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

To develop a reliable and valid clinical scale measuring the severity of ataxia. 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. The mean time to administer SARA in patients was 14.2 +/- 7.5...

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Veröffentlicht in:Neurology 2006-06, Vol.66 (11), p.1717-1720
Hauptverfasser: SCHMITZ-HÜBSCH, T, TEZENAS DU MONTCEL, S, KREMER, B, MARIOTTI, C, MELEGH, B, PANDOLFO, M, RAKOWICZ, M, RIBAI, P, ROLA, R, SCHOLS, L, SZYMANSKI, S, VAN DE WARRENBURG, B. P, BALIKO, L, DÜRR, A, KLOCKGETHER, T, BERCIANO, J, BOESCH, S, DEPONDT, C, GIUNTI, P, GLOBAS, C, INFANTE, J, KANG, J.-S
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container_end_page 1720
container_issue 11
container_start_page 1717
container_title Neurology
container_volume 66
creator SCHMITZ-HÜBSCH, T
TEZENAS DU MONTCEL, S
KREMER, B
MARIOTTI, C
MELEGH, B
PANDOLFO, M
RAKOWICZ, M
RIBAI, P
ROLA, R
SCHOLS, L
SZYMANSKI, S
VAN DE WARRENBURG, B. P
BALIKO, L
DÜRR, A
KLOCKGETHER, T
BERCIANO, J
BOESCH, S
DEPONDT, C
GIUNTI, P
GLOBAS, C
INFANTE, J
KANG, J.-S
description To develop a reliable and valid clinical scale measuring the severity of ataxia. 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. 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). 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.
doi_str_mv 10.1212/01.wnl.0000219042.60538.92
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subjects Adolescent
Adult
Aged
Aged, 80 and over
Biological and medical sciences
Development. Senescence. Regeneration. Transplantation
Female
Fundamental and applied biological sciences. Psychology
Health Status Indicators
Humans
Male
Medical sciences
Middle Aged
Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis
Neurologic Examination - methods
Neurology
Outcome Assessment (Health Care) - methods
Reproducibility of Results
Sensitivity and Specificity
Severity of Illness Index
Spinocerebellar Ataxias - classification
Spinocerebellar Ataxias - diagnosis
Vertebrates: nervous system and sense organs
title Scale for the assessment and rating of ataxia : Development of a new clinical scale
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