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Flashcards in Path Notes Deck (32):
1

What is the key to pathogenesis of CNS Dementia and neurodegenerative disorders?

Abnormal protein homeostasis

2

Gender difference in Alzheimer's

Women slightly more likely
-odds for any one woman now exceeds breast cancer

3

Where do amyloid plaques tend to be more numerous?

Deep cortical layers

4

What are filamentous intracellular inclusions made of? What are they called?

-Tau protein
-Neurfibrillary tangles (NFTs)
-Neuropil thrads (NTs)

5

What part of the brain does plaques and tangles spare?

Occipital lobe, primary motor cortex

6

Where can plaques and tangles be found early on in Alzheimers?

Hippocampus

7

What are the 2 AB peptides?

AB 40 = bad
AB 42 = worse, seed

8

What is amyloid angiopathy?

AB deposits in cortical and leptomeningeal arteries and arterioles

9

What are Hirano bodies?

Intracellular aggregates of actin and actin-associated proteins

10

Where are Hirano bodies frequently found?

Hippocampal pyramidal cells

11

What is Tau?

microtubule associated protein

12

What happens to Tau in Alzheimer's?

-Hyperphosphorylated
-Lose ability to bind to microtubules

13

What ApoE alleles have higher/lower risk for AD?

E4 = higher risk
E2 = lower risk

14

What 3 Genes are associated with early onset AD

-APP
-Presenilin 1
-Presenilin 2

All autosomal dominant

15

3 Types of Frontotemporal Lobar Degeneration (FTLD):

-Tau
-TDP
-FUS

16

Knife-edge gross examination of brain suggests:

Tau form of Frontotemporal Lobar Degeneration (FTLD)

17

Which type of FTLD is related to ALS?

FTLD-TDP

18

Which type of FTLD is most common?

Tau type

19

3 Hallmark symptoms of Parkinson's:

Tremor
bradykinesia
Rigidity

20

What are Lewy Bodies made of?

Alpha-synuclein

21

Difference of Progressive Supranuclear Palsy vs. Parkinson's

No Lewy Bodies in PSP

22

Important exam finding in PSP:

Difficulty with voluntary eye movements

23

Histological findings in PSP:

-Globose tangle
-Tufted astrocyte

24

Exam and histological findings in Corticobasal Degernation (CBD)

Exam: jerking of limbs, dyspraxia (cognitive dysfunction)

Histology: Ballooned neuron

25

When does trinucleotide repeat expansion occur in Huntingtons?

Spermatogenesis

26

Where does atrophy occur in Huntington's?

Caudate and Striate

27

Freidreich's ataxia age of onset:

1st or 2nd decade of life

28

Disease associated with Freidreich's Ataxia:

Diabetes (1/4 of patients)

29

Genetics of Freidreich's Ataxia:

-GAA trinucleotide repeat on 9q13 frataxin protein
-Autosomal recessive

30

Exam finding of ALS:

-Foot drop
-Muscle cramps
-Sensation preserved

31

Proteins associated with Spinal Muscular Atrophy

-SMN1, SMN2
-Gene needed for motor neuron survival

32

Diseases with Tau:

-Alzheimer's
-FTLD
-Parkinson's
-PSP
-CBD