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 guidelin
Contact: Kathy Stone
American Academy of Neurology