HY Concept 779

[tommyk]

Case; A pt presents with symptoms of Wilson's dx (copper in liver). Symptoms include rigidity, chorea, tremor. WHERE is the lesion? What is diagnostic lesion in the eye? Liver?













A) Likely the Globus Pallidus and Putamen. Copper deposition in cornea gives Kayser Fleishcher rings (look at it on an atlas). Copper in liver leads to cirrhosis!

[Anonymous]

Here's some additional info and a pic to aide in Tommy's concept



A Kayser-Fleischer ring is a ring of greenish-brown discoloration seen just within the corneoscleral margin of patients with hepatolenticular degeneration (Wilson's disease). Wilson's disease is due to inherited defect in copper metabolism; patients have decreased plasma levels of ceruloplasmin and copper but increased copper in their urine and tissues. Kayser-Fleischer rings are pathognomonic for this disease and result from deposition of copper deposited within Descemet's membrane. Hepatolenticular degeneration refers to the developement of cirrhosis and degeneration of the basal ganglia which results from this excessive copper deposition within the liver and the brain.




ref: medsteps.com