(Shmuely, 2016). This study reviewed more than 70 previously published articles and meeting extracts on mortality in Dravet syndrome and analyzed 177 cases of death in Dravet syndrome. Sudden unexpected death in epilepsy (SUDEP) was the cause of death in nearly half of those cases, followed by status epilepticus in 32%, drowning/accidental death in 8%, infections in 5%, and other causes/unknown causes in 6%. The mean age of death was 8.7 years (with a large standard deviation of 9.8 years), and 73% died before age 10. While this article did not estimate the incidence of mortality in Dravet syndrome, it certainly highlights the large percentage of SUDEP-related deaths and emphasizes the importance of further study in this area.

Shmuely, S., Sisodiya, S.M., Gunning, W.B., Sander, J.W., Thijs, R.D. (2016). Mortality in Dravet syndrome: A review. Epilepsy & Behavior. Oct 9;64(Pt A):69-74. doi: 10.1016/j.yebeh.2016.09.007. https://www.ncbi.nlm.nih.gov/pubmed/27732919