Literature DB >> 21569025

Ketogenic diet also benefits Dravet syndrome patients receiving stiripentol: a prospective pilot study.

Rima Nabbout1, Cristiana Copioli, Mathilde Chipaux, Nicole Chemaly, Isabelle Desguerre, Olivier Dulac, Catherine Chiron.   

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. Wiley Periodicals, Inc.
© 2011 International League Against Epilepsy.

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Year:  2011        PMID: 21569025     DOI: 10.1111/j.1528-1167.2011.03107.x

Source DB:  PubMed          Journal:  Epilepsia        ISSN: 0013-9580            Impact factor:   5.864


  22 in total

Review 1.  Safety and tolerability of the ketogenic diet used for the treatment of refractory childhood epilepsy: a systematic review of published prospective studies.

Authors:  Qian-Yun Cai; Zhong-Jie Zhou; Rong Luo; Jing Gan; Shi-Ping Li; De-Zhi Mu; Chao-Min Wan
Journal:  World J Pediatr       Date:  2017-07-12       Impact factor: 2.764

2.  Dynamic changes of depolarizing GABA in a computational model of epileptogenic brain: Insight for Dravet syndrome.

Authors:  P Kurbatova; F Wendling; A Kaminska; A Rosati; R Nabbout; R Guerrini; O Dulac; G Pons; C Cornu; P Nony; C Chiron; P Benquet
Journal:  Exp Neurol       Date:  2016-05-28       Impact factor: 5.330

3.  Of fish and men.

Authors:  Gustavo A Patino; Jack M Parent
Journal:  Epilepsy Curr       Date:  2014-03       Impact factor: 7.500

Review 4.  Treatment Strategies for Dravet Syndrome.

Authors:  Kelly G Knupp; Elaine C Wirrell
Journal:  CNS Drugs       Date:  2018-04       Impact factor: 5.749

Review 5.  Current Treatment Strategies and Future Treatment Options for Dravet Syndrome.

Authors:  Julie Ziobro; Krista Eschbach; Joseph E Sullivan; Kelly G Knupp
Journal:  Curr Treat Options Neurol       Date:  2018-10-13       Impact factor: 3.598

Review 6.  Pharmacotherapy for Dravet Syndrome.

Authors:  Adam Wallace; Elaine Wirrell; Daniel L Kenney-Jung
Journal:  Paediatr Drugs       Date:  2016-06       Impact factor: 3.022

Review 7.  Stiripentol : in severe myoclonic epilepsy of infancy (dravet syndrome).

Authors:  Greg L Plosker
Journal:  CNS Drugs       Date:  2012-11       Impact factor: 5.749

Review 8.  Novel animal models of pediatric epilepsy.

Authors:  Stéphane Auvin; Eduardo Pineda; Don Shin; Pierre Gressens; Andrey Mazarati
Journal:  Neurotherapeutics       Date:  2012-04       Impact factor: 7.620

9.  A novel variant in the 3' UTR of human SCN1A gene from a patient with Dravet syndrome decreases mRNA stability mediated by GAPDH's binding.

Authors:  Tao Zeng; Zhao-Fei Dong; Shu-Jing Liu; Rui-Ping Wan; Ling-Jia Tang; Ting Liu; Qi-Hua Zhao; Yi-Wu Shi; Yong-Hong Yi; Wei-Ping Liao; Yue-Sheng Long
Journal:  Hum Genet       Date:  2014-01-25       Impact factor: 4.132

Review 10.  Recent Advances in the Drug Treatment of Dravet Syndrome.

Authors:  Elaine C Wirrell; Rima Nabbout
Journal:  CNS Drugs       Date:  2019-09       Impact factor: 5.749

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