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The international cooperative ataxia rating scale in Machado-Joseph disease. Comparison with the unified multiple system atrophy rating scale

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Abstract

Our purpose was to evaluate and compare the international cooperative ataxia rating scale (ICARS) and the unified multiple system atrophy rating scale (UMSARS) in patients with Machado-Joseph disease (MJD). We assessed 52 consecutive subjects with MJD using each scale. Both scales had adequate internal consistency (α > 0.90), except for the oculomotor (OD) subscore (α = 0.08). Patients with dystonia had the highest scores in both scales, and symptoms other than ataxia clearly confounded the total ICARS score. There was a very strong correlation between the ICARS and UMSARS-II (motor function), and the correlations between the ICARS and UMSARS-I (r = 0.79) (history) and UMSARS-IV (r = 0.69) (disability) were also statistically significant. We found no significant changes in scores after a mean interval of 7.7 months, although there was after a mean interval of 13.3 months. We conclude that the total ICARS score is a reliable method for longitudinal evaluation of ataxia in MJD, but a disease specific scale should be developed. © 2007 Movement Disorder Society

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