Slow Virus and Prion Disease

Question 1

Nature of disease cause by prions forms:

Answer 1

Spongiform encephalopathy

Question 2

What group of viruses cause Visna?

Answer 2

retroviruses

Question 3

Hosts of Chronic wasting diseases that cause spongiform encephalopathy

Answer 3

Mule, deer, elk

Question 4

Conventional viruses that cause Chronic Degenerative CNS Disease

Answer 4

Measles
JC Virus
Rubella Virus

Question 5

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

Answer 5

Visna virus or Progressive Pneumonia Viruses

Question 6

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

Answer 6

in vivo

Question 7

Chronic Degenerative CNS Disease resulting from oligodendrocyte infections by polyomavirus

Answer 7

Progressive multifocal leukoencephalopathy (PML)

Question 8

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

Answer 8

Subacute sclerosing panencephalitis (SSPE)

Question 9

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

Answer 9

Progressive Rubella Panencephalitis (PRP)

Question 10

PRP develops during which decade of life?

Answer 10

second decade (8-19 yo)

Question 11

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

Answer 11

M protein - frequently lacking

Question 12

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

Answer 12

Monoclonal antibodies

Question 13

Signs and Symptoms of PML

Answer 13

memory loss
loss of coordination
mentation problems
vision problems

Question 14

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

Answer 14

T

Question 15

Proteinaceous material devoid of detectable amounts of nucleic acid

Answer 15

Prions

Question 16

Give 2 characteristics common to both classic viruses and prions

Answer 16

Filterable infectious agents
Presence of protein

Question 17

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

Answer 17

Guanidine thiocyanate

Question 18

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

Answer 18

C.

Question 19

PrPˢᶜ vs PrPᶜ:
Multimeric

Answer 19

PrPˢᶜ

Question 20

PrPˢᶜ vs PrPᶜ:
Cytoplasmic vesicles

Answer 20

PrPˢᶜ

Question 21

PrPˢᶜ vs PrPᶜ:
Hourly turnover

Answer 21

PrPᶜ

Question 22

PrPˢᶜ vs PrPᶜ:
Cell free

Answer 22

PrPˢᶜ

Question 23

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

Answer 23

PrPᶜ

Question 24

PrPˢᶜ vs PrPᶜ:
Protease resistant

Answer 24

PrPˢᶜ

Question 25

PrPˢᶜ vs PrPᶜ: Protease resistant

Answer 25

PrPˢᶜ

Question 26

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

Answer 26

PrPᶜ

Question 27

PrPˢᶜ vs PrPᶜ: beta pleated sheet structure

Answer 27

PrPˢᶜ

Question 28

PrPˢᶜ vs PrPᶜ: Turnover is Days

Answer 28

PrPˢᶜ

Question 29

Human version of PrPᶜ is encoded on which chromosome?

Answer 29

chromosome 20

Question 30

2 Basic features of Prion diseases

Answer 30

Neurodegeneration and Spongiform changes

Question 31

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

Answer 31

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

Question 32

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

Answer 32

D.

Question 33

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

Answer 33

tau protein 14-3-3 brain protein

Question 34

2 human spongiform encephalopathies

Answer 34

Kuru Classic CJD

Question 35

2 familial forms of CJD

Answer 35

GSS and FFI

Question 36

The new variant CJD is related to which animal spongiform encephalopathy

Answer 36

BSE

Question 37

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

Answer 37

1 year

Question 38

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

Answer 38

Myoclonus Visual or cerebellar symptoms Pyramidal or extrapyramidal symptoms Akinetic mutism

Question 39

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

Answer 39

A

Question 40

Clinical triad of CJD in 75% of cases includes:

Answer 40

Mental deterioration Myoclonus Periodic sharp EEG complex

Question 41

What is the usual prodrome of CJD?

Answer 41

vague personality changes

Question 42

3 major histological changes in CJD

Answer 42

Spongiform changes Gliosis Neuronal loss

Question 43

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

Answer 43

Variant CJD:

Question 44

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

Answer 44

Variant CJD:

Question 45

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

Answer 45

Classic CJD

Question 46

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

Answer 46

Variant CJD:

Question 47

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

Answer 47

Classic CJD

Question 48

Classic CJD vs Variant CJD: Dementia and early neurologic symptoms

Answer 48

Classic CJD

Question 49

Classic CJD vs Variant CJD: Pulvinar sign on MRI

Answer 49

Variant CJD:

Question 50

Classic CJD vs Variant CJD: psychiatric and painful sensory signs

Answer 50

Variant CJD:

Question 51

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

Answer 51

Classic CJD

Question 52

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

Answer 52

Kuru

Question 53

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

Answer 53

T

Question 54

Kuru is limited where?

Answer 54

New Guinea highlands (from the Fore tribe)

Question 55

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

Answer 55

Gerstmann-Straussler-Scheinker Syndrome

Question 56

Early Symptom of GSS

Answer 56

ataxia

Question 57

Which familial CJD can result to blindness and deafness

Answer 57

GSS

Question 58

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

Answer 58

FFI

Question 59

Familial CJD manifesting with hallucinations and ferocious insomnia

Answer 59

Fatal Familial Insomnia

Question 60

Initial diagnosis of prion disease is made on:

Answer 60

Clinical grounds

Question 61

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

Answer 61

D.

Question 62

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

Answer 62

PMCA protein-misfolding cyclic assay

Question 63

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

Answer 63

D.