Mortality in Dravet syndrome

MS Cooper, A Mcintosh, DE Crompton, JM McMahon… - Epilepsy research, 2016 - Elsevier
We measured the mortality rate and the rate of Sudden Unexpected Death in Epilepsy
(SUDEP) in Dravet Syndrome (DS). We studied a cohort of 100 consecutively recruited,
unrelated patients with DS; 87 had SCN1A mutations. Living cases had a median follow-up
of 17 years. Seventeen patients died, at a median age of seven years (inter-quartile range 3–
11 years) with causes of death: 10 SUDEP, four status epilepticus, two drowning and one
asphyxia. The SUDEP classification included three Definite, one Definite Plus and six …