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Pathoma Rev > 17.7 Dementia & Degen Disorders > Flashcards

17.7 Dementia & Degen Disorders Flashcards

1
Q

What is the protein that carries an INcreased risk of developing AD? Why?

A

-Epsilon-4-ApoE-Increases APP conversion to the beta form

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2
Q

What causes the suppressed expression of genes with Huntington’s disease?

A

Deacetylation of histones

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3
Q

What is the pathophysiology of normal pressure hydrocephalus?

A

Unknown etiology of inability to resorb CSF in the arachnoid granulations

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4
Q

What is the normal configuration of prion protein? Abnormal?

A

-Normal = Alpha (PrP^c)-Abnormal = beta sheet (PrP^sc)

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5
Q

What acute treatment can be performed for normal pressure hydrocephalus?

A

LP

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6
Q

What are the characteristics of the EEG with CJD?

A

Spike wave complexes

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7
Q

What are Lewy bodies composed of?

A

Alpha-synuclein

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8
Q

What are the major clinical features of PD? (4)

A

-Tremor-Rigidity-Akinesia/bradykinesia -Postural instability and shuffling gait

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9
Q

Cogwheel rigidity = ?

A

PD

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10
Q

What is the major pathophysiology of Huntington’s disease?

A

Degeneration of the GABAergic neurons in the caudate nucleus of the BG

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11
Q

True or false: most cases of Alzheimer’s are inherited

A

False–sporadic

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12
Q

What are characteristic of the hallucinations of lewy body dementia?

A

Vivid and benign

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13
Q

Is huntington’s disease AD or AR? What is the genetic defect, and what protein is affected?

A

AD repeat of CAG in the huntingtin gene

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14
Q

Why is it that patients with Down syndrome develop Alzheimer’s?

A

Chromosome 21 is where APP is—thus they express more of it

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15
Q

What are the most important risk factors for the development of early AD? (2)

A

-Presenilin 1 or 2 gene-Down Syndrome

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16
Q

What is the natural h/o CJD?

A

Usually results in death in less than 1 year

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17
Q

What is the protein that carries a DEcreased risk of developing AD?

A

-Epsilon-2-ApoE

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18
Q

What are the two major gray matter areas in the brain?

A

-Cerebral hemispheres-BG

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19
Q

Are there focal neurological deficits with Alzheimer’s?

A

No

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20
Q

What are the genes that increase the risk for later Alzheimer’s disease?

A

Epsilon-ApoE4

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21
Q

What is the protein that accumulates in Pick’s disease?

A

-Spherical tau protein aggregates-TDP43

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22
Q

Degeneration of the brainstem and BG leads to what?

A

Movement disorders

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23
Q

What is the treatment for normal pressure hydrocephalus?

A

VP shunt

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24
Q

What is the key gross finding of Huntington’s disease?

A

Loss of the caudate nucleus and resulting hydrocephalus ex vacuo

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25
Q

What are the characteristics of Lewy body dementia?

A

-Dementia-Hallucinations-Parkinsonian features

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26
Q

Mask like facies = ?

A

PD

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27
Q

What is the usual presentation of CJD? (3)

A

-Rapidly progressive dementia -ataxia-startle myoclonus

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28
Q

What is vCJD?

A

Exposure to bovine protein

29
Q

What is the most common cause of dementia?

A

Alzheimers

30
Q

What is the key risk factor for the development of PD?

A

Aging

31
Q

Where does the substantia nigra project to? What neurotransmitters are released here?

A

-Dopamine-Striatum

32
Q

What is cerebral amyloid angiopathy?

A

A-beta amyloid in AD deposits around blood vessels, weakening them and leading to hemorrhage

33
Q

What causes the anticipation seen in HD?

A

Further expansion of CAG during spermatogenesis

34
Q

What is the usual cause of CJD?

A

Sporadic–rarely arises due to exposure to prion infected human tissue

35
Q

What are the two major gray matter structures of the striatum?

A

-Caudate-Putamen

36
Q

What is the cause of Parkinson’s disease?

A

Degenerative loss of dopaminergic neurons in the substantia nigra pars compacta of the BG

37
Q

What is the drug that is known for causing PD?

A

MPTP

38
Q

What generally causes vascular dementia?

A

HTN, atherosclerosis, or vasculitis

39
Q

What, generally, is hydrocephalus ex vacuo?

A

The appearance of enlarged ventricles 2/2 loss of cerebral matter

40
Q

What is the role of the caudate on the cortex?

A

Project GABA to inhibit movement

41
Q

What are the genes that increase the risk for early Alzheimer’s disease?

A

Presenilin 1 or 2

42
Q

What is the effect of beta (PrP^sc) on other proteins in the brain?

A

Produces more of itself, which is not degradable

43
Q

What is the pathogenesis of Alzheimer’s disease?

A

Disruption of the degradation of APP into beta amyloid, leading to A-beta-amyloid deposition

44
Q

True or false: AD is a diagnosis of exclusion

A

True–confirmed with pathology at autopsy

45
Q

What is the effect of D1 and D2 binding on the striatum, on the cortex?

A

D1 binding = increased stimulationD2 = decreased inhibition

46
Q

What is familial fatal insomnia?

A

Prion disease that causes progressive insomnia, exaggerated startle response, and death

47
Q

What is the basic pathogenesis of dementia?

A

Loss of neurons within the gray matter

48
Q

What is normal pressure hydrocephalus?

A

CSF builds up, resulting in dilated ventricles

49
Q

What are the three classic symptoms of normal pressure hydrocephalus?

A

DementiaAtaxiaUrinary incontinence(“wacky, wobbly, and wet”)

50
Q

Pill rolling tremor = ?

A

PD

51
Q

What is normal role of Tau protein in neurons?

A

Helps to organize microtubules

52
Q

What is the second most common cause of dementia?

A

Vascular dementia

53
Q

What is the natural h/o vascular dementia?

A

Stepwise deterioration of mental functioning

54
Q

What are the three ways to acquire prion disease?

A

-Sporadically-Inherited-Transmitted

55
Q

What are the first few symptoms of Pick’s disease?

A

Early behavior and language symptoms, followed by dementia

56
Q

What are the nerve fibers that run along the edges of the ventricles, and are stretched in normal pressure hydrocephalus (which causes s/sx)?

A

Corona radiata

57
Q

Degeneration of what large area of the brain leads to dementia?

A

Cortex

58
Q

What are the characteristics hallmarks of spongiform encephalopathy?

A

Vacuoles in the brain

59
Q

What is Pick’s disease (aka frontotemporal dementia)?

A

Degenerative disease of the frontal and temporal cortex, that spares the parietal and occipital lobes

60
Q

Is the memory loss with Alzheimer’s fast or slow?

A

-Slow memory loss-Progressive disorientation-Loss of learned motor skills-Behavior and personality changes-Mute and bedridden

61
Q

Where are Lewy bodies found in lewy body dementia?

A

Cortices

62
Q

What are the neurofibrillary tangles seen in AD?

A

Hyperphosphorylated Tau protein in cells

63
Q

What are the neuritic plaques seen in AD?

A

Pink extracellular AB amyloid, surrounded by entrapped neuritic processes

64
Q

What are the gross characteristics of brains with AD?

A

Diffuse cerebral atrophy:-Narrowing of gyri-Widening of the sulci

65
Q

What is the most important risk factor for the development of sporadic AD?

A

Age

66
Q

Early onset dementia in the setting of suspected PD, is called what?

A

Lewy body dementia–although it is the same pathophysiologic progress underpinning it.

67
Q

What separates the caudate and the putamen on a coronal cut of the brain?

A

Anterior segment of the internal capsule

68
Q

What is the startle myoclonus seen with CJD?

A

Minimal stimuli causes myoclonus

69
Q

What is the area of the BG that sends either stimulatory or inhibitory signals to the cortex?

A

Striatum

Pathoma Rev (4 decks)

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