Endo Makiko, Odaira Kaori, Ono Ryohei, Kurauchi Go, Koseki Atsushi, Goto Momoko, Sato Yumi, Kon Seiko, Watanabe Norio, Sugawara Norio, Takada Hiroto, Kimura En
Clinical Research Unit, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira, Tokyo 187-8551, Japan,
Regional Medical Liaison Office, National Hospital Organization, Aomori Hospital, Namioka, Aomori 038-1331, Japan.
Neuropsychiatr Dis Treat. 2019 Jan 14;15:219-226. doi: 10.2147/NDT.S187607. eCollection 2019.
Myotonic dystrophy type 1 (DM1) is a common form of muscular dystrophy that presents with a variety of symptoms that can affect patients' quality of life (QoL). Despite the importance of clarifying patients' subjective experience in both physical and psychosocial aspects for improved symptom management, there is lack of evidence concerning QoL of patients with DM1 in Japan.
A cross-sectional study was performed with 51 DM1 patients who completed questionnaires that measured health-related QoL (HRQoL), depression, and daytime sleepiness. Activities of daily living, body mass index (BMI), and genetic information were also collected, together with general demographic information. Correlation analyses using these variables were performed. Furthermore, regression analysis was utilized to assess the relationship that HRQoL, depression, and daytime sleepiness scores have with other variables.
Physical component summary (PCS) score was affected by the disease more than the mental component summary (MCS) score among study participants. Moderate correlation was observed between PCS and depression, PCS and Barthel index, and depression and daytime sleepiness. Regression analysis revealed that age, sex, cytosine-thymine-guanine repeats, and BMI did not predict the aforementioned dependent variables.
DM1 symptoms influenced physical component scores more than mental component scores, although the state of physical wellness seemed to affect patients' mood. Explaining the QoL of these patients only using biologic and genetic characteristics was not sufficient. We conclude that social and psychological aspects of these patients' lives and the nature of adjustments made by patients due to DM1 to require further examination in order to improve the standard of care.
1型强直性肌营养不良症（DM1）是一种常见的肌营养不良症，表现出多种可影响患者生活质量（QoL）的症状。尽管明确患者在身体和心理社会方面的主观体验对于改善症状管理很重要，但在日本，缺乏关于DM1患者生活质量的证据。
对51名DM1患者进行了一项横断面研究，这些患者完成了测量健康相关生活质量（HRQoL）、抑郁和日间嗜睡情况的问卷。还收集了日常生活活动、体重指数（BMI）和基因信息，以及一般人口统计学信息。使用这些变量进行了相关性分析。此外，利用回归分析评估HRQoL、抑郁和日间嗜睡评分与其他变量之间的关系。
在研究参与者中，身体成分总结（PCS）得分比心理成分总结（MCS）得分受疾病影响更大。观察到PCS与抑郁、PCS与巴氏指数以及抑郁与日间嗜睡之间存在中度相关性。回归分析显示，年龄、性别、胞嘧啶 - 胸腺嘧啶 - 鸟嘌呤重复序列和BMI不能预测上述因变量。
DM1症状对身体成分得分的影响大于心理成分得分，尽管身体健康状况似乎会影响患者的情绪。仅用生物学和遗传特征来解释这些患者的生活质量是不够的。我们得出结论，这些患者生活的社会和心理方面以及患者因DM1所做调整的性质需要进一步研究，以提高护理标准。
