Variant Creutzfeldt–Jakob disease

Variant Creutzfeldt–Jakob disease is a rare, fatal prion disease linked chiefly to exposure to bovine spongiform encephalopathy prions, usually through contaminated beef.

Prion: An infectious, misfolded protein that propagates by inducing normal versions of the same protein to misfold. Variant CJD is caused by prions that convert the brain’s normal prion protein into an abnormal form.
United Kingdom BSE outbreak: The large cattle epidemic of bovine spongiform encephalopathy that peaked in the United Kingdom in the late 1980s and early 1990s. The outbreak preceded the first recognized variant CJD cases and supplied the suspected exposure source.
Specified risk material: Animal tissues judged likely to contain high levels of transmissible spongiform encephalopathy infectivity and removed from the food chain. Removing these tissues from cattle products reduced potential exposure to BSE prions.
Iatrogenic Creutzfeldt–Jakob disease: Creutzfeldt–Jakob disease acquired through medical exposure to infectious prions, such as contaminated instruments or biological products. Both are acquired prion diseases, but variant CJD is linked to BSE rather than medical procedures.
Bovine spongiform encephalopathy: A fatal prion disease of cattle, also called BSE or mad cow disease. The cattle disease’s prions are linked to the human infections that define variant CJD.
Variant Creutzfeldt–Jakob disease in the United Kingdom: The cluster of variant CJD cases first recognized in the United Kingdom in 1996. These early cases established the human disease’s clinical and pathological pattern.
BSE control measures: Policies used to limit bovine spongiform encephalopathy, including feed restrictions, surveillance, and removal of infected cattle. These interventions targeted the animal epidemic associated with variant CJD.
Familial Creutzfeldt–Jakob disease: Inherited Creutzfeldt–Jakob disease caused by pathogenic variants in the PRNP gene. Its inherited origin contrasts with variant CJD’s association with an external prion exposure.
PRNP: The human gene that encodes prion protein. A PRNP codon 129 variant strongly influences susceptibility and disease incubation.
Blood transfusion transmission of variant CJD: The documented transmission of variant CJD through transfused blood from infected donors. Transfusion cases showed that infectious prions could circulate before symptoms and spread through blood.

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