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MICROBIOLOGY MED2 > Slow Virus and Prion Disease > Flashcards

Slow Virus and Prion Disease Flashcards

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1
Q

Nature of disease cause by prions forms:

A

Spongiform encephalopathy

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2
Q

What group of viruses cause Visna?

A

retroviruses

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3
Q

Hosts of Chronic wasting diseases that cause spongiform encephalopathy

A

Mule, deer, elk

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4
Q

Conventional viruses that cause Chronic Degenerative CNS Disease

A

Measles
JC Virus
Rubella Virus

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5
Q

This slow virus infects all the organs of the body of the infected sheep resulting to pathologic changes confined primarily to the Brain, Lungs, Reticuloendothelial system

A

Visna virus or Progressive Pneumonia Viruses

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6
Q

Viral expression of Visna viruses is restricted in (vivo or vitro)?

A

in vivo

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7
Q

Chronic Degenerative CNS Disease resulting from oligodendrocyte infections by polyomavirus

A

Progressive multifocal leukoencephalopathy (PML)

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8
Q

Rare disease of young adults resulting from the reduced efficiency of measles virus transcription in differentiated brain cells maintaining persistent infection in the CNS

A

Subacute sclerosing panencephalitis (SSPE)

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9
Q

Rare complication of congenital rubella that results to severe neurologic (mental and motor) deterioration that progresses to death

A

Progressive Rubella Panencephalitis (PRP)

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10
Q

PRP develops during which decade of life?

A

second decade (8-19 yo)

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11
Q

In SSPE, laboratory findings will show high titers of anti-measles except for which protein?

A

M protein - frequently lacking

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12
Q

PML is also a rare complication of which therapeutic agent for MS?

A

Monoclonal antibodies

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13
Q

Signs and Symptoms of PML

A

memory loss
loss of coordination
mentation problems
vision problems

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14
Q

T/F: Long-term persistent infections of Maedi virus results to Antigenic Variation

A

T

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15
Q

Proteinaceous material devoid of detectable amounts of nucleic acid

A

Prions

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16
Q

Give 2 characteristics common to both classic viruses and prions

A

Filterable infectious agents
Presence of protein

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17
Q

Prions are sensitive to this agent which is effective in decontamination of medical supplies and instruments

A

Guanidine thiocyanate

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18
Q

Which of the following is not effective for Prions?
A. 90% phenol
B. 10% sodium dodecyl sulfate
C. 3.7% Formaldehyde
D. Autoclaving for 1 hour, 121 deg C

A

C.

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19
Q

PrPˢᶜ vs PrPᶜ:
Multimeric

A

PrPˢᶜ

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20
Q

PrPˢᶜ vs PrPᶜ:
Cytoplasmic vesicles

A

PrPˢᶜ

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21
Q

PrPˢᶜ vs PrPᶜ:
Hourly turnover

A

PrPᶜ

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22
Q

PrPˢᶜ vs PrPᶜ:
Cell free

A

PrPˢᶜ

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23
Q

PrPˢᶜ vs PrPᶜ:
Held in the cell membrane by linkage between terminal serine and GPI

A

PrPᶜ

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24
Q

PrPˢᶜ vs PrPᶜ:
Protease resistant

A

PrPˢᶜ

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25
Q

PrPˢᶜ vs PrPᶜ:
Protease resistant

A

PrPˢᶜ

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26
Q

PrPˢᶜ vs PrPᶜ:
Interacts with potassium channels, NMDA receptors and neural cell adhesion molecules

A

PrPᶜ

27
Q

PrPˢᶜ vs PrPᶜ:
beta pleated sheet structure

A

PrPˢᶜ

28
Q

PrPˢᶜ vs PrPᶜ:
Turnover is Days

A

PrPˢᶜ

29
Q

Human version of PrPᶜ is encoded on which chromosome?

A

chromosome 20

30
Q

2 Basic features of Prion diseases

A

Neurodegeneration and Spongiform changes

31
Q

T/F: Host immunosuppression has no effect on pathogenesis of prion diseases

A

T: but chronic inflammation induced by other factors may affect pathogenesis

32
Q

Observations in Spongiform encephalopathy ERXCEPT:
A. Amyloid-containing plaques and fibrils
B. Proliferation of astrocytes
C. Vacuolation of neurons and glial cells
D. Atrophy of astrocytes

A

D.

33
Q

Give the 2 nonspecific protein markers detected in CSF of symptomatic patients with spongiform encephalopathy

A

tau protein
14-3-3 brain protein

34
Q

2 human spongiform encephalopathies

A

Kuru
Classic CJD

35
Q

2 familial forms of CJD

A

GSS and FFI

36
Q

The new variant CJD is related to which animal spongiform encephalopathy

A

BSE

37
Q

Vast majority of CJD patients usually die within ___ yr/s of illness onset

A

1 year

38
Q

4 clinical features included in the criteria for probable and possible classic CJD

A

Myoclonus
Visual or cerebellar symptoms
Pyramidal or extrapyramidal symptoms
Akinetic mutism

39
Q

Which of the following is not a criterion for Familial CJD
A. Definite or probable CJD + possible CJD in a first degree relative
B. Neuropsychiatric disorder plus disease specific PrP gene mutation
C. Definite or probable CJD + Definite or probable CJD in a first degree relative

A

A

40
Q

Clinical triad of CJD in 75% of cases includes:

A

Mental deterioration
Myoclonus
Periodic sharp EEG complex

41
Q

What is the usual prodrome of CJD?

A

vague personality changes

42
Q

3 major histological changes in CJD

A

Spongiform changes
Gliosis
Neuronal loss

43
Q

Classic CJD vs Variant CJD:
13-14mos median duration of illness

A

Variant CJD:

44
Q

Classic CJD vs Variant CJD:
Marked accumulation of protease resistance prion protein

A

Variant CJD:

45
Q

Classic CJD vs Variant CJD:
Variable accumulation of protease resistant prion proteins

A

Classic CJD

46
Q

Classic CJD vs Variant CJD:
Agent readily detected in lymphoid tissue

A

Variant CJD:

47
Q

Classic CJD vs Variant CJD:
Periodic sharp waves often present in EEG

A

Classic CJD

48
Q

Classic CJD vs Variant CJD:
Dementia and early neurologic symptoms

A

Classic CJD

49
Q

Classic CJD vs Variant CJD:
Pulvinar sign on MRI

A

Variant CJD:

50
Q

Classic CJD vs Variant CJD:
psychiatric and painful sensory signs

A

Variant CJD:

51
Q

Classic CJD vs Variant CJD:
Median age of death is 68 yrs

A

Classic CJD

52
Q

Prion disease that is a cerebellar syndrome that means shivering or trembling

A

Kuru

53
Q

T/F: Most patients with Kuru die within a year after symptom onset

A

T

54
Q

Kuru is limited where?

A

New Guinea highlands (from the Fore tribe)

55
Q

An autosomal dominant disease resulting from the defect of prion protein gene PRNP which results in multicentric amyloid plaques in the brain and neurofibrillary degeneration

A

Gerstmann-Straussler-Scheinker Syndrome

56
Q

Early Symptom of GSS

A

ataxia

57
Q

Which familial CJD can result to blindness and deafness

A

GSS

58
Q

Autosomal dominant prion disease that results to Autonomic, Cognitive, Motor and Endocrine changes

A

FFI

59
Q

Familial CJD manifesting with hallucinations and ferocious insomnia

A

Fatal Familial Insomnia

60
Q

Initial diagnosis of prion disease is made on:

A

Clinical grounds

61
Q

Confirmation of prion disease can be made by the following except:
A. MRI
B. CSF tau or 14-3-3 protein
C. protein K-resistant form of PrP in Western blot in tonsil biopsy
D. EEG

A

D.

62
Q

What is used to initiate polymerization of PrP and amplify the number of scrapie-like prior protein units to detect for presence of prions

A

PMCA

protein-misfolding cyclic assay

63
Q

Which of the following can be used to decontaminate for prions?
A. 5% hypochlorite
B. 1.0 M sodium hydroxide
C. autoclaving for 15 psi for 1 hour
D. AOTA

A

D.

MICROBIOLOGY MED2 (9 decks)

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