A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83)

Prion disease represents a group of conditions that affect the nervous system in humans and animals. In people, these conditions impair brain function, causing changes in memory, personality, and behavior; a decline in intellectual function (dementia); and abnormal movements, particularly difficulty with coordinating movements (ataxia). The signs and symptoms of prion disease typically begin in adulthood and worsen with time, leading to death within a few months to several years.

(transmissible spongiform encephalopathy) A transmissible, infectious disease that is caused by a protein that is able to induce abnormal folding of normal cellular proteins, leading to characteristic spongiform brain changes, which are associated with neuronal loss without an inflammatory response. Such disorders have typically long incubation periods, but are then generally rapidly progressive and are uniformly fatal.

(transmissible spongiform encephalopathy(tse)) A group of rare degenerative brain disorders associated with orions for example Creutzfeldt-Jakob disease (CJD) or Bovine Spongiform Encephalopathy (BSE).

Prion diseases (or transmissible spongiform encephalopathies) are a group of uniformly fatal neurodegenerative diseases characterized by progressive dementia and motor dysfunction. These diseases occur in spontaneous, genetic, and acquired forms. Patients commonly present with behavioral or personality changes, myoclonus, visual...