A Case of Wilson's Disease.

Kim, Hun Soo; Kim, Sang Jin; Jo, Hang Im

Case Report

Journal of the Korean Ophthalmological Society 1987;28(3):661-665.
Published online March 1, 1987.

A Case of Wilson's Disease.

¹Department of Ophthalmology, College of Medicine, Keimyung University, Daegu, Korea.
²Department of Ophthalmology, St. Benedict Hospital, Pusan, Korea.

Wilson 씨 병 1 예

김헌수(Hun Soo Kim),김상진(Sang Jin Kim),조행임(Hang Im Jo)

Abstract

Wilson's disease is associated with abnormal deposition of copper in the brain, liver, kidneys and other body tissues, apparently due to an inherited defect in copper metabolism. Clinically the disorder is manifested by signs and symptoms of basal ganglia disease, postnecrotic hepatic cirrhosis, Kayser-Fleischer rings, hypoceruloplasminemia, hypocupremia, hypouricemia, cupruresis, and aminoaciduria. The authors experienced a case of Wilson's disease showing the characteristic signs such as Kayser-Fleischer rings, dysarthria, dysphagia and muscular rigidity, without any signs of liver involvement. A review of this case is provided with the literature.