This new disease could eventually kill tens of thousands of people in Britain or, given the many uncertainties about prion diseases and long incubation periods, it may eventually die out, he said. Unlike the mis folded prions in sporadic C.J.D., which are found exclusively in brain tissue, new variant prions are found in the brain, tonsils, appendix, spleen and lymph nodes. One of the biggest mysteries is why the new variant form of the disease attacks young people, Dr. Will said. The British victims were 13 to 43 years old, whereas sporadic C.J.D. affects much older people. After meat is removed from the carcass, he said, it is compressed with a machine not unlike an automobile crusher used in wrecking yards.
Bone comes out one end and a meat-like goo at the other end.
It was fed to children all over the country, but the link between eating the food and getting the disease has not been proved. No cases of new variant C.J.D. have been found in the United States, said Dr. Lawrence Schonberger, an assistant director for public health at the Centers for Disease Control and Prevention in Atlanta. There have been a few instances of deaths of younger Americans from C.J.D., but tests of their brain tissue showed that they had sporadic forms of the disease, he said. But according to Dr. Michael Hansen, a research associate at Consumers Union in Yonkers, deer and elk in several western states are experiencing a growing epidemic of a prion disease called chronic wasting disease. Some experts fear that many hunters and their families have almost certainly been exposed to the misfolded proteins from eating infected deer and elk meat, Dr. Hansen said.