New Guidelines Issued on Treatment of Infantile Spasms

Laurie Barclay, MD

May 24, 2004

May 24, 2004 — The American Academy of Neurology (AAN) and the Child Neurology Society (CNS) developed new guidelines for the treatment of infantile spasms, which are published in the May 25 issue of Neurology.

"We wanted to develop this guideline because surveys of pediatric neurologists in the United States, Japan, and the United Kingdom found very different approaches to treatment," lead author Mark Mackay, MBBS, FRACP, from the Royal Children's Hospital in Victoria, Australia, says in an news release. "We wanted to see if the scientific evidence could give us some answers as to the best practices in the treatment of infantile spasms."

Infantile spasms, or West syndrome, is a rare disorder with onset at age four to six months and resolution by age two to four years. Most children with infantile spasms have developmental disabilities later in life. Although the spasms are a type of seizure associated with sudden flexion or extension, they rarely respond to typical antiepileptic drugs.

After review of all of the MEDLINE literature from 1966 and EMBASE literature from 1980 on infantile spasms associated with hypsarrythmia, the authors selected 159 articles for detailed review.

"Unfortunately, there are not many well-designed, prospective studies on infantile spasms," Dr. Mackay says. "More research is needed to answer many of our questions about the treatment of this disorder."

Although the guidelines determined that adrenocorticotropic hormone (ACTH) is probably effective for the short-term treatment of infantile spasms, there is insufficient evidence available to recommend the optimum dose and duration of ACTH treatment.

Vigabatrin is possibly effective for the short-term treatment of infantile spasms and for tuberous sclerosis. Because of possible retinal toxicity of vigabatrin, serial ophthalmologic screening is required, but data are insufficient to recommend the frequency or type of screening.

"This guideline will be helpful for parents because many parents are understandably concerned about the potentially serious side effects of ACTH and vigabatrin," Dr. Mackay says. "Even though there are other anticonvulsant drugs that have less serious side effects, a careful review of the medical literature suggests that ACTH and vigabatrin offer the best chance of seizure control and reversal of the developmental regression that is often associated with this severe form of epilepsy."

According to the guidelines, evidence is insufficient to recommend oral corticosteroids or other treatments for infantile spasms, or to conclude that successful treatment of infantile spasms improves the long-term prognosis.

"There is a great need for more, high-quality studies on the treatment of infantile spasms," says senior author O. Carter Snead III, MD, from the University of Toronto in Ontario, Canada. "There is also a need for the development of an animal model of the disorder so we can investigate how the disease develops and identify new targets for potential treatments."

The Bloorview Children's Hospital Foundation helped support development of these guidelines. Several authors report various financial arrangements with one or more pharmaceutical companies.

Neurology. 2004;62:1668-1681

Reviewed by Gary D. Vogin, MD

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