Masters, Colin L. National Creutzfeldt-Jakob Disease Registry, Department of Pathology, University of Melbourne, Melbourne, Australia.
Collins, Steven J. The Australian National Creutzfeldt-Jakob Disease (CJD) Registry and Department of Pathology, University of Melbourne, Melbourne, Australia.
Last reviewed:April 2018
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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: Animal prions; Brain; Nervous system disorders; Protein; Scrapie
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