患有马查多-约瑟夫病的澳大利亚原住民的睡眠障碍：一项评估该疾病患者及其照料者经历的多方法研究的定量结果。
Sleep disorders among Aboriginal Australians with Machado-Joseph Disease: Quantitative results from a multiple methods study to assess the experience of people living with the disease and their caregivers.
作者信息
LaGrappe Desireé, Massey Libby, Kruavit Anuk, Howarth Timothy, Lalara Gayangwa, Daniels Bronwyn, Wunungmurra Julie Gungunbuy, Flavell Kimberley, Barker Ruth, Flavell Howard, Heraganahally Subash S
机构信息
Centre for Disease Control, Public Health Unit, Northern Territory Government Department of Health, O'Keefe House, Katherine Hospital, George Rd, Katherine, Northern Territory, 0850, Australia.
Judith Lumley Centre, School of Nursing and Midwifery, La Trobe University, Level 3, George Sinclair Building, Bundoora, Victoria, 3086, Australia.
出版信息
Neurobiol Sleep Circadian Rhythms. 2022 Apr 22;12:100075. doi: 10.1016/j.nbscr.2022.100075. eCollection 2022 May.
BACKGROUND
Machado-Joseph Disease (MJD), or Spinocerebellar Ataxia Type 3 (SCA3), is a genetic disorder that causes progressive muscle weakness, loss of motor control, ataxia and permanent physical disability. Sleep disturbances are associated with MJD but remain poorly understood.
OBJECTIVE
To investigate frequency and characteristics of sleep disorders and their association with health-related quality of life and psychosocial wellbeing for Aboriginal Australians living with MJD.
METHODS
A convenience sample of MJD participants n = 24 participated in a semi-attended, ambulatory diagnostic sleep study to capture polysomnography, actigraphy and sleep diary data. Self-report measures collected were the Pittsburgh Sleep Quality Index (PSQI), STOP-BANG Questionnaire for Obstructive Sleep Apnoea (OSA), International Restless Legs Syndrome Study Group rating scale (IRLS), Kessler-5 (K5) and EuroQoL-5 Dimension (EQ5D). Caregivers (n = 22) reported EQ-5D, K5 and bed partners' sleep behaviour (Mayo Sleep Questionnaire-Informant). Environmental factors were measured.
RESULTS
We observed Nocturia, Sleep Related Leg Cramps, OSA, REM Behaviour Disorder, and RLS, respectively in 100%, 71%, 47%, 43%, and 33% of participants with a significant positive correlation between Body mass index (BMI) and Apnoea hypopnea index (AHI). The majority of sleep was spent in non-rapid eye movement sleep (NREM)-N2 stage (77.8% (67.7, 81.6)). Overcrowding (92%) and overnight care needs (42%) interrupted sleep. MJD participants and caregivers reported high psychological distress (K5 median 12.5 IQR 7, 16.5 & 8 IQR 6, 12 respectively).
CONCLUSION
Poor sleep quality and sleep disturbances are prevalent among this cohort. Disease manifestations and environmental factors are driving factors. Larger sample sizes are required to predict risk factors and confirm observed associations.
背景
马查多-约瑟夫病（MJD），即3型脊髓小脑共济失调（SCA3），是一种遗传性疾病，可导致进行性肌肉无力、运动控制丧失、共济失调和永久性身体残疾。睡眠障碍与MJD有关，但人们对此仍知之甚少。
目的
调查患有MJD的澳大利亚原住民睡眠障碍的频率和特征，以及它们与健康相关生活质量和心理社会幸福感的关联。
方法
选取24名MJD参与者的便利样本，参加一项半陪同的动态诊断睡眠研究，以获取多导睡眠图、活动记录仪和睡眠日记数据。收集的自我报告测量指标包括匹兹堡睡眠质量指数（PSQI）、阻塞性睡眠呼吸暂停（OSA）的STOP-BANG问卷、国际不宁腿综合征研究组评定量表（IRLS）、凯斯勒5项心理困扰量表（K5）和欧洲五维健康量表（EQ5D）。照顾者（22名）报告EQ-5D、K5和床伴的睡眠行为（梅奥睡眠问卷- informant）。测量环境因素。
结果
我们分别在100%、71%、47%、43%和33%的参与者中观察到夜尿症、睡眠相关腿部痉挛、OSA、快速眼动睡眠行为障碍和不宁腿综合征，体重指数（BMI）与呼吸暂停低通气指数（AHI）之间存在显著正相关。大部分睡眠处于非快速眼动睡眠（NREM）-N2阶段（77.8%（67.7，81.6））。过度拥挤（92%）和夜间护理需求（42%）干扰了睡眠。MJD参与者和照顾者报告有高度的心理困扰（K5中位数分别为12.5，四分位距7，16.5和8，四分位距6，12）。
结论
该队列中睡眠质量差和睡眠障碍很普遍。疾病表现和环境因素是驱动因素。需要更大的样本量来预测风险因素并确认观察到的关联。
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