Prions

Question 1

What is it a causative agent of?

Answer 1

Spongiform encephalopathies of huamns and animals

Question 2

Characteristic

Genome? Size

Answer 2

Small size (<30nm), consists of proteinaceous material devoid of nucleic acids
- Not conventional viruses (incorrectly referred to as slow viruses)

Question 3

What is it resistant to?

Answer 3

High resistance to chemical and physical agents

Question 4

Is it immunogenic?

Answer 4

No

Question 5

How is it distributed in tissues?

Answer 5

Not uniformly distributed
Concentrated in brain tissues

Question 6

How does it replicate?

Answer 6

Abnormal prions combine with normal cellular protein in brain to induce replication of abnormal form

Question 7

What is the name of the disease it causes?

Answer 7

Creutzfeldt-Jacob disease

Question 8

What is the clinical presentation of Creutzfeldt-Jacob disease?

Answer 8

Dementia, ataxia, myoclojnic jerks, death within 1 year

Question 9

Describe the pathology of Creutzfeldt-Jacob disease

Answer 9

Degeneration of CNS with vacuolated neurons (Spongiform apperance), fibrous gliosis (holes in brain)

Question 10

How long is the incubation period of Creutzfeldt-Jacob disease?

Answer 10

Long - years

Question 11

What does EEG for Creutzfeldt-Jacob disease show?

Answer 11

Periodic sharp wave complexes