Abstract
Spinal cerebellar ataxia type 3 is a common SCA subtype in the world. It is a neurodegenerative disease characterized by ataxia. Patients exhibit common neuropsychological symptoms such as depression and anxiety. Some patients have suicidal tendencies when they are severely depressed. So, it is very important to study the severity of depression and clinical symptoms (SARA), to find out the patient’s psychological state in time and to help patients actively respond to treatment. A total of 97 Chinese SCA3 patients were enrolled in the study. The Beck Depression Scale was used to investigate the prevalence of depression in the confirmed patients. The distribution of depression data in these patients was investigated. Then, the quantifier was used to model the depression status of Chinese SCA3 patients. An analysis was conducted to identify the key factors affecting depression under different quantiles. Studies have shown that SARA and gender are important factors affecting depression; the effect of initial SARA is small, then the degree of influence increases, and the degree of influence decreases in the later period, but it is always positively correlated with depression; the development of women’s SARA is gentler than that of men, and the degree of depression is lower than that of men.
Highlights
Machado–Joseph disease or spinocerebellar ataxia 3 (MJD/ SCA3) is a clinically heterogeneous, neurodegenerative disorder characterized by varying degrees of ataxia, ophthalmoplegia, peripheral neuropathy, pyramidal dysfunction, and movement disorder
MJD/SCA3 is caused by a CAG repeat expansion mutation in the protein coding region of the ATXN3 gene located at chromosome 14q32.1 [1]
Dementia and hyporeflexia were more frequent in patients with SCA2, while spasticity, hyperreflexia, and Babinski signs were more frequent in patients with SCA3/MJD, and those might be helpful in clinical work to primarily distinguish patients with
Summary
Machado–Joseph disease or spinocerebellar ataxia 3 (MJD/ SCA3) is a clinically heterogeneous, neurodegenerative disorder characterized by varying degrees of ataxia, ophthalmoplegia, peripheral neuropathy, pyramidal dysfunction, and movement disorder. MJD/SCA3 is caused by a CAG repeat expansion mutation in the protein coding region of the ATXN3 gene located at chromosome 14q32.1 [1]. Previous studies on SCA3 focused the pathogenesis of SCA3, CAG mutation amplification, and ethnic differences. In the study of the role of the proteasome in the pathogenesis of SCA3/MJD, it was found that the proteasome plays a direct role in suppressing polyglutamine aggregation in disease. There are a few studies on depression in Chinese SCA3 patients, and the main method is linear regression. Ere are a few studies combining the quantile regression method with the influencing factors of depression in Chinese SCA3 patients. Erefore, it is necessary to study the factors affecting the quantile regression in SCA3 patients with depression Considering the quantile, regression can effectively avoid the heteroscedasticity and nonnormal distribution of the data, and with the change of the quantile used, it can more accurately describe the influence of independent variables on dependent variables and characterize the conditional distribution. erefore, it is necessary to study the factors affecting the quantile regression in SCA3 patients with depression
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