MRI in the diagnosis of sporadic Creutzfeldt-Jakob disease: a study on inter-observer agreement.

摘要: According to the current WHO criteria, technical investigations included in clinical diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) are electroencephalogram (EEG) and CSF-analysis for 14-3-3 proteins. MRI is not a criterion sCJD, although typical changes have been described. We investigated reliability sCJD diagnosis, evaluated sequences compared with EEG 14-3-3. This study includes 193 consecutive suspected patients who had referred German CJD Surveillance Unit from 2001 2003. Three observers independently analysed scans, blinded data. was rated as 'typical sCJD' if increased signal intensity detected caudate nucleus putamen. 442 scans [184 T2-weighted sequences, 132 fluid attenuated inversion recovery (FLAIR) 75 diffusion-weighted 51 proton-density weighted sequences]. Inter-observer agreement 123 or 63.7% (overall kappa = 0.53). Sensitivity clinically probable autopsy-proven 59.7% Observer 1, 58.3% 2 70.8% 3; specificity high (84.2, 89.5 81.6%, respectively). Diffusion-weighted best showed pathologic changes, followed by FLAIR. Periodic sharp slow wave complexes were 32% (sensitivity), proteins CSF elevated 91%. conclude that detection hyperintense basal ganglia helps improve therefore, we propose incorporate diagnostic criteria sCJD.