Study on the relationship between urinary copper deficiency and neurological symptoms exacerbation after treatment in Wilson's disease

Abstract

Abstract Objective: To study the clinical characteristics and response to chelating agent therapy in patients of Wilson's disease (WD) with insufficient urinary copper exclusion, and to analyze the factors of insufficient urinary copper exclusion. Methods: Collect 40 WD patients with deficiency urinary copper exclusion (the highest urinary copper <500mg/L), 40 WD patients with normal urinary copper exclusion, and 10 normal control cases. The modified Young scale, Child grading, metal metabolism were checked. Magnetic sensitive imaging, dispersion tensor imaging, magnetic resonance imaging checks were performed. The values of phase value (CP), fractional anisotropy (FA), N-acetyl nmda/Creatine (NAA/Cr) were measured. According to the imaging results, WD patients were divided into clinical stages (metal deposition stage, fiber damage stage and neuron necrosis stage). All patients were treated with metal chelating agent for 3 months.Results: 26 cases were in the stage of neuronal necrosis in the group with insufficient urinary copper exclusion. After D-penicillamine (DPA) and sodium dimercaptopropansulfonate (DMPS) treatment, the modified Young scale score of group with insufficient copper exclusion was higher than that of the group with normal copper exclusion (P=0.013, 0.027). The factors influencing the deficiency of urinary copper exclusion were disease type (OR value: 1.22, P=0.031), age of onset (OR value: -0.19, P=0.041), and total score of modified Young scale (OR value: 0.14, P=0.020).Conclusion: WD patients with insufficient urinary copper exclusion have high severity of neurological symptoms. After DPA and DMPS treatment, WD patients with insufficient urinary copper exclusion are prone to worsen neurological symptoms.