Related factors of ICARS and SARA scores on spinocerebellar ataxia type 3/Machado-Joseph disease

Jie Zhou, Lifang Lei, Xinxin Liao, Junling Wang, Hong Jiang, Beisha Tang, Lu Shen
Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences 2011, 36 (6): 498-503

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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