse effects were compared among the groups divided by clinical factors: dosage, treatment lag, onset age, and cause.


RESULTS
One hundred thirteen patients had seizure control with ACTH. For more than 1 year after ACTH, 59 remained seizure free. Adverse effects were observed in 57, and ACTH therapy was discontinued in 23. The lowest dosage group (0.0125 mg/kg/d) had fewer episodes of discontinuation (P<.05), whereas differences in efficacy between different dosages were insignificant. None of the clinical factors correlated with initial effect. The earlier-onset group (<4 months) showed unfavorable seizure outcome 1 year after ACTH (P<.01). The cryptogenic patients showed better seizure outcome (P<.05) compared with the symptomatic.


CONCLUSIONS
Synthetic ACTH therapy at a lower dosage is as effective as natural ACTH therapy at a higher dosage. Considering the adverse effects and the benefits for seizure control, the ACTH dosage of 0.0125 mg/kg/d (synthetic analogue) is more favorable than larger dosage.
Hamano
Shin-ichiro
S
Division of Neurology, Saitama Children's Medical Center, and Department of Pediatrics, Jikei University School of Medicine, Japan. a1091170@pref.saitama.lg.jp
Yamashita
Shintaro
S
Tanaka
Manabu
M
Yoshinari
Satoshi
S
Minamitani
Motoyuki
M
Eto
Yoshikatsu
Y
D017668:Age of Onset; D003366:Cosyntropin / Q000008:administration & dosage* / Q000009:adverse effects / Q000494:pharmacology; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome
D003366:Cosyntropin