title: Is low serum tocopherol in Wilson's disease a significant symptom?
abstract: BACKGROUND
  Free radical mediated injury is increasingly recognized in many metabolic diseases including Wilson's disease (WD). Use of antioxidants as an adjunctive therapy in WD may have therapeutic significance.
  
  
  OBJECTIVE
  The aim of the study was to correlate serum levels of tocopherols with serum copper and ceruloplasmin and clinical status of these patients.
  
  
  METHODS
  Serum levels of tocopherol of were measured spectrophotometrically using the Emmerie-Engel reaction in 34 patients from a large cohort of WD being followed up at a tertiary care center.
  
  
  RESULTS
  Majority of patients were male (M/F=23:11). The mean serum copper was 43.6+/-26.2 microg/dl (range=10-121 microg/dl) and serum ceruloplasmin was 5.6+/-5.5 mg/dl (range=0-30 mg/dl). The mean serum tocopherol level was 0.68+/-0.18 mg/dl (range=0.23-1.14 mg/dl) and compared to the control (1.07+/-0.17 mg/dl), nearly 59% of patients had decreased levels (p<0.001). No significant correlation was noted between low serum tocopherol levels and serum copper levels, Mini Mental Status Examination (MMSE) scores and CHU staging. However, serum tocopherol levels were lower in patients with relatively short duration of treatment (7.8 years vs. 12.4 years).
  
  
  CONCLUSIONS
  Decreased levels of serum tocopherol were detected in 59% of patients compared to controls. However, low tocopherol levels did not correlate with clinical status or biochemical parameters of WD, except for relatively shorter duration of treatment. Further studies, especially in newly diagnosed patients, need to be done to validate the role of low tocopherol levels in Wilson's disease.
authors.affiliation: Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Hosur Road, Bangalore-560 029, India.
authors.forename: Sanjib
authors.initials: S
authors.lastname: Sinha
authors.forename: Rita
authors.initials: R
authors.lastname: Christopher
authors.forename: G R
authors.initials: GR
authors.lastname: Arunodaya
authors.forename: L K
authors.initials: LK
authors.lastname: Prashanth
authors.forename: G
authors.initials: G
authors.lastname: Gopinath
authors.forename: H S
authors.initials: HS
authors.lastname: Swamy
authors.forename: A B
authors.initials: AB
authors.lastname: Taly
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002570:Ceruloplasmin / Q000378:metabolism; D002648:Child; D003072:Cognition Disorders / Q000097:blood / Q000175:diagnosis / Q000209:etiology; D003300:Copper / Q000097:blood; D005260:Female; D005609:Free Radicals / Q000378:metabolism; D006527:Hepatolenticular Degeneration / Q000097:blood* / Q000150:complications / Q000503:physiopathology; D006801:Humans; D008297:Male; D013223:Statistics as Topic; D024505:Tocopherols / Q000097:blood*; D014811:Vitamin E Deficiency / Q000097:blood* / Q000150:complications / Q000503:physiopathology
chemical_list: D005609:Free Radicals; D003300:Copper; D002570:Ceruloplasmin; D024505:Tocopherols