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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
Lewy bodies
round eosinophilic inclusion of α-synuclein (PRIMARILY CORTICAL)
26
Creutzfeldt-Jakob disease - histology
spongiform cortex
27
Creutzfeldt-Jakob disease - mechanism
prions (PrPc-->PrPsc sheet) | β-pleated sheet resistant to protease
28
Creutzfeldt-Jakob disease - sporadic or familiar
typically sporadic | some familiar forms
29
Frontotemporal dementia - is also called
Pick disease
30
Frontotemporal dementia (Pick disease) - gross appearance
Frontotemporal lobe degeneration
31
Frontotemporal dementia (Pick disease) - histology
1. silver-staining spherical tau protein aggregates (Pick bodies) 2. inclusions of ubiquitinated TDP-43
32
Frontotemporal dementia (Pick disease) - Pick bodies
silver-staining spherical tau protein aggregates
33
Frontotemporal dementia (Pick disease) - symptoms
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
Frontotemporal dementia (Pick disease) - affected areas
- frontal and temporal cortex | - spares parietal lobe, occipital lobe and posterior 2/3 of superor temporal gyrus
35
vitamins deficiency associated dementia - vitamins
Vitamins B1, B3 or B12
36
Dementia - definition
dementia is a decreasing in cognitive, memory or functions with intact consciousness
37
Alzheimer disease - neurotransmitter alternations
decrease of Ach
38
Creutzfeldt-Jakob disease - clinical presentation
rapidly progressive (weeks to months) dementia with myoclonus) (startle myoclonus)
39
demetia with startle myoclonus
Creutzfeldt-Jakob disease
40
Parkinson disease is a .... of CNS
degenerative disorder
41
Parkinson disease - histology
1. Lewy bodies | 2. loss of dopaminergic neurons of substantia nigra pars compacta (de-pigmentation)
42
Parkinson disease - Lewy bodies
composed of a-synuclein - intracellilar eosnophilic inclusions
43
Parkinson disease - neurotransmitter alternations
1. decreased dopamine 2. increased Ach 3. Increased serotonin
44
Parkinson disease - symproms
MNEMONIC: TRAP 1. Tremor (pill-rolling tremor at rest) 2. Rigidity (cogwheel) 3. Akinesia (or bradykinesia) 4. Postural instability 5. Shuffling gait
45
Parkinson disease - Tremor / gait
- resting tremor (pill-rolling tremor at rest) | - Shuffling gait
46
Huntington disease - mode of inferitence
AD trinucleotide repeat disorder (anticipation)
47
Huntington disease - chromosome of trinucleotide / trinucleotide repeat / age
ch 4 CAG 20-50
48
Huntington disease - symptoms
1. choreiform movements 2. aggression 3. depression 4. dementia
49
Huntington disease - movement disorders
1. athetosis | 2. chorea
50
Huntington disease - neurotransmitter alternations
1. increased dopamine 2. decreased GABA 3. decreased Ach
51
Huntington disease - MRI
Atrophy of caudate and putamen nuclei with hydrocephalus ex vacuo
52
Huntington disease - a common cause of death
suicide
53
Huntington disease - pathogenesis
neuronal death via NMDA-R binding and glutamate toxicity
54
Lewy body dementia - area in the CNS
cortical
55
Vascular dementia - result of
multiple arterial infracts and/or chronic ischemia
56
Vascular dementia - presentation
step-wise decline in cognitive ability with late-onset memory impairment
57
Huntungton is sometimes mistaken for
substance abuse

neurology FA (31 decks)

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