Mortality in Dravet Syndrome: Difference between revisions
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Cooper MS, Mcintosh A, Crompton DE, et al. (2016) Mortality in Dravet Syndrome. Epilepsy Res. 2016 Dec;128:43-47. | |||
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== | https://ac.els-cdn.com/S0920121116302443/1-s2.0-S0920121116302443-main.pdf?_tid=25816326-c591-4cc8-a5e4-44e3268626a8&acdnat=1531182097_cae60ddf6676fc5da9ee8e40c3cb2471 | ||
== | |abstract= | ||
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. | |||
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Dravet syndrome; Epilepsy; Mortality; Sudden unexpected death in epilepsy | |||
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Latest revision as of 17:43, 17 June 2019
Cooper MS, Mcintosh A, Crompton DE, et al. (2016) Mortality in Dravet Syndrome. Epilepsy Res. 2016 Dec;128:43-47.
Abstract: 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.
Keywords: Dravet syndrome; Epilepsy; Mortality; Sudden unexpected death in epilepsy