Regression of Kayser-Fleischer rings during oral zinc therapy: correlation with systemic manifestations of Wilson's disease.
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.
Department of Ophthalmology, W.K. Kellogg Eye Center, Ann Arbor, MI 48105, USA.
B
B
Esmaeli
M A
MA
Burnstine
C L
CL
Martonyi
A
A
Sugar
V
V
Johnson
G J
GJ
Brewer
D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D001927:Brain Diseases / Q000473:pathology; D003300:Copper / Q000652:urine; D003315:Cornea / Q000187:drug effects / Q000503:physiopathology*; D003316:Corneal Diseases / Q000188:drug therapy / Q000503:physiopathology*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy / Q000503:physiopathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008982:Molybdenum / Q000008:administration & dosage / Q000627:therapeutic use; D011446:Prospective Studies; D015032:Zinc / Q000008:administration & dosage / Q000627:therapeutic use*
D003300:Copper; D008982:Molybdenum; C020809:tetrathiomolybdate; D015032:Zinc