Department of Neurology, Adnan Menderes University Medical Faculty, Aydın, Turkey.
Department of Neurology, Hacettepe University Medical Faculty, Ankara, Turkey.
Sleep Med. 2022 Jun;94:17-25. doi: 10.1016/j.sleep.2022.03.025. Epub 2022 Apr 2.
Narcolepsy type 1 (NT1) is caused by hypocretin deficiency, the pathophysiology of narcolepsy type 2 (NT2) has not been delineated. Except for the hypocretin deficiency and cataplexy, all clinical and laboratory features used in the diagnosis of NT2 are identical to those used for NT1. The aim of this study was to assess the rapid eye movement (REM) sleep-related characteristics in the patients with narcolepsy; the characteristics of REM sleep in polysomnography (PSG) and multiple sleep latency test (MSLT) recordings, the quantification of REM sleep without atonia (RSWA) and atonia index, and the analysis of rapid eye movements (REMs) during REM sleep.
This study was planned by the Sleep Medicine Study Group of the Turkish Neurology Society, and conducted in 11 centers in eight cities in Turkey. The analysis of RSWA was analyzed by reviewing all REM sleep periods on nocturnal PSG and MSLT recordings per standard criteria. The total duration of the increased muscle tone during REM sleep in the chin and bilateral leg electromyography (EMG) recordings was calculated as RSWA index. The REMs index was also investigated the relation to the RSWA.
A total of 274 patients were involved; 147 patients (53.6%) were males and 127 patients (46.4%) were females; the mean age was 29.1 ± 12.0 years. The diagnosis of NT1 was made in 166 patients (60.6%), and 108 patients (39.4%) were diagnosed as having NT2. The mean Epworth sleepiness scale score was significantly higher in patients with NT1 than the patients with NT2 (P = 0.001). The diagnosis of REM sleep behavior disorder (RBD) was made in 19.3% of the patients with NT1 versus in 2.8% of the patients with NT2 (P < 0.001). The percentage of SOREMP in PSG recordings was significantly higher in patients with NT1 (37.1%) than those with NT2 (18.9%, P = 0.001). MSLT showed that the mean sleep latency was shorter in patients with NT1 compared to those with NT2 (P < 0.001). The total duration of REMs on electrooculography recordings was also significantly higher in patients with RSWA in comparison with the patients without RSWA (P = 0.002). Total duration of REMs was significantly and positively correlated with the duration of RSWA on chin-EMG and leg-EMG recordings (P = 0.001). ROC analyses showed an RSWA index of ≥2% for the RSWA on chin-EMG with a sensitivity of 86.7% and a specificity of 71.3% (P < 0.001). The REMs index ≥20% was associated with the presence of RSWA with a sensitivity of 70.0% and a specificity of 57.1% (P = 0.008).
In this nation-wide study, we identified for the first time that the increase in REMs density during REM sleep may be a major correlate of the RSWA. Significant positive correlations were demonstrated between the total duration of REMs on electrooculography recordings and the mean durations of RSWA in both chin and leg EMG recordings. A REMs index of >20% was demonstrated to have a moderate sensitivity and specificity in the diagnosis of RSWA. As observed in chin RSWA index, REMs index also showed a significantly high association with RBD, in comparison to RSWA per standard criteria.
1 型发作性睡病(NT1)是由下丘脑分泌素缺乏引起的,2 型发作性睡病(NT2)的病理生理学尚未明确。除了下丘脑分泌素缺乏和猝倒外,用于诊断 NT2 的所有临床和实验室特征与用于诊断 NT1 的特征相同。本研究旨在评估发作性睡病患者的快速眼动(REM)睡眠相关特征;多导睡眠图(PSG)和多次小睡潜伏期试验(MSLT)记录中的 REM 睡眠特征、REM 睡眠无张力(RSWA)和张力指数的定量分析,以及 REM 睡眠期间的快速眼动(REMs)分析。
本研究由土耳其神经病学会睡眠医学研究小组计划,并在土耳其八个城市的 11 个中心进行。通过审查夜间 PSG 和 MSLT 记录中的所有 REM 睡眠期,按照标准标准分析 RSWA。在颏肌和双侧腿部肌电图(EMG)记录中,计算 REM 睡眠期间增加的肌肉张力的总持续时间,作为 RSWA 指数。还研究了 REMs 指数与 RSWA 的关系。
共有 274 名患者参与;147 名患者(53.6%)为男性,127 名患者(46.4%)为女性;平均年龄为 29.1±12.0 岁。166 名患者(60.6%)被诊断为 NT1,108 名患者(39.4%)被诊断为 NT2。与 NT2 患者相比,NT1 患者的 Epworth 嗜睡量表评分明显更高(P=0.001)。在 NT1 患者中,REM 睡眠行为障碍(RBD)的诊断率为 19.3%,而在 NT2 患者中为 2.8%(P<0.001)。PSG 记录中的 SOREMP 百分比在 NT1 患者中(37.1%)明显高于 NT2 患者(18.9%,P=0.001)。MSLT 显示,与 NT2 患者相比,NT1 患者的平均睡眠潜伏期更短(P<0.001)。与无 RSWA 的患者相比,RSWA 患者的眼电图记录中的 REMs 总持续时间也明显更高(P=0.002)。REM 总持续时间与颏肌和腿部 EMG 记录中的 RSWA 持续时间呈显著正相关(P=0.001)。ROC 分析显示,颏肌 EMG 的 RSWA 指数≥2%时,RSWA 的灵敏度为 86.7%,特异性为 71.3%(P<0.001)。REMs 指数≥20%与 RSWA 的存在相关,灵敏度为 70.0%,特异性为 57.1%(P=0.008)。
在这项全国性研究中,我们首次发现 REM 密度的增加可能是 RSWA 的主要相关因素。在眼电图记录的 REMs 总持续时间和颏肌和腿部 EMG 记录中的 RSWA 平均持续时间之间,显示出显著的正相关。REMs 指数>20%在诊断 RSWA 时具有中等的灵敏度和特异性。与标准标准的 RSWA 指数相比,REMs 指数也与 RBD 有显著的高关联。