Related factors of ICARS and SARA scores on spinocerebellar ataxia type 3/Machado-Joseph disease
10.3969/j.issn.1672-7347.2011.06.005
- VernacularTitle:脊髓小脑性共济失调3型/Machado-Joseph 病ICARS与SARA评分相关因素分析
- Author:
Jie ZHOU
;
Lifang LEI
;
Xinxin LIAO
;
Junling WANG
;
Hong JIANG
;
Beisha TANG
;
Lu SHEN
- Publication Type:Journal Article
- Keywords:
spinocerebellar ataxia type 3;
Machado-Joseph disease;
international cooperative ataxia rating scale (ICARS);
scale for the assessment and rating of ataxia (SARA);
clinical assessment
- From:
Journal of Central South University(Medical Sciences)
2011;36(6):498-503
- CountryChina
- Language:Chinese
-
Abstract:
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: Y1(total ICARS score)=13.072+2.388 X2(disease duration)(F=68.874,P＜0.05); Y2(total SARA score)=4.403+ 0.961 X2(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.
