Monitoring progression in Friedreich ataxia (FRDA): the use of clinical scales
Version of Record online: 17 JUL 2013
© 2013 International Society for Neurochemistry
Journal of Neurochemistry
Special Issue: 150 Years of Friedreich Ataxia Research. The International Society for Neurochemistry and Wiley have published this supplement without financial support
Volume 126, Issue Supplement s1, pages 118–124, August 2013
How to Cite
J. Neurochem.(2013) 126 (Suppl. 1),118–124.
- Issue online: 17 JUL 2013
- Version of Record online: 17 JUL 2013
- Manuscript Accepted: 16 MAY 2013
- Manuscript Revised: 15 MAY 2013
- Manuscript Received: 13 MAY 2013
- FRDA ;
Friedreich ataxia (FRDA) is a progressive neurodegenerative disorder associated with ataxia, dysarthria, pyramidal tract signs, sensory loss, cardiomyopathy and diabetes. There is no cure for FRDA so far. Studies of the natural history of the disease and future therapeutic trials require development of appropriate outcome markers. Since any therapeutic benefit is expected to modulate deterioration over time rather than to reverse disability, potential outcome measures must be sensitive instruments carefully analysed for their significance. Clinical scales may represent an appropriate measuring tool. Over the last few years the construction, evaluation and validation of sensitive clinical scales for the assessment of disease severity and progression in ataxia have had considerable impact on our understanding of the disease. Currently, there are three different scales that are most frequently applied: The International Cooperative Ataxia Rating Scale (ICARS), the Friedreich Ataxia Rating Scale (FARS) and the Scale for the Assessment and Rating of Ataxia (SARA). All scales have been validated and compared with regard to their testing properties.