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neurology FA > dementia, Parkinson, Huntington disease > Flashcards

dementia, Parkinson, Huntington disease Flashcards

1
Q

Dementia - definition

A

dementia is a decreasing in cognitive, memory or functions with intact consciousness

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2
Q

causes of dementia

A
  1. Alzheimer 2. Frontotemporal dementia (Pick disease) 3. Lewy body dementia 4. Creutzfeldt-Jakob disease 5. other causes
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3
Q

other causes of dementia

A
  1. Multi-infarct (vascular) 2. Syphilis 3. HIV 4. Wilson disease 5. Normal pressure hydrocephalus
  2. Vitamins B1, B3 or B12 deficiency
  3. Chronic substance abuse (neurotoxicity of drugs)
  4. Huntington
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4
Q

MCC of dementia in elderly

A

Alzheimer disease

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5
Q

2nd MCC of dementia in elderly

A

Multi-infract (aka vascular)

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6
Q

which patients have increased risk for Alzheimer disease

A

Down syndrome patients

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7
Q

familiar form of Alzheimer disease - proportion and proteins

A

10%

APP, presenilin-1, presenilin-2

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8
Q

Altered proteins - Alzheimer disease

A

altered proteins: ApoE2, ApoE4, APP, presenilin-1, presenilin-2

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9
Q

Alzheimer disease - presenilin 2

A

increased risk for early onset (familiar)

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10
Q

Alzheimer disease - ApoE4

A

increased risk of sporadic

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11
Q

Alzheimer disease - presenilin 1

A

increased risk for early onset (familiar)

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12
Q

Alzheimer disease - ApoE2

A

decreased risk of sporadic

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13
Q

familiar form of Alzheimer disease - APP

A

increased risk for early onset (familiar)

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14
Q

Alzheimer disease - gross appearance

A
  • widespread cortical atrophy

- Narrowing of gyri and widening of sulci

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15
Q

Alzheimer disease - histology

A
  1. loss of ach neurons
  2. senile plaques in gray matter
  3. Neurofibrillary tangles
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16
Q

Alzheimer disease - senile plaques

A

extracellular β-amyloid (Aβ) core

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17
Q

Alzheimer disease - senile plaques - β-amyloid is synthesized by

A

cleaving amyloid precursor protein (APP)

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18
Q

APP

A

amyloid precursor protein

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19
Q

Alzheimer disease - senile plaques CAN CAUSE (how)

A

amyloid angiopathy –> intracranial haemorrhage

deposition around vessels

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20
Q

Alzheimer disease - Neurofibrillary tangles

A

intracellular, hyperphosphorylated tau protein

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21
Q

tau protein

A

insoluble cytoskeletal elements

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22
Q

Alzheimer disease - number of Neurofibrillary tangles ….

A

correlates with degree of dementia

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23
Q

Lewy body dementia - clinical course

A

initially dementia and visual hallucinations followed by parkinsonian features

24
Q

Lewy body dementia - histology

A
  1. loss of pigmented neurons in substantial nigra

2. lewy bodies (PRIMARILY CORTICAL)

25
Q

Lewy bodies

A

round eosinophilic inclusion of α-synuclein (PRIMARILY CORTICAL)

26
Q

Creutzfeldt-Jakob disease - histology

A

spongiform cortex

27
Q

Creutzfeldt-Jakob disease - mechanism

A

prions (PrPc–>PrPsc sheet)

β-pleated sheet resistant to protease

28
Q

Creutzfeldt-Jakob disease - sporadic or familiar

A

typically sporadic

some familiar forms

29
Q

Frontotemporal dementia - is also called

A

Pick disease

30
Q

Frontotemporal dementia (Pick disease) - gross appearance

A

Frontotemporal lobe degeneration

31
Q

Frontotemporal dementia (Pick disease) - histology

A
  1. silver-staining spherical tau protein aggregates (Pick bodies)
  2. inclusions of ubiquitinated TDP-43
32
Q

Frontotemporal dementia (Pick disease) - Pick bodies

A

silver-staining spherical tau protein aggregates

33
Q

Frontotemporal dementia (Pick disease) - symptoms

A
  1. Dementia
  2. aphasia (early) (primary progressive aphasia)
  3. movement disorders (eg. parkinsonism, ALS-like UMN/LMN degeneration)
  4. early change in personality and behaviour
34
Q

Frontotemporal dementia (Pick disease) - affected areas

A
  • frontal and temporal cortex

- spares parietal lobe, occipital lobe and posterior 2/3 of superor temporal gyrus

35
Q

vitamins deficiency associated dementia - vitamins

A

Vitamins B1, B3 or B12

36
Q

Dementia - definition

A

dementia is a decreasing in cognitive, memory or functions with intact consciousness

37
Q

Alzheimer disease - neurotransmitter alternations

A

decrease of Ach

38
Q

Creutzfeldt-Jakob disease - clinical presentation

A

rapidly progressive (weeks to months) dementia with myoclonus) (startle myoclonus)

39
Q

demetia with startle myoclonus

A

Creutzfeldt-Jakob disease

40
Q

Parkinson disease is a …. of CNS

A

degenerative disorder

41
Q

Parkinson disease - histology

A
  1. Lewy bodies

2. loss of dopaminergic neurons of substantia nigra pars compacta (de-pigmentation)

42
Q

Parkinson disease - Lewy bodies

A

composed of a-synuclein - intracellilar eosnophilic inclusions

43
Q

Parkinson disease - neurotransmitter alternations

A
  1. decreased dopamine
  2. increased Ach
  3. Increased serotonin
44
Q

Parkinson disease - symproms

A

MNEMONIC: TRAP

  1. Tremor (pill-rolling tremor at rest)
  2. Rigidity (cogwheel)
  3. Akinesia (or bradykinesia)
  4. Postural instability
  5. Shuffling gait
45
Q

Parkinson disease - Tremor / gait

A
  • resting tremor (pill-rolling tremor at rest)

- Shuffling gait

46
Q

Huntington disease - mode of inferitence

A

AD trinucleotide repeat disorder (anticipation)

47
Q

Huntington disease - chromosome of trinucleotide / trinucleotide repeat / age

A

ch 4
CAG
20-50

48
Q

Huntington disease - symptoms

A
  1. choreiform movements
  2. aggression
  3. depression
  4. dementia
49
Q

Huntington disease - movement disorders

A
  1. athetosis

2. chorea

50
Q

Huntington disease - neurotransmitter alternations

A
  1. increased dopamine
  2. decreased GABA
  3. decreased Ach
51
Q

Huntington disease - MRI

A

Atrophy of caudate and putamen nuclei with hydrocephalus ex vacuo

52
Q

Huntington disease - a common cause of death

A

suicide

53
Q

Huntington disease - pathogenesis

A

neuronal death via NMDA-R binding and glutamate toxicity

54
Q

Lewy body dementia - area in the CNS

A

cortical

55
Q

Vascular dementia - result of

A

multiple arterial infracts and/or chronic ischemia

56
Q

Vascular dementia - presentation

A

step-wise decline in cognitive ability with late-onset memory impairment

57
Q

Huntungton is sometimes mistaken for

A

substance abuse

neurology FA (31 decks)

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