Mayo Center for Sleep Medicine, Department of Neurology, Division of Cardiovascular Diseases, St. Mary's Hospital, Mayo Clinic and Foundation, Rochester, MN, USA.
Neurology. 2013 Jul 9;81(2):157-64. doi: 10.1212/WNL.0b013e31829a340f. Epub 2013 Jun 7.
Although sleep disturbances are common in myotonic dystrophy type 1 (DM1), sleep disturbances in myotonic dystrophy type 2 (DM2) have not been well-characterized. We aimed to determine the frequency of sleep disturbances in DM2.
We conducted a case-control study of 54 genetically confirmed DM2 subjects and 104 medical controls without DM1 or DM2, and surveyed common sleep disturbances, including symptoms of probable restless legs syndrome (RLS), excessive daytime sleepiness (EDS), sleep quality, fatigue, obstructive sleep apnea (OSA), probable REM sleep behavior disorder (pRBD), and pain. Thirty patients with DM2 and 43 controls responded to the survey. Group comparisons with parametric statistical tests and multiple linear and logistic regression analyses were conducted for the dependent variables of EDS and poor sleep quality.
The mean ages of patients with DM2 and controls were 63.8 and 64.5 years, respectively. Significant sleep disturbances in patients with DM2 compared to controls included probable RLS (60.0% vs 14.0%, p < 0.0001), EDS (p < 0.001), sleep quality (p = 0.02), and fatigue (p < 0.0001). EDS and fatigue symptoms were independently associated with DM2 diagnosis (p < 0.01) after controlling for age, sex, RLS, and pain scores. There were no group differences in OSA (p = 0.87) or pRBD (p = 0.12) scores.
RLS, EDS, and fatigue are frequent sleep disturbances in patients with DM2, while OSA and pRBD symptoms are not. EDS was independently associated with DM2 diagnosis, suggesting possible primary CNS hypersomnia mechanisms. Further studies utilizing objective sleep measures are needed to better characterize sleep comorbidities in DM2.
尽管肌强直性营养不良 1 型(DM1)患者常出现睡眠障碍,但肌强直性营养不良 2 型(DM2)患者的睡眠障碍尚未得到充分描述。我们旨在确定 DM2 患者睡眠障碍的发生频率。
我们对 54 名经基因确认的 DM2 患者和 104 名无 DM1 或 DM2 的医学对照进行了病例对照研究,并调查了常见的睡眠障碍,包括可能的不宁腿综合征(RLS)症状、日间嗜睡(EDS)、睡眠质量、疲劳、阻塞性睡眠呼吸暂停(OSA)、可能的 REM 睡眠行为障碍(pRBD)和疼痛。30 名 DM2 患者和 43 名对照者对该调查作出了回应。采用参数统计检验和多元线性及逻辑回归分析,对 EDS 和睡眠质量差等因变量进行组间比较。
DM2 患者和对照组的平均年龄分别为 63.8 岁和 64.5 岁。与对照组相比,DM2 患者存在显著的睡眠障碍,包括可能的 RLS(60.0% vs 14.0%,p < 0.0001)、EDS(p < 0.001)、睡眠质量(p = 0.02)和疲劳(p < 0.0001)。在控制年龄、性别、RLS 和疼痛评分后,EDS 和疲劳症状与 DM2 诊断独立相关(p < 0.01)。两组在 OSA(p = 0.87)或 pRBD(p = 0.12)评分上无差异。
RLS、EDS 和疲劳是 DM2 患者常见的睡眠障碍,而 OSA 和 pRBD 症状则不然。EDS 与 DM2 诊断独立相关,提示可能存在中枢神经系统原发性过度嗜睡机制。需要进一步的研究,利用客观的睡眠测量来更好地描述 DM2 的睡眠合并症。