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Microbiology · Virology: prion disease
Sample itemA 62-year-old woman has rapidly progressive dementia and startle myoclonus over 3 months. EEG shows periodic sharp wave complexes, and brain pathology shows spongiform change. Which property of the causative agent is correct?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. A misfolded protein that is resistant to proteinase K and to routine sterilisation
Creutzfeldt-Jakob disease is a prion disease in which the normal cellular protein PrPC is converted into a beta-sheet-rich form, PrPSc, that resists proteases and conventional sterilisation. There is no nucleic acid and no inflammatory response, and CSF 14-3-3 protein may be raised. Special decontamination is needed for instruments.
Option by option
A. An enveloped RNA virus inactivated by formalin
The agent has no RNA genome and is not inactivated by formalin.
B. A DNA virus that causes latent infection in neurons
No DNA virus is responsible, as the agent lacks nucleic acid.
C. A misfolded protein that is resistant to proteinase K and to routine sterilisation (correct)
Correct. The infectious agent is a protease-resistant misfolded protein.
D. A cell-wall-deficient bacterium sensitive to doxycycline
The agent is not a bacterium, so antibiotics are ineffective.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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