行为和认知障碍对1型强直性肌营养不良患者生活质量有显著影响。
Significant impact of behavioral and cognitive impairment on quality of life in patients with myotonic dystrophy type 1.
作者信息
Rakocevic-Stojanovic V, Peric S, Madzarevic R, Dobricic V, Ralic V, Ilic V, Basta I, Nikolic A, Stefanova E
机构信息
Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia.
Institute of Neurology, Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, Belgrade, Serbia.
出版信息
Clin Neurol Neurosurg. 2014 Nov;126:76-81. doi: 10.1016/j.clineuro.2014.08.021. Epub 2014 Aug 30.
OBJECTIVE
To assess an impact of cognitive and behavioral impairment on QoL in a larger cohort of patients with DM1.
METHODS
Sixty six genetically confirmed DM1 patients (22 with juvenile (jDM1) and 44 with adult form (aDM1) of the disease) were recruited. Following behavioral tests were used: Hamilton scales for depression and anxiety (HamD and HamA), Daytime Sleepiness Scale (DSS), and Krupp's Fatigue Severity Scale (FSS). Patients also underwent detailed classic neuropsychological investigation and Cambridge Neuropsychological Test Automated Battery (CANTAB). Individualized Neuromuscular Quality of Life questionnaire (INQoL) was used as a measure of QoL.
RESULTS
Patients with jDM1 scored lower than aDM1 patients regarding total INQoL score and all INQoL subdomains, except for myotonia. Significant predictors of total INQoL score in patients with jDM1 were severity of fatigue (β=+0.60, p<0.01) and percentage of correct responses on Spatial Recognition Memory test from CANTAB that measures visuospatial abilities (β=-0.38, p<0.05). The most important predictors of total INQoL score in patients with aDM1 were severity of fatigue (β=+0.36, p<0.05) and level of education (β=-0.29, p<0.05).
CONCLUSION
Our results showed clear influence of different central manifestations on QoL in patients with both aDM1 and jDM1.
目的
在更大规模的1型糖尿病患者队列中评估认知和行为障碍对生活质量的影响。
方法
招募了66名经基因确诊的1型糖尿病患者（22名青少年型（jDM1）和44名成人型（aDM1））。使用了以下行为测试：汉密尔顿抑郁量表和焦虑量表（HamD和HamA）、日间嗜睡量表（DSS）以及克鲁普疲劳严重程度量表（FSS）。患者还接受了详细的经典神经心理学调查和剑桥神经心理测试自动成套系统（CANTAB）。使用个体化神经肌肉生活质量问卷（INQoL）作为生活质量的衡量指标。
结果
jDM1患者在INQoL总分及除肌强直外的所有INQoL子领域得分均低于aDM1患者。jDM1患者INQoL总分的显著预测因素是疲劳严重程度（β=+0.60，p<0.01）以及CANTAB中测量视觉空间能力的空间识别记忆测试的正确反应百分比（β=-0.38，p<0.05）。aDM1患者INQoL总分的最重要预测因素是疲劳严重程度（β=+0.36，p<0.05）和教育水平（β=-0.29，p<0.05）。
结论
我们的结果表明，不同的中枢表现对aDM1和jDM1患者的生活质量有明显影响。
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