Infantile spasms are epileptic seizures that are unique to a very limited time frame during infancy. The triad of West syndrome consists of infantile spasms, hypsarrhythmic electroencephalography, and arrested psychomotor development.
Spasms may consist of flexor, extensor, or mixed flexor-extensor types. Infantile spasms are broadly classified into symptomatic and cryptogenic. The list of causes in the symptomatic category is extensive. The severity of the outcome is, in large part, determined by the underlying abnormality, but even in cryptogenic cases the risk of abnormal mental development is high. The mainstay of treatment of infantile spasms remains intramuscular injection with adrenocorticotropin hormone, although there are reports of the successful use of vigabatrin, particularly in cases of infantile spasms secondary to tuberous sclerosis. Other medications have also been reported to be effective in children with infantile spasms.
Much remains unknown about the developmental aspects of the syndrome accounting for its age-specific nature. Similarly, the mechanism of action of adrenocorticotropin hormone therapy remains enigmatic. Prospective, multicenter studies comparing different treatment regimens are needed to determine the optimal management of this condition.
(THE NEUROLOGIST 6:298-301, 2000)