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

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Anqin Wang ◽  
Hongli Wu ◽  
Chunsheng Xu ◽  
Lanfeng Tang ◽  
Jaeyoun Lee ◽  
...  

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.

2013 ◽  
Vol 28 (3) ◽  
pp. 455-462 ◽  
Author(s):  
Rakesh Jadav ◽  
Jitender Saini ◽  
Sanjib Sinha ◽  
Bhavanishankara Bagepally ◽  
S. Rao ◽  
...  

2019 ◽  
Vol 39 (3) ◽  
Author(s):  
Ting Dong ◽  
Wen-ming Yang ◽  
Ming-cai Wu ◽  
Juan Zhang ◽  
Peng Huang ◽  
...  

Abstract Purpose: Wilson’s disease (WD) is a genetic disorder of copper metabolism with pathological copper accumulation in the brain. The purpose of the present study was to evaluate the relationship between the damaged white matter and the impaired cognitive function in WD patients. Materials and methods: Thirty WD adolescents and thirty age- and sex-matched healthy controls (HC) were enrolled. All subjects had received brain MRI, including conventional and diffusion-tensor imaging (DTI) scans. The DTI parameter of fractional anisotropy (FA) was calculated by diffusion kurtosis estimator software. The t test was used to compare the differences between two groups. The correlation between cognitive function and whiter matter disorders were analyzed by linear regression. The results of FA parameter and MD parameter intergroup analysis were both corrected with False Discovery Rate (FDR) simulations by SPSS. Results: WD adolescents showed significantly lower scores of time-based prospective memory (TBPM) and verbal fluency test (VFT) compared with HC. We found significantly higher FA in the right thalamus, right lentiform nucleus, left thalamus, left lentiform nucleus, and brain stem in WD adolescents. Besides, WD adolescents exhibited significantly lower FA in right cerebellum and cingulum and left middle frontal lobe compared with controls (P<0.05). There were significantly negative correlations between FA in bilateral lentiform and thalamus and cognitive impairment in WD adolescents (P<0.05). Conclusion: The whiter matter of WD adolescents was impaired and mainly distributed in subcortical brain regions. The impaired cognitive function was affected by the damaged whiter matter. The present study may be helpful for recognition and understanding of WD.


2014 ◽  
Vol 69 (3) ◽  
pp. 294-298 ◽  
Author(s):  
G. Li ◽  
X. Zhou ◽  
P. Xu ◽  
X. Pan ◽  
Y. Chen

2021 ◽  
Author(s):  
Francisco Costa Beber Lemanski ◽  
Vitor Dalepiane Rossato ◽  
Nathalia Beck Corrêa ◽  
Letícia Reginato ◽  
Gabriel Tarasconi Zanin

Background: Wilson’s disease (DW) is a genetic disorder characterized by the accumulation of copper in the body. The copper accumulation is systemic and occurs in several tissues, with the central nervous system (CNS) being one of the most affected sites. The use of imaging tests is not necessary for the diagnosis. However, in the suspicion of neurological damage, Magnetic Resonance Imaging (MRI) plays an important role in the assessment of the metal deposit in the CNS and in the clinico-anatomical correlation in symptomatic patients. Objectives: to identify the characteristic findings of DW in neuroimaging exams. Methods: a narrative literature review. Results: in MRI, the most affected sites in the CNS are the basal ganglia (mainly the outermost portion of the putamen), followed by the midbrain, the pons, and the thalamus. Alterations are bilaterally and symmetrically. T2 sequence reveals hyperintensity in putamen, the most common abnormality, as well as in the rest of the basal ganglia. Eventually, it is possible to identify the “panda sign” in the axial section of the midbrain, due to the involvement of the tegmental region associated with the normal signal of the red nuclei and hypointensity of the superior colliculus, characteristic of DW. In the T1 sequence, patients with neurological symptoms present hypointense images. Conclusions: MRI has a diagnostic and prognostic role in DW. The putamen is the most affected structure, but abnormalities in the pons, midbrain, and thalamus are part of the neuroimaging spectrum of Wilson’s disease. The “panda sign” is the classic MRI finding.


2022 ◽  
Author(s):  
Xiaoying Wang ◽  
Wenhui Guo ◽  
Yingying Zhang ◽  
Dan Liu ◽  
Qing Gao ◽  
...  

Abstract Background: Posture/balance disorder and pain are both present in Parkinson's patients, but their neural basis remain unclear. To investigate the central mechanism of posture/balance disorder and PD-related pain in Parkinson's disease by using diffusion tensor imaging (DTI) and tract-based spatial statistics (TBSS), combined with Transcranial Doppler (TCD). Results: It was found that the dose of levodopa, UPDRSⅡ and UPDRSⅢ were higher value in the group with higher score of posture/balance. In the more severe posture/balance disorder group, the fiber bundles of the prefrontal cortex, anterior cingulate cortex and basal ganglia were more likely to be affected. In addition, the DTI parameter values of the three brain regions had a significant correlation with the parameter values of the corresponding arteries. In the analysis of PD-related pain, the white matter fiber bundles from the midbrain to the basal ganglia increased in patients with PD-related pain. There were no statistic difference in prevalence of PD-related pain was found between different groups according to posture/balance. Conclusions: Posture and balance in PD were correlated with the severity of the disease and the dosage of compound levodopa. Posture and balance in PD were related to changes in the white matter integrity of the prefrontal cortex, anterior cingulate cortex and basal ganglia. The function of cerebral arteries had contributions to white matter integrity of these area and posture/balance. PD-related pain was positively correlated with sleep score. Patients with PD-related pain had an increase in the fiber projection from the midbrain to the basal ganglia. No relation was found between posture/balance disorder with PD-related pain.


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