Neurológia pre prax 5/2024

Treatment of Dravet syndrome

Dravet syndrome is a developmental and epileptic encephalopathy starting in infancy and its main features are drug –resistant epilepsy and several co-morbidities. Seizures are typically provoked by increased temperature. The treatment of Dravet syndrome is challenging. The first antiseizure drug should be valproic acid, while clobazam, stiripentol, fenfluramine, canabidiol or topiramate are usually added later. All the patients must have rescue medication for home management of seizures. Sodium channel blockers should not be used for chronic treatment, but phenytoin can be administered to stop status epilepticus. Non-pharmacological treatment of co-morbidities should be addressed as well.

Keywords: Dravet syndrome, stiripentol, fenfluramine, canabidiol