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. J. Magn. Reson. Imaging 2012;35:998‐1012. © 2012 Wiley Periodicals, Inc.

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AMK and RKK contributed to the drafting and revision of the manuscript. CJW and RW provided neuroimaging and expert interpretation of the findings. KLT provided expert revision of the manuscript.

In this report, an 8-month-old calf (crossbred, Holstein × Japanese Black) developed fever and accompanied abomasum displacement. Blood chemical test showed remarkably high values of white blood cell count and heteromorphic lymphocytes. In pathological appraisal, enlarged splenomegaly and swelling of the lymph nodes were observed. Histopathological examination revealed invasion of tumor cells derived from B1 cells into systemic lymph nodes, liver and spleen. The provirus loads of bovine leukemia virus (BLV) was 1,439 copies per 10 ng DNA by using real time PCR. In conclusion, this case was diagnosed as bovine leukemia caused by BLV infection with a huge splenomegaly.