Monday, January 05, 2009



IN regards to CWD, baiting, and game farms, science shows us that game farms can be nothing short of a Petri dish for prions. Science shows us also that BOTH (game farms and baiting) enhances the spreading of CWD, and with baiting, the enhancements comes from multiple routes i.e. feeding of TSE tainted feed to cervids and the horizontal transmission from humans by _non-natural_ enhancement of congregating cervids by feeding. So, if your feeding them 50 lb. bags of potentially tainted TSE feed, and or planting a food plot, or throwing a bucket of beets out, you enhance the potential for CWD. I see no political agenda for the ban on baiting and or the ban on game farms, if that is what it takes. ...


posted April 01, 2005 04:11 PM

-------- Original Message --------

Subject: A Risk-based Audit of the Captive/Privately-owned Cervid Industry in Michigan

Date: Fri, 1 Apr 2005 15:48:08 -0600

From: "Terry S. Singeltary Sr." <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!x-usc:mailto:flounder@WT.NET>

Reply-To: Bovine Spongiform Encephalopathy <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!x-usc:mailto:BSE-L@KALIV.UNI-KARLSRUHE.DE>

To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!x-usc:mailto:BSE-L@KALIV.UNI-KARLSRUHE.DE

##################### Bovine Spongiform Encephalopathy #####################

A Risk-based Audit of the Captive/Privatelyowned Cervid Industry in Michigan Michigan Department of Natural Resources Report Series Issue Report No. 1 March 10, 2005

Data Analysis and Final Report Committee Michigan Department of Natural Resources

D. OBrien, Wildlife Division (Chair) P. Bernardi, Law Enforcement Division S. Dubay, Wildlife Division S. Mayhew, Wildlife Division W. Moritz, Wildlife Division D. Purol, Law Enforcement Division ii

Executive Summary

Deer and closely related species such as elk (Cervus elaphus nelsoni), moose (Alces alces), and caribou (Rangifer tarandus), scientifically classified as members of the Family Cervidae are collectively referred to as Cervids. While the general public commonly considers cervids wildlife, cervids raised in enclosures and cared for by humans (variously called captive, privately-owned,1 or farmed) form a group distinct from free-ranging (i.e., wild) cervids. Management of these captive/privately-owned cervids (C/P-OC) presents a number of unique challenges and opportunities. Because C/P-OC management involves aspects relevant to both agriculture and resource conservation, both the Michigan Departments of Agriculture (MDA) and Natural Resources (MDNR) have responsibilities in C/P-OC regulation. Both agencies recognize the potential of diseases, specifically Chronic Wasting Disease and Bovine Tuberculosis, to negatively impact both privately owned livestock and wildlife in Michigan. The term Captive/Privately Owned Cervid Industry refers to the collective body of enclosures. This industry is composed of 740 facilities located throughout the State, ranging in size from less than 1 acre to over 5,000 acres. Facilities are classified into 4 categories based on function: Hobby, Exhibition, Ranch, and Full Registration. While Hobby and Exhibition are self explanatory, Ranches provide shooting opportunities, and Full Registration facilities provide breeding stock, shooting stock, and sale of live animals for hobby and exhibition operations. As a result of recommendations from the Michigan CWD Task Force and an Executive Order of the Governor, a risk-based audit of the states C/P -OC industry was carried out not to be punitive, but to find any flaws or weaknesses in the current system that might lead to the entrance of CWD into Michigans captive and wild cervid herds. With the cooperation of the MDAs Animal Industry Division and C/P-OC producers around the state, the Law Enforcement and Wildlife Divisions of the MDNR audited 584 C/P-OC facilities throughout the state between June 15, 2004 and October 26, 2004, of which 506 were active operations. Auditors collected data on a variety of factors related to the risk of introduction and spread of CWD in the state, including number and types of cervids held, the places from which they were obtained, how they were identified, the types, heights and conditions of fences, and information about CWD testing and escapes. During the period of the audit, audited facilities housed a total of 32,493 C/P-OC based on facility owner information. More than 30,000 (30,616 or 94.2%) of those animals were of species known or anticipated to be susceptible to CWD. The vast majority (25,976 or 84.8%) were white-tailed deer. Elk were second most abundant at 4,029 animals (13.2%), and 611 animals (2.0%) were red deer (Cervus elaphus elaphus). Full Registration facilities housed 13,840 (42.6%) C/P -OC while Ranches housed 18,394 (56.6%). Overall, auditors determined that 37% of all C/P-OC facilities were not in compliance with current regulations at the time of the audit. The principal areas of deficiency related to the identification of animals, the rate of CWD testing, conditions of fences, and the rate and reporting of escaped animals. In spite of the unique characteristics of CWD as a disease, many of the risks for its introduction and propagation identified during the course of this audit are recurring themes in the surveillance and control of other contagious diseases in other species. While many issues of note, both positive and n gative, were found in these inspections of Michigan C/P-OC facilities, the following stand out as deserving comments and recommendations: · Efforts to minimize the risks of introduction and propagation of CWD via C/P-OC in Michigan begin and end with individual animal identification. The current animal identification regulations are inadequate because they do not require facility owners to identify all C/P-OC or to identify them all in 1 The terms used to refer these animals differ between stakeholder groups. In Michigan, agricultural groups prefer the term privately-owned cervids, whereas natural resource groups more commonly recognize the term captive cervids. To avoid confusion of either group, for the purposes of this report they are referred to collectively as captive/privately-owned cervids (abbreviated C/P-OC). iii a unique and uniform way. A system must be implemented that is mandatory, uniform across all facilities and classes, and that provides unique and visible identification to each individual by which the animal can be traced throughout its lifetime. All animals must be identified by 1 year of age , and the appropriate state agency must issue and administer the identification system. The identification must also be easily visible so that each and every animal is clearly identified as a C/P -OC in the event of escape. In calling for this requirement, we understand that identification of every animal may be very difficult for Ranch facilities because of their size and their inherently less intensive management and handling of the animals. Nonetheless, individual animal identification is so critical to minimizing and managing disease risk that facilities such as Ranches that cannot reliably and verifiably identify each and every individual should be subject to more stringent and vigorously enforced fencing and biosecurity regulations to ensure that unmarked animals do not le ave the facility alive under any circumstances. · Along with animal identification, CWD testing of Michigan C/P-OC, or more accurately, the lack of testing, was the greatest risk for introduction and propagation of the disease identified during this audit. In spite of a mandatory testing program for all C/P-OC over 16 months of age that die plus a representative percentage of culls, nearly 90% of the reported C/P-OC deaths were not tested for CWD. While some facilities have tested in good faith, nearly half of the audited Ranch and Full Registration facilities reported that they had submitted no CWD tests at all. Without adequate CWD testing, the introduction of CWD into the States C/P -OC cannot be detected. More ominously, this same lack of testing means that we cannot rule out the possibility the disease is already here and currently propagating undetected. Steps have been taken jointly by MDA and MDNR to notify producers of testing requirements and provide information about sample submission (letter dated Nov 15, 2004). · The lack of a specified protocol for de-commissioning or de-registering a C/P-OC facility is a risk for introduction and propagation of CWD. Audit teams found a number of facilities that wanted to leave the C/P-OC business but had little guidance from regulations on how to decommission. As a result, understandably frustrated facility owners may deal with the situation in a way they deem appropriate, which, at worst, could mean releasing the ir C/P-OC into the free-ranging cervid population. Appropriate regulations should be developed speedily, and those regulations should provide for an outreach/education program to inform and assist C/P-OC producers who wish to leave the business and get rid of their animals. · Procedures to deal with facility abandonment, are conspicuously absent and critically needed. As an example, when inspectors visited a facility during the audit, fences were down, the C/P-OC were gone, and the owner had moved out of state. In such cases, given the currently inadequate regulatory provisions for individual animal identification and recordkeeping, there is no way to be sure what happene to the animals or verify the CWD risk those animals, or the land once used as a C/P-OC facility, pose to the free-ranging cervid population. Penalties for cases where an owner just walks away from a facility should be sufficiently severe to provide a strong deterrent for this unacceptable behavior. · Another area of risk for CWD introduction and propagation for which both C/P-OC facilities and regulating state agencies bear some burden of responsibility is thao inadequate recordkeeping. To the credit of the C/P -OC industry, the vast majority of inspected facilities not only keep records, but the records they keep were judged to be in compliance with current regulations. However, the current regulations are not particularly stringent when viewed in the context of what is required of a recordkeeping system in order to minimize disease risks. For example, most of the records kept are on paper, and while they comply with current regulations, lack of simultaneous accessibility of these iv records by the multiple parties necessary to ensure adequate disease surveillance presents an obvious risk. In addition to the issues discussed relative to animal identification, the State needs to reevaluate and improve the way it gathers and stores regulatory information from C/P-OC facilities so that the information is rapidly, efficiently, and widely accessible to multiple agencies and producers, and so that important data linkages are maintained. The development of an electronic data collection, archiving, and reporting system to aid compliance, enforcement, and disease risk assessment should be a high priority. Such a system is currently lacking, and its design, development, and implementation should involve both information technology and disease control specialists to ensure an adequate system is developed. · These audit findings also revealed the risk of C/P-OC escapes. In spite of the fact that reporting of releases is mandatory in current regulations, it is clear not only that escapes occur but that they are rarely reported. Of 464 escapes reported to audit inspectors, only 8 releases were apparently reported to MDA. Twenty percent of Class IV and about 14% of Class III C/P-OC facilities experienced escapes, which is likely to be an underestimate. Adding to the risk is the fact that only half of the escaped C/P -OC from Ranches bore identification. Most escaped C/P-OC were reported to have been recovered, yet the time allowed for reporting and recovery under current regulations is suffic ient to add substantial risk of CWD introduction even for recovered animals. The development of more stringent escape and recovery protocols, along with enforcement and stiffening of penalties for nonreporting, is critical. Consideration should be given to measures which would allow agencies to dictate the rapidity and conduct of recovery operations based on risk and automatically make unreported escaped C/P -OC public property and subject to immediate harvest. These protocols should include measures to explicitly provide authority to agencies to manage the harvest of nonnative cervid species. The Natural Resources Commission approved regulations to allow harvest of escaped exotic Cervids in January 2005. The documentation by this audit of another practice, the intentional release of C/P-OC into the wild, is also both notable and deeply troubling. · Uniform regulatory requirements for the composition and maintenance of perimeter fencing should be developed and enforced. Current regulations specify that fences be constructed only of woven wire, yet in practice, C/P-OC facilities use a variety of other materials that agencies consider to be in compliance with the standards. Some of these materials very likely are adequate. Updated regulations should include specific guidance such as (but not limited to) minimum gauge of wire, mesh size, and distance between posts. In addition, the revised regulations need to address the current problematic conflict in fencing standards, which both specify minimum fence heights by species, yet also specifyth t fences need to prevent the ingress and egress of any cervid species. We cannot overstate the crucial role of fences in minimizing the risks of CWD introduction and propagation. In spite of their similar appearances, C/P-OC and free-ranging cervids are separate populations from the standpoint of disease control, and the separation between those populations should be maintained at all times. Good fences not only protect free-ranging cervids from C/P-OC, but vice ers . · Some summary mention of Ranch facilities is warranted because of their unique characteristics and the unique risks they hold for CWD introduction and propagation. This audit found that of the 4 facility classes, Ranches enclosed the largest number of CWD-susceptible C/P-OC (>18,000 statewide), imported the largest numbers of C/P-OC from out-of-state sources (including from CWDpositive states), had the largest percentage of animals lacking individual identification, had the lowest rate of CWD testing, and had the lowest rates of recovery and identification of escapees. In addition, Ranch facilities are located in areas with some of the highest free-ranging WTD densities in the state. If CWD were to infect C/P-OC that subsequently escape from one of these facilities, propagation of CWD in the surrounding free-ranging population would likely be rapid. We do not intend these remarks to stigmatize all Ranch facilities. Some of the best managed C/P-OC facilities in the state are Ranches. However, because of this combination of factors that increase CWD risks, serious v consideration should be given to making registration and fencing requirements for Ranches more stringent than those for other classes of C/P-OC facilities. This may help provide greater assurance that registered facilities will be well managed and economically self-sufficient, and capable of providing needed disease surveillance and management safeguards. · An emerging issue with respect to the risks of CWD introduction and propagation is potential environmental contamination via the manure or carcasses of infected animals. This audit was able to gather some of the first information on the ways that C/P -OC facilities manage and dispose of these materials. This is an area where development of workable regulations should be an ongoing priority for both agriculture and natural resource agencies. While the attention paid to issues of carcass and manure management and disposal is likely to increase in the future because of recent research findings, agencies and the industry must also keep the place of these items in proper perspective within the context of the overall risks of CWD transmission. The available research and the current scientific opinions of preeminent CWD scientists agree that the highest risks for introduction and propagation of the disease are the movements of, and contact between, live animals. The role played by carcasses and manure from infected animals, while by no means negligible, is a distant second in terms of risk importance, with contamination of machinery and equipment an even more distant third. It is critical that disease control experts and policy makers keep this relative risk ranking in mind so that attention, as well as limited time and resources, are not diverted from the most important sources of CWD risk. · Measures of the overall non-compliance of C/P-OC facilities (37% of C/P-OC facilities judged noncompliant by audit inspectors) essentially speak for themselves. While the validity and meaning of these measures can be debated, clearly an appreciable amount of non-compliance exists among C/POC facilities, and there is substantial room for improvement. In many respects, identifying the need for improvements in the C/P-OC industry to minimize the risks of introduction and propagation of CWD, and even suggesting remedies, is the easy part of the process. Much more difficult is the task of finding and applying sufficient resources to make the remedies happen. Agencies and policy makers should harbor no illusions about the amount of funding, personnel, and time neede to ensure the implementation and enforcement of the measures suggested in this report. All will be sizeable, but such support will be necessary if Michigan is serious about minimizing disease risks. It is only fair to point out that many of the problems identified with respect to current C/P -OC regulations and their implementation may have been largely due to a failure to provide the money and expertise necessary to do the job properly. In the end, measures take to revent the introduction and spread of CWD to Michigan will benefit both free-ranging cervids and C/P-OC, and the methods devised to fund risk mitigation measures should reflect that fact. vi Acknowledgments The authors want to acknowledge that even though our names appear on this Final Report, others completed the vast majority of the work of this audit. They deserve a great deal of credit for their efforts, efforts made over and above work schedules that for most were already strained. Primary credit lies with the teams of Conservation Officers and Wildlife Biologists who conducted the audit inspections and with D. Dominic and D. Purol who organized, scheduled, and managed the logistical challenges of hundreds of inspections. Credit is also due to T. Pullen who helped in the organization of photographic data, to J. Kennedy and T. Riebow who spent many hours entering questionnaire data by hand, and to K. Gardiner, Y. Li, T. Oliver and M. Strong who provided mapping expertise. The members of the various audit planning and training committees are too numerous to name here, but their efforts to plan and organize the audit from scratch in less than a months time were indispensable and nothing short of amazing. Thanks are also due to the Animal Industry Division of the Michigan Department of Agriculture for providing full cooperation and access to their data and records. Finally, credit and thanks are extended to the captive/privately-owned cervid facilities of Michigan and their producer groups, whose cooperation under difficult circumstances allowed this audit to be completed. Preface In writing this Report, we had three primary goals: 1) to provide the best context we could for the potential disease risks (or lack of them) associated with the audits findings; 2) to comprehensively and accurately document the findings of the audit inspections in the interest of transparency; and 3) to meticulously document the environment, planning, and conduct of the audit, hopefully to provide some guidance and assistance to other groups or agencies faced with a similar task in the future. The Report is long, but this was the unavoidable consequence of our effort to be comprehensive. All audit data that could be summarized and presented in a reasonably concise way are included here, either in the Results themselves or in Appendices. The Report was not written with the intent that everyone would read it cover to cover. Rather, it is organized into sections which were intended to stand on their own. As a result, some issues are covered repeatedly. Of necessity, some topics overlap. The Table of Contents is organized so that a reader with a specific interest in a particular risk topic can locate that topic easily and view a summary of the audit findings relevant to it without having to read the entire report.

snip...Full Text 168 pages...TSS

" Captive/Privately Owned Cervid Facility Audit Report

PDF icon An audit of captive/privately owned cervid facilities that house deer, elk and other animals around the state showed that 37 percent of the facilities are not in compliance with current regulations for the industry. Executive Summary

Synopsis of the report


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Brief Communications
Nature 425, 35-36 (4 September 2003) doi:10.1038/425035a

Prion disease: Horizontal prion transmission in mule deer Michael W. Miller1 & Elizabeth S. Williams2


The gathering of deer during winter may foster the spread of chronic wasting disease.

Epidemics of contagious prion1 diseases can be perpetuated by horizontal (animal to animal) and maternal (dam to offspring, before or after birth) transmission2, 3, 4, 5, 6, 7, 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.


The accumulation of abnormal prion protein in the peripheral lymphatic tissue of a host species seems to be strongly related to the horizontal transmissibility of prion diseases, and so might be a better predictor of contagiousness than the prion strain itself. The demonstration of abnormal prion protein in gut-associated lymphoid tissues10,11 but not in placental tissues11 of mule deer infected with CWD is consistent with an alimentary shedding route8, as has been suggested for scrapie12. Both direct and indirect transmission of CWD can probably occur8, and concentrating deer in captivity or by feeding them artificially may facilitate transmission. The social behaviour of deer, particularly their tendency to gather and yard together during winter, might also foster epidemics in the wild. The efficiency of horizontal CWD transmission, exacerbated by behavioural ecology, might explain the high probability ofCWD’s becoming established in deer populations once it has been introduced either by natural spread or by the commercial movement of infected animals.

Transmission and Detection of Prions in Feces

Posted Jun 24 08 5:07pm The Journal of Infectious Diseases 2008;198:81–89 © 2008 by the Infectious Diseases Society of America. All rights reserved. 0022-1899/2008/19801-0015$15.00 DOI: 10.1086/588193


Transmission and Detection of Prions in Feces

Jiri G. Safar,1,2 Pierre Lessard,1 Gültekin Tamgüney,1,2 Yevgeniy Freyman,1 Camille Deering,1 Frederic Letessier,1 Stephen J. DeArmond,1,3 and Stanley B. Prusiner1,2,4

1Institute for Neurodegenerative Diseases, Departments of 2Neurology, 3Pathology, and 4Biochemistry and Biophysics, University of California, San Francisco, San Francisco

In chronic wasting disease (CWD) in cervids and in scrapie in sheep, prions appear to be transmitted horizontally. Oral exposure to prion-tainted blood, urine, saliva, and feces has been suggested as the mode of transmission of CWD and scrapie among herbivores susceptible to these prion diseases. To explore the transmission of prions through feces, uninoculated Syrian hamsters (SHas) were cohabitated with or exposed to the bedding of SHas orally infected with Sc237 prions. Incubation times of 140 days and a rate of prion infection of 80%-100% among exposed animals suggested transmission by feces, probably via coprophagy. We measured the disease-causing isoform of the prion protein (PrPSc) in feces by use of the conformation-dependent immunoassay, and we titrated the irradiated feces intracerebrally in transgenic mice that overexpressed SHa prion protein (SHaPrP). Fecal samples collected from infected SHas in the first 7 days after oral challenge harbored 60 ng/g PrPSc and prion titers of 106.6 ID50/g. Excretion of infectious prions continued at lower levels throughout the asymptomatic phase of the incubation period, most likely by the shedding of prions from infected Peyer patches. Our findings suggest that horizontal transmission of disease among herbivores may occur through the consumption of feces or foodstuff tainted with prions from feces of CWD-infected cervids and scrapie-infected sheep.

Received 9 October 2007; accepted 15 November 2007; electronically published 27 May 2008.

(See the editorial commentary by Bosque and Tyler, on pages 8-9.)

Potential conflicts of interest: none reported.

Financial support: National Institutes of Health (grants AG02132, AG010770, NS22786, and NS14069); G. Harold and Leila Y. Mathers Foundation; Sherman Fairchild Foundation.

Reprints or correspondence: Dr. Stanley B. Prusiner, 513 Parnassus Ave., HSE-774, San Francisco, CA 94143-0518 (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!

WLA December 2008 • Vol. 16, No. 6

Chronic Wasting Disease Poised to Cross Species Barrier By Vivian Pharis, AWA Board Member

In 2007 and early 2008, more than 5,000 deer were killed by hunters and another nearly 3,500 through culls in wildlife zones that lie roughly between Wainwright and Empress. Twenty-one mule and three white-tailed deer were found with CWD, bringing the total in the Alberta wild to 53 cases since first detected just over two years earlier. In 2008 the first wild Saskatchewan elk were found with CWD. A January 2008 Alberta government bulletin tried to put a positive spin on the situation, claiming that because the number of new CWD cases remains “low,” their culling programs are effective in their “aim to reduce and eventually eliminate CWD in Alberta.” AWA seriously questions such optimism in light of research which increasingly indicates that once contamination of soils occurs, CWD will persist and will spread from the environment to animals. Its expanding range in the U.S. wild despite hunter and official culls, and even the banning of game farms in some states, fuels our fears. A new live-animal test being trialed in 2008 on captured elk in Colorado’s heavily elk-populated Rocky Mountain National Park indicated an astounding CWD infection rate of 11 percent, or 13 of 117 animals. The area’s typical disease rate is 1 to 2 percent. CWD appears thoroughly ensconced in Colorado’s wildlife and has even crossed the Continental Divide to the west.


We are told CWD cannot infect us, so why the concern? Apart from the toll it is taking on our wildlife and the possibility of its spread to species like moose and caribou, there are disturbing new reasons for humans to be vigilant for their own safety. Velvet antler, full of blood and nerve tissue, was the lucrative product of Canada’s game farms, marketed mainly to South Korea for medicinal uses. But Korea, which had taken 86 percent of Canada’s velvet antler, said no to Canadian velvet in 2000 after CWD was discovered on game farms in Saskatchewan. Since then it has been a downhill slide for the game industry, which is now largely upheld through penned hunts in Saskatchewan and government subsidies in both provinces. What remains of the industry is making a desperate bid to sell velvet within North America, and plenty of websites extol its medicinal virtues. Since its efficacy has never been shown scientifically despite decades of research, the sales pitch has broadened from humans to the pet market. Type in “deer velvet” and “pets,” and hundreds of websites appear trying to sell velvet cures for Fuzzy and Fido’s various ills.


The world outside our Alberta and Saskatchewan backdoors has gotten scarier. And it’s looking more likely that Alberta government attempts to eradicate CWD from the wild through deer culling have about as much hope of success as does a deer that eats dirt-clinging prions. If your heart rate is still normal, then extrapolate from the conclusion of the Wisconsin group’s paper, and you may feel your hair stand on end. The group realized that all three soil types that act to enhance the effectiveness of TSE prions – bentonite, kaolin, and silica – are common food additives in our Western diet. They are used as fillers and stabilizers in everything from pills to

Saturday, September 06, 2008

Chronic wasting disease in a Wisconsin white-tailed deer farm 79% INFECTION RATE Contents: September 1 2008, Volume 20, Issue 5

Journal of Veterinary Diagnostic Investigation Vol. 20 Issue 5, 698-703 Copyright © 2008 by the American Association of Veterinary Laboratory Diagnosticians


Case Reports

Chronic wasting disease in a Wisconsin white-tailed deer farm

Delwyn P. Keane1, Daniel J. Barr, Philip N. Bochsler, S. Mark Hall, Thomas Gidlewski, Katherine I. O'Rourke, Terry R. Spraker and Michael D. Samuel Correspondence: 1Corresponding Author: Delwyn Keane, University of Wisconsin, Wisconsin Veterinary Diagnostic Laboratory, 445 Easterday Lane, Madison, WI 53706. mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!

In September 2002, chronic wasting disease (CWD), a prion disorder of captive and wild cervids, was diagnosed in a white-tailed deer (Odocoileus virginianus) from a captive farm in Wisconsin. The facility was subsequently quarantined, and in January 2006 the remaining 76 deer were depopulated. Sixty animals (79%) were found to be positive by immunohistochemical staining for the abnormal prion protein (PrPCWD) in at least one tissue; the prevalence of positive staining was high even in young deer. Although none of the deer displayed clinical signs suggestive of CWD at depopulation, 49 deer had considerable accumulation of the abnormal prion in the medulla at the level of the obex. Extraneural accumulation of the abnormal protein was observed in 59 deer, with accumulation in the retropharyngeal lymph node in 58 of 59 (98%), in the tonsil in 56 of 59 (95%), and in the rectal mucosal lymphoid tissue in 48 of 58 (83%). The retina was positive in 4 deer, all with marked accumulation of prion in the obex. One deer was considered positive for PrPCWD in the brain but not in the extraneural tissue, a novel observation in white-tailed deer. The infection rate in captive deer was 20-fold higher than in wild deer. Although weakly related to infection rates in extraneural tissues, prion genotype was strongly linked to progression of prion accumulation in the obex. Antemortem testing by biopsy of recto–anal mucosal-associated lymphoid tissue (or other peripheral lymphoid tissue) may be a useful adjunct to tonsil biopsy for surveillance in captive herds at risk for CWD infection.


Title: Chronic wasting disease in a Wisconsin captive white-tailed deer farm


Keane, Delwyn - U OF WIS, WIS VET DIAG LA Barr, Daniel - U OF WIS, WIS VET DIAG LA Bochsler, Philip - U OF WIS, WIS VET DIAG LA Hall, S - USDA, APHIS, VS, NVSL Gidlewski, Thomas - USDA, APHIS, VS O`rourke, Katherine Spraker, Terry - CO STATE UNIVERSITY Samuel, Michael - US GEOLOGIC SERVICE

Submitted to: Journal of Veterinary Diagnostic Investigation Publication Type: Peer Reviewed Journal Publication Acceptance Date: May 5, 2008 Publication Date: N/A

Interpretive Summary: Chronic wasting disease is a fatal disease of deer and elk. Clinical signs, including weight loss, frequent urination, excessive thirst, and changes in behavior and gait, have been reported in mule deer and elk with this disorder. Clinical signs in captive white tailed deer are less well understood. In a previous study, a captive facility housed 200 deer, of which half were positive for the disease with no clinical signs reported. In this study, we examined 78 white tailed deer from a captive facility with a history of chronic wasting disease and no animals with clinical signs. Examination of the brain and lymph nodes demonstrated that the abnormal prion protein, a marker for disease, was observed in 60 of the deer. Biopsy of the rectal mucosa, a test that can be performed on live deer, detected 83% of the infected animals. The prion genetics of the deer was strongly linked to the rate of infection and to disease progression. The results demonstrate that clinical signs are a poor indicator of the disease in captive white tailed deer and that routine testing of live deer and comprehensive necropsy surveillance may be needed to identify infected herds. Technical Abstract: Chronic wasting disease CWD is a transmissible spongiform encephalopathy or prion disease of deer and elk in North America. All diseases in this family are characterized by long preclinical incubation periods following by a relatively short clinical course. Endpoint disease is characterized by extensive deposits of aggregates of the abnormal prion protein in the central nervous system,. In deer, the abnormal prion proteins accumulate in some peripheral lymphoid tissues early in disease and are therefore suitable for antemortem and preclinical postmortem diagnostics and for determining disease progression in infected deer. In this study, a herd of deer with previous CWD diagnoses was depopulated. No clinical suspects were identified at that time. Examination of the brain and nodes demonstrated that 79% of the deer were infected. Of the deer with abnormal prion in the peripheral lymphoid system, the retropharyngeal lymph node was the most reliable diagnostic tissue. Biopsy of the rectal mucosal tissue, a site readily sampled in the restrained or chemically immobilized deer, provided an accurate diagnosis in 83% of the infected deer. The retina in the eye of the deer was positive only in late stage cases. This study demonstrated that clinical signs are a poor indicator of disease, supports the use of the retropharyngeal lymph node as the most appropriate postmortem sample, and supports a further evaluation of the rectal mucosal tissue biopsy as an antemortem test on a herd basis.


Minnesota has announced the finding of CWD in a captive elk in Aitkin County. The animal was a five-year-old male. It had been purchased from a captive facility in Stearns County in August of 2000. The herd where the elk was found has been placed under quarantine as has two additional facilities where the infected elk had resided prior to it coming to the farm in Aitkin County. Minnesota DNR officials will test wild deer in the area to determine if there is any sign of CWD in the free-ranging population. This is the first case of CWD in either captive or freeranging cervids in Minnesota. Several more states have passed bans on the importation of deer and elk carcasses from states where CWD has been found in wild animals. Previously the states of Colorado, Illinois and Iowa and the province of Manitoba had passed such bans. The states of Vermont, Oregon and Missouri have enacted similar bans. Numerous states have issue voluntary advisories to their out-of-state hunters encouraging them not to bring the carcass or carcass parts of deer and elk into their state. The bans do permit the importation of boned out meat, hides or cape with no meat attached, clean skull cap with antler attached, finished taxidermy heads or the ivories of elk. The state of Georgia has recently banned the importation of live cervids into that state also. Some citizens of Colorado have formed a new political action group called Colorado Wildlife Defense (just happens that the acronym is CWD). The stated goal of this group are; Elimination of big game diseases, especially CWD; promotion of healthy wildlife habitat; promotion of scientifically sound wildlife research; promotion of a discussion of the ethics of hunting and wildlife management; education of the hunting and non hunting public. Their action plan calls for; requiring double fencing of all game farms at owners expense; all game farmers provide annual proof of bonding; prohibit new licenses for deer and elk farms; prohibit expansion in acreage of existing game farms; prohibit the transfer of game farm licenses; prohibit charging for hunting behind high wire; prohibit blocking of traditional migratory paths by high fences; requiring game farms to maintain environmental controls and prohibit the escape of contaminated water or soil; requiring immediate reporting of missing deer or elk from game farms; and requiring all game farm deer and elk to be tested for brucellosis and TB. Wisconsin has announced that 7 more free-ranging deer have tested positive for CWD. They have expanded their eradication zone by an additional 15 square miles to cover these findings. The total number of free-ranging CWD positive in Wisconsin is now 31 white-tail deer.

In 2000, a elk farmer in Wisconsin received elk from a CWD exposed herd in Colorado. At that time, the farmer advised the Wisconsin Department of Agriculture that both animals from the exposed herd in Colorado were dead. He has now advised Wisconsin Ag. that he was mistaken and that one of the animals is still alive in his herd. The second draft of the implementation documents for the National CWD Plan was distributed to committee members and others on Friday, August 30. The final documents are due to APHIS and USFWS on Friday, September 13. The herd of captive elk in Oklahoma that had been exposed to CWD will be destroyed this week. This herd had an elk test positive for CWD in 1997 but the depopulation of the herd was not agreed to by the owners and federal representatives until this week. Since the discovery of CWD in the herd, the remaining animals have been under quarantine, however, in the meantime the herd has dropped from 150 animals to 74. Due to a lack of communication, not all of the 76 animals that died in the interim were tested for CWD. All remaining animals will be tested but the true degree of infection rate of the herd will never be known.

The owners of the facility will not be permitted to restock the area with cervids for a period of five years. A New York based organization, BioTech Research Fund I LLC has committed a $1 million line of credit to fund commercialization of tests for brain-wasting disorders and production of various vaccines to Gene-Thera of Wheat Ridge, Colorado. Gene-Thera has spent three years developing new ways not only to diagnose CWD, but create vaccines for mad cow disease, E. coli contaminants and foot-and-mouth disease. Its tests for CWD have been successful in more than 100 samples from Colorado and Wisconsin according to company officials. Gene-Thera plans to license and market some o fits disease test kits by the end of the year, then begin volume distribution by mid-2003. The abstracts of the presentations from the CWD Conference in Denver August 6 and 7 have been posted on the Colorado Division of Wildlife web site. You will need adobe acrobat reader to read them. The Division web site is: If you don’t have adobe acrobat, you can download it free at

Minnesota: Second case in a game farmed elk discovered in Stearns Co. This is a trace forward from the previously affected game farm in Aitkins Co. An additional game farm in Benton Co is under quarantine.


Supporting Documents: Colorado: CWD-Exposed Elk Used in 1990 Study- Wildlife officials call W. Slope move a mistake Date: January 17, 2003 Source: Denver Post Contacts: Theo Stein Environment Writer

The Colorado Division of Wildlife knowingly used a herd of captive elk exposed to chronic wasting disease in a grazing study on the Western Slope in January 1990, possibly introducing the disease to the elk-rich area. "It was a bad call," said Jeff Ver Steeg, the division's top game manager. "I can't deny it." About 150 wild elk were allowed to graze in the same pens near Maybell after the research herd was removed and may have picked up the abnormal protein that causes the disease from the feces and urine left by the captive elk. While the Division of Wildlife has expressed concern before that its animals might have helped spread CWD, this is the first time the agency has acknowledged it knowingly moved elk exposed to CWD deep into an area where the disease was not known to already exist. Studies that could help determine the source of CWD on the Western Slope are incomplete, and officials say what data that do exist are so new and so spotty they may not provide all the answers. So far, it appears that less than 1 percent of deer and elk in the area are infected, compared with as much as 15 to 20 percent in hotspots in northeastern Colorado. But as wildlife officials grapple with CWD's appearance in northwestern Colorado, officials now admit the decision to continue the grazing study over the objections of some biologists was an error. At the time, biologists wanted to see whether elk grazing on winter range depleted forage that ranchers wanted for fattening cattle in spring. "I think in hindsight a lot of good people probably did some dumb things, myself included," said Bruce Gill, a retired wildlife manager who oversaw research efforts and remembers the debate over the project. "Had we known CWD would explode into such a potentially volatile ecologic and economic issue, we wouldn't have done it." Elk ranchers, who have been blamed for exporting the disease from its stronghold on the Colorado and Wyoming plains to seven states and two Canadian provinces, say the agency's belated disclosure smacks of a coverup. "It's pure negligence," said Jerry Perkins, a Delta banker and rancher who is now demanding a legislative inquiry. "If I'd have moved animals I knew to be infected around like that, I'd be in jail." Grand Junction veterinarian and sportsman Dick Steele said he faults the agency for not disclosing information about CWD-exposed research animals before October, when information was posted on the Division of Wildlife website. "This went way beyond poor judgment," he said. "My main concern is that this has been hidden for the last 12 years. It would have been real important to our decision-making process on how to deal with CWD." While the Maybell information is new, Perkins and other ranchers have long suspected Division of Wildlife research facilities near Meeker and Kremmling, which temporarily housed mule deer kept in heavily infected pens at the Fort Collins facility, have leaked CWD to the wild. Fear of an outbreak led the agency to sample 450 deer around the Meeker and Kremmling facilities. None tested positive, but the sample size was only large enough to detect cases if the infection rate was greater than 1 percent. This fall, tests on 23,000 deer and elk submitted by hunters statewide have revealed 48 CWD cases north of Interstate 70 and west of the Continental Divide. Biologists believe the infection rate in that area, which includes the Maybell, Meeker and Kremmling sites, is still well below 1 percent. But CWD has never been contained in a wild population, so experts fear the problem will grow worse.

The Division of Wildlife says it will be months before a statistical analysis of the fall's sampling results can be completed, an exercise that may shed light on the disease's origin on the Western Slope. "We're just not going to speculate at this point," said Ver Steeg of the possible Maybell connection. "This is one possibility, but certainly not the only possibility." Some biologists think a defunct elk ranch near Pagoda, which had dozens of unexplained deaths in the mid-'90s, is another, a suggestion Perkins rejects. "It may be inconclusive to them," said Perkins. "It isn't inconclusive to us."

A Guide to Reporting on Chronic Wasting Disease December 2002 National Wildlife Federation


Game Ranching

Game ranching and CWD intersect at several points. It is likely that the crowded conditions at some game ranches where captive animals congregate at man-made feed and water stations are conducive to a more rapid transmission of the disease from infected to non-infected animals than in the wild. In one extreme case, more than 50 percent of all the elk in a Nebraska game ranch tested positive for CWD. In addition, as noted above, the transport of captive game animals from one state to another has probably introduced the disease into some areas where it had not previously existed. This concern recently led several states to enact bans on the import of captive deer and elk. Infection in a captive herd also may be spread to wild animals by interactions through fencing, or due to downed fencing.

Game Ranching and Cervid (Deer and Elk) Import Bans


Michigan State law: Emergency moratorium banned entry of cervids April 26, 2002 (reindeer allowed with permits). Game ranches: 980. Regulated by: Michigan Department of Agriculture, contact: Dr. Rachel Zesar, State Veterinarian, Division of Animal Industries: (517) 241-1557.

States sorry they ever allowed game farms

Elk & game farming in other states

CWD in Captive Cervids Although chronic wasting disease (CWD) was recognized in captive deer held in publicly owned research facilities in Colorado in the 1960s, it was not detected in privately owned commercial cervids until 1996 in Saskatchewan. Since then, 1 or more cases of CWD have been confirmed in elk in 39 captive herds in Saskatchewan. Primary or secondary epidemiological links have been found between every infected herd and a single source herd in the province that apparently imported infected elk from a private South Dakota herd in 1989. In another example of international movement of CWD, the disease was diagnosed in captive elk in Korea that reportedly had been imported from Saskatchewan. It was announced in early 2002 that one infected captive elk herd had been found in Alberta, and an epidemiological investigation is underway. CWD is a reportable disease under Canada's federal Health of Animals Act, and the Canada Food Inspection Agency has responsibility for controlling the disease. Nearly 8,000 captive elk in Saskatchewan have been destroyed in eradication efforts to date at a cost of more than $19,000,000 for indemnification and cleanup. In 1997, South Dakota became the first state to detect CWD within the commercial elk industry. As of May 2002, CWD has been diagnosed in 20 privately owned elk herds in Colorado (7), South Dakota (7) Kansas (1), Montana (1), Nebraska (3), and Oklahoma (1). Epidemiological investigations have disclosed animal movement among some of the infected elk ranches. A group of interconnected facilities near Rapid City, South Dakota, that experienced particularly severe infection appeared to be the original source of CWD for other South Dakota game farms. Additional investigations have revealed that two herds of the infected elk in Nebraska originated in Colorado, infected elk in Oklahoma apparently originated in Montana, and CWD subsequently was confirmed in the

Colorado and Montana source herds. In late 2001, CWD was found in captive elk shipped from an infected Colorado elk ranch to two herds in Colorado and one herd in Kansas. Over the course of several years, exposed animals from the source herd in Colorado had been shipped to captive elk operations in 19 states as well as to more than 40 other commercial facilities in Colorado. The other living elk shipped from this facility have been traced, euthanized, and tested for CWD with negative results. All but two of the infected captive herds found in the United States have been depopulated. One Nebraska herd was quarantined, monitored for additional cases of CWD, and released from quarantine after no more cases were found in 3 years. The Oklahoma herd has not been depopulated despite the recognition of infected elk in 1998, but it recently was appraised in preparation for euthanasia, testing, and disposal of the animals. As is the case with the Oklahoma herd, there are other situations where there was an extended delay between the diagnosis of CWD and herd depopulation. In several instances this delay occurred while arrangements were made with state and federal animal health authorities for compensation of the owner. Indemnification for captive elk destroyed in CWD control efforts is one component of the proposed program currently being developed by USDA's Animal and Plant Health Inspection Service (APHIS). The APHIS program, which was requested by the commercial elk industry, is being put together with their assistance as well as with input from animal health officials, wildlife management agencies, and other organizations. Drafts of the proposed program have been informally reviewed, and a formal document is being prepared for publication in the Federal Register. The major components of the program are indemnification for elk destroyed in CWD-control efforts, a monitoring and certification program for captive elk herds, and for herd plan guidelines for positive facilities. The programs as currently proposed would be voluntary unless interstate shipment of animals is involved and will apply only to captive elk with no provisions for other cervid species. In September 2001, the USDA issued a declaration of emergency regarding CWD, and approximately $2.6 million were made available in Commodity Credit Corporation (CCC) funds for indemnification and surveillance. In February 2002, an Interim Rule was published in the Federal Register regarding CWD indemnification and another $12.2 million in CCC funds were released to support CWD control efforts. In addition to compensating owners for the destruction of positive or exposed cervids, the USDA recently purchased approximately 1,350 elk in the northeastern portion of Colorado where CWD is endemic in wild deer and elk. Owners of nearly all of the captive elk herds in the endemic area participated in this buyout and agreed to never restock their property with cervids because of the continued disease risk. There will be no new captive cervid facilities constructed in Colorado's endemic area. Requests for similar federal assistance are expected from other states that recently have found CWD in wild animals. Individual states may implement more stringent regulations than are outlined in the proposed federal CWD control program. In response to the identification of CWD in captive and wild cervids in several locations, numerous states recently have imposed bans or stringent regulations on live cervid movement. In some states, these regulations apply to animals originating from areas where CWD has been detected, while in others there is total prohibition of live cervid importation. Additionally, some states now require mandatory CWD surveillance of captive deer and elk. (Prepared by John Fischer) Additional sources of information:

Canadian Food Inspection Agency ( SCWDS BRIEFS, April 2002, Vol. 18, No. 1 -13- USDA's Animal and Plant Health Inspection Service (


In addition to cases in captive research and free-ranging deer and elk, CWD has been diagnosed in privately owned elk on game farms in several states beginning in 1996. Infection has been particularly severe in a group of interconnected facilities near Rapid City, South Dakota that appear to be the original source of infection for other South Dakota game farms as well as the Saskatchewan epidemic. In contrast, infected elk in two of three Nebraska farms originated in Colorado, and infected elk in Oklahoma apparently originated in Montana; CWD has been confirmed in the Montana and Colorado source herds.

The incidence of CWD can be remarkably high in captive cervid populations. In one infected research facility,

more than 90% of mule deer over 2 years died or were euthanized due to illness from CWD.(5)

Recently, a high CWD prevalence (about 50%) has been demonstrated in white-tailed deer confined at an infected Nebraska elk farm.(6)

Among captive elk, CWD was the primary cause of adult mortality (five out of seven, 71%; four out of 23, 17%) in two research herds and a high prevalence (59%) was detected in a group of 17 elk slaughtered from an infected game farm herd.(7)

The potential for density-dependent disease transmission is greater among animals in captivity than in free-ranging wildlife. Captive animals are often held at higher than natural densities and thus are more frequently in direct contact and are more consistently stressed.(2)(3) Their repeated exposure to the same (potentially contaminated) soil may exacerbate effects of density on captive cervids.

CWD may be transmitted between captive and wild cervid populations, in either direction, and there is concern that transmission between cervids and cattle is possible, but this has only been demonstrated experimentally.(8) To date, cattle have rarely become infected when experimentally inoculated with CWD via intracerebral injection.(8)

The History of Chronic Wasting Disease

Dr. Trent Bollinger, CCWHC

One World, One Health Symposium

Sept. 29, 2004


In 1981 CWD was recognized in a free-ranging elk in Colorado. Subsequent surveillance of free-ranging cervids indicated the disease was present in northern Colorado and SE Wyoming. The overall prevalence of disease is given in the slide; however in some deer management zones the prevalence was approximately 15%. Through the 1980s and 1990s the disease was thought to be confined to this area.

In 1996 CWD was diagnosed in a farmed elk in Saskatchewan and a second case was diagnosed in 1998. Both of these index cases could be linked to a farm in west-central Saskatchewan and ultimately to elk imported from South Dakota in the late 1980s. CWD likely had been on the farms in South Dakota and Lloydminster since the 1980s and had gone undiagnosed. CWD was eventually detected in 40 Saskatchewan game farms as part of a CWD management and surveillance program. Farmed cervids in Saskatchewan have been free of the disease for the last 2 years however the eradication program has cost well in excess of 30 million dollars. In 1994 and 1997 a total of 125 elk were shipped from Saskatchewan to South Korea from a farm that was subsequently found to have CWD and 1 of these elk was confirmed positive for CWD in 2001. This is the only case of CWD outside North America.

A surveillance program to look for CWD in wild cervids in Saskatchewan began in 1997 and the first case of CWD in a mule deer was diagnosed in 2000. We now have diagnosed CWD in 32 mule deer and 2 white-tailed deer from 3 geographically distinct areas as shown on this map.

This scenario has played out in areas of the United States as well. Wisconsin detected their first case of CWD in wild white-tailed deer in 2001 in a relatively localized area in the south-central area of the state. This was followed by detection of the disease in wild white-tails in northern Illinois. About the same time CWD was detected in a single wild mule deer in southern New Mexico, in wild cervids in Nebraska and South Dakota and more recently cases have been detected in Utah and there has been spread to new foci in Colorado and Wyoming.

This map shows where CWD has been detected in farmed and wild cervids. In some jurisdictions the disease has since been eradicated from farmed species. Nowhere has it been eradicated in the wild. The known distribution of this disease has changed dramatically over the last 5 years in part due to increased surveillance and detection of the disease, in part due to natural spread and in part due to humans moving CWD infected deer and elk to new areas and in some cases with subsequent spread to wild cervids.


Department of Agriculture & Markets News Wednesday, April 13, 2005 Contact: Jessica A. Chittenden 518-457-3136 mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/! DEC TO TEST FOR CHRONIC WASTING DISEASE IN HAMILTON COUNTY

DEC Contact: Michael Fraser

(518) 402-8000

The New York State Department of Environmental Conservation (DEC) today announced it will sample approximately 20 wild deer in the Town of Arietta, Hamilton County in order to test for chronic wasting disease (CWD).

Recently, CWD was found in two captive white-tailed deer herds in Oneida County, marking the first incidents of CWD in New York State. The State Department of Agriculture and Markets (DAM) completed testing for CWD on the two herds in an effort to control the possible spread of the disease in New York State. Five of the 22 deer from the two captive herds tested positive for CWD.

DAM has been investigating other captive deer herds associated with the index herds. One of those herds that supplied deer in the mid-1990s to the index herd in Oneida County was located in the Town of Arietta in Hamilton County. Since the herd no longer exists and the owner of the herd is now deceased, DEC will sample approximately 20 wild deer the area surrounding the former captive herd to determine if there is any reason to believe the former captive herd may have been infected with CWD.

The sample tissues will be sent to the New York State Veterinary Diagnostic Laboratory at Cornell University to be analyzed for CWD. Results of the analysis will be made available as soon as they are confirmed, which is expected to take approximately seven to 10 days.

In addition to Hamilton County, DEC has initiated intensive monitoring of the wild deer population surrounding both herds in Oneida County to determine if CWD has spread to wild deer. Extending the effort to the specified area in Hamilton County will provide essential information on the health of wild deer and assist DEC and DAM in possibly identifying the source of the disease in New York State.

CWD is a transmissible disease that affects the brain and central nervous system of certain deer and elk. There is no evidence that CWD is linked to disease in humans or domestic livestock other than deer and elk.

More information on CWD can be found at the State Department of Agriculture and Markets’ website at, at DEC’s website at or the State Department of Health’s website at



Test results from the two white-tailed deer herds confirmed positive for Chronic Wasting Disease (CWD) in Oneida County that were sampled earlier this week have revealed three additional deer infected with CWD. The New York State Veterinary Diagnostic Laboratory at Cornell University conducted the CWD tests on the twenty deer, and notified the Department of Agriculture and Markets (DAM) of the three positive test results, late last night. The three white-tailed deer that tested positive for CWD all came from the index herd, owned by John Palmer, who at the time had 18 deer on his premises. Although DAM is still investigating the source of the infection, the prevalence of CWD in the Palmer herd provides some indication that the disease may be a more recent infection. The prevalence may also provide clues as to the source of infection and the risk to other captive herds and the surrounding wild deer population.

The detection of CWD in New York was due to the State's aggressive surveillance and monitoring program, which requires all captive deer herds to conduct routine sampling. After confirmation of two positive CWD cases in two separate captive white-tailed deer herds in Oneida County last week, DAM ordered the depopulation and testing of both herds in an effort to control the possible spread of the disease in New York State. No additional positives were found in the two white-tailed deer tested at the second confirmed herd, owned by Martin Proper.

The New York State Department of Environmental Conservation (DEC) plans to conduct intensive monitoring of the wild deer population surrounding both herds to determine if CWD has spread to wild deer. DEC will begin sampling the surrounding wild deer population next week to determine CWD status. Input and assistance from the public and local landowners will be essential in DEC's sampling efforts. DEC and DAM will hold two public meetings to inform the public of efforts being made in Oneida County in response to CWD. The first meeting will be held tonight, Friday, April 8, 2005, at 7 p.m. at the Oneida County Cornell Cooperative Extension office in Oriskany. The second meeting will be held tomorrow, April 9, 2005 at 1:00 p.m. at the Westmoreland Middle School. CWD is a transmissible disease that affects the brain and central nervous system of certain deer and elk. There is no evidence that CWD is linked to disease in humans or domestic livestock other than deer and elk.

More information on CWD can be found at the State Department of Agriculture and Markets' website at <>, at the State Department of Environmental Conservation's website at <> or the State Department of Health's website at <>. ###

Department of Agriculture & Markets News Friday, April 08, 2005 Contact: Jessica A. Chittenden 518-457-3136 mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!

Three Additional Deer test positive for CWD

ALBANY, N.Y. Three more captive deer in New York have tested positive for chronic wasting disease.

Agricultural officials say the three deer belonged to the same Oneida County herd that yielded the first positive last week. There are now five deer in New York with confirmed cases of C-W-D. All of the cases come from captive herds -- though state officials are checking to see if the deadly disease spread into the wild.C-W-D is a transmissible disease that affects the brain and central nervous system of certain deer and elk. There is no evidence that C-W-D is linked to disease in humans or domestic livestock other than deer and elk. Copyright 2005 Associated Press. All rights reserved. This material may not be published, broadcast, rewritten, or redistributed.

Directions to Oneida County Cornell Cooperative Extension

From the NYS Thruway:

Take Exit 32 (Westmoreland) and turn left onto Cider Street. Turn left onto Judd Road. Drive approximately two-thirds of a mile and take a left onto Second Street. The office is on the left, with a sign posted "Oneida County Farm and Home Center."

From Rome: Take Route 233 South to Sutliff Road. Drive past the Sheriff's Department to a traffic light. Proceed through the intersection to second building on the right.

From Utica: Take Oriskany Boulevard to Route 69. Drive through Whitesboro past Oriskany and turn left onto Airport Road. Drive 1 mile, and turn left at traffic light. Building is second on the right.


Subject: CWD disease detected on Lac qui Parle County cervid farm southwestern Minnesota (2006-03-15) Date: March 15, 2006 at 12:36 pm PST

Chronic wasting disease detected on Lac qui Parle County cervid farm (2006-03-15) The Board of Animal Health announced today that chronic wasting disease (CWD) has been detected in one domestic white-tailed deer on a cervid farm in Lac qui Parle County, which is located in southwestern Minnesota.

Immediately, DNR officials will conduct a local deer survey to determine the number of wild deer in the area. It is expected that not many deer will be found because the area is highly agricultural, with little deer habitat surrounding the farm. DNR will conduct opportunistic sampling of deer, like road kills, in the immediate area now and will conduct intensive hunter-harvested surveillance during the 2006 firearm deer season.

Although this positive animal is a captive deer, DNR has conducted surveillance for CWD in wild deer in the area. The farm is located near the northern boundary of deer permit area 447, where wild deer surveillance for CWD last occurred in 2003.

Lou Cornicelli, DNR big game program coordinator, said, "In 2003, we conducted wild deer CWD surveillance in adjoining permit areas 433, 446 and 447. In total, we collected 392 samples from those permit areas during the regular firearm deer season and CWD was not detected."

The sampling of wild deer was designed statistically to have a 95 percent confidence of detecting a 1 percent infection rate, according to Mike DonCarlos, DNR wildlife programs manager.

"This situation is very similar to the positive elk farm discovered in Stearns County in 2003, which followed the first discovery of CWD in an Aitkin County elk farm," DonCarlos said. “The DNR response will be similar to the Stearns County action and will include an initial assessment of wild deer populations in the area and development of a surveillance program for next fall."

From 2002 to 2004, DNR staff collected nearly 28,000 CWD samples statewide and no disease found in the wild herd.

"The intensive surveillance conducted in 2003 indicated CWD was not present in wild deer," Cornicelli said. “In addition, all indications are that this positive captive deer has not contacted any wild deer, but we will conduct additional surveillance this fall to be sure."

Subject: CWD disease detected on Lac qui Parle County cervid farm southwestern Minnesota (2006-03-15) Date: March 15, 2006 at 12:36 pm PST Chronic wasting disease detected on Lac qui Parle County cervid farm (2006-03-15) The Board of Animal Health announced today that chronic wasting disease (CWD) has been detected in one domestic white-tailed deer on a cervid farm in Lac qui Parle County, which is located in southwestern Minnesota.

Immediately, DNR officials will conduct a local deer survey to determine the number of wild deer in the area. It is expected that not many deer will be found because the area is highly agricultural, with little deer habitat surrounding the farm. DNR will conduct opportunistic sampling of deer, like road kills, in the immediate area now and will conduct intensive hunter-harvested surveillance during the 2006 firearm deer season.

Although this positive animal is a captive deer, DNR has conducted surveillance for CWD in wild deer in the area. The farm is located near the northern boundary of deer permit area 447, where wild deer surveillance for CWD last occurred in 2003.

Lou Cornicelli, DNR big game program coordinator, said, "In 2003, we conducted wild deer CWD surveillance in adjoining permit areas 433, 446 and 447. In total, we collected 392 samples from those permit areas during the regular firearm deer season and CWD was not detected."

The sampling of wild deer was designed statistically to have a 95 percent confidence of detecting a 1 percent infection rate, according to Mike DonCarlos, DNR wildlife programs manager.

"This situation is very similar to the positive elk farm discovered in Stearns County in 2003, which followed the first discovery of CWD in an Aitkin County elk farm," DonCarlos said. “The DNR response will be similar to the Stearns County action and will include an initial assessment of wild deer populations in the area and development of a surveillance program for next fall."

From 2002 to 2004, DNR staff collected nearly 28,000 CWD samples statewide and no disease found in the wild herd.

"The intensive surveillance conducted in 2003 indicated CWD was not present in wild deer," Cornicelli said. “In addition, all indications are that this positive captive deer has not contacted any wild deer, but we will conduct additional surveillance this fall to be sure."

Summary of Chronic Wasting Disease in Nebraska Domesticated Cervine Animal Facility permits were issued to 73 cervid herds in Nebraska in January, 2006. These facilities contained 2,182 elk, 698 fallow deer, 102 Sika deer, 441 mule deer and about 103 deer of various other breeds. Herds with elk and mule deer are required to have all animals over 12 months of age identified with a visible plastic tag and a permanent form of identification (metal USDA eartag or tattoo). These herds are also required to keep a current herd inventory on all animals, which is verified annually by a Bureau of Animal Industry representative. All elk and mule deer over 16 months of age which die from any cause, are to be reported to the Bureau and have the brain stem submitted for Chronic Wasting Disease (CWD) testing. As of January 1, 2006 over 3,800 animals have been submitted since testing began in 1998. Four Nebraska herds have been found to be CWD-positive as a result of this testing.

The first case of CWD was found in a Cherry county elk farm in March of 1998. This herd has tested over 186 animals negative after finding only one positive animal, and was released from quarantine. The second positive herd was found in Cheyenne county in April of 1999 and was depopulated. A third herd in Sioux county was found positive in December of 2000. This herd was a hunt operation and had an elk pasture and a deer pasture. The elk pasture had 15 positive elk in a herd of 150 animals and the deer pasture had 98 positive whitetail deer in a herd of 196 animals. Both pastures were completely depopulated. One positive elk was found in a fourth herd during depopulation of cervid herds in the endemic area (see below) in 2002. No captive cervid herds in Nebraska are known to be infected with CWD at this time.

Import and movement restrictions have been enacted that require CWD herd certification for a minimum of 36 months for intrastate movement and 60 months for import from another state. Fifty-two herds have applied for and been granted CWD Monitored Status under the Nebraska Chronic Wasting Disease Cervid Monitoring Program.

Surveillance in wild deer began in 1997 and the first wild deer to be found positive was in the fall of 2000. Since that time 24,849 wild deer have been tested in Nebraska with a total of 94 positive animals found. Map 1, shows where the positive animals were located.

Chronic Wasting Disease.

In May 2003, the NWRC received $500,000 from the USDA-APHIS-VS line item in the FY03 budget with Congressional language to address CWD. As a result, the NWRC Wildlife Disease Research Program developed a CWD research project, selected a Project Leader and devoted the remainder of FY03 to project planning and meeting with federal, state, and academic scientists involved with CWD. The initial funding was used to initiate the project and develop infrastructure and to establish cooperative research studies in several states. These field studies are providing basic information on CWD epidemiology, and are developing and implementing methods for decreasing prevalence and transmission within and among cervid species and between captive and wild cervids. Funding for research in FY04 increased 50% and has allowed us to expand ongoing collaborative studies and initiate new research. One of the primary concerns of APHIS was the potential transmission of CWD between captive and free-ranging cervids (Miller and 130 D. USAHA SCIENTIFIC PAPERS Williams, 2004) and NWRC has begun research to understand the rates and types of contacts between them and is developing cost-effective barrier techniques and strategies to reduce or eliminate contact. We are using track plots and motion-activated video to determine how common interactions through game farm fences are between farmed and wild cervids (mule deer [Odocoileus hemionus], white-tailed deer, and Rocky Mountain elk [Cervus elaphus nelsoni]). Our primary objective is to determine if disease transmission risk exists along game farm fences. We are evaluating 9 fences around elk farms in Colorado and 5 fences around white-tailed deer farms in Michigan. Track-plot data are collected bi-weekly and video data are collected continuously. Track plots document where animals visited the same point during a 24-hour period. Video documents when wild or farmed animals were at the fence and the nature of interactions. We are finding considerable variation in the species, sex, age class, and number of wild cervids that frequent game farm fence lines. Preliminarily, direct interactions between farmed and wild white-tailed deer (1) appear less common than between farmed and wild elk (71). We are using a Geographic Information System (GIS) to document relationships between farmed and wild cervid interactions and landscape attributes. Game farm management practices appear to impact fence-line activity. Stocking rates, proximity of males to females, feeding procedures, and fence construction all appear to contribute directly to the potential for interaction. Based on our results, we will develop recommendations for methods of reducing interactions. The NWRC is investigating and comparing the density, movement, and habitat use of white-tailed deer and mule deer and how these characteristics relate to the manifestation, transmission, and spread of CWD in Nebraska. In western Nebraska, where CWD occurs, NWRC is using telemetry to learn about the ranges and movements of mule and white-tailed deer. Concurrently, we are conducting fine-scale surveillance to locate infected deer. Deer range in the area centers on the North Platte River, and the potential exists that CWD could move east along the river rather quickly if management actions are not taken. At the same time, we are continuing a long-term study of the ecology of deer along the Missouri River. Data from this study will be used in the development of models and formation of management decisions. Investigations of a potential CWD vaccine using scrapie prions in mouse and rabbit models have begun at NWRC as well as collaboration on the development of rapid tests to identify prions in biological and environmental samples and on methods to decontaminated surfaces and environmental samples. Studies are planned to investigate the role of predators and scavengers in the possible transmission and/ or dissemination of CWD and to improve capture and census techniques for wild cervids.




February 17, 2006


5. Predicted population effects on free-ranging elk based on captive elk chronically exposed to the CWD prion. Forty-three female elk calves were trapped at the National Elk Refuge and transported to Sybille in February 2002. Elk were housed in pens, assumed to be environmentally contaminated with the CWD prion. Elk will be held throughout their lifetimes. Elk dying will be examined and cause of death determined. From these data, it will should be possible to model free-ranging elk mortality and population dynamics under extreme circumstances of CWD prion exposure and transmission. As of December 2005 (46 months post capture), 11 of 43 elk have died due to CWD. This compares to 100% mortality in less than 25 months in elk orally inoculated with different dosages of the CWD prion.



correct urls.....sorry;



This story is featured in Montana Outdoors September–October 2003


Though CWD has not been found in Montana’s wild deer or elk populations, in 1999 it was diagnosed in nine captive elk on an alternative livestock facility (game farm) near Philipsburg. All the animals there were destroyed and the facility was quarantined. Montana voters passed an initiative the following year that prohibits transfer of existing game farm licenses, ends new licensing, and forbids shooting captive elk. Still, roughly 75 of the facilities still exist, selling elk for meat, antlers, and breeding.

Date: 31 Mar 2002 From: ProMED-mail <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!> Source: Rocky Mountain News, 30 Mar 2002 [edited],1299,DRMN_21_1059119,00.html

Nebraska: Wasting illness hit 50% of herd

About half of the white-tailed deer within an 800-acre enclosure in northwestern Nebraska have tested positive for chronic wasting disease, a staggering total surpassing by far any known infection rate for the deadly disease.

Though test results from the herd have trickled in for months, the new numbers -- 79 positives out of 154 deer -- are nearly final, pending a handful of additional lab results. They show a spread of the disease unmatched outside of research pens in Fort Collins.

It's tempting to assume that chronic wasting disease began out of nowhere in the Fort Collins research pens or on the Wyoming Game and Fish Department's Sybille Wildlife Research Unit in Southeastern Wyoming. Analysis of the infection rates in wild deer and elk along with mathematical modeling suggest that the Fort Collins area may, in fact, be one focus of infection, but deer from the Colorado research facility were routinely moved to Sybille and vice versa. The Wyoming research pens seem to be another long-term focus of infection. All the research deer tract their lineage back to the wild, so there is some chance that the disease was brought into the research units. Speculation on the source of the disease is easy to find, but it’s likely that no one will ever know for sure how or where CWD started.

CWD occurred principally in two locations, this one at Sybille and in a similar faccility at Fort Collins, Colorado, some 120 miles southwest. It was estimated that in total probably 60-70 cases of CWD have occurred.

It was difficult to gain a clear account of incidence and temporal sequence of events (-this presumably is data awaiting publication - see below) but during the period 1981-1984, 10-15 cases occurred at the Sybille facility.

The moribidity amongst mule deer in the facilities ie. those of the natural potentially exposed group has been about 90% with 100% mortality.


Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.


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.***


Epidemiology Epidemiologic studies suggest that animals contract the disease by external exposure to the infectious agent, such as by eating contaminated feed. No evidence suggests that the TME agent spreads by contact between unrelated mink or from mother to nursing young. The disease has been identified in both genders and all color phases in animals greater than 1 year old. The first documented TME outbreak in the United States occurred in 1947 on one ranch in Wisconsin and then on a ranch in Minnesota that had received mink from the Wisconsin ranch. In 1961, TME outbreaks occurred on five ranches in Wisconsin. In Factsheet Veterinary Services February 2002 APHIS 1963, outbreaks occurred in Idaho, Minnesota, and Wisconsin. Epidemiologic data from the Minnesota and Wisconsin outbreaks trace the cases in those States to one common purchased food source.


The 1985 Stetsonville Outbreak The most recent TME outbreak occurred on one mink ranch in Stetsonville, WI, in 1985. In the herd of 7,300 adult mink, 60 percent of the animals died. Clinical signs included tail arching, incoordination, and hyperexcitability. At the most advanced stages of the disease, the animals were in trancelike states and eventually died. The outbreak lasted 5 months. Microscopic examination of sections of the brain confirmed the spongelike changes characteristic of TME. Diagnostic tests identified the prion protein. The following year, mink born during the outbreak showed no signs of TME. The late Richard Marsh, a veterinary virologist at the University of Wisconsin who studied the transmission of TME and other TSE’s, investigated this outbreak. Marsh learned that the mink were fed a diet composed of fresh meat products from “downer cattle” and commercial sources of fish, poultry, and cereal. Downer cattle are nonambulatory and cannot rise because they are affected with a condition such as a metabolic disease, broken limbs, or a central nervous system disorder. Marsh theorized that the meat from these downer cattle introduced a TSE agent to the mink in which TME resulted. Although Marsh’s hypothesis is based on speculation and anecdotal evidence, in 1993 APHIS adjusted its national BSE surveillance program to include testing downer cattle for evidence of a TSE. The brains of more than 20,141 cattle have been examined at APHIS’ National Veterinary Services Laboratories and other State diagnostic laboratories. Not a single tissue sample has revealed evidence of BSE or another TSE in cattle.

AND as everyone knows, the rest is history, those dead-stock downers, the most high risk cattle, were NOT tested, and in FACT, was a major source of YOUR CHILDRENS SCHOOL LUNCH PROGRAM, all across the Nation. sorry, these are the most high risk cattle for TSE aka mad cow disease, and i am a bit touchy about this topic. ...sorry. ...terry



In April of 1985, a mink rancher in Wisconsin reported a debilitating neurologic disease in his herd which we diagnosed as TME by histopathologic findings confirmed by experimental transmission to mink and squirrel monkeys. The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle and a few horses. She had never been fed.

We believe that these findings may indicate the presence of a previously unrecognized scrapie-like disease in cattle and wish to alert dairy practitioners to this possibility.



NOW, back to those mad mink i.e. TME. let me throw a curve ball here ;

Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model Thierry Baron,* Anna Bencsik,* Anne-Gaëlle Biacabe,* Eric Morignat,* and Richard A. Bessen† Emerging Infectious

Transmissible mink encepholapathy (TME) is a foodborne transmissible spongiform encephalopathy (TSE) of ranch-raised mink; infection with a ruminant TSE has been proposed as the cause, but the precise origin of TME is unknown. To compare the phenotypes of each TSE, bovine- passaged TME isolate and 3 distinct natural bovine spongiform encephalopathy (BSE) agents (typical BSE, Htype BSE, and L-type BSE) were inoculated into an ovine transgenic mouse line (TgOvPrP4). Transgenic mice were susceptible to infection with bovine-passaged TME, typical BSE, and L-type BSE but not to H-type BSE. Based on survival periods, brain lesions profi les, disease-associated prion protein brain distribution, and biochemical properties of protease-resistant prion protein, typical BSE had a distint phenotype in ovine transgenic mice compared to L-type BSE and bovine TME. The similar phenotypic properties of L-type BSE and bovine TME in TgOvPrP4 mice suggest that L-type BSE is a much more likely candidate for the origin of TME than is typical BSE. Transmissible mink encephalopathy (TME) is a rare prion disease in ranch-raised mink (Mustela vison) in North America and Europe (1–4). Six outbreaks have been reported from 1947 through 1985 in North America, and several have been linked to contaminated commercial feed (1). Although contamination of feed with scrapie-infected sheep parts has been proposed as the cause of TME, the origin of the disease remains elusive. The idea that scrapie in sheep may be a source of TME infection is supported by fi ndings that scrapie-infected mink have a similar distribution of vacuolar pathologic features in the brain and the same clinical signs as mink with natural and experimental TME (5). However, mink are not susceptible to scrapie infection following oral exposure for up to 4 years postinoculation, which suggests that either the scrapie agent may not be the source of natural TME infection or that only specifi c strains of the scrapie agent are able to induce TME (6,7). Epidemiologic investigations in the Stetsonville, Wisconsin, outbreak of TME in 1985 suggested a possible cattle origin, since mink were primarily fed downer or dead dairy cattle but not sheep products (8). Experimental transmission of Stetsonville TME into cattle resulted in transmissible spongiform encephalopathy (TSE) disease with an incubation period of 18.5 months. Back passage of bovine TME into mink resulted in incubation periods of 4 and 7 months after oral or intracerebral inoculation, respectively, which was similar to that found following inoculation of Stetsonville TME into mink by these same routes (8). These fi ndings indicated that cattle are susceptible to TME, and that bovine-passaged TME did not result in a reduced pathogenicity for mink. These studies raised the question as to whether an unknown TSE in cattle was the source of TME infection in the Stetsonville outbreak. Several additional TME outbreaks in the United States have been associated with mink diet that contained downer or dead cattle (9). ...

snip...full text ;

CWD, is but a small piece, of a very big puzzle. ...TSS

*** PLEASE NOTE, before i forget ;

THE IHC test has been proven to be the LEAST LIKELY to detect BSE/TSE in the bovine, and these were probably from the most high risk cattle pool, the ones the USDA et al, SHOULD have been testing. ...TSS

USDA 2003

We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back. Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS.

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.


Dr. Detwiler: It seems a good idea, but I'm not aware of it. Another important thing to get across to the public is that the negatives do not guarantee absence of infectivity. The animal could be early in the disease and the incubation period. Even sample collection is so important. If you're not collecting the right area of the brain in sheep, or if collecting lymphoreticular tissue, and you don't get a good biopsy, you could miss the area with the PRP in it and come up with a negative test. There's a new, unusual form of Scrapie that's been detected in Norway. We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS .

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.



Completely Edited Version PRION ROUNDTABLE

Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado



North American Cervids Harbor Two Distinct CWD Strains


Angers, R. Seward, T, Napier, D., Browning, S., Miller, M., Balachandran A., McKenzie, D., Hoover, E., Telling, G. 'University of Kentucky; Colorado Division of Wildlife, Canadian Food Inspection Agency; University Of Wisconsin; Colorado State University.


Despite the increasing geographic distribution and host range of CWD, little is known about the prion strain(s) responsible for distinct outbreaks of the disease. To address this we inoculated CWD-susceptible Tg(CerPrP)1536+/· mice with 29 individual prion samples from various geographic locations in North America. Upon serial passage, intrastudy incubation periods consistently diverged and clustered into two main groups with means around 210 and 290 days, with corresponding differences in neuropathology. Prion strain designations were utilized to distinguish between the two groups: Type I CWD mice succumbed to disease in the 200 day range and displayed a symmetrical pattern of vacuolation and PrPSc deposition, whereas Type II CWD mice succumbed to disease near 300 days and displayed a strikingly different pattern characterized by large local accumulations of florid plaques distributed asymmetrically. Type II CWD bears a striking resemblance to unstable parental scrapie strains such as 87A which give rise to stable, short incubation period strains such as ME7 under certain passage conditions. In agreement, the only groups of CWD-inoculated mice with unwavering incubation periods were those with Type I CWD. Additionally, following endpoint titration of a CWD sample, Type I CWD could be recovered only at the lowest dilution tested (10-1), whereas Type II CWD was detected in mice inoculated with all dilutions resulting in disease. Although strain properties are believed to be encoded in the tertiary structure of the infectious prion protein, we found no biochemical differences between Type I and Type II CWD. Our data confirm the co·existence of two distinct prion strains in CWD-infected cervids and suggest that Type II CWD is the parent strain of Type I CWD.

see page 29, and see other CWD studies ;

Sunday, November 23, 2008

PRION October 8th - 10th 2008 Book of Abstracts

Saturday, September 06, 2008 Chronic wasting disease in a Wisconsin white-tailed deer farm 79% INFECTION RATE Contents: September 1 2008, Volume 20, Issue 5

snip...see full text ;

Thursday, April 03, 2008

A prion disease of cervids: Chronic wasting disease

2008 1: Vet Res. 2008 Apr 3;39(4):41

A prion disease of cervids: Chronic wasting disease

Sigurdson CJ.


*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,


full text ;

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: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/! .



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.

Wednesday, December 17, 2008


The prion strain phenomenon: Molecular basis and unprecedented features


There is currently no mean to predict which will be the conformation of a newly generated strain and how this new PrPSc conformation could affect other species. One interesting new prion disease is CWD, a disease affecting farm and wild species of cervids [68,69]. The origin of CWD and its potential to transmit to humans are currently unknown. This is worrisome, considering that CWD has became endemic in some parts of USA and the number of cases continues to increase [69]. It is presumed that a large number of hunters in the US have been in contact or consumed CWD-infected meat [70]. CWD transmissibility studies have been performed in many species in order to predict how this disease could be spread by consumption of CWD meat [71–73]. In these studies, a special attention has been done to scavenging animals [74], which are presumed to be exposed to high concentration of cervid prions, resulting in the putative generation of many new forms of TSEs. Fortunately negative results were obtained in one experiment done in raccoons infected with CWD [74]. Transmission of CWD to humans cannot be ruled out at present and a similar infective episode to BSE involving CWD could result in catastrophic events, spreading the disease in a very dangerous way through the human population. No clinical evidence linking CWD exposed humans and CJD patients have been found [70], but experimental inoculation of CWD prions into squirrel monkeys propagated the disease [71]. It is important to mention that the species barrier between humans and cervids appears to be greater than with cattle, as judged by experiments with transgenic mice models [75]. Finally, it is important to be aware about CWD transmissibility to other species in which a “conformational intermediary” could be formed, facilitating human infection.


From: TSS (


Date: September 30, 2002 at 7:06 am PST

From: "Belay, Ermias"


Cc: "Race, Richard (NIH)" ; ; "Belay,


Sent: Monday, September 30, 2002 9:22 AM


Dear Sir/Madam,

In the Archives of Neurology you quoted (the abstract of which was

attached to your email), we did not say CWD in humans will present like

variant CJD.

That assumption would be wrong. I encourage you to read the whole

article and call me if you have questions or need more clarification

(phone: 404-639-3091). Also, we do not claim that "no-one has ever been

infected with prion disease from eating venison." Our conclusion stating

that we found no strong evidence of CWD transmission to humans in the

article you quoted or in any other forum is limited to the patients we


Ermias Belay, M.D.

Centers for Disease Control and Prevention

-----Original Message-----


Sent: Sunday, September 29, 2002 10:15 AM

To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!x-usc:mailto:ebb8@CDC.GOV



Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS


A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most

serious because of rapid horizontal spread and higher prevalence than BSE in

UK, up to 15% in some populations. Also may be a risk to humans - evidence

that it is not dangerous to humans is thin.

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:


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...full text ;

Volume 12, Number 10-October 2006


Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease

Samantha MaWhinney,* W. John Pape,? Jeri E. Forster,* C. Alan Anderson,?§ Patrick Bosque,?¶ and Michael W. Miller#

*University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA; ?Colorado Department of Public Health and Environment, Denver, Colorado, USA; ?University of Colorado School of Medicine, Denver, Colorado, USA; §Denver Veteran's Affairs Medical Center, Denver, Colorado, USA; ¶Denver Health Medical Center, Denver, Colorado, USA; and #Colorado Division of Wildlife, Fort Collins, Colorado, USA

Suggested citation for this article

The transmission of the prion disease bovine spongiform encephalopathy (BSE) to humans raises concern about chronic wasting disease (CWD), a prion disease of deer and elk. In 7 Colorado counties with high CWD prevalence, 75% of state hunting licenses are issued locally, which suggests that residents consume most regionally harvested game. We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD). The relative risk (RR) of CJD for CWD-endemic county residents was not significantly increased (RR 0.81, 95% confidence interval [CI] 0.40-1.63), and the rate of CJD did not increase over time (5-year RR 0.92, 95% CI 0.73-1.16). In Colorado, human prion disease resulting from CWD exposure is rare or nonexistent. However, given uncertainties about the incubation period, exposure, and clinical presentation, the possibility that the CWD agent might cause human disease cannot be eliminated.

snip... full text ;

full text ;

RE: USA: Safety of dietary supplements questioned by report; will approval seals work?

cwd, feeding, and baiting piles

Posted Sep 01 08 7:10pm

Oral transmission and early lymphoid tropism of chronic wasting disease PrPres in mule deer fawns (Odocoileus hemionus ) Christina J. Sigurdson1, Elizabeth S. Williams2, Michael W. Miller3, Terry R. Spraker1,4, Katherine I. O'Rourke5 and Edward A. Hoover1

Mule deer fawns (Odocoileus hemionus) were inoculated orally with a brain homogenate prepared from mule deer with naturally occurring chronic wasting disease (CWD), a prion-induced transmissible spongiform encephalopathy. Fawns were necropsied and examined for PrP res, the abnormal prion protein isoform, at 10, 42, 53, 77, 78 and 80 days post-inoculation (p.i.) using an immunohistochemistry assay modified to enhance sensitivity. PrPres was detected in alimentary-tract-associated lymphoid tissues (one or more of the following: retropharyngeal lymph node, tonsil, Peyer's patch and ileocaecal lymph node) as early as 42 days p.i. and in all fawns examined thereafter (53 to 80 days p.i.). No PrPres staining was detected in lymphoid tissue of three control fawns receiving a control brain inoculum, nor was PrPres detectable in neural tissue of any fawn. PrPres-specific staining was markedly enhanced by sequential tissue treatment with formic acid, proteinase K and hydrated autoclaving prior to immunohistochemical staining with monoclonal antibody F89/160.1.5. These results indicate that CWD PrP res can be detected in lymphoid tissues draining the alimentary tract within a few weeks after oral exposure to infectious prions and may reflect the initial pathway of CWD infection in deer. The rapid infection of deer fawns following exposure by the most plausible natural route is consistent with the efficient horizontal transmission of CWD in nature and enables accelerated studies of transmission and pathogenesis in the native species.


These results indicate that mule deer fawns develop detectable PrP res after oral exposure to an inoculum containing CWD prions. In the earliest post-exposure period, CWD PrPres was traced to the lymphoid tissues draining the oral and intestinal mucosa (i.e. the retropharyngeal lymph nodes, tonsil, ileal Peyer's patches and ileocaecal lymph nodes), which probably received the highest initial exposure to the inoculum. Hadlow et al. (1982) demonstrated scrapie agent in the tonsil, retropharyngeal and mesenteric lymph nodes, ileum and spleen in a 10-month-old naturally infected lamb by mouse bioassay. Eight of nine sheep had infectivity in the retropharyngeal lymph node. He concluded that the tissue distribution suggested primary infection via the gastrointestinal tract. The tissue distribution of PrPres in the early stages of infection in the fawns is strikingly similar to that seen in naturally infected sheep with scrapie. These findings support oral exposure as a natural route of CWD infection in deer and support oral inoculation as a reasonable exposure route for experimental studies of CWD.





Kunkle, Robert Hamir, Amirali Miller, Janice - ARS RETIRED Hall, S - USDA-VS-APHIS-NVSL, AMES Richt, Juergen Greenlee, Justin Williams, Elizabeth - UNIVERSITY OF WYOMING

Submitted to: United States Animal Health Association Proceedings Publication Type: Abstract Publication Acceptance Date: August 1, 2006 Publication Date: October 12, 2006 Citation: Kunkle, R.A., Hamir, A.N., Miller, J.M., Hall, S.M., Richt, J.A., Greenlee, J.J., Williams, E.S. 2006. Experimental transmission of chronic wasting disease (CWD) of elk (Cervus Elaphus Nelsoni), white-tailed deer (Odocoileus Virginianus), and mule deer (Odocoileus Hemionus Hemionus) to white-tailed deer by intracerebral route [abstract]. United States Animal Health Association. p. 221.

Technical Abstract: Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) affecting elk, white-tailed deer, and mule deer. Intra-species transmission of CWD is readily accomplished via oral administration of CWD-affected brain. And while the exact mode of natural transmission is unclear, horizontal transmission within species has been demonstrated. The TSE¿s are prion-associated diseases. Differentiable prion strains are associated with variations in clinical course and pathology in susceptible animal hosts. To determine the potential existence of CWD pathotype strain differences, groups of 5 white-tailed deer were inoculated by intracerebral route (IC) with 1 ml of 10% (wt/vol) brain homogenates derived from CWD-affected elk, white-tailed deer, and mule deer. Two non-inoculated deer served as negative controls. All deer were homozygous at PrP gene polymorphic sites 95 (glutamine) and 138 (serine). Deer homozygous (glycine/glycine) and heterozygous (glycine/serine) at codon 96 were approximately divided between treatment groups. One deer from each treatment group was euthanized 10 months post-inoculation (PI); findings between groups were similar and included limited or mild spongiform encephalopathy (SE) and immunohistochemical (IHC) detection of prion in lymphoid tissue follicles and in the CNS which was pronounced in subependymal areas. The remaining deer were euthanized at the terminal stage of disease. The clinical course of CWD appeared similar between groups. The survival period did not differ between groups, ranged from 14 to 26 months, with an average mean of 20 months. The severity of SE and magnitude of IHC staining appeared proportional to incubation period. Microscopic lesions in the CNS were typical of previously reported CWD SE including the presence of cerebral florid plaques. IHC staining was multifocally extensive to diffuse, and perineuronal, subependymal, and neuropil associated. Staining was pronounced in the midbrain and relatively sparse in the hippocampus. Differences in histopathologic and IHC findings between groups were not noted. Negative control deer sacrificed at 26 months PI did not have SE and were IHC negative. The composite findings indicate the clinical course and pathology of CWD in IC challenged white-tailed deer was not influenced by the inoculum source or PrP gene polymorphism at codon 96.

Last Modified: 01/04/2009

Subject: MAD DEER/ELK DISEASE AND POTENTIAL SOURCES Date: Sat, 25 May 2002 18:41:46 -0700 From: "Terry S. Singeltary Sr." Reply-To: BSE-L To: BSE-L

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April 9, 2001 WARNING LETTER


Brian J. Raymond, Owner Sandy Lake Mills 26 Mill Street P.O. Box 117 Sandy Lake, PA 16145 PHILADELPHIA DISTRICT

Tel: 215-597-4390

Dear Mr. Raymond:

Food and Drug Administration Investigator Gregory E. Beichner conducted an inspection of your animal feed manufacturing operation, located in Sandy Lake, Pennsylvania, on March 23, 2001, and determined that your firm manufactures animal feeds including feeds containing prohibited materials. The inspection found significant deviations from the requirements set forth in Title 21, code of Federal Regulations, part 589.2000 - Animal Proteins Prohibited in Ruminant Feed. The regulation is intended to prevent the establishment and amplification of Bovine Spongiform Encephalopathy (BSE) . Such deviations cause products being manufactured at this facility to be misbranded within the meaning of Section 403(f), of the Federal Food, Drug, and Cosmetic Act (the Act).

Our investigation found failure to label your swine feed with the required cautionary statement "Do Not Feed to cattle or other Ruminants" The FDA suggests that the statement be distinguished by different type-size or color or other means of highlighting the statement so that it is easily noticed by a purchaser.

In addition, we note that you are using approximately 140 pounds of cracked corn to flush your mixer used in the manufacture of animal feeds containing prohibited material. This flushed material is fed to wild game including deer, a ruminant animal. Feed material which may potentially contain prohibited material should not be fed to ruminant animals which may become part of the food chain.

The above is not intended to be an all-inclusive list of deviations from the regulations. As a manufacturer of materials intended for animal feed use, you are responsible for assuring that your overall operation and the products you manufacture and distribute are in compliance with the law. We have enclosed a copy of FDA's Small Entity Compliance Guide to assist you with complying with the regulation... blah, blah, blah...

Thursday, December 25, 2008

Elk meat recalled due to CWD Boulder County Health Department and Colorado Department of Public Health and Environment

Monday, December 1, 2008 When Atypical Scrapie cross species barriers

ONE FINAL THOUGHT on [QUOTE=Buckup;2485167][B]Spinning Animal Disease For Political Gain[/B]

FACTS speak for themselves here ;

Sunday, December 28, 2008

MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy



CATTLE With CNS Symptoms Were NOT Always Tested


Between FYs 2002 and 2004, FSIS condemned 680 cattle of all ages due to CNS symptoms. About 357 of these could be classified as adult. We could validate that ONLY 162 were tested for BSE (per APHIS records. ...


WE interviewed officials at five laboratories that test for rabies. Those officials CONFIRMED THEY ARE NOT REQUIRED TO SUBMIT RABIES-NEGATIVE SAMPLES TO APHIS FOR BSE TESTING. A South Dakota laboratory official said they were not aware they could submit rabies-negative samples to APHIS for BSE testing. A laboratory official in another State said all rabies-negative cases were not submitted to APHIS because BSE was ''NOT ON THEIR RADAR SCREEN." Officials from New York, Wisconsin, TEXAS, and Iowa advised they would NOT submit samples from animals they consider too young. Four of the five States contacted defined this age as 24 months; Wisconsin defined it as 30 months. TEXAS officials also advised that they do not always have sufficient tissue remaining to submit a BSE sample. ...



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.)

Wednesday, December 10, 2008

Evaluation of FSIS Management Controls Over Pre-Slaughter Activities (Audit Report 24601-7-KC) USDA CERTIFIED DEADSTOCK DOWNER COW SCHOOL LUNCH PROGRAM

Wednesday, August 20, 2008 Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?

A New Prionopathy OR more of the same old BSe and sporadic CJD

Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP],F2400_P1001_PUB_MAIL_ID:1010,39963

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

sporadic Fatal Familial Insomnia


Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam


Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003

doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI

Tracking spongiform encephalopathies in North America

Original TextXavier Bosch

"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem."

49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realized that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.

Singeltary was greatly demoralized at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.

Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.

To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.

Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticized for not having performed enough prionic tests in farm deer and elk.

Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.

Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.

Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modeled on that established in the UK, he points out.

Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunterstwo of whom were friendswho died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.

CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited.


>>> he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.<<<


MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:


I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported 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. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr Bacliff, Tex

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT

2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.

Creutzfeldt Jakob Disease


Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

CJD TEXAS (cjd clusters)


The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

Attending Dr.: Date / Time Admitted : 12/14/97 1228

UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 Pathology Report

FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858


I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.

NO test tube TSE by either Prusiner or Soto, to date, have ever produced a TSE identical to any natural case of sporadic CJD. IN fact, no test tube TSE has ever been produced that resembles _any_ natural field TSE.

IF you feed BSE tainted materials to cattle and primate, you have BSE i.e. nvCJD in humans.

IF you feed USA sheep scrapie strain to USA cattle, you get USA TSE, not like the typical UK BSE.

IF you feed USA tainted atypical h-BSE cattle to humans, what do you get ?

IF you feed sporadic CJD to primate you get a CJD infected primate. NOTHING spontaneous about it at all.

USA is in a very unique situation. there are more documented TSE in different species than any other country, all of which have been rendered and fed back to animals for human and animal consumption, for decades. Millions exposed, and of these Millions, how many surgical and dental procedures have been done on these exposed, to pass on to others, via the 'friendly fire' mode of transmission?

IF, the spontaneous TSE was true, then this would be Prusiner and everyone else that is trying to cash in on this agent with there TSE rapid test, this would be there dream come true. IT would require mandatory BSE/TSE testing of all species, due to the fact you could not ever eradicate it through any intervention.

85%+ of all CJD i.e. sporadic, all spontaneous without route(S), without source(S), just some protein that flips out in all but a few cases ???

How long can this charade continue $

How many more will become exposed and have to die $

UKBSEnvCJD only theory, i don't think so. ...

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE

Evaluation of the Human Transmission Risk of an Atypical Bovine Spongiform Encephalopathy Prion Strain

Qingzhong Kong,1* Mengjie Zheng,1 Cristina Casalone,2 Liuting Qing,1 Shenghai Huang,1? Bikram Chakraborty,1 Ping Wang,1 Fusong Chen,1 Ignazio Cali,1 Cristiano Corona,2 Francesca Martucci,2 Barbara Iulini,2 Pierluigi Acutis,2 Lan Wang,1 Jingjing Liang,1 Meiling Wang,1 Xinyi Li,1 Salvatore Monaco,3 Gianluigi Zanusso,3 Wen-Quan Zou,1 Maria Caramelli,2 and Pierluigi Gambetti1* Department of Pathology, Case Western Reserve University, Cleveland, Ohio 44106,1 CEA, Istituto Zooprofilattico Sperimentale, 10154 Torino, Italy,2 Department of Neurological and Visual Sciences, University of Verona, 37134 Verona, Italy3 *Corresponding author. Mailing address: Department of Pathology, Case Western Reserve University, Cleveland, OH 44106. Phone for Pierluigi Gambetti: (216) 368-0586. Fax: (216) 368-2546. E-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/! . Phone for Qingzhong Kong: (216) 368-1756. Fax: (216) 368-2546. E-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/! ?Present address: Department of Patient Education and Health Information, Cleveland Clinic Foundation, Cleveland, OH 44195. Received November 30, 2007; Accepted January 16, 2008.

Bovine spongiform encephalopathy (BSE), the prion disease in cattle, was widely believed to be caused by only one strain, BSE-C. BSE-C causes the fatal prion disease named new variant Creutzfeldt-Jacob disease in humans. Two atypical BSE strains, bovine amyloidotic spongiform encephalopathy (BASE, also named BSE-L) and BSE-H, have been discovered in several countries since 2004; their transmissibility and phenotypes in humans are unknown. We investigated the infectivity and human phenotype of BASE strains by inoculating transgenic (Tg) mice expressing the human prion protein with brain homogenates from two BASE strain-infected cattle. Sixty percent of the inoculated Tg mice became infected after 20 to 22 months of incubation, a transmission rate higher than those reported for BSE-C. A quarter of BASE strain-infected Tg mice, but none of the Tg mice infected with prions causing a sporadic human prion disease, showed the presence of pathogenic prion protein isoforms in the spleen, indicating that the BASE prion is intrinsically lymphotropic. The pathological prion protein isoforms in BASE strain-infected humanized Tg mouse brains are different from those from the original cattle BASE or sporadic human prion disease. Minimal brain spongiosis and long incubation times are observed for the BASE strain-infected Tg mice. These results suggest that in humans, the BASE strain is a more virulent BSE strain and likely lymphotropic.

Thursday, December 04, 2008 2:37 PM

"we have found that H-BSE can infect humans."

personal communication with Professor Kong. ...TSS

Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived from Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001 Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Public Submission APHIS-2006-0041-0006 Public Submission Title Comment from Terry S Singletary Sr

From: Terry S. Singeltary Sr.

To: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!x-usc:mailto:FREAS@CBER.FDA.GOV

Cc: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/! ; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!

Sent: Friday, December 01, 2006 2:59 PM

Subject: Re: TSE advisory committee for the meeting December 15, 2006 [TSS SUBMISSION


ONE FINAL COMMENT PLEASE, (i know this is long Dr. Freas but please bear with me)

THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted blood from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone.

These are the facts as i have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species. ...

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

snip... 48 pages...

Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary

Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008


Tissue infectivity and strain typing of the many variants Manuscript of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...


Docket APHIS-2007-0033 Docket Title Agricultural Bioterrorism Protection Act of 2002; Biennial Review and Republication of the Select Agent and Toxin List Docket Type Rulemaking Document APHIS-2007-0033-0001 Document Title Agricultural Bioterrorism Protection Act of 2002; Biennial Review and Republication of the Select Agent and Toxin List Public Submission APHIS-2007-0033-0002.1 Public Submission Title Attachment to Singeltary comment

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

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