66 Pathology: Neurodegeneration Flashcards

1
Q

Alzheimer Disease

  • How common is this?
  • How often is their a genetic component?
  • When do most sporadic cases manifest?
A
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2
Q

Alzheimer Disease

  • What 3 symptoms does this usually manifest with?
  • What 2 symptoms are indicated of server cortical dysfunction?
  • How does end-stage AD present?
  • What is usually the cause of death?
A
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3
Q

Alzheimer Disease

  • What is the fundamental abnormality of AD?
    • Does location matter?
A
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4
Q

Alzheimer Disease

  • Where do A-Beta peptides aggregate?
  • Where do Tau tangles develop?
    • Where do they eventually end up?
A
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5
Q

Alzheimer Disease

  • What is the probably the initiating event for AD pathology?
A
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6
Q

Alzheimer Disease

A
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7
Q

Alzheimer Disease

  • What genetic condition affects chromosomes and is related to an increased risk for AD
A
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8
Q

Alzheimer Disease

  • What happens between AB generation and visible plaque formation?
A
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9
Q

Alzheimer Disease

  • What kind of protein is 2?
  • What are the 3 things that happen to tangle development?
  • What 2 pathways have been suggested that cause injury to neurons?
A
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10
Q

Alzheimer Disease

  • Genetic Risk Factors
    • What protein, located on chromosome 19, has been implicated in AD development?
      • What Isoform increases the risk for AD?
      • Is this isoform this increase AB or Tau generation?
A
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11
Q

Alzheimer Disease

  • Inflammation
    • What 2 cells does AB elicit an inflammatory response from?
      • What is good about this response?
      • What is bad about it?
A
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12
Q

Alzheimer Disease

  • Relate the appearance of AB plaques and Tau tangles to cognitive impairment.
  • What is a better indicator of severe dementia: High # of tangles or high # of AB?
A
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13
Q

Alzheimer Disease

  • Morphology
    • What happens to the cerebral sulci?
      • Where is this most prominent?
    • What happens to the ventricles?
A
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14
Q

Alzheimer Disease

  • Histology
    • What 2 things can diagnose AD at this level?
      • Which one is intracellular?
      • Which one is extracellular?
A
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15
Q

Alzheimer Disease

  • Neuritic plaques
    • What are these derived from?
    • What do they surround?
      • What does this contain?
    • What 3 regions of the brain do they deposit in?
    • What notable regions do they NOT deposit in, until late stages of the disease?
      *
A
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16
Q

Alzheimer Disease

  • Neurofibrillary Tangles
    • What are these bundles of?
    • What do they bind to; acids or bases?
    • Where are they located in the cell?
      • What do they do to the cell’s nucleus?
      • What happens when the cell dies?
    • What are the 5 cells that these are common in?
A
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17
Q

Alzheimer Disease

  • Where is the plaque?
  • Where is the tangle?
  • What is the brown stuff?
A
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18
Q

Frontotemporal Lobar Degeneration

  • What deficits to patients do patients usually have?
    • Which ones appear first?
    • How does this help differentiate this condition from Alzheimer Disease?
      • How does the age of the patient help differentiate this condition from Alzheimer Disease?
A
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19
Q

Frontotemporal Lobar Degeneration

  • What are the subgroups of this condition?
    • Which one contains inclusions?
    • Which one contains DNA/RNA-binding proteins?
A
20
Q

Frontotemporal Lobar Degeneration

  • What is shown in these two images?
A
21
Q

Frontotemporal Lobar Degeneration

  • How does the FTLD subgroup that presents with PICK BODIES relate to Alzheimer’s Disease?
A
22
Q

Frontotemporal Lobar Degeneration

  • In FTLD-TDP, what is NOT seen in nuclear staining of neurons?
A
23
Q

Parkinsons Disease

  • What are the 4 things that characterize the clinical symptoms of this disease?
    • What is the difference between parkinsonism and PD?
    • What neuronal inclusions are associated with PD?
A
24
Q

Parkinson’s Disease

  • What two cellular processes are defective in this disease?
  • What protein is inside a Lewy body?
    • What function is this protein involved in?
A
25
Q

Parkinson’s Disease

  • What can cause autosomal dominant PD?
  • How does Gaucher disease relate to PD?
  • What is Glucocerebrosidase?
A
26
Q

Parkinson’s Disease

  • What two gross structures appear to pale on autopsy?
    • Which one is associated with norepinephrine?
A
27
Q

Parkinson’s Disease

  • Cells are lost in the Substantia Nigra Compact as well as the locus coeruleus. In the cells that don’t die, what inclussion rematins in the cytoplasm?
    • What immune cell is assoicated with these
A
28
Q

Parkinson’s Disease

  • What are the clinical manifestations of PD?
A
29
Q

Parkinson’s Disease

  • How lond does the disease take to produce severe immobility?
  • What does death usually result from? (2)
A
30
Q

Parkinson’s Disease

  • When is L-DOPA most helpful?
  • What non-Rx therapy can help reduce L-DOPA dosage in later stages of the disease?
    • What strucutres are associated with this nonRx therapy?
A
31
Q

Huntingtons

  • What kind of inheritance does this have?
  • What degnerates?
A
32
Q

Huntingtons

  • What type of movements are characteristic of this?
A
33
Q

Huntingtons

  • How long do patients live after symptoms start?
  • Whatare the early cognitive symptoms?
  • What do HD patients have a high risk for?
A
34
Q

Huntingtons

  • Where is the mutaiton?
    • What kind of mutation?
    • What does a high copy number present as?
A
35
Q

Huntingtons

  • Is this a loss or gain of function mutation?
  • Mutant PRotein
    • What 2 things can cause the large intranuclear aggreates?
A
36
Q

Huntingtons

  • Which part of the striatum has the most atrophy?
    • How does this present on a gross scale?
A
37
Q

Huntingtons

  • Aside from the striatum, what other basal ganglia structure may atrophy?
  • Which lobe in the brain frequently atrophies?
A
38
Q

Huntingtons

  • What is left in striatal cells that do not die?
A
39
Q

ALS

  • Where are Betz cells?
  • What happens when lower motor neurons are loss?
  • What happens when upper motor neurons are lost?
  • What is usaully spaired in this disease?
A
40
Q

ALS

  • Who gets this more: men or women?
    • At what age?
  • What happens at the start of symptoms?
  • What happens as this progresses?
  • What is the usual cause of death for patients?
A
41
Q

ALS

  • What percent of cases genetic, and what type of ingeritence is seen?
    • What gene accounts for about 20% of genetic cases, and what does this mutation trigger in cells?
  • What is the m ost common cause of familial ALS?
A
42
Q

ALS

  • What is the most common gross morpholic change?
  • What gross changes happen within the brain in severe cases?
A
43
Q

ALS

  • Which spinal cord has ALS?
A
44
Q

ALS

  • What three things are seen on histologic cross section of the spinal cord?
A
45
Q
A