Mortality in Dravet syndrome

doi:10.1016/j.eplepsyres.2016.10.006

. 2016 Dec:128:43-47.

doi: 10.1016/j.eplepsyres.2016.10.006. Epub 2016 Oct 26.

Mortality in Dravet syndrome

Monica S Cooper¹, Anne Mcintosh², Douglas E Crompton³, Jacinta M McMahon⁴, Amy Schneider⁴, Kevin Farrell⁵, Vijeya Ganesan⁶, Deepak Gill⁷, Sara Kivity⁸, Tally Lerman-Sagie⁹, Ailsa McLellan¹⁰, James Pelekanos¹¹, Venkateswaran Ramesh¹², Lynette Sadleir¹³, Elaine Wirrell¹⁴, Ingrid E Scheffer¹⁵

Affiliations

¹ Department of Developmental Medicine, Royal Children's Hospital, Melbourne, Australia,; Department of Paediatrics, University of Melbourne, Royal Children's Hospital, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
² Epilepsy Research Centre, Department of Medicine, University of Melbourne, Austin Health, Australia; Melbourne Brain Centre, The Royal Melbourne Hospital, University of Melbourne, Australia.
³ Epilepsy Research Centre, Department of Medicine, University of Melbourne, Austin Health, Australia; Department of Neurology, Northern Health, Melbourne, Australia.
⁴ Epilepsy Research Centre, Department of Medicine, University of Melbourne, Austin Health, Australia.
⁵ Division of Neurology, Department of Paediatrics, University of British Columbia, Child and Family Research Institute, Vancouver, Canada.
⁶ Department of Neurology, Great Ormond Street Hospital and Institute of Child Health, London, United Kingdom.
⁷ TY Nelson Department of Neurology, The Children's Hospital at Westmead, Sydney, Australia.
⁸ Institute of Pediatric Neurology, Schneider Children's Medical Centre of Israel, Petach Tikvah, Israel.
⁹ Pediatric Neurology Unit, Wolfson Medical Centre, Sackler School of Medicine, Tel-Aviv University, Holon, Israel.
¹⁰ Department of Paediatric Neurosciences, Royal Hospital For Sick Children, Edinburgh, United Kingdom.
¹¹ Paediatric Neurologist, Brisbane, Queensland, Australia.
¹² Department of Paediatric Neurology, Newcastle General Hospital, Newcastle Upon Tyne, United Kingdom.
¹³ Department of Paediatrics, Wellington Children's Hospital, New Zealand; University of Otago, Wellington, New Zealand.
¹⁴ Division of Child and Adolescent Neurology and Epilepsy, Mayo Clinic, Rochester, MN, USA.
¹⁵ Department of Paediatrics, University of Melbourne, Royal Children's Hospital, Melbourne, Australia; Epilepsy Research Centre, Department of Medicine, University of Melbourne, Austin Health, Australia; Florey Institute of Neuroscience and Mental Health, University of Melbourne, Australia. Electronic address: [email protected].

PMID: 27810515
DOI: 10.1016/j.eplepsyres.2016.10.006

Mortality in Dravet syndrome

Monica S Cooper et al. Epilepsy Res. 2016 Dec.

. 2016 Dec:128:43-47.

doi: 10.1016/j.eplepsyres.2016.10.006. Epub 2016 Oct 26.

Authors

Affiliations

¹ Department of Developmental Medicine, Royal Children's Hospital, Melbourne, Australia,; Department of Paediatrics, University of Melbourne, Royal Children's Hospital, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
² Epilepsy Research Centre, Department of Medicine, University of Melbourne, Austin Health, Australia; Melbourne Brain Centre, The Royal Melbourne Hospital, University of Melbourne, Australia.
³ Epilepsy Research Centre, Department of Medicine, University of Melbourne, Austin Health, Australia; Department of Neurology, Northern Health, Melbourne, Australia.
⁴ Epilepsy Research Centre, Department of Medicine, University of Melbourne, Austin Health, Australia.
⁵ Division of Neurology, Department of Paediatrics, University of British Columbia, Child and Family Research Institute, Vancouver, Canada.
⁶ Department of Neurology, Great Ormond Street Hospital and Institute of Child Health, London, United Kingdom.
⁷ TY Nelson Department of Neurology, The Children's Hospital at Westmead, Sydney, Australia.
⁸ Institute of Pediatric Neurology, Schneider Children's Medical Centre of Israel, Petach Tikvah, Israel.
⁹ Pediatric Neurology Unit, Wolfson Medical Centre, Sackler School of Medicine, Tel-Aviv University, Holon, Israel.
¹⁰ Department of Paediatric Neurosciences, Royal Hospital For Sick Children, Edinburgh, United Kingdom.
¹¹ Paediatric Neurologist, Brisbane, Queensland, Australia.
¹² Department of Paediatric Neurology, Newcastle General Hospital, Newcastle Upon Tyne, United Kingdom.
¹³ Department of Paediatrics, Wellington Children's Hospital, New Zealand; University of Otago, Wellington, New Zealand.
¹⁴ Division of Child and Adolescent Neurology and Epilepsy, Mayo Clinic, Rochester, MN, USA.
¹⁵ Department of Paediatrics, University of Melbourne, Royal Children's Hospital, Melbourne, Australia; Epilepsy Research Centre, Department of Medicine, University of Melbourne, Austin Health, Australia; Florey Institute of Neuroscience and Mental Health, University of Melbourne, Australia. Electronic address: [email protected].

PMID: 27810515
DOI: 10.1016/j.eplepsyres.2016.10.006

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.

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