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Creutzfeld Jakobs disease
A protein, a so-called ”prion”, causes Creutzfeld Jacobs Disease, CJD.
Infection
CJD occur spontaneously mostly in people over 60 years. Variant CJD, vCJD, occur in younger people and is transmitted by consumption of nerve tissue from animals infected with the prion.
There is so far no indication that vCJD is transmitted through pure meat and blood transfusion, but is it not entirely excluded.
Vaccination
There is no vaccine.
Symptoms
Rapidly progressing dementia with loss of coordination of movements and paresis.
Diagnosis
Clinical examination, brain scans and brain biopsy.
Treatment
There is no treatment.
Prevention
vCJD is transmitted through cows meat, and separating nerve tissue from the meat proper, which is now standard procedure, can reduce the risk of infection.
More about vCJD
Everybody has the gene coding for the protein affected by CJD.
In CJD and vCJD the normal protein is attacked by the CJD protein and transformed to the abnormal protein.
Boiling does not destroy the CJD protein.
The CJD protein can be found in different tissue like brain, nerves, liver and spleen.
vCJD in the United Kingdom
In the United Kingdom experienced an outbreak of “Mad Cows Disease” also called Bovine Spongiform Encephalitis or BSE.
BSE is caused by a prion very similar to vCJD, and it is almost certain that it was the BSE agent from cattle that has caused the vCJD outbreak in humans.
BSE is no longer a problem in cattle in the UK, but cases of vCJD are still seen, and the incubation period has not been determined.
By the June 30th 2002 the total number diagnosed was 124 of which 115 has died.
Edited 3 November 2003
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