Spasms typically present during the first of life, most often between 4 and 6 months of age. The spasms result from underlying neurologic disease in a majority of cases, with tuberous sclerosis , cerebral dysgenesis, and hypoxic-ischemic encephalopathy the most frequently identified causes. Hypsarrhythmia consists of a unique, chaotic pattern of asynchronous, high amplitude slow waves and spikes which is peculiar to this disorder. Treatment of infantile spasms is problematic, since response to traditional anticonvulsant agents is poor. Parenteral ACTH is generally considered to be first-line therapy.
Side effects are frequent, including hyppertension, Cushing syndrome, and irritability.
Children with infantile spasms face considerable risks for long term neurologic morbidity.