Neuroimaging of prion diseases

The role of neuroimaging in prion disease has greatly evolved over the past decade, in which brain magnetic resonance imaging (MRI), particularly diffusion imaging, has a very high diagnostic utility. The non-invasive nature of MRI, the development of new magnetic resonance sequences, and the availability of clinical and neuropathological correlation have led to a more prominent role of neuroimaging in the diagnosis, and possibly an understanding of these enigmatic disorders. This chapter explores the key characteristics of MRI findings in the various types and subtypes of prion disease, and also offers an insight into the differential diagnosis and radiological mimickers of this ever-challenging disease.