NS Infection - Prions Flashcards

1
Q

Whats the main Prion disease?

A

Creutzfeldt-Jakob Disease (CJD)

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2
Q

What is a prion?

A

Transmissable Proteinaceous particle

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3
Q

What are the types of CJD?

A
  • Sporadic
  • New Variant
  • Familial
  • Acquired (Cadeveric Growth Hormone treatment, Dura Mater graft, blood transfusion)
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4
Q

What are the clinical features of Sporadic CJD?

A
  • Slow insidious onset usually in the >60s
  • Early behavioural abnormalities
  • > Rapidly progressing dementia
  • > Global Neurological Decline

Can also cause myoclonus, motor abnormalities, cortical blindness and seizures

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5
Q

DDx for CJD?

A

Alzheimer’s with myoclonus (More prolonged than CJD)

Subacute Sclerosing Panencephalitis (A rare chronic infection with defective measles virus)

CNS vasculitis

Inflammatory Encephalopathies

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6
Q

Prognosis for CJD?

A

Rapid decline then death (within 6 months mostly)

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7
Q

How does New Variant CJD present differently from Sporadic?

A

A younger onset (<40)

Early behavioural changes are much more prominent

Longer course (Avg = 13 months)

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8
Q

How does New Variant CJD reach humans?

A

Eating infected material, mainly cattle.

There’s possibly a genetic susceptibility involved too

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9
Q

How do we investigate CJD?

A

MRI
EEG
LP (with immunoassay)

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