Structural and neurochemical evaluation of the brain and pons in patients with Wilson’s disease

Algın, Oktay; Taşkapılıoğlu, Özlem; Hakyemez, Bahattin; Ocakoğlu, Gökhan; Yurtoğulları, Şükran; Erer, Sevda; Parlak, Müfıt

doi:10.1007/s11604-010-0491-4

Cited by 13 publications

(8 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…No significant correlations were found between GM atrophy of BG and PM, which is inconsistent with a previous study (Dong et al, 2016). However, many previous studies have revealed the widespread alternations in BG which mainly lead to extrapyramidal symptoms such as tremors, ataxia, and dystonic syndrome (Ala et al, 2007;Algin et al, 2010). The possible interpretation is that PM impairment is easily masked by other symptoms (Lorincz, 2010) that did not manifest in structural damage in BG in the present study.…”

Section: Discussionmentioning

confidence: 44%

Structural and Functional Changes Are Related to Cognitive Status in Wilson’s Disease

et al. 2021

Front. Hum. Neurosci.

View full text Add to dashboard Cite

Patients with Wilson’s disease (WD) suffer from prospective memory (PM) impairment, and some of patients develop cognitive impairment. However, very little is known about how brain structure and function changes effect PM in WD. Here, we employed multimodal neuroimaging data acquired from 22 WD patients and 26 healthy controls (HC) who underwent three-dimensional T1-weighted, diffusion tensor imaging (DTI), and resting state functional magnetic resonance imaging (RS-fMRI). We investigated gray matter (GM) volumes with voxel-based morphometry, DTI metrics using the fiber tractography method, and RS-fMRI using the seed-based functional connectivity method. Compared with HC, WD patients showed GM volume reductions in the basal ganglia (BG) and occipital fusiform gyrus, as well as volume increase in the visual association cortex. Moreover, whiter matter (WM) tracks of WD were widely impaired in association and limbic fibers. WM tracks in association fibers are significant related to PM in WD patients. Relative to HC, WD patients showed that the visual association cortex functionally connects to the thalamus and hippocampus, which is associated with global cognitive function in patients with WD. Together, these findings suggested that PM impairment in WD may be modulated by aberrant WM in association fibers, and that GM volume changes in the association cortex has no direct effect on cognitive status, but indirectly affect global cognitive function by its aberrant functional connectivity (FC) in patients with WD. Our findings may provide a new window to further study how WD develops into cognitive impairment, and deepen our understanding of the cognitive status and neuropathology of WD.

show abstract

Section: Discussionmentioning

confidence: 44%

Structural and Functional Changes Are Related to Cognitive Status in Wilson’s Disease

et al. 2021

Front. Hum. Neurosci.

View full text Add to dashboard Cite

show abstract

“…Areas of symmetric hypointensity on T1-weighted images and symmetric hyperintensity on T2-weighted images were presented in the basal ganglia, thalamus, and brain stem [12]. Diffusion tenser imaging (DTI) is a noninvasive technique to measure microstructural organization in brain tissues in vivo, based on the properties of water diffusion [13].…”

Section: Introductionmentioning

confidence: 99%

Study on Lesion Assessment of Cerebello-Thalamo-Cortical Network in Wilson’s Disease with Diffusion Tensor Imaging

Wang

Wu²,

et al. 2017

Neural Plasticity

View full text Add to dashboard Cite

Wilson's disease (WD) is a genetic disorder of copper metabolism with pathological copper accumulation in the brain and any other tissues. This article aimed to assess lesions in cerebello-thalamo-cortical network with an advanced technique of diffusion tensor imaging (DTI) in WD. 35 WD patients and 30 age- and sex-matched healthy volunteers were recruited to accept diffusion-weighted images with 15 gradient vectors and conventional magnetic resonance imaging (MRI). The DTI parameters, including fractional anisotropy (FA) and mean diffusion (MD), were calculated by diffusion kurtosis estimator software. After registration, patient groups with FA mappings and MD mappings and normal groups were compared with 3dttest and receiver-operating characteristic (ROC) curve analysis, corrected with FDR simulations (p = 0.001, α = 0.05, cluster size = 326). We found that the degree of FA increased in the bilateral head of the caudate nucleus (HCN), lenticular nucleus (LN), ventral thalamus, substantia nigra (SN), red nucleus (RN), right dentate nucleus (DN), and decreased in the mediodorsal thalamus and extensive white matter. The value of MD increased in HCN, LN, SN, RN, and extensive white matter. The technique of DTI provides higher sensitivity and specificity than conventional MRI to detect Wilson's disease. Besides, lesions in the basal ganglia, thalamus, and cerebellum might disconnect the basal ganglia-thalamo-cortical circuits or dentato-rubro-thalamic (DRT) track and disrupt cerebello-thalamo-cortical network finally, which may cause clinical extrapyramidal symptoms.

show abstract

“…Various organs, including the liver, kidneys, and brain, are impaired in untreated WD . Copper accumulation in the brain can induce changes such as myelin degeneration, gliosis, and profound neuronal loss . Among all brain structures (including the cerebellum), the gray matter in the basal ganglia is affected the most, followed by the brainstem (red nucleus and substantia nigra [SN]) and the cortex (especially the frontal lobes and the parieto‐occipital zones) .…”

mentioning

confidence: 99%

“…9 Copper accumulation in the brain can induce changes such as myelin degeneration, gliosis, and profound neuronal loss. 10,11 Among all brain structures (including the cerebellum), the gray matter in the basal ganglia [11][12][13][14][15] is affected the most, followed by the brainstem (red nucleus and substantia nigra [SN]) 16,17 and the cortex (especially the frontal lobes and the parietooccipital zones). 14,18 Additionally, emotion research has revealed that the psychiatric impairments of WD, including emotional lability, personality disorders, and mania, are caused by abnormalities in the limbic system 19 and basal ganglia.…”

mentioning

confidence: 99%

Regional morphometric abnormalities and clinical relevance in Wilson's disease

et al. 2019

View full text Add to dashboard Cite

Background Morphology builds Wilson's disease's clincal basis. Objectives To detect and quantify regional morphometric abnormalities, in terms of both volume and shape, in patients with Wilson's disease. Methods Twenty‐seven Wilson's disease patients and 24 healthy controls were enrolled. Specific brain structures, including the bilateral caudate, putamen, globus pallidus, thalamus, amygdala, hippocampus, red nucleus, and substantia nigra (SN), were automatically extracted from each participant's T1‐weighted image. Volume abnormalities and correlations with the modified Young scale were investigated. Furthermore, vertex‐based shape analysis was performed to explore region‐specific morphometric abnormalities. Results Significant global volume atrophy and local shape abnormalities were detected and quantified in the bilateral caudate, putamen, globus pallidus, thalamus, amygdala, red nucleus, and SN. Morphometric abnormalities of the caudate, putamen, and thalamus were strong, whereas those of the globus pallidus, amygdala, red nucleus, and SN were weaker. No hippocampal abnormalities were observed. The modified Young scale was found to correlate significantly with the volumes of the bilateral putamen and the right globus pallidus. Shape analysis revealed subregion‐specific atrophy of the bilateral caudate and putamen. They were concentrated in the subregions that project to the limbic and executive cortices. Significant region‐specific atrophy was also detected in the bilateral thalamic subregions that project to the primary motor, sensory, and premotor cortices. Conclusions We found significant morphometric abnormalities of specific structures of interest in patients with Wilson's disease, both globally and locally. These morphometric abnormalities may serve as useful imaging biomarkers for Wilson's disease. © 2019 International Parkinson and Movement Disorder Society

show abstract

Structural and neurochemical evaluation of the brain and pons in patients with Wilson’s disease

Abstract: MRS is a noninvasive, valuable modality for detecting structural-neurochemical changes of the brain stem and deep gray matter in patients with WD. The contribution of DWI in these patients is limited.

Cited by 13 publications

References 29 publications

Structural and Functional Changes Are Related to Cognitive Status in Wilson’s Disease

Structural and Functional Changes Are Related to Cognitive Status in Wilson’s Disease

Study on Lesion Assessment of Cerebello-Thalamo-Cortical Network in Wilson’s Disease with Diffusion Tensor Imaging

Regional morphometric abnormalities and clinical relevance in Wilson's disease

Contact Info

Product

Resources

About