Abstracts

Rationale: The ketogenic diet (KD) is a therapeutic option in pharmacoresistant epilepsy and is particularly effective in GLUT-1 deficiency syndrome, Myoclonic Atonic Epilepsy (MAE) and a few other specific disorders. It is rarely used as a first-line treatment. The purpose of this study was to look at the impact of KD in treating a broader range of pediatric epilepsy syndromes and its success when used earlier vs later in the course of treatment. Methods: We retrospectively reviewed a consecutive series of patients treated with KD at Children s Memorial Hospital (CMH) from May 2005- August 2009. We recorded gender, age of onset, type and cause of epilepsy, prior treatments, age of diet initiation, impact on seizure frequency, diet ratio, administration route, and side effects. Results: We initiated 64 patients on the KD. Patients followed at other institutions (N=4) or without seizures (N=1 with a metabolic condition) were excluded. Of the remaining 59, 29 (49%) were girls. Average age of onset of epilepsy was 1.1y (range 0-8y) and at initiation 4.5y (range 0.2 to 22y); 20 (34%) were ? 5y. Most patients, 46 (78%), came from CMH; 13 (22%) were referred by outside providers. 24 (41%) patients had received 0-4 treatments prior to initiating the diet; 35 patients had tried 5-11 different treatments. Route of feeding was PO for 40 (59%) and tube for 24 (41%). A ratio of 3:1 to 4:1 was maintained in 84% of patients. Significant improvement in seizure frequency occurred in 42 (71%) including 9 (15%) who became completely seizure-free. Patients referred from outside CMH differed slightly from those from within in that they were less likely to be tube fed 31% vs 44% and more likely to have tried 4+ other tx prior to KD initiation 69% vs 57% (both P-values >0.40). Earlier (<4 tx) vs. later (?4 tx) initiation was marginally associated with better response (83% vs 63%, p=0.09). Positive response was comparable in children initiated at <5y vs ? 5y (72% vs. 70%, N.S.) and in those who were tube vs. PO fed (74% vs. 67%, N.S.). Positive response to KD was comparable across different forms of epilepsy (West, 5/8 (63%); DS, 2/2 (100%); MAE, 5/7 (71%); GLUT1-DS, 2/2 (100%); Jeavons syndrome, 0/1 (0%); Lennox Gastaut Syndrome, 5/6 (83%); and nonspecific epilepsies, 23/33 (70%). Duration of treatment ranged from 0 to 5.5y (mean=2y). 30 (51%) children remain on the diet including 70% of those who showed improvement and 6% of those who did not. One or more side effects was noted in 58%, including constipation (39%); kidney stones (5%); acidosis (21%); nausea/emesis(14%); increased seizures (7%). Conclusions: Ketogenic diet can be used to treat a broad range of pediatric epilepsies. It can be successfully initiated in less severely impaired children who do not require tube feeding and in older children. The ketogenic diet should be considered as a therapeutic option earlier in the course of treatment for children with epilepsy, even in those whose epilepsy syndrome is not precisely characterized.