Wilson’s disease is an autosomal recessive disorder in which excessive amounts of copper accumulate in body tissues, particularly in the liver, kidneys, cornea, and brain. It presents initially at ages 8–12 with hepatic, neurological, or psychiatric disturbances including movement disorders (dysarthria, rigidity, tremor) and psychotic behavior. There are characteristic Kayser-Fleischer rings caused by copper in the limbus of the cornea. Serum ceruloplasmin and copper levels are low, and urinary copper is increased.
KeywordsCaudate Nucleus Globus Pallidus Dentate Nucleus Autosomal Recessive Disorder Menkes Disease
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16 Basal Ganglia Diseases
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