摘要：

目的 通过多导睡眠图监测肌萎缩侧索硬化症患者睡眠结构和睡眠呼吸事件，探讨其睡眠障碍和睡眠呼吸障碍特点。方法 共36 例患者根据是否存在延髓症状分为肢体受累组（14 例）和延髓麻痹组（22 例），记录一般资料以及睡眠障碍和睡眠呼吸障碍相关主诉，包括入睡困难、睡眠维持困难或早醒、呼吸不畅或鼾症、夜尿症、不宁腿综合征、肌肉疼痛等；Appel 肌萎缩侧索硬化症量表（AALS）评价延髓功能、呼吸功能、上下肢肌力和肌肉功能；多导睡眠图监测脑电图、眼动图、心电图、肌电图、体位、鼾声、口鼻气流量、胸腹式呼吸和指端脉搏血氧饱和度，以及睡眠相关参数包括总睡眠时间、睡眠效率、睡眠潜伏期、觉醒次数、非快速眼动睡眠期各期和快速眼动睡眠期比例、睡眠呼吸暂停低通气指数。Pearson 相关分析评价快速眼动睡眠期睡眠呼吸暂停低通气指数和周期性腿动与临床资料和AALS 评分的相关性。结果 延髓麻痹组患者AALS 总评分（P = 0.007）、延髓功能评分（P = 0.000）和呼吸功能评分（P = 0.000）高于，上肢肌力（P = 0.016）低于肢体受累组。两组患者均出现睡眠结构紊乱，表现为睡眠片段化，其中延髓麻痹组觉醒次数多于（P = 0.027）、快速眼动睡眠期比例低于（P = 0.009）、周期性腿动次数少于（P = 0.020）肢体受累组；两组患者睡眠呼吸障碍主要表现为低通气，其中延髓麻痹组睡眠呼吸暂停低通气指数（P = 0.038）、快速眼动睡眠期和非快速眼动睡眠期睡眠呼吸暂停低通气指数（P = 0.031，0.049）高于肢体受累组。Pearson 相关分析显示，快速眼动睡眠期睡眠呼吸暂停低通气指数与病程（r =0.654，P = 0.028）以及AALS总评分（r = 0.458，P = 0.034）、延髓功能评分（r = 0.572，P = 0.030）、呼吸功能评分（r = 0.756，P = 0.002）呈正相关，周期性腿动与病程（r = 0.574，P = 0.030）以及AALS 总评分（r = 0.321，P = 0.042）、上肢肌肉功能（r = 0.656，P = 0.028）和下肢肌肉功能（r = 0.754，P = 0.015）评分呈正相关。结论 肌萎缩侧索硬化症患者存在失眠、睡眠呼吸障碍、周期性肢体运动障碍等多种形式的睡眠障碍。多导睡眠图可以监测到睡眠呼吸障碍，为尽早进行无创性正压通气提供临床依据。

关键词: 肌萎缩侧索硬化, 睡眠障碍, 多道睡眠描记术

Abstract:

Objective  To study the characteristics of sleep architecture and sleep - disordered breathing (SDB) in patients with amyotrophic lateral sclerosis (ALS) using polysomnography (PSG).  Methods  A total of 36 patients with ALS were recruited in this study. According to symptoms of medulla oblongata, the patients were divided into limb involvement group (N = 14) and bulbar palsy group (N = 22). Detailed record of the patients was made including general information and chief complaints of sleep dysfunction and SDB, which covered sleep initiation and maintenance disorders, arousals, difficulty in breathing and snoring, nocturnal polyuria, restless legs syndrome (RLS) and muscle soreness. Appel Amyotrophic Lateral Sclerosis (AALS) Scores were used to assess bulbar function, breathing function,myodynamia and limbs function. PSG was performed to monitor EEG, EOG, EMG, ECG, position, snore, gas flow of mouth and nose, chest breathing, pulse oxygen saturation (SpO₂) and sleep-related parameters including total sleep time (TST), sleep efficiency (SE), sleep latency (SL), awakening times, percentage of different non-rapid eye movement (NREM) and rapial eye movement (REM), and apnea hypopnea index (AHI). Pearson correlation analysis evaluated the relationship between AHI of REM, periodic limb movements (PLM) and clinical information, AALS Scores.  Results  Bulbar palsy group had higher scores in AALS Scores (P = 0.007), bulbar function (P = 0.000) and breathing function (P = 0.000), and lower score in upper limb myodynamia (P = 0.016) than limb involvement group. Both 2 groups showed disturbed sleep architecture in the performance of sleep fragmentation. Bulbar palsy group had more awakening times (P = 0.027), lower percentage of REM sleep (P = 0.009) and less PLM (P = 0.020) than limb involvement group. The main respiratory event of 2 groups was hypopnea. Bulbar palsy group had higher AHI (P = 0.038) and AHI of REM and NREM (P = 0.031, 0.049) than limb involvement group. Pearson correlation analysis showed that AHI of REM was positively correlated with duration (r = 0.654, P = 0.028), AALS total score (r = 0.458, P = 0.034), bulbar function score (r = 0.572, P = 0.030) and breathing function (r = 0.756, P = 0.002). PLM was also positively correlated with duration (r = 0.574, P = 0.030), AALS total score (r = 0.321, P = 0.042), upper limb function (r = 0.656, P = 0.028) and lower limb function (r = 0.754, P = 0.015). Conclusions Patients with ALS have many types of sleep disorders including insomnia, SDB and periodic limb movements disorder (PLMD). PSG can monitor respiratory event thus providing clinical evidence for the non-invasive ventilation intervention.

Key words: Amyotrophic lateral sclerosis, Sleep disorders, Polysomnography