A SIMPLE, EFFECTIVE AND WELL-TOLERATED TREATMENT REGIME FOR WEST SYNDROME

The authors systematically treated 94 patients with West syndrome usin g the same protocol of sodium valproate and steroids, starting with hy drocortisone (HC) orally for two weeks. If seizures stopped, HC was wi thdrawn; if they persisted, tetracosactrin (TA; synthetic ACTH) was ad ministered for another two weeks then HC was slowly withdrawn. 90 per cent of the symptomatic cases were controlled by HC, the remainder by TA. 65 per cent of symptomatic cases were controlled by HC; this rose to 78 per cent if patients treated by HC then TA were included. At 31 months follow-up, the percentage of favourable results was 72 per cent for cryptogenic and 60 per cent for symptomatic cases. For the latter , best results were obtained in patients with periventricular leukomal acia, postnatal distress and porencephaly. Patients suffering from seq uelae of full-term distress or encephalopathy of unknown aetiology wer e the most resistant.