Dravet Syndrome is characterised by high epilepsy-related premature mortality and a marked young age at death. Sudden Unexpected Death in Epilepsy (SUDEP) is the leading reported cause of death in Dravet syndrome, accounting for nearly half of all deaths. DSEF wants to contribute to better understanding of SUDEP in Dravet Syndrome with the ultimate goal to reduce the number of SUDEP cases dramatically. A working group was installed within the DSEF in 2016 to make an action plan.
What is SUDEP?
It refers to a death in a person with epilepsy in which there is no other clear cause of death (such a cancer, stroke, heart disease or an accident). In cases of SUDEP, there is often evidence of an associated seizure.
The most common criteria used to determine whether a death is due to SUDEP are:
At the moment physiopatological mechanisms that lead to SUDEP are unknown and there are currently no proven ways to prevent it.
International group of collaborating researchers are currently dedicated to uncovering the risk factors, causes, and mechanism of SUDEP to improve future epilepsy treatment and SUDEP prevention. Patients community can help studies sharing data and materials.
DSEF ACTION PLAN
In the general assembly in Prague 2016 DSEF agreed to work on the following goals, as proposed by the SUDEP working group:
Several concrete action points have been formulated to achieve these aims.
 Shmuely S, Sisodiya SM, Gunning WB, Sander JW, Thijs RD. Mortality in Dravet syndrome: A review. Epilepsy & Behavior 2016; 64: 69-74