The changes of extrapyramidal network studied by Diffusion tensor imaging in WD patients after 5 years of copper excluding treatment

Objective: To evaluate the changes of extrapyramidal network in Wilson disease (WD) patients after 5 years of copper excluding treatment using diffusion tensor imaging (DTI). Methods: 50 patients with WD, including 41 with cerebral type and 9 with hepatic type, and 10 age-matched healthy controls were enrolled. Neurological symptoms were scored using the modified Young Scale. Patients with cerebral type WD were divided into four subgroups: those with (1) hypokinesia, (2) parkinsonism, (3) mouth and throat dystonia, and (4) psychiatric symptoms. All study subjects underwent DTI of the brain. Five regions of interest (ROIs) were chosen. Fractional anisotropy (FA) and fiber volumes between ROIs were determined. After 5 years of copper excluding treatment, DTI review was performed.Results: After 5 years of copper excluding treatment, the volumes of fibers between the globus pallidus (GP) and the putamen (PU), the GP and the caudate nucleus (CA), the substantia nigra (SN)and the PU, the SN and the thalamus (TH), the PU and the TH, the TH and the CA, the TH and the cerebellum were also significantly higher in WD patients (P=0.013,0.040,0.037, 0.021,0.042, 0.038, 0.019). The volumes of fibers between the SN and the PU were higher in WD patients of group 3 after therapy (P=0.027). FA values of the fibers between the GP and the PU, the GP and the CA were significantly higher in WD patients of group 4 after therapy (P=0.026, 0.047). FA values of the fibers between the GP and the CA in WD patients with hepatic type were higher in WD patients after therapy (P=0.019).Conclusions: After the treatment with metal chelating agent, the damage of extrapyramidal neural network connection fibers in WD patients were repaired to a certain extent. Different types of WD patients have different responses to copper excluding treatment.