Transmissible Spongiform Diseases Flashcards
What is the infectious agent that causes Transmissible Spongiform Diseases?
Prions
Name 4 things that prion can resist degredation by
- High Temperatures
- UV and ionising radiation
- Formaldehyde Treatment
- Proteases
What causes TSE
In terms of the prion
An infectious agent (protein) in a misfolded form
How do prions primarily spread?
Via horizontal transmission e.g saliva, blood, urine
What is the pathogenesis of the diseased/ beta form of the prion?
- Alters proteins
- Changes their conformation and turn sthem into diseased proteins
Name three characteristics of the beta sheet folding pattern
- resistant to proteases
- Aggregate and accumulate as an insoluble amyloid in neurons and neuropil
- Form large amyloid and fibrous plaques
- cause neuronal dysfunction
What happens when the activated prions enter the nervous system
they act as this transcription template
What is their general pathogenesis?
- Ingested by the small intestine
- Use M cells to enter peyers patches and dendritic cells
- Spreads through lymphatic nodules and the spleen
- Prions are then released from the dendritic cells and then enter the nervous endings in the lymphoid tissue
- Spread throughout the CNS
What is the pathogenesis of Scrapie prions?
specific to scrapie
- Amplify in large numbers in the lymphoreticuar system before spreading to the brain via the ANS
What is the pathogenesis of BSE prions?
specific
- Colonise MALT
- Spread via the ANS to the CNS
- Amplify in large numbers in the CNS and PNS
What do the macroscopic lesions of TSE look like?
- Intracytoplamsic vacuoles in neurons and neuropil
- Neuronal loss
- Gliosis
- Absence of leukocytic inflammation
What are the clinical signs of classical scrapie
- Pruritus, neurological signs (e.g ataxia)
- It is infectious
What are the clinical signs of atypical scrapie?
- Neurological signs but typically no pruritus and wool loss
- Sporadic/ Spontaneous (not transmissible in field situations)
Where are atypical scrapie lesions predominantly found?
In the cerebellum rather than the medulla oblongata
What agents cause atypical BSE?
Caused by H and L type agents
* naturally sporadic occuring form