Participation and the Role of Neuropsychological Functioning in Myotonic Dystrophy Type 1.
Participation and the Role of Neuropsychological Functioning in Myotonic Dystrophy Type 1.
复制标题
神经心理功能在 1 型强直性肌营养不良中的参与和作用。
DOI：
10.3233/jnd-170246
复制
发表时间：
2018
影响因子：
3.3
通讯作者：
J. Visser
中科院分区：
文献类型：
--
作者：
C. V. van Heugten;Susan Meuleman;D. Hellebrekers;E. K. Kruitwagen;J. Visser
背景
强直性肌营养不良 1 型 (DM1) 主要是一种神经肌肉疾病，但也可能产生神经心理学后果（即认知、情感和行为），从而影响日常生活和社会参与。对于 DM1 患者及其亲属的参与水平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，并识别那些面临长期参与问题风险的人。
目标
调查DM1患者及其伴侣的参与水平及其决定因素。
方法
使用乌得勒支康复参与评估量表进行横断面研究。决定因素是人口特征、疾病相关特征和神经心理学特征。进行了 Spearman 相关性和向后多元回归分析。
结果
在 66 名 DM1 参与者中，54% 的人报告严重疲劳，47% 的人表现冷漠。经历过的参与限制最常见的是家政（74%）、体育（68%）和户外活动（55%）。参与者对自己的参与程度相对满意，但不满意的地方主要是家政（58%）、户外活动（55%）和体育（54%）。年龄和冷漠是参与多变量模型的频率 (R2 = 10.4) 和限制 (R2 = 14.4) 的显着预测因素。情绪功能是参与满意度的唯一显着预测因素（R2 = 23.8）。
结论
相当一部分DM1患者除了神经肌肉受限外，还存在疲劳、冷漠等中枢神经系统相关问题。参与度较低的风险因素包括年龄、冷漠和情绪问题。建议进行身体和神经心理康复计划。
BACKGROUND
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.
OBJECTIVE
To investigate the level of participation of DM1 patients and their partners and the determinants.
METHODS
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.
RESULTS
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).
CONCLUSIONS
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.