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New page: * 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 m...
* 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
* 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.
* 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.
* 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
* 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.
* 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.