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首页> 外文期刊>Journal of neurology >How does performance of the Friedreich Ataxia Functional Composite compare to rating scales?
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How does performance of the Friedreich Ataxia Functional Composite compare to rating scales?

机译：Friedreich Ataxia功能复合的表现如何与评级尺度相比？

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摘要

Progression of Friedreich ataxia (FRDA) is often measured using neurological rating scales such as the Friedreich Ataxia Rating Scale (FARS). Performance scales comprising functional measures have been used in other conditions due to their increased sensitivity and reproducibility and may replace examination-based measures. The aims of this study were to examine the relationship between the Friedreich Ataxia Functional Composite (FAFC) measures and characteristics of FRDA to determine if the FAFC is more sensitive to clinical change over time compared to its components. One hundred and twenty-two individuals completed the timed 25-foot walk (T25FW), 9-Hole Peg Test (9HPT) and the low-contrast letter acuity (LCLA) test at baseline, 63 at year 1, 34 at year 2 and 25 at year 3. Composite scores, Z2 (T25FW and 9HPT) and Z3 (T25FW, 9HPT and LCLA) were created. Correlation analyses were conducted. Change in FAFC components were examined over 1, 2, and 3 years. The FARS, Z2, Z3 and 9HPT showed significant change over all time points compared to baseline. The T25FW only demonstrated significant change over 3 years. The LCLA demonstrated no significant change over any of the time points. The FAFC shows significant change over time and indicates disease progression, however, this may result from individual components driving the differences. The LCLA showed no change over time, rendering Z3 redundant. The FAFC is of limited value in cohorts with non-ambulant individuals as it leads to skewing of the dataset and is better suited to less affected populations.
机译：Friedreich Ataxia（FRDA）的进展通常使用神经学评级等级来衡量，例如Friedreich Ataxia评级规模（FARS）。包括功能措施的性能尺度已在其他条件下使用，因为它们增加了灵敏度和再现性，并且可以取代基于检查的措施。本研究的目的是检查Friedreich Ataxia功能复合物（FAFC）措施和FRDA特征之间的关系，以确定FAFC是否与其组件相比随着时间的推移对临床变化更敏感。一百二十二个人完成了定时25英尺的步行（T25FW），9孔PEG测试（9HPT）和基线的低对比度敏锐度（LCLA）测试，第1岁及24年，34年，34年， 25年35日，创建了综合评分，Z2（T25FW和9HPT）和Z3（T25FW，9HPT和LCLA）。进行相关分析。在1,2和3年内检查FAFC组件的变化。与基线相比，FARS，Z2，Z3和9HPT显示出所有时间点的显着变化。 T25FW只表现出3年超过3年的重大变化。 LCLA在任何时间点都证明没有重大变化。 FAFC随着时间的推移显示出显着的变化，并且表明疾病进展，然而，这可能是推动差异的各个组件。 LCLA显示随着时间的推移没有变化，渲染Z3冗余。 FAFC在与非驻守个人的群组中的价值有限，因为它导致数据集的偏差，更适合受影响的人口。

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