Teaching File - Creutzfeldt-Jakob Disease (CJD)

Axial DWI MRI images of a 68-year-old man who presented with rapidly progressive dementia showed high signal intensity in the regions of cerebral cortex of the frontal and temporal lobes (arrowheads) and deep nuclei of basal ganglia (arrows). More subtle changes are also noted in the thalami. Findings are consistent with Creutzfeldt-Jakob disease.

Creutzfeldt-Jakob Disease (CJD)

* Progressive, fatal spongioform encephalopathy
* Transmissable disease
* Clinical: rapidly progressive dementia (<2>

MRI of CJD

* Distribution of abnormality: usually symmetric, deep gray nuclei (caudate head, putamen, thalamus), cerebral cortex
* Characters: high signal on T2 and FLAIR, restricted diffusion on DWI
* DWI may be the most sensitive imaging method for the early clinical diagnosis of CJD

Reference:
Shiga Y, et al. Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology 2004;63:443-449.