INFANTILE SPASMS - FAVORABLE RESPONSE TO STEROID THERAPY

Infantile spasms have many causes, but most cases are idiopathic, and the patients with this type are the ones who respond best to steroid therapy. Indeed, in such patients, mental deterioration may sometimes be prevented, or at least arrested, by such treatment. The secondary cases result from a variety of causes; indeed, any insult to the brain during fetal or early infantile life appears capable of evoking this type of epilepsy. In such secondary cases steroid therapy has been disappointing. In spite of early favorable response, the relaspse rate has been high and the prognosis for mentality poor. In a few of these patients I have had the impression that mental retardation was less severe after steroid therapy than it would have been without it, but none of the patients in this series with secondary infantile spasms has achieved normal intelligence with or without steroid therapy. In the group with secondary infantile spasms, the incidence of tuberous sclerosis was remarkably high (7 of 19). This diagnosis is easily missed unless the clinician is aware of the significance of multiple depigmented spots on the skin of an infant with seizures. The drug of choice for the treatment of infantile spasms at this time is hydrocortisone, or its analogues. In selected patients with idiopathic infantile spasms treated early and vigorously not only can we stop the seizures, but we can actually prevent mental deficiency.