Over time, with careful delineation of Dravet syndrome, doctors 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. This study proposes an algorithm for management, but appreciate that the positioning of newer agents is yet to be established.
Key Points
- An early accurate diagnosis is key to optimal treatment in Dravet syndrome
- Prompt rescue treatment with personalized protocols for prolonged seizures is key, as well as avoidance of precipitants of seizures
- Clobazam, stiripentol, valproate, and more recently cannabidiol appear to be effective treatments
- Fenfluramine is a promising agent demonstrating specific efficacy in trials and should be considered for the future