@article {Schmitz-H {\“u} bsch678,作者= {T。Schmitz-H {\“u} bsch r . Fimmers和m . Rakowicz r·劳拉和大肠Zdzienicka r . Fancellu和c . Mariotti c . Linnemann和l .原理图{\ " o} ls a和d . Timmann Filla大肠萨尔瓦多和j .亲王p Giunti r .上唇和b·克雷默B.P.C. van de Warrenburg l . Baliko和b . Melegh c Depondt j·舒尔茨和美国Tezenas du Montcel t Klockgether}, title ={响应不同的评级工具在脊髓小脑的共济失调患者},体积={74}={8},页面= {678 - 684}= {2010},doi = {10.1212 / WNL。出版商0 b013e3181d1a6c9} = {Wolters Kluwer健康,公司代表美国神经病学学会},文摘={目的:确定最近开发的纵向指标属性脊髓小脑的共济失调的临床评估工具(SCA)首页。方法:171例患者的一个子集的EUROSCA自然历史研究队列(61年43 SCA1、SCA2、37 SCA3和30 SCA6)经过1年的随访检查。分数变化和效果指标计算临床量表(量表的评估和评级共济失调(SARA),库存Non-Ataxia症状[在]),功能测试(SCA功能指数(SCAFI)和组件),基于规模和主观健康状况(EQ-5D视觉模拟量表(EQVAS))。响应性是决定与病人{\ textquoteright}年代全球印象(PGI)的变化和再现性描述为重新测试可靠性稳定组和最小可检测的变化。结果:1年随访期内,莎拉,SCAFI艾娜,但不是EQVAS恶化表示在整个组和组与主观(PGI)恶化。SCAFI及其9洞小钉板(9成稳定组组件还在恶化。标准化反应意味着最高9 hpt(-0.67),萨拉(0.50),和SCAFI(-0.48)与相应的较低的样本大小的估计143,250,或275每组2-arm介入试验,旨在降低疾病进展50 \ %。莎拉和EQVAS表现最好区分组织列为PGI更糟。所有尺度上除了EQVAS达到的标准测试的可靠性。 Conclusion: While both the Scale for the Assessment and Rating of Ataxia and the SCA Functional Index (SCAFI) (and its 9-hole pegboard component) had favorable measurement precision, the clinical relevance of SCAFI and 9-hole pegboard score changes warrants further exploration. The EQ-5D visual analogue scale proved insufficient for longitudinal assessment, but validly reflected patients{\textquoteright} impression of change. 8MW=8-m walking time; 9HPT=9-hole pegboard; AUC=area under the curve; EQVAS=EQ-5D visual analogue scale; ES=effect size; ICC=intraclass correlation coefficient; INAS=Inventory of Non-Ataxia Symptoms; PATA=rate of PATA repetition; PGI=patient{\textquoteright}s global impression; SARA=Scale for the Assessment and Rating of Ataxia; SCA=spinocerebellar ataxia; SCAFI=SCA Functional Index; SDC=smallest detectable change; SEM=standard errors of measurement; SRM=standardized response mean.}, issn = {0028-3878}, URL = {//www.ez-admanager.com/content/74/8/678}, eprint = {//www.ez-admanager.com/content/74/8/678.full.pdf}, journal = {Neurology} }