Mechanisms of sudden unexpected death in epilepsy: the pathway to prevention.
Mechanisms of sudden unexpected death in epilepsy: the pathway to prevention.
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DOI：
10.1038/nrneurol.2014.64
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发表时间：
2014-05
期刊：
影响因子：
--
通讯作者：
Richerson GB
中科院分区：
文献类型：
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作者：
Massey CA;Sowers LP;Dlouhy BJ;Richerson GB
癫痫猝死（SUDEP）是难治性癫痫患者死亡的主要原因，但许多医生并不知道这种猝死的高风险。人们普遍认为SUDEP是由于发作后的心脏异常引起的。然而，最近的研究表明，癫痫发作后呼吸抑制很常见，并且可能严重到导致血氧饱和度大幅下降。在这篇综述中，我们总结了发作期和发作后心脏、呼吸和觉醒异常的证据以及这些异常的潜在机制。我们讨论了癫痫引起的死亡的小鼠模型，以及这些模型如何有助于理解 SUDEP 的机制。其中一些是由于与人类综合症对应的基因突变造成的。关于心力衰竭与呼吸骤停作为主要死亡原因的相对重要性仍存在争议。解决这一争议需要同时监测人类近 SUDEP 病例期间的心脏和呼吸参数，以及癫痫引起的死亡期间动物模型的详细病理生理学数据。高危患者的有效预防策略将依赖于定义引发从癫痫发作到死亡的一系列事件的机制。
Sudden unexpected death in epilepsy (SUDEP) is the leading cause of death in patients with refractory epilepsy, and yet many physicians do not know about this high risk of sudden death. It has been widely believed that SUDEP is due to cardiac abnormalities during the post-ictal period. However, recent studies have demonstrated that respiratory depression is common following a seizure, and can be severe enough to cause a substantial decrease in oxygen saturation. In this review we summarize evidence for cardiac, respiratory, and arousal abnormalities during the ictal and post-ictal period and potential mechanisms for these abnormalities. We discuss mouse models of seizure-induced death and how these models are useful for understanding the mechanisms that underlie SUDEP. Some of these are due to genetic mutations that have counterparts in human syndromes. Controversy remains regarding the relative importance of cardiac failure versus respiratory arrest as the primary cause of death. Resolving this controversy will require simultaneous monitoring of cardiac and respiratory parameters during cases of near SUDEP in humans and detailed pathophysiological data from animal models during seizure-induced death. Effective preventive strategies in high-risk patients will rely on defining the mechanisms that initiate the sequence of events that lead from seizures to death.