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Flashcards in Path Notes Deck (32)
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1
Q

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

A

Abnormal protein homeostasis

2
Q

Gender difference in Alzheimer’s

A

Women slightly more likely

-odds for any one woman now exceeds breast cancer

3
Q

Where do amyloid plaques tend to be more numerous?

A

Deep cortical layers

4
Q

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

A
  • Tau protein
  • Neurfibrillary tangles (NFTs)
  • Neuropil thrads (NTs)
5
Q

What part of the brain does plaques and tangles spare?

A

Occipital lobe, primary motor cortex

6
Q

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

A

Hippocampus

7
Q

What are the 2 AB peptides?

A

AB 40 = bad

AB 42 = worse, seed

8
Q

What is amyloid angiopathy?

A

AB deposits in cortical and leptomeningeal arteries and arterioles

9
Q

What are Hirano bodies?

A

Intracellular aggregates of actin and actin-associated proteins

10
Q

Where are Hirano bodies frequently found?

A

Hippocampal pyramidal cells

11
Q

What is Tau?

A

microtubule associated protein

12
Q

What happens to Tau in Alzheimer’s?

A
  • Hyperphosphorylated

- Lose ability to bind to microtubules

13
Q

What ApoE alleles have higher/lower risk for AD?

A
E4 = higher risk
E2 = lower risk
14
Q

What 3 Genes are associated with early onset AD

A
  • APP
  • Presenilin 1
  • Presenilin 2

All autosomal dominant

15
Q

3 Types of Frontotemporal Lobar Degeneration (FTLD):

A
  • Tau
  • TDP
  • FUS
16
Q

Knife-edge gross examination of brain suggests:

A

Tau form of Frontotemporal Lobar Degeneration (FTLD)

17
Q

Which type of FTLD is related to ALS?

A

FTLD-TDP

18
Q

Which type of FTLD is most common?

A

Tau type

19
Q

3 Hallmark symptoms of Parkinson’s:

A

Tremor
bradykinesia
Rigidity

20
Q

What are Lewy Bodies made of?

A

Alpha-synuclein

21
Q

Difference of Progressive Supranuclear Palsy vs. Parkinson’s

A

No Lewy Bodies in PSP

22
Q

Important exam finding in PSP:

A

Difficulty with voluntary eye movements

23
Q

Histological findings in PSP:

A
  • Globose tangle

- Tufted astrocyte

24
Q

Exam and histological findings in Corticobasal Degernation (CBD)

A

Exam: jerking of limbs, dyspraxia (cognitive dysfunction)

Histology: Ballooned neuron

25
Q

When does trinucleotide repeat expansion occur in Huntingtons?

A

Spermatogenesis

26
Q

Where does atrophy occur in Huntington’s?

A

Caudate and Striate

27
Q

Freidreich’s ataxia age of onset:

A

1st or 2nd decade of life

28
Q

Disease associated with Freidreich’s Ataxia:

A

Diabetes (1/4 of patients)

29
Q

Genetics of Freidreich’s Ataxia:

A
  • GAA trinucleotide repeat on 9q13 frataxin protein

- Autosomal recessive

30
Q

Exam finding of ALS:

A
  • Foot drop
  • Muscle cramps
  • Sensation preserved
31
Q

Proteins associated with Spinal Muscular Atrophy

A
  • SMN1, SMN2

- Gene needed for motor neuron survival

32
Q

Diseases with Tau:

A
  • Alzheimer’s
  • FTLD
  • Parkinson’s
  • PSP
  • CBD