title: Ketogenic diet also benefits Dravet syndrome patients receiving stiripentol: a prospective pilot study.
abstract: We aimed to test the efficacy of ketogenic diet (KD) in patients with Dravet syndrome (DS) not satisfactorily controlled by antiepileptic drugs (AEDs). We included prospectively 15 DS patients aged >3 years with partial response to AEDs including stiripentol. All patients had a seizure diary and clinical examination with Conners and Achenbach scales before KD, at 1 month following onset and every 3 months thereafter. At 1 month, 10 patients (66%) had a decrease of seizure frequency ≥75%. Efficacy was maintained in eight responders at 3 and 6 months and in six responders at 9 months. Five patients (33%) remained on KD over 12 months, and one was seizure-free. In addition to efficacy on seizure frequency, KD was beneficial on behavior disturbances including hyperactivity. This effect was reported in all responders and in a few nonresponders. KD might have a double effect, on seizure control and on hyperactivity and behavior disturbances in patients with DS.
authors.affiliation: Department of Neuropediatrics, National Referral Center for Rare Epilepsies, Necker-Enfants Malades Hospital, 149 rue de Sévres, Paris, France. rimanabbout@yahoo.com
authors.forename: Rima
authors.initials: R
authors.lastname: Nabbout
authors.forename: Cristiana
authors.initials: C
authors.lastname: Copioli
authors.forename: Mathilde
authors.initials: M
authors.lastname: Chipaux
authors.forename: Nicole
authors.initials: N
authors.lastname: Chemaly
authors.forename: Isabelle
authors.initials: I
authors.lastname: Desguerre
authors.forename: Olivier
authors.initials: O
authors.lastname: Dulac
authors.forename: Catherine
authors.initials: C
authors.lastname: Chiron
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D055423:Diet, Ketogenic; D004148:Dioxolanes / Q000627:therapeutic use*; D004831:Epilepsies, Myoclonic / Q000178:diet therapy* / Q000188:drug therapy; D005260:Female; D006801:Humans; D008297:Male; D010865:Pilot Projects; D011446:Prospective Studies; D012640:Seizures / Q000188:drug therapy* / Q000517:prevention & control; D013577:Syndrome
chemical_list: D000927:Anticonvulsants; D004148:Dioxolanes; C021092:stiripentol