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Dravet syndrome: Management and prognosis

Authors
Fabio A Nascimento, MD
Danielle M Andrade, MD, MSc, FRCPC.
Section Editor
Douglas R Nordli, Jr, MD
Deputy Editor
April F Eichler, MD, MPH

INTRODUCTION

Dravet syndrome (DS) (OMIM # 607208), previously known as severe myoclonic epilepsy of infancy (SMEI), is a rare pediatric genetic epilepsy syndrome characterized by refractory epilepsy and neurodevelopmental problems beginning in infancy. Mutations in the alpha-1 subunit of the voltage-gated sodium channel (SCN1A) gene are identified in 70 to 80 percent of patients with DS.

Drug resistance is a well-recognized feature of seizures in this syndrome, and antiseizure drug therapies have overall limited efficacy. Nonetheless, every effort should be made to avoid seizure triggers and to control seizures and status epilepticus as best as possible. Pharmacologic therapy remains the mainstay of treatment, and ketogenic diet and neuromodulation are viable options in selected patients.

The goals of treatment are to reduce both the length and number of seizures and prevent status epilepticus, limit adverse effects of antiseizure drugs to promote better neurocognitive development, and improve quality of life.

The management and prognosis of DS will be reviewed here. The epidemiology, genetics, clinical features, and diagnosis of DS are reviewed separately. (See "Dravet syndrome: Genetics, clinical features, and diagnosis".)

AVOIDANCE OF SEIZURE TRIGGERS

Patients with Dravet syndrome (DS) are more sensitive to certain seizure triggers than the general epilepsy population, and anticipatory guidance about common triggers is an important component of management.

                       

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Literature review current through: Nov 2016. | This topic last updated: Wed Sep 21 00:00:00 GMT+00:00 2016.
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