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Title: Mortality in Dravet syndrome.
Austin Authors: Cooper, Monica S;Mcintosh, Anne M;Crompton, Douglas E;McMahon, Jacinta M;Schneider, Amy L ;Farrell, Kevin;Ganesan, Vijeya;Gill, Deepak;Kivity, Sara;Lerman-Sagie, Tally;McLellan, Ailsa;Pelekanos, James;Ramesh, Venkateswaran;Sadleir, Lynette;Wirrell, Elaine;Scheffer, Ingrid E 
Affiliation: Department of Developmental Medicine, Royal Children's Hospital, Melbourne, Australia
Department of Paediatrics, University of Melbourne, Royal Children's Hospital, Melbourne, Australia
Epilepsy Research Centre, Department of Medicine, Austin Health, The University of Melbourne, Heidelberg, Victoria, Australia
Melbourne Brain Centre, The Royal Melbourne Hospital, University of Melbourne, Australia
Department of Neurology, Northern Health, Melbourne, Australia
Division of Neurology, Department of Paediatrics, University of British Columbia, Child and Family Research Institute, Vancouver, Canada
Department of Neurology, Great Ormond Street Hospital and Institute of Child Health, London, United Kingdom
TY Nelson Department of Neurology, The Children's Hospital at Westmead, Sydney, Australia
Institute of Pediatric Neurology, Schneider Children's Medical Centre of Israel, Petach Tikvah, Israel
Pediatric Neurology Unit, Wolfson Medical Centre, Sackler School of Medicine, Tel-Aviv University, Holon, Israel
Department of Paediatric Neurosciences, Royal Hospital For Sick Children, Edinburgh, United Kingdom
Paediatric Neurologist, Brisbane, Queensland, Australia
Department of Paediatric Neurology, Newcastle General Hospital, Newcastle Upon Tyne, United Kingdom
Department of Paediatrics, Wellington Children's Hospital, New Zealand; University of Otago, Wellington, New Zealand
Division of Child and Adolescent Neurology and Epilepsy, Mayo Clinic, Rochester, MN, USA
Murdoch Children's Research Institute, Melbourne, Australia
Issue Date: Dec-2016
Date: 2016
Publication information: Epilepsy research 2016; 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.
DOI: 10.1016/j.eplepsyres.2016.10.006
ORCID: 0000-0002-0776-1203
Journal: Epilepsy research
PubMed URL: 27810515
PubMed URL:
Type: Journal Article
Subjects: Dravet syndrome
Sudden unexpected death in epilepsy
Appears in Collections:Journal articles

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