Whole brain volume and cortical thickness abnormalities in Wilson’s disease: a clinical correlation study

Wilson’s disease (WD) is an inherited autosomal recessive disorder of copper metabolism, and its neurological and neuropsychiatric manifestations are associated with copper accumulation in brain. A few neuroimaging studies have shown that gray matter atrophy in WD affects both subcortical structures...

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Veröffentlicht in:Brain imaging and behavior 2021-08, Vol.15 (4), p.1778-1787
Hauptverfasser: Song, Yukun, Zou, Lin, Zhao, Jing, Zhou, Xiangxue, Huang, Yingqian, Qiu, Haishan, Han, Haiwei, Yang, Zhiyun, Li, Xunhua, Tang, Xiaoying, Chu, Jianping
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container_title Brain imaging and behavior
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creator Song, Yukun
Zou, Lin
Zhao, Jing
Zhou, Xiangxue
Huang, Yingqian
Qiu, Haishan
Han, Haiwei
Yang, Zhiyun
Li, Xunhua
Tang, Xiaoying
Chu, Jianping
description Wilson’s disease (WD) is an inherited autosomal recessive disorder of copper metabolism, and its neurological and neuropsychiatric manifestations are associated with copper accumulation in brain. A few neuroimaging studies have shown that gray matter atrophy in WD affects both subcortical structures and cortex. This study aims to quantitatively evaluate the morphometric brain abnormalities in patients with WD in terms of whole brain volume and cortical thickness and their associations with clinical severity of WD. Thirty patients clinically diagnosed as WD with neurological manifestations and 25 healthy controls (HC) were recruited. 3D T1-weighted images were segmented into 276 whole-brain regions of interest (ROIs) and 68 cortical ROIs. WD-vs-HC group comparisons were then conducted for each ROI. The associations between those morphometric measurements and the Global Assessment Scale (GAS) score for WD were analyzed. Compared with HC, significant WD-related volumetric decreases were found in the bilateral subcortical nuclei (putamen, globus pallidus, caudate nucleus, substantia nigra, red nucleus and thalamus), diffuse white matter and several gray matter regions. WD patients showed reduced cortical thickness in the left precentral gyrus and the left insula. Further, the volumes of the right globus pallidus, bilateral putamen, right external capsule and left superior longitudinal fasciculus were negatively correlated with GAS. Our results indicated that significant WD-related morphometric abnormalities were quantified in terms of whole-brain volumes and cortical thicknesses, some of which correlated significantly to the clinical severity of WD. Those morphometrics may provide a potentially effective biomarker of WD.
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WD patients showed reduced cortical thickness in the left precentral gyrus and the left insula. Further, the volumes of the right globus pallidus, bilateral putamen, right external capsule and left superior longitudinal fasciculus were negatively correlated with GAS. Our results indicated that significant WD-related morphometric abnormalities were quantified in terms of whole-brain volumes and cortical thicknesses, some of which correlated significantly to the clinical severity of WD. Those morphometrics may provide a potentially effective biomarker of WD.</abstract><cop>New York</cop><pub>Springer US</pub><doi>10.1007/s11682-020-00373-9</doi><tpages>10</tpages><orcidid>https://orcid.org/0000-0001-5483-2169</orcidid></addata></record>
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subjects Abnormalities
Age
Alzheimer's disease
Atrophy
Biomarkers
Biomedical and Life Sciences
Biomedicine
Brain
Brain research
Caudate nucleus
Copper
Globus pallidus
Hereditary diseases
Hospitals
Medical imaging
Mental disorders
Metabolism
Morphometry
Neuroimaging
Neuropsychology
Neuroradiology
Neurosciences
Original Research
Parameter estimation
Patients
Precentral gyrus
Psychiatry
Putamen
Red nucleus
Registration
Substantia alba
Substantia grisea
Substantia nigra
Thalamus
Thickness
Volumetric analysis
Wilson's disease
title Whole brain volume and cortical thickness abnormalities in Wilson’s disease: a clinical correlation study
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