Abstract

ObjectiveTo investigate the cause of the motor asymmetry in Wilson's disease (WD) patients using functional MRI.MethodsFifty patients with WD and 20 age‐matched healthy controls were enrolled. Neurological symptoms were scored using the modified Young Scale. All study subjects underwent diffusion tensor imaging (DTI), susceptibility‐weighted imaging (SWI), and resting‐state functional MRI (rs‐fMRI) of the brain. Six regions of interest (ROI) were chosen. Fiber volumes between ROIs on DTI, corrected phase (CP) values on SWI, amplitude of low‐frequency fluctuation (ALFF), and regional homogeneity (REHO) values on rs‐fMRI were determined. Asymmetry index (right or left value/left or right value) was evaluated.ResultsAsymmetry of rigidity, tremor, choreic movement, and gait abnormality (asymmetry index = 1.33, 1.39, 1.36, 1.40), fiber tracts between the GP and substantia nigra (SN), GP and PU, SN and thalamus (TH), SN and cerebellum, head of the caudate nucleus (CA) and SN, PU and CA, CA and TH, TH and cerebellum (asymmetry index = 1.233, 1.260, 1.269, 1.437, 1.503, 1.138, 1.145, 1.279), CP values in the TH, SN (asymmetry index = 1.327, 1.166), ALFF values, and REHO values of the TH (asymmetry index = 1.192, 1.233) were found. Positive correlation between asymmetry index of rigidity and fiber volumes between the GP and SN, SN and TH (r = .221, .133, p = .043, .036), and tremor and fiber volumes between the CA and TH (r = .045, p = .040) was found.ConclusionsThe neurological symptoms of patients with WD were asymmetry. The asymmetry of fiber projections may be the main cause of motor asymmetry in patients with WD.

Highlights

  • | MATERIALS AND METHODSWilson’s disease (WD) is an autosomal recessive inherited disorder of copper metabolism, characterized by excessive copper deposition, especially in the liver and brain

  • We hypothesized that asymmetry in performance in patients with Wilson’s disease (WD) may be a manifestation of asymmetrical metal deposition and damage to both subcortical nuclei and fiber projections between nuclei

  • We studied the relationship of asymmetry of neurological symptoms and metal deposition and damage to both subcortical nuclei and fiber projections

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Summary

| MATERIALS AND METHODS

Wilson’s disease (WD) is an autosomal recessive inherited disorder of copper metabolism, characterized by excessive copper deposition, especially in the liver and brain. There is asymmetry in performance of gait, tremor, and rigidity in patients with WD (Valtteri, 2016) Both the manifestation and extent of motor symptoms on the two sides of patients with WD are quite different. Resting-­state functional MRI (rs-f­ MRI) is a method that has been used to detect functional activity and functional conjunction of the nuclei in the brain In this series, we tried to study the characteristic and mechanism of motor asymmetry in patients with WD. We estimated brain metal deposition using SWI to evaluate fiber tracts’ abnormalities using DTI, to evaluate functional activity of the subcortical nuclei using rs-­fMRI, and to investigate the correlations between motor asymmetry and asymmetry of imaging metrics in patients with WD. We found no correlation between symptom asymmetry and asymmetry of CP values, and asymmetry of rs-­fMRI parameters, respectively

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