Skip to main content
SpringerLink
Log in

15-White Dots APP-Coo-Test: a reliable touch-screen application for assessing upper limb movement impairment in patients with cerebellar ataxias

  • Original Communication
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Background

The use of objective measurements is essential to assess disease progression and to evaluate the effectiveness of rehabilitation protocols and clinical treatments.

Aim

The purpose of this study was to develop a touch-screen application, that we named 15-White Dots APP-Coo-Test (15-WDACT), able to carry out quantitative and objective measurements of the rapid and coordinated upper limb movements, typically impaired in patients with cerebellar ataxias (CA).

Methods

A total of 87 CA patients and 170 healthy subjects participated in this study. The subject was asked to touch with their index finger a white dot, appearing consecutively and randomly on the screen at different positions, for a total of 15 dots per session. The score is the execution time of a single session.

Results

15-WDACT measurements have highly correlated with the scores obtained with the Scale for the Assessment and Rating of Ataxia (SARA), with the Composite Cerebellar Functional Severity (CCFS) and with the measurements obtained using two validated evaluating systems, i.e., the Nine Hole Pegboard test (9HPT) and the Click Test. We also observed high internal consistency and an excellent intra-rater and test–retest reliability. We found a small Standard Error of Measurement (SEM) and an excellent Minimal Detectable Change (MDC), indicating that even small variations in the 15-WDACT measurements are to be associated with real changes in performance.

Conclusions

We have concluded that 15-WDACT is an easy, fast and reliable tool to assess the severity of the upper limb ataxia in patients with CA.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Bodranghien F, Bastian A, Casali C, Hallett M, Louis ED, Manto M et al (2016) Consensus paper: revisiting the symptoms and signs of cerebellar syndrome. Cerebellum 15(3):369–391

    Article  PubMed  PubMed Central  Google Scholar 

  2. Patel M, Isaacs CJ, Seyer L, Brigatti K, Gelbard S, Strawer C et al (2016) Progression of Friedreich ataxia: quantitative characterization over 5 years. Ann Clin Transl Neurol 3:684–694

    Article  PubMed  PubMed Central  Google Scholar 

  3. Reetz K, Dogan I, Hilgers RD, Giunti P, Mariotti C, Durr A et al (2016) Biological and clinical characteristics of the European Friedreich’s Ataxia Consortium for Translational Studies (EFACTS) cohort: a cross-sectional analysis of baseline data. Lancet Neurol 15:1346–1354

    Article  PubMed  Google Scholar 

  4. Diallo A, Jacobi H, Cook A, Labrum R, Durr A, Brice A et al (2018) Survival in patients with spinocerebellar ataxias types 1,2,3 and 6 (EUROSCA): a longitudinal cohort study. Lancet Neurol 17:327–334

    Article  PubMed  Google Scholar 

  5. Yabe I, Matsushima M (2008) Usefulness of the scale for the assessment and rating of ataxia (SARA). J Neurol Sci 266:164–166

    Article  PubMed  Google Scholar 

  6. Schmitz-Hubsch T et al (2006) Scale for the assessment of ataxia: development of a new clinical scale. Neurology 66:1717–1720

    Article  CAS  Google Scholar 

  7. Weyer A, Abele M, Schmitz-Hubsch T (2007) Reliability and validity of the scale for the assessment and rating of ataxia: a study in 64 ataxia patients. Mov Disord 22:1633–1637

    Article  PubMed  Google Scholar 

  8. Bürk K, Sival DA (2018) Scales for clinical evaluation of cerebellar disorders. Hand Clin Neurol 154:329–339

    Article  Google Scholar 

  9. Tanguy Melac A, Mariotti C, Filipovic Pierucci A, Giunti P, Arpa J, Boesch S et al (2018) Friedreich and dominant ataxias: quantitative differences in cerebellar dysfunction measurements. J Neurol Neurosurg Psychiatry 89:559–565

    Article  PubMed  Google Scholar 

  10. Schmitz-Hübsch T, Tezenas-Du-Montcel S, Baliko L, Boesch S, Bonato S, Fancellu R et al (2005) Reliability and validity of the international cooperative ataxia rating scale: a study in 156 spinocerebellar ataxia patients. Mov Disord 21:699–704

    Article  Google Scholar 

  11. Tison F, Yekhlef F (2002) Application of the international cooperative ataxia scale rating in multiple system atrophy. Mov Disord 17(6):1248–1254

    Article  PubMed  Google Scholar 

  12. Schmitz-Hübsch T, Giunti P, Stephenson DA, Globas C, Baliko L, Saccà F et al (2008) SCA Functional Index—usefulness of a compound performance measure in spinocerebellar ataxia patients. Neurology 71:486–492

    Article  PubMed  Google Scholar 

  13. Schmitz-Hübsch T, Fimmers R, Rakowicz M, Rola R, Zdzienicka E, Fancellu R et al (2010) Responsiveness of different rating instruments in spinocerebellar ataxia patients. Neurology 74:678–684

    Article  PubMed  Google Scholar 

  14. du Montcel ST, Charles P, Ribai P, Goizet C, Le Bayon A, Labauge P et al (2008) Composite cerebellar functional severity score: validation of a quantitative score of cerebellar impairment. Brain 131:1352–1361

    Article  PubMed  Google Scholar 

  15. Mathiowetz V, Weber K, Kashman N, Volland G (1985) Adult norms for the nine hole peg test of finger dexterity. Occup Ther J Res 5(1):24–38

    Article  Google Scholar 

  16. Oxford Grice K, Vogel KA, Le V, Mitchell A, Muniz S, Vollmer MA (2003) Adult norms for a commercially available nine hole peg test for finger dexterity. Am J Occup Ther 57(5):570–573

    Article  PubMed  Google Scholar 

  17. Paap BK, Roeske S, Durr A, Schöls L, Ashizawa T, Boesch S, Bunn LM et al (2016) Standardized assessment of hereditary ataxia patients in clinical studies. Mov Disord Clin Pract 3(3):230–240

    Article  PubMed  PubMed Central  Google Scholar 

  18. Tanguy Melac A, Mariotti C, Filipovic Pierucci A, Giunti P, Arpa J, Boesch S, Klopstock T, Müller Vom Hagen J, Klockgether T, Bürk K, Schulz JB, Reetz K, Pandolfo M, Durr A, Tezenas du Montcel S (2018) Friedreich and dominant ataxias: quantitative differences in cerebellar dysfunction measurements. J Neurol Neurosurg Psychiatry 89(6):559–565

    Article  PubMed  Google Scholar 

  19. Portney LG, Watkins MP (2009) Foundations of clinical research: applications to practice. Pearson/Prentice Hall, Upper Saddle River

    Google Scholar 

  20. Baumgartner TA, Chung H (2001) Confidence limits for intraclass reliability coefficients. Meas Phys Educ Exerc Sci 5:179–188

    Article  Google Scholar 

  21. Harvill L (1991) Standard error of measurement. Educ Meas 10:33–41

    Article  Google Scholar 

  22. Donoghue D, Stokes EK (2009) How much change is true change? The minimum detectable change of the Berg Balance Scale in elderly people. J Rehabil Med 41:343–346

    Article  PubMed  Google Scholar 

  23. Haley SM, Fragala-Pinkham MA (2006) Interpreting change scores of tests and measures used in physical therapy. Phys Ther 86:735–743

    PubMed  Google Scholar 

  24. Serrao M, Pierelli F, Ranavolo A, Draicchio F, Conte C, Casali C et al (2012) Gait pattern in inherited cerebellar ataxias. Cerebellum 11(1):194–211

    Article  PubMed  Google Scholar 

  25. Fujioka S, Sundal C, Zbigniew K (2013) Autosomal dominant cerebellar ataxia type III: a review of the phenotypic and genotypic characteristics. Orphanet J Rare Dis 18(8):14

    Article  Google Scholar 

  26. Schols L, Bauer P, Schmidt T, Schulte T, Riess O (2004) Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis. Lancet Neurol 3:291–304

    Article  Google Scholar 

  27. Basri R, Yabe I, Soma H, Sasaki H (2007) Spectrum and prevalence of autosomal dominant spinocerebellar ataxia in Hokkaido, the northern island of Japan: a study of 113 Japanese families. J Hum Genet 52:848–855

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

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

    Giuseppe Arcuria, Christian Marcotulli, Claudio Galasso, Francesco Pierelli & Carlo Casali

Authors
  1. Giuseppe Arcuria
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Christian Marcotulli
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Claudio Galasso
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Francesco Pierelli
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Carlo Casali
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Giuseppe Arcuria.

Ethics declarations

Conflicts of interest

The manuscript has been approved by all the authors. The authors declare that they have no financial kind of relationships that might lead to a conflict of interest.

Ethical approval

This study was conducted in the Department of Medical and Surgical Sciences and Biotechnologies, “Sapienza” University of Rome, at “Marco Pasquali” Institute—ICOT (Latina), from October 2016 to September 2018. All patients who participated to the study were informed of the use of measurements for research purposes and gave their written informed consent. All the procedures performed were in accordance with the ethical standards of the institutional and national research committee. The regulations of our institution concerning intellectual property has been respected. The ethical standards, laid down in 1964 Declaration of Helsinki and its later amendments, were respected.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 How to select the APP-Coo-Test format (a) and how to download the PDF report (b) (PDF 194 kb)

415_2019_9299_MOESM2_ESM.pdf

Supplementary material 2 Four different examples of reports generated by the APP-Coo-Test. a refers to a healthy subject, while b, c and d refer to three patients with different ataxia levels as reported by a SARA score of 10, 20 and 30, respectively (PDF 107 kb)

415_2019_9299_MOESM3_ESM.pdf

Supplementary material 3 Some phases of the 15-WDACT: The patient selects the 15-WDACT on the screen (a), then presses the start button (b); the countdown that prepares the patient to perform the test starts (c, d, e); the first dot appears on the screen and the test begins (f); the patient types with their index finger on the 15 white dots that will appear on the screen sequentially in random positions (g–i, l–v); after reaching all the 15th white dots the test stops and the execution time appears on the screen (z) (PDF 307 kb)

Supplementary material 4 (MPG 1368 kb)

Supplementary material 5 (PDF 33 kb)

Supplementary material 6 (PDF 75 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Arcuria, G., Marcotulli, C., Galasso, C. et al. 15-White Dots APP-Coo-Test: a reliable touch-screen application for assessing upper limb movement impairment in patients with cerebellar ataxias. J Neurol 266, 1611–1622 (2019). https://doi.org/10.1007/s00415-019-09299-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-019-09299-9

Keywords

Search

Navigation