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Regression of Kayser-Fleischer rings during oral zinc therapy: correlation with systemic manifestations of Wilson's disease.

Abstract
Fourteen patients presenting with neuropsychiatric manifestations of Wilson's disease were treated with oral tetrathiomolybdate (TM) for 8 weeks followed by oral zinc (Zn) maintenance therapy. The patients were evaluated prospectively at baseline and at yearly intervals for up to 5 years by slit-lamp biomicroscopy and photography, quantitative neurologic and speech pathology examinations, 24-h urine copper collection, and a quantitative scoring of magnetic resonance imaging (MRI) of the brain. Kayser-Fleischer (KF) ring size decreased significantly during the 5-year study period (p < 0.0001). Although results of neurologic examination, speech pathology examination, and 24-h urine copper level analysis in symptomatic Wilson's disease patients improved during the study period, KF ring regression did not correlate with the improvement in these clinical parameters (p > 0.05). However, there was a correlation between MRI scores and KF ring regression (p = 0.02). Anticopper therapy with TM followed by zinc maintenance therapy is a safe and effective treatment for patients with neurologically symptomatic Wilson's disease. This treatment leads to reduction in KF ring size; however, KF ring reduction is not a good predictor of clinical improvement for patients with neuropsychiatric manifestations of Wilson's disease.
AuthorsB Esmaeli, M A Burnstine, C L Martonyi, A Sugar, V Johnson, G J Brewer
JournalCornea (Cornea) Vol. 15 Issue 6 Pg. 582-8 (Nov 1996) ISSN: 0277-3740 [Print] United States
PMID8899270 (Publication Type: Journal Article)
Chemical References
  • Copper
  • Molybdenum
  • tetrathiomolybdate
  • Zinc
Topics
  • Administration, Oral
  • Adolescent
  • Adult
  • Brain Diseases (pathology)
  • Copper (urine)
  • Cornea (drug effects, physiopathology)
  • Corneal Diseases (drug therapy, physiopathology)
  • Female
  • Hepatolenticular Degeneration (drug therapy, physiopathology)
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Molybdenum (administration & dosage, therapeutic use)
  • Prospective Studies
  • Zinc (administration & dosage, therapeutic use)

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