title: Efficacy of a ketogenic diet in resistant myoclono-astatic epilepsy: A French multicenter retrospective study.
abstract: 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.
authors.affiliation: Hôpital Universitaire de Strasbourg, Centre de Référence des Epilepsies Rares, France.
authors.forename: Elodie
authors.initials: E
authors.lastname: Stenger
authors.affiliation: Hôpital Universitaire de Strasbourg, Centre de Référence des Epilepsies Rares, France.
authors.forename: Mickael
authors.initials: M
authors.lastname: Schaeffer
authors.affiliation: Hôpital Universitaire de Toulouse, France.
authors.forename: Claude
authors.initials: C
authors.lastname: Cances
authors.affiliation: Hôpital Universitaire de Reims, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: Motte
authors.affiliation: Hôpital Robert Debré, Paris, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Auvin
authors.affiliation: Hôpital Universitaire de Lyon, Paris, France.
authors.forename: Dorothée
authors.initials: D
authors.lastname: Ville
authors.affiliation: Hôpital du Kremlin Bicêtre, Paris, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Maurey
authors.affiliation: Hôpital Necker Enfants Malades, Centre de Référence des Epilepsies Rares, Paris, France.
authors.forename: Rima
authors.initials: R
authors.lastname: Nabbout
authors.affiliation: Hôpital Universitaire de Strasbourg, Centre de Référence des Epilepsies Rares, France. Electronic address: anne.desaintmartin@chru-strasbourg.fr.
authors.forename: Anne
authors.initials: A
authors.lastname: de Saint-Martin
mesh_terms: D002675:Child, Preschool; D055423:Diet, Ketogenic / Q000379:methods* / Q000639:trends; D000069279:Drug Resistant Epilepsy / Q000175:diagnosis / Q000178:diet therapy* / Q000453:epidemiology*; D004569:Electroencephalography / Q000639:trends; D004831:Epilepsies, Myoclonic / Q000175:diagnosis / Q000178:diet therapy* / Q000453:epidemiology*; D005260:Female; D005500:Follow-Up Studies; D005602:France / Q000453:epidemiology; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Doose syndrome; Epileptic encephalopathy; Ketogenic diet; Myoclono-astatic epilepsy; Resistant epilepsy