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In prion disease, classically, the :

  • A.Liver becomes excessively enlarged
  • B.Liver becomes sponge like
  • C.Brain ventricles are squeezed
  • D.Brain becomes sponge like

Answer: D

In prion disease the brain becomes sponge like.

Prion diseases such as Creutzfeldt-Jakob disease, kuru, bovine spongiform encephalopathy and scrapie are caused by misfolded prion protein (PrPSc) that converts normal PrP into the abnormal form. Neurons die and vacuoles form in the grey matter, giving the brain a spongy appearance, hence the name transmissible spongiform encephalopathies.

The damage is in the central nervous system, not the liver, so the two liver options are wrong. Squeezed ventricles are not the characteristic change; brain atrophy, if anything, enlarges them.

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