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Flashcards in dementia, Parkinson, Huntington disease Deck (57):
1

Dementia - definition

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

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causes of dementia

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

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other causes of dementia

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

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MCC of dementia in elderly

Alzheimer disease

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2nd MCC of dementia in elderly

Multi-infract (aka vascular)

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which patients have increased risk for Alzheimer disease

Down syndrome patients

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familiar form of Alzheimer disease - proportion and proteins

10%
APP, presenilin-1, presenilin-2

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Altered proteins - Alzheimer disease

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

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Alzheimer disease - presenilin 2

increased risk for early onset (familiar)

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Alzheimer disease - ApoE4

increased risk of sporadic

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Alzheimer disease - presenilin 1

increased risk for early onset (familiar)

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Alzheimer disease - ApoE2

decreased risk of sporadic

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familiar form of Alzheimer disease - APP

increased risk for early onset (familiar)

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Alzheimer disease - gross appearance

- widespread cortical atrophy
- Narrowing of gyri and widening of sulci

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Alzheimer disease - histology

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

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Alzheimer disease - senile plaques

extracellular β-amyloid (Aβ) core

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

cleaving amyloid precursor protein (APP)

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APP

amyloid precursor protein

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Alzheimer disease - senile plaques CAN CAUSE (how)

amyloid angiopathy --> intracranial haemorrhage
(deposition around vessels)

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Alzheimer disease - Neurofibrillary tangles

intracellular, hyperphosphorylated tau protein

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tau protein

insoluble cytoskeletal elements

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Alzheimer disease - number of Neurofibrillary tangles ....

correlates with degree of dementia

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Lewy body dementia - clinical course

initially dementia and visual hallucinations followed by parkinsonian features

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Lewy body dementia - histology

1. loss of pigmented neurons in substantial nigra
2. lewy bodies (PRIMARILY CORTICAL)

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Lewy bodies

round eosinophilic inclusion of α-synuclein (PRIMARILY CORTICAL)

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Creutzfeldt-Jakob disease - histology

spongiform cortex

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Creutzfeldt-Jakob disease - mechanism

prions (PrPc-->PrPsc sheet)
β-pleated sheet resistant to protease

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Creutzfeldt-Jakob disease - sporadic or familiar

typically sporadic
some familiar forms

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Frontotemporal dementia - is also called

Pick disease

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Frontotemporal dementia (Pick disease) - gross appearance

Frontotemporal lobe degeneration

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Frontotemporal dementia (Pick disease) - histology

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

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Frontotemporal dementia (Pick disease) - Pick bodies

silver-staining spherical tau protein aggregates

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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

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Frontotemporal dementia (Pick disease) - affected areas

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

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vitamins deficiency associated dementia - vitamins

Vitamins B1, B3 or B12

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Dementia - definition

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

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Alzheimer disease - neurotransmitter alternations

decrease of Ach

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Creutzfeldt-Jakob disease - clinical presentation

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

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demetia with startle myoclonus

Creutzfeldt-Jakob disease

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Parkinson disease is a .... of CNS

degenerative disorder

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Parkinson disease - histology

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

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Parkinson disease - Lewy bodies

composed of a-synuclein - intracellilar eosnophilic inclusions

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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

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Parkinson disease - Tremor / gait

- resting tremor (pill-rolling tremor at rest)
- Shuffling gait

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Huntington disease - mode of inferitence

AD trinucleotide repeat disorder (anticipation)

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Huntington disease - chromosome of trinucleotide / trinucleotide repeat / age

ch 4
CAG
20-50

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Huntington disease - symptoms

1. choreiform movements
2. aggression
3. depression
4. dementia

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Huntington disease - movement disorders

1. athetosis
2. chorea

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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

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Huntington disease - a common cause of death

suicide

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Huntington disease - pathogenesis

neuronal death via NMDA-R binding and glutamate toxicity

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Lewy body dementia - area in the CNS

cortical

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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

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Huntungton is sometimes mistaken for

substance abuse