Abstracts

Rationale: Recent studies suggest that early introduction of ketogenic diet (KD) could improve seizure control in myoclono-astatic epilepsy (MAE). This multicenter retrospective observational study aimed to identify the benefits of KD use on seizure control and epilepsy, and on developmental outcome in children with resistant MAE.Methods: The data of 50 children diagnosed with severe MAE in the French network of Reference Centers for Rare Epilepsies, and treated with KD between 2000 and 2013, have been reviewed. Seizure frequency, EEG recordings were assessed 2 weeks before KD introduction, 2 and 6 months after, and at last follow-up, with developmental outcome.Results: Patients had a median follow up of 52 months (range 13-136) and received 4.3 antiepileptic drugs [2-9] before KD introduction. 54% of our patients were seizure free after 6 months of KD or more, and 86% experienced more than 70% seizure reduction after 2 months of KD. 44% of them had a clear benefit of early KD treatment (after 4 AEDs failed). Early KD treatment, didn’t lead to higher seizure reduction (p=0.055) but significantly led to remission (p<0.028). 50% patients with resistant MAE had normal development outcome. Early KD treatment, after 3 AEDs failed, is correlated with a better cognitive outcome (p<0.01).Conclusions: Early introduction of KD treatment in resistant MAE has a strong anticonvulsant effect, but also a long-lasting effect, with long term remission and better cognitive outcome, and should prevent from a chronic epileptic encephalopathy.