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

T Schmitz-Hübsch, S Tezenas du Montcel, L Baliko, 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, Roberto Fancellu

Research output: Contribution to journalArticleAcademicpeer-review

1507 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 alpha 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(2) = 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 - 13-Jun-2006
Externally publishedYes

Keywords

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Female
  • Health Status Indicators
  • Humans
  • Male
  • Middle Aged
  • Neurologic Examination
  • Outcome Assessment (Health Care)
  • Reproducibility of Results
  • Sensitivity and Specificity
  • Severity of Illness Index
  • Spinocerebellar Ataxias
  • Journal Article
  • Randomized Controlled Trial
  • Research Support, Non-U.S. Gov't

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