Saturday, February 18, 2017

Ketogenic diet in resistant myoclono-astatic epilepsy

Elodie Stenger, Mickael Schaeffer, Claude Cances, Jacques Motte, Stéphane Auvin, Dorothée Ville, Hélène Maurey, Rima Nabbout, Anne de Saint-Martin.  Efficacy of a ketogenic diet in resistant myoclono-astatic epilepsy: A French multicenter retrospective study.  Epilepsy Research.  In press.

•A Ketogenic diet has a strong anticonvulsant effect in myoclono–astatic epilepsy
•Early introduction of a ketogenic diet after three antiepileptic drugs allows for a high rate of remission
•A good cognitive outcome is correlated with an early introduction of a ketogenic diet.

Recent studies have suggested that the early introduction of a ketogenic diet (KD) could improve seizure control in myoclono-astatic epilepsy (MAE). This multicenter study sought to identify the benefits of KD use on seizure control and epilepsy and on developmental outcomes in children with resistant MAE.

Fifty children who were diagnosed with severe MAE in the French network of Reference Centers for Rare Epilepsies and who were treated with KD between 2000 and 2013 were included in this study. The seizure frequency and EEG recordings were assessed two weeks before KD introduction, 2 and 6 months after, and during the last follow-up, which also included an assessment of developmental outcome.

Patients had a median follow up of 52 months (range 13 −136) and received 4.3 antiepileptic drugs [2-9] before KD introduction. Fifty-four percent (54%) of our patients were seizure-free after 6 months of KD or more, and 86% experienced more than a 70% seizure reduction after 2 months of KD. Forty-four percent (44%) of them had a clear benefit of early KD treatment (after four AEDs failed). Early KD treatment did not result in a greater seizure reduction (p = 0.055), but significantly resulted in remission (p < 0.028). Fifty percent of patients with resistant MAE had normal development outcomes. Earlier KD treatment, after three AEDs failed, was correlated with a better cognitive outcome (p < 0.01).

Early introduction of KD treatment in resistant MAE has a strong, persistent anticonvulsant effect with long-term remission and better cognitive outcomes.

Courtesy of:


  1. Wiemer-Kruel A, Haberlandt E, Hartmann H, Wohlrab G, Bast T. Modified Atkins diet is an effective treatment for children with Doose syndrome. Epilepsia. 2017 Feb 23. doi: 10.1111/epi.13701. [Epub ahead of print]

    Children with myoclonic astatic epilepsy (MAE; Doose syndrome) whose seizures do not respond immediately to standard antiepileptic drugs (AEDs) are at high risk of developing an epileptic encephalopathy with cognitive decline. A classic ketogenic diet (KD) is a highly effective alternative to AEDs. To date, there are only limited data on the effectiveness of the modified Atkins diet (MAD), which is less restrictive and more compatible with daily life. We report findings from a retrospective study on 30 MAE patients treated with MAD.
    Four participating centers retrospectively identified all patients with MAE in whom a MAD had been started before June 2015. Seven children were recruited from a cohort included in an open prospective controlled trial. A retrospective review of all available charts was performed in the other patients.
    Thirty patients (24 boys) were included. Mean age at epilepsy onset was 3.1 years (range 1.5-5.6). MAD was started at a mean age of 4.5 years (range 2.2-9.1) after the children had received an average of six different AEDs (range 2-15). Mean MAD observation time was 18.7 months (range 1.5-61.5). Twenty of 30 patients were still on MAD at the end of study (duration range 1.5-61.5, mean 18.5 months). MAD was stopped without relapse in three patients after sustained seizure freedom for >2 years. For the other seven cases, ineffectiveness (three patients), loss of efficacy (two), or noncompliance (two) led to termination. No severe adverse effects were noted. By the end of the observation period, 25 (83%) of 30 patients experienced a seizure reduction by ≥50% and 14 (47%) of 30 were seizure-free. None of the evaluated factors differed significantly between the groups of seizure-free and non-seizure-free children.
    MAD is an effective treatment for MAE. It should be considered as an alternative to AEDs or the more restrictive classic ketogenic diet.

  2. Simard-Tremblay E, Berry P, Owens A, Cook WB, Sittner HR, Mazzanti M, Huber J, Warner M, Shurtleff H, Saneto RP. High-fat diets and seizure control in myoclonic-astatic epilepsy: a single center's experience. Seizure. 2015 Feb;25:184-6.

    To determine the efficacy of the Modified Atkins Diet (MAD) and Ketogenic Diet (KD) in seizure control within a population of myoclonic-astatic epilepsy (MAE) patients.
    This was a retrospective, single center study evaluating the seizure control by high fat diets. Seizure diaries kept by the parents performed seizure counts. All patients met the clinical criteria for MAE.
    Nine patients met the clinical criteria. We found that both the MAD and KD were efficacious in complete seizure control and allowed other medications to be stopped in seven patients. Two patients had greater than 90% seizure control without medications, one on the KD and the other on the MAD. Seizure freedom has ranged from 13 to 36 months, and during this time four patients have been fully weaned off of diet management. One patient was found to have a mutation in SLC2A1.
    Our results suggest that strictly defined MAE patients respond to the MAD with prolonged seizure control. Some patients may require the KD for seizure freedom, suggesting a common pathway of increased requirement for fats. Once controlled, those fully responsive to the Diet(s) could be weaned off traditional seizure medications and in many, subsequently off the MAD or KD.