Wilsons disease

Wilson's disease, or hepatolenticular degeneration, is an autosomal recessive disorder affecting between one and three persons per 100 000 of the population. The abnormality in Wilson's disease is defective hepatic excretion of copper. The consequence is copper deposition and injury to many organs, particularly the liver and the brain. The genetic defect occurs on chromosome 13, and the gene product is probably a transmembrane copper transporter. (54) Because copper accumulation is slow, signs and symptoms do not appear before the age of 6 years. Most patients present with manifestations of organ damage between the ages of 8 and 20. Prolonged extrahepatic release of copper not bound to ceruloplasmin causes basal ganglion destruction, and sometimes cerebral cortex destruction. Prominent neuropsychiatric symptoms include tremor, muscular rigidity, and dystonic postures. Patients with Wilson's disease exhibit behavioural abnormalities that can include irritability, aggression, disinhibition, and recklessness. Depressive features are common, but psychosis is rarely seen. Almost invariably, the severity of psychiatric symptoms correlates with the severity of neurological symptoms, especially dystonic and bulbar manifestations.(55) Treatment for Wilson's disease is with D-penicillamine.

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