Community School of Naples, Naples, Florida.
University of North Carolina Wilmington, Wilmington, North Carolina.
Muscle Nerve. 2022 Oct;66(4):487-494. doi: 10.1002/mus.27688. Epub 2022 Aug 9.
INTRODUCTION/AIMS: Facioscapulohumeral muscular dystrophy (FSHD) causes weakness and secondary associations, such as respiratory complications and pain, that can be linked to abnormal sleep patterns. Limited studies have focused on sleep in FSHD. The purpose of this study was to identify the prevalence of, and clinical features associated with, self-reported lowered sleep quality (SQ) and excessive daytime sleepiness (DS) in a large group of participants with FSHD.
We conducted a prospective survey of individuals with self-reported FSHD enrolled in the FSHD Society Registry. The survey consisted of demographic and clinical characteristics, the Pittsburgh Sleep Quality Index (PSQI), and the Epworth Sleepiness Scale. Descriptive statistics were evaluated, and associations between clinical characteristics and SQ and DS were explored using one-way analysis of variance tests. Small effect size was identified as 0.01 ≥ η > 0.06, medium was 0.06 ≥ η > 0.14, and large was 0.14 ≥ η .
Six hundred ninety individuals responded to the survey, equally distributed between men and women, and spanning the age range from under 12 to 74 years of age or older. Sixty-six percent of the respondents showed reduced SQ (PSQI > 5) (n = 392; 95% confidence interval [CI], 62.4-70.0), and 15% showed excessive DS (>10) (n = 89; 95% CI, 12.2-17.9). There was a significant association between SQ and DS. Nocturnal pain had a large significant effect on lowering SQ (P < .001, η = 0.192). Factors including age and gender had minor effects on SQ.
Physicians should monitor sleep quality of patients with FSHD as a routine part of care, with special attention to potentially modifiable factors. Future research should address the physiological effects of pain in sleep.
简介/目的：面肩肱型肌营养不良症（FSHD）会导致肌肉无力，并引起如呼吸并发症和疼痛等继发性并发症，这些症状可能与睡眠模式异常有关。目前，针对 FSHD 患者睡眠情况的研究较为有限。本研究的目的是在较大的 FSHD 患者群体中，确定自我报告的睡眠质量下降（SQ）和日间嗜睡（DS）的发生率和与临床特征相关的因素。
我们对登记在 FSHD 协会注册中心的 FSHD 自我报告患者进行了前瞻性调查。该调查包括人口统计学和临床特征、匹兹堡睡眠质量指数（PSQI）和 Epworth 嗜睡量表。我们对描述性统计数据进行了评估，并使用单因素方差检验探讨了临床特征与 SQ 和 DS 之间的关系。小效应量被定义为 0.01≥η＞0.06，中效应量为 0.06≥η＞0.14，大效应量为 0.14≥η。
共有 690 名患者对调查做出了回应，其中男女比例相等，年龄从 12 岁以下到 74 岁或以上不等。66%的受访者存在睡眠质量下降（PSQI＞5）（n=392；95%置信区间[CI]，62.4-70.0），15%的受访者存在日间嗜睡过度（＞10）（n=89；95%CI，12.2-17.9）。SQ 和 DS 之间存在显著关联。夜间疼痛对降低 SQ 有较大的显著影响（P＜0.001，η=0.192）。年龄和性别等因素对 SQ 有较小的影响。
医生应将监测 FSHD 患者的睡眠质量作为常规护理的一部分，特别注意可能可改变的因素。未来的研究应关注疼痛对睡眠的生理影响。
