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Peripheral blood leukocytes are indicative of the immune status in neurodegenerative diseases. However, alterations in the characteristics of peripheral blood leukocytes at different stages of SCA3 and their potential roles in disease progression remain unclear.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>The goal was to identify leukocyte profiles alterations at different stages of SCA3 and analyze their correlation with disease severity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This cross\u2010sectional study included 150 total <jats:italic>ATXN3<\/jats:italic> expansion carriers (20 pre\u2010ataxic and 130 ataxic) and 113 healthy controls. Differences in leukocyte profiles were analyzed, and correlations with clinical characteristics were assessed using Spearman and partial correlation. Logistic regression and receiver operating characteristic curve identified independent factors associated with disease severity. Mediation analysis evaluated the effect of lymphocyte counts on the relationship between disease duration and SARA scores.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Alterations in neutrophil, lymphocyte, eosinophil, and basophil counts were observed across <jats:italic>ATXN3<\/jats:italic> expansion carriers, including those in pre\u2010ataxic and ataxic stages, compared with healthy controls. Lymphocyte counts negatively correlated with SARA and International Cooperative Ataxia Rating Scale scores, particularly in speech function. Leukocyte, monocyte, and basophil counts were also negatively associated with ataxia scales scores or sub\u2010item scores. Lymphocyte counts distinguished disease severities, and partially mediated the effect of disease duration on ataxia scale scores in ataxic SCA3.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Leukocyte profiles alterations at different stages of SCA3 may be closely linked to disease progression and severity. Lymphocyte counts may serve as valuable indicators of disease severity. \u00a9 2025 International Parkinson and Movement Disorder Society.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.30189","type":"journal-article","created":{"date-parts":[[2025,4,10]],"date-time":"2025-04-10T04:39:06Z","timestamp":1744259946000},"page":"1379-1387","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Decreased Peripheral Blood Lymphocytes in Spinocerebellar Ataxia Type 3 Correlate with Disease Severity"],"prefix":"10.1002","volume":"40","author":[{"given":"Qi","family":"Deng","sequence":"first","affiliation":[{"name":"Department of Neurology Xiangya Hospital, Central South University  Changsha Hunan China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cuiling","family":"Tang","sequence":"additional","affiliation":[{"name":"Department of Neurology Xiangya Hospital, Central South University  Changsha Hunan 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