Micro 2 Prions Flashcards by Philip Lichtenstein

Q

Normal protein and gene

A

PrPc

PRNP

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Q

Histological Dx

A

Spongiform pattern with no inflammation

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Q

Most common CJD

A

Sporadic

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Q

fCJD inheritance

A

Autosomal dominance

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Q

PrP plaque stain color

A

Brown

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Q

Classic v. Variant CJD histopathology

A

Even spread v. Florid plaques (clumps)

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Q

vCJD changes

A

Most evident in BG & Thalamus
florid plaques
psychiatric -> cerebellar

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Q

vCJD inheritance

A

none LOL

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Q

CJD v vCJD on MRI

A

Pulvinar sign on vCJD

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Q

Kuru progression

A

Ataxia -> Dementia

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Q

vCJD progression

A

Psychiatric -> Ataxia -> Dementia (14 months)

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Q

CJD progression

A

Dementia/Ataxia (5 months)

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Q

Gerstmann-Straussler Scheinker Syndrome

A

Pronounced Cerebellar degeneration

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Q

Fatal Familial Insomnia

A

Insomnia first

D178N mutation

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Q

Myoclonus

A

sCJD

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Q

vCDJ allele

A

Methionine at codon 129