Lang Christina
Department of Neurology, University Hospital of Ulm.
Curr Opin Neurol. 2025 Oct 1;38(5):606-613. doi: 10.1097/WCO.0000000000001424. Epub 2025 Aug 20.
This review summarizes recent evidence on sleep disturbances in amyotrophic lateral sclerosis (ALS), emphasizing their role as intrinsic features of the disease process rather than consequence of motor decline.
Emerging data suggest that sleep disturbances such as sleep fragmentation, rapid eye movement sleep (REM) and non rapid eye movement sleep (NREM) alterations and circadian changes often precede classic motor symptoms. Structural and functional hypothalamic changes have been observed in early ALS, suggesting a direct role in sleep-wake dysregulation. In addition, impaired glymphatic clearance during sleep may contribute to neurodegeneration by impairing the removal of protein waste. Polysomnographic studies and cohort data support the presence of prodromal sleep abnormalities in both symptomatic patients and gene mutation carriers. Noninvasive ventilation has shown benefits not only in respiratory management but also in improving sleep quality and overall prognosis.
Sleep alterations in ALS are increasingly recognized as early indicators and potential modulators of disease progression. The hypothalamus and the glymphatic system emerge as key contributors to these disturbances, highlighting sleep as a therapeutic target. Understanding the role of sleep in ALS pathophysiology may aid in earlier diagnosis and novel intervention strategies aimed at modifying disease course.
本综述总结了肌萎缩侧索硬化症(ALS)睡眠障碍的最新证据,强调其作为疾病进程内在特征的作用,而非运动功能衰退的结果。
新出现的数据表明,睡眠障碍如睡眠片段化、快速眼动睡眠(REM)和非快速眼动睡眠(NREM)改变以及昼夜节律变化往往先于典型运动症状出现。在早期ALS患者中观察到下丘脑的结构和功能改变,提示其在睡眠 - 觉醒失调中起直接作用。此外,睡眠期间类淋巴系统清除功能受损可能通过损害蛋白质废物的清除而导致神经退行性变。多导睡眠图研究和队列数据支持有症状患者和基因突变携带者存在前驱性睡眠异常。无创通气不仅在呼吸管理方面显示出益处,而且在改善睡眠质量和总体预后方面也有效果。
ALS患者的睡眠改变越来越被认为是疾病进展的早期指标和潜在调节因素。下丘脑和类淋巴系统成为这些障碍的关键因素,突出了睡眠作为治疗靶点的重要性。了解睡眠在ALS病理生理学中的作用可能有助于早期诊断以及制定旨在改变疾病进程的新干预策略。