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Developing an objective evaluating system to quantify the degree of upper limb movement impairment in patients with severe Friedreich’s ataxia

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Abstract

Background

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.

Aim

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.

Methods

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).

Results

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.

Conclusion

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.

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Acknowledgments

We thank the patients and healthy subjects who participated in our study.

Author information

Authors and Affiliations

  1. Department of Medical and Surgical Sciences and Biotechnologies, Sapienza - University of Rome - Polo Pontino, Via Faggiana 34, 40100, Latina, Italy

    Giuseppe Arcuria, Christian Marcotulli, Claudio Galasso & Carlo Casali

  2. Department of Science and Information Technology, I.I.S. Ettore Majorana, Piazza Sen. Marescalchi 2, Piazza Armerina, EN, Italy

    Raffaele Amuso

  3. Department of Mathematical, Physical and Natural Sciences, University of Rome “Sapienza”, Piazzale Aldo Moro 5, 00185, Rome, Italy

    Giuliano Dattilo

  4. IRCCS Neuromed, Pozzilli, IS, Italy

    Francesco Pierelli

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  1. Giuseppe Arcuria
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  7. Carlo Casali
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Contributions

All authors contributed to the study conception and design. Material preparation was performed by Giuseppe Arcuria and Raffaele Amuso. Data collection and analysis were performed by Giuseppe Arcuria, Christian Marcotulli, and Claudio Galasso. Giuseppe Arcuria and Giuliano Dattilo did the statistical analyses. All authors contributed to interpretation of the results. The first draft of the manuscript was written by Giuseppe Arcuria and Giuliano Dattilo. Review and supervision were performed by Francesco Pierelli and Carlo Casali. All authors commented on previous versions of the manuscript and read and approved the final manuscript.

Corresponding author

Correspondence to Giuseppe Arcuria.

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The authors declare that they have no conflict of interest.

Ethical approval

All the procedures performed in this study were in accordance with ethical standards laid down in 1964 Declaration of Helsinki and its later amendments or comparable ethical standards. All patients gave their written informed consent before study enrollment.

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Arcuria, G., Marcotulli, C., Amuso, R. et al. Developing an objective evaluating system to quantify the degree of upper limb movement impairment in patients with severe Friedreich’s ataxia. Neurol Sci 41, 1577–1587 (2020). https://doi.org/10.1007/s10072-020-04249-0

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