title: The international cooperative ataxia rating scale in Machado-Joseph disease. Comparison with the unified multiple system atrophy rating scale.
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 (alpha > 0.90), except for the oculomotor (OD) subscore (alpha = 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.
authors: Neurology Department, Cidade Universitária Zeferino Vaz, Campinas-SP, Brazil.
authors: Anelyssa
authors: A
authors: D'Abreu
authors: Marcondes
authors: M
authors: Franca
authors: Iscia
authors: I
authors: Lopes-Cendes
authors: Fernando
authors: F
authors: Cendes
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002869:Chromosome Aberrations; D004185:Disability Evaluation; D004421:Dystonia / Q000175:diagnosis / Q000235:genetics; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D017827:Machado-Joseph Disease / Q000175:diagnosis* / Q000235:genetics; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000175:diagnosis* / Q000235:genetics; D009460:Neurologic Examination / Q000379:methods*; D015203:Reproducibility of Results; D018911:Trinucleotide Repeats