Week 6.5 Flashcards

1
Q

What is the protein aggregate found in AD, PD, HD, CJD, and ALS?

A
  • AD: beta-amyloid, tau tangles
  • PD: alpha-synuclein
  • HD: huntingtin
  • CJD: prion
  • ALS: TDP-43
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2
Q

Alzheimer’s has a typical onset of what age?

A

sixty years old

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3
Q

Describe the gross pathology of Alzheimer’s disease.

A
  • early medial temporal atrophy
  • occipital lobe relatively spared
  • large ventricles and hydrocephalus ex vacuo
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4
Q

Alzheimer’s typically spares what lobe of the brain?

A

occipital

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5
Q

What vascular disease is associated with Alzheimer’s disease?

A

cerebral beta-amyloid angiopathy

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6
Q

What are Hirano bodies?

A

actin aggregates common in the hippocampal neurons of those with Alzheimer’s

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7
Q

Beta-amyloid is a product of what metabolic process?

A

cleavage of the APP protein by beta- and gamma-secretase

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8
Q

The APP gene is located on which chromosome?

A

21

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9
Q

Down’s syndrome patients often suffer from early onset of what dementia?

A

Alzheimer’s

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10
Q

A functional tau protein plays what physiologic role?

A

it stabilizes microtubules

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11
Q

Which protein aggregate is more strongly associated with the onset of symptoms in Alzheimer’s?

A

the appearance of tau tangles

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12
Q

Tau tangles preferentially affect which brain regions?

A
  • amygdala
  • hippocampus
  • lateral geniculate nucleus
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13
Q

What causes tau to become pathological?

A

it is phosphorylated

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14
Q

What are some genetic risk factors for Alzheimer’s disease?

A

presenilin-1 and ApoE4

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15
Q

The ApoE gene encodes a protein that does what?

A

it is a cholesterol transporter apolipoprotein

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16
Q

What is donepezil?

A

a centrally acting reversible AChE inhibitor that works to treat those with Alzheimer’s disease

17
Q

Which NT system is affected in those with Alzheimer’s?

A

reduced acetylcholine

18
Q

Name two drugs commonly used to treat Alzheimer’s.

A
  • donepezil (AChE inhibitor)

- memantine (NMDA antagonist)

19
Q

Lewy Bodies are a feature of what two disease?

A

Parkinson’s and dementia with Lewy Bodies

20
Q

What are the symptoms of Dementia with Lewy Bodies?

A
  • progressive dementia with fluctuations
  • REM sleep disorder
  • visual hallucinations
  • variable parkinsonism
21
Q

Lewy bodies are comprised of what protein?

A

alpha-synuclein

22
Q

What genetic risk factor exists for dementia with levy bodies?

A

ApoE4

23
Q

Autosomal dominant parkinson’s diseases is the result of a mutation in which gene?

A

leucine rich repeat kinase 2

24
Q

Sporadic parkinson’s has been linked to mutations in which gene?

A

glucocerebrosidase

25
Q

Frontotemporal lobar dementias share what features?

A
  • progressive language deterioration
  • personality changes
  • atrophy of temporal and frontal lobes with sparing of parietal and occipital
26
Q

Pick’s diseases is characterized by what protein aggregate?

A

tauopathy

27
Q

Pick’s disease has an onset of when?

A

20-60 years of age

28
Q

What are the symptoms of Pick’s disease?

A
  • personality change
  • loss of motivation
  • lack of empathy
  • inappropriate behavior
  • language disturbance
  • lack of insight
29
Q

What is the most common genetic cause of ALS?

A

C9orf72

30
Q

What protein aggregate is the hallmark of ALS?

A

TDP-43

31
Q

What is the normal function of TDP-43?

A

work with FUS to ensure the long-term survival of motor neurons

32
Q

What white mater tract is most affected by ALS?

A

anterior and lateral corticospinal tracts