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From: TSS ()
##################### Bovine Spongiform Encephalopathy ##################### Tests show another woman died from Creutzfeldt-Jakob disease TWIN FALLS -- Final test results on brain tissue have confirmed another Idaho woman died from the classic form of Creutzfeldt-Jakob disease and not the variant form related to mad cow disease, according to the Idaho Department of Health and Welfare. Since January, the Idaho Department of Health and Welfare has received nine reports of people -- seven women and two men -- diagnosed with Creutzfeldt-Jakob disease -- or CJD -- a fatal brain-wasting disease carried by prions, an abnormal form of protein in the bloodstream. Prions cause folding of normal protein in the brain, leading to brain damage. Symptoms include dementia and other neurological signs. Its victims usually die within four or five months after onset of the disease, according to the Centers for Disease Control and Prevention. The cases include four women from Twin Falls County, a woman from Minidoka County, a woman from Benewah County in northern Idaho, a woman from Bear Lake County in the southern corner of Idaho on the Utah border, a man from Elmore County and a man from Caribou County in southeastern Idaho. Of the nine people in Idaho who have died, five had autopsies and their brain tissue was sent to the National Prion Disease Pathology Surveillance Center at Cleveland's Case Western Reserve University. Of those five, three women -- two women from Twin Falls County and the woman from Benewah County -- tested positive for a prion disease, and now final results on two of them showed they died of classic CJD and not the variant form that is caused by eating meat from a cow with bovine spongiform encephalopathy, commonly known as mad cow disease. Health officials are still waiting for the final results on the third woman, said Tom Shanahan, spokesman for the Idaho Department of Health and Welfare on Wednesday. Two people, including the Elmore County man and a Twin Falls woman, tested negative for a prion disease. Autopsies were not performed on the other four suspected CJD victims. However, a CDC neurologist is reviewing their medical records, Shanahan said. The number of cases is highly unusual. Normally, there is one case of CJD per million people a year. Between 1984 and 2004, Idaho averaged 1.2 cases a year, Shanahan said. He said there was one year during that period when Idaho had three cases. Because of their ages -- all of the victims except one were over the age of 60 -- health officials suspect they died of Classic CJD, and not the variant. However, the only way to confirm CJD is by testing brain tissue, according to the National Prion Disease Pathology Surveillance Center. Shanahan said there have been no additional reports of CJD diagnoses in Idaho this year. Times-News writer Sandy Miller can be reached at 735-3264 or by e-mail at smiller@magicvalley.com. http://www.magicvalley.com/articles/2005/12/01/news_localstate/news_local_state.2.txt #################### https://lists.aegee.org/bse-l.html #################### >>> Of those five, three women -- two women from Twin Falls County and the woman from Benewah County -- tested positive for a prion disease, and now final results on two of them showed they died of classic CJD and not the variant form that is caused by eating meat from a cow with bovine spongiform encephalopathy, commonly known as mad cow disease. <<< Creutzfeldt-Jakob disease Magdalini Polymenidou, Katharina Stoeck, Markus Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi Summary Background The molecular typing of sporadic Creutzfeldt-Jakob disease (CJD) is based on the size and glycoform ratio of protease-resistant prion protein (PrPSc), and on PRNP haplotype. On digestion with proteinase K, type 1 and type 2 PrPSc display unglycosylated core fragments of 21 kDa and 19 kDa, resulting from cleavage around amino acids 82 and 97, respectively. Methods We generated anti-PrP monoclonal antibodies to epitopes immediately preceding the differential proteinase K cleavage sites. These antibodies, which were designated POM2 and POM12, recognise type 1, but not type 2, PrPSc. Findings We studied 114 brain samples from 70 patients with sporadic CJD and three patients with variant CJD. Every patient classified as CJD type 2, and all variant CJD patients, showed POM2/POM12 reactivity in the cerebellum and other PrPSc-rich brain areas, with a typical PrPSc type 1 migration pattern. Interpretation The regular coexistence of multiple PrPSc types in patients with CJD casts doubts on the validity of electrophoretic PrPSc mobilities as surrogates for prion strains, and questions the rational basis of current CJD classifications. into debate and introduce interesting questions about human CJD types. For example, do human prion types exist in a dynamic equilibrium in the brains of affected individuals? Do they coexist in most or even all CJD cases? Is the biochemically identified PrPSc type simply the dominant type, and not the only PrPSc species?
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