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Practice Parameter: Treatment of Infantile Spasms Is Underdeveloped

Adverse drug effects and uncertain efficacy highlight the need for more research.

West syndrome is a unique epilepsy syndrome characterized by flurries of brief, tonic-like seizures and a severely abnormal EEG. The syndrome is confined to early infancy and often is catastrophic, with resulting arrest or regression of development. Despite the initial description of the condition by West in 1841 (Lancet 1841; 1:724), there is no consensus on how to treat these children. Therapies have included vitamin B6, the ketogenic diet, surgery, steroids, and virtually all of the conventional antiepileptic drugs.

In this practice parameter on the medical treatment of infantile spasms (as West syndrome is now known), the authors reviewed all pertinent research and used an evidence-based classification scheme to assess efficacy. They conclude that adrenocorticotropic hormone (ACTH) probably is effective for short-term treatment of infantile spasm, although there is insufficient evidence to recommend either dosage or duration of therapy. They deem vigabatrin (not licensed for use in the U.S. because of associated retinal toxicity) possibly effective for short-term treatment of spasms. There is insufficient evidence to make recommendations regarding other therapies for infantile spasms.

Comment: It is disappointing that, 163 years after the description of infantile spasms, we can conclude only that ACTH is probably effective and vigabatrin is possibly effective. This is particularly disturbing because both drugs can have serious adverse effects. This important and timely practice parameter emphasizes how little we know about the pathophysiologic basis of infantile spasms. As the authors appropriately note, an animal model must be developed to allow investigation of mechanisms of infantile spasms, which we can hope will lead to novel therapeutics. In addition, more multicenter, prospective studies are urgently needed to identify a better therapy for this devastating condition.

— Gregory L. Holmes, MD

Dr. Holmes is Chief of Neurology, Dartmouth Medical School, Lebanon, NH.

Published in Journal Watch Neurology July 22, 2004

Citation(s):

Mackay MT et al. Practice parameter: Medical treatment of infantile spasms: Report of the American Academy of Neurology and the Child Neurology Society. Neurology 2004 May 25; 62:1668-81.

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