Serial diffusion-weighted MRI of Creutzfeldt-Jakob disease

The objective of our study was to evaluate the clinical usefulness of MRI findings, including diffusion-weighted imaging, in relation to the clinical signs and symptoms of Creutzfeldt-Jakob disease (CJD).

We reviewed nine cases of CJD in which MRI was performed from the early to terminal phase of the disease. MRI findings were correlated before (early phase) and after (intermediate phase) the onset of the characteristic clinical findings of myoclonus and periodic synchronous discharges on electroencephalograms. The chronologic changes in imaging findings were followed from the akinetic mutism to the terminal phase of the disease (terminal phase). T2-weighted images had been obtained in all the patients, and diffusion-weighted images and FLAIR images had been obtained in six patients. We evaluated the images for the presence and location of abnormal signal intensities.

During the early phase, the T2-weighted images showed no abnormal findings. The diffusion-weighted images, however, revealed abnormal high signal intensities in the cortex in all patients and in the basal ganglia in five patients. In two cases, there were abnormal signals on FLAIR images that corresponded to diffusion-weighted imaging abnormalities. During the intermediate phase, the area of the high signal intensities on the diffusion-weighted images had expanded and progressive cerebral atrophy had become apparent. During the terminal phase, abnormal high signal intensities in the cerebral cortex and basal ganglia on the diffusion-weighted images in one patient disappeared.

Diffusion-weighted imaging is extremely useful in detecting CJD during the very early phase-even before the onset of characteristic clinical findings.