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Clinical Article
Effect of structural abnormality of gray matter on motor and non-motor function in spinocerebellar ataxia type 3
LI Mengfei  JIANG Zhenzhen  CHEN Hui  LIU Chen  WANG Jian 


[Abstract] Objective To explore the structural abnormality of gray matter and its effect on non-motor and motor function in patients with spinocerebellar ataxia type 3 (SCA3), so as to provide a theoretical basis for early diagnosis and proper treatment of SCA3.Materials and Methods One hundred and two subjects [49 patients and 53 age- and sex- matched healthy controls (HC) ] were enrolled in the study. The participants were assessed by the neuropsychological scale, including the Montreal Cognitive Assessment (MoCA), the Mini Mental State Examination (MMSE), the Rapid Verbal Retrieve (RVR), Digit Span Test (DST), the Activities of Daily Living (ADL), and the Hamilton Depression Scale (HAMD) . They also participated in the MRI evaluation. Voxel-based morphometry (VBM) was conducted to analyse the gray matter volume (GMV) alterations between the patients and the healthy controls. The partial correlation analysis was performed to identify the partial correlation between GMV and clinical scale scores with sex, age, and total intracranial volume (TIV) as covariables.Results Compared with the healthy control group, the scores of MoCA (Z=-4.578, P<0.001), MMSE (Z=-4.725, P<0.001) and RVR (Z=-5.773, P<0.001) in the SCA3 group decreased significantly, while the scores of ADL (Z=-6.447, P<0.001) and HAMD (Z=-5.285, P<0.001) increased significantly. The brain volume atrophied were discovered in the vermis Ⅸ lobe, bilateral cerebellum, caudate nucleus, cingulate gyrus, frontal lobe, hippocampus, precentral gyrus, putamen, supplementary motor area, and temporal lobe, left calcarine cortex, paracentral lobule, and para-hippocampal gyrus, right cuneus, fusiform gyrus and occipital lobe (P<0.001, FDR corrected). The increased ones were intralaminar nucleus, dorsal medial nucleus of left thalamus and ventral anterior nucleus of right thalamus (P<0.001, FDR corrected). The correlation analysis between differential brain area and the neuropsychological scale showed that Scale for the Assessment and Rating of Ataxia (SARA) and International Cooperative Ataxia Rating Scale (ICARS) scores were negatively correlated with the vermis Ⅸ lobule, bilateral cerebellum, precentral gyrus, and temporal lobe; left calcarine cortex, right caudate nucleus, cingulate gyrus, frontal lobe, fusiform gyrus and occipital lobe, while positively correlated with the intralaminar nucleus of left thalamus. MoCA score was positively correlated with vermis Ⅸ lobule and bilateral cerebellum. ADL score was negatively correlated with vermis Ⅸ lobule, bilateral cerebellum, left calcarine cortex, temporal lobe and right fusiform gyrus; HAMD score was negatively correlated with bilateral cerebellum, right frontal lobe and left temporal lobe; RVR score was positively correlated with bilateral cerebellum, right frontal lobe, left temporal lobe and Ⅸ lobule of vermis.Conclusions In addition to cerebellar structural damage, there are also extensive cognitive, memory and emotional brain damage in SCA3, which is closely related to motor and non-motor functional impairment, which provides theoretical guidance for the next selection of therapeutic targets for SCA3.
[Keywords] spinocerebellar ataxia type 3;magnetic resonance imaging;voxel-based morphometry;cerebellum

LI Mengfei   JIANG Zhenzhen   CHEN Hui   LIU Chen   WANG Jian*  

Department of Radiology, the First Affiliated Hospital of the Army Military Medical University, Chongqing 400038, China

Corresponding author: Wang J, E-mail:

Conflicts of interest   None.

Received  2022-10-17
Accepted  2023-05-06
DOI: 10.12015/issn.1674-8034.2023.05.012

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