脊髓小脑共济失调3型ICARS评分相关因素分析  

Analysis of the related factors of ICARS score on spinocerebellar ataxia type 3

作　　者：顾卫红[1] 王国相[1] 王康[1] 孙瑞华[2] 杨斯柳[1] 郝莹[1] 王桂芳[2] 

机构地区：[1]中日友好医院神经内科,北京100029 [2]中日友好医院科教部,北京100029

出　　处：《中国现代神经疾病杂志》2008年第2期139-141,共3页Chinese Journal of Contemporary Neurology and Neurosurgery

基　　金：卫生部临床学科重点项目(项目编号:2004-2006)

摘　　要：目的探讨国际协作共济失调评估量表(ICARS)评估脊髓小脑共济失调3型患者的相关因素。方法应用ICARS对29例经基因学检查明确诊断的脊髓小脑共济失调3型家系先证者进行评估,通过统计学方法分析其发病年龄、病程和SCA3/MJD基因胞嘧啶-腺嘌呤-鸟嘌呤(CAG)重复数目与ICARS总评分的相关性。结果简单线性回归分析结果显示,ICARS总评分与病程呈正相关关系(r=0.576,P=0.004),回归方程为Y=18.992+2.282X2(病程)(F=10.020,P=0.004)。多重线性回归分析结果显示,ICARS总评分与发病年龄、病程和CAG重复数目呈正相关关系,回归方程为Y=-110.744+0.974X1(发病年龄)+2.310X2(病程)+1.446X3(CAG重复数目)(F=6.690,P=0.002);3项因素的标准化偏回归系数分别为0.681、0.527和0.575。结论脊髓小脑共济失调3型患者的ICARS总评分与发病年龄、病程和CAG重复数目呈正相关关系。ICARS为评估共济失调的严重程度提供了可靠的方法。Objective To investigate the-related factors Of International Cooperative Ataxia Rating Scale （ICARS） score on spinocerebellar ataxia type 3 （SCA3） patients. Methods Twenty-nine patients （familial probands） with genetically determined SCA3 were assessed by ICARS. The relation between total ICARS score and age of onset, disease duration and expanded cytosine-adenine-guanine （CAG） repeat number of SCA3/MJD gene were statistically analysed. Results Simple linear regression analysis showed that total ICARS score was positively related with the disease duration （r = 0.576, P= 0.004）. Simple linear regression equation was Y = 18.992 ＋ 2.282X2 （disease duration） （F= 10.020, P= 0.004）. Multiple linear regression analysis showed that total ICARS score was positively related with the age of onset, disease duration and expanded CAG repeat number of SCA3/MJD gene. Multiple linear regression equation was Y=-110.744＋0.974X1 （age of onset） ＋ 2.310X2 （disease duration） ＋ 1.446X3 （expanded CAG repeat number） （F= 6.690, P= 0.002）. The standardized partial regression coefficients of the 3 factors were 0.681, 0.527 and 0.575, respectively. Condusion These findings indicate that the total ICARS scores of SCA3 eases are positively related with the age of onset, disease duration and expanded CAG repeat number of SCA3/MJD gene. The ICARS is a reliable method to evaluate the severity of ataxia.

关 键 词：脊髓小脑变性 重复序列 核酸 神经病学检查 

分 类 号：R744[医药卫生—神经病学与精神病学]