Comparison of short‐ versus long‐term ketogenic diet for intractable infantile spasms

Abstract

SUMMARYPurpose: To compare the prognoses between short-term (8 months) and conventional long-term (>2 years) trials involving patients with refractory infantile spasms who successfully completed the ketogenic diet (KD). Methods: Of 40 patients who achieved seizure-free outcomes and showed improvement in hypsarrhythmic patterns within 6 months of the KD, with a 3:1 fat to nonfat ratio as an add-on treatment, 16 patients were randomized into the short-term trial group and the diet was tapered throughout two additional months. Twenty-four patients were randomized into a long-term trial group, and 19 patients could successfully discontinue the diet after 2 years. Primary outcome measures included seizure relapse and frequency of 35 patients for >12 months after successful completion of the KD. Key Findings: Of 16 patients in the short-term trial group, two patients relapsed with clusters of spasms, and one patient had recurrence of occasional focal seizures. Of 19 patients in the long-term trial group, two patients progressed to Lennox-Gastaut syndrome and one patient experienced recurrence of occasional focal seizures with secondary generalization. An early response to the KD, evidenced by short latency before seizure freedom and disappearance of hypsarrythmia and cryptogenic etiology, may indicate a successful early discontinuation of the KD. Significant growth failure was complicated only in conventional long-term trial group. Significance: Use of the KD for only 8 months in children who become spasm-free appears to be justified, with similar outcomes, recurrence rate, and less growth disturbance than a longer-term, traditional use.

Ketogenic diet guidelines for infants with refractory epilepsy

“…However, there is evidence that they may tolerate a reduced ratio (with increased carbohydrate intake) in a longer term. In the case of IS, a study randomizing the seizure-free patients to either discontinue the diet in the short-term (8 months) or long term (2 years) showed no difference in the rate of seizure relapse between the two groups [58]. Considering the possible occurrence of side effects including growth consequences, a shorter duration of KD in IS might be considered.…”

Summary of recommendations for the management of infantile seizures: TaskForceReport for theILAE Commission ofPediatrics

“…Class 4 evidence supports the role of the ketogenic diet for infants younger than 1 year of age, and class 3 evidence for children aged 12-24 months. [12][13][14][15][16][17][18][19][20] There is evidence that ketosis can be sustained in infants.…”

Danger in the Pipeline for the Ketogenic Diet?

“…Lastly, even though the study from Sweden suggested that carotid stiffness may improve after 12 months of dietary therapy, it would seem logical, based on the Italian results, to consider diet discontinuation at 1-2 years if medically indicated to allow blood vessels to normalize. For some conditions such as infantile spasms, 6 months only of dietary therapy may be sufficient (8).…”