Diet May Eliminate Spasms for Infants with Epilepsy

Infantile spasms are a severe and potentially devastating epilepsy condition affecting children aged typically 4-8 months. In a new study appearing in Epilepsia, researchers have found that the ketogenic diet, a high fat, low carbohydrate diet more traditionally used for intractable childhood epilepsy, may be an effective first-line treatment option for this condition. The study is the first description of use of the ketogenic diet for infantile spasms before starting medications.

ACTH and vigabatrin are medications that are commonly-used as first treatments worldwide, but these medications can have potentially-serious side effects such as hypertension, gastric ulceration, cortical atrophy and visual field constriction. ACTH, though it is effective in 60-70 percent of cases, costs more than $80,000 for a one-month supply and vigabatrin is not currently available in the U.S. Both drugs have about a 30-40 percent recurrence rate of spasms as well.

"We decided to review our experience at Johns Hopkins using the ketogenic diet to treat infantile spasms before medications were tried and compare this to our use of ACTH over the same time period," says Eric Kossoff, M.D, a pediatric neurologist at Johns Hopkins Hospital and lead author of the study. "We knew that the ketogenic diet worked well for difficult-to-control infantile spasms, so we thought it would also be effective earlier."

If the diet stopped the spasms, infants were kept on it for usually 6 months. The diet worked in 8-of-13 infants within approximately one week. Only 1-of-8 had recurring spasms, and that infant was controlled again with the addition of the medication topiramate. Side effects that were monitored in this study were fewer with the diet than with ACTH, and the recurrence rate was also lower with the diet. In the 5 patients in which the diet did not work, ACTH was started immediately; it worked quickly in 4 of the 5 infants. ACTH did lead to a normal EEG quicker, but there were no obvious differences in development of the children.

Editor’s note: Findings in this study should be interpreted with some caution and viewed as preliminary information due to common limitations of early, pilot studies. In a small number of patients in both treatment groups, a retrospective approach (reviewing what happened after the epilepsy treatments were given), was used and there were some dissimilarities in the clinical characteristics of the two groups.

However, any indication of an effective treatment option for children with this devastating seizure disorder is welcome news. Future prospective studies with a larger number of patients will help to further evaluate and compare the effectiveness of these different treatment options and also detect potentially small but clinically important differences in side effects or in neurodevelopment.

Source: Wiley-Blackwell
Reviewed by: Raman Sankar, M.D., Ph.D., Professor, Division of Pediatric Neurology, UCLA