USMLE Step 1 Review 25 22 Wilson’s Disease

USMLE Step 1 Review 25 22 Wilson’s Disease

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Play USMLE Audio MP3 25 22 Wilson’s Disease Below

Begin 25 22 Wilson’s Disease Transcription

 

Moving on to a new topic, what disease can cause damage to the caudate nucleus, putamen, the globus pallidus, cerebral cortex and cerebellum, due to the deposition of copper?

  • This is Wilson’s disease.

Now Wilson’s disease is what type of metabolic disease?

  • It is a disorder of copper metabolism that results in copper deposition in many tissues.

And what are the genetics of Wilson’s disease?

  • It is an autosomal recessive disorder.

Where has the gene defect in Wilson’s disease been localized?

  • The long arm of chromosome 13.

While the exact mechanism of Wilson’s disease is unknown, its pathogenesis appears to involve decreased binding of copper to what transport protein?

  • Ceruloplasm.

And what is the most common ocular finding in Wilson’s disease, the name of it? Kaiser-Flescher rings. How are these Kaiser-Flescher rings described?

  • Bilateral brown corneal rings that result from copper deposition.

In what membrane are the Kaiser-Flescher rings actually located?

  • In Descemet’s membrane.

How is Descemet’s name spelled?

  • Descemet.

Now, in addition to increased copper deposition, what is increased in the serum?

  • There are increased serum levels of ceruloplasmin.

Now according to some sources, which part of the basal ganglia is most particularly damaged in Wilson’s disease?

  • This is the globus pallidus.

****END OF TRANSCRIPTION****

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