Microbiology 5: Prion Diseases Flashcards

1
Q

Which test detects active syphilis ?

A

RPR (Rapid plasma reagin)

can also send VDRL

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2
Q

Which protein is a marker of rapid neurodegeneration ?

A

14-3-3

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3
Q

What is the difference between PrP and PrP^sc ?

A

PrP is the normal prion protein found in the body. It forms alpha helical configurations and is soluble.

PrP^sc is the pathological prion protein seen in prion diseases. It forms beta pleated sheets which are insoluble and deposit in the brain. PrP^sc acts as a template causing rapid conversion of normal PrP to PrP^sc

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4
Q

Name a sporadic prion disease ?

A

Creutzfeldt-Jakob disease (CJD)

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5
Q

Name an acquired Prion disease ?

A

Kuru
Variant CJD
Iatrogenic CJD

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6
Q

Name a genetically acquired prion disease ?

A

Gerstmann-straussler-sheinker syndrome (GSSS)

Familial fatal insomnia

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7
Q

List 4 signs of CJD ?

A

Dementia
Myoclonus
Cortical blindness
Akinetic mutism (loses ability to speak and mobilise)

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8
Q

Which EEG finding which may suggest sporadic CJD ?

A

Periodic triphasic complexes

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9
Q

What is the characteristic histological finding in CJD ?

A

Spongiform vacuolisation

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10
Q

Which symptoms are seen in variant CJD that are not seen in sporadic CJD ?

A

Psychiatric signs: hallucinations, dysphoria, anxiety

Peripheral sensory changes

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11
Q

What is the characteristic MRI sign for variant CJD ?

A

Pulvinar sign (high signal in the putamen)

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12
Q

What is the diagnostic test for variant CJD ?

A

Tonsillar biopsy

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13
Q

List features of Sporadic CJD ?

A

4LAMB

  • 4 months to live after onset of rapidly deteriorating dementia
  • Lower motor neurone signs
  • Akinetic mutism
  • Myoclonus
  • Cortical blindness
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