{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,13]],"date-time":"2026-09-13T01:15:05Z","timestamp":1789262105264,"version":"build-2803163510"},"reference-count":25,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2019,5,23]],"date-time":"2019-05-23T00:00:00Z","timestamp":1558569600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["81100851"],"award-info":[{"award-number":["81100851"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","award":["U1505222"],"award-info":[{"award-number":["U1505222"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003392","name":"Natural Science Foundation of Fujian Province","doi-asserted-by":"publisher","award":["2018J01156"],"award-info":[{"award-number":["2018J01156"]}],"id":[{"id":"10.13039\/501100003392","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Molec Gen &amp; Gen Med"],"published-print":{"date-parts":[[2019,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Spinocerebellar ataxia type 3 (SCA3) is a rare, inherited form of ataxia that leads to progressive neurodegeneration. The initial symptoms could affect clinical phenotypes in neurodegenerative diseases, such as Parkinson's disease and amyotrophic lateral sclerosis. However, the contribution of initial symptoms to the phenotypes of SCA3 has been scarcely investigated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In the present study, 143 SCA3 patients from China were recruited and divided into two groups of gait\u2010onset and non\u2010gait\u2010onset. For determining the influences of initial symptoms on age at onset (AAO), the severity and progression of ataxia, and the possible factors affecting the initial symptoms, multivariable linear regression, and multivariate logistic regression were performed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We found that the frequency of gait\u2010onset was 87.41%, and the frequency of non\u2010gait\u2010onset was 12.59% (diplopia: 7.69%, dysarthria: 4.20%, dystonia: 0.70%). Compared to the non\u2010gait\u2010onset group, the gait\u2010onset group had significantly more severe ataxia (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.046), while the initial symptoms had no effect on AAO (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.109) and progression of ataxia (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.265). We failed to find the existence of any factors affecting initial symptoms.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>These findings collectively suggested that initial symptoms influenced phenotypes in SCA3 and that neurodegeneration in different parts of brain may induce different disease severity in SCA3.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mgg3.719","type":"journal-article","created":{"date-parts":[[2019,5,24]],"date-time":"2019-05-24T04:14:09Z","timestamp":1558671249000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["The influence of initial symptoms on phenotypes in spinocerebellar ataxia type 3"],"prefix":"10.1002","volume":"7","author":[{"given":"Hao\u2010Ling","family":"Xu","sequence":"first","affiliation":[{"name":"Department of Neurology and Institute of Neurology First Affiliated Hospital, Fujian Medical University Fuzhou 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Affiliated Hospital, Fujian Medical University Fuzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ning","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Neurology and Institute of Neurology First Affiliated Hospital, Fujian Medical University Fuzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Neurology and Institute of Neurology First Affiliated Hospital, Fujian Medical University Fuzhou China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7552-5141","authenticated-orcid":false,"given":"Shi\u2010Rui","family":"Gan","sequence":"additional","affiliation":[{"name":"Department of Neurology and Institute of Neurology First Affiliated Hospital, Fujian Medical University Fuzhou 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