Infantile spasms

Infantile spasms constitute a relatively rare disorder of infancy and early childhood; their onset is usually within the first 6 to 8 months of life. A large percentage of patients with this disorder (85-90 per cent) show various degrees of retardation. Infantile spasms typically occur in clusters immediately on arousal, or soon thereafter, but rarely occur while the infant is actually asleep. The usual interictal EEG pattern associated with infantile spasms is hypsarrhythmia, but infantile spasms may occur in the absence of this EEG pattern. The pathophysiology of infantile spasms is not known, but recent evidence suggests that certain regions in the brain stem that are associated with sleep cycling may be responsible for the clinical and EEG manifestations of this disorder. At present, the only known effective treatment for infantile spasms is ACTH or corticosteroids. The therapeutic efficacy of these two agents is relatively equal, and one drug may be effective if the other drug fails. The effectiveness of certain traditional anticonvulsants (valproic acid and the benzodiazepines) and pyridoxine in the treatment of infantile spasms has not been adequately assessed. The long-term mental and developmental outcome of patients with infantile spasms is poor. The only factor that appears to be important in terms of long-term outcome is whether the patient is initially classified as cryptogenic or symptomatic, with the cryptogenic patients having the better outcomes. Approximately half of the infantile spasm patients will continue to have other types of seizures after their spasms stop.