SUDEP in the North American SUDEP Registry The full spectrum of epilepsies
SUDEP in the North American SUDEP Registry The full spectrum of epilepsies
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DOI：
10.1212/wnl.0000000000007778
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发表时间：
2019-07-16
期刊：
影响因子：
9.9
通讯作者：
Devinsky, Orrin
中科院分区：
文献类型：
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作者：
Verducci, Chloe;Hussain, Fizza;Devinsky, Orrin
目的获取癫痫猝死（SUDEP）病例的病历、家庭访谈和死亡相关报告，以更好地了解SUDEP.Methods2011年10月至2018年6月期间所有提交北美SUDEP登记处（NASR）的病例;通过共识审查确定死亡原因。审查了所有SUDEP病例的可用医疗记录、死亡现场调查报告、尸检报告和近亲访谈。癫痫发作类型，脑电图，MRI，和SUDEP分类由2 epileptologist.ResultsThere是237个明确的和可能的情况下，SUDEP 530 NASR参与者。SUDEP死亡者的中位年龄为26岁（范围1-70），38%为女性。在143例有足够信息的患者中，40%为全身性癫痫，60%为局灶性癫痫。SUDEP影响癫痫的全部谱，从具有中央颞区棘波的良性癫痫（n = 3，1%）到难治性癫痫性脑病（n = 27，11%）。大多数（93%）SUDEP是无人目击的; 70%发生在明显的睡眠期间; 69%的患者是俯卧的。只有37%的SUDEP病例服用了最后一剂抗癫痫药物（ASMs）。终生全身强直阵挛发作（GTCS）< 10次者占33%，0次者占4%.ConclusionsNASR参与者通常具有与SUDEP风险相关的临床特征，如年轻成人年龄、ASM不依从和频繁GTCS。然而，相当一部分SUDEP发生在被认为对治疗有反应或患有良性癫痫的患者中。这些结果强调了SUDEP教育在癫痫严重程度范围内的重要性。我们的目标是为研究人员提供NASR数据和生物标本，以促进SUDEP的理解和预防。
ObjectiveTo obtain medical records, family interviews, and death-related reports of sudden unexpected death in epilepsy (SUDEP) cases to better understand SUDEP.MethodsAll cases referred to the North American SUDEP Registry (NASR) between October 2011 and June 2018 were reviewed; cause of death was determined by consensus review. Available medical records, death scene investigation reports, autopsy reports, and next-of-kin interviews were reviewed for all cases of SUDEP. Seizure type, EEG, MRI, and SUDEP classification were adjudicated by 2 epileptologists.ResultsThere were 237 definite and probable cases of SUDEP among 530 NASR participants. SUDEP decedents had a median age of 26 (range 1-70) years at death, and 38% were female. In 143 with sufficient information, 40% had generalized and 60% had focal epilepsy. SUDEP affected the full spectrum of epilepsies, from benign epilepsy with centrotemporal spikes (n = 3, 1%) to intractable epileptic encephalopathies (n = 27, 11%). Most (93%) SUDEPs were unwitnessed; 70% occurred during apparent sleep; and 69% of patients were prone. Only 37% of cases of SUDEP took their last dose of antiseizure medications (ASMs). Reported lifetime generalized tonic-clonic seizures (GTCS) were < 10 in 33% and 0 in 4%.ConclusionsNASR participants commonly have clinical features that have been previously been associated with SUDEP risk such as young adult age, ASM nonadherence, and frequent GTCS. However, a sizeable minority of SUDEP occurred in patients thought to be treatment responsive or to have benign epilepsies. These results emphasize the importance of SUDEP education across the spectrum of epilepsy severities. We aim to make NASR data and biospecimens available for researchers to advance SUDEP understanding and prevention.