Clinical Study Of ~1H-MRS In The Evalution Of The Wilson's Disease

Objective The purpose of this study was to determine the brain metabolic changes due to Wilson's Disease by using MRS. To describe and correlate the MRI abnormalities of the brain with clinical features in WD. To evaluate the usefulness of MRS in the early diagnosis of brain injury in Wilson's Disease patients.Materials and methods 22 patients of WD and 20controls were examined at the same time with MRI and MRS(20 patients of WD) in the symmetrical putamen, thalami, frontal and parieto white matter. There are 4 patients without evidence of neurologic (hWD) and 15 patients with neurologic features (nWD). The amplitude of the peaks in the time domain were measured at the following resonances: N-acetylaspartate(NAA), creatine and phosphocreatine (Cr), choline-containing compounds (Cho). The mean FWHM was also measured in each spectrum studied. We compared NAA/Cr, Cho/Cr, NAA/Cho and FWHM obtained in the symmetrical putamen, thalami, FWM and PWM of the patients among WD and controls, hWD, nWD and controls. MRI grading and clinical manifestations scores for severity of disease were noted. The relationship of MRI grading and disease severity scores was analysed.Results MRI changes correlated with NSS (P<0.001) , MRI and NSS did not correlate with the duration of illness. The mean FWHM was significantly larger in the putamen and thalami of patients with WD than in control subjects (p<0.001) . The mean FWHM was larger in the putame of hWD than in control (p<0.05) . The mean FWHM was larger in the thalami and putame of nWD than control (p<0.05). MRS can find abnormal metabolites in all patients of WD ,in which decrease of NAA/Cr was most common (p<0.001) . The decrease of the radio of NAA/Cr and NAA/Cho in the putamen of patients with WD was more than in control (the former p<0.001, the later p<0.05) . The decrease of the radio of NAA/Cr in the thalami of patients with WD was more than in control (p<0.001) . The decrease of the radio of Cho/Cr and the increase of the radio of NAA/Cho in the frontal white matter of patients with WD were more than in control (p<0.05). The decrease of the radio of NAA/Cr in the putamen and thalami of nWD was more than in hWD and control (p<0.05) . The increase of the radio of Cho/Cr in the putamen of hWD was more than in nWD and control (p<0.05) .Conclusion1,The characteristic MRI presentation of WD are: symmetrical T₂WI hyperintensities in the putamen, caudate, thalamus, "face of giant panda" sign, different degree of brain atrophy, mostly seen in the cerebellum;2,MRI correlated with NSS, MRI and NSS did not correlate with the duration of illness; 3,The increase of the mean FWHM and abnormal metabolites in the ROI of WD groups indicated that copper deposition lead to heterogeneity of the magnetic field and abnormal metabolite changes. FWHM with MRS can be used to analyze the causes of abnormal microstructures and metabolite changes of WD, which are helpful in the early diagnosis of brain injury and evaluation of the effectiveness of therapy.