Path 28d: CNS

43 cards

Path 28d: CNS ��� ����


 
  
Created Nov 18, 2011
by
mtsuer

 

 
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1
Animal prion disease
 
BSE in cows (bovine spongiform enceph)
2
Prions are...
 
transmissable damaged PrP protein coded by PRNP on chromosome 20. resistant to proteases.
3
How is PrP damaged in prions?
 
alpha-PrPc to beta-PRPsc
4
Two causes of iatrogenic (from a medical tx) infections of CJ Disease
 
corneal transplants hGH
5
Clinical findings in CJ Disease
 
Rapidly progressing dementia Startle myoclonus (involuntary jerking when surprised)  
6
Prognosis for CJ patient
 
0--it is always fatal. Avg of 7 months
7
How does variant CJ D present differently?
 
younger people neuro sx progress slower, but behavior issues noticeable earlier no alterations...
8
What is vCJ D linked to?
 
BSE
9
**Microscopic findings for vCJ?
 
cortical plaques surrounded by a halo of spongiform change
10
In fatal familial insomnia, what does it have? 1. Spongiform change 2. Neuronal loss 3....
 
No Yes Yes Yes
11
This inherited prion disease begins with cerebellar ataxia then progressive dementia. Death...
 
Gerstmann-Straussler-Scheinker S
12
Microscopic findings of GSSS?
 
Kuru plaques--aggregates of PrPsc
13
Most common demyelinating disorder
 
MS
14
Immune mechanism that damges myelin in MS
 
CD4+ TH1 cells vs. myelin --> ING-g --> MACs
15
Where do you see the gross glassy, gry plaques in MS?
 
White matter; around the lateral ventricles

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