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Treatment Strategies for Dravet Syndrome

Kelly G Knupp¹, Elaine C Wirrell²

¹Departments of Pediatrics and Neurology, School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO, USA.

|March 30, 2018

Insights

Dravet syndrome (DS) is a severe epilepsy in infants, often resistant to treatment. New therapies like fenfluramine and cannabidiol show promise for seizure control and managing co-morbidities.

Area of Science:

Neurology
Pediatric Epilepsy
Clinical Trials

Background:

Dravet syndrome (DS) is a severe, drug-resistant epilepsy beginning in infancy.
Characterized by prolonged, fever-triggered seizures and evolving co-morbidities like intellectual disability and gait issues.
Current treatments offer limited seizure control and must balance efficacy with adverse effects.

Purpose of the Study:

To review current and emerging treatment strategies for Dravet syndrome.
To highlight the importance of managing co-morbidities in DS patients.
To emphasize the need for home rescue therapies and seizure action plans.

Main Methods:

Literature review of existing and investigational therapies for Dravet syndrome.
Analysis of treatment outcomes, focusing on seizure reduction and co-morbidity management.
Evaluation of safety profiles and adverse effects of different treatment modalities.

Main Results:

First-line treatments (clobazam, valproic acid) offer limited efficacy in DS.
Agents like stiripentol, topiramate, levetiracetam, ketogenic diet, and VNS show benefit.
Investigational drugs fenfluramine and cannabidiol demonstrate positive results in clinical trials.
Sodium-channel blockers may exacerbate seizures in DS patients.

Conclusions:

Comprehensive management of Dravet syndrome requires addressing both seizures and co-morbidities.
Emerging therapies offer new hope for improved seizure control in DS.
Standardized home rescue protocols and seizure action plans are crucial for patient safety.

Read full analysis

Abstract:

Dravet syndrome (DS) is a medically refractory epilepsy that onsets in the first year of life with prolonged seizures, often triggered by fever. Over time, patients develop other seizure types (myoclonic, atypical absences, drops), intellectual disability, crouch gait and other co-morbidities (sleep problems, autonomic dysfunction). Complete seizure control is generally not achievable with current therapies, and the goals of treatment are to balance reduction of seizure burden with adverse effects of therapies. Treatment of co-morbidities must also be addressed, as they have a significant impact on the quality of life of patients with DS. Seizures are typically worsened with sodium-channel agents. Accepted first-line agents include clobazam and valproic acid, although these rarely provide adequate seizure control. Benefit has also been noted with stiripentol, topiramate, levetiracetam, the ketogenic diet and vagal nerve stimulation. Several agents presently in development, specifically fenfluramine and cannabidiol, have shown efficacy in clinical trials. Status epilepticus is a recurring problem for patients with DS, particularly in their early childhood years. All patients should be prescribed a home rescue therapy (usually a benzodiazepine) but should also have a written seizure action plan that outlines when rescue should be given and further steps to take in the local hospital if the seizure persists despite home rescue therapy.

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