ABSTRACT
Objective To investigate the value of neurite orientation dispersion and density imaging (NODDI) in quantitative evaluation of the microstructural changes in basal ganglia and thalamus in Wilson's disease (WD) patients,and to assess the diagnostic efficacy of NODDI.Methods Totally 27 WD patients (WD group) and 26 age-and sex-matched controls (control group) were enrolled.All subjects underwent MR scanning with NODDI.Parameters of NODDI,including intracellular volume fraction (Vic),orientation dispersion index (ODI) and isotropic volume fraction (Viso) of bilateral caudate nucleus,globus pallidus,putamen and thalamus were compared between the 2 groups.Correlation analysis was performed between each parameter of NODDI and clinical Young scores.Random Forest model was used to assess the relative importance of each parameter and to evaluate the diagnostic efficacy.Results The Vic and ODI of bilateral caudate nucleus,globus pallidus and putamen in WD patients were significantly lower than those in normal controls (all P＜0.05),while Viso was significantly higher than that in normal controls (all P＜0.05).The Vic of bilateral thalamus was lower,while Viso was higher in WD patients than those in normal controls (all P＜0.05),and ODI had no significant difference between the 2 groups (P=0.055).In WD patients,Vic and ODI of bilateral caudate nucleus,globus pallidus and putamen were negatively correlated with clinical Young scores.Viso of globus pallidus and putamen were positively correlated with clinical scores.The prediction accuracy of NODDI was 96.23％,and the area under ROC curve was 0.96.Conclusion NODDI can effectively evaluate changes in microstructures and metabolism during copper deposition in WD patients,and it may be useful in detecting changes of brain deep nuclei and assessing the progression of WD.
ABSTRACT
Objective To evaluate functional activity of the subcortical nuclei in Wilson's disease (WD) using resting state functional MRI (rs-fMRI),and to evaluate damage to the functional conjunction in the extracorticospinal tract in WD patients.Methods Twenty-two patients with WD (between January 2015 and January 2016),including 18 with cerebral type and 4 with hepatic type,and 20 age-matched healthy controls were enrolled.Neurological symptoms were scored using the modified Young Scale.Patients with cerebral type WD were divided into 4 subgroups.All study subjects underwent rs-fMRI of the brain.The values of amplitude of low-frequency fluctuation (ALFF) and regional homogeneity (REHO) in the thalamus,caudate nucleus,putamen and globus pallidus were determined.The relationships between rsfMRI metrics and clinical status were evaluated.Results ALFF values were lower in the caudate nucleus,putamen and right thalamus of WD patients than in controls (t =-3.07,-3.00,-3.12,-2.46,-2.20;P =0.005,0.006,0.004,0.020,0.036),while REHO values were lower in the left caudate nucleus and left thalamus of WD patients (t =-2.38,-2.16;P =0.025,0.040).In the caudate nucleus (P =0.032,0.029,0.023),thalamus (P =0.022,0.041,0.035) ALFF values were lower in group 4 than in other groups.REHO values of the putamen (P =0.040,0.017,0.040) and thalamus (P =0.024,0.029 7,0.041) were higher in group 4 than in other groups.ALFF values in the caudate nucleus (t =-0.29,P=0.037),and thalamus (t =-1.77,P =0.042) were lower,and REHO values in the caudate nncleus (t =-1.46,P =0.040) were lower,in patients of cerebral type than in hepatic type patients.Conclusions The damage to the functional activity of the subcortical nuclei may occur in the WD patients.The functional activity of nuclei may be different between hepatic and cerebral type patients.Damage to the activity of neurons in the putamen and thalamus may correlate with psychiatric symptoms in WD patients.