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Kat's Pathology > Neurodegeneration > Flashcards

Neurodegeneration Flashcards

1
Q

What are prion diseases

A

A series of diseases with a common molecular pathology.

Prion = proteinaceous infectious only.

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2
Q

What is the pathology of prion disease

A

A transmissible factor, with no DNA or RNA involved.

Involves the misfolding of PrP proteins.

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3
Q

What are some prion diseases in humans (4)

A

Creutzfeldt-Jakob disease (CJD).
Gerstmann-Straussler-Sheinker syndrome.
Kuru
Fatal familial insomnia

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4
Q

What is the histopathology of prion disease (2)

A

Spongiform change

Prion protein deposits

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5
Q

What is New Variant CJD (vCJD)

A

A prion disease.

A sporadic neuropsychiatric disorder.

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6
Q

How does vCJD differ to CJD

A

vCJD has a longer duration than CJD

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7
Q

What age group does vCJD affect

A

Patients <45 years old

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8
Q

What are the clinical features of vCJD (2)

A

Cerebellar ataxia

Dementia

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9
Q

What is vCJD linked to

A

BSE

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10
Q

What are the neuropathological features of alzheimer’s disease (4)

A

Extracellular plaques (senile plaques)
Neurofibrillary tangles
Cerebral amyloid angiopathy (CAA)
Neuronal loss (cerebral atrophy)

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11
Q

What is the protein deposited and accumulated in AD

A

Amyloid-beta plaques.

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12
Q

What is amyloid-beta formed from

A

Byproduct of APP processing.

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13
Q

What are the deposits seen in AD (2)

A

Amyloid-beta.

Tau

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14
Q

What does CTE stand for

A

Chronic traumatic encephalopathy

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15
Q

What is CTE

A

A form of dementia characterised by repeated head trauma (e.g. in boxers) with an early age of onset

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16
Q

What is the histological hallmark of PD

A

Lewy bodies

17
Q

What is the protein found within lewy bodies

A

Alpha-synuclein

18
Q

What is considered the gold standard for diagnosing PD

A

Alpha-synuclein immunostaining

19
Q

What is the staging system for PD called

A

Braak

20
Q

What are some causes of Parkinsonism (8)

A 
Idiopathic Parkinson's disease. 
Drug-induced Parkinsonism 
Multiple system atrophy 
Progressive supranuclear palsy 
Corticobasal degeneration 
Vascular pseudoparkinsonism 
Alzheimer's changes 
Fronto-temporal neurodegenerative disorders
21
Q

What are the histological hallmarks of Picks disease (4)

A

Fronto-temporal atrophy
Marked gliosis and neuronal loss
Balloon neurons
Tau positive Pick bodies.

22
Q

What is the inheritance pattern of FTDP-17 syndromes

A

Autosomal dominant

23
Q

What is the structure of tau (6)

A

Single gene on 17q21
16 exons
Alternative splicing gives rise to 6 isoforms
3R or 4R tau (microtubule binding domains)
Two further inserts with unknown function
Shortest form (3R0/0N)

24
Q

What is used to help diagnose dementias molecularly

A

Tau molecular analysis

25
Q

Where to get the rest of the info for this lecture

A

Year 4 notes!

Kat's Pathology (105 decks)

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