Abstract
Oxidative stress has been reported in Wilson’s disease with neurological manifestation (WDNM), but there is a paucity of studies on the role of adjunctive antioxidant therapy. This study aims to evaluate the efficacy of adjunctive vitamin C and E treatment in reducing oxidative stress and improving clinical outcomes. Forty-nine patients with WDNM were included and their clinical details were noted. Glutathione (GSH), total antioxidant capacity (TAC), and malondialdehyde (MDA) were measured using spectrophotometer at baseline and follow-up. All patients received zinc with or without chelating therapy, and 32 of them prescribed vitamin C (500 mg/day) and E (400 mg/day). Clinical outcomes at 6, 12, and 24 months were categorized as improved, static, or worsened based on improvement in Burke-Fahn-Marsden (BFM) score (>10%) and/or severity grade (> 1). Baseline parameters were similar between two groups; except BFM score was higher in the antioxidant group. At follow-up, the antioxidant group had higher GSH, TAC, and lower MDA levels compared with baseline. Patients on antioxidant treatment experienced improvement more frequently at 6 (53.1% vs. 29.4%), 12 (62.5% vs. 29.4%), and 24 months (68.8% vs. 35.3%) compared with those without antioxidant treatment. In WDNM, adjunctive vitamin C and E treatment reduce oxidative stress and improve clinical outcome.
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Abbreviations
BFM Burke-Fahn-MarsdenCNS Central nervous systemCu CopperGSH GlutathioneLPO Lipid peroxidation MRI Magnetic resonance imagingMDA MalondialdehydeWD Wilson’s diseaseTAC Total antioxidant capacity
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Highlights
Free copper induces oxidative stress in Wilson’s disease.
Vitamin C and E treatment lower oxidative stress.
Adjunctive antioxidant therapy improves outcomes.
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Kalita, J., Kumar, V., Misra, U.K. et al. Adjunctive Antioxidant Therapy in Neurologic Wilson’s Disease Improves the Outcomes. J Mol Neurosci 70, 378–385 (2020). https://doi.org/10.1007/s12031-019-01423-8
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DOI: https://doi.org/10.1007/s12031-019-01423-8