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.
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Authors | B Esmaeli, M A Burnstine, C L Martonyi, A Sugar, V Johnson, G J Brewer |
Journal | Cornea
(Cornea)
Vol. 15
Issue 6
Pg. 582-8
(Nov 1996)
ISSN: 0277-3740 [Print] United States |
PMID | 8899270
(Publication Type: Journal Article)
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Chemical References |
- Copper
- Molybdenum
- tetrathiomolybdate
- Zinc
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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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