JOURNAL ARTICLE
RESEARCH SUPPORT, NON-U.S. GOV'T
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Related factors of ICARS and SARA scores on spinocerebellar ataxia type 3/Machado-Joseph disease.

OBJECTIVE: To investigate the related factors of international cooperative ataxia rating scale (ICARS) and scale for the assessment and rating of ataxia scores (SARA) in patients with spinocerebellar ataxia type 3/Machado-Joseph disease.

METHODS: A total of 126 SCA3/MJD patients were assessed by ICARS and SARA. The relation between ICARS or SARA scores and age of onset, disease duration and CAG repeat size was analyzed.

RESULTS: Either the total ICARS or the total SARA score was positively related with the disease duration(r=0.586,P<0.05;r=0.643,P<0.05). Simple linear regression equations were: Y(1)(total ICARS score)=13.072+2.388 X(2)(disease duration)(F=68.874,P<0.05); Y(2)(total SARA score)=4.403+ 0.961 X(2)(disease duration)(F=87.254, P<0.05). Either age adjusted the total ICARS score or age adjusted the total SARA score was positively related with CAG repeat size(r=0.328, P<0.05; r=0.335, P<0.05). Both the ICARS subscores and the SARA subscores were positively related with the disease duration(r=0.257-0.589, P<0.05; r=0.432-0.623, P<0.05). Both age adjusted ICARS subscores and age adjusted SARA subscores were positively related with CAG repeat size(r=0.263-0.403, P<0.05; r=0.189-0.366, P<0.05). Analysis of variance showed that the total ICARS score and the total SARA score increased with the disease stage.

CONCLUSION: ICARS and SARA are both reliable and effective scales in assessing the severity of ataxia in patients with SCA3/MJD, and researchers can choose the most suitable scale according to specific requirement.

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