title: Modified Atkins diet is an effective treatment for children with Doose syndrome.
abstract: 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.
authors.affiliation: Epilepsy Centre Kork, Clinic for Children and Adolescents, Kehl-Kork, Germany.
authors.forename: Adelheid
authors.initials: A
authors.lastname: Wiemer-Kruel
authors.affiliation: Department for Neuropediatrics, Children's University Hospital, Innsbruck, Austria.
authors.forename: Edda
authors.initials: E
authors.lastname: Haberlandt
authors.affiliation: Department of Pediatrics, Hannover Medical School, Hannover, Germany.
authors.forename: Hans
authors.initials: H
authors.lastname: Hartmann
authors.affiliation: Department for Neuropediatrics, Children's University Hospital, Zürich, Switzerland.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Wohlrab
authors.affiliation: Epilepsy Centre Kork, Clinic for Children and Adolescents, Kehl-Kork, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Bast
mesh_terms: D002648:Child; D002675:Child, Preschool; D050528:Diet, Carbohydrate-Restricted / Q000379:methods*; D004831:Epilepsies, Myoclonic / Q000178:diet therapy*; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: Ketogenic diet; Myoclonic astatic epilepsy; Pharmacoresistance