European Radiology

, Volume 17, Issue 5, pp 1200–1211 | Cite as

Radiological assessment of Creutzfeldt-Jakob disease

  • Henriette J. TschampaEmail author
  • Inga Zerr
  • Horst Urbach


Creutzfeldt-Jakob disease is a rare fatal neurodegenerative disorder, characterized by rapidly progressive dementia and neurological signs. There is a need for early and accurate clinical diagnosis in order to exclude any treatable disorder. Additionally, it is of public interest to differentiate the sporadic form of the disease from the variant CJD type (vCJD), which is probably transmitted from cattle infected with bovine spongiform encephalopathy (BSE). High signal in the striatum on T2-weighted, FLAIR and diffusion weighted (DW) MRI as well as cortical high signal in FLAIR and DW MRI are the classical findings in sCJD. The “pulvinar sign”, defined as high signal in the pulvinar thalami that is brighter than potential additional high signal in the basal ganglia, is considered pathognomonic for vCJD.


Creutzfeldt-Jakob disease MRI Diagnostic criteria Diffusion Dementia Prion 



bovine spongiform encephalopathy


diffusion weighted




fatal familial insomnia


fluid attenuated inversion recovery


proton density


periodic sharp wave complexes


sporadic Creutzfeldt-Jakob disease


sporadic fatal insomnia


transmissible spongiform encephalopathy


variant Creutzfeldt-Jakob disease



We thank Hanno Schimikowski for his help with figure editing and Carsten Krautmacher for critical reading of the manuscript.


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Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Henriette J. Tschampa
    • 1
    Email author
  • Inga Zerr
    • 2
  • Horst Urbach
    • 1
  1. 1.Department of RadiologyUniversity of BonnBonnGermany
  2. 2.National Reference Center for TSE Surveillance at the Department of NeurologyUniversity of GöttingenGöttingenGermany

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