Practice Guideline Summary: Sudden Unexpected Death in Epilepsy Incidence Rates and Risk Factors: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Epilepsy Society

Cynthia Harden; Torbjörn Tomson; David Gloss; Jeffrey Buchhalter; J Helen Cross; Elizabeth Donner; Jacqueline A French; Anthony Gil-Nagel; Dale C Hesdorffer; W Henry Smithson; Mark C Spitz; Thaddeus S Walczak; Josemir W Sander; Philippe Ryvlin

doi:10.5698/1535-7511.17.3.180

Practice Guideline Summary: Sudden Unexpected Death in Epilepsy Incidence Rates and Risk Factors: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Epilepsy Society

Epilepsy Curr. 2017 May-Jun;17(3):180-187. doi: 10.5698/1535-7511.17.3.180.

Authors

Affiliations

¹ Department of Neurology, Mount Sinai Health System, New York, NY.
² Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
³ Department of Neurology, CAMC Physicians, Charleston, WV.
⁴ Alberta Children's Hospital, Departments of Pediatrics and Clinical Neurosciences, University of Calgary, Calgary, Canada.
⁵ Department of Clinical Neurosciences, University College London - Institute of Child Health, and Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
⁶ Division of Neurology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.
⁷ Department of Neurology, New York University Langone Comprehensive Epilepsy Center, New York, NY.
⁸ Department of Neurology, Hospital Ruber Internacional, Madrid, Spain.
⁹ Gertrude H. Sergievsky Center and Department of Epidemiology, Columbia University Medical Center, New York, NY.
¹⁰ Department of General Practice, University College Cork, Cork, Ireland.
¹¹ Anschutz Outpatient Pavilion, University of Colorado Health, Aurora, CO.
¹² Neurology Clinic, University of Minnesota, Minneapolis, MN.
¹³ University College London Institute of Neurology, London, United Kingdom, and Stichting Epilepsie Instellingen Nederland (SEIN), Heemstede, Netherlands.
¹⁴ Department of Clinical Neurosciences, CHUV, Lausanne, Switzerland.

Abstract

Objective: To determine the incidence rates of sudden unexpected death in epilepsy (SUDEP) in different epilepsy populations and address the question of whether risk factors for SUDEP have been identified.

Methods: Systematic review of evidence; modified Grading Recommendations Assessment, Development and Evaluation process for developing conclusions; recommendations developed by consensus.

Results: Findings for incidence rates based on 12 Class I studies include the following: SUDEP risk in children with epilepsy (aged 0-17 years) is 0.22/1,000 patient-years (95% CI 0.16-0.31) (high confidence in evidence). SUDEP risk increases in adults to 1.2/1,000 patient-years (95% CI 0.64-2.32) (low confidence in evidence). The major risk factor for SUDEP is the occurrence of generalized tonic-clonic seizures (GTCS); the SUDEP risk increases in association with increasing frequency of GTCS occurrence (high confidence in evidence).

Recommendations: Level B: Clinicians caring for young children with epilepsy should inform parents/guardians that in 1 year, SUDEP typically affects 1 in 4,500 children; therefore, 4,499 of 4,500 children will not be affected. Clinicians should inform adult patients with epilepsy that SUDEP typically affects 1 in 1,000 adults with epilepsy per year; therefore, annually 999 of 1,000 adults will not be affected. For persons with epilepsy who continue to experience GTCS, clinicians should continue to actively manage epilepsy therapies to reduce seizures and SUDEP risk while incorporating patient preferences and weighing the risks and benefits of any new approach. Clinicians should inform persons with epilepsy that seizure freedom, particularly freedom from GTCS, is strongly associated with decreased SUDEP risk.