A case-control evaluation of the ketogenic diet versus ACTH for new-onset infantile spasms.
OBJECTIVE
ACTH is currently the standard first-line therapy for new-onset infantile spasms, but it has significant side effects. We hypothesized the ketogenic diet (KD), previously reported as beneficial for intractable infantile spasms, would have similar efficacy, but better tolerability than ACTH when used first-line.


METHODS
We conducted a retrospective chart review of all infants started on the KD (n = 13) and high-dose ACTH (n = 20) for new-onset infantile spasms at our institution since 1996.


RESULTS
Infants were spasm-free in 8 of 13 (62%) infants treated with the KD within 1 month, compared to 18 of 20 (90%) treated initially with ACTH, p = 0.06. When effective, median time to spasm freedom was similar between ACTH and the KD (4.0 vs. 6.5 days, p = 0.18). Those treated with ACTH were more likely to have a normal EEG at 1 month (53% vs. 9%, p = 0.02), however, use of the KD led to EEG normalization within 2-5 months in all eight who became spasm-free. In the five children in whom the KD was unsuccessful, four became spasm-free subsequently with ACTH or topiramate immediately. Side effects (31% vs. 80%, p = 0.006) and relapse rate after initial success (12.5% vs. 33%, p = 0.23) were lower with the KD.


CONCLUSIONS
In this retrospective study, the KD stopped spasms in nearly two-thirds of cases, and had fewer side effects and relapses than ACTH. ACTH normalized the EEG more rapidly, however. Further prospective study of the KD as, with a 2-week time limit if unsuccesful, first-line therapy for infantile spasms is warranted.
Kossoff
Eric H
EH
Department of Neurology, John M. Freeman Pediatric Epilepsy Center, Johns Hopkins Medical Institutes, Baltimore, Maryland 21287-1000, USA. ekossoff@jhmi.edu
Hedderick
Erika F
EF
Turner
Zahava
Z
Freeman
John M
JM
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use*; D000927:Anticonvulsants / Q000627:therapeutic use*; D016022:Case-Control Studies; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D007657:Ketone Bodies / Q000378:metabolism*; D008297:Male; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000178:diet therapy* / Q000188:drug therapy / Q000378:metabolism*
D000927:Anticonvulsants; D007657:Ketone Bodies; D000324:Adrenocorticotropic Hormone