Abstracts

Rationale: Aim of this study is to investigate the efficacy of ketogenic diet (KD) in children with Dravet syndrome (DS), especially focus on cognitive development outcome. Methods: In this retrospective study, we evaluated the clinical records of 46 patients that met the diagnostic criteria of DS treated with KD. These patients were seen at the department of neurology in our center between February 2005 and May 2011. All the patients underwent cognitive assessment by Bayley scales, WPPSI or WISC. Results: Forty-six subjects (25 boys and 21 girls), with a median (range) follow-up of 24 (4-67) months were identified. Age at seizure onset was a median of 9 (2-60) months. Ages at initiation of the KD were 10 and 165 months (mean, 48 months) and duration of the KD was 4 and 67 months (mean, 24 months). One year after initiating the diet, 32 (70%) of the patients remained on the diet. Eleven (30%) patients were seizure free, 18 (56%) patients had a 75-99% decrease in seizure. Thus 1 year after starting the diet, 29 (90%) children had achieved a >75% decrease in their seizures. 19 patients have been off the diet for > 2 years: six (31%) patients were seizure free, eight (42%) patients had a 75-99% decrease in seizure. After the diet, MDI in Bayley scales(n=9) improved and remained significant in statistically (p=0.049). However, PDI and SQ did not differ significantly as a function of age.Conclusions: We consider that children with DS should be offered the KD for developmental progression, further studies with large patient are necessary.