Correlation of diffusion-weighted magnetic resonance imaging with neuropathology in Creutzfeldt-Jakob disease

Background: Although the diagnosis of Creutzfeld-Jakob disease (CJD) is straightforward in fully developed cases, a definitive diagnosis can be difficult early in the course of the illness. T2-weighted magnetic resonance imaging (MRI) signal abnormalities, and recently, diffusion-weighted MRI abnormalities, have been described in patients with CJD, suggesting the utility of MRI in the early recognition of CJD. Objective: To correlate diffusion-weighted MRI signal abnormalities with neuropathologic changes in CJD. Materials and Methods: Diffusion-weighted MRI and neuropathologic changes of 2 patients with autopsyproven CJD were examined in a blinded fashion by a neuroradiologist and a neuropathologist. Results: Areas of bright signal on diffusion-weighted MRI correlated with a higher degree of spongiform changes. Conclusion: Diffusion-weighted MRI in CJD demonstrates specific-signal abnormalities that correlate well with areas of the most severe and characteristic neuropathologic changes.