Dravet syndrome: Treatment options and management of prolonged seizures.

J Helen Cross, Roberto H Caraballo, Rima Nabbout, Federico Vigevano, Renzo Guerrini, Lieven Lagae

Journal: Epilepsia 2020;60 Suppl 3():S39-S48

PMID: 31904119

Full Text on Ovid Technologies, Inc. View on PubMed

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.

Address: University College London National Institute for Health Research Biomedical Research Centre Great Ormond Street Institute of Child Health, London, UK.; Great Ormond Street Hospital for Children, London, UK.; Department of Neurology, J. P. Garrahan Hospital, Capital Federal, Buenos Aires, Argentina.; Référence centre for rare épilepsies, department of pediatric neurology, necker enfants malades hospital, aphp, Paris Descartes university, Paris, France.; Imagine institute, inserm, U1163, Paris, France.; Pediatric Neurology Unit, Bambino Gesù Children's Hospital, Rome, Italy.; Pediatric Neurology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.; Department of Pediatric Neurology, UZ Leuven, Leuven, Belgium.

DOI: 10.1111/epi.16334

Link outs

Free resources

Medical:

https://medlineplus.gov/seizures.html

Subscription / membership required

Full Text Sources:

MeSH Terms: Anticonvulsants, Diet, Ketogenic, Dioxolanes, Epilepsies, Myoclonic, Humans, Seizures, Valproic Acid