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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. | URI: | https://ahro.austin.org.au/austinjspui/handle/1/17144 | DOI: | 10.1016/j.eplepsyres.2016.10.006 | ORCID: | 0000-0002-0776-1203 0000-0002-5020-260X 0000-0002-2859-132X 0000-0002-2311-2174 |
Journal: | Epilepsy research | PubMed URL: | 27810515 | PubMed URL: | https://pubmed.ncbi.nlm.nih.gov/27810515 | Type: | Journal Article | Subjects: | Dravet syndrome Epilepsy Mortality Sudden unexpected death in epilepsy |
Appears in Collections: | Journal articles |
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