New Set of Recommendations for Better Diagnosis and Management of Dravet Syndrome Developed
A consensus panel of epilepsy specialists, experts in Dravet syndrome, and parents of children with Dravet syndrome came together to develop a set of recommendations for the better diagnosis and management of the condition. The recommendations were published in the journal Pediatric Neurology.
“We were able to identify areas where there was strong consensus that we hope will (1) inform health care providers on optimal diagnosis and management of patients with Dravet syndrome, (2) support reimbursement from insurance companies for genetic testing and Dravet syndrome-specific therapies, and (3) improve quality of life for patients with Dravet syndrome and their families by avoidance of unnecessary testing and provision of an early accurate diagnosis allowing optimal selection of therapeutic strategies,” the authors wrote.
The panel consisted of 13 physicians and five family members who had an enhanced experience and understanding of the condition through the active roles they were playing in Dravet syndrome associations. Three rounds of online questionnaires were conducted to identify areas of consensus and contention about the diagnosis and management of Dravet syndrome.
Strong consensus was reached among panelists in the following areas: typical clinical presentation of Dravet syndrome, range of EEG and MRI findings, need for genetic testing, critical information that should be conveyed to families at the time of diagnosis, priorities for seizure control, factors triggering seizures and recommendations to avoid these, first- and second-line therapies for seizures, requirement and indications for rescue therapy, specific recommendations for screening for other diseases that may co-occur at the same time as Dravet syndrome, and the need for family support.
Consensus was not as strong regarding later therapies, such as vagus nerve stimulation and surgery, and for specific therapies of associated diseases.
Apart from the initial treatment with drugs called benzodiazepines and the use of valproate, no consensus was reached on the best way to manage convulsive status epilepticus in a hospital setting.
Dravet syndrome is a type of childhood epilepsy affecting around one in 40,000 to one in 20.000 children. It is characterised by prolonged seizures that may require emergency intervention. It is usually managed with antiepileptic drugs (AEDs) but these may not be able to suppress seizures completely.
Author: Dr Özge Özkaya
What is Dravet Syndrome?