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Epilepsia. 2019 Dec;60 Suppl 3:S39-S48. doi: 10.1111/epi.16334.

Dravet syndrome: Treatment options and management of prolonged seizures.

Author information

1
University College London National Institute for Health Research Biomedical Research Centre Great Ormond Street Institute of Child Health, London, UK.
2
Great Ormond Street Hospital for Children, London, UK.
3
Department of Neurology, J. P. Garrahan Hospital, Capital Federal, Buenos Aires, Argentina.
4
Référence centre for rare épilepsies, department of pediatric neurology, necker enfants malades hospital, aphp, Paris Descartes university, Paris, France.
5
Imagine institute, inserm, U1163, Paris, France.
6
Pediatric Neurology Unit, Bambino Gesù Children's Hospital, Rome, Italy.
7
Pediatric Neurology Unit, A. Meyer Children's Hospital, University of Florence, Florence, Italy.
8
Department of Pediatric Neurology, UZ Leuven, Leuven, Belgium.

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.

KEYWORDS:

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

PMID:
31904119
DOI:
10.1111/epi.16334

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