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Scale for the assessment and rating of ataxia

Development of a new clinical scale

From the Department of Neurology (T.S.-H., T.K.), University Hospital of Bonn, Bonn, Germany; Department of Biostatistics and Medical Informatics (S.T.M.), Hôpital de la Pitié-Salpêtrière, Assistance Publique-Hôpitaux, Paris, France; Modeling in Clinical Research (S.T.M.), EA 3974, University Pierre et Marie Curie, Paris, France; Department of Neurology and Stroke (L.B.), County Hospital, Veszprém, Hungary; Department of Neurology (J.B., J.I.), University Hospital Marqués de Valldecilla, Santander, Spain; Department of Neurology (S.B.), University of Innsbruck, Innsbruck, Austria; Department of Neurology (C.D., M.P.), Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium; Department of Biochemistry and Genetics (R.F., C.M.), Istituto Nazionale Neurologico C. Besta, Milan, Italy; Department of Molecular Neuroscience (P.G.), Institute of Neurology, Queen Square, London, United Kingdom; Department of Neurology and Hertie-Institute for Clinical Brain Research (C.G., L.S.), University of Tübingen, Tübingen, Germany; Department of Neurology (J.-S.K.), University of Frankfurt, Frankfurt/M, Germany; Department of Neurology (B.K., B.P.W.), Radboud University Nijmegen Medical Center, Nijmegen, Netherlands; Department of Medical Genetics and Child Development (M.B.), University of Pécs, Pécs, Hungary; Institute of Psychiatry and Neurology (M.R., R.R.), Warsaw, Poland; INSERM U679 and Department of Genetics (P.R., A.D.), Cytogenetics and Embryology, Hôpital de la Pitié-Salpêtrière, Paris, France; Department of Neurology (S.S.), St. Josef Hospital, University Hospital of Bochum, Bochum, Germany.

Address correspondence and reprint requests to Dr. Thomas Klockgether, Department of Neurology, University Hospital of Bonn, Sigmund-Freud-Str. 25, D-53105 Bonn, Germany; e-mail: klockgether{at}uni-bonn.de

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 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, r² = 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.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the June 13 issue to find the title link for this article.

See also page 1711

Supported by grant EUROSCA/LSHM-CT-2004-503304 from the European Union, grant GeneMove/01 GM 0503 from the German Ministry of Education and Research, and grant 3 PO5B 019 24 from the Polish Ministry of Scientific Research and Information Technology.

Disclosure: The authors report no conflicts of interest.

Received October 10, 2005. Accepted in final form March 6, 2006.

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