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From the Royal Children’s Hospital (Dr. Mackay), Victoria, Australia; Hospital for Sick Children (Drs. Weiss and Snead, T. Adams-Webber and D. Stephens) and Faculty of Medicine (Drs. Weiss and Snead, D. Stephens), University of Toronto, Ontario, Canada; Loma Linda University School of Medicine (Dr. Ashwal), CA; Montefiore Medical Center (Drs. Ballaban-Gill and Shinnar), Albert Einstein College of Medicine, New York; University of California at Irvine (Dr. Baram) and Mattel Children’s Hospital, University of California at Los Angeles (Dr. Shields); Miami Children’s Hospital (Dr. Duchowny), FL; National Institute of Neurological Disorders and Stroke (Dr. Hirtz), NIH, Washington, DC; Medical College of Virginia (Dr. Pellock), Virginia Commonwealth University, Richmond; and Cleveland Clinic Foundation (Dr. Wyllie), OH.
Address correspondence and reprint requests to the Quality Standards Subcommittee of the American Academy of Neurology, 1080 Montreal Avenue, St. Paul, MN 55116.
Objective: To determine the current best practice for treatment of infantile spasms in children.
Methods: Database searches of MEDLINE from 1966 and EMBASE from 1980 and searches of reference lists of retrieved articles were performed. Inclusion criteria were the documented presence of infantile spasms and hypsarrhythmia. Outcome measures included complete cessation of spasms, resolution of hypsarrhythmia, relapse rate, developmental outcome, and presence or absence of epilepsy or an epileptiform EEG. One hundred fifty-nine articles were selected for detailed review. Recommendations were based on a four-tiered classification scheme.
Results: Adrenocorticotropic hormone (ACTH) is probably effective for the short-term treatment of infantile spasms, but there is insufficient evidence to recommend the optimum dosage and duration of treatment. There is insufficient evidence to determine whether oral corticosteroids are effective. Vigabatrin is possibly effective for the short-term treatment of infantile spasm and is possibly also effective for children with tuberous sclerosis. Concerns about retinal toxicity suggest that serial ophthalmologic screening is required in patients on vigabatrin; however, the data are insufficient to make recommendations regarding the frequency or type of screening. There is insufficient evidence to recommend any other treatment of infantile spasms. There is insufficient evidence to conclude that successful treatment of infantile spasms improves the long-term prognosis.
Conclusions: ACTH is probably an effective agent in the short-term treatment of infantile spasms. Vigabatrin is possibly effective.
Received September 3, 2003. Accepted in final form March 17, 2004.
Approved by the Quality Standards Subcommittee on July 26, 2003. Approved by the AAN Practice Committee on November 16, 2003. Approved by the AAN Board of Directors in January, 2004. This practice parameter has been endorsed by the American Epilepsy Society.
Dr. Mackay has received honoraria from GlaxoSmithKline, and Janssen Cilag. Dr. Mackay has no equity, stock, or any other ownership interests in these companies. Dr. Pellock has received grants/research support in excess of $10,000 and is a paid consultant for Abbott Laboratories, Aventis, Carter Wallace (MedPointe), Elan Pharmaceuticals, GlaxoSmithKline, Ortho McNeil/Johnson & Johnson, and UCB Pharmaceuticals. Dr. Shinnar has received grants/research support from Abbott Laboratories, Elan Pharmaceuticals, and Xcel Pharmaceuticals. He is a paid consultant for Abbott Laboratories, Cephalon, Inc., Elan Pharmaceuticals, Ovation, Pfizer Laboratories, and Xcel Pharmaceuticals. He also has received honoraria from Abbott Laboratories, Cephalon, Inc., Elan Pharmaceuticals, Pfizer Inc., the R.W. Johnson Pharmaceutical Research Institute, UCB Pharmaceuticals, Inc., and Xcel Pharmaceuticals. Dr. Shinnar has no equity, stock, or any other ownership interest in any of these companies. Dr. Shields participated in a study of vigabatrin that was partially supported by an unrestricted grant from Aventis.
This article has been cited by other articles:
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 J. M. Wild, C. R. Robson, A. L. Jones, I. A. Cunliffe, and P. E. M. Smith Detecting vigabatrin toxicity by imaging of the retinal nerve fiber layer. Invest. Ophthalmol. Vis. Sci., March 1, 2006; 47(3): 917 - 924. [Abstract] [Full Text] [PDF]
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S. Shinnar and J. M. Pellock The trials and tribulations of pediatric drug trials Neurology, November 8, 2005; 65(9): 1348 - 1349. [Full Text] [PDF]
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 R Gupta and R Appleton Corticosteroids in the management of the paediatric epilepsies Arch. Dis. Child., April 1, 2005; 90(4): 379 - 384. [Abstract] [Full Text] [PDF]
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R. Sankar Initial treatment of epilepsy with antiepileptic drugs: Pediatric issues Neurology, November 23, 2004; 63(10_suppl_4): S30 - S39. [Abstract] [Full Text]
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 Practice Parameter: Treatment of Infantile Spasms Is Underdeveloped Journal Watch Neurology, July 22, 2004; 2004(722): 1 - 1. [Full Text]
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