Distribution of perivascular spaces distribution and relate to the clinical features of SCA3
Abstract Background Spinocerebellar ataxia type 3 (SCA3) is a rare neurodegenerative condition. Prior research has established perivascular spaces (PVS) expansion has been implicated in the pathogenesis and prognosis of various neurodegenerative diseases. To examine PVS changes in SCA3 by comparing...
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| Format: | Article |
| Language: | English |
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BMC
2025-08-01
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| Series: | Orphanet Journal of Rare Diseases |
| Online Access: | https://doi.org/10.1186/s13023-025-03954-3 |
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| author | Xinyuan Chen Yanhua Lian Wei Lin Xiaoyue Xia Lin Zhang Zhuoying Huang Maolin Cui Ruying Yuan Mengcheng Li Ziqiang Huang Naping Chen Yuqing Tu Jianping Hu Ning Wang Qunlin Chen Shirui Gan |
| author_facet | Xinyuan Chen Yanhua Lian Wei Lin Xiaoyue Xia Lin Zhang Zhuoying Huang Maolin Cui Ruying Yuan Mengcheng Li Ziqiang Huang Naping Chen Yuqing Tu Jianping Hu Ning Wang Qunlin Chen Shirui Gan |
| author_sort | Xinyuan Chen |
| collection | DOAJ |
| description | Abstract Background Spinocerebellar ataxia type 3 (SCA3) is a rare neurodegenerative condition. Prior research has established perivascular spaces (PVS) expansion has been implicated in the pathogenesis and prognosis of various neurodegenerative diseases. To examine PVS changes in SCA3 by comparing patients and matched healthy controls and to identify potential connection of PVS for clinical features. Methods We conducted MRI scans on 91 SCA3 patients and 64 healthy controls. We utilized visual semi-quantitative methods to assess PVS in various brain regions, including the center of the semiovale (CSO), basal ganglia (BG), and midbrain-pons, as well as combinations (BG + CSO, BG + CSO + midbrain-pons). To differentiate SCA3 patients from healthy controls, we compared the area under the curve (AUC) of the receiver operating characteristic curve between the two groups. Additionally, we employed Pearson’s correlation coefficient to examine the relationship between PVS scores in each brain region and clinical indicators among SCA3 patients. Results In the SCA3 group, we observed higher levels of PVS in the BG, CSO + BG, and three brain regions compared to the healthy control group. PVS in the CSO and CSO + BG also showed positive correlations with age and disease duration, and negative correlations with the number of CAG repeats. Furthermore, PVS in three brain regions exhibited negative correlation with the number of CAG repeats. Conclusions This study represents an initial investigation into the relationship between PVS and SCA3 disease. Our findings suggest that PVS might indicate the degree of cerebellar ataxia to a certain extent. |
| format | Article |
| id | doaj-art-bd0a050d680e47debf36601d4d0763c6 |
| institution | Kabale University |
| issn | 1750-1172 |
| language | English |
| publishDate | 2025-08-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj-art-bd0a050d680e47debf36601d4d0763c62025-08-20T04:02:41ZengBMCOrphanet Journal of Rare Diseases1750-11722025-08-012011910.1186/s13023-025-03954-3Distribution of perivascular spaces distribution and relate to the clinical features of SCA3Xinyuan Chen0Yanhua Lian1Wei Lin2Xiaoyue Xia3Lin Zhang4Zhuoying Huang5Maolin Cui6Ruying Yuan7Mengcheng Li8Ziqiang Huang9Naping Chen10Yuqing Tu11Jianping Hu12Ning Wang13Qunlin Chen14Shirui Gan15Department of Rehabilitation Medicine, The First Affiliated Hospital, Fujian Medical UniversityFuzhou Second General HospitalDepartment of Neurology, Institute of Neurology of First Affiliated Hospital, Institute of Neuroscience, Fujian Key Laboratory of Molecular Neurology, Fujian Medical UniversityDepartment of Radiology, The First Affiliated Hospital, Fujian Medical UniversityDepartment of Radiology, The First Affiliated Hospital, Fujian Medical UniversityDepartment of Neurology, Institute of Neurology of First Affiliated Hospital, Institute of Neuroscience, Fujian Key Laboratory of Molecular Neurology, Fujian Medical UniversityDepartment of Neurology, Institute of Neurology of First Affiliated Hospital, Institute of Neuroscience, Fujian Key Laboratory of Molecular Neurology, Fujian Medical UniversityDepartment of Neurology, Institute of Neurology of First Affiliated Hospital, Institute of Neuroscience, Fujian Key Laboratory of Molecular Neurology, Fujian Medical UniversityDepartment of Radiology, The First Affiliated Hospital, Fujian Medical UniversityDepartment of Radiology, The First Affiliated Hospital, Fujian Medical UniversityDepartment of Radiology, The First Affiliated Hospital, Fujian Medical UniversityDepartment of Radiology, The First Affiliated Hospital, Fujian Medical UniversityDepartment of Radiology, The First Affiliated Hospital, Fujian Medical UniversityDepartment of Neurology, Institute of Neurology of First Affiliated Hospital, Institute of Neuroscience, Fujian Key Laboratory of Molecular Neurology, Fujian Medical UniversityDepartment of Radiology, The First Affiliated Hospital, Fujian Medical UniversityDepartment of Neurology, Institute of Neurology of First Affiliated Hospital, Institute of Neuroscience, Fujian Key Laboratory of Molecular Neurology, Fujian Medical UniversityAbstract Background Spinocerebellar ataxia type 3 (SCA3) is a rare neurodegenerative condition. Prior research has established perivascular spaces (PVS) expansion has been implicated in the pathogenesis and prognosis of various neurodegenerative diseases. To examine PVS changes in SCA3 by comparing patients and matched healthy controls and to identify potential connection of PVS for clinical features. Methods We conducted MRI scans on 91 SCA3 patients and 64 healthy controls. We utilized visual semi-quantitative methods to assess PVS in various brain regions, including the center of the semiovale (CSO), basal ganglia (BG), and midbrain-pons, as well as combinations (BG + CSO, BG + CSO + midbrain-pons). To differentiate SCA3 patients from healthy controls, we compared the area under the curve (AUC) of the receiver operating characteristic curve between the two groups. Additionally, we employed Pearson’s correlation coefficient to examine the relationship between PVS scores in each brain region and clinical indicators among SCA3 patients. Results In the SCA3 group, we observed higher levels of PVS in the BG, CSO + BG, and three brain regions compared to the healthy control group. PVS in the CSO and CSO + BG also showed positive correlations with age and disease duration, and negative correlations with the number of CAG repeats. Furthermore, PVS in three brain regions exhibited negative correlation with the number of CAG repeats. Conclusions This study represents an initial investigation into the relationship between PVS and SCA3 disease. Our findings suggest that PVS might indicate the degree of cerebellar ataxia to a certain extent.https://doi.org/10.1186/s13023-025-03954-3 |
| spellingShingle | Xinyuan Chen Yanhua Lian Wei Lin Xiaoyue Xia Lin Zhang Zhuoying Huang Maolin Cui Ruying Yuan Mengcheng Li Ziqiang Huang Naping Chen Yuqing Tu Jianping Hu Ning Wang Qunlin Chen Shirui Gan Distribution of perivascular spaces distribution and relate to the clinical features of SCA3 Orphanet Journal of Rare Diseases |
| title | Distribution of perivascular spaces distribution and relate to the clinical features of SCA3 |
| title_full | Distribution of perivascular spaces distribution and relate to the clinical features of SCA3 |
| title_fullStr | Distribution of perivascular spaces distribution and relate to the clinical features of SCA3 |
| title_full_unstemmed | Distribution of perivascular spaces distribution and relate to the clinical features of SCA3 |
| title_short | Distribution of perivascular spaces distribution and relate to the clinical features of SCA3 |
| title_sort | distribution of perivascular spaces distribution and relate to the clinical features of sca3 |
| url | https://doi.org/10.1186/s13023-025-03954-3 |
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