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Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale
S H Subramony1, W May, D Lynch
1Department of Neurology, University of Mississippi Medical Center, Jackson 39216, USA. ssubramony@neurology.umsmed.edu
Abstract:
Measuring the severity of neurologic dysfunction in patients with inherited ataxias, including Friedreich ataxia (FA), is difficult because of the variable rate of progression, the variable age at onset and the variety of neural systems that may be affected. The authors discuss the problems related to rating scales in the ataxias, report a neurologic rating scale for FA, and demonstrate acceptable interrater reliability of the instrument.