On these “Gold Standard Step 1 Facts” pages you will find Free:
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Let’s start our USMLE Review with Anatomy from the Gold Standard USMLE Step 1 Audio Review program.
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?
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?
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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