title: [Outcome of initial treatment with high-dose vitamin B6, valproate sodium or clonazepam in West syndrome].
abstract: We reviewed the outcome (seizures and development) of 25 cases with West syndrome in which antiepileptic drugs (valproate sodium, clonazepam) or high doses of vitamin B6, instead of ACTH therapy, were administered for the initial treatment. Five of 9 cryptogenic cases (56%) and 4 of 16 symptomatic cases (25%) showed complete cessation of spasms. In cryptogenic cases, response to vitamin B6, valproate sodium or clonazepam was not predicted by clinical features (age of onset, seizure type, initial EEG finding or treatment lag). On the other hand, response to these drugs was correlated with some clinical findings in symptomatic cases; all infants with neurocutaneous syndrome (tuberous sclerosis, neurofibromatosis type 1) had controlled infantile spasms, while none of patients with severe neonatal asphyxia or with prior other seizures responded. Twenty of 25 patients have been followed-up. The average age at follow-up was 5 years and 8 months. Prognosis of both cryptogenic and symptomatic "responders" was favorable; all had seizures controlled, and 50% had normal psychomotor development or only mild impairment (DQ > 70). Symptomatic "nonresponders" had the worst prognosis. Our results suggest that choice of drug in the initial treatment of West syndrome should be determined by clinical features (especially etiology).
authors.affiliation: Department of Pediatrics, Osaka University Medical School.
authors.forename: Y
authors.initials: Y
authors.lastname: Suzuki
authors.forename: T
authors.initials: T
authors.lastname: Kita
authors.forename: T
authors.initials: T
authors.lastname: Mano
authors.forename: H
authors.initials: H
authors.lastname: Arai
authors.forename: T
authors.initials: T
authors.lastname: Matsuoka
authors.forename: R
authors.initials: R
authors.lastname: Kodaka
authors.forename: K
authors.initials: K
authors.lastname: Imai
authors.forename: T
authors.initials: T
authors.lastname: Nagai
authors.forename: S
authors.initials: S
authors.lastname: Okada
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002998:Clonazepam / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011736:Pyridoxine / Q000008:administration & dosage*; D013036:Spasms, Infantile / Q000188:drug therapy*; D014635:Valproic Acid / Q000627:therapeutic use
chemical_list: D000927:Anticonvulsants; D002998:Clonazepam; D014635:Valproic Acid; D011736:Pyridoxine