Abstracts

RATIONALE: Cerebral dysplasias and other migrational abnormalities are increasingly recognized as an underlying cause of intractable epilepsy. Seizure control with traditional AEDs in these children is difficult and many children are not epilepsysurgery candidates. The ketogenic diet (KD), an alternative treatment for intractable epilepsy has in general an accepted efficacy rate of 50-75% (and 1/3 seizure freedom). However, the success rate in this subset of most intractable epilepsy patients has not been investigated.
METHODS: Children followed in the Pediatric Regional Epilepsy Program at the Children[ssquote]s Hospital of Philadelphia with intractable epilepsy (failed 3 drugs) and dysplasias (diagnosed by MRI) who were not an epilepsy surgery candidate were offered the traditional 4:1 KD. Eleven subjects were enrolled over a 4 year period, 6 girls and 5 boys, age 7.7 (range 1.2-11.3 yrs). Dysplasias included: 2 double cortex syndrome with bilateral band heterotopias; 1 band heterotopia and pachygyria; 2 schizencephaly(1 diffuse involvement and single hemisphere); 1 bilateral polymicrogyria; 1 Aicardi; 4 with multilobar bihemispheric dysgenesis. One subject had a previous failed surgical resection but the others were not considered surgical candidates. One subject had a VNS implanted. Additional diagnoses included: 90% mental retardation, 18% microcephaly, 54% cerebral palsy,and 37% were nonambulating. The average number AEDs tried prior to KD = 7 (range 5-12) and the average AEDs used at the time the KD was started was 2.3 (range 1-4). Average age of first seizure 3.8 yrs (range 0.08-14.8 yrs). Duration of seizures prior to starting the KD was 3.86 yrs (range 1.2-11.3 yrs). The subjects were admitted to the hospital and started on the traditional 4:1 KD preceded by a fast. In subjects with multiple seizure types the most disabling seizure was identified and designated as the target seizure. Efficacy was assessed at 3 mo. Any subject with [gt]50% reduction in seizure frequency was considered a responder.
RESULTS: 63% of these subjects were reponders. Duration on the diet ranged from 1-58 mos. 27% became seizure free and AEDs were sucessfully weaned in these subjects. The seizure free subjects included the two with double cortex syndrome and one with schizencephaly. Only one of the seizure free subjects was successfully weaned off the KD without recurrence of seizures.
CONCLUSIONS: 1. The KD is an efficacious alternative treatment for intractable epilepsy.
2. The efficacy of the KD in children with diffuse cerebral dysgenesis in this series is comparable to that of other intractable epilepsies.
3. Seizure freedom is obtained using the KD with the same frequency(1/4-1/3) in this group of very difficult to treat subjects with epilepsy.
Support: This work is supported in part by 1k23-RR16074-02.