Literature DB >> 31904119

Dravet syndrome: Treatment options and management of prolonged seizures.

J Helen Cross1,2, Roberto H Caraballo3, Rima Nabbout4,5, Federico Vigevano6, Renzo Guerrini7, Lieven Lagae8.   

Abstract

Over time, with careful delineation of Dravet syndrome, we have gained experience in treatments most likely to lead to improvement in seizures, as well as those that should be avoided. Sodium valproate, clobazam, stiripentol, and topiramate are all medications that may lead to benefit, as well as the ketogenic diet. Bromides may be utilized in resistant cases. However, equally important are outlining prompt rescue treatment for prolonged seizures and avoidance of precipitants. Newer agents including cannabidiol and fenfluramine have been demonstrated to be of benefit in clinical trials. We propose an algorithm for management, but appreciate that the positioning of newer agents is yet to be established. Wiley Periodicals, Inc.
© 2019 International League Against Epilepsy.

Entities:  

Keywords:  Dravet syndrome; antiepileptic drugs; cannabidiol; fenfluramine; stiripentol; treatment

Mesh:

Substances:

Year:  2019        PMID: 31904119     DOI: 10.1111/epi.16334

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


  18 in total

1.  NaV1.1 haploinsufficiency impairs glutamatergic and GABAergic neuron function in the thalamus.

Authors:  Carleigh Studtmann; Marek Ladislav; Mackenzie A Topolski; Mona Safari; Sharon A Swanger
Journal:  Neurobiol Dis       Date:  2022-02-24       Impact factor: 5.996

Review 2.  Dravet Syndrome: Novel Approaches for the Most Common Genetic Epilepsy.

Authors:  Lori L Isom; Kelly G Knupp
Journal:  Neurotherapeutics       Date:  2021-08-10       Impact factor: 6.088

Review 3.  Precision medicine for genetic epilepsy on the horizon: Recent advances, present challenges, and suggestions for continued progress.

Authors:  Juliet K Knowles; Ingo Helbig; Cameron S Metcalf; Laura S Lubbers; Lori L Isom; Scott Demarest; Ethan M Goldberg; Alfred L George; Holger Lerche; Sarah Weckhuysen; Vicky Whittemore; Samuel F Berkovic; Daniel H Lowenstein
Journal:  Epilepsia       Date:  2022-07-17       Impact factor: 6.740

4.  Dravet Syndrome-The Polish Family's Perspective Study.

Authors:  Justyna Paprocka; Anita Lewandowska; Piotr Zieliński; Bartłomiej Kurczab; Ewa Emich-Widera; Tomasz Mazurczak
Journal:  J Clin Med       Date:  2021-04-28       Impact factor: 4.241

5.  A ketogenic diet protects DBA/1 and Scn1aR1407X/+ mice against seizure-induced respiratory arrest independent of ketosis.

Authors:  Megan S Crotts; YuJaung Kim; Eduardo Bravo; George B Richerson; Frida A Teran
Journal:  Epilepsy Behav       Date:  2021-09-29       Impact factor: 2.937

6.  The Recommendations for the Management of Chinese Children With Epilepsy During the COVID-19 Outbreak.

Authors:  Baiyu Chen; Miriam Kessi; Shimeng Chen; Juan Xiong; Liwen Wu; Xiaolu Deng; Lifen Yang; Fang He; Fei Yin; Jing Peng
Journal:  Front Pediatr       Date:  2020-08-25       Impact factor: 3.569

7.  Recommendations for the treatment of epilepsy in adult and pediatric patients in Belgium: 2020 update.

Authors:  Susana Ferrao Santos; Anna C Jansen; Lieven Lagae; Benjamin Legros; Sarah Weckhuysen; Paul Boon
Journal:  Acta Neurol Belg       Date:  2020-10-13       Impact factor: 2.396

8.  Development of an antiseizure drug screening platform for Dravet syndrome at the NINDS contract site for the Epilepsy Therapy Screening Program.

Authors:  Chelsea D Pernici; Jeffrey A Mensah; E Jill Dahle; Kristina J Johnson; Laura Handy; Lauren Buxton; Misty D Smith; Peter J West; Cameron S Metcalf; Karen S Wilcox
Journal:  Epilepsia       Date:  2021-05-17       Impact factor: 6.740

9.  Fenfluramine: New Treatment for Seizures in Dravet Syndrome.

Authors:  Joanna Garcia Pierce; Divakar S Mithal
Journal:  Pediatr Neurol Briefs       Date:  2020-03-12

10.  Efficacy of Fenfluramine and Norfenfluramine Enantiomers and Various Antiepileptic Drugs in a Zebrafish Model of Dravet Syndrome.

Authors:  Jing Li; Maxim Nelis; Jo Sourbron; Daniëlle Copmans; Lieven Lagae; Deirdre Cabooter; Peter A M de Witte
Journal:  Neurochem Res       Date:  2021-05-26       Impact factor: 3.996

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