Boentert Matthias
Department of Neurology, University Hospital Muenster, Muenster, Germany.
Nat Sci Sleep. 2019 Aug 9;11:97-111. doi: 10.2147/NSS.S183504. eCollection 2019.
Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease inevitably leading to generalized muscle weakness and premature death. Sleep disturbances are extremely common in patients with ALS and substantially add to the burden of disease for both patients and caregivers. Disruption of sleep can be caused by physical symptoms, such as muscle cramps, pain, reduced mobility, spasticity, mucus retention, and restless legs syndrome. In addition, depression and anxiety may lead to significant insomnia. In a small subset of patients, rapid eye movement (REM) sleep behavioral disorder may be present, reflecting neurodegeneration of central nervous system pathways which are involved in REM sleep regulation. With regard to overall prognosis, sleep-disordered breathing (SDB) and nocturnal hypoventilation (NH) are of utmost importance, particularly because NH precedes respiratory failure. Timely mechanical ventilation is one of the most significant therapeutic measures to prolong life span in ALS, and transcutaneous capnometry is superior to pulse oxymetry to detect NH early. In addition, it has been shown that in patients on home ventilatory support, survival time depends on whether normocapnia, normoxia, and elimination of apneic events during sleep can be reliably achieved. Several studies have investigated sleep patterns and clinical determinants of sleep disruption in ALS, but exact prevalence numbers are unknown. Thus, constant awareness for sleep-related symptoms is appropriate. Since no curative treatment can be offered to affected patients, sleep complaints should be thoroughly investigated in order to identify any treatable etiology and improve or stabilize quality of life as much as possible. The use of hypnotics should be confined to palliation during the terminal phase and refractory insomnia in earlier stages of the disease, taking into account that most compounds potentially aggravate SDB.
肌萎缩侧索硬化症（ALS）是一种进行性运动神经元疾病，不可避免地导致全身肌肉无力和过早死亡。睡眠障碍在ALS患者中极为常见，极大地加重了患者及其护理人员的疾病负担。睡眠中断可能由身体症状引起，如肌肉痉挛、疼痛、活动能力下降、痉挛、黏液潴留和不安腿综合征。此外，抑郁和焦虑可能导致严重失眠。在一小部分患者中，可能存在快速眼动（REM）睡眠行为障碍，这反映了参与REM睡眠调节的中枢神经系统通路的神经变性。关于总体预后，睡眠呼吸障碍（SDB）和夜间通气不足（NH）至关重要，特别是因为NH先于呼吸衰竭出现。及时进行机械通气是延长ALS患者寿命的最重要治疗措施之一，经皮二氧化碳监测在早期检测NH方面优于脉搏血氧饱和度测定。此外，研究表明，对于接受家庭通气支持的患者，生存时间取决于能否在睡眠期间可靠地实现正常碳酸血症、正常氧合和消除呼吸暂停事件。多项研究调查了ALS患者的睡眠模式和睡眠中断的临床决定因素，但确切的患病率尚不清楚。因此，持续关注与睡眠相关的症状是合适的。由于无法为受影响的患者提供治愈性治疗，应彻底调查睡眠主诉，以确定任何可治疗的病因，并尽可能改善或稳定生活质量。考虑到大多数药物可能会加重SDB，催眠药的使用应仅限于疾病终末期的姑息治疗和早期难治性失眠。
