LAUSR

BACKGROUND The ketogenic diet is an accepted treatment modality in refractory childhood epilepsy. In this study, we analyzed the efficacy and tolerability of the ketogenic and modified Atkins diets in children with refractory epilepsy of genetic etiology and studied the effect of the diet on seizure frequency. METHODS The records of children with a genetic etiology for refractory epilepsy treated with ketogenic and modified Atkins diet between September 2005 and July 2016 were reviewed. We documented age of seizure and diet onset, seizure characteristics, and specific genetic etiology. The proportion of children remaining on the diet and responder rates (greater than 50% seizure reduction) were noted at one, three, six, 12, and 24 months after diet initiation. Tolerability and safety profile were also recorded. RESULTS Fifty-nine children with a genetic etiology (63% females, median age at diet onset 2.2 years) were initiated on the diet at our center. Fifty-three (90%) were started on a traditional ketogenic diet, whereas six started a modified Atkins diet. The adverse events at the initiation of diet were vomiting (24%), hypoglycemia (15%), and refusal to feed (11%). Three children stopped the diet before discharge because of poor compliance, severe reflux, and ketoacidosis (n = 1 each). The proportion of children remaining on the diet at one, three, six, 12, and 24 months was 95%, 86%, 69%, 64%, and 47%. The responder rates were 63%, 61%, 54%, 53%, and 41% at one, three, six, 12, and 24 months, respectively. CONCLUSIONS The ketogenic diet is an effective treatment modality in children with refractory epilepsy of genetic etiology.