Pathology: Degenerative Diseases and Neoplasias Flashcards
How is a degenerative disease of the nervous system characterized on pathology?
- Loss of cells or cell components
- Bilateral symmetry
- Usually slow progression
- Commonly start at a young age
- Peripheral or central
- Pattern recognition
note: most degenerative diseases are suspected to be of genetic origin
How is pattern recognition of a degenerative neuropathy helpful in diagnosis?
Depending on the disease, species and breed will depend on the process and pathology, thus diagnosis
Define a Lysosomal Storage Disease
Lysosomal storage diseases are inherited metabolic diseases that are characterized by an abnormal build-up of various toxic materials in the body’s cells as a result of enzyme deficiencies
note: often the CNS is involved
note: these diseases are progressive and fatal
How can Lysosomal Storage Diseases be classified in pathology?
Based on the accumulated material found in the cells:
- Glycoproteinosis
- Sphingolipidosis
- Mucopolysaccharidosis
- Glycogenosis
- Proteinosis
- Gangliosidosis
Give an example of a lysosomal storage disease in Friesian calves
GM1: Gangliosidosis
- the accumulation of ganglioside due to an enzyme defect in beta-galactosidase
Give an example of a lysosomal storage disease in Jacob Sheep
GM2: Gangliosidosis
- the accumulation of ganglioside due to an enzyme defect in beta-hexasaminidase
Give an example of a lysosomal storage disease in Cairn and West Highland White Terriers
Krabbe disease or globoid cell leukodystrophy (GLD)
- defective lysosomal hydrolysis of specific galactolipids found primarily in myelin
What is a Transmissable Spongiform Encephalopathy (TSE)?
Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of rare degenerative brain disorders characterized by tiny holes that give the brain a “spongy” appearance.
Give an example of a Transmissable Spongiform Encephalopathy (TSE) in cattle
BSE: Bovine Spongiform Encephalopathy
Give an example of a Transmissable Spongiform Encephalopathy (TSE) in sheep
Scrapie
Give an example of a Transmissable Spongiform Encephalopathy (TSE) in felines
FSE: Feline Spongiform Encephalopathy
Give an example of a Transmissable Spongiform Encephalopathy (TSE) in deer and elk
CWD: Chronic Wasting Disease
Give an example of a Transmissable Spongiform Encephalopathy (TSE) in humans
CJD: Creutzfeldt-Jacob Disease
Kuru
vCJD
What is the human concern when it comes to Bovine Spongiform Encephalopathy (BSE)?
BSE spread to humans is believed to result in variant Creutzfeldt–Jakob disease (vCJD) in humans
How can BSE be diagnosed in a cow?
- Submission of the obex region for testing
- Tests based on protease resistance of diseased prion
How can TSE’s be diagnosed?
- Rapid Tests
- Western Blot
- Histopathology
- Immunohistochemistry
- Strain typing
Is Scrapie in goats and sheep transmissable to humans?
Apparently not
What is Scrapie?
Scrapie is a fatal, degenerative disease affecting the nervous systems of sheep and goats. It is one of several transmissible spongiform encephalopathies (TSEs), and as such it is thought to be caused by a prion