Brain Advance Access originally published online on June 15, 2005
Brain 2005 128(9):2026-2033; doi:10.1093/brain/awh575
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MRI in the diagnosis of sporadic Creutzfeldt–Jakob disease: a study on inter-observer agreement
1 Department of Radiology and 2 Institute for Medical Biometry, Informatics and Epidemiology, University of Bonn, 3 Department of Neuroradiology and 4 Department of Neurology, Georg-August University, Göttingen and 5 Institute for Neuropathology, Ludwig-Maximilians University, München, Germany
Correspondence to: Dr Henriette J. Tschampa, Department of Radiology, University of Bonn, Sigmund-Freud-Strasse 25, D-53105 Bonn, Germany E-mail: Henriette.Tschampa{at}ukb.uni-bonn.de
According to the current WHO criteria, technical investigations included in the clinical diagnosis of sporadic Creutzfeldt–Jakob disease (sCJD) are electroencephalogram (EEG) and CSF-analysis for 14-3-3 proteins. MRI is not a criterion for the diagnosis of sCJD, although typical changes have been described. We investigated the reliability of MRI in the sCJD diagnosis, evaluated MRI sequences and compared MRI with EEG and 14-3-3. This study includes 193 consecutive suspected sCJD patients who had been referred to the German CJD Surveillance Unit from 2001 to 2003. Three observers independently analysed MRI scans, blinded to clinical data. MRI was rated as ‘typical for sCJD’ if increased signal intensity was detected in the caudate nucleus and putamen. We analysed 442 MRI scans [184 T2-weighted sequences, 132 fluid attenuated inversion recovery (FLAIR) sequences, 75 diffusion-weighted sequences and 51 proton-density weighted sequences]. Inter-observer agreement was 123 of 193 patients or 63.7% (overall 
= 0.53). Sensitivity of MRI in clinically probable or autopsy-proven sCJD was 59.7% for Observer 1, 58.3% for Observer 2 and 70.8% for Observer 3; specificity was high (84.2, 89.5 and 81.6%, respectively). Diffusion-weighted sequences best showed the pathologic changes, followed by FLAIR. Periodic sharp and slow wave complexes were detected in the EEG in 32% (sensitivity), the 14-3-3 proteins in CSF were elevated in 91%. We conclude that the detection of hyperintense basal ganglia in MRI helps to improve the clinical diagnosis, and therefore, we propose to incorporate MRI in the diagnostic criteria for sCJD.
Key Words: Creutzfeldt–Jakob disease; dementia; diagnostic criteria; diffusion; MRI
Abbreviations: CJD = Creutzfeldt–Jakob disease; DW = diffusion-weighted; EEG = electroencephalogram; FLAIR = fluid attenuated inversion recovery; PD = proton-density; PSWCs = periodic sharp wave complexes; sCJD = sporadic CJD; vCJD = variant of CJD
Received December 23, 2004. Revised May 15, 2005. Accepted May 19, 2005.
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