(2) Slow Virus and Prions

Question 1

Slow or chronic infections of the CNS

Answer 1

Slow Virus Infections

Question 2

Incubation period for Slow Virus infection

Answer 2

YEAAAAAARSSSS

Question 3

what are the 2 mentions slow virus infections

Answer 3

• Subacute Sclerosing Panencephalitis (SSPE)
• Progressive Multifocal Leukoencephalopathy (PML)

Question 4

Slow Virus

• Rare disease caused by Measles virus
• occurs 2-10 years after recovery from measles

Answer 4

Subacute Sclerosing Panencephalitis (SSPE)

Question 5

Slow Virus

what happens with the CNS in Subacute Sclerosing Panencephalitis (SSPE)

Answer 5

Slow progressive demyelination in the CNS = ending in death :(

Question 6

Slow Virus

what are the initial symptoms of Subacute Sclerosing Panencephalitis (SSPE)

Answer 6

• memory loss
• irritability
• seizures
• involuntary muscle movements
• and/or behavioral changes

all symptoms leading to neurological deterioration and death

Question 7

Slow Virus

what are found in the neurons and glial cells with SSPE infection

Answer 7

Large numbers of viral nucleocapsid

Question 8

Slow Virus

Caused by JC virus (a polyomavirus)

Answer 8

Progressive Multifocal Leukoencephalopathy (PML)

Question 9

Slow Virus

John Cunningham virus, a ds-DNA, icosahedral, and
naked

Answer 9

JC virus (a polyomavirus)

Question 10

Slow virus

• Rare
• 85% of adults in the general population have the virus

Answer 10

Progressive Multifocal Leukoencephalopathy (PML)

Question 11

Slow virus

PML virus usually remain dormant in what sites of the body

Answer 11

lymph nodes, bone
marrow, or kidneys throughout our lifetime

Question 12

TOF
PML can cause CNS complication that occurs in immunocompromised
individuals (e.g., AIDS patients)

Answer 12

True

Question 13

• Small, proteinaceous particles
• no nucleic acid

Answer 13

Prions

Question 14

Cellular prion protein

Answer 14

PrPC

Question 15

A modified prion

Answer 15

PrPSC

Question 16

Prion

A normal protein in humans and other animals which is in extended form of recent infection with sars-cov-2

Answer 16

PrPC

Question 17

Prion

Globular – resistant to proteases

Answer 17

PrPSC

Question 18

Responsible for the disease causing of Prion

Answer 18

PrPSC

Question 19

who suggested the model of how prions cause disease?

Answer 19

Stanley Prusiner

Question 20

Prion - Pathogenesis

(1) PrPsc binds to the

Answer 20

normal PrPc on the cell surface and cause it to be processed into PrPsc

Question 21

Prion - Pathogenesis

(2) The cell replenishes the ____, and the cycle continues.

Answer 21

PrPc

Question 22

Prion - Pathogenesis

(3) ____ aggregates as amyloid-like plaques in the brain

Answer 22

PrPsc

Question 23

Prion - Pathogenesis

what causes the aggregates as amyloid-like plaques in the brain

Answer 23

PrPsc

Question 24

Prion - Pathogenesis

(4) e plaques consist of host protein might explain the lack of an immune response to these agents in ____

Answer 24

spongiform encephalopathies

Question 25

SYmptoms of prion disease

Answer 25

- Loss of muscle control - shivering - tremors - dementia

Question 26

# Prions - Symptoms post mortem appearance of the brain with large vacuoles in the cortex and cerebellum

Answer 26

Spongiform encephalopathies

Question 27

TOF Vaccine is available for treatment of Prions

Answer 27

F (No treatment available)

Question 28

This the only way to minimize or prevent the prion disease

Answer 28

Control

Question 29

Control for KURU

Answer 29

cessation of ritual of cannibalism

Question 30

Control for GSS and CJD

Answer 30

disinfection of neurosurgical tools and electrodes in 5% hypochlorite, 1.0 M NaOH, or autoclaving at 15 psi for 1 hour

Question 31

# Prions - What DIsease Host: Human Mechanism: Cannibalism

Answer 31

Kuru

Question 32

# Prions - What DIsease Host: Human Mechanism: Spontaneous PrPc to PrPsc conversion or somatic mutation

Answer 32

Sporadic Creutzfeldt-Jacob Disease (CJD) and Sporadic fatal insomnia

Question 33

# Prions - What DIsease Host: Human Mechanism: nfection from prioncontaining material, e.g., dura mater, electrode

Answer 33

Iatrogenic CJD

Question 34

# Prions - What DIsease Host: Human Mechanism: Mutation in the PrP gene

Answer 34

Familial CJD and Gerstmann-Straussler Scheincker (GSS) disease

Question 35

# Prions - What DIsease Host: Human Mechanism: Infection from BSE (bovine spongifrom encelopathy) (mad cow)

Answer 35

vCJD

Question 36

# Prions - What DIsease Host: Human Mechanism: D178N mutation in the PrP gene, with M129 polymorphism

Answer 36

Familial Fatal Insomnia (FFI)

Question 37

# Prions - What DIsease Host: Sheep Mechanism: Infection in susceptible sheep

Answer 37

Scrapie

Question 38

# Prions - What DIsease Host: Cattle Mechanism: Infection from contaminated food

Answer 38

Bovine Spongiform Encephalopathy (BSE)

Question 39

# Prions - What DIsease Host: Mink Mechanism: Infection from sheep or cattle in food

Answer 39

Transmissible mink encephalopathy (TME)