title: Comparison of three clinical rating scales in Friedreich ataxia (FRDA).
abstract: 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.
authors: Department of Neurodegeneration and Restorative Research, Centers of Molecular Physiology of the Brain and Neurological Medicine, University of Göttingen, Göttingen, Germany. buerk@ngi.de
authors: Katrin
authors: K
authors: Bürk
authors: Ulrike
authors: U
authors: Mälzig
authors: Stefanie
authors: S
authors: Wolf
authors: Suzette
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authors: Heck
authors: Konstantinos
authors: K
authors: Dimitriadis
authors: Tanja
authors: T
authors: Schmitz-Hübsch
authors: Sascha
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authors: Hering
authors: Tobias M
authors: TM
authors: Lindig
authors: Verena
authors: V
authors: Haug
authors: Dagmar
authors: D
authors: Timmann
authors: Ingrid
authors: I
authors: Degen
authors: Bernd
authors: B
authors: Kruse
authors: Jan-Markus
authors: JM
authors: Dörr
authors: Susanne
authors: S
authors: Ratzka
authors: Anja
authors: A
authors: Ivo
authors: Ludger
authors: L
authors: Schöls
authors: Sylvia
authors: S
authors: Boesch
authors: Thomas
authors: T
authors: Klockgether
authors: Thomas
authors: T
authors: Klopstock
authors: Jörg B
authors: JB
authors: Schulz
mesh_terms: 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