A Case of Wilson's Disease.
- Author:
Hun Soo KIM
1
;
Sang Jin KIM
;
Hang Im JO
Author Information
1. Department of Ophthalmology, College of Medicine, Keimyung University, Daegu, Korea.
- Publication Type:Case Report
- MeSH:
Basal Ganglia Diseases;
Brain;
Copper;
Deglutition Disorders;
Dysarthria;
Hepatolenticular Degeneration*;
Kidney;
Liver;
Liver Cirrhosis;
Metabolism;
Muscle Rigidity
- From:Journal of the Korean Ophthalmological Society
1987;28(3):661-665
- CountryRepublic of Korea
- Language:Korean
-
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.