Department of Medical and Surgical Sciences and Biotechnologies, Sapienza - University of Rome - Polo Pontino, Via Faggiana 34, 40100, Latina, Italy.
Department of Science and Information Technology, I.I.S. Ettore Majorana, Piazza Sen. Marescalchi 2, Piazza Armerina, EN, Italy.
Neurol Sci. 2020 Jun;41(6):1577-1587. doi: 10.1007/s10072-020-04249-0. Epub 2020 Jan 28.
The use of standardized tools and objective measurements is essential to test the effectiveness of new drugs or rehabilitative protocols. Friedreich's ataxia (FRDA) patients with severe disease are often unable to perform the quantitative measurement tests currently used.
The purpose of our study was to develop an easy-to-use application, for touchscreen devices, able to quantify the degree of upper limb movement impairment in patients with severe Friedreich's ataxia. The APP, which we named "Twelve-Red-Squares App-Coo-Test" (12-RSACT), assesses the upper limb ataxia by measuring the test execution time.
All patients were clinically evaluated using the Composite Cerebellar Functional Severity (CCFS) and the Scale for the Assessment and Rating of Ataxia (SARA). We recruited 92 healthy subjects and 36 FRDA patients with a SARA mean value of 28.8.1 ± 8.2. All participants in our study underwent upper limb movement assessment using the new 12-RSACT, the Click Test, and a well-established system, i.e., the Nine-Hole Peg Test (9HPT).
We observed a strong linear correlation between the measurements obtained with the 12-RSACT and those obtained with 9HPT, Click Test, CCFS, and SARA. The 12-RSACT was characterized by excellent internal consistency and intra-rater and test-retest reliability. The minimal detectable change (MDC%) was excellent too. Additionally, the 12-RSACT turned out to be faster and easier to perform compared with the 9HPT.
The 12-RSACT is an inexpensive test and is easy to use, which can be administered quickly. Therefore, 12-RSACT is a promising tool to assess the upper limb ataxia in FRDA patients and even those with severe diseases.
使用标准化工具和客观测量对于测试新药或康复方案的有效性至关重要。患有严重疾病的弗里德里希共济失调（FRDA）患者通常无法进行当前使用的定量测量测试。
我们的研究目的是开发一种易于使用的应用程序，用于触摸屏设备，能够量化严重 FRDA 患者上肢运动障碍的程度。我们将该应用程序命名为“十二红方块应用程序-Coo-Test”（12-RSACT），通过测量测试执行时间来评估上肢共济失调。
所有患者均通过复合小脑功能严重程度（CCFS）和共济失调评估和评分量表（SARA）进行临床评估。我们招募了 92 名健康受试者和 36 名 FRDA 患者，他们的 SARA 平均值为 28.8.1 ± 8.2。我们的研究中的所有参与者均使用新的 12-RSACT、点击测试以及经过验证的系统（即九孔钉测试（9HPT））进行上肢运动评估。
我们观察到 12-RSACT 测量值与 9HPT、点击测试、CCFS 和 SARA 测量值之间存在很强的线性相关性。12-RSACT 具有出色的内部一致性和内部评分者和测试-重测可靠性。最小可检测变化（MDC%）也非常出色。此外，与 9HPT 相比，12-RSACT 操作更快、更容易。
12-RSACT 是一种廉价的测试方法，易于使用，可以快速进行。因此，12-RSACT 是评估 FRDA 患者上肢共济失调的一种很有前途的工具，甚至对患有严重疾病的患者也有效。
