School for Mental Health and Neuroscience, Maastricht University Medical Center, Maastricht, The Netherlands.
Department of Neuropsychology and Psychopharmacology, Maastricht University, Maastricht, The Netherlands.
J Neuromuscul Dis. 2018;5(2):205-214. doi: 10.3233/JND-170246.
Myotonic Dystrophy type 1 (DM1) is primarily a neuromuscular disease but can also have neuropsychological consequences (i.e.cognitive, emotional and behavioural) which can influence daily living and societal participation. Not much is known about the level of participation of DM1 patients and their relatives and the factors influencing participation. This information can guide the development of rehabilitation programs and identify those at risk of long-term participation problems.
To investigate the level of participation of DM1 patients and their partners and the determinants.
Cross-sectional study using the Utrecht Scale for Evaluation of Rehabilitation-Participation. Determinants were demographic, disease related, and neuropsychological characteristics. Spearman correlations and backward multiple regression analyses were performed.
Of the 66 DM1 participants, 54% reported severe fatigue and 47% apathy. Experienced participation restrictions were most prevalent in housekeeping (74%), sports (68%) and outdoor activities (55%). Participants were relatively satisfied with their level of participation, but dissatisfaction occurred mostly in housekeeping (58%), outdoor activities (55%), and sports (54%). Age and apathy were significant predictors of frequency of (R2 = 10.4) and restrictions in participation in the multivariate model (R2 = 14.4). Emotional functioning was the only significant predictor of satisfaction with participation (R2 = 23.8).
A considerable number of DM1 patients have Central Nervous System-related problems such as fatigue and apathy in addition to neuromuscular restrictions. Risk factors for lower participation are age, apathy, and emotional problems. Physical as well as neuropsychological rehabilitation programs are recommended.
1 型肌强直性营养不良（DM1）主要是一种神经肌肉疾病，但也可能有神经心理学后果（即认知、情感和行为），这可能会影响日常生活和社会参与。关于 DM1 患者及其家属的参与程度以及影响参与的因素知之甚少。这些信息可以指导康复计划的制定，并确定那些有长期参与问题风险的人。
调查 DM1 患者及其伴侣的参与程度及其决定因素。
使用乌得勒支康复参与评估量表进行横断面研究。决定因素包括人口统计学、疾病相关和神经心理学特征。进行了 Spearman 相关分析和向后多元回归分析。
在 66 名 DM1 参与者中，54%报告严重疲劳，47%报告冷漠。在家庭管理（74%）、运动（68%）和户外活动（55%）方面，经历的参与限制最为普遍。参与者对自己的参与水平相对满意，但不满主要发生在家庭管理（58%）、户外活动（55%）和运动（54%）方面。年龄和冷漠是参与频率（R2=10.4）和参与受限的多变量模型（R2=14.4）的显著预测因子。情感功能是参与满意度的唯一显著预测因子（R2=23.8）。
除了神经肌肉限制外，相当一部分 DM1 患者还存在与中枢神经系统相关的问题，如疲劳和冷漠。参与度较低的风险因素是年龄、冷漠和情绪问题。建议进行身体和神经心理学康复计划。
