Adjunctive Antioxidant Therapy in Neurologic Wilson’s Disease Improves the Outcomes
- 31 Downloads
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.
KeywordsWilson’s disease Oxidative stress Copper Vitamin C and E
Compliance with Ethical Standards
Conflict of Interest
On behalf of all the authors, the corresponding author states that there are no conflicts of interest.
The research has been approved by the Institutional Ethics Committee, SGPGIMS, Lucknow (Ethic No. A-03: PGI/IMP/IEC/56/19.08.2011).
BFM Burke-Fahn-MarsdenCNS Central nervous systemCu CopperGSH GlutathioneLPO Lipid peroxidation MRI Magnetic resonance imagingMDA MalondialdehydeWD Wilson’s diseaseTAC Total antioxidant capacity
- Bernardo-Colon A, Vest V, Clark A, Cooper ML, Calkins DJ, Harrison FE, Rex TS (2018) Antioxidants prevent inflammation and preserve the optic projection and visual function in experimental neurotrauma. Cell Death Dis 9:1097. https://doi.org/10.1038/s41419-018-1061-4 CrossRefPubMedPubMedCentralGoogle Scholar
- Ferenci P (2017) Diagnosis of Wilson disease. Handbook of clinical neurology 142:171–180. https://doi.org/10.1016/B978-0-444-63625-6.00014-8 CrossRefGoogle Scholar
- Mehvari J, Motlagh FG, Najafi M, Ghazvini MR, Naeini AA, Zare M (2016) Effects of vitamin E on seizure frequency, electroencephalogram findings, and oxidative stress status of refractory epileptic patients. Adv Biomed Res 5:36. https://doi.org/10.4103/2277-9175.178780 CrossRefPubMedPubMedCentralGoogle Scholar
- Smirnova J, Kabin E, Järving I, Bragina O, Tõugu V, Plitz T, Palumaa P (2018) Copper(I)-binding properties of de-coppering drugs for the treatment of Wilson disease. α-Lipoic acid as a potential anti-copper agent. Sci Rep 8:1463–1463. https://doi.org/10.1038/s41598-018-19873-2 CrossRefPubMedPubMedCentralGoogle Scholar
- Wiles C (1990) Quantification of neurologic deficit. Journal of Neurology Neurosurgery & Psychiatry 53:628Google Scholar