Comparison of three clinical rating scales in Friedreich ataxia (FRDA).
To test the validity and reliability of the scale for the assessment and rating of ataxia (SARA) in Friedreich ataxia (FRDA). SARA is limited to eight items and can be performed rapidly. Ninety-six patients with a molecular genetic diagnosis of FRDA were rated using three different clinical scales, the FRDA Rating Scale (FARS), the International Cooperative Ataxia Rating Scale (ICARS), and SARA. Despite considerable discrepancies in scale size and subscale structure, SARA total scores were significantly correlated with ICARS (r = 0.953, P < 0.0001) and FARS (r = 0.938, P < 0.0001) total scores. SARA total scores also correlated with the activities of daily living (ADL, r = 0.929, P < 0.0001). Although originally developed for the use in dominantly inherited ataxias, which are primarily ataxias of the cerebellar type, SARA can also be used successfully to assess afferent ataxia, which is the predominant form in FRDA. Because SARA is characterized by high interrater reliability and practicability, SARA is applicable and well suited forclinical trials of FRDA.
Bürk
Katrin
K
Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen, Göttingen, Germany. buerk@ngi.de
Mälzig
Ulrike
U
Wolf
Stefanie
S
Heck
Suzette
S
Dimitriadis
Konstantinos
K
Schmitz-Hübsch
Tanja
T
Hering
Sascha
S
Lindig
Tobias M
TM
Haug
Verena
V
Timmann
Dagmar
D
Degen
Ingrid
I
Kruse
Bernd
B
Dörr
Jan-Markus
JM
Ratzka
Susanne
S
Ivo
Anja
A
Schöls
Ludger
L
Boesch
Sylvia
S
Klockgether
Thomas
T
Klopstock
Thomas
T
Schulz
Jörg B
JB
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D004185:Disability Evaluation; D005260:Female; D005621:Friedreich Ataxia / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D025341:Principal Component Analysis; D011594:Psychometrics; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D013223:Statistics as Topic; D055815:Young Adult