Department of Sport and Exercise Sciences, Manchester Metropolitan University, Manchester, United Kingdom.
The Neuromuscular Centre, Winsford, United Kingdom.
PLoS One. 2022 Sep 22;17(9):e0274970. doi: 10.1371/journal.pone.0274970. eCollection 2022.
Despite poor sleep quality being recognised in Duchenne Muscular Dystrophy, reports from milder forms of Muscular Dystrophy (MD), and accompanied associations with quality of life (QoL), pain and fatigue, remain limited however.
Adult males (n = 15 Beckers MD (BMD), n = 12 Limb-Girdle MD (LGMD), n = 12 Fascioscapulohumeral (FSHD), n = 14 non-MD (CTRL)) completed assessments of body composition (Bio-electrical impedance), sleep (7-day 24-hour tri-axial accelerometer, Pittsburgh Sleep Quality Index (PSQI) and Insomnia Severity Index, QoL (SF36-v2), pain (Visual analogue scale), fatigue (Modified Fatigue Index Scale) and functional assessments (Brookes and Vignos).
FSHD and BMD reported worse sleep than CTRL on the PSQI. FSHD scored worse than CTRL on the Insomnia Severity Index (P<0.05). 25-63% and 50-81% of adults with MD reported poor sleep quality using the Insomnia Severity Index and PSQI, respectively. Accelerometery identified no difference in sleep quality between groups. Associations were identified between sleep measures (PSQI global and insomnia severity) with mental or physical QoL in LGMD, BMD and FSHD. Multiple regression identified associations between sleep impairment and fatigue severity (all MDs), body composition (BMD & LGMD), upper and lower limb function (LGMD, FSHD) and age (FSHD).
25-81% of men with MD, depending on classification, experience sleep impairment, using self-report sleep measures. Whilst BMD and FSHD showed worse sleep outcomes than CTRL, no group difference was observed between LGMD and CTRL, however all groups showed associations with sleep impairment and higher levels of fatigue. These findings, and associations with measures of health and wellbeing, highlight an area for further research which could impact QoL in adults with MD.
尽管人们已经认识到杜氏肌营养不良症（Duchenne Muscular Dystrophy）患者的睡眠质量较差，但对于病情较轻的肌营养不良症（Muscular Dystrophy，MD）患者以及与生活质量（Quality of Life，QoL）、疼痛和疲劳相关的报告仍然有限。
15 名贝克肌营养不良症（Beckers MD，BMD）男性患者、12 名肢带型肌营养不良症（Limb-Girdle MD，LGMD）男性患者、12 名面肩肱型肌营养不良症（Fascioscapulohumeral，FSHD）男性患者和 14 名非肌营养不良症（Non-MD，CTRL）男性志愿者参与了本次研究。研究采用了生物电阻抗分析、7 天 24 小时三轴加速度计、匹兹堡睡眠质量指数（Pittsburgh Sleep Quality Index，PSQI）和失眠严重程度指数（Insomnia Severity Index，ISI）、健康调查简表 36 项（Short Form 36-v2，SF36-v2）、视觉模拟量表（Visual Analogue Scale，VAS）、改良疲劳指数量表（Modified Fatigue Index Scale，MFIS）和Brookes 和 Vignos 评估等方法来评估身体成分、睡眠、生活质量、疼痛和疲劳等方面的情况。
FSHD 和 BMD 患者的 PSQI 睡眠评分明显差于 CTRL 组。FSHD 患者的 ISI 评分明显差于 CTRL 组（P<0.05）。使用 ISI 和 PSQI 评估时，分别有 25-63%和 50-81%的 MD 患者报告睡眠质量差。加速计并未发现各组之间的睡眠质量存在差异。在 LGMD、BMD 和 FSHD 患者中，睡眠测量（PSQI 总分和失眠严重程度）与心理或身体 QoL 之间存在关联。多元回归分析显示，睡眠障碍与所有 MD 患者的疲劳严重程度、身体成分（BMD 和 LGMD）、上下肢功能（LGMD、FSHD）和年龄（FSHD）相关。
根据分类，25-81%的 MD 男性患者存在睡眠障碍，使用自我报告的睡眠测量方法可以评估。虽然 BMD 和 FSHD 患者的睡眠结果明显差于 CTRL 组，但 LGMD 组与 CTRL 组之间没有差异，然而所有组都与睡眠障碍和更高水平的疲劳有关。这些发现以及与健康和幸福相关的测量结果，突出了进一步研究的领域，这可能会影响 MD 患者的生活质量。
