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Scientific foundations Of neuropsychiatry > Neuropathology Of Neurodegeneration > Flashcards

Neuropathology Of Neurodegeneration Flashcards

1
Q

What is a neurodegenerative disease?

A

Condition which primarily affects the neurones in the human brain and/or spinal cord resulting in a loss of their structure and/or function.

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2
Q

What is dementia?

A

The development of multiple cognitive deficits that represent d a decline from previous level of functioning and is severe enough to impair occupational and/or social functioning

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3
Q

What does disease of the parietal lobe lead to?

A

Visuospatial impairment
Impaired integration of sensory inputs
Agnosia
Apraxia
e.g. neglect, Gerstmann

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4
Q

What does disease of the occipital lobe lead to?

A

Visual impairment
(e.g. posterior cortical atrophy)

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5
Q

What does disease of the temporal lobe lead to?

A

Lateral: receptive dysphasia (Wernicke’s), automatisms

Medial: disorders of memory, hallucinations

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6
Q

What does disease of the frontal lobe lead to?

A

Impairments in judgement, abstract reasoning, inhibition, planning

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7
Q

What is the unifying pathology of neurodegenerative diseases?

A

Accumulation of filamentous proteins (amyloid)

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8
Q

Which disorders show extra cellular protein aggregates?

A

Prion diseases - PrP
Alzheimer’s - amyloid beta

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9
Q

Which disorders show cytoplasmic/neuritic protein aggregates?

A

Parkinson’s, DLB - alpha synuclein (neurons)
MSA - alpha synuclein (glia)

ALS, FTD (FUS, TDP-43)

AD, ALS, PSP, CBD, GGT, Pick’s - Tau

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10
Q

Which disorders show intranuclear protein aggregates?

A

Huntington’s - Huntingtin
SCA - Ataxin
DRPLA - atrophin-1

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11
Q

How many repeats might you see in a Ray doublet or triplet band?

A

3 or 4

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12
Q

What is the age of onset of Alzheimer’s?

A

40-90

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13
Q

What is the average time from Alzheimer’s onset to death?

A

9-12 years

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14
Q

What are the genes associated with familial AD?

A

Chromosome 21 - APP
Chromosome 19 - APOE E4
Chromosome 14 - PSEN1
Chromosome 1 - PSEN2

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15
Q

What would you see histologically in an Alzheimer’s brain

A

Extracellular neurotic plaques (AB)
Intracellular neurofibrillary tangles (tau)

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16
Q

What is Braak staging associated with?

A

Tau pathology

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17
Q

What are the Braak stages 1 and 2?

A

Transentorhinal - asymptomatic

1: small density of NFTs in transentorhinal cortex

2: moderate density in transentorhinal cortex and small density in hippocampus CA1

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18
Q

What are the Braak stages 3 and 4?

A

Limbic - incipient AD

3: Moderate density throughout CA1, introduction to fusiform gyrus, amygdala

4: Severe involvement in same areas, introduction to cortex

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19
Q

What are the Braak stages 5 and 6?

A

Isocortocal - symptomatic

5: tangles throughout the hippocampus, sorted through the isocortex but soaring sensory and motor cortices

6: increased density of tangles, involvement of thalamus, hypothalamus, substantia nigra

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20
Q

What is the staging for amyloid beta?

A

Thal 1-5

21
Q

What is amyloid angiopathy?

A

Amyloid beta forms in the blood vessel walls, weakening the vessels
Can cause infarcts or haemorrhages

Important co-pathology with AD
Occurs without AD also

22
Q

What staining can you use for amyloid?

A

Congo red
Silver
Immunohistochemistry

23
Q

What are some problems with the amyloid cascade hypothesis?

A

Disease symptoms correlate better with tau than AB pathology

Drug trials using anti AB treatments haven’t been so effective (aducanumab)

24
Q

How is it hypothesised that tau proteins accumulate?

A

Like prion proteins - seeding and propagation model

Recent results using tau contradict this

25
Q

What are the diagnostic criteria for Parkinsonism?

A

2 out of 3:
Bradykinesia
Rigidity
Tremor

26
Q

What are the hallmark features of idiopathic Parkinson’s disease?

A

Mask like face
Forward tilt
Reduced arm swing
Shuffling gait
Trembling

27
Q

What are the pathological hallmarks of Parkinson’s?

A

Lewy bodies
Absence of neuromelanjn in the substantia nigra

28
Q

Where are Lewy bodies seen in LBD?

A

Substantia nigra and cortex

29
Q

How do Lewy bodies in the cortex differ from those in the substantia nigra?

A

No halo
Harder to detect on HNE stain
Requires immunohistochemistry

30
Q

What other conditions show Parkinsonism?

A

Progressive supranuclear palsy
Corticobasal degeneration
Multiple system atrophy
Vascular dementia
PDC Guam

31
Q

What drugs can give Parkinsonian symptoms?

A

Dopamine antagonists
(Antipsychotics)

32
Q

What are the three types of vascular dementia?

A

Multi infarct dementia

Diffuse white matter changes - Binswanger type

Strategic infarct

33
Q

What movement abnormalities are associated with vascular dementia?

A

Hemiparesis bellismus
Parkinsonism

34
Q

What are the pathological hallmarks of Huntington’s Disease?

A

Shrinkage of the caudate and putamen
Ubiquitinated huntingtin inclusions (intranuclear)

35
Q

What are the three main syndromes of FTD?

A

Behavioural variant
Progressive non fluent aphasia
Semantic variant

36
Q

What are Pick inclusions composed of?

A

Tau

37
Q

What would be seen macroscopically in the FTD brain?

A

Frontotemporal atrophy
Widening of sulci
Shrinkage of gyri

38
Q

What is the most common type of FTD inclusion?

A

TFP-43

39
Q

What is the prognosis for ALS/MND?

A

Less than a year

40
Q

What are hallmarks of symptoms of ALS/MND?

A

Atrophy/weakness of muscles
Upper motor neuron signs - hyperreflexia
Bulbar palsy - problems with tongue and swallowing

41
Q

What are the genes associated with familial ALS?

A

C9ORF72 (repeat expansion)
TARDP
SOD1
FUS

42
Q

Where are most mutalstiond in TDP43?

A

Glycine rich region

43
Q

What is the most common inclusion in ALS?

A

TFP-43

44
Q

How did iatrogenic CJD come about?

A

Growth hormone from pituitary
Dural and corneal transplants

45
Q

How does CJD present?

A

Ataxia
Myoclonus
Motor neurone disorders

46
Q

What is the protein only hypothesis of prion disease?

A

Prion protein undergoes a conformational change (from alpha helix to beta pleated sheet) which acts as a template for more protein conversion

47
Q

What changes are seen histologically in prion disease?

A

Spongiform change in the brain
Prion protein deposition

48
Q

What is the histological hallmark of vCJD?

A

Florida plaque

Scientific foundations Of neuropsychiatry (26 decks)

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