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AWilson's disease, or hepatolenticular degeneration, is an autosomal recessive disorders of copper (Cu) metabolism characterised by an inability of the liver to excrete Cu into bile and to incorporate Cu into ceruloplasmin.
Clinically, Wilson's disease is characterised by neurologic, psychiatric and/or hepatic abnormalities. Movement disorders associated with Wilson's disease often take the form of action tremor, focal dystonia, rigidity, slowed movements and characteristic speech disorders. The psychiatric profile of Wilson's disease includes disturbances such as anxiety, irritability, and alteration in personality, forgetfulness, depression, mania, and rapid mood swings.
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Pathology MCQs
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