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

Q2 Medicine

Neurobiology of Sleep and Circadian Rhythms Pub Date : 2022-05-01 DOI:10.1016/j.nbscr.2022.100075

Desireé LaGrappe , Libby Massey , Anuk Kruavit , Timothy Howarth , Gayangwa Lalara , Bronwyn Daniels , Julie Gungunbuy Wunungmurra , Kimberley Flavell , Ruth Barker , Howard Flavell , Subash S. Heraganahally

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引用次数: 9

Abstract

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.

查看原文本刊更多论文

患有Machado Joseph病的澳大利亚原住民的睡眠障碍：评估该病患者及其护理人员经历的多种方法研究的定量结果

machado - joseph病(MJD)，或脊髓小脑性共济失调3型(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，但人们对其了解甚少。目的调查澳大利亚土著MJD患者睡眠障碍的频率和特征及其与健康相关的生活质量和心理社会健康的关系。方法选取24名MJD参与者作为方便样本，参与了一项半旁听、动态睡眠诊断研究，以获取多导睡眠图、活动图和睡眠日记数据。收集的自我报告测量方法为匹兹堡睡眠质量指数(PSQI)、阻塞性睡眠呼吸暂停(OSA) STOP-BANG问卷、国际不宁腿综合征研究组评定量表(IRLS)、Kessler-5 (K5)和EuroQoL-5维度(EQ5D)。护理人员(n = 22)报告了EQ-5D、K5和床伴的睡眠行为(梅奥睡眠问卷-被调查者)。测量环境因素。结果夜尿症、睡眠相关性腿痉挛、OSA、REM行为障碍和RLS分别在100%、71%、47%、43%和33%的参与者中出现，体重指数(BMI)与呼吸暂停低通气指数(AHI)呈显著正相关。大部分睡眠时间为非快速眼动睡眠(NREM)-N2期(77.8%(67.7,81.6))。过度拥挤(92%)和夜间护理需求(42%)导致睡眠中断。MJD参与者和照顾者报告了较高的心理困扰(K5中位数12.5 IQR 7, 16.5 &8 IQR 6, 12分别)。结论该人群普遍存在睡眠质量差和睡眠障碍。疾病表现和环境因素是驱动因素。预测危险因素和确认观察到的关联需要更大的样本量。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Neurobiology of Sleep and Circadian Rhythms Neuroscience-Behavioral Neuroscience

CiteScore

4.50

自引率

0.00%

发文量

审稿时长

69 days

期刊介绍： Neurobiology of Sleep and Circadian Rhythms is a multidisciplinary journal for the publication of original research and review articles on basic and translational research into sleep and circadian rhythms. The journal focuses on topics covering the mechanisms of sleep/wake and circadian regulation from molecular to systems level, and on the functional consequences of sleep and circadian disruption. A key aim of the journal is the translation of basic research findings to understand and treat sleep and circadian disorders. Topics include, but are not limited to: Basic and translational research, Molecular mechanisms, Genetics and epigenetics, Inflammation and immunology, Memory and learning, Neurological and neurodegenerative diseases, Neuropsychopharmacology and neuroendocrinology, Behavioral sleep and circadian disorders, Shiftwork, Social jetlag.