Dysarthria in Spinocerebellar Ataxia Type 3: Prevalence and Disease Progression.

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Title: Dysarthria in Spinocerebellar Ataxia Type 3: Prevalence and Disease Progression.
Authors: Ji, Hai-Ping1, Cui, Mao-Lin1,2, Lin, Wei1, Huang, Zhuo-Ying1, Ye, Bei-Ning1, Gan, Shi-Rui1,3,4 ganshirui@fjmu.edu.cn
Source: Journal of Speech, Language & Hearing Research. Sep2025, Vol. 68 Issue 9, p4256-4262. 7p.
Subject Terms: *Dysarthria, *Data analysis, *Retrospective studies, Spinocerebellar ataxia, Genomics, T-test (Statistics), Research funding, Polymerase chain reaction, Interviewing, Multiple regression analysis, DNA, Chi-squared test, Mann Whitney U Test, Descriptive statistics, Statistics, Health outcome assessment, Data analysis software, Disease progression, Disease risk factors, Disease complications
Abstract: Background: Spinocerebellar ataxia type 3 (SCA3), a common genetic disorder, results from an expanded CAG repeat in the ATXN3 gene. It often leads to dysarthria, which impacts patients' quality of life. Yet, there is limited research on how dysarthria's prevalence relates to clinical features and disease progression in SCA3. Method: We retrospectively analyzed 183 SCA3 patients, dividing them into dysarthria and non-dysarthria groups based on their "speech disturbance" subscale scores from the Scale for the Assessment and Rating of Ataxia (SARA). Patients with a score of zero were classified as non-dysarthria, while those with higher scores were classified as dysarthria. Spearman's rho tested factor associations with dysarthria; logistic regression identified dysarthria risk factors. Kaplan--Meier curves were employed to demonstrate the onset of dysarthria over the disease duration. Results: We identified a 78.7% prevalence of dysarthria among SCA3 patients. Patients with dysarthria had significantly higher SARA scores (p < .001) and longer disease durations (p < .001). Disease duration showed the strongest association with the occurrence of dysarthria (r = .319, p < .001) and emerged as an independent prognostic factor (p < .001). By the eighth year, 50% of patients exhibited dysarthria, with the highest incidence occurring in the later stages of the first decade. Conclusions: Dysarthria is common in SCA3 patients, with disease duration as the main prognostic factor. The prevalence of dysarthria increases as the disease progresses, particularly during the later stages of the first decade. These findings underscore the need for early intervention to address dysarthria in SCA3 patients, especially as they approach critical disease milestones. [ABSTRACT FROM AUTHOR]
Copyright of Journal of Speech, Language & Hearing Research is the property of American Speech-Language-Hearing Association and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: Dysarthria in Spinocerebellar Ataxia Type 3: Prevalence and Disease Progression.
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  Data: &lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Ji%2C+Hai-Ping%22&quot;&gt;Ji, Hai-Ping&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Cui%2C+Mao-Lin%22&quot;&gt;Cui, Mao-Lin&lt;/searchLink&gt;&lt;relatesTo&gt;1,2&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Lin%2C+Wei%22&quot;&gt;Lin, Wei&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Huang%2C+Zhuo-Ying%22&quot;&gt;Huang, Zhuo-Ying&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Ye%2C+Bei-Ning%22&quot;&gt;Ye, Bei-Ning&lt;/searchLink&gt;&lt;relatesTo&gt;1&lt;/relatesTo&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Gan%2C+Shi-Rui%22&quot;&gt;Gan, Shi-Rui&lt;/searchLink&gt;&lt;relatesTo&gt;1,3,4&lt;/relatesTo&gt;&lt;i&gt; ganshirui@fjmu.edu.cn&lt;/i&gt;
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  Data: *&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Dysarthria%22&quot;&gt;Dysarthria&lt;/searchLink&gt;&lt;br /&gt;*&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Data+analysis%22&quot;&gt;Data analysis&lt;/searchLink&gt;&lt;br /&gt;*&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Retrospective+studies%22&quot;&gt;Retrospective studies&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Spinocerebellar+ataxia%22&quot;&gt;Spinocerebellar ataxia&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Genomics%22&quot;&gt;Genomics&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22T-test+%28Statistics%29%22&quot;&gt;T-test (Statistics)&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Research+funding%22&quot;&gt;Research funding&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Polymerase+chain+reaction%22&quot;&gt;Polymerase chain reaction&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Interviewing%22&quot;&gt;Interviewing&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Multiple+regression+analysis%22&quot;&gt;Multiple regression analysis&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22DNA%22&quot;&gt;DNA&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Chi-squared+test%22&quot;&gt;Chi-squared test&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Mann+Whitney+U+Test%22&quot;&gt;Mann Whitney U Test&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Descriptive+statistics%22&quot;&gt;Descriptive statistics&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Statistics%22&quot;&gt;Statistics&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Health+outcome+assessment%22&quot;&gt;Health outcome assessment&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Data+analysis+software%22&quot;&gt;Data analysis software&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Disease+progression%22&quot;&gt;Disease progression&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Disease+risk+factors%22&quot;&gt;Disease risk factors&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Disease+complications%22&quot;&gt;Disease complications&lt;/searchLink&gt;
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Background: Spinocerebellar ataxia type 3 (SCA3), a common genetic disorder, results from an expanded CAG repeat in the ATXN3 gene. It often leads to dysarthria, which impacts patients&#39; quality of life. Yet, there is limited research on how dysarthria&#39;s prevalence relates to clinical features and disease progression in SCA3. Method: We retrospectively analyzed 183 SCA3 patients, dividing them into dysarthria and non-dysarthria groups based on their &quot;speech disturbance&quot; subscale scores from the Scale for the Assessment and Rating of Ataxia (SARA). Patients with a score of zero were classified as non-dysarthria, while those with higher scores were classified as dysarthria. Spearman&#39;s rho tested factor associations with dysarthria; logistic regression identified dysarthria risk factors. Kaplan--Meier curves were employed to demonstrate the onset of dysarthria over the disease duration. Results: We identified a 78.7% prevalence of dysarthria among SCA3 patients. Patients with dysarthria had significantly higher SARA scores (p &lt; .001) and longer disease durations (p &lt; .001). Disease duration showed the strongest association with the occurrence of dysarthria (r = .319, p &lt; .001) and emerged as an independent prognostic factor (p &lt; .001). By the eighth year, 50% of patients exhibited dysarthria, with the highest incidence occurring in the later stages of the first decade. Conclusions: Dysarthria is common in SCA3 patients, with disease duration as the main prognostic factor. The prevalence of dysarthria increases as the disease progresses, particularly during the later stages of the first decade. These findings underscore the need for early intervention to address dysarthria in SCA3 patients, especially as they approach critical disease milestones. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
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  Data: &lt;i&gt;Copyright of Journal of Speech, Language &amp; Hearing Research is the property of American Speech-Language-Hearing Association and its content may not be copied or emailed to multiple sites without the copyright holder&#39;s express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.&lt;/i&gt; (Copyright applies to all Abstracts.)
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RecordInfo BibRecord:
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    Identifiers:
      – Type: doi
        Value: 10.1044/2025_JSLHR-24-00845
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      – Code: eng
        Text: English
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      – SubjectFull: Dysarthria
        Type: general
      – SubjectFull: Data analysis
        Type: general
      – SubjectFull: Retrospective studies
        Type: general
      – SubjectFull: Spinocerebellar ataxia
        Type: general
      – SubjectFull: Genomics
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      – SubjectFull: T-test (Statistics)
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      – SubjectFull: Research funding
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      – SubjectFull: Polymerase chain reaction
        Type: general
      – SubjectFull: Interviewing
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      – SubjectFull: Multiple regression analysis
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      – SubjectFull: DNA
        Type: general
      – SubjectFull: Chi-squared test
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      – SubjectFull: Mann Whitney U Test
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      – SubjectFull: Disease progression
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      – SubjectFull: Disease complications
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      – TitleFull: Dysarthria in Spinocerebellar Ataxia Type 3: Prevalence and Disease Progression.
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              M: 09
              Text: Sep2025
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