[Difference in imaging and metal metabolism between hepatic and cerebral type Wilson disease]

Objective: To evaluate the difference of metal metabolism, damage to structure and functional activity in brains between hepatic and cerebral type Wilson disease (WD). Methods: Forty patients with WD, including 20 with cerebral type and 20 with hepatic type, and 20 age-matched healthy controls in the First Affiliated Hospital, Sun Yat-sen University between Jul 2013 and May 2016 were enrolled.All study subjects underwent diffusion tensor imaging (DTI), resting state functional MRI (rs-fMRI) and susceptibility weighted imaging (SWI) of the brain.Six regions of interest (ROIs) were chosen.The values of fractional anisotropy (FA), λ in ROIs were determined on DTI, FA and fiber volumes between ROIs were also determined on DTI.The values of amplitude of low frequency fluctuation (ALFF) and regional homogeneity (REHO) in ROIs were determined on rs-fMRI.The values of corrected phase (CP) were calculated on SWI.The copper and iron content were measured.The difference of imaging and metal metrics between cerebral type and hepatic type WD were evaluated. Results: DTI metrics differed between patients with the cerebral and hepatic types of WD.ALFF values in the caudate nucleus, and thalamus were lower (P=0.037, 0.040), and REHO values in the caudate nucleus were lower (P=0.029), in patients of cerebral type than in hepatic type patients.CP values of the right caudate nucleus and left putamen in cerebral type WD patients were lower than in hepatic type patients (P=0.020, 0.23). The serum iron content of hepatic type WD patients was higher than the normal (P=0.013), and the urine copper content was higher than the cerebral type patients (P=0.021). Conclusions: Metal deposition and damage to the structure and functional activity in the brain may occur in hepatic type WD patients.The structural and functional activity damage of the brain in hepatic type is less severe than that in cerebral type patients, while the metal deposition is not significant different between hepatic and cerebral type.