Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3
Background: Spinocerebellar ataxia type 3 (SCA) is a cerebellum-dominant degenerative disorder that is characterized primarily by infratentorial damage, although less severe supratentorial involvement may contribute to the clinical manifestation. These impairments may result from the efferent loss o...
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doaj-7387b5c1ef8245a8933b7213abdb00dd2020-11-25T01:38:27ZengFrontiers Media S.A.Frontiers in Neurology1664-22952020-03-011110.3389/fneur.2020.00124507183Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3Po-Shan Wang0Po-Shan Wang1Po-Shan Wang2Yu-Te Wu3Yu-Te Wu4Yu-Te Wu5Tzu-Yun Wang6Hsiu-Mei Wu7Bing-Wen Soong8Bing-Wen Soong9Bing-Wen Soong10Bing-Wen Soong11Chi-Wen Jao12Chi-Wen Jao13Chi-Wen Jao14Brain Research Center, National Yang-Ming University, Taipei, TaiwanInstitute of Biophotonics, National Yang-Ming University, Taipei, TaiwanDepartment of Neurology, Taipei Municipal Gan-Dau Hospital, Taipei, TaiwanBrain Research Center, National Yang-Ming University, Taipei, TaiwanInstitute of Biophotonics, National Yang-Ming University, Taipei, TaiwanDepartment of Biomedical Imaging and Radiological Sciences, National Yang-Ming University, Taipei, TaiwanDepartment of Biomedical Imaging and Radiological Sciences, National Yang-Ming University, Taipei, TaiwanDepartment of Radiology, Taipei Veterans General Hospital, Taipei, TaiwanBrain Research Center, National Yang-Ming University, Taipei, TaiwanTaipei Neuroscience Institute, Taipei Medical University, Taipei, TaiwanDepartment of Neurology, Taipei Medical University-Shuang Ho Hospital, New Taipei City, TaiwanDepartment of Neurology, Taipei Veterans General Hospital, Taipei, TaiwanBrain Research Center, National Yang-Ming University, Taipei, TaiwanInstitute of Biophotonics, National Yang-Ming University, Taipei, TaiwanDepartment of Neurology, Shin-Kong Wu Ho Su Memorial Hospital, Taipei, TaiwanBackground: Spinocerebellar ataxia type 3 (SCA) is a cerebellum-dominant degenerative disorder that is characterized primarily by infratentorial damage, although less severe supratentorial involvement may contribute to the clinical manifestation. These impairments may result from the efferent loss of the cerebellar cortex and degeneration of the cerebral cortex.Method: We used the three-dimensional fractal dimension (3D-FD) method to quantify the morphological changes in the supratentorial regions and assessed atrophy in the relatively focal regions in patients with SCA3. A total of 48 patients with SCA3 and 50 sex- and age-matched healthy individuals, as the control group, participated in this study. The 3D-FD method was proposed to distinguish 97 automatic anatomical label regions of gray matter (left cerebrum: 45, right cerebrum: 45, cerebellum: 7) between healthy individuals and patients with SCA3.Results: Patients with SCA3 exhibited reduced brain complexity within both the traditional olivopontocerebellar atrophy (OPCA) pattern and specific supratentorial regions. The study results confirmed the extensive involvement of extracerebellar regions in SCA3. The atrophied regions of SCA3 in infratentorial and supratentorial cortex showed a wide range of overlapped areas as in two functional cortexes, namely cerebellum-related cortex and basal ganglia-related cortex.Conclusions: Our results found that the atrophy of the SCA3 are not only limited in the infratentorial regions. Both cerebellar related cortex and basal ganglia related cortex were affected in the disease process of SCA3. Our findings might correlate to the common symptoms of SCA3, such as ataxia, Parkinsonism, dysarthria, and dysmetria. SCA3 should no longer be considered a disease limited to the cerebellum and its connections; rather, it should be considered a pathology affecting the whole brain.https://www.frontiersin.org/article/10.3389/fneur.2020.00124/fullspinocerebellar ataxia type 3 (SCA3)supratentorial involvementfractal dimensionmorphological changesMRI |
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DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Po-Shan Wang Po-Shan Wang Po-Shan Wang Yu-Te Wu Yu-Te Wu Yu-Te Wu Tzu-Yun Wang Hsiu-Mei Wu Bing-Wen Soong Bing-Wen Soong Bing-Wen Soong Bing-Wen Soong Chi-Wen Jao Chi-Wen Jao Chi-Wen Jao |
spellingShingle |
Po-Shan Wang Po-Shan Wang Po-Shan Wang Yu-Te Wu Yu-Te Wu Yu-Te Wu Tzu-Yun Wang Hsiu-Mei Wu Bing-Wen Soong Bing-Wen Soong Bing-Wen Soong Bing-Wen Soong Chi-Wen Jao Chi-Wen Jao Chi-Wen Jao Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3 Frontiers in Neurology spinocerebellar ataxia type 3 (SCA3) supratentorial involvement fractal dimension morphological changes MRI |
author_facet |
Po-Shan Wang Po-Shan Wang Po-Shan Wang Yu-Te Wu Yu-Te Wu Yu-Te Wu Tzu-Yun Wang Hsiu-Mei Wu Bing-Wen Soong Bing-Wen Soong Bing-Wen Soong Bing-Wen Soong Chi-Wen Jao Chi-Wen Jao Chi-Wen Jao |
author_sort |
Po-Shan Wang |
title |
Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3 |
title_short |
Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3 |
title_full |
Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3 |
title_fullStr |
Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3 |
title_full_unstemmed |
Supratentorial and Infratentorial Lesions in Spinocerebellar Ataxia Type 3 |
title_sort |
supratentorial and infratentorial lesions in spinocerebellar ataxia type 3 |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Neurology |
issn |
1664-2295 |
publishDate |
2020-03-01 |
description |
Background: Spinocerebellar ataxia type 3 (SCA) is a cerebellum-dominant degenerative disorder that is characterized primarily by infratentorial damage, although less severe supratentorial involvement may contribute to the clinical manifestation. These impairments may result from the efferent loss of the cerebellar cortex and degeneration of the cerebral cortex.Method: We used the three-dimensional fractal dimension (3D-FD) method to quantify the morphological changes in the supratentorial regions and assessed atrophy in the relatively focal regions in patients with SCA3. A total of 48 patients with SCA3 and 50 sex- and age-matched healthy individuals, as the control group, participated in this study. The 3D-FD method was proposed to distinguish 97 automatic anatomical label regions of gray matter (left cerebrum: 45, right cerebrum: 45, cerebellum: 7) between healthy individuals and patients with SCA3.Results: Patients with SCA3 exhibited reduced brain complexity within both the traditional olivopontocerebellar atrophy (OPCA) pattern and specific supratentorial regions. The study results confirmed the extensive involvement of extracerebellar regions in SCA3. The atrophied regions of SCA3 in infratentorial and supratentorial cortex showed a wide range of overlapped areas as in two functional cortexes, namely cerebellum-related cortex and basal ganglia-related cortex.Conclusions: Our results found that the atrophy of the SCA3 are not only limited in the infratentorial regions. Both cerebellar related cortex and basal ganglia related cortex were affected in the disease process of SCA3. Our findings might correlate to the common symptoms of SCA3, such as ataxia, Parkinsonism, dysarthria, and dysmetria. SCA3 should no longer be considered a disease limited to the cerebellum and its connections; rather, it should be considered a pathology affecting the whole brain. |
topic |
spinocerebellar ataxia type 3 (SCA3) supratentorial involvement fractal dimension morphological changes MRI |
url |
https://www.frontiersin.org/article/10.3389/fneur.2020.00124/full |
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