Efficacy of the ketogenic diet for infantile spasms.
OBJECTIVE
The objective of this study was to determine whether the ketogenic diet is safe, well-tolerated, and efficacious in the treatment of infantile spasms.


METHODS
During a 4-year period, 23 children with infantile spasms, aged 5 months to 2 years, were started on the ketogenic diet; 9 (39%) had symptomatic infantile spasms, and 16 (70%) had hypsarrhythmia. Children had an average prediet exposure to 3.3 anticonvulsants. Two children were enrolled before any medication had been tried. Seizure reduction was analyzed retrospectively, using parent reports and electroencephalograms (EEGs) when available.


RESULTS
At 3, 6, 9, and 12 months, 38%, 39%, 53%, and 46%, respectively, of all patients currently on the diet were >90% improved (3 were seizure-free at 12 months); 67%, 72%, 93%, and 100% were >50% improved. Fifty-six percent remained on the diet at 12 months, 46% of whom were >90% improved and 100% were >50%. Fifty percent of those with hypsarrhythmia and follow-up EEGs had EEG improvement. Fifty-seven percent had their medications reduced or discontinued by 12 months. Fifty-seven percent had improvement in development, which was correlated with seizure control. Independent factors that predicted improvement included age younger than 1 year and previous exposure to 3 or fewer anticonvulsants. No child has died, and 7 children had diet-related adverse reactions (nephrolithiasis, gastroesophageal reflux).


CONCLUSIONS
The ketogenic diet is a safe, well-tolerated, and possibly effective potential alternative to other therapies for infantile spasms.
Kossoff
Eric H
EH
Department of Neurology, Pediatric Epilepsy Center, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA. ekossoff@jhmi.edu
Pyzik
Paula L
PL
McGrogan
Jane R
JR
Vining
Eileen P G
EP
Freeman
John M
JM
D002675:Child, Preschool; D004040:Dietary Carbohydrates / Q000008:administration & dosage; D004041:Dietary Fats / Q000008:administration & dosage; D004044:Dietary Proteins / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D005526:Food, Formulated; D006801:Humans; D007223:Infant; D007662:Ketosis / Q000139:chemically induced*; D008297:Male; D018570:Risk Assessment; D013036:Spasms, Infantile / Q000178:diet therapy*; D016896:Treatment Outcome
D004040:Dietary Carbohydrates; D004041:Dietary Fats; D004044:Dietary Proteins