Definitions

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BSE and CJD -  Definitions  


  
 

Bovine Spongiform Encephalopathy (BSE) or Mad Cow Disease: This is a brain disease in cattle.

Creutzfeldt–Jakob Disease or CJD: This is the brain disease in humans.

CJD can be variant CJD. This was linked with BSE in the U.K. in 1996. See the video for the announcement by the Health Minister in March 1996.

CJD can also be sporadic or classical. This appears to occur in a random manner.

The incidence of sporadic CJD was 1 in a million or less.

It is possible that the increased incidence of sporadic CJD is linked to BSE.

Chronic Wasting Disease (CWD): This is the brain disease in deer, elk and moose.

These diseases are TSE’s: Transmissible Spongiform Encephalopathies. This is a general term which covers a range of brain diseases in animals and humans. TSE’s are prion diseases. Prions are infectious proteins. These infectious proteins cause disease by crowding in a ‘dense cluster’ outside of brain cells.

► Variant CJD could be caused by BSE through beef consumption. BSE has been linked to Mechanically Recovered Meat (MRM). This is a meat product which is made by forcing beef though a machine to separate any meat from the bone. It is also known as mechanically separated meat and was banned in 1995 according to the U.K. Food Standards Agency.

Vaccines may also be relevant to some cases of vCJD in the United Kingdom.

Kuru is a Transmissible Spongiform Encephalopathy (TSE) caused by prions found in humans.

It was found in a tribe in Papua New Guinea and as a human brain disease it has helped explain variant CJD. In particular, the disease affected people decades after transmission of the disease. This pattern may repeat itself with vCJD.

The origin of the disease was due to the tribe's consumption of a deceased individual's brain. Such brains were contaminated from individuals who had sporadic CJD.

Ingestion of prion particles can cause the disease. It is possible that the disease was also transmitted through open sores and cuts on hands as members of the tribe dealt with deceased relatives.

Scrapie is a TSE found in sheep and goats. It was found in the 18th century and does not seem to be linked to humans and variant CJD.

Food Safety and Food Hygiene

Food safety can be about the cooking, handling, preparation and storage of food.

General Terms

Risk is about the probability of a harmful occurrence, such as a disease.

Therefore, safety can be seen as the opposite. It is about the probability of not suffering from a harmful outcome.

Hazard is the potential to cause harm.

Key Issues

How many people have died from CJD?  The measurement of CJD deaths has been contested and may increase in future years.

One of the concerns, is whether CJD was avoidable.  If this was the case, then it makes the hazard more painful; with regret from knowing that the disease could have been avoided.

Improved accountability of scientists, and greater public control over science, is needed so that greater care is taken.  This should avoid  similar problems in the future. 

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