Annals of Indian Academy of Neurology (Jan 2004)

Malondialdehyde Levels In Wilson′s Disease

  • Sinha Sanjib,
  • Christopher R,
  • Prashanth L K,
  • Vidya N,
  • Arunodaya G R,
  • Rao S,
  • Taly A B

Journal volume & issue
Vol. 7, no. 4
pp. 507 – 510

Abstract

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Background: Copper ion is a pro-oxidant metal and may be responsible for free radical mediated damage in Wilson′s disease (WD). Estimation of serum malondialdehyde (MDA), a standard test to detect oxidative damage, may support this pathogenesis. Aim: The aim of the study was to analyze the serum levels of MDA and correlate with clinical status in patients of WD. Patients and Methods: Forty five patients, from a large cohort of proven WD at various stages of treatment were evaluated. Their clinical parameters were noted and assessment of disability and handicap were done. The laboratory tests were carried out using standard methods. Results: The mean age of patients was 21.1+9.1 years (range: 6-50 years) with M: F ratio of 1.6:1. The mean age at presentation was 14.4+6.9 years (range: 6-42 years) and the mean duration of treatment was 14.4+6.9 years (range: 0-24 years). Their mean serum copper and ceruloplasmin levels were 41.4+22.9 g/d1 (range: 4 -103 g/dl) and 5.8+3.4 mg/dl (range: 2-18 mg/dl) respectively. Thirty eight of them had neurological involvement, four had hepatic form, two had both hepatic and neurological involvement while one was presymptomatic sibling. Patients were assessed using CHU staging (mean: 1.5+0.6), modified schwab and England Activities of Daily Living (MSEADL) scale (mean: 94.44+19.55) and Neurologic symptom Score (NSS) (mean: 3.96+5.2). The mean serum MDA level was 0.65 + 0.34 moles/1 and only four (9.4%) patients had increased levels of MDA (mean: 1.32 + 0.05 moles/1), compared to control (mean: 0.56 + 0.36 moles/1) but it was not statistically significant. No significant correlation was noted between MDA levels and NSS, CHU score, MSEADL score and serum copper level. Conclusion: Increased levels of MDA were detected in only 4 out of 45 patients but those with or without increased MDA levels were not different in their clinical status. These patients were on long term treatment, which might alter the oxidative state. Mechanisms other than oxidative damage may underlie WD. Further studies, especially on newly diagnosed patients, need to be done to validate the mechanism of copper induced damage.