Bovine Spongiform Encephalopathy
Bovine Spongiform Encephalopathy (BSE or “Mad Cow Disease”) is a type of transmissible spongiform encephalopathy (TSE) that affects cattle. TSEs are progressive and fatal neurodegenerative diseases.
There are multiple TSEs which affect different species of animals including scrapie in sheep, feline spongiform encephalopathy (FSE), chronic wasting disease (CWD) in deer and elk, and a spongiform encephalopathy of exotic ruminants. BSE has also been linked to a variant of Creutzfeldt-Jakob disease (CJD) in humans.
The abnormal prion protein, which is believed to be the cause of BSE, is resistant to enzymatic breakdown and most disinfection treatments. Classical and atypical are the two forms of BSE. The incubation period for classical BSE for time of infection until the onset of clinical signs averages three to six years. The primary source of infection for classical BSE is feed contaminated with the infectious prion agent, such as meat-and-bone meal containing protein derived from rendered infected cattle. Regulations from the FDA have prohibited the inclusion of mammalian protein in feed for cattle and other ruminants and have also prohibited high risk tissue materials in all animal feed. Atypical BSE occurs in older cattle, usually 8 years of age or greater, and does not appear to be associated with contaminated feed.
BSE presents a public health concern because occurrences of variant CJD in humans have been linked to the consumption of food containing ingredients derived from BSE-infected cattle.
BSE is an exotic disease and authorities must be notified immediately of any suspicious cases.
What can I expect during an inspection? Take a look at the BSE Inspection Checklist
Visit the FDA and USDA websites for more BSE information.