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Mental disorders associated with hepatolenticular degeneration

Hepatolenticular degeneration (HLD) is an autosomal recessive disorder caused by a decrease in the level of ceruloplasmin (a copper binding enzyme) in plasma. It was first reported by Wilson in 1912, so it is also called Wilson's disease. Due to the disorder of copper metabolism in the body, this disease leads to liver, brain and other tissue lesions. The clinical features are extrapyramidal symptoms, liver damage and characteristic corneal pigment ring, accompanied by varying degrees of mental disorders. The age of onset ranges from juvenile to adult, and about half of the patients are 20 ~ 30 years old. Often accompanied by mental symptoms, it is easy to be misdiagnosed as primary mental disorder. The disease progresses continuously, and most of them have a poor prognosis.


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