A retrospective cohort study: vaccination status and safety analysis of SARS-CoV-2 vaccine in patients with Wilson's disease.

BACKGROUND: Wilson's disease (WD) is a rare hepatic and neurological disorder, which can dramatically worsen by traumatic injuries, surgeries, and infections. No studies have reported safety data of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination in WD patients. We aimed to investigate the SARS-CoV-2 vaccination status and post-vaccination adverse events in WD patients. METHODS: This is a multicenter, retrospective, observational study. We investigated the vaccination rates, the type of vaccine, subjective reasons for non-vaccination, and the adverse events following vaccination. Logistic regression analysis was used to assess the correlation between vaccination status and increased Unified Wilson's Disease Rating Scale (UWDRS) scores. RESULTS: A total of 554 WD patients with a mean (SD) age of 25.3 (10.85) years were included in this study, of whom 336 (60.6%) were males and 218 (39.4%) were females. 368 (66.4%) patients received at least one dose of the SARS-CoV-2 vaccine.186 (33.6%) patients were unvaccinated. Logistic regression analysis showed that vaccination against SARS-CoV-2 was not significantly associated with increased UWDRS scores. The safety analysis demonstrated that 21.2% had post-vaccination adverse events. CONCLUSIONS: In this study, vaccination against SARS-CoV-2 was safe in WD patients, providing evidence for the safety of vaccination in WD patients.

Disrupted topological organization of the motor execution network in Wilson's disease.

Objective: There are a number of symptoms associated with Wilson's disease (WD), including motor function damage. The neuropathological mechanisms underlying motor impairments in WD are, however, little understood. In this study, we explored changes in the motor execution network topology in WD. Methods: We conducted resting-state functional magnetic resonance imaging (fMRI) on 38 right-handed individuals, including 23 WD patients and 15 healthy controls of the same age. Based on graph theory, a motor execution network was constructed and analyzed. In this study, global, nodal, and edge topological properties of motor execution networks were compared. Results: The global topological organization of the motor execution network in the two groups did not differ significantly across groups. In the cerebellum, WD patients had a higher nodal degree. At the edge level, a cerebello-thalamo-striato-cortical circuit with altered functional connectivity strength in WD patients was observed. Specifically, the strength of the functional connections between the cerebellum and thalamus increased, whereas the cortical-thalamic, cortical-striatum and cortical-cerebellar connections exhibited a decrease in the strength of the functional connection. Conclusion: There is a disruption of the topology of the motor execution network in WD patients, which may be the potential basis for WD motor dysfunction and may provide important insights into neurobiological research related to WD motor dysfunction.