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Flashcards in Neurodegenerative Diseases Deck (24)
1

What is the most common neurodegenerative disease of the cortex?

Alzheimers

2

What is the prognosis for AD?

3-20 years

3

What is the incidence of AD among 85 year olds?

40%

4

What do AD patients usually die from?

infection - often pneumonia

5

What are the grossa and macroscopic findings in AD?

gross = brain atrophy
microscopic = tangles and plaques

6

Where in the cortex does AD usually start? Progresses?

starts in the hippocampus, then to the frontal lobe, then to the rest of the cortex

7

What are the plaques made of in AD?

AB amyloid

8

What are the tangles made of and where are they located?

tau protein

located in the neuron

9

What are some other pathological changes you see with AD?

1. neuronal loss and gliosis
2. cerebral amyloid angiopathy
3. granulovacuolar degeneration
4. hirano bodies

10

How can you stain for amyloid?

Congo red will stain is slightly redder, but when you turn the slide to a certain angle, it will look green

11

What does the AB peptide come from in AD?

It's an alternate cleaving of amyloid precursor protein.

it's usually cleaved by alpha secretase and gamma secretase into dissolvable parts. But in AD, it's cleaved by gamma and beta secretase. This forms an AB segment that is not soluble and will form aggregates

12

What forms first in AD, the amyloid plaques or the tangles?

the plaques are first and the tau tangles are secondary - maybe directly caused by the plaques damaging the microtubules

13

What parts of the brain are affected in Pick Disease?

It's a distinctive dementia that affects the frontal lobe and temporal lobes only

14

What do you see on pathology in Pick Disease?

you get severe atrophy of the frontal and temporal lobes with neuronal loss, swolen neurons (pick cells) with cytoplasmic neuronal inclusions (pick bodies)

15

True or false: Parkinsons's disease diminishes life expectancy.

false

16

What will you see on pathology in PD?

loss of pigemented neurons causing pallor of substantia nigra

Lewy body formation in the remaining neurons

17

What protein is contained in Lewy Bodies (but we don't really know what it does?

alpha-synuclein

18

What degenerates in amyotrophic lateral sclerosis?

motor neurons

19

Is cognition and sensation affected in ALS?

Nope - just motor

20

What is usually the cause of death in ALS?

Death occurs usually witnin 2-3 years due to the respiratory compromise

21

"Progressive musclar artophy" is a subtype of ALS that is characterized by what?

loss of upper motor neurons only - not LMNs

22

Progressive bulbar palsy is a subtype of ALS that is characterized by what?

cranial nerve signs - especially difficulty swallowing and speaking

23

What proteins are potentially mutated in ALS?

superoxide dismutase
ubiquilin 2 (a housekeeping protein)

24

What will you see on pathology in ALS?

thin anterior roots of spinal cord
reduction in anterior horne neurons
skeletal muscles atrophies
degeneration of corticospinal tracts