Imagerie de la maladie de Creutzfeldt-Jakob

Creutzfeldt-Jakob disease is a fatal neurodegenerative disease characterized by an abnormal accumulation of human prion protein PrPSc. It causes a rapidly progressive dementia syndrome. The imaging work-up is based on MRI which often discloses the typical disease anomaly seen as high signal intensity in the basal ganglia and cerebral cortex on Flair sequences and/or more readily on diffusion weighted sequences. This work recalls our fundamental knowledge concerning this disease and presents MRI data from 7 cases and a review of the literature.