Histopathology 13 - Neurodegenerative diseases

Question 1

is there any DNA or RNA involved in prion?

Answer 1

no

Question 2

What does Prion stand for

Answer 2

PRoteinaceous Infections ONly

Question 3

Histology of prion disease

Answer 3

Spongeiform change and prion protein deposits

Question 4

Prion disease endemic to papua new guinuea

Answer 4

Kuru

Question 5

difference between CJD and vCJD

Answer 5

in vCJD patients <45, linked to BSE, cerebellar ataxia and dementia /

Question 6

Most common type of prion disease

Answer 6

Sporadic CJD

Question 7

What is an example of familial prion disease?

Answer 7

Fatal familial insomnia (AD)

Question 8

Alzheimer’s dementia neuropathology

Answer 8

Senile plaques of Amyloid-beta protein
Neurofibrillary tangles (Tau)

Question 9

Which area of the brain is commonly affected in AD?

Answer 9

Hippocampus

Question 10

Why do neurofibrillary tangles form?

Answer 10

Tau is abnormal as it is hyperphosphorylated which self aggregates –> NFTs which are neurotoxic –> microtubule instability

Question 11

How is amyloid beta protein formed?

Answer 11

Cleavage of amyloid precursor protein (APP)

Question 12

Normal pathophysiology of APP cleavage vs abnormal

Answer 12

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

Question 13

Staging system used in Tau pathologies

Answer 13

BRAAK staging

Question 14

Name of the tauopathy seen in boxers

Answer 14

Chronic traumatic encephalopathy

Question 15

Main protein involved in PD?

Answer 15

Alpha synuclein

Question 16

What are Lewy bodies?

Answer 16

Cells with alpha synuclein

Question 17

Which cells are lost in PD

Answer 17

Loss of dopaminergic cells of substantia nigra –> coloration of SN lost

Question 18

Where do cells of the substantial nigra project to?

Answer 18

Basal ganglia

Question 19

What functions is the basal ganglia heavily involved in?

Answer 19

Movement

Question 20

Gold standard diagnostic for PD

Answer 20

Alpha synuclein immunostaining

Question 21

Multisystem atrophy - what is it?

Answer 21

Synucleinopathy targeting GLIAL cells in cerebellum, patients tends to present with falls

Question 22

Progressive supra nuclear palsy - which protein involved?

Answer 22

TAU

Question 23

WHAT ARE THE two forms of TAU?

Answer 23

3r OR 4r

Question 24

mYELIN basic protein and proteo-lipid protein

Answer 24

MS

Question 25

whICH CELLS produce myelin?

Answer 25

Oligodendrocytes