Imaging of prion diseases

Prion diseases are caused by self-replicating proteins that induce lethal neurodegenerative disorders. In the last decade, the understanding of the different clinical, pathological, and neuroimaging phenotypes of this group of disorders has evolved paralleling the advances in prion molecular biology. From an imaging standpoint, the implementation of diffusion-weighted imaging in routine practice has markedly facilitated the detection of prion diseases, especially Creutzfeldt-Jakob. Less frequent prion-related disorders, including genetic diseases, may also benefit from progresses in the field of quantitative diffusion-weighted imaging, MR spectroscopy or molecular imaging. Herein, we present a review of the neuroimaging features of the prion disorders known to affect humans emphasizing the important contribution of MRI in the diagnosis of this group of disorders.