2006/01/31 by Tanja Schmitz‐Hübsch, Sophie Tézenas du Montcel, László Balikó +18 · 2 citations
Neuroscience · Biochemistry, Genetics and Molecular Biology · Medicine · #Genetic Neurodegenerative Diseases #Mitochondrial Function and Pathology #Neurological disorders and treatments
paper · doi:10.1002/mds.20781
openalex publication_date 2006/01/31 · openalex created_date 2025/10/10 · openalex updated_date 2026/07/26
To evaluate the efficacy of treatments in spinocerebellar ataxias (SCAs), appropriate clinical scales are required. This study evaluated metric properties of the International Cooperative Ataxia Rating Scale (ICARS) in 156 SCA patients and 8 controls. ICARS was found to be a reliable scale satisfying accepted criteria for interrater reliability, test-retest reliability, and internal consistency. Although validity testing was limited, we found evidence of validity of ICARS when ataxia disease stages and Barthel index were used as external criteria. On the other hand, our study revealed two major problems associated with the use of ICARS. First, the redundant and overlapping nature of several items gave rise to a considerable number of contradictory ratings. Second, a factorial analysis showed that the rating results were determined by four different factors that did not coincide with the ICARS subscales, thus questioning the justification of ICARS subscore analysis in clinical trials.