West syndrome: individualized ACTH therapy.
Individualized ACTH treatment of the West syndrome (WS) was assessed in a prospective multicenter study, in which each patient's dosage was increased stepwise according to response. Our series included six patients with cryptogenic and 24 with symptomatic infantile spasms. During the treatment period the total ACTH dose ranged from 58 to 373 i.u./kg. In the cryptogenic group one patient responded to pre-ACTH pyridoxine and four to the lowest dosage of ACTH (3 i.u./kg daily) with cessation of spasms and good outcome; one patient needed the highest dosage (12 i.u./kg daily) for cessation of seizures and became developmentally retarded. In the symptomatic group, 21 of the 24 patients needed 6-12 i.u./kg daily; 12 became seizure-free or having infrequent non-IS fits. Complications such as arterial hypertension, cerebral ventricle dilatation, cardiac hypertrophy, and prolonged adrenocortical hyporesponsiveness were related to the dose. The individualization provides all the benefits of ACTH treatment with minimal side effects and cost.
Heiskala
H
H
Children's Hospital, University of Helsinki, Finland.
Riikonen
R
R
Santavuori
P
P
Simell
O
O
Airaksinen
E
E
Nuutila
A
A
Perheentupa
J
J
D000324:Adrenocorticotropic Hormone / Q000008:administration & dosage*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D011446:Prospective Studies; D011736:Pyridoxine / Q000008:administration & dosage; D013036:Spasms, Infantile / Q000188:drug therapy*; D016896:Treatment Outcome
D000324:Adrenocorticotropic Hormone; D011736:Pyridoxine