Slow Viruses/ Agent disease Flashcards

1
Q

how are slow virus or slow agent disease characterized by

A

incubation period period lasting for many months to years

- usually ends in death

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2
Q

what is the difference between convention and unconventional agent replication process

A

conventional: in vitro, produce CPE
unconventional: no CPE in vitro

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3
Q

conventional agents do what to the central nervous system

A

evoke inflammatory reaction of CNS

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4
Q

unconventional agents do not cause waht

A

no inflammatory response
no Ab response
no interferon production

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5
Q

what is Scrapie

A

disease of sheep marked by tremors and ataxia

- no deymyelination, no pleocytosis, no febrile reaction, no meningeal signs

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6
Q

what does Scrapie cause in gray matter

A

spongiform changes in gray matter- destruction of neurons

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7
Q

how does one get Scrapie

A

genetic and/or infectious in origin

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8
Q

physically what is Scrapie

A

PRION - aberrant protein PrPsc

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9
Q

PrPsc form what

A

scrapie associated fibrils found in brain

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10
Q

what does PrPc stand for

A

normal cell

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11
Q

PrPc is anchored to what

A

phosphatidylinositol glycan

- embedded in cell surface membrane

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12
Q

how does one get Human Ecephalopathies

A

genetically based disease

- familial transmission

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13
Q

how is Human encephalopathies transmitted

A

infecitous transmission

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14
Q

familial origins of prion disease results from mutations in the what normal gene? where is this gene located

A

PrP gene

human chromosome 20

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15
Q

what are mutations in the PrP gene

A

CJD

GSS

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16
Q

Scrapie agent is resistant to what

A

UV irradiation

ionizing irradtion

17
Q

what inactivates scrapie

A

chlorox ( sodium hypochlorite)

18
Q

what is the only immunity the body has to scrapie

A

no immunity except for Ab to neurofilaments

19
Q

how is scrapie’s transmissible

A

animals

20
Q

what does not inactivate scrapie agent

A

formalin- brain tissue in formalin still infectious after 7 years - threat to pathologists

21
Q

where does PrP sc go in the cell

A

accumulates in cells and is deposited in cytoplasmic vesicles or it can be released from the cell surface

22
Q

how can PrPsc cause disease and be transmissible

A

abnormal tertiary level folding

  • causes CNS degernation
  • interact with normal PrPc
23
Q

what is kuru

A

spongiform encephalitis in man similar to scrapie in sheep

24
Q

clinical symptoms of kuru

A
tremors 
little or no demenita
incoordination
ataxia
somnolence
sleepiness
25
Q

how has Kuru been passed

A

eating body parts of dead people who have it

26
Q

clinical symptoms of Creutzfeld-Jacob disease

A
presenile dementia
ataxia
myoclonic fasciculations 
semiconsciousness 
involuntary contraction of groups of muscle fibers
27
Q

what is found in the infectious tissue of Creutzfeld-Jakob disease

A

SAF

28
Q

how is Ceutzfeld-Jakob transmitted

A

corneal transplants
contaminated brain electrodes
maybe sheep to human

29
Q

what are diseases that are possibly caused by unconventional agents

A

alpers
picks
alzheimers

30
Q

what are 2 diseases caused by conventional agents (viruses)

A

Subacute sclerosing panencephalitis (SSPE)

Progressive Multifocal Leukoencephalopathy (PML)- papovavirus

31
Q

what happens inside the body for SSPE

A
progressive demyelination
degenerative neurological disease
fatal
enormous increase in hypertrophic astrocytes
proliferation of microglial cells
32
Q

what is a causative agent for SSPE

A

measles virus

33
Q

what are 2 viruses associated with PML

A

SV40

JC

34
Q

PML is associated with what disorders

A

lymphoproliferative disorders