ACTH and corticosteroids have for many years been considered the standard therapy, in spite of limited controlled data and serious risks of medium-term toxicity. ACTH is usually preferred to oral corticosteroids. The usual initial recommended daily dose of ACTH is 40 IU (3-6 IU/kg), given for between 1 and 5 months. If seizures relapse either on therapy or after withdrawal, the ACTH should be recommenced immediately and doses of 60-80 IU may be needed. The incidence of adverse events is very high and almost all children develop cushingoid symptoms. Other common adverse effects include infections, increased arterial blood pressure, gastritis and hyperexcitability.
Oral steroids are less extensively prescribed, although they seem to be better tolerated than ACTH. In a prospective, randomized, blinded study, the efficacy of prednisone (2 mg/kg/day) was inferior to that of high-dose ACTH
(150 IU/day) given for 2 weeks, but no differences were found when ACTH was administered at lower doses. Spasms are immediately controlled in about 60% of children with spasms following therapy with ACTH or corticosteroids, but there is a relapse rate of about 20%. Uncontrolled evidence suggests that intellectual outcome in survivors is better with ACTH than oral corticosteroids.
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