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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
Neuro

Abstract

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.

Keywords

Creutzfeldt-Jakob disease MRI Diagnostic criteria Diffusion Dementia Prion 

Abbreviations

BSE

bovine spongiform encephalopathy

DW

diffusion weighted

EEG

electroencephalogram

FFI

fatal familial insomnia

FLAIR

fluid attenuated inversion recovery

PD

proton density

PSWCs

periodic sharp wave complexes

sCJD

sporadic Creutzfeldt-Jakob disease

SFI

sporadic fatal insomnia

TSE

transmissible spongiform encephalopathy

vCJD

variant Creutzfeldt-Jakob disease

Notes

Acknowledgements

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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