West's syndrome--etiology, treatment and prognosis

Forty two children with West's syndrome who had been treated in the Clinic of Paediatrics, Higher Medical Institute, Plovdiv in the last 10 years were entered into the present study. Analysis is made of the aetiology of the disease, the results of treatment and development of the children. All children were followed up from 6 months to 10 years. The West's syndrome was idiopathic in four children and symptomatic in 38 children (90.5%). It had perinatal aetiology in 76.5% of the patients, prenatal in 21%, and postnatal in 2.6%. Complete seizure control was achieved in 17 children (40.5%) treated only with antiepileptic drugs. Synacthen was included in the treatment of the remaining 22 children in three therapeutic doses--0.0125 mg/kg/day (n = 8), 0.025 mg/kg/day (n = 8), and > or = 0.05 mg/kg/day (n = 6). Treatment with different doses of Synacthen showed no statistically significant differences in the three groups. The side effects of the treatment occurred more frequently and were more severe in the groups with a high-dose Synacthen treatment. The follow-up established mental retardation and/or neurological deficit in 88.1% of the children. One infant died during the treatment with Synacthen and another two with severe mental retardation--one year after treatment. In about one third of the cases transition was observed to other epileptic syndromes. Synacthen is concluded to be efficacious in the treatment of West's syndrome. If antiepileptic drugs fail to produce any effect Synacthen should be included in the therapy in due time, preferably in small doses in order to avoid severe and unwanted side effects.