Comparison of three clinical rating scales in Friedreich ataxia (FRDA)

Katrin Bürk, Ulrike Mälzig, Stefanie Wolf, Suzette Heck, Konstantinos Dimitriadis, Tanja Schmitz-Hübsch, Sascha Hering, Tobias M Lindig, Verena Haug, Dagmar Timmann, Ingrid Degen, Bernd Kruse, Jan-Markus Dörr, Susanne Ratzka, Anja Ivo, Ludger Schöls, Sylvia Boesch, Thomas Klockgether, Thomas Klopstock, Jörg B Schulz
Movement Disorders: Official Journal of the Movement Disorder Society 2009 September 15, 24 (12): 1779-84
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.

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