More information on CJD
Transmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative disorders caused by the change in conformation of a normal cellular protein. Disease is caused by the prion protein (PrP) which converts from the normal cellular protein, PrPC to the disease-associated scrapie conformation PrPSc.
TSEs belong to a larger family of degenerative disorders caused by protein misfolding and aggregation that include Alzheimer’s disease, Parkinson’s disease and type II diabetes. TSEs differ from other misfolding disorders as they can transmit disease within and between species.
In humans the most common form of TSE disease is Creutzfeldt Jakob disease (CJD) which typically occurs sporadically. In 1996 a new form of CJD – unusually affecting young people – became known as vCJD.
The appearance of vCJD followed the bovine spongiform encephalopathy epidemic in the United Kingdom which peaked in the mid to late 1980s. Transmission of BSE to humans was later confirmed when it was demonstrated that vCJD and BSE had similar molecular features.
Without a reliable test to identify that people are carrying the disease the UK adopted several control measures to reduce the chance of human-to-human disease transmission through contaminated surgical instruments or blood transfusion.
Three of the 177 clinical cases of vCJD are thought to have resulted from contaminated blood products before the control measures were introduced.
Identifying people incubating vCJD remains the most effective way to reduce disease transmission. There has been significant effort put into developing such a test but a major obstacle has been the lack of relevant samples for evaluating assay suitability.