Difference between revisions of "Prion Disease"
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* They are a group of fatal neurodegenerative diseases which occur in a number of species, including man. | * They are a group of fatal neurodegenerative diseases which occur in a number of species, including man. | ||
** For example: | ** For example: | ||
− | *** Bovine spongiform encephalopathy (BSE) in cattle | + | *** [[Bovine Spongiform Encephalopathy|Bovine spongiform encephalopathy (BSE)]] in cattle |
*** Scrapie in sheep | *** Scrapie in sheep | ||
*** Chronic wasting disease in elk | *** Chronic wasting disease in elk |
Revision as of 14:35, 30 May 2011
- Prion diseases are also knowns as transmissible spongiform encephalopathies (TSEs).
- They are a group of fatal neurodegenerative diseases which occur in a number of species, including man.
- For example:
- Bovine spongiform encephalopathy (BSE) in cattle
- Scrapie in sheep
- Chronic wasting disease in elk
- Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and kuru in man
- For example:
- TSEs have a long incubation period, making them difficult to diagnose.
Aetiology
- The aetiology of prion diseases is still highly controversial.
- However, an abnormal isoform of the host-encoded prion protein (PrP) is seen in the brains of affected animals.
- The normal host PrP changes its structure into the disease-associated form PrPSc.
- The abnormal proteint (PrPSc) accumulates as amyloid fibrils in nervous tissue.
- However, an abnormal isoform of the host-encoded prion protein (PrP) is seen in the brains of affected animals.
- The agent appears to be highly resistant.
Pathology
Gross
- Prion diseases cause NO GROSS LESIONS.
Histological
- TSEs typically cause what is known as the "microscopic triad":
- Spongiform change.
- Vacuolation of neurons.
- Particularly in nuclei.
- Astrogliosis
- Amyloid plaques
- These are not always seen.
- Spongiform change.