Guideline evaluates treatment of infantile spasms

05/17/04

ST. PAUL, Minn. – A new guideline developed by the American Academy of Neurology and the Child Neurology Society evaluates the treatment of infantile spasms. The guideline is published in the May 25 issue of Neurology, the scientific journal of the American Academy of Neurology.

Infantile spasms, also called West syndrome, is a rare disorder that usually begins in infants ages 4 to 6 months and stops by the age of 2 to 4. The spasms are a type of seizure associated with sudden flexion or extension movements. Infantile spasms rarely respond to the usual anti-seizure medications. Most children with infantile spasms have developmental disabilities later in life.

"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," said guideline co-author Mark Mackay, MBBS, FRACP, of the Royal Children's Hospital in Victoria, Australia. "We wanted to see if the scientific evidence could give us some answers as to the best practices in the treatment of infantile spasms."

To develop the guideline, the authors reviewed all of the scientific studies on the topic. "Unfortunately, there are not many well-designed, prospective studies on infantile spasms," Mackay said. "More research is needed to answer many of our questions about the treatment of this disorder."

The guideline determined that adrenocorticotropic hormone (ACTH) is probably effective for the short-term treatment of infantile spasms. However, the guideline states that there is not enough evidence available to recommend the optimum dosage and length of treatment. There is also insufficient evidence to determine whether oral corticosteroids are an effective treatment for infantile spasms.

The drug vigabatrin is possibly effective for the short-term treatment of infantile spasms and is possibly also effective for children with tuberous sclerosis, according to the guideline. The evidence suggests that vision of children taking vigabatrin should be tested regularly, as the drug can affect the retina.

The guideline determined that there is insufficient evidence to recommend other treatments for infantile spasms.

"This guideline will be helpful for parents because many parents are understandably concerned about the potentially serious side effects of ACTH and vigabatrin," Mackay said. "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."

There is insufficient evidence to conclude that successful treatment of infantile spasms improves the long-term prognosis, according to the guideline.

"There is a great need for more, high-quality studies on the treatment of infantile spasms," said guideline co-author O. Carter Snead III, MD, of the Hospital for Sick Children and the University of Toronto in Toronto, Ontario. "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."

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Last reviewed: By John M. Grohol, Psy.D. on 21 Feb 2009
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