Department of Neurology, Fatih Sultan Mehmet Education and Research Hospital, Health Sciences University, Istanbul, Turkey.
Sleep Disorders Unit, Department of Neurology, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey,
Neurodegener Dis. 2019;19(3-4):148-154. doi: 10.1159/000505575. Epub 2020 Feb 28.
BACKGROUND/OBJECTIVE: In this study, we demonstrated the effects of obstructive sleep apnea syndrome (OSAS) on the prognosis of amyotrophic lateral sclerosis (ALS). However, the effects of sleep-related disorders other than breathing problems are still waiting to be delineated.
In this longitudinal retrospective and prospective study, we investigated 73 patients with ALS compared to 20 healthy subjects, to determine sleep-related disorders and their impact on disease prognosis.
In patients with ALS, the mean respiratory disturbance index (RDI) was 28.0/h, which was significantly higher than that in the controls (p < 0.001). OSAS was present in 67% of the patients, sleep-related hypoxemia was observed in 13.7% of the patients, and 5.4% had central sleep apnea syndrome. In patients with bulbar-onset disease, higher RDI was almost significantly associated with lower survival (p = 0.056). The mean index of periodic leg movements in sleep was significantly higher in patients with ALS (34.0 ± 19.9/h) than that in the controls (12.5 ± 15.5/h; p < 0.001). Periodic leg movements disorder (PLMD) was diagnosed in 23 patients with ALS (31.5%); fragmentary myoclonus was present in 13.7% of patients, and REM-sleep behavior disorder was diagnosed in 4 patients (5.4%). The presence of PLMD in addition to OSAS was significantly associated with worse prognosis and poorer survival (p = 0.040).
These findings emphasize that sleep-related disorders other than OSAS deserve attention in ALS. ALS is a catastrophic and frustrating disease for both patients and physicians; thus, the diagnosis and treatment of comorbid sleep disorders could improve the survival of patients with ALS.
背景/目的：本研究旨在探讨阻塞性睡眠呼吸暂停综合征（OSAS）对肌萎缩侧索硬化症（ALS）预后的影响。然而，除呼吸问题以外的睡眠相关障碍的影响仍有待阐明。
本研究为纵向回顾性和前瞻性研究，共纳入 73 例 ALS 患者和 20 名健康对照者，旨在调查睡眠相关障碍及其对疾病预后的影响。
在 ALS 患者中，平均呼吸紊乱指数（RDI）为 28.0/h，明显高于对照组（p＜0.001）。67%的患者存在 OSAS，13.7%的患者出现睡眠相关低氧血症，5.4%的患者存在中枢性睡眠呼吸暂停综合征。在延髓起病的患者中，较高的 RDI 与较低的生存率呈显著相关（p=0.056）。ALS 患者的睡眠周期性肢体运动指数（PLMS）明显高于对照组（34.0±19.9/h 比 12.5±15.5/h；p＜0.001）。23 例 ALS 患者（31.5%）被诊断为 PLMD，13.7%的患者存在片段性肌阵挛，4 例（5.4%）患者被诊断为 REM 睡眠行为障碍。除 OSAS 外还存在 PLMD 与更差的预后和生存率降低显著相关（p=0.040）。
这些发现强调了除 OSAS 以外的睡眠相关障碍在 ALS 中值得关注。ALS 对患者和医生来说都是一种灾难性和令人沮丧的疾病；因此，合并睡眠障碍的诊断和治疗可能会改善 ALS 患者的生存率。
