Epilepsy research · 2016
Mortality in Dravet syndrome.
СтатьяНазвание и аннотация: Перевод в очереди
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Cooper Monica S, Mcintosh Anne, Crompton Douglas E, McMahon Jacinta M, Schneider Amy, Farrell Kevin, Ganesan Vijeya, Gill Deepak, Kivity Sara, Lerman-Sagie Tally, McLellan Ailsa, Pelekanos James, Ramesh Venkateswaran, Sadleir Lynette, Wirrell Elaine, Scheffer Ingrid E
Аннотация
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 Probable. The Dravet-specific mortality rate/1000-person-years was 15.84 (98% CI 9.01-27.85). The Dravet-specific SUDEP rate was 9.32/1000-person-years (98% CI 4.46-19.45). The Dravet-specific SUDEP rate is the only documented syndrome-specific SUDEP rate. SUDEP in DS occurs mainly in childhood. It is also the highest SUDEP rate, considerably higher than the recent 5.1 SUDEP rate/1000-person-years for adults with refractory epilepsy.