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

Mov Disord. 2009 Sep 15;24(12):1779-84. doi: 10.1002/mds.22660.


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.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Disability Evaluation*
  • Female
  • Friedreich Ataxia / diagnosis*
  • Friedreich Ataxia / genetics
  • Friedreich Ataxia / physiopathology
  • Humans
  • Male
  • Middle Aged
  • Outcome Assessment, Health Care*
  • Principal Component Analysis
  • Psychometrics
  • Reproducibility of Results
  • Severity of Illness Index*
  • Statistics as Topic
  • Young Adult