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Neurology > Prions > Flashcards

Prions Flashcards

1
Q

TSE’s?

A

Transmissable Spongiform Encephalopathy

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2
Q

What causes TSEs?

A

Prions (infectious proteins)

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3
Q

TSE disease process

A

Long incubation period but then rapidly progressive. Usually leads to a fatal dimentia
Pathology shows multifocal spongiform changes

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4
Q

Are there amyloid plaques present in TSE

A

not usually

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5
Q

Inflammation in TSE (transmissable spongiform enceph)

A

minimal

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6
Q

Human TSE

A
  • kuru
  • creutzfeld-jakob
  • Gertsmann Straussler Scheinker disease
  • Fatal familial insomnia
  • Variant CJD
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7
Q

Animal TSE

A

Scrapie (seen in sheeps and goats)
Bovine spongiform encephalopathy (BSE)
Chronic Wasting Disease (deer and elk)

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8
Q

Agents that cuase TSEs are resisitant to what

A

Regular virus treatments which damage nucleic acids.

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9
Q

Prion stands for

A

Proteinacious infectious particles

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10
Q

Prion protein is highly conserved among humans and animals

A

KNOW

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11
Q

where is the prion protein encoded>

A

on a single axon on human chromosome 20

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12
Q

Prion protein is highly expressed where

A

neurons and lymphocytes

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13
Q

Prion protein final product

A

a 209 amino acid glycoprotein that is anchored to cell surfaces by a fatty acid

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14
Q

WHat are the two general forms of prions

A

The physiologic type: PrPc

The patologic type: PrPsc or PrP res
Stands for PrP scrapie or PrP resistant

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15
Q

KNOW PrPc and PrPsc can have identical sequences yet they are different because of what

A

They way they fold.

PrPsc is unusually stable and only partially degraded by proteases. Aggregates in cell cytoplasm and leads to neuron apoptosis

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16
Q

Remember…you must express PrPc in order to contract disease. ALso remember that there are probably chaperone proteins involved in this process since simly mixing PrPsc and PrPc together doesnt generate scrapie protein

A

ok

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17
Q

What the fuck is the scrapie disease

A
  • subacute, progressive ataxia of sheep
  • first found at one year of age in lymphatics and tissues
  • by 2 yrs the brain is infected
18
Q

First documented human TSE

A

Kuru

19
Q

Most common human TSE

A

Creutzfeld Jakob (CJD)

20
Q

Three forms of CJD

A

sporadic- most common
Iatrogenic
Familial

21
Q

classic CJD quadrad

A
  • Dementia
  • Myoclonus
  • Ataxia
  • Mutism
22
Q

Sporadic CJD onset age

A

around 60

23
Q

Mean survival time for sporadic CJD

A

only 5-8 mos

24
Q

Cause of sporadic CJD

A

pretty much unknown

25
Q

How do you get iatrogenic CJD

A

Dural grats
Corneal grafts
HGH

NOT BLOOD TRANSMISSION

26
Q

Familial CJD inheritance

A

Auto dominant

age of onset 45-50
survival 2-4 yrs

27
Q

CJD diagnosis

A
  • CLinical course: rapid dementia and myoclonus
  • histology shows spongiform change and no amyloid which rules out alzheimers
  • Check CSF to rule out tertiary syphillis
28
Q

Gertzman Schausler Shanker (sp?) follows what inheritance patterns

A

AD

29
Q

What mutation is GSS associated with

A

codon 102 of PrP

30
Q

Clinical features of GSS

A

avg age of onset is 48
mean time to death is 5 years
gait abnormalities and ataxia
dementia is less common

31
Q

KNOW>..GSS shows amyloid plaques and spongiform changes

A

ok

32
Q

FFI inheritance

A

AD

33
Q

Clinical features of FFI

A
  • sleep disturbaces

- autonomic dysfunction

34
Q

histo of FFI

A

no spongiform changes. just neuronal loss

35
Q

Variant CJD

A

NEW!! found in UK in 90’s

36
Q

the causative agent of CJD is the same as what

A

The same as BSE (mad cow). So it came from contaminated beef or blood transfusion which is different that the CJD we discussed earlier

37
Q

One major difference between regular CJD and variant is

A

Regular affects pretty much only the CNS

Variant = CNS, retina, DG, bone marrow, tonsils, spleen, lymphnodes, appendix, blood

Also:
Age of onset is 29 in variant (compared to 60)
mean survivial is 14 in variant (compared to 5-8)
SEnsory abnormalities , anxiety, depression

38
Q

ALL INDIVIDUALS WITH vCJD were homozygous for

A

methionine at position 129 of Prp

39
Q

Diagnosis of vCJD

A

prion positive tonsil biopsy

histology shows spongiform changes in basal ganglia…flower appearance

40
Q

how do you properly sterilize graft equit to protect from iatrogenic CJD

A

soak in 1 M sodium hydroxide for an hour followed by autoclave at 134 C

Neurology (38 decks)

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