Diffusion-weighted magnetic resonance imaging in probable Creutzfeldt-Jakob disease - A clinical-anatomic correlation

Background: Creutzfeldt-Jakob disease (CJD) is a rare transmissible disease that typically causes a rapidly progressive dementia and leads to death in less than 1 year. Although a few anecdotal reports suggest that diffusion- weighted magnetic resonance imaging may help substantiate premortem diagnos is of CJD, detailed correlation between radiographic data and clinical, ele ctrophysiologic, and metabolic parameters is not available. Methods: Signal abnormalities on diffusion-weighted images in 3 consecutive patients with probable CJD were correlated with psychometric features, ele ctroencephalographic findings, and functional images with either positron e mission tomography or single photon emission computed tomography. Results: Focality of abnormalities on diffusion-weighted image, not apparen t on routine magnetic resonance images, correlated closely with clinical ma nifestations of CJD. The topographic distribution of signal abnormality on diffusion-weighted image corresponded with abnormal metabolism or perfusion on positron emission and single photon emission computed tomographic scans . In 2 cases, the laterality of diffusion abnormalities correlated with per iodic sharp wave activity on electroencephalograms. Conclusion: These findings extend previous observations that suggested a di agnostic and localizing utility of diffusion-weighted imaging in CJD.