Effect of reduced glutathione in combination with sodium dimercaptosulphonate in the treatment of neurological Wilson‘s disease

Abstract

"Objective To evaluate the effectiveness and safety of reduced glutathione (rGSH) in combination with sodium dimercaptosulphonate (DMPS) for neurological Wilson’s disease (NWD), and to explore the effect of the combined treatment on oxidative stress levels. Methods Retrospective analysis of clinical data of 118 NWD patients who received rGSH combined with DMPS (observation group, n=62) or DMPS monotherapy (control group, n=56) at the First Affiliated Hospital of Anhui University of Traditional Chinese Medicine from May 2020 to March 2022. Clinical data such as age, gender, clinical outcome, adverse effects, modified Young scale score, white blood cells, platelets, ceruloplasmin (CER), aspartate aminotransferase (AST), alanine aminotransferase (ALT), superoxide dismutase (SOD) and malondialdehyde (MDA) were collected and statistically analysed. Results Of the 62 NWD patients in the observation group, neurological symptoms improved in 46 cases and remained stable in 15 cases, while worsened in 1 case. Of the 56 NWD patients in the control group, neurological symptoms improved in 31 cases and remained stable in 18 cases, while worsened in 7 cases. The improvement of neurological symptoms in the observation group was better than that in the control group (U=2.381, P=0.017). The modified Young scale score was significantly decreased in both groups (P＜0.05), and that in the observation group was lower compared to the control group (P＜0.05). The levels of white blood cell, platelet, and CER remained stable in both groups (P＞0.05). The combination treatment greatly facilitated 24-hour urinary copper excretion, and improved liver function (AST, ALT) and anti-oxidative stress levels (SOD, MDA) (P＜0.05). Similar adverse reactions occurred in both groups (χ2=0.102, P＞0.05). Conclusion Compared to DMPS monotherapy, rGSH plus DMPS for NWD patients can effectively ameliorate neurological symptoms, enhance urinary copper excretion and improve anti-oxidative stress levels with a high safety."

Keywords

• wilson’s disease， reduced glutathione，sodium dimercaptosulphonate，abnormal copper metabolism，neurological symptom， oxidative stress