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| 1 |
Animal prion disease
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BSE in cows (bovine spongiform enceph)
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| 2 |
Prions are...
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transmissable damaged PrP protein coded by PRNP on chromosome 20. resistant to proteases.
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How is PrP damaged in prions?
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alpha-PrPc to beta-PRPsc
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Two causes of iatrogenic (from a medical tx) infections of CJ Disease
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corneal transplants
hGH
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Clinical findings in CJ Disease
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Rapidly progressing dementia
Startle myoclonus (involuntary jerking when surprised)
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Prognosis for CJ patient
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0--it is always fatal. Avg of 7 months
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How does variant CJ D present differently?
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younger people
neuro sx progress slower, but behavior issues noticeable earlier
no alterations...
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What is vCJ D linked to?
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BSE
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**Microscopic findings for vCJ?
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cortical plaques surrounded by a halo of spongiform change
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| 10 |
In fatal familial insomnia, what does it have?
1. Spongiform change
2. Neuronal loss
3....
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No
Yes
Yes
Yes
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| 11 |
This inherited prion disease begins with cerebellar ataxia then progressive dementia. Death...
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Gerstmann-Straussler-Scheinker S
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| 12 |
Microscopic findings of GSSS?
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Kuru plaques--aggregates of PrPsc
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| 13 |
Most common demyelinating disorder
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MS
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Immune mechanism that damges myelin in MS
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CD4+ TH1 cells vs. myelin --> ING-g --> MACs
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Where do you see the gross glassy, gry plaques in MS?
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White matter; around the lateral ventricles
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