Neurology: Case of the Month

Test Yourself — Patient 52

Creutzfeldt-Jakob Disease

1. What are the characteristic findings of sporadic CJD on MRI?
   • [ A ] Signal abnormalities on T1-weighted MR sequences.
   • [ B ] Signal abnormalities on T2-weighted MRI and hyperintensities on FLAIR.
   • [ C ] Signal abnormalities on T2-weighted MR imaging alone.
   • [ D ] Normal appearing MRI.
   • [ E ] None of the above.
2. What is the causative agent of CJD?
   • [ A ] The JC virus.
   • [ B ] The cause is unknown.
   • [ C ] Abnormally conformed prion protein acquired from infected sources.
   • [ D ] Abnormally conformed prion protein arising from somatic mutations in the prion protein (PRNP) gene, or from spontaneous misfolding of prion protein.
   • [ E ] Both C and D.
3. What are the possible EEG findings of CJD?
   • [ A ] Normal EEG.
   • [ B ] Slow background activity.
   • [ C ] Periodic complexes occurring every 0.5-2.0 seconds.
   • [ D ] All of the above.
   • [ E ] None of the above.
4. What is the likely cause of new variant CJD?
   • [ A ] Heterozygosity for polymorphisms in the prion protein (PRNP) gene.
   • [ B ] Infected dural or corneal grafts.
   • [ C ] Infected neurosurgical instruments or brain electrodes.
   • [ D ] Interspecies transmission of bovine spongiform encephalopathy.
   • [ E ] Spontaneous somatic mutations in the PRNP gene.
5. What is the treatment for CJD?
   • [ A ] Cadaveric human growth hormone.
   • [ B ] Palliative care only.
   • [ C ] Immunization with normal prion protein.
   • [ D ] Surgical removal of the accumulations of prion protein.
   • [ E ] Taking a bath with sodium hypochlorite.

 

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