Quera Salva Maria Antonia, Hartley Sarah, Léger Damien, Dauvilliers Yves A
AP-HP Hôpital Raymond Poincaré, Sleep Unit, Physiology Department, Versailles Saint-Quentin-en-Yvelines University, Garches, France.
Université Paris Descartes, Sorbonne Paris Cité, APHP, Hôtel-Dieu, Centre du Sommeil et de la Vigilance et, Paris, France.
Front Neurol. 2017 Dec 18;8:686. doi: 10.3389/fneur.2017.00686. eCollection 2017.
Several aspects of human physiology and behavior are dominated by 24-h circadian rhythms with key impacts on health and well-being. These include mainly the sleep-wake cycle, vigilance and performance patterns, and some hormone secretions. The rhythms are generated spontaneously by an internal "pacemaker," the suprachiasmatic nuclei within the anterior hypothalamus. This master clock has, for most humans, an intrinsic rhythm slightly longer than 24 h. Daily retinal light exposure is necessary for the synchronization of the circadian rhythms with the external 24-h solar environment. This daily synchronization process generally poses no problems for sighted individuals except in the context of jetlag or working night shifts being conditions of circadian desynchrony. However, many blind subjects with no light perception had periodical circadian desynchrony, in the absence of light information to the master clock leading to poor circadian rhythm synchronization. Affected patients experience cyclical or periodic episodes of poor sleep and daytime dysfunction, severely interfering with social, academic, and professional life. The diagnosis of Non-24 Sleep-Wake Rhythm Disorder, also named free-running disorder, non-entrained disorder, or hypernycthemeral syndrome, remains challenging from a clinical point of view due to the cyclical symptoms and should be confirmed by measurements of circadian biomarkers such as urinary melatonin to demonstrate a circadian period outside the normal range. Management includes behavioral modification and melatonin. Tasimelteon, a novel melatonin receptor 1 and 2 agonist, has demonstrated its effectiveness and safety with an evening dose of 20 mg and is currently the only treatment approved by the FDA and the European Medicines Agency.
人类生理和行为的几个方面受24小时昼夜节律主导，对健康和幸福有着关键影响。这些主要包括睡眠-觉醒周期、警觉性和表现模式以及一些激素分泌。这些节律由位于下丘脑前部的视交叉上核这个内部“起搏器”自发产生。对大多数人来说，这个主时钟的内在节律略长于24小时。每日视网膜接受光照对于昼夜节律与外部24小时的太阳环境同步是必要的。除了在时差反应或夜班工作这种昼夜不同步的情况下，这种每日同步过程对有视力的人通常没有问题。然而，许多没有光感的盲人存在周期性的昼夜不同步，因为主时钟没有接收到光信息，导致昼夜节律同步不佳。受影响的患者会经历睡眠不佳和白天功能障碍的周期性发作，严重干扰社交、学业和职业生活。非24小时睡眠-觉醒节律障碍，也称为自由运行障碍、未同步障碍或超昼夜节律综合征，从临床角度来看，由于其周期性症状，诊断仍然具有挑战性，应通过测量昼夜生物标志物如尿褪黑激素来证实昼夜周期超出正常范围。治疗方法包括行为调整和使用褪黑激素。他司美琼是一种新型的褪黑激素受体1和2激动剂，已证明其在晚上服用20毫克时的有效性和安全性，目前是美国食品药品监督管理局和欧洲药品管理局批准的唯一治疗药物。
