Abstract:

Infantile Spasms is a rare disorder consisting of epileptic spasms that occur before the age of one year. This severe epilepsy affects about 0.31 in 1000 births with an average age at onset of 6 months. Treatment usually consists of hormonal therapies (adrenocorticotropic hormone or corticosteroids) or vigabatrin. Decisions about choice of therapy are complicated because these first-line treatments can have significant side effects, are often expensive, and there are few randomized, controlled, blinded studies to guide therapy. Hormonal therapies are the most common first-line treatments and multiple trials suggest that they are the most effective. Vigabatrin may be more effective in treating infantile spasms in patients with tuberous sclerosis. Studies evaluating long-term cognitive outcomes in patients with infantile spasms have failed to show that any therapy produces superior long-term cognitive outcomes in all types of patients. However, there are some data suggesting that early treatment with hormonal therapies improves outcomes in patients for whom a cause of infantile spasms is never found. In this article, we review the evidence for available treatments for infantile spasms.

Dr. Knupp has received research funding from Zogenix that is unrelated to the content of this article.
Dr. Smith has no relevant conflicts to disclose.

Keywords:

infantile spasms, ACTH, vigabatrin, prednisolone, prednisone, West Syndrome,Anschutz Medical Campus, Aurora, CO