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

被引:1459
作者
Schmitz-Huebsch, T.
du Montcel, S. Tezenas
Baliko, 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.
Schoels, L.
Szymanski, S.
de Warrenburg, B. P. van
Durr, A.
Klockgether, T.
机构
[1] Univ Hosp, Dept Neurol, D-53105 Bonn, Germany
[2] Hop La Pitie Salpetriere, Assistance Publ Hop, Dept Biostat & Med Informat, Paris, France
[3] Univ Paris 06, EA 3974, Paris, France
[4] Cty Hosp, Dept Neurol & Stroke, Veszprem, Hungary
[5] Univ Hosp Marques Valldecilla, Dept Neurol, Santander, Spain
[6] Univ Innsbruck, Dept Neurol, A-6020 Innsbruck, Austria
[7] Univ Libre Bruxelles, Hop Erasme, Dept Neurol, Brussels, Belgium
[8] Ist Nazl Neurol Carlo Besta, Dept Biochem & Genet, Milan, Italy
[9] Inst Neurol, Dept Mol Sci, London WC1N 3BG, England
[10] Univ Tubingen, Dept Neurol, D-7400 Tubingen, Germany
[11] Univ Tubingen, Hertie Inst Clin Brain Res, D-7400 Tubingen, Germany
[12] Goethe Univ Frankfurt, Dept Neurol, D-6000 Frankfurt, Germany
[13] Radboud Univ Nijmegen, Med Ctr, Dept Neurol, Nijmegen, Netherlands
[14] Univ Pecs, Dept Med Genet & Child Dev, Pecs, Hungary
[15] Inst Psychiat & Neurol, Warsaw, Poland
[16] Hop La Pitie Salpetriere, INSERM, U679, Paris, France
[17] Hop La Pitie Salpetriere, Dept Genet, Paris, France
[18] Univ Hosp Bochum, St Josef Hosp, Dept Neurol, Bochum, Germany
关键词
D O I
10.1212/01.wnl.0000219042.60538.92
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
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收藏
页码:1717 / 1720
页数:4
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