Harden Cynthia, Tomson Torbjörn, Gloss David, Buchhalter Jeffrey, Cross J Helen, Donner Elizabeth, French Jacqueline A, Gil-Nagel Anthony, Hesdorffer Dale C, Smithson W Henry, Spitz Mark C, Walczak Thaddeus S, Sander Josemir W, Ryvlin Philippe
From the Department of Neurology (C.H.), Mount Sinai Health System, New York, NY; Department of Clinical Neuroscience (T.T.), Karolinska Institutet, Stockholm, Sweden; Department of Neurology (D.G.), CAMC Physicians, Charleston, WV; Departments of Pediatrics and Clinical Neurosciences (J.B.), Alberta Children's Hospital, University of Calgary, Canada; Department of Clinical Neurosciences, Institute of Child Health (J.H.C.), and Institute of Neurology (J.W.S.), University College London; Great Ormond Street Hospital for Children NHS Foundation Trust (J.H.C.), London, UK; Department of Paediatrics (E.D.), Division of Neurology, The Hospital for Sick Children, University of Toronto, Canada; Department of Neurology (J.A.F.), New York University Langone Comprehensive Epilepsy Center, New York; Department of Neurology (A.G.-N.), Hospital Ruber Internacional, Madrid, Spain; Gertrude H. Sergievsky Center and Department of Epidemiology (D.C.H.), Columbia University Medical Center, New York, NY; Department of General Practice (W.H.S.), University College Cork, Ireland; Anschutz Outpatient Pavilion (M.C.S.), University of Colorado Health, Aurora; Neurology Clinic (T.S.W.), University of Minnesota, Minneapolis; Stichting Epilepsie Instellingen Nederland (SEIN) (J.W.S.), Heemstede, the Netherlands; and the Department of Clinical Neurosciences (P.R.), CHUV, Lausanne, Switzerland.
Neurology. 2017 Apr 25;88(17):1674-1680. doi: 10.1212/WNL.0000000000003685.
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.
Systematic review of evidence; modified Grading Recommendations Assessment, Development, and Evaluation process for developing conclusions; recommendations developed by consensus.
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% confidence interval [CI] 0.16-0.31) (moderate 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).
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.
确定不同癫痫人群中癫痫性猝死（SUDEP）的发病率，并探讨是否已识别出SUDEP的危险因素。
对证据进行系统评价；采用改良的分级推荐评估、发展和评价过程得出结论；通过共识形成推荐意见。
基于12项I级研究得出的发病率结果如下：癫痫患儿（0 - 17岁）的SUDEP风险为0.22/1000患者年（95%置信区间[CI] 0.16 - 0.31）（证据可信度中等）。成人的SUDEP风险增至1.2/1000患者年（95% CI 0.64 - 2.32）（证据可信度低）。SUDEP的主要危险因素是全面强直阵挛发作（GTCS）的发生；SUDEP风险随GTCS发作频率增加而升高（证据可信度高）。
B级：照料癫痫患儿的临床医生应告知家长/监护人，在1年中，SUDEP通常影响4500名儿童中的1名；因此，4500名儿童中有4499名不会受到影响。临床医生应告知成年癫痫患者，SUDEP通常每年影响1000名成年癫痫患者中的1名；因此，每年1000名成年人中有999名不会受到影响。对于持续经历GTCS的癫痫患者，临床医生应继续积极管理癫痫治疗以减少发作和SUDEP风险，同时纳入患者偏好并权衡任何新方法的风险和益处。临床医生应告知癫痫患者，无发作，尤其是无GTCS发作，与降低SUDEP风险密切相关。
