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Current Treatment Strategies and Future Treatment Options for Dravet Syndrome

  • Julie Ziobro
  • Krista Eschbach
  • Joseph E. Sullivan
  • Kelly G. Knupp
Pediatric Neurology (A Yeshokumar, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Pediatric Neurology

Abstract

Purpose of review

Dravet syndrome is a rare but severe genetic epilepsy that has unique treatment challenges. This is a review of current and future potential treatment options.

Recent findings

Treatment for Dravet syndrome should encompass many aspects of the syndrome such as gait, behavior, and nutrition, as well as focus on seizure control. Many sodium channel blockers should be avoided as they are likely to exacerbate seizures. Current options for treatment include valproic acid, clobazam, stiripentol, and ketogenic diet. Testing is underway for several new treatment options with unique mechanisms of action and therapeutic targets, including the serotonin system and genetic modulation.

Summary

Accurate and early diagnosis of Dravet syndrome will lead to avoidance of medications that may exacerbate seizures. Additionally, a multi-disciplinary approach and careful planning for management of episodes of status epilepticus may lead to improved outcomes. Ongoing research for novel approaches to treatment creates optimism for future improvement in outcomes.

Keywords

Dravet syndrome SCN1A Stiripentol Fenfluramine 

Notes

Compliance with Ethical Standards

Conflict of Interest

Kelly G. Knupp reports grants from Zogenix Inc., grants from West Therapeutics, grants from Pediatric epilepsy research fund, personal fees from Stoke therapeutics, and grants from Colorado Department of Public Health, outside the submitted work. Joseph E. Sullivan reports grants from Zogenix, personal fees from Epygenix, and personal fees from Miller Medical, outside the submitted work; and Medical Advisory Board-Dravet Syndrome Foundation. Julie Ziobro and Krista Eschbach each declare no potential conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Julie Ziobro
    • 1
  • Krista Eschbach
    • 2
  • Joseph E. Sullivan
    • 3
  • Kelly G. Knupp
    • 2
  1. 1.Division of Pediatric NeurologyUniversity of MichiganAnn ArborUSA
  2. 2.Children’s Hospital ColoradoUniversity of Colorado DenverAuroraUSA
  3. 3.Benioff Children’s HospitalUniversity of California San FranciscoSan FranciscoUSA

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