Mortality in Dravet syndrome

Monica S. Cooper; Anne Mcintosh; Douglas E. Crompton; Jacinta M. McMahon; Amy Schneider; Kevin Farrell; Vijeya Ganesan; Deepak Gill; Sara Kivity; Tally Lerman-Sagie; Ailsa McLellan; James Pelekanos; Venkateswaran Ramesh; Lynette Sadleir; Elaine Wirrell; Ingrid E. Scheffer

doi:10.1016/j.eplepsyres.2016.10.006

Mortality in Dravet syndrome

Monica S. Cooper, Anne Mcintosh, Douglas E. Crompton, Jacinta M. McMahon, Amy Schneider, Kevin Farrell, Vijeya Ganesan, Deepak Gill, Sara Kivity, Tally Lerman-Sagie, Ailsa McLellan, James Pelekanos, Venkateswaran Ramesh, Lynette Sadleir, Elaine Wirrell, Ingrid E. Scheffer

Neurology

Research output: Contribution to journal › Article › peer-review

103 Scopus citations

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.

Original language	English (US)
Pages (from-to)	43-47
Number of pages	5
Journal	Epilepsy Research
Volume	128
DOIs	https://doi.org/10.1016/j.eplepsyres.2016.10.006
State	Published - Dec 1 2016

Keywords

Dravet syndrome
Epilepsy
Mortality
Sudden unexpected death in epilepsy

ASJC Scopus subject areas

Neurology
Clinical Neurology

Access to Document

10.1016/j.eplepsyres.2016.10.006

Cite this

@article{7891ce31d9c149d6b9b26691bd9481eb,

title = "Mortality in Dravet syndrome",

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",

author = "Cooper, {Monica S.} and Anne Mcintosh and Crompton, {Douglas E.} and McMahon, {Jacinta M.} and Amy Schneider and Kevin Farrell and Vijeya Ganesan and Deepak Gill and Sara Kivity and Tally Lerman-Sagie and Ailsa McLellan and James Pelekanos and Venkateswaran Ramesh and Lynette Sadleir and Elaine Wirrell and Scheffer, {Ingrid E.}",

note = "Publisher Copyright: {\textcopyright} 2016 Elsevier B.V.",

year = "2016",

month = dec,

day = "1",

doi = "10.1016/j.eplepsyres.2016.10.006",

language = "English (US)",

volume = "128",

pages = "43--47",

journal = "Journal of Epilepsy",

issn = "0920-1211",

publisher = "Elsevier",

}

TY - JOUR

T1 - Mortality in Dravet syndrome

AU - Cooper, Monica S.

AU - Mcintosh, Anne

AU - Crompton, Douglas E.

AU - McMahon, Jacinta M.

AU - Schneider, Amy

AU - Farrell, Kevin

AU - Ganesan, Vijeya

AU - Gill, Deepak

AU - Kivity, Sara

AU - Lerman-Sagie, Tally

AU - McLellan, Ailsa

AU - Pelekanos, James

AU - Ramesh, Venkateswaran

AU - Sadleir, Lynette

AU - Wirrell, Elaine

AU - Scheffer, Ingrid E.

PY - 2016/12/1

Y1 - 2016/12/1

N2 - 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.

AB - 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.

KW - Dravet syndrome

KW - Epilepsy

KW - Mortality

KW - Sudden unexpected death in epilepsy

UR - http://www.scopus.com/inward/record.url?scp=84994036111&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84994036111&partnerID=8YFLogxK

U2 - 10.1016/j.eplepsyres.2016.10.006

DO - 10.1016/j.eplepsyres.2016.10.006

M3 - Article

C2 - 27810515

AN - SCOPUS:84994036111

SN - 0920-1211

VL - 128

SP - 43

EP - 47

JO - Journal of Epilepsy

JF - Journal of Epilepsy

ER -

Mortality in Dravet syndrome

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this