Scale for the assessment and rating of ataxia: development of a new clinical scale.
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.
Schmitz-Hübsch
T
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Department of Neurology, University Hospital of Bonn, Sigmund-Freud-Str. 25, D-53105 Bonn, Germany.
du Montcel
S Tezenas
ST
Baliko
L
L
Berciano
J
J
Boesch
S
S
Depondt
C
C
Giunti
P
P
Globas
C
C
Infante
J
J
Kang
J-S
JS
Kremer
B
B
Mariotti
C
C
Melegh
B
B
Pandolfo
M
M
Rakowicz
M
M
Ribai
P
P
Rola
R
R
Schöls
L
L
Szymanski
S
S
van de Warrenburg
B P
BP
Dürr
A
A
Klockgether
T
T
Fancellu
Roberto
R
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006305:Health Status Indicators; D006801:Humans; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination / Q000379:methods*; D017063:Outcome Assessment, Health Care / Q000379:methods*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D020754:Spinocerebellar Ataxias / Q000145:classification* / Q000175:diagnosis*