What is the autosomal recessive disorder of copper metabolism characterized by behavioral changes and neurodegenerative symptoms?

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Wilson's disease is an autosomal recessive disorder that affects copper metabolism in the body. It is caused by a mutation in the ATP7B gene, which is responsible for the transport of copper in the liver. When this gene is dysfunctional, copper builds up in the liver and eventually spills into the bloodstream, leading to accumulation in other tissues, particularly the brain and cornea.

One of the prominent features of Wilson's disease is the neuropsychiatric symptoms that can arise due to the toxic effects of excess copper in the brain. Patients may experience behavioral changes, such as personality shifts, mood disorders, and cognitive decline, which can resemble other neurodegenerative conditions. Additionally, Wilson's disease can lead to neurological symptoms, including movement disorders and tremors, similar to those observed in Parkinson's disease.

Recognizing Wilson's disease is crucial because, with early diagnosis and treatment, which typically involves chelating agents to reduce copper levels, patients can manage symptoms and prevent further neurological damage. The other conditions listed, while they may present with various neurological symptoms, do not specifically relate to copper metabolism and its associated behavioral changes in the same clear context as Wilson's disease.

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