Kalkman Joke S, Schillings Maartje L, Zwarts Machiel J, van Engelen Baziel G M, Bleijenberg Gijs
Expert Centre Chronic Fatigue, Radboud University Nijmegen Medical Centre, The Netherlands.
J Psychosom Res. 2007 May;62(5):571-9. doi: 10.1016/j.jpsychores.2006.11.014.
Severe fatigue is reported by the majority of patients with three relatively common types of neuromuscular disorders.
This study aimed to identify predictors of fatigue in a longitudinal study and to develop a model of fatigue in patients with three neuromuscular disorders.
One hundred ninety-eight patients [60 facioscapulohumeral muscular dystrophy (FSHD), 70 adult-onset myotonic dystrophy (MD), and 68 hereditary motor and sensory neuropathy type I (HMSN-I) patients] were studied twice during an 18-month period. Fatigue severity was assessed by the Checklist Individual Strength. A multidimensional assessment method was used, including self-report questionnaires, a daily Self-Observation List, and physical activity (actometer). Muscle strength was determined using the Medical Research Council scale. Structural equation modeling was used to develop and test a model of factors contributing to the persistence of experienced fatigue.
Muscle strength, self-reported physical activity, sleep disturbances, and pain at baseline contributed directly or indirectly to fatigue and impairment at follow-up. Lower muscle strength contributed to lower levels of physical activity, which, in turn, contributed to fatigue severity. The model showed excellent fit for the whole group of neuromuscular disorders. In FSHD, pain also contributed to physical activity. A model with the actometer as measurement for actual physical activity instead of self-report showed an excellent model fit in FSHD and HMSN but an insufficient fit in MD.
The model of perpetuating factors for fatigue in FSHD and HMSN is different from the model in MD. The main difference is in physical (in)activity. These differences have implications for interventions based on these models.
大多数患有三种相对常见类型神经肌肉疾病的患者都报告有严重疲劳。
本研究旨在通过纵向研究确定疲劳的预测因素，并建立三种神经肌肉疾病患者的疲劳模型。
198名患者[60例面肩肱型肌营养不良（FSHD）、70例成人起病的强直性肌营养不良（MD）和68例I型遗传性运动和感觉神经病（HMSN-I）患者]在18个月期间接受了两次研究。通过个体力量检查表评估疲劳严重程度。采用多维度评估方法，包括自我报告问卷、每日自我观察清单和身体活动（活动计）。使用医学研究委员会量表测定肌肉力量。采用结构方程模型来建立和检验导致持续性疲劳的因素模型。
基线时的肌肉力量、自我报告的身体活动、睡眠障碍和疼痛直接或间接导致随访时的疲劳和功能损害。较低的肌肉力量导致身体活动水平降低，进而导致疲劳严重程度增加。该模型对整个神经肌肉疾病组显示出良好的拟合度。在FSHD中，疼痛也会影响身体活动。以活动计测量实际身体活动而非自我报告的模型在FSHD和HMSN中显示出良好的模型拟合度，但在MD中拟合度不足。
FSHD和HMSN中疲劳持续因素的模型与MD中的模型不同。主要差异在于身体活动（不活动）情况。这些差异对基于这些模型的干预措施具有启示意义。
