Department of Pharmacology, University of Cambridge, Tennis Court Road, Cambridge CB2 1PD, UK.
Exp Neurol. 2013 May;243:34-44. doi: 10.1016/j.expneurol.2012.10.014. Epub 2012 Oct 22.
Huntington's disease is a progressive neurological disorder that starts insidiously with motor, cognitive or psychiatric disturbance, and progresses through a distressing range of symptoms to end with a devastating loss of function, both motor and executive. There is a growing awareness that, in addition to cognitive and psychiatric symptoms, there are other important non-motor symptoms in HD, including sleep and circadian abnormalities. It is not clear if sleep-wake changes are caused directly by HD gene-related pathology, or if they are simply a consequence of having a neurodegenerative disease. From a patient point of view, the answer is irrelevant, since sleep and circadian disturbances are deleterious to good daily living, even in neurologically normal people. The assumption should be that, at the very least, sleep and/or circadian disturbance in HD patients will contribute to their symptoms. At worst, they may contribute to the progressive decline in HD. Here I review the state of our understanding of sleep and circadian abnormalities in HD. I also outline a set of simple rules that can be followed to improve the chances of a good night's sleep, since preventing any 'preventable' symptoms is the a logical first step in treating disease. The long-term impact of sleep disruption in HD is unknown. There have been no large-scale systematic studies of in sleep in HD. Furthermore, there has never been a study of the efficacy of pharmaceuticals that are typically used to treat sleep deficits in HD patients. Thus treatment of sleep disturbance in HD is necessarily empirical. A better understanding of the relationship between sleep/circadian abnormalities and HD pathology is needed, if treatment of this aspect of HD is to be optimized.
亨廷顿病是一种进行性神经疾病，最初会出现运动、认知或精神障碍，然后逐渐出现一系列令人痛苦的症状，最终导致运动和执行功能的严重丧失。人们越来越意识到，除了认知和精神症状外，HD 还存在其他重要的非运动症状，包括睡眠和昼夜节律异常。目前尚不清楚睡眠-觉醒变化是由 HD 基因相关病理直接引起的，还是仅仅是患有神经退行性疾病的结果。从患者的角度来看，答案并不重要，因为睡眠和昼夜节律紊乱即使在神经正常的人中也会对良好的日常生活造成损害。假设至少在 HD 患者中，睡眠和/或昼夜节律紊乱会加重他们的症状。在最坏的情况下，它们可能会导致 HD 的进行性下降。在这里，我回顾了我们对 HD 中睡眠和昼夜节律异常的理解现状。我还概述了一套简单的规则，可以遵循这些规则来提高良好睡眠的机会，因为预防任何“可预防”的症状是治疗疾病的合乎逻辑的第一步。HD 中睡眠中断的长期影响尚不清楚。目前还没有关于 HD 中睡眠的大规模系统研究。此外，从未有过研究过用于治疗 HD 患者睡眠缺陷的药物的疗效。因此，HD 中睡眠障碍的治疗必然是经验性的。如果要优化对 HD 这一方面的治疗，就需要更好地了解睡眠/昼夜节律异常与 HD 病理之间的关系。
