Prion disease

Article By:

Masters, Colin L. Australian National Creutzfeldt-Jakob Disease Registry, and Department of Pathology, University of Melbourne, Melbourne, Australia.

Collins, Steven J. Australian National Creutzfeldt-Jakob Disease Registry, and Department of Pathology, University of Melbourne, Melbourne, Australia.

Last reviewed:June 2021

DOI:https://doi.org/10.1036/1097-8542.757460

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• Prion disease, published June 2014:Download PDF Get Adobe Acrobat Reader

Any of the transmissible spongiform encephalopathies in humans and other animals. Prion diseases comprise a family of progressive neurodegenerative conditions. These rare and fatal brain disorders are caused by misfolding and aggregation of prion proteins and are characterized at a neuropathological level by vacuolation of the brain's gray matter (spongiform change) and widespread loss of nerve cells. There are several forms of prion disease in humans and other animals. Scrapie is the most common form seen in nonhuman animals, whereas the most prevalent form in humans is Creutzfeldt-Jakob disease (Fig. 1). See also: Brain; Degenerative neural diseases; Nervous system disorders; Prion; Protein; Protein folding; Scrapie

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