A Case if Wilson's Disease

Wilson's disease is an autosomal recessive abnormality in the hepatic excretion of copper that results in toxic accumulation of the metal in liver, brain, and the other organs. Wilson's disease associated with brain lesion was found in 25-year-old woman who had dysarthria, wing-beating tremor, and Kayser-Fleisher ring. Laboratory data revealed hypoceruloplasminemia, hypercuprinuria, hypocupronemia and increased copper deposition in the liver biopsy specimen. The brain CT showed mild cortical atropy and bilateral low attenuation in lentiform nucleus.