Microbiology 5: Prion Diseases Flashcards
Which test detects active syphilis ?
RPR (Rapid plasma reagin)
can also send VDRL
Which protein is a marker of rapid neurodegeneration ?
14-3-3
What is the difference between PrP and PrP^sc ?
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
Name a sporadic prion disease ?
Creutzfeldt-Jakob disease (CJD)
Name an acquired Prion disease ?
Kuru
Variant CJD
Iatrogenic CJD
Name a genetically acquired prion disease ?
Gerstmann-straussler-sheinker syndrome (GSSS)
Familial fatal insomnia
List 4 signs of CJD ?
Dementia
Myoclonus
Cortical blindness
Akinetic mutism (loses ability to speak and mobilise)
Which EEG finding which may suggest sporadic CJD ?
Periodic triphasic complexes
What is the characteristic histological finding in CJD ?
Spongiform vacuolisation
Which symptoms are seen in variant CJD that are not seen in sporadic CJD ?
Psychiatric signs: hallucinations, dysphoria, anxiety
Peripheral sensory changes
What is the characteristic MRI sign for variant CJD ?
Pulvinar sign (high signal in the putamen)
What is the diagnostic test for variant CJD ?
Tonsillar biopsy
List features of Sporadic CJD ?
4LAMB
- 4 months to live after onset of rapidly deteriorating dementia
- Lower motor neurone signs
- Akinetic mutism
- Myoclonus
- Cortical blindness
