Scale for the assessment and rating of ataxia: Development of a new clinical scale

T. Schmitz-Hübsch, S. Tezenas Du Montcel, L. Balikó, J. Berciano, S. Boesch, C. Depondt, P. Giunti, C. Globas, J. Infante, J. S. Kang, B. Kremer, C. Mariotti, B. Melegh, M. Pandolfo, M. Rakowicz, P. Ribai, R. Rola, L. Schöls, S. Szymanski, B. P. Van De WarrenburgA. Dürr, T. Klockgether

Research output: Article

682 Citations (Scopus)

Abstract

OBJECTIVE: To develop a reliable and valid clinical scale measuring the severity of ataxia. METHODS: The authors devised the Scale for the Assessment and Rating of Ataxia (SARA) and tested it in two trials of 167 and 119 patients with spinocerebellar ataxia. RESULTS: The mean time to administer SARA in patients was 14.2 ± 7.5 minutes (range 5 to 40). Interrater reliability was high, with an intraclass coefficient (ICC) of 0.98. Test-retest reliability was high with an ICC of 0.90. Internal consistency was high as indicated by Cronbach's α of 0.94. Factorial analysis revealed that the rating results were determined by a single factor. SARA ratings showed a linear relation to global assessments using a visual analogue scale, suggesting linearity of the scale (p <0.0001, r = 0.98). SARA score increased with the disease stage (p <0.001) and was closely correlated with the Barthel Index (r = -0.80, p <0.001) and part IV (functional assessment) of the Unified Huntington's Disease Rating Scale (UHDRS-IV) (r = -0.89, p <0.0001), whereas it had only a weak correlation with disease duration (r = 0.34, p <0.0002) CONCLUSIONS: The Scale for the Assessment and Rating of Ataxia is a reliable and valid measure of ataxia, making it an appropriate primary outcome measure for clinical trials.

Original languageEnglish
Pages (from-to)1717-1720
Number of pages4
JournalNeurology
Volume66
Issue number11
DOIs
Publication statusPublished - jún. 2006

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Ataxia
Spinocerebellar Ataxias
Huntington Disease
Visual Analog Scale
Reproducibility of Results
Outcome Assessment (Health Care)
Clinical Trials

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Schmitz-Hübsch, T., Du Montcel, S. T., Balikó, L., Berciano, J., Boesch, S., Depondt, C., ... Klockgether, T. (2006). Scale for the assessment and rating of ataxia: Development of a new clinical scale. Neurology, 66(11), 1717-1720. https://doi.org/10.1212/01.wnl.0000219042.60538.92

Scale for the assessment and rating of ataxia : Development of a new clinical scale. / Schmitz-Hübsch, T.; Du Montcel, S. Tezenas; Balikó, L.; Berciano, J.; Boesch, S.; Depondt, C.; Giunti, P.; Globas, C.; Infante, J.; Kang, J. S.; Kremer, B.; Mariotti, C.; Melegh, B.; Pandolfo, M.; Rakowicz, M.; Ribai, P.; Rola, R.; Schöls, L.; Szymanski, S.; Van De Warrenburg, B. P.; Dürr, A.; Klockgether, T.

In: Neurology, Vol. 66, No. 11, 06.2006, p. 1717-1720.

Research output: Article

Schmitz-Hübsch, T, Du Montcel, ST, Balikó, L, Berciano, J, Boesch, S, Depondt, C, Giunti, P, Globas, C, Infante, J, Kang, JS, Kremer, B, Mariotti, C, Melegh, B, Pandolfo, M, Rakowicz, M, Ribai, P, Rola, R, Schöls, L, Szymanski, S, Van De Warrenburg, BP, Dürr, A & Klockgether, T 2006, 'Scale for the assessment and rating of ataxia: Development of a new clinical scale', Neurology, vol. 66, no. 11, pp. 1717-1720. https://doi.org/10.1212/01.wnl.0000219042.60538.92
Schmitz-Hübsch T, Du Montcel ST, Balikó L, Berciano J, Boesch S, Depondt C et al. Scale for the assessment and rating of ataxia: Development of a new clinical scale. Neurology. 2006 jún.;66(11):1717-1720. https://doi.org/10.1212/01.wnl.0000219042.60538.92
Schmitz-Hübsch, T. ; Du Montcel, S. Tezenas ; Balikó, L. ; Berciano, J. ; Boesch, S. ; Depondt, C. ; Giunti, P. ; Globas, C. ; Infante, J. ; Kang, J. S. ; Kremer, B. ; Mariotti, C. ; Melegh, B. ; Pandolfo, M. ; Rakowicz, M. ; Ribai, P. ; Rola, R. ; Schöls, L. ; Szymanski, S. ; Van De Warrenburg, B. P. ; Dürr, A. ; Klockgether, T. / Scale for the assessment and rating of ataxia : Development of a new clinical scale. In: Neurology. 2006 ; Vol. 66, No. 11. pp. 1717-1720.
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T2 - Development of a new clinical scale

AU - Schmitz-Hübsch, T.

AU - Du Montcel, S. Tezenas

AU - Balikó, L.

AU - Berciano, J.

AU - Boesch, S.

AU - Depondt, C.

AU - Giunti, P.

AU - Globas, C.

AU - Infante, J.

AU - Kang, J. S.

AU - Kremer, B.

AU - Mariotti, C.

AU - Melegh, B.

AU - Pandolfo, M.

AU - Rakowicz, M.

AU - Ribai, P.

AU - Rola, R.

AU - Schöls, L.

AU - Szymanski, S.

AU - Van De Warrenburg, B. P.

AU - Dürr, A.

AU - Klockgether, T.

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Y1 - 2006/6

N2 - OBJECTIVE: To develop a reliable and valid clinical scale measuring the severity of ataxia. METHODS: The authors devised the Scale for the Assessment and Rating of Ataxia (SARA) and tested it in two trials of 167 and 119 patients with spinocerebellar ataxia. RESULTS: The mean time to administer SARA in patients was 14.2 ± 7.5 minutes (range 5 to 40). Interrater reliability was high, with an intraclass coefficient (ICC) of 0.98. Test-retest reliability was high with an ICC of 0.90. Internal consistency was high as indicated by Cronbach's α of 0.94. Factorial analysis revealed that the rating results were determined by a single factor. SARA ratings showed a linear relation to global assessments using a visual analogue scale, suggesting linearity of the scale (p <0.0001, r = 0.98). SARA score increased with the disease stage (p <0.001) and was closely correlated with the Barthel Index (r = -0.80, p <0.001) and part IV (functional assessment) of the Unified Huntington's Disease Rating Scale (UHDRS-IV) (r = -0.89, p <0.0001), whereas it had only a weak correlation with disease duration (r = 0.34, p <0.0002) CONCLUSIONS: The Scale for the Assessment and Rating of Ataxia is a reliable and valid measure of ataxia, making it an appropriate primary outcome measure for clinical trials.

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