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目的设计用于评价肝豆状核变性(Wilson disease,WD)神经系统症状的量表(改良Young量表),并对该量表的信度和效度及临床意义进行了研究。方法选取住院的脑型WD初诊患者60例,对所有WD患者分别用改良Young量表、WD全面评价量表(Globle assessment scale,GAS量表)、帕金森联合量表(Unified parkinson dis-ease rating scale,UPDRS量表)、日常生活活动量表(activity of daily living scale,ADL)进行评分。间隔1 d后再次用改良Young量表进行评分。所有患者进行脑脊液铜检查。接受二巯基丙磺钠排铜治疗1个月后再次用上述量表进行评分。应用统计方法分析量表的信度、效度、反应度。并结合WD患者脑脊液铜来分析量表在WD的临床意义。结果改良Young量表的内部一致性系数Cronbachα系数=0.8733,重测信度系数在0.8以上,评定者间一致性系数维持在0.75到0.94。结构效度因子分析证明量表由肌张力障碍、其他运动障碍、高级神经功能3个方面构成,与临床构想一致。改良Young量表得分与GAS量表、UPDRS量表、ADL量表得分具有相关性。改良Young量表评分与脑脊液铜有相关性,与临床症状严重程度的相关系数高于其他量表。量表的效标尺度((治疗后得分-治疗前得分)/治疗前得分)为0.23。结论改良Young量表有较好的信度和效度,有较高的灵敏度。能有效反映WD的症状严重程度和生活质量,可监测治疗效果,在一定程度上能反映WD患者脑内的代谢异常程度。
Objective To design a scale (improved Young scale) for evaluating the neurological symptoms of Wilson’s disease (WD) and to evaluate its reliability, validity and clinical significance. Methods Sixty-six newly diagnosed brain-type WD patients were enrolled in this study. All WD patients were evaluated by Modified Young Scale, WD Comprehensive Assessment Scale (GAS scale), Parkinson’s dis-ease rating scale, UPDRS scale), activity of daily living scale (ADL). After an interval of 1 d again with a modified Young scale score. All patients were tested for cerebrospinal fluid copper. After receiving sodium dimercaptopropane sodium for 1 month, they were again scored on the above scale. Application of statistical methods to analyze the scale of reliability, validity, responsiveness. Combined with cerebrospinal fluid copper in patients with WD to analyze the clinical significance of the scale in WD. Results The internal consistency coefficient of the improved Young scale was Cronbachα coefficient = 0.8733, the retest reliability coefficient was above 0.8, and the consistency coefficient between the assessors was maintained at 0.75 to 0.94. Structural validity factor analysis showed that the scale consists of dystonia, other movement disorders, advanced neurological function in three aspects, consistent with clinical conception. The modified Young scale score was correlated with the GAS scale, UPDRS scale, and ADL scale score. Correlation between the Modified Young Scale and cerebrospinal fluid copper was higher than that of other scales in relation to the severity of clinical symptoms. Scale scale ((post-treatment score - pre-treatment score) / pre-treatment score) was 0.23. Conclusion The modified Young scale has better reliability and validity and higher sensitivity. Can effectively reflect the severity of symptoms and quality of life of WD, can monitor the therapeutic effect, to some extent, can reflect the degree of metabolic abnormalities in the brain of WD patients.