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 C Wirrell, Ingrid E. Scheffer

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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 languageEnglish (US)
Pages (from-to)43-47
Number of pages5
JournalEpilepsy Research
Volume128
DOIs
StatePublished - Dec 1 2016

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Keywords

  • Dravet syndrome
  • Epilepsy
  • Mortality
  • Sudden unexpected death in epilepsy

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Cooper, M. S., Mcintosh, A., Crompton, D. E., McMahon, J. M., Schneider, A., Farrell, K., Ganesan, V., Gill, D., Kivity, S., Lerman-Sagie, T., McLellan, A., Pelekanos, J., Ramesh, V., Sadleir, L., Wirrell, E. C., & Scheffer, I. E. (2016). Mortality in Dravet syndrome. Epilepsy Research, 128, 43-47. https://doi.org/10.1016/j.eplepsyres.2016.10.006