There is no evidence of CWD/scrapie in people ? WRONG!!!

----- Original Message ----- From: Terry S. Singeltary Sr. To: dsapatkin@phillynews.com Cc: philly_feedback@knightridder.com Sent: Saturday, November 26, 2005 10:43 AM Subject: There is no evidence of CWD/scrapie in people ? WRONG!!! re-Nascent disease troubles hunters Known cases: There is no evidence of CWD in people ??? Related diseases: Scrapie has been found for hundreds of years in domestic goats and sheep, with no known human infections. Bovine spongiform encephalopathy (mad cow) can be passed to humans who eat infected beef; it is fatal. PLEASE, this is such BSe (bull shit encephalopathy). PLEASE, read the science. I urge you to read the data. you are just printing and rubber stamping the lies the feds have been telling us, while this agent continues to spread and expose millions via a multitude of proven routes and sources i.e. CWD and Scrapie, both transmit to primates and cattle. the myth of no humans ever contracting scrapie or CWD is an unproven myth, another pipedream. i lost my mother to the Heidenhain Variant of CJD (confirmed), and since then wasted 8 years of my life trying to get the truth out (mad cow disease has been here for decades, and that the UKBSEnvCJD only theory was total BSe. how could it be true when there are over 20 strains of sheep scrapie with new atypical strains showing up now? NOW, Aguzzi et al proves me right, and is what i have been saying all along, that the UKBSEnvCJD only theory was a pipe dream. BUT GWs BSEMRR policy (the legal trading of all human/animal TSEs globally), and (the ENHANCED BSE COVER-UP OF JUNE 2004), shows just how corrupt our gov is. all one has to do is look at the 7+ month delay in confirming the TEXAS MAD COW, and you must not forget the TEXAS stumbling and staggering mad cow that was completely cover up; http://www.fda.gov/bbs/topics/news/2004/NEW01061.html oops! or the mad cow feed TEXAS cattle feed on ; http://www.fda.gov/bbs/topics/NEWS/2001/NEW00752.html when in reality on the oral dose of BSE in primates--look at the table and you'll see that as little as 1 mg (or 0.001 gm) caused 7% (1 of 14) of the cows to come down with BSE ; Risk of oral infection with bovine spongiform encephalopathy agent in primates Corinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog, Franck Mouthon, Timm Konold, Frédéric Auvré, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Nicole Salès, Gerald Wells, Paul Brown, Jean-Philippe Deslys The uncertain extent of human exposure to bovine spongiform encephalopathy (BSE)—which can lead to variant Creutzfeldt-Jakob disease (vCJD)—is compounded by incomplete knowledge about the efficiency of oral infection and the magnitude of any bovine-to-human biological barrier to transmission. We therefore investigated oral transmission of BSE to non-human primates. We gave two macaques a 5 g oral dose of brain homogenate from a BSE-infected cow. One macaque developed vCJD-like neurological disease 60 months after exposure, whereas the other remained free of disease at 76 months. On the basis of these findings and data from other studies, we made a preliminary estimate of the food exposure risk for man, which provides additional assurance that existing public health measures can prevent transmission of BSE to man. Published online January 27, 2005 http://image.thelancet.com/ extras/05let1056web.pdf Commissariat à l’Energie Atomique/Direction des Sciences du Vivant/Départment de Recherche Médicale, 18 Route du Panorama, 92265 Fontenay-aux-Roses, France (C I Lasmézas DrMedVet, E Comoy DrMedVet, C Herzog DipBiol, F Mouthon DipBiol, F Auvré, E Correia, N Lescoutra-Etchegaray DipBiol, Prof N Salès PhD, J-P Deslys MD); Veterinary Laboratories Agency, New Haw, Addlestone, UK (S Hawkins MIBiol, T Konold DrMedVet, G Wells BVetMed); and 7815 Exeter Road, Bethesda, MD 20814, USA (P Brown PhD) Correspondence to: Dr Jean-Philippe Deslys e-mail: jpdeslys@cea.fr ========================== yep, i remember the same rhetoric with BSE/nvCJD. the above statement is nothing more than wishful thinking. i hope it's true, but the evidence speaks for itself, and the above statement is not 'sound science'. it's the same old song and dance, 'protect the industry at all cost'. why dont you just say, we don't know, but the present science to date shows that ; The EFSA Journal (2004) 70, 1-7, Opinion on a surveillance programme for Chronic Wasting Disease in the European Union http://www.efsa.eu.int 3 of 7 BACKGROUND 1.1. Scientific Steering Committee opinion At its meeting of 6-7 March 2003, the Scientific Steering Committee (SSC) produced an opinion on “Chronic Wasting Disease and tissues that might carry a risk for human and animal feed chains”. In this opinion, the SSC recommended the instigation of a surveillance program in the EU, which might initially target the examination of cervids dying in or culled from zoological collections and fallen stock in farmed cervid populations, prior to decisions on the screening of free-ranging cervids. snip... 8. Even though human TSE-exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familiar) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available. http://www.seac.gov.uk/papers/efsa-annex%205.pdf Research Project: Transmission, Differentiation, and Pathobiology of Transmissible Spongiform Encephalopathies Location: Virus and Prion Diseases of Livestock Title: Experimental Second Passage of Chronic Wasting Disease (Cwd-Mule Deer) Agent to Cattle Authors Hamir, Amirali Kunkle, Robert - bob Miller, Janice - ARS RETIRED Greenlee, Justin Richt, Juergen Submitted to: Journal Of Comparative Pathology Publication Acceptance Date: July 25, 2005 Publication Date: N/A Interpretive Summary: To compare the findings of experimental first and second passage of chronic wasting disease (CWD) in cattle, 6 calves were inoculated into the brain with CWD-mule deer agent previously (first) passaged in cattle. Two other uninoculated calves served as controls. Beginning 10-12 months post inoculation (PI), all inoculates lost appetite and weight. Five animals subsequently developed clinical signs of central nervous system (CNS) abnormality. By 16.5 months PI, all cattle had been euthanized because of poor prognosis. None of the animals showed microscopic lesions of spongiform encephalopathy (SE) but the CWD agent was detected in their CNS tissues by 2 laboratory techniques (IHC and WB). These findings demonstrate that inoculated cattle amplify CWD agent but also develop clinical CNS signs without manifestation of microscopic lesions of SE. This situation has also been shown to occur following inoculation of cattle with another TSE agent, namely, sheep scrapie. The current study confirms previous work that indicates that the diagnostic tests currently used for confirmation of bovine spongiform encephalopathy (BSE) in the U.S. would detect CWD in cattle, should it occur naturally. Furthermore, it raises the possibility of distinguishing CWD from BSE in cattle due to the absence of microscopic lesions and a unique multifocal distribution of PrPres, as demonstrated by IHC, which in this study, appears to be more sensitive than the WB. Technical Abstract: To compare clinicopathological findings of first and second passage of chronic wasting disease (CWD) in cattle, a group of calves (n=6) were intracerebrally inoculated with CWD-mule deer agent previously (first) passaged in cattle. Two other uninoculated calves served as controls. Beginning 10-12 months post inoculation (PI), all inoculates lost appetite and lost weight. Five animals subsequently developed clinical signs of central nervous system (CNS) abnormality. By 16.5 months PI, all cattle had been euthanized because of poor prognosis. None of the animals showed microscopic lesions of spongiform encephalopathy (SE) but PrPres was detected in their CNS tissues by immunohistochemistry (IHC) and Western blot (WB) techniques. These findings demonstrate that intracerebrally inoculated cattle not only amplify CWD PrPres but also develop clinical CNS signs without manifestation of morphologic lesions of SE. This situation has also been shown to occur following inoculation of cattle with another TSE agent, scrapie. The current study confirms previous work that indicates the diagnostic techniques currently used for confirmation of bovine spongiform encephalopathy (BSE) in the U.S. would detect CWD in cattle, should it occur naturally. Furthermore, it raises the possibility of distinguishing CWD from BSE in cattle due to the absence of neuropathologic lesions and a unique multifocal distribution of PrPres, as demonstrated by IHC, which in this study, appears to be more sensitive than the WB. http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=178318 ----- Original Message ----- From: "Terry S. Singeltary Sr." To: Sent: Wednesday, October 19, 2005 10:47 AM Subject: Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel Monkeys (Saimiri sciureus) ##################### Bovine Spongiform Encephalopathy ##################### From: TSS () Subject: Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel Monkeys (Saimiri sciureus) Date: October 19, 2005 at 8:33 am PST 0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005 Copyright © 2005, American Society for Microbiology. All Rights Reserved. Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel Monkeys (Saimiri sciureus) Richard F. Marsh,1, Anthony E. Kincaid,2 Richard A. Bessen,3 and Jason C. Bartz4* Department of Animal Health and Biomedical Sciences, University of Wisconsin, Madison 53706,1 Department of Physical Therapy,2 Department of Medical Microbiology and Immunology, Creighton University, Omaha, Nebraska 68178,4 Department of Veterinary Molecular Biology, Montana State University, Bozeman, Montana 597183 Received 3 May 2005/ Accepted 10 August 2005 Chronic wasting disease (CWD) is an emerging prion disease of deer and elk. The risk of CWD transmission to humans following exposure to CWD-infected tissues is unknown. To assess the susceptibility of nonhuman primates to CWD, two squirrel monkeys were inoculated with brain tissue from a CWD-infected mule deer. The CWD-inoculated squirrel monkeys developed a progressive neurodegenerative disease and were euthanized at 31 and 34 months postinfection. Brain tissue from the CWD-infected squirrel monkeys contained the abnormal isoform of the prion protein, PrP-res, and displayed spongiform degeneration. This is the first reported transmission of CWD to primates. ---------------------------------------------------------------------------- ---- * Corresponding author. Mailing address: Department of Medical Microbiology and Immunology, Creighton University, 2500 California Plaza, Omaha, NE 68178. Phone: (402) 280-1811. Fax: (402) 280-1875. E-mail: jbartz@creighton.edu . Deceased. ---------------------------------------------------------------------------- ---- Journal of Virology, November 2005, p. 13794-13796, Vol. 79, No. 21 0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005 Copyright © 2005, American Society for Microbiology. All Rights Reserved. http://jvi.asm.org/cgi/content/abstract/79/21/13794maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=cwd&searchid=1129736446553_4280&stored_search=&FIRSTINDEX=0&volume=79&issue=21&journalcode=jvi The Ten Biggest Lies in CWD: 1- "CWD occurs naturally at a low background level in wild animals." [All CWD to date -- including zoo and game farm -- traces back to the original scrapie sheep-to-deer transmission at CDOW Foothills Research Station in 1967. No natural prion disease has ever been found in any animal species anywhere in the world, outside of human.] 2- "CWD does not transmit to human, so hunters are not at risk." [Too soon to say, TSEs can have 50 year incubations. No test -- no one knows how to distinguish CWD in human from sporadic CJDs. Epidemiology has poor power. Preclinical cases can propagate without species barrier via blood and tissue donation. Same was said about mad cow disease, wrongly as it turned out.] 3- "I've hunted for years in the CWD hot zone, eaten brain sausage, and I feel great, therefore it must be ok." [Needs no comment, too stupid, yet 2 WY and 1 CO researcher have said this repeatedly to Denver Post.] 4- "The deer I shot tested negative, so no problem." [Preclinical animals may be *more* infectious than overtly sick animals because of higher prion abundance in more commonly eaten tissues. Game processors commonly pool 100 animals prior to a rendering run; there is no effective way to decontaminate equipment between batches. False negatives are quite common when only obex is used. Hunters commonly sever the spinal cord without wearing Biohazard Level III clothing.] 5- "Absence of evidence is evidence of absence" [Needs no comment, too stupid, yet most common spin put out to journalists by CDC, Colorado officials.] 6- "CWD is restricted to cervids; a species barrier prevents transmission to bears, wolves, wolverines, cougars, livestock etc." [If you don't look, you don't find: zero testing has been on predators and scavengers, other than preliminary cougar and raccoon work. Species barriers are initial inefficiencies, not absolute; second and later passages are highly efficient.] 7- "Trust me, I'm an expert." [Few officials have read or understood the 7028 published papers on prion disease. Few veterinarians and epidemiologists are trained in protein chemistry and molecular biology, the realm of prion disease.] 8- "You can test your way out of this disease." [Facilities like game farms become hopelessly contaminated; culling clinically ill animals is too late; live animal tests are difficult and not sufficiently sensitive.] 9- "CWD is self-limited to the Tri-State area because of natural selection." [Animals with CWD can reach reproductive age before dying; migrations cover huge areas; game farm trafficking is effectively unregulated; escapes, releases, entrapment of wild animals and rut breakin are common.] 10-- "There's scientific uncertainty about transmission mechanisms, so nothing can be done about CWD in the wild." [Containment facilities are notorious for high incidence of CWD; artifically high winter densities -- the govt runs 23 huge elk feedlots in Wyoming -- mimic game farms and research facilities. The Grand Teton feedlots could be closed and elk dispersed.] Possible CWD Surprises (over next year or two): -- A live animal blood test might be approved. If sufficiently sensitive (no false negatives, esp. animals early on in infection), animal roundups for testing might be presented as an option. However, concentration of animals in a confinement facility such as winter feedlot would only result in further spread of CWD prior to ability to cull and incinerate infected animals. -- Transmission to cows or sheep on public (or private) lands might be documented. This would bring calls for eradication of deer and elk or restriction to national parks, rather than allotment retirement. So far, experimental transmission to cattle has been difficult. Back transmission to sheep has not been tested. There are many evolving strains of CWD as each species has a unique prion protein sequence. Species barriers can change dramatically upon passage to a new species such as moose. -- Transmission to humans might be proven. This could cause a plunge in out-fof-state hunting tags which in Colorado account fo over half the Fish and Game budget. Right now, CWD has no distinctive signature (unlike florid plaques of mad cow disease) that separates it from sporadic CJD. It is all but impossible to prove epidemiologically as hunters are hard to track over long incubation periods and often have other exposures such as decades of eating mutton or beef or UK visits. Asymptomatic hunters could spread the disease widely into non-hunters through blood donations, corneal transplants, endoscopes, root canals, etc. What species have gotten CWD already? Note CWD has already passaged to carnivore and primate: CWD-Donor CWD-Recipient Method Year sheep white-tail deer shared pen at Foothills 1967+ sheep black-tail deer shared pen at Foothills 1967+ sheep mule deer shared pen at Foothills 1967+ sheep rocky mtn elk shared pen at Foothills 1967+ sheep rocky mtn elk intracerebral injection 2004 cervid moose unknown Colorado wild 2005 mule deer cow intracerebral injection 2001 white-tail cow in vitro conversion 2000 white-tail sheep in vitro conversion 2000 mule deer goat intracerebral injection 2001 mule deer sheep intracerebral injection 2001 white-tail human in vitro conversion 2000 mule deer squirrel monkey intracerebral injection 2005 white-tail ferret intracerebral injection 1998 ferret hamster intracerebral injection 1998 elk humanized mice intracerebral injection 2005* elk elkized mice intracerebral injection 2005 mule deer muleized mice intracerebral injection 2004 *first passage negative, second passage underway Abstracts for published scientific articles on CWD: PubMed using "chronic wasting disease" as search term. Also you can sign up for automatic email alerts there. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?CMD=Limits&DB=PubMed Extensive history of CWD: use 'advanced search' google restricted to url, http://www.mad-cow.org Some great eat-more-rotten-venison-today newspaper quotes from CO and WY game and fish staffers. An email/phone directory to about 200 scientists working in prion disease can be found at http://mad-cow.org/researchers.html Some recent abstracts Vet Rec. 2003 Jul 26;153(4):121-3. [no abstract, unclear if raccoon, another Carnivora, became infected] Experimental inoculation of scrapie and chronic wasting disease agents in raccoons (Procyon lotor). Hamir AN, Miller JM, Cutlip RC, Stack MJ, Chaplin MJ, Jenny AL, Williams ES. National Animal Disease Center, ARS, USDA, 2300 Dayton Avenue, PO Box 70, Ames, IA 50010, USA. PLOS Biology 2005: "Unlike BSE, CWD is not thought to be transmitted through feed. But three species of cervids are naturally susceptible, and the question arises of how many other species might be in danger. To help answer that question, Michael Samuel and colleagues at the University of Wisconsin are staking out deer carcasses to see which scavengers come to feed. With flashlit photography, they've discovered “an amazing cast of characters,” including hawks, owls, crows, dogs, cats, coyotes, raccoons, skunks, mink, foxes, and opossums (Figure 2). Mammalian scavengers in the state's CWD-affected region will later be examined for disease." J Vet Diagn Invest. 2004 Jul;16(4):316-21. [cwd looks just like scrapie, no surprise given inadvertent transmission at Foothills Research Station] Transmission of sheep scrapie to elk (Cervus elaphus nelsoni) by intracerebral inoculation: final outcome of the experiment. Hamir AN, Miller JM, Cutlip RC, Kunkle RA, Jenny AL, Stack MJ, Chaplin MJ, Richt JA. National Animal Disease Center, ARS, USDA, Ames, IA 50010, USA. This is a final report of an experimental transmission of sheep scrapie agent by intracerebral inoculation to Rocky Mountain elk (Cervus elaphus nelsoni). It documents results obtained in experimental (n = 6) and control (n = 2) elk. During the first 2 years postinoculation (PI), 3 animals died or were euthanized because of infection or injuries other than spongiform encephalopathy (SE). In years 3 and 4 PI, 3 other inoculated elk died after brief terminal neurological episodes. Necropsy of these animals revealed moderate weight loss but no other gross lesions. Microscopically, characteristic lesions of SE were seen throughout the brain and spinal cord, and the tissue was positive for proteinase K-resistant prion protein (PrPres) by immunohistochemistry (IHC) and by Western blot. Scrapie-associated fibrils (SAF) were observed by negative-stain electron microscopy in the brain of elk with neurologic signs. PrPres and SAF were not detected in the 3 inoculated elk necropsied during the first 2 years or in the 2 control animals. Retrospective analysis of the gene-encoding cervid PrP revealed a polymorphism at codon 132. The elk with SE were either homozygous (MM) or heterozygous (LM). These findings confirm that intracerebral inoculation of sheep scrapie agent results in SE with accumulations of PrPres in the central nervous system of elk. Based on morphologic and IHC findings, the experimentally induced SE cannot be distinguished from chronic wasting disease of elk with currently available diagnostic techniques. J Gen Virol. 2005 Aug;86(Pt 8):2127-34. [explores possible natural occurence of genetic resistance due to prion gene polymorphisms] Low frequency of PrP genotype 225SF among free-ranging mule deer (Odocoileus hemionus) with chronic wasting disease. Jewell JE, Conner MM, Wolfe LL, Miller MW, Williams ES. Department of Veterinary Sciences, University of Wyoming, Wyoming State Veterinary Laboratory, Laramie, WY. jjewell@uwyo.edu The prion protein (PrP) gene was characterized in 1482 free-ranging mule deer (Odocoileus hemionus) from Wyoming and Colorado. Using DNA sequences from 363 deer, dimorphisms at codons 20 (aspartate/glycine) and 225 [serine (S)/phenylalanine (F)] were found; silent changes occurred at codons 131 (tyrosine) and 247 (isoleucine). The remaining samples were surveyed for codon 225 genotype and all were characterized for chronic wasting disease (CWD) infection status. A total of 112 deer with the genotype 225SF or FF were found, of which one was CWD-positive; 1370 were 225SS, with 289 positive for CWD. Among CWD-negative deer, the frequency of 225SF/FF genotypes was 9.3 % but among CWD-positive deer it was only 0.3 %. For all samples combined, CWD status was not independent of codon 225 genotype (P<0.0001). The odds that a deer of the 225SS genotype was CWD-infected were 30 times greater (95 % confidence intervals=4-213) than for a 225SF deer. The proportion of 225SF animals in sampled subpopulations varied from 0 to 18 %; the CWD prevalence varied from 0 to 25 %. However, no relationship was observed between genotype frequency and CWD prevalence in different areas. The PrP sequences of experimentally infected mule deer were analysed from pre-existing projects and 10 animals were found with 225SF genotypes, all of which were positive for CWD. Data available from some of these animals suggest that the 225SF genotype could be associated with longer incubation periods in CWD infection compared with the 225SS genotype. J Vet Diagn Invest. 2003 Jul;15(4):320-3. [O'Rourke monoclonal antibody can be used in highly inconvenient live test using tonsil biopsy] Abundant PrP(CWD) in tonsil from mule deer with preclinical chronic wasting disease. O'Rourke KI, Zhuang D, Lyda A, Gomez G, Williams ES, Tuo W, Miller MW. Agricultural Research Service, U.S. Department of Agriculture Pullman, WA 99164, USA. A monoclonal antibody dot-blot assay was used to evaluate detergent lysates of tonsil tissue from mule deer to detect PrP(CWD), the marker for the cervid transmissible spongiform encephalopathy chronic wasting disease (CWD). Samples of formalin-fixed brain and tonsil tissues from mule deer were examined for PrP(CWD) using immunohistochemistry (IHC) with Mab F99/97.6.1, the gold standard for diagnosis of preclinical CWD. The contralateral tonsil from each of the 143 deer was prepared for confirmatory IHC and as a 10% (wt/vol) detergent lysate without purification or enrichment steps for monoclonal antibody dot-blot assay. PrP(CWD) was detected by dot-blot assay in 49 of 50 samples considered positive by IHC. Forty-eight of the positive samples were evaluated with a quantitative dot-blot assay calibrated with recombinant PrP. Tonsillar PrP(CWD) concentrations ranged from 34 to 1,188 ng per 0.5 mg starting wet weight of tissue. The abundant PrP(CWD) in mule deer tonsil will facilitate development and validation of high-throughput screening tests for CWD in large populations of free-ranging deer. J Vet Diagn Invest. 2003 May;15(3):274-7 [no transmission observed yet deer-to-cattle in the field] Survey of cattle in northeast Colorado for evidence of chronic wasting disease: geographical and high-risk targeted sample. Gould DH, Voss JL, Miller MW, Bachand AM, Cummings BA, Frank AA. Department of Pathology, Veterinary Medicine and Biomedical Sciences, Colorado State University, Ft. Collins, CO. A geographically targeted survey of potentially high-risk, adult cattle in chronic wasting disease (CWD)-endemic areas in Colorado was initiated to assess the possibility of the spread of CWD from deer to cattle under natural conditions. Surveyed cattle were sympatric with free-roaming deer in geographically defined areas where CWD occurs and where CWD prevalence has been estimated. To qualify for inclusion in the survey, cattle had to be at least 4 years old and had to have spent a minimum of 4 years in surveyed areas. Brains from culled cattle were examined microscopically and immunohistochemically for tissue alterations indicative of a transmissible spongiform encephalopathy (TSE). Two hundred sixty-two brains were suitable for evaluation and were found to lack changes indicative of a TSE infection. Prion deposition was not demonstrable using a method involving formic acid and proteinase-K treatment before application of monoclonal antibody to bovine prion protein (F99/97.6.1). Some incidental neuropathologic changes unrelated to those of TSEs were detected. Findings from this study suggest that large-scale spread of CWD from deer to cattle under natural range conditions in CWD-endemic areas of northeast Colorado is unlikely. J Gen Virol. 2004 May;85(Pt 5):1339-46. Polymorphisms in the prion precursor functional gene but not the pseudogene are associated with susceptibility to chronic wasting disease in white-tailed deer. O'Rourke KI, Spraker TR, Hamburg LK, Besser TE, Brayton KA, Knowles DP. US Department of Agriculture, Agricultural Research Service Pullman, WA 99164, USA. korourke@vetmed.wsu.edu Chronic wasting disease (CWD) status and PrP genotypes were determined for a group of 133 wild white-tailed deer in a 780 acre enclosure in western Nebraska, USA. Approximately half of the deer tested showed evidence of PrPd in the brainstem or lymphoid tissues. Four PRNP alleles encoding amino acid substitutions were identified, with substitutions at residues 95 (Q-->H), 96 (G-->S) or 116 (A-->G), each with serine (S) at residue 138. In addition, a processed pseudogene with two alleles encoding five or six copies of the octapeptide repeat was identified in 26 % of the deer. Both alleles encoded asparagine (N) at residue 138. The functional gene alleles sorted into five major diploid genotypes and four rare genotypes. Although all five major diploid genotypes were found in deer with CWD, unaffected deer were less likely to have the allele QGAS and more likely to have QSAS compared with CWD-affected deer. Late-stage disease (PrPd in brainstem) was noted in deer less than 1 year of age, although no single genotype was associated with this rapid neuroinvasion. Early-stage disease (PrPd distribution limited to the lymphoid system) was observed in deer estimated to be more than 5 years old, suggesting that they were infected as adults or that the incubation time might be extremely long in some individuals. The pseudogene was found in deer of all major PRNP genotypes and was not correlated with CWD status. The large number of susceptible genotypes and the possibility of adult-to-adult transmission suggest that much of the white-tailed deer population may be at risk for disease following exposure to CWD, despite the association of specific genotypes with CWD noted here. Nature. 2003 Sep 4;425(6953):35-6. [incidence of cwd in confined herds becomes astronomic] Prion disease: horizontal prion transmission in mule deer. Miller MW, Williams ES. Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, Colorado 80526, USA. mike.miller@state.co.us Epidemics of contagious prion diseases can be perpetuated by horizontal (animal to animal) and maternal (dam to offspring, before or after birth) transmission, but the relative importance of each mechanism is unclear. Here we compare the incidence of chronic wasting disease (CWD) in captive mule deer (Odocoileus hemionus) that is attributable to horizontal or maternal transmission. We find that horizontal transmission is remarkably efficient, producing a high incidence of disease (89%) in a cohort of deer in which maternal transmission was improbable. Our results indicate that horizontal transmission is likely to be important in sustaining CWD epidemics. Vet Rec. 2004 Sep 4;155(10):295-302. [obex testing alone seriously understates actual cwd incidence] Variable patterns of distribution of PrP(CWD) in the obex and cranial lymphoid tissues of Rocky Mountain elk (Cervus elaphus nelsoni) with subclinical chronic wasting disease. Spraker TR, Balachandran A, Zhuang D, O'Rourke KI. Colorado State University, Fort Collins, CO, USA. Sections of medulla oblongata, taken at the level of the obex, palatine tonsil and medial retropharyngeal lymph node from 10,269 captive Rocky Mountain elk (Cervus elaphus nelsoni), were examined by immunohistochemical staining with monoclonal antibody for the prion protein associated with the transmissible spongiform encephalopathy of cervids, chronic wasting disease (PrP(CWD)). The protein was detected in 226 of them. On the basis of the anatomical location of the deposits in the brainstem of 183 elk, four distinct patterns of distribution of PrP(CWD) within the parasympathetic region of the dorsal motor nucleus of the vagus nerve and the adjacent nuclei were observed. Mild gross lesions of chronic wasting disease (serous atrophy of fat) were observed in only three elk, all with spongiform degeneration; the other elk were considered to be in the preclinical stage of the disease. In contrast with the relatively predictable distribution of prion protein (PrP) in the brain and cranial nodes of sheep and mule deer, the distribution of PrP(CWD) in the brain and nodes of the elk was more variable and unrelated to their PrP genotype. One hundred and fifty-five of the 226 positive elk had deposits of PrP(CWD) in the brainstem and lymphoid tissues, 43 had deposits only in the lymphoid tissue and 28 had deposits only in the brainstem. ........snip.........end Aguzzi warns of CWD danger The TSE family of diseases also includes chronic wasting disease (CWD) in deer, a condition that has spread in the US in recent years (Nature 416, 569; 2002). Speaking at the Days of Molecular Medicine conference in La Jolla in March, prion expert Adriano Aguzzi issued a strong warning against underestimating this form of TSE. "For more than a decade, the US has by-and-large considered mad cows to be an exquisitely European problem. The perceived need to protect US citizens from this alien threat has even prompted the deferral of blood donors from Europe," he said. "Yet the threat-from-within posed by CWD needs careful consideration, since the evidence that CWD is less dangerous to humans than BSE is less-than-complete. Aguzzi went on to point out that CWD is arguably the most mysterious of all prion diseases. "Its horizontal spread among the wild population is exceedingly efficient, and appears to have reached a prevalence unprecedented even by BSE in the UK at its peak. The pathogenesis of CWD, therefore, deserves a vigorous research effort. Europeans also need to think about this problem, and it would be timely and appropriate to increase CWD surveillance in Europe too." Aguzzi has secured funding from the National Institutes of Health to investigate CWD, and the effort will be lead by Christina Sigurdson in his department at the University of Zurich. KAREN BIRMINGHAM, LONDON This quote from Dr. Gambetti is especially significant since he is the rather cautious TSE researcher under contract with the Centers for Disease Control to examine the brains of individuals who have died of CJD. ----------------- Pierluigi Gambetti, director of the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, said all deer should be tested for chronic wasting disease before any processing is done. "There is no way around it," he said. "Nobody should touch that meat unless it has been tested." http://www.ledger-enquirer.com/mld/...ion/3954298.htm CWD TO HUMANS = sCJD ??? AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease. snip... http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf ATYPICAL TSEs in USA CATTLE AND SHEEP ? http://www.bseinquiry.gov.uk/files/sc/seac17/tab03.pdf TSS #################### https://lists.aegee.org/bse-l.html #################### Perspective Chronic Wasting Disease and Potential Transmission to Humans Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,† Michael W. Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger* *Centers for Disease Control and Prevention, Atlanta, Georgia, USA; †University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve University, Cleveland, Ohio, USA Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from: http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm -------------------------------------------------------------------------------- Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions. snip... Risk for Transmission to Humans Epidemiologic Studies The increasing detection of CWD in a wider geographic area and the presumed foodborne transmission of BSE to humans, resulting in cases of vCJD, have raised concerns about the possible zoonotic transmission of CWD (32). In the late 1990s, such concerns were heightened by the occurrence of CJD among three patients 30 years of age who were deer hunters or ate deer and elk meat harvested by family members (Table 2). However, epidemiologic and laboratory investigations of these case-patients indicated no strong evidence for a causal link between CWD and their CJD illness (33). None of the patients were reported to have hunted deer or eaten deer meat harvested in the CWD-endemic areas of Colorado and Wyoming. Such a history in unusually young CJD patients, if present, would have supported a causal link with CWD. Moreover, the testing of brain tissues from >1,000 deer and elk harvested from areas where the patients hunted or their venison originated did not show any evidence of CWD (33). In addition, the lack of homogeneity in the clinicopathologic manifestation and codon 129 of the prion protein gene among the three patients suggested that their illnesses could not be explained by exposure to the same prion strain. In vCJD, homogeneity of the genotype at codon 129 and the clinical and pathologic phenotype were attributed to the patients' exposure to the same prion strain, the agent of BSE. In 2001, the case of a 25-year-old man who reportedly died of a prion disease after an illness lasting ˜22 months was investigated (Table 2). Although this man had hunted deer only rarely, his grandfather hunted deer and elk throughout much of the 1980s and 1990s and regularly shared the venison with the case-patient's family. The grandfather primarily hunted in southeastern Wyoming, around the known CWD-endemic area. The case-patient's illness began with a seizure and progressed to fatigue, poor concentration, and depression. Memory loss, ataxia, speech abnormalities, combative behavior, and recurrent seizures also developed. Histopathologic, immunohistochemical, and Western blot testing of brain autopsy samples confirmed a prion disease diagnosis. Analysis of the prion protein gene indicated a P102L mutation coupled with valine at the polymorphic codon 129 in the mutant allele, confirming a diagnosis of Gerstmann-Sträussler-Scheinker syndrome (GSS). This case-patient was unusually young even for a person with a GSS P102L mutation. It remains unknown whether the possible exposure of the case-patient to CWD-infected venison potentially contributed to the early onset of his prion disease. In 2001, two additional CJD patients 26 and 28 years of age were reported from a single state (Table 2) (34). The patients grew up in adjacent counties and had illness onset within several months of each other. As a result of this fact and their unusually young age, a possible environmental source of infection, including exposure to CWD-infected venison, was considered. One of the patients died after an illness lasting 5–6 months that was characterized by progressive aphasia, memory loss, social withdrawal, vision disturbances, and seizure activity leading to status epilepticus and induced coma. Histopathologic, immunohistochemical, and Western blot testing of brain biopsy and autopsy samples confirmed a CJD diagnosis. The patient's disease phenotype corresponded to the MM2 sporadic CJD subtype reported by Parchi et al. (35). This patient did not hunt, and family members provided no history of regularly eating venison. The patient may have occasionally eaten venison originating from the Upper Peninsula of Michigan while away from home during his college years. However, ongoing surveillance has not detected CWD in Michigan deer (36). The second patient died from an illness lasting <16 months. The patient's illness began with behavioral changes, including unusual outbursts of anger and depression. Confusion, memory loss, gait disturbances, incontinence, headaches, and photophobia also developed. Western blot analysis of frozen brain biopsy tissue confirmed a prion disease diagnosis. Immunohistochemical analysis of brain tissue obtained after the patient's death showed prion deposition consistent with GSS. A prion protein gene analysis could not be performed because appropriate samples were lacking. However, prion protein gene analysis of a blood sample from one of the patient's parents indicated a GSS P102L mutation. The patient did not hunt but may have eaten venison from Michigan once when he was 1–2 years old. The GSS diagnosis greatly reduced the likelihood that the two patients reported from adjacent counties had disease with a common origin. Recently, rare neurologic disorders resulting in the deaths of three men who participated in "wild game feasts" in a cabin owned by one of the decedents created concern about the possible relationship of their illnesses with CWD (Table 2) (37). Two of the patients reportedly died of CJD, and the third died from Pick's disease. More than 50 persons were identified as possibly participating in these feasts; the three patients were the only participants reported to have died of a degenerative neurologic disorder. Reanalysis of autopsy brain tissues from the three patients at the National Prion Disease Pathology Surveillance Center indicated that two of them had no evidence of a prion disease by immunohistochemical analysis. CJD was confirmed in the third patient, who had clinicopathologic, codon 129, and prion characteristics similar to the most common sporadic CJD subtype (MM1/MV1) (35). This patient participated in the feasts only once, perhaps in the mid-1980s. In addition, the investigation found no evidence that the deer and elk meat served during the feasts originated from the known CWD-endemic areas of Colorado and Wyoming. In 2003, CJD in two deer and elk hunters (54 and 66 years of age) was reported (38). The report implied that the patients had striking neuropathologic similarities and that their illness may represent a new entity in the spectrum of prion diseases. A third patient (63 years of age), who was also purported to have been a big game hunter, was subsequently reported from the same area. However, none of the three patients were reported to have eaten venison from the CWD-endemic areas of the western United States. The 66-year-old patient hunted most of his life in Washington State. Although information about the 54-year-old patient was limited, there was no evidence that he hunted in CWD-endemic areas. The third patient was not a hunter but ate venison harvested from Pennsylvania and Washington. The neuropathologic changes, Western blot profile, and genotype at codon 129 of the three patients each fit the MM1, VV1, or VV2 sporadic CJD subtype, indicating absence of phenotypic similarity among the cases or atypical neuropathologic features (35). To date, only two nonfamilial CJD cases with a positive history of exposure to venison obtained from the known CWD-endemic areas have been reported. One of the patients was a 61-year-old woman who grew up in an area where this disease is known to be endemic, and she ate venison harvested locally. She died in 2000, and analysis of autopsy brain specimens confirmed that the patient's CJD phenotype fit the MM1 subtype, with no atypical neuropathologic features. The second patient was a 66-year-old man who was reported to have eaten venison from two deer harvested in a CWD-endemic area. Both deer tested negative for CWD, and the patient's illness was consistent with the MM1 CJD phenotype. Despite the decades-long endemicity of CWD in Colorado and Wyoming, the incidence of CJD and the age distribution of CJD case-patients in these two states are similar to those seen in other parts of the United States. From 1979 to 2000, 67 CJD cases from Colorado and 7 from Wyoming were reported to the national multiple cause-of-death database. The average annual age-adjusted CJD death rate was 1.2 per million persons in Colorado and 0.8 in Wyoming. The proportion of CJD patients who died before age 55 in Colorado (13.4%) was similar to that of the national (10.2%). The only CJD case-patient <30 years of age in Colorado had iatrogenic CJD linked to receipt of human growth hormone injections. CJD was not reported in persons <55 years of age in Wyoming during the 22-year surveillance period. snip... Conclusions The lack of evidence of a link between CWD transmission and unusual cases of CJD, despite several epidemiologic investigations, and the absence of an increase in CJD incidence in Colorado and Wyoming suggest that the risk, if any, of transmission of CWD to humans is low. Although the in vitro studies indicating inefficient conversion of human prion protein by CWD-associated prions raise the possibility of low-level transmission of CWD to humans, no human cases of prion disease with strong evidence of a link with CWD have been identified. However, the transmission of BSE to humans and the resulting vCJD indicate that, provided sufficient exposure, the species barrier may not completely protect humans from animal prion diseases. Because CWD has occurred in a limited geographic area for decades, an adequate number of people may not have been exposed to the CWD agent to result in a clinically recognizable human disease. The level and frequency of human exposure to the CWD agent may increase with the spread of CWD in the United States. Because the number of studies seeking evidence for CWD transmission to humans is limited, more epidemiologic and laboratory studies should be conducted to monitor the possibility of such transmissions. Studies involving transgenic mice expressing human and cervid prion protein are in progress to further assess the potential for the CWD agent to cause human disease. Epidemiologic studies have also been initiated to identify human cases of prion disease among persons with an increased risk for exposure to potentially CWD-infected deer or elk meat (47). If such cases are identified, laboratory data showing similarities of the etiologic agent to that of the CWD agent would strengthen the conclusion for a causal link. Surveillance for human prion diseases, particularly in areas where CWD has been detected, remains important to effectively monitor the possible transmission of CWD to humans. Because of the long incubation period associated with prion diseases, convincing negative results from epidemiologic and experimental laboratory studies would likely require years of follow-up. In the meantime, to minimize the risk for exposure to the CWD agent, hunters should consult with their state wildlife agencies to identify areas where CWD occurs and continue to follow advice provided by public health and wildlife agencies. Hunters should avoid eating meat from deer and elk that look sick or test positive for CWD. They should wear gloves when field-dressing carcasses, bone-out the meat from the animal, and minimize handling of brain and spinal cord tissues. As a precaution, hunters should avoid eating deer and elk tissues known to harbor the CWD agent (e.g., brain, spinal cord, eyes, spleen, tonsils, lymph nodes) from areas where CWD has been identified. Acknowledgments snip...END http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm ================================== BUT here is the most damning evidence of all. may be the beginning of the END of the damn UKBSEnvCJD ONLY THEORY; TSS Coexistence of multiple PrPSc types in individuals with CJD Fri Nov 18, 2005 10:05 70.110.89.13 Lancet Neurology 2005; 4:805-814 DOI:10.1016/S1474-4422(05)70225-8 Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease Magdalini Polymenidou a, Katharina Stoeck a, Markus Glatzel a b, Martin Vey c, Anne Bellon c and Adriano Aguzzi a 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. Affiliations a Institute of Neuropathology, University Hospital Zurich, Switzerland b Present address: Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany c ZLB Behring, Marburg, Germany Correspondence to: Dr Adriano Aguzzi, Institute of Neuropathology, University Hospital of Zürich, Schmelzbergstrasse 12, CH-8091 Zürich, Switzerland http://www.thelancet.com/journals/laneur/article/PIIS1474442205702258/abstract what i been saying for years, that the diagnostic criteria differentiating between the nvCJD (i.e. 'the chosen ones') and the sCJD (i.e. 'the forgotten ones') has been terribly flawed from the beginning. ....TSS AND WHAT ABOUT THIS DRAMATIC INCREASE IN SPORADIC CJD IN THE USA AND WHAT ABOUT THE UNKNOWN TYPES DOCUMENTED RECENTLY ??? just more of the infamous sporadic CJD, no route, no source, just pops out of thin air, spontaneously, in-VIVO they call it, another PIPEDREAM and or wishful thinking by the industry ; STRICTLY PRIVATE AND CONFIDENTIAL 25, AUGUST 1995 snip... To minimise the risk of farmers' claims for compensation from feed compounders. To minimise the potential damage to compound feed markets through adverse publicity. To maximise freedom of action for feed compounders, notably by maintaining the availability of meat and bone meal as a raw material in animal feeds, and ensuring time is available to make any changes which may be required. snip... THE FUTURE 4.......... MAFF remains under pressure in Brussels and is not skilled at handling potentially explosive issues. 5. Tests _may_ show that ruminant feeds have been sold which contain illegal traces of ruminant protein. More likely, a few positive test results will turn up but proof that a particular feed mill knowingly supplied it to a particular farm will be difficult if not impossible. 6. The threat remains real and it will be some years before feed compounders are free of it. The longer we can avoid any direct linkage between feed milling _practices_ and actual BSE cases, the more likely it is that serious damage can be avoided. ... SEE full text ; http://www.bseinquiry.gov.uk/files/yb/1995/08/24002001.pdf SPORADIC CJD USA TO 2005 (only the documented ones, with a human TSE surveillance system that is about like the USA BSE surveillance system in cattle, non-existance...TSS) Animal Prion Diseases Relevant to Humans (unknown types?) Thu Oct 27, 2005 12:05 71.248.128.109 About Human Prion Diseases / Animal Prion Diseases Relevant to Humans Bovine Spongiform Encephalopathy (BSE) is a prion disease of cattle. Since 1986, when BSE was recognized, over 180,000 cattle in the UK have developed the disease, and approximately one to three million are likely to have been infected with the BSE agent, most of which were slaughtered for human consumption before developing signs of the disease. The origin of the first case of BSE is unknown, but the epidemic was caused by the recycling of processed waste parts of cattle, some of which were infected with the BSE agent and given to other cattle in feed. Control measures have resulted in the consistent decline of the epidemic in the UK since 1992. Infected cattle and feed exported from the UK have resulted in smaller epidemics in other European countries, where control measures were applied later. Compelling evidence indicates that BSE can be transmitted to humans through the consumption of prion contaminated meat. BSE-infected individuals eventually develop vCJD with an incubation time believed to be on average 10 years. As of November 2004, three cases of BSE have been reported in North America. One had been imported to Canada from the UK, one was grown in Canada, and one discovered in the USA but of Canadian origin. There has been only one case of vCJD reported in the USA, but the patient most likely acquired the disease in the United Kingdom. If current control measures intended to protect public and animal health are well enforced, the cattle epidemic should be largely under control and any remaining risk to humans through beef consumption should be very small. (For more details see Smith et al. British Medical Bulletin, 66: 185. 2003.) Chronic Wasting Disease (CWD) is a prion disease of elk and deer, both free range and in captivity. CWD is endemic in areas of Colorado, Wyoming, and Nebraska, but new foci of this disease have been detected in Nebraska, South Dakota, New Mexico, Wisconsin, Mississippi Kansas, Oklahoma, Minnesota, Montana, and Canada. Since there are an estimated 22 million elk and deer in the USA and a large number of hunters who consume elk and deer meat, there is the possibility that CWD can be transmitted from elk and deer to humans. As of November 2004, the NPDPSC has examined 26 hunters with a suspected prion disease. However, all of them appeared to have either typical sporadic or familial forms of the disease. The NPDPSC coordinates with the Centers for Disease Control and state health departments to monitor cases from CWD-endemic areas. Furthermore, it is doing experimental research on CWD transmissibility using animal models. (For details see Sigurdson et al. British Medical Bulletin. 66: 199. 2003 and Belay et al. Emerging Infectious Diseases. 10(6): 977. 2004.) http://www.cjdsurveillance.com/abouthpd-animal.html SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2004. SPORADIC CJD CASES TRIPLED, and that is with a human TSE surveillance system that is terrible flawed. in 1997 cases of the _reported_ cases of cjd were at 54, to 163 _reported_ cases in 2004. see stats here; p.s. please note the 47 PENDING CASES to Sept. 2005 p.s. please note the 2005 Prion D. total 120(8) 8=includes 51 type pending, 1 TYPE UNKNOWN ??? p.s. please note sporadic CJD 2002(1) 1=3 TYPE UNKNOWN??? p.s. please note 2004 prion disease (6) 6=7 TYPE UNKNOWN??? http://www.cjdsurveillance.com/resources-casereport.html NOW, for the good part ; > Discussion > > > WE conclude that American sources of sheep scrapie are transmissible to > cattle by direct intracerebral inoculation but the disease induced is NOT > identical to BSE as seen in the United Kingdom. While there were > similarities in clinical signs between this experimental disease and BSE, > there was no evidence of aggressiveness, hyperexcitability, hyperesthesia > (tactile or auditory), or hyperemetria of limbs as has been reported for BSE > (9). Neither were there extensive neurologic lesions, which are primary for > BSE, such as severe vacuolation of neurons and neuropil or neuronal necrosis > and gliosis. Although some vacuolation of neuropil, chromotolysis in > neurons, and gliosis were seen in the brains of some affected calves, these > were industinguishable from those of controls. Vacuolated neurons in the red > nucleus of both challenged and normal calves were considered normal for the > bovines as previously described (50). > > > PrP-res was found in ALL CHALLENGED CALVES REGARDLESS OF CLINCIAL SIGNS, and > the amount of PrP-res positively related to the length of the incubation. > ... > > > snip... > > > WE also conclude from these studies that scrapie in cattle MIGHT NOT BE > RECOGNIZED BY ROUTINE HISTOPATHOLOGICAL EXAMINATION OF THE BRAIN AND SUGGEST > THAT DETECTION OF PrP-res by immunohistochemistry or immunoblotting is > necessary to make a definitive diagnosis. THUS, undiagnosed scrapie > infection could contribute to the ''DOWNER-COW'' syndrome and could be > responsible for some outbreaks of transmissible mink encephalopathy proposed > by Burger and Hartsough (8) and Marsh and harsough (52). ... > > > snip...tss http://www.bseinquiry.gov.uk/files/sc/seac17/tab03.pdf http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf nope, no mad cows in the USA and all the demented are just a happenstance of badluck, spontaneous BSe what i call it. CORPORATE MAD COW DISEASE IN SHEEP, DEER/ELK, COWS AND HUMANS, why not, we been feeding scrapie infected sheep and goats, CWD infected deer and elk, with a few TME mink thrown in (not too many, but a few via RENDER), plus the undocumented TSE in cattle that has been here for decades; Gerald Wells: Report of the Visit to USA, April-May 1989 snip... The general opinion of those present was that BSE, as an overt disease phenomenon, _could exist in the USA, but if it did, it was very rare. The need for improved and specific surveillance methods to detect it as recognised... snip... It is clear that USDA have little information and _no_ regulatory responsibility for rendering plants in the US... snip... 3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a _very low profile indeed_. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be _avoided_ in the US _at all costs_... snip... http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf To be published in the Proceedings of the Fourth International Scientific Congress in Fur Animal Production. Toronto, Canada, August 21-28, 1988 Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle R.F. Marsh* and G.R. Hartsough •Department of Veterinary Science, University of Wisconsin-Madison, Madison, Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service, Thiensville, Wisconsin 53092 ABSTRACT Epidemiologic investigation of a new incidence of transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin suggests that the disease may have resulted from feeding infected cattle to mink. This observation is supported by the transmission of a TME-like disease to experimentally inoculated cattle, and by the recent report of a new bovine spongiform encephalopathy in England. INTRODUCTION Transmissible mink encephalopathy (TME) was first reported in 1965 by Hartsough and Burger who demonstrated that the disease was transmissible with a long incubation period, and that affected mink had a spongiform encephalopathy similar to that found in scrapie-affecied sheep (Hartsough and Burger, 1965; Burger and Hartsough, 1965). Because of the similarity between TME and scrapie, and the subsequent finding that the two transmissible agents were indistinguishable (Marsh and Hanson, 1969), it was concluded that TME most likely resulted from feeding mink scrapie-infecied sheep. The experimental transmission of sheep scrapie to mink (Hanson et al., 1971) confirmed the close association of TME and scrapie, but at the same time provided evidence that they may be different. Epidemiologic studies on previous incidences of TME indicated that the incubation periods in field cases were between six months and one year in length (Harxsough and Burger, 1965). Experimentally, scrapie could not be transmitted to mink in less than one year. To investigate the possibility that TME may be caused by a (particular strain of scrapie which might be highly pathogenic for mink, 21 different strains of the scrapie agent, including their sheep or goat sources, were inoculated into a total of 61 mink. Only one mink developed a progressive neurologic disease after an incubation period of 22 mon..s (Marsh and Hanson, 1979). These results indicated that TME was either caused by a strain of sheep scrapie not yet tested, or was due to exposure to a scrapie-like agent from an unidentified source. OBSERVATIONS AND RESULTS A New Incidence of TME. In April of 1985, a mink rancher in Stetsonville, Wisconsin reported that many of his mink were "acting funny", and some had died. At this time, we visited the farm and found that approximately 10% of all adult mink were showing typical signs of TME: insidious onset characterized by subtle behavioral changes, loss of normal habits of cleanliness, deposition of droppings throughout the pen rather than in a single area, hyperexcitability, difficulty in chewing and swallowing, and tails arched over their _backs like squirrels. These signs were followed by progressive deterioration of neurologic function beginning with locomoior incoordination, long periods of somnolence in which the affected mink would stand motionless with its head in the corner of the cage, complete debilitation, and death. Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. Since previous incidences of TME were associated with common or shared feeding practices, we obtained a careful history of feed ingredients used over the past 12-18 months. The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy cattle and a few horses. Sheep had never been fed. Experimental Transmission. The clinical diagnosis of TME was confirmed by histopaihologic examination and by experimental transmission to mink after incubation periods of four months. To investigate the possible involvement of cattle in this disease cycle, two six-week old castrated Holstein bull calves were inoculated intracerebrally with a brain suspension from affected mink. Each developed a fatal spongiform encephalopathy after incubation periods of 18 and 19 months. DISCUSSION These findings suggest that TME may result from feeding mink infected cattle and we have alerted bovine practitioners that there may exist an as yet unrecognized scrapie-like disease of cattle in the United States (Marsh and Hartsough, 1986). A new bovine spongiform encephalopathy has recently been reported in England (Wells et al., 1987), and investigators are presently studying its transmissibility and possible relationship to scrapie. Because this new bovine disease in England is characterized by behavioral changes, hyperexcitability, and agressiveness, it is very likely it would be confused with rabies in the United Stales and not be diagnosed. Presently, brains from cattle in the United States which are suspected of rabies infection are only tested with anti-rabies virus antibody and are not examined histopathologically for lesions of spongiform encephalopathy. We are presently pursuing additional studies to further examine the possible involvement of cattle in the epidemiology of TME. One of these is the backpassage of our experimental bovine encephalopathy to mink. Because (here are as yet no agent- specific proteins or nucleic acids identified for these transmissible neuropathogens, one means of distinguishing them is by animal passage and selection of the biotype which grows best in a particular host. This procedure has been used to separate hamster- adapted and mink-udapted TME agents (Marsh and Hanson, 1979). The intracerebral backpassage of the experimental bovine agent resulted in incubations of only four months indicating no de-adaptation of the Stetsonville agent for mink after bovine passage. Mink fed infected bovine brain remain normal after six months. It will be essential to demonstrate oral transmission fiom bovine to mink it this proposed epidemiologic association is to be confirmed. ACKNOWLEDGEMENTS These studies were supported by the College of Agricultural and Life Sciences, University of Wisconsin-Madison and by a grant (85-CRCR-1-1812) from the United States Department of Agriculture. The authors also wish to acknowledge the help and encouragement of Robert Hanson who died during the course of these investigations. REFERENCES Burger, D. and Hartsough, G.R. 1965. Encephalopathy of mink. II. Experimental and natural transmission. J. Infec. Dis. 115:393-399. Hanson, R.P., Eckroade, R.3., Marsh, R.F., ZuRhein, C.M., Kanitz, C.L. and Gustatson, D.P. 1971. Susceptibility of mink to sheep scrapie. Science 172:859-861. Hansough, G.R. and Burger, D. 1965. Encephalopathy of mink. I. Epizoociologic and clinical observations. 3. Infec. Dis. 115:387-392. Marsh, R.F. and Hanson, R.P. 1969. Physical and chemical properties of the transmissible mink encephalopathy agent. 3. ViroL 3:176-180. Marsh, R.F. and Hanson, R.P. 1979. On the origin of transmissible mink encephalopathy. In Hadlow, W.J. and Prusiner, S.P. (eds.) Slow transmissible diseases of the nervous system. Vol. 1, Academic Press, New York, pp 451-460. Marsh, R.F. and Hartsough, G.R. 1986. Is there a scrapie-like disease in cattle? Proceedings of the Seventh Annual Western Conference for Food Animal Veterinary Medicine. University of Arizona, pp 20. Wells, G.A.H., Scott, A.C., Johnson, C.T., Cunning, R.F., Hancock, R.D., Jeffrey, M., Dawson, M. and Bradley, R. 1987. A novel progressive spongiform encephalopathy in cattle. Vet. Rec. 121:419-420. MARSH http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf 7 OF 10 LITTLE PIGGIES WENT ON TO DEVELOP BSE; 1: J Comp Pathol. 2000 Feb-Apr; 122(2-3): 131-43. Related Articles, Links Click here to read The neuropathology of experimental bovine spongiform encephalopathy in the pig. Ryder SJ, Hawkins SA, Dawson M, Wells GA. Veterinary Laboratories Agency Weybridge, Woodham Lane, New Haw, Addlestone, Surrey, KT15 3NB, UK. In an experimental study of the transmissibility of BSE to the pig, seven of 10 pigs, infected at 1-2 weeks of age by multiple-route parenteral inoculation with a homogenate of bovine brain from natural BSE cases developed lesions typical of spongiform encephalopathy. The lesions consisted principally of severe neuropil vacuolation affecting most areas of the brain, but mainly the forebrain. In addition, some vacuolar change was identified in the rostral colliculi and hypothalamic areas of normal control pigs. PrP accumulations were detected immunocytochemically in the brains of BSE-infected animals. PrP accumulation was sparse in many areas and its density was not obviously related to the degree of vacuolation. The patterns of PrP immunolabelling in control pigs differed strikingly from those in the infected animals. PMID: 10684682 [PubMed - indexed for MEDLINE] http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?holding=npg&cmd=Retrieve&db=PubMed&list_uids=10684682&dopt=Abstract confindential pigs & pharmaceuticals http://www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf http://www.bseinquiry.gov.uk/files/yb/1990/08/23002001.pdf http://www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf http://www.bseinquiry.gov.uk/files/yb/1990/08/29003001.pdf Infected and Source Flocks As of August 31, 2005, there were 115 scrapie infected and source flocks (figure 3). There were 3 new infected and source flocks reported in August (Figure 4) with a total of 148 flocks reported for FY 2005 (Figure 5). The total infected and source flocks that have been released in FY 2005 are 102 (Figure 6), with 5 flocks released in August. The ratio of infected and source flocks released to newly infected and source flocks for FY 2005 = 0.69 : 1. In addition, as of August 31, 2005, 574 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 122 were RSSS cases (Figure 7). This includes 55 newly confirmed cases in August 2005 (Figure 8). Fifteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat case was reported in May 2005. snip... full text ; http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html SCRAPIE USA JULY 2005 UPDATE AS of July 31, 2005, there were 120 scrapie infected soure flocks (figure 3). There were 16 new infected and source flocks reorted in July (Figure 4) with a total of 143 flocks reported for FY 2005 (Figure 5). The total infected and source flocks that have been released in FY 2005 are 89 (Figure 6), with 8 flocks released in July. The ratio of infected and source flocks released to newly infected and source flocks for FY = 0.62 : 1. IN addition, as of July 31, 2005, 524 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 116 were RSSS cases (Figure 7). This includes 76 newly confirmed cases in July 2005 (Figure 8). Fifteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat case was reported in May 2005. ........... snip... http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html SCRAPIE USA JUNE 2005 UPDATE AS of June 30, 2005, there were 114 scrapie infected and source flocks (Figure 3). There were 14 new infected and source flocks reported in June (Figure 4) with a total of 123 flocks reported for FY 2005 (Figure 5). snip... In addition, as of June 30, 2005, 448 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 106 were RSSS cases (Figure 7). This includes 81 newly confirmed cases in June 2005 (Figure 8). Fifteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat case was reported in May 2005. snip...end http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html From: TSS () Subject: SCRAPIE USA UPDATE MARCH - JUNE 2005 Date: August 24, 2005 at 7:03 pm PST SCRAPIE USA MONTHLY REPORT 2005 AS of March 31, 2005, there were 70 scrapie infected source flocks (Figure 3). There were 11 new infected and source flocks reported in March (Figure 4) with a total of 51 flocks reported for FY 2005 (Figure 5). The total infected and source flocks that have been released in FY 2005 are 39 (Figure 6), with 1 flock released in March. The ratio of infected and source flocks released to newly infected and source flocks for FY 2005 = 0.76 : 1. IN addition, as of March 31, 2005, 225 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), of which 53 were RSSS cases (Figure 7). This includes 57 newly confirmed cases in March 2005 (Figure 8). Fourteen cases of scrapie in goats have been reported since 1990 (Figure 9). The last goat cases was reported in January 2005. New infected flocks, source flocks, and flocks released or put on clean-up plans for FY 2005 are depicted in Figure 10. ... FULL TEXT ; http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html Published online before print October 20, 2005 Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102 Medical Sciences A newly identified type of scrapie agent can naturally infect sheep with resistant PrP genotypes ( sheep prion | transgenic mice ) Annick Le Dur *, Vincent Béringue *, Olivier Andréoletti , Fabienne Reine *, Thanh Lan Laï *, Thierry Baron , Bjørn Bratberg ¶, Jean-Luc Vilotte ||, Pierre Sarradin **, Sylvie L. Benestad ¶, and Hubert Laude * *Virologie Immunologie Moléculaires and ||Génétique Biochimique et Cytogénétique, Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut National de la Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005) Scrapie in small ruminants belongs to transmissible spongiform encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative disorders that affect humans and animals and can transmit within and between species by ingestion or inoculation. Conversion of the host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified surveillance of scrapie in the European Union, together with the improvement of PrPSc detection techniques, has led to the discovery of a growing number of so-called atypical scrapie cases. These include clinical Nor98 cases first identified in Norwegian sheep on the basis of unusual pathological and PrPSc molecular features and "cases" that produced discordant responses in the rapid tests currently applied to the large-scale random screening of slaughtered or fallen animals. Worryingly, a substantial proportion of such cases involved sheep with PrP genotypes known until now to confer natural resistance to conventional scrapie. Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health. -------------------------------------------------------------------------------- Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R., T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B. contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data; and H.L. wrote the paper. A.L.D. and V.B. contributed equally to this work. To whom correspondence should be addressed. Hubert Laude, E-mail: laude@jouy.inra.fr www.pnas.org/cgi/doi/10.1073/pnas.0502296102 http://www.pnas.org/cgi/content/abstract/0502296102v1 TSS #################### https://lists.aegee.org/bse-l.html #################### ######## Bovine Spongiform Encephalopathy ######### 12/10/76 AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE Office Note CHAIRMAN: PROFESSOR PETER WILDY snip... A The Present Position with respect to Scrapie A] The Problem Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries. The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep. It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible. Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously. snip... 76/10.12/4.6 http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf 1: J Infect Dis 1980 Aug;142(2):205-8 Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates. Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC. Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation. PMID: 6997404 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract NOPE, you folks in the media will not print the truth, just rubber stamp the bull shit the feds are telling you, while the agent continues to spread $$$ [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE SAFEGUARDS (comment submission) https://web01.aphis.usda.gov/regpublic.nsf/0/eff9eff1f7c5cf2b87256ecf000df08d?OpenDocument Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA https://web01.aphis.usda.gov/BSEcom.nsf/0/b78ba677e2b0c12185256dd300649f9d?OpenDocument&AutoFramed Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION] http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt Docket Management Docket: 02N-0273 - Substances Prohibited From Use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed Comment Number: EC -10 Accepted - Volume 2 http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html PART 2 http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be09.html PDF]Freas, William TSS SUBMISSION File Format: PDF/Adobe Acrobat - Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary Sr. [flounder@wt.net] Monday, January 08,200l 3:03 PM freas ... http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf Asante/Collinge et al, that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest _sporadic_ CJD; http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice in Manufacturing, Packing, or Holding Dietary Ingredients a Comment Number: EC -2 Accepted - Volume 7 http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm i am no doctor, i have NO PhDs, but the facts and 'sound science' speak for themselves. the amplification and transmission is not 'rocket science' ... Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518