Julie Ziobro1, Krista Eschbach2, Joseph E Sullivan3, Kelly G Knupp4. 1. Division of Pediatric Neurology, University of Michigan, 1540 East Hospital Drive, Ann Arbor, MI, 48109, USA. 2. Children's Hospital Colorado, University of Colorado Denver, Anschutz Medical Campus, 13123 E 16th Ave, Aurora, CO, 80045, USA. 3. Benioff Children's Hospital, University of California San Francisco, 550 16th Street, 4th Floor, San Francisco, CA, 94143, USA. 4. Children's Hospital Colorado, University of Colorado Denver, Anschutz Medical Campus, 13123 E 16th Ave, Aurora, CO, 80045, USA. kelly.knupp@childrenscolorado.org.
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. 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.
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. 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.
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