Mad cow disease--the OR connection

Creutzfeldt-Jakob disease (CJD) is one of the transmissible spongiform encephalopathies, a group of fatal, neurodegenerative disorders affecting both humans and animals. The causative agent is the prion, which is still being researched and is controversial. In the 1980s, bovine spongiform encephalopathy brought much media attention to these diseases. Bovine spongiform encephalopathy is the result of faulty industrial practices that produced cattle feed contaminated by prions. In the 1990s, a new variant of CJD (i.e., nvCJD) appeared in Britain. Researchers believe that nvCJD was passed to humans through oral consumption of contaminated beef. This article describes the history, causative agent, mode of transmission, clinical features and course, diagnosis, treatment, and decontamination and sterilization guidelines for this baffling disease.