Histopathology 13 - Neurodegenerative diseases Flashcards
is there any DNA or RNA involved in prion?
no
What does Prion stand for
PRoteinaceous Infections ONly
Histology of prion disease
Spongeiform change and prion protein deposits
Prion disease endemic to papua new guinuea
Kuru
difference between CJD and vCJD
in vCJD patients <45, linked to BSE, cerebellar ataxia and dementia /
Most common type of prion disease
Sporadic CJD
What is an example of familial prion disease?
Fatal familial insomnia (AD)
Alzheimer’s dementia neuropathology
Senile plaques of Amyloid-beta protein Neurofibrillary tangles (Tau)
Which area of the brain is commonly affected in AD?
Hippocampus
Why do neurofibrillary tangles form?
Tau is abnormal as it is hyperphosphorylated which self aggregates –> NFTs which are neurotoxic –> microtubule instability
How is amyloid beta protein formed?
Cleavage of amyloid precursor protein (APP)
Normal pathophysiology of APP cleavage vs abnormal
Normal: APP cleaved by alpha secretase and products of its breakdown can be removes
Abnormal: APP cleaved by beta secretase –> production of AB protein which aggregates
Staging system used in Tau pathologies
BRAAK staging
Name of the tauopathy seen in boxers
Chronic traumatic encephalopathy
Main protein involved in PD?
Alpha synuclein
What are Lewy bodies?
Cells with alpha synuclein
Which cells are lost in PD
Loss of dopaminergic cells of substantia nigra –> coloration of SN lost
Where do cells of the substantial nigra project to?
Basal ganglia
What functions is the basal ganglia heavily involved in?
Movement
Gold standard diagnostic for PD
Alpha synuclein immunostaining
Multisystem atrophy - what is it?
Synucleinopathy targeting GLIAL cells in cerebellum, patients tends to present with falls
Progressive supra nuclear palsy - which protein involved?
TAU
WHAT ARE THE two forms of TAU?
3r OR 4r
mYELIN basic protein and proteo-lipid protein
MS
