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While highly infectious tissues and fluids are found in the central nervous system, in vCJD, wider tissue distribution of [PrP.sup.Sc] has been observed including evidence of [PrP.sup.Sc] in the appendix, tonsil, and spleen.
The neuropathological features of vCJD are characterized by the presence of abundant florid PrP plaques and the propagation of type 4 disease-related [PrP.sup.Sc] in the brain (1,11).
VCJD is transmitted by a protein called prion which builds up in the brain.
Variant CJD (vCJD) has been confirmed as being caused by the same prion strain as bovine spongiform encephalopathy (BSE) found in cattle.
Bite: Gummer The link between eating beef and vCJD was confirmed six years later.
VCJD progressively causes the brain to become riddled with holes, leading to mental problems, loss of body function, and eventual death.
One test from researchers at the National Institute of Allergy and Infectious Diseases uses an antibody-based technique to isolate abnormal prion proteins and detect them at levels 10,000 times more sensitive than in previous tests for detecting vCJD. Another test from researchers at University College London Institute of Neurology and the National Hospital for Neurology and Neurosurgery in London was able to detect vCJD prions in the presence of the normal prion protein with a sensitivity of 71% and specificity of 100%.
However, they pointed out that the test has not been studied in plasma or CSF from vCJD patients and that "further studies will be required to assess the diagnostic utility of eQuIC based on the detection of vCJD seeding activity that is endogenous to these and other specimens."
Between 1990 and the start of May 2011 there have been 171 confirmed and probable vCJD deaths reported to the National Creutzfeldt-Jakob Disease Research and Surveillance Unit in Edinburgh.
VCJD causes the brain to become full of holes, leading to mental problems, loss of body function and death.
Lawyers representing the families, who have all lost loved ones as a result of vCJD, said some found the claims process extremely painful and highly distressing.
Whether or not they develop the terrible symptoms of vCJD and die from the condition would depend on how long it takes to develop.
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