REGRESSION OF KAYSER-FLEISCHER RINGS DURING ORAL ZINC THERAPY - CORRELATION WITH SYSTEMIC MANIFESTATIONS OF WILSONS-DISEASE

Fourteen patients presenting with neuropsychiatric manifestations of W ilson's disease were treated with oral tetrathiomolybdate (TM) for 8 w eeks 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 neur ologic and speech pathology examinations, 24-h urine copper collection , and a quantitative scoring of magnetic resonance imaging (MRI) of th e brain. Kayser-Fleischer (KF) ring size decreased significantly durin g the 5-year study period (p < 0.0001). Although results of neurologic examination, speech pathology examination, and 24-h urine copper leve l analysis in symptomatic Wilson's disease patients improved during th e study period, KF ring regression did not correlate with the improvem ent in these clinical parameters (p > 0.05). However, there was a corr elation between MRI scores and KF ring regression (p = 0.02). Anticopp er therapy with TM followed by zinc maintenance therapy is a safe and effective treatment for patients with neurologically symptomatic Wilso n's disease. This treatment leads to reduction in KF ring size; howeve r, KF ring reduction is not a good predictor of clinical improvement f or patients with neuropsychiatric manifestations of Wilson's disease.