Wednesday, June 01, 2011

Management of CWD in Canada: Past Practices, Current Conditions, Current Science, Future Risks and Options

Management of CWD in Canada: Past Practices, Current Conditions, Current Science, Future Risks and Options

February 9-10, 2011, Edmonton

Executive Summary

A workshop was held in Edmonton, Alberta, on February 9-10, 2011, as part of a two-step process to review and draft a proposed update to Canada’s National Chronic Wasting Disease Control Strategy. This Strategy was prepared for the Federal-Provincial/Territorial Resource Ministers Council in October, 2005. A copy may be viewed at: .

The Edmonton Workshop brought us up to date on the many developments regarding Chronic Wasting Disease (CWD) that have occurred since 2005.

We heard from U.S. state and Canadian provincial disease control experts as well as from academics about the significant spread of this disease in both farmed and wild cervids through Saskatchewan, into Alberta and perhaps beyond. Practitioners described the difficulty of designing programs to control CWD in wild cervids, especially when these involve culls or other measures that the public and hunters find difficult to support. Meanwhile, CWD eradication from farmed cervids has proven costly and had limited success. These developments and challenges have led state, provincial and Canadian federal officials to consider control rather than eradication as an objective of their programs.

We also heard about variable understanding of this disease by First Nations communities, hunters and the general public, as well as the challenges and uncertainties involved in calculating the full economic, social and cultural impacts of the disease.

On a more positive note, much has been learned about CWD since 2005. We now have important insights into its spread within herds by age and gender, as well as geographically, for example along river basins. Research is also improving our understanding of how the disease may spread between farmed and wild populations, insights that may lead to improved methods for bio-containment. These insights may improve disease control methods in the short and medium term. Meanwhile, observations on genetic susceptibility of host species and the impact of environmental factors on the disease agent may lead to new approaches to disease control in the longer term.

Last, but not least, we heard from a representative of the Public Health Agency of Canada and other speakers concerning expert views on the possibility that CWD might be transmitted to humans, including the possibility that this risk couldevolve with the changes to the agent itself and its spread in wild or farmed cervids.

These observations and discussions of the Edmonton workshop will be considered in the updating of the Strategy.



CWD Management in Wildlife and Farmed Stock

1. Chronic Wasting Disease in Colorado: Trends, Observations & Implications

Dr. Michael Miller, Senior Wildlife Veterinarian, Wildlife Health Program, Colorado Division of Wildlife

Chronic wasting disease has infected wild cervid populations in Colorado, USA, since at least the early 1980s. Knowledge about the distribution and occurrence of CWD has improved markedly since 2001. Cases have been diagnosed in all four of Colorado’s native cervid species (mule deer, white-tailed deer, elk, and moose), but occurs and is currently at higher rates in mule deer than in the other three species. CWD appears to be restricted to the cervid family, as no other native or domestic ruminants or other mammalian species have shown evidence of natural susceptibility.

Thus far there does not appear to be any evidence of selection for natural resistance. However, variations have been seen in the prion gene between and within cervid species that appear to be important in modulating the course of the disease and perhaps susceptibility.

Through the study of demographic and spatial patterns in Colorado over the past 10 to 15 years, a number of observations have been made on CWD. These include:

• Herd demographics, movement and congregation patterns appear to influence the rate of infection.

• There seems to be higher infection rates in males than in females, and infection rates tend to increase with age (at least to a point).

• There has been both natural and human assistance to the spread of CWD.

• Harvest strategies and other management practices may influence prevalence.

• The prevalence of CWD has been increasing.

There is evidence that the environment plays a role and that prions can be transmitted through environmental sources, such as soils and reservoirs. Clay-rich soils in particular have demonstrated an ability to increase the incidence of the disease (e.g., a 1% increase in clay content of soil led to a 9% increase in the odds of CWD infection in some areas). This correlation may be useful for assessing risk of outbreaks, identifying surveillance target areas, and developing disease control strategies.

Assessing CWD prevalence is difficult. For example, the apparent higher rate of CWD in older male cervids may bias estimates based on hunter-submitted samples because these cervids are more frequently harvested and tested in some areas. Wild cervids infected with CWD tend to die fairly quickly, so not many cases are seen by people. This also makes it difficult to convey the importance of the disease to the public.

Although some attempts were made to either control or contain CWD in Colorado, there was no clear evidence that they had an effect on the disease’s prevalence over the few years that control measures were applied. In addition, there was limited public support for policy changes, management approaches or surveillance strategies. The imperative to act was also reduced by the lack of evidence that CWD is readily transmissible to livestock and humans. As a result, these efforts were abandoned.

Although the CWD transmission process and disease detection methods are understood, the long-term ecological impacts are not. Practical management approaches that don’t affect sport hunting or recreation activities are needed.

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MANAGEMENT OF CWD IN CANADA: Past Practices, Current Conditions, Current Science, Future Risks and Options | February 9-10, 2011, Edmonton | Workshop Summary

2. Chronic Wasting Disease in Wisconsin: Management and Research Updates

Dr. Michael Samuel, Department of Forest and Wildlife Ecology, University of Wisconsin

The prevalence of chronic wasting disease in Wisconsin in 2002 was approximately 5% of the white-tailed deer population, located in a fairly concentrated area. Today, this percentage has increased significantly and there are two focal areas, one in the south central part of the state and one near the Wisconsin-Illinois border. These two areas appear to be growing together, even though the habitats and deer behaviour patterns are different between the two areas. Most of the data on CWD in Wisconsin has been collected in the south central area.

Wisconsin has very high deer density. The deer population in the south central area has more than doubled over the past 25 years. Even with the introduction of CWD, the population seems to be steady. Testing and monitoring of harvested deer in one of the affected areas has shown recent increases in disease prevalence especially in adult male deer.

Wisconsin’s initial CWD management goals were to limit the spread of the disease from known infected areas, eradicate the disease in known infected areas, expand surveillance of both wild and captive cervid populations, and to increase scientific knowledge and understanding of CWD. Hunters were considered a key partner in implementing solutions; however, their contributions were not as effective as anticipated. Wisconsin has now shifted from a CWD eradication approach to a CWD containment policy that includes prevention, monitoring and response. However, there is limited state funding and few concrete, tested strategies.

Additional emphasis has also been given to developing a public relations message about CWD management and public contribution to containment efforts. To this end, Wisconsin hired a private marketing firm to survey hunters and landowners. Results showed that hunters are better informed about the effects of CWD in Wisconsin than in other CWD affected areas. Many consider CWD to be a moderate to serious problem, one that will increase if not addressed. More than half support disease containment or control measures, but do not support reducing the size of the deer herd.

Current CWD research includes studies on density-dependent transmission, which has indicated that CWD transmission to young deer is related to the abundance of infected deer on the landscape. These results suggest that strategies for disease control should consider both CWD prevalence and deer density. Evaluation of PrP genotypes in Wisconsin deer has shown much higher infection rates and lower post-infection survival of deer with the common homozygous PrP. Although genetic selection for resistance may be occurring, this is likely to be a slow process with little potential for CWD management in wild deer populations.

Other research areas include selection pressure and PrP genotypes, the rate of CWD spread, modelling transmission and management strategies, landscape genetics for disease spread, infected mothers and fawns, CWD detection in environment, vaccine tests and herd immunity.

Understanding the rate of CWD spread is important for predicting future CWD distribution. A preliminary estimate of disease spread appears to be one to two miles per year. However, the Wisconsin deer population has very "simple" behaviour patterns with limited migration.

MANAGEMENT OF CWD IN CANADA: Past Practices, Current Conditions, Current Science, Future Risks and Options | February 9-10, 2011, Edmonton | Workshop Summary

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3. The Perils of Chronic Wasting Disease Regulation

Dr. Greg Douglas, Chief Veterinary Officer, Saskatchewan Ministry of Agriculture

Since the detection of chronic wasting disease in Saskatchewan in 1996 in farmed elk, a variety of regulatory approaches have been attempted at both the federal and provincial level. As of February 1, 2011, 64 cervid farms have been infected with CWD in Canada (all but two have been in Saskatchewan). There are currently seven farms under quarantine in Saskatchewan, and 12 farms have been declared "highly contaminated premises" and are under permanent quarantine.

From 1996 to 2002, evidence suggested that CWD occurred through direct transmission. Since 2002, direct transmission by farmed cervids is not as apparent and indirect transmission is increasingly considered a factor. In some cases, investigators were unable to determine the source of the CWD introduction, as multiple possibilities exist (environment, soil, feed, etc.).

CWD has been a federally reportable disease since 2001. An eradication approach is currently in place; however, policy will be shifting to one of control and zoning. This involves killing of the herd and all trace-outs up to 36 months; quarantine and surveillance of trace-outs from 36 to 60 months; and cleaning and disinfection. Compensation is provided to producers. In addition, mandatory provincial surveillance programs exist in four provinces (Manitoba, Alberta, Saskatchewan and the Yukon) and all on-farm deaths are tested. A national CWD voluntary herd certification program for producers has been in place since 2003, and is monitored by the Canadian Food Inspection Agency (CFIA). Wild cervid surveillance is under provincial/territorial authority. The federal Health of Animals Act requires a permit prior to movement of both wild and captive cervids within Canada.

The cervid industry includes three main components: the hunt farm industry (only in Saskatchewan and Quebec); the velvet industry (medicinal use of antlers, mainly for export); and the meat (venison) industry. The collective contribution to Saskatchewan’s economy is approximately $15M (cash receipts) annually, with the hunt industry accounting for more than two-thirds of that amount. The economic impacts of hunting in Saskatchewan are approximately $200M annually.

Most states and provinces allow farming of domestic cervids, and most jurisdictions allow the importation of cervids. Ninety percent of imported cervids, which are predominantly from Alberta, go to hunt farms. In 2005, the farmed cervids population in Saskatchewan was approximately 54,000. By 2010, the population had declined to 22,000 (6,000 white-tailed deer and 16,000 elk). The number of licensed game farms has also declined, from 616 in 2002 to 421 in 2011.

In the early years of the cervid industry, mandatory surveillance, movement permits, traceability systems, testing, and other regulations were accepted by the industry. The provincial regulations package has been a challenge to implement.

CWD has social, political, economic, environmental and other challenges. The control of CWD should take a "one health" approach that involves wildlife, environment and human health interests. Saskatchewan believes that we have a responsibility to prevent the spread of CWD to wild populations if it is possible to do so.

MANAGEMENT OF CWD IN CANADA: Past Practices, Current Conditions, Current Science, Future Risks and Options | February 9-10, 2011, Edmonton | Workshop Summary

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4. The Alberta Advantage: Integrated Surveillance, Management and Research during the Early Stages of Chronic Wasting Disease Intrusion–

Dr. Margo Pybus, Provincial Wildlife Disease Specialist, Fish and Wildlife Division, University of Alberta (presenting); Dr. Mark Ball and Jim Allen, Fish and Wildlife Division, University of Alberta; Drs. Evelyn Merrill, David Coltman, and Vic Adamowicz, University of Alberta

Alberta has been actively engaged with chronic wasting disease since 1998 when ongoing surveillance of wild and farmed cervids began. As of February 8, 2011, we have detected 91 cases of CWD in wild cervids in Alberta: 82 cases were mule deer and 9 were white-tailed deer; 30 animals were female and 61 were male. There is a significantly lower survival rate for adult male mule deer.

Ongoing research in Alberta on deer biology has shown that mule deer and white-tailed deer share the landscape, although there are local areas of only mule deer or only white-tailed deer. The majority of both deer species are sedentary, occupying relatively small summer and winter ranges. Mule deer have a significantly higher winter group size. However, the home range does not change as deer density increases.

Implications for CWD include:

•Genetic relatedness appears to be a factor in CWD infection: CWD-positive deer are more closely related than CWD-negative deer. Genetic relatedness is limited to about 1 km.

•There is some evidence that CWD is becoming more established in the deer population, as there has been a significant increase in the number of animals with late stage CWD.

•There appears to be a close correlation between CWD infection and watersheds, with the disease appearing to spread within continuous deer habitat along river valleys.

•There is also indication that the disease is continuing to spread westward from the core Saskatchewan/Alberta border areas.

From 2005 to 2008, Alberta took an aggressive approach to CWD control. If a deer hunted in the fall tested positive for CWD, a targeted winter cull would be undertaken within the area where the positive deer was taken. This local herd reduction efficiently removed other infected deer and created a lower deer density, thereby reducing the risk of transmission among the remaining deer. During these years, the overall prevalence of CWD noted through hunter surveillance was steady at around 0.15%. Since the disease control was stopped, the prevalence has risen to 0.25% in 2009 and 0.40% in 2010.

Research on public perceptions related to CWD indicated that:

•70% of Albertans are aware of CWD, although they are uncertain about its effects.

•80% of Albertans support eliminating CWD from the province.

•The hunting public is more concerned with increased prevalence than increased spread of CWD.

•Albertans are willing to pay higher taxes to reduce the perceived threat.

•Despite some aversion to culling, aggressive management efforts are generally well-received.

The success of any surveillance and management program is tied to public and political education and understanding. A key barrier to effective CWD management is the lack of political will to provide long-term funding support. This undermines the public message that CWD is of concern, and that its spread will continue in the absence of active control.

MANAGEMENT OF CWD IN CANADA: Past Practices, Current Conditions, Current Science, Future Risks and Options | February 9-10, 2011, Edmonton | Workshop Summary

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5. CWD in Wild Cervids in Saskatchewan

Dr. Trent Bollinger, Department of Veterinary Pathology, Western College of Veterinary Medicine, University of Saskatchewan

Chronic wasting disease was introduced into Saskatchewan through the importation of infected farmed elk from the United States in the late 1980s. Further cases were detected in other Saskatchewan game farms as part of the CWD management and surveillance program; these infections were linked back to the original source.

The disease then spread from the elk and deer farms to wild cervids in at least three locations in Saskatchewan, and was first detected in a wild mule deer near the Alberta border in 2000. Since then, CWD has spread to cover much of the south-western part of the province and into eastern Alberta; it has also spread easterly and is now within ~100 km of the Manitoba border.

Incentives for hunters were introduced to reduce deer populations in CWD areas, including free hunting permits, increases to the number of animals per license, and expansion of permits to allow harvesting of younger males and females. The programs had mixed results: in some areas the cervid population decreased; in other areas it increased. Over the ten years since the introduction of CWD, its prevalence in mule deer has increased to approximately 5% in some areas. One cluster or "hot spot" has a CWD infection rate of 25%.

Management efforts are now focused on tracking movement of the disease. Mobile CWD test stations are used to collect samples from hunters in zones where CWD has not yet been detected. Incentives are used to encourage hunters to submit samples.

Current research efforts are focused in predicting rates and direction of geographic spread; determining factors affecting transmission; predicting effects on cervid populations; and developing strategies for CWD management. Research findings include:

•No evidence of subpopulations, so no barriers to gene flow or CWD spread.

•Mule deer families within 2 km of one another are likely to be related; cervids have a matriarchal social structure.

•Within a local area, CWD infected animals are more likely to be related to one another than non-infected animals; CWD transmission is facilitated within female family groups.

•Focal herd reduction is not an effective management strategy to prevent CWD spread: by the time CWD is detected in a new area, it has likely been there for some time.

•Deer migration and movement of dispersing young deer can be up to 100 km; CWD management zones are typically too small to be effective.

•CWD is transmitted through both direct and indirect contact, but the relative role of each is uncertain.

Management strategies need to look beyond just reducing deer numbers to encompass multifaceted approaches for both wild and farmed cervids. A five-year commitment of funds and resources is needed in order to establish and evaluate CWD management strategies and control measures.

MANAGEMENT OF CWD IN CANADA: Past Practices, Current Conditions, Current Science, Future Risks and Options | February 9-10, 2011, Edmonton | Workshop Summary

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Public Awareness and Economic Impacts

6. An Update on the Economic Impacts of Chronic Wasting Disease and Hunting/Recreation/Wildlife

Dr. Vic Adamowicz, Department of Rural Economy, University of Alberta


Molecular Biology and Public Health

11. Chronic Wasting Disease Strains

Judd Aiken, Ph.D., Professor, Centre for Prions and Protein Folding Diseases, University of Alberta

CWD is a highly contagious brain disease that is always fatal and for which there is no treatment or cure. It is spread animal-to-animal and animal-to-environment. Detection of CWD is complicated and infected cervids appear healthy for a long time. The disease appears to spread slowly. The disease persists in the environment for decades, and in some cases it becomes more infectious in the environment.

Little is currently known about interspecies transmission; at this time, CWD appears to be restricted to cervids. Although both CWD and BSE, a known zoonotic, are prion diseases, there is no direct evidence of transmission of CWD to humans.

Prion strains are prion disease variants that can exhibit unique clinical symptoms, disease incubation period, biochemical characteristics of the prion protein, and transmission characteristics. Prion strains have been identified in virtually every species affected by these transmissible neurological disorders. The implications of prion strains relate to detection, tissue distribution of infectivity and inter-species transmission.

Studies have identified prion protein gene variants in white-tailed deer. Genetic analysis of hunter harvest data as well as experimental CWD transmission studies in deer demonstrate that two of the prion protein variants are linked to disease prolongation. Prions isolated from these variants exhibit unique biochemical characteristics indicative of unique CWD strains in white-tailed deer. There is also evidence of amino acid changes that indicate increased susceptibility to CWD. Other studies have indicated that there are differences in CWD between regions.

A study published in January 2011 suggests that CWD has the potential to transfer to humans. Using protein misfolding cyclic amplification (PMCA), a means of replicating prions in a test tube, CWD prion strains were shown to have the ability to convert to human prions.

Although it is known that CWD strains exist, they have not yet been fully characterized. The biology of CWD remains unknown.

12. Are We Prepared for Human Chronic Wasting Disease?

Zheng Wang, M.D., Canadian Creutzfeldt-Jakob Disease Surveillance System, Public Health

Agency of Canada (presenter); Michael B. Coulthart, PhD, Canadian Creutzfeldt-Jakob Disease

Surveillance System, Public Health Agency of Canada

The risk to human health of chronic wasting disease is poorly known. The current risk profile is drawn from results of animal and in vitro studies, including the following:

• Studies in transgenic mice expressing human PrP failed to demonstrate that CWD prions can induce prion disease after intracerebral inoculation. One study showed that the species barrier between cervids and humans may be strain-dependent. This suggests that other CWD strains, either naturally occurring or adapted in cervids from other species, could be highly transmissible to humans.

• In studies on non-human primates, squirrel monkeys succumbed to the prion disease following intracerebral and oral inoculation of CWD prions.

• In vitro studies using cell free conversion (CFC) experiments have suggested that transmission risk to humans is low as a result of incompatibility of the donor and recipient PrP sequence; however, it has not been ruled out.

• In vitro studies using PMCA have shown that CWD PrPSc may have the potential to trigger conversion of human PrPC after in vitro or in vivo adaptation and stabilization.

Assessment of these studies suggests that while a strong species barrier exists between cervids and humans, there is insufficient evidence to rule out a potential risk of CWD transmission to humans. Human surveillance to monitor occurrence of potential human CWD is needed, and reduction of the risk of human exposure to CWD remains strongly recommended.

More than twelve years of national Creutzfeldt-Jakob disease (CJD) surveillance in Canada has not detected any cases of human CWD. The appearance of CJD in CWD endemic provinces (Saskatchewan and Alberta) from 1998 to 2009 was consistent with known CJD characteristics worldwide. Available results from detailed case investigations do not suggest that any cases were causally linked with CWD.

Although the comprehensive national surveillance of CJD can reveal unusual disease phenotypes, instances of transmission could be missed due to imperfect case detection, unknown phenotype or subclinical infection. Improved collection and sharing of data on harvested (wild and farmed) CWD-positive animals and on CWD-positive cervid meat consumption would enhance the Canadian CJD surveillance program. New and/or improved collaborations are needed between animal and human health authorities within and between provinces, between federal and provincial authorities, and across federal departments and agencies.

Risk tolerance for human CWD is extremely low: a single case could create a crisis (e.g., regarding safety of the Canadian blood supply). Ongoing assessment of the magnitude of this risk and its potential downstream consequences is crucial. Given the likelihood that occasional human exposure to CWD is likely to continue and even increase as CWD spreads in cervid populations, maintenance of appropriate public health precautions therefore remains warranted.

13. Challenges in Managing Emerging Prion Risks: Applications to Chronic Wasting Disease Management in Canada

Dr. Margit Westphal, McLaughlin Centre for Population Health Risk Assessment, University of Ottawa

Lessons learned from bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob disease (CJD) can be applied to management approaches related to chronic wasting disease.

The first North American case of BSE was found in Canada in May 2003, leading the US and many other countries to close their borders to Canadian beef. The socio-economic cost of the outbreak was estimated to be in the range of $10 billion. It was also determined that vCJD can be transmitted through blood transfusion.

An integrated risk management framework for BSE and vCJD was developed over a five-year period. It drew from case studies from 27 countries and regions around the world and looked at risk management approaches and interventions, international trade policy, and lessons learned.

In Canada and the US, animal health practices have been implemented to control the spread of BSE, including bans on specified risk materials in fertilizers and feed and guidelines for handling risk materials. Canada is the first country in North America with a National Cattle Identification System, which supports the containment and eradication of BSE through its trace-back capabilities. Effective safeguards have also been implemented in the food chain and for blood donation.

As of March 5, 2010, there have been 18 reported cases of BSE and one case of vCJD in Canada (the person is believed to have consumed the contaminated meat in the U.K.). A mathematical probability model suggests that there are 200 undetected cases of BSE in Canada, but they would be older animals and not used for meat consumption.

In 2009, an expert panel on transmissible spongiform encephalopathy (TSE) was convened to evaluate the knowledge gaps related to prion disease risk using a structured elicitation procedure for weighting and pooling expert judgment. When asked, "What is the likelihood that chronic wasting disease can transmit to humans?", panel members responded that it is possible.

Another expert elicitation exercise will take place in May 2011 and will focus on knowledge gaps associated with CWD, including those related to disease transmission, species barriers, environmental exposure, prion resistance, infectious periods, diagnostic test development and vaccine development. A key objective will be to obtain the values needed for accurate mathematical modelling of CWD environmental transmission and spread.

CWD disease exists in 18 U.S. states and two Canadian provinces in both free-ranging and farmed cervids. Effective management of CWD requires the development and application of an integrated risk management framework based on sound principles of risk assessment and management, the harmonization of regulations across jurisdictions, and continued surveillance. A key goal should be the prevention of CWD from entering new provinces/territories and species.

snip...see full report 31 pages here;

Tuesday, May 31, 2011

Chronic Wasting Disease DOI: 10.1007/128_2011_159 # Springer-Verlag Berlin Heidelberg 2011

Thursday, May 26, 2011

Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007 FoodNet Population Survey

Journal of the American Dietetic Association Volume 111, Issue 6 , Pages 858-863, June 2011.

Saturday, May 14, 2011

Modeling Routes of Chronic Wasting Disease Transmission: Environmental Prion Persistence Promotes Deer Population Decline and Extinction

Thursday, April 28, 2011

Chronic Wasting Disease Testing and Prevalence Wisconsin April 2011

Tuesday, January 25, 2011

Generation of a new form of human PrPSc in vitro by inter-species transmission from cervids prions

Wednesday, April 06, 2011

Presence and Seeding Activity of Pathological Prion Protein (PrPTSE) in Skeletal Muscles of White-Tailed Deer Infected with Chronic Wasting Disease

Wednesday, January 5, 2011



David W. Colby1,* and Stanley B. Prusiner1,2


Wednesday, September 08, 2010


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 ;

2003 The Pathological Protein

The Fort Collins facility became a CWD death trap. Between 1970 and 1981, 90 percent of the deer that stayed more than two years died from the disease or had to be euthanized. In 1980 the scourge emerged outside Colorado, at the Sybille Research Unit in southeastern Wyoming, 120 miles northwest of Fort Collins. The two facilities had exchanged deer for breeding purposes, thus indicating that the disease was infectious--even to a different species: soon the elk at the facilities contracted the disease. (Deer and elk both belong to the cervid family.)

For years, researchers thought CWD resulted from nutritional deficiencies, poisoning, or stress from confinement. But in 1977 Elizabeth S. Williams, studying for her doctorate at Colorado State University, discovered that this view was mistaken. When Williams looked at brain slices from infected animals, she saw that the tissue was full of microscopic holes. "I happened to be taking a course in neuropathology and had studied a lot of brain lesions," she recalls. The holes were unmistakably like scrapie, the sheep sickness that was the first documented spongiform encephalopathy.


In fact, CWD appears to have originated from scrapie. Richard E. Race of the National Institutes of Health Rocky Mountain Laboratories in Hamilton, Mont., conducted test tube studies that revealed no distinction between the malformed PrP of scrapie sheep and CWD cervids. Consistent with this discovery, Amir Hamir of the U.S. Department of Agriculture's National Animal Disease Center in Ames, Iowa, found no difference in the appearance of brain samples from elk with CWD and elk experimentally infected with scrapie. (BSE also probably arose from scrapie, after cows ate feed derived from infected sheep.)



By 1985 veterinarians discovered CWD in free-ranging deer and elk, generally within about 30 miles of the two wildlife facilities. Whether the disease originated in the wild and spread to the captives, or vice versa, is not known. The two populations had plenty of time to mingle. Especially during mating season, wild cervids nosed up to captives through the chain-link fences. Incubating deer could also have escaped or been released.

Both facilities tried hard to eradicate CWD. The Sybille center killed all the deer and elk in the affected area and waited a year to introduce new animals; four years later deer and elk started coming down with CWD. The Fort Collins facility acted more aggressively. Officials first killed off all the resident deer and elk; then they turned several inches of soil and repeatedly sprayed structures and pastures with swimming-pool chlorine, which readily wipes out bacteria and viruses. After waiting a year, they brought in 12 elk calves, but a few years afterward two of those elk contracted CWD.


Philip Yam is Scientific American's news editor. This article is adapted from his book, The Pathological Protein: Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases, published in June.

also, see ;



Perceptions of unconventional slow virus diseases of animals in the USA

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 about 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. Whether they were scrapie infected sheep or not is unclear.



October 1994

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ

Dear Mr Elmhirst,


Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.

The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

and why do we not want to do TSE transmission studies on chimpanzees $


5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.



Topics in Current Chemistry, 2011, 1-28, DOI: 10.1007/128_2011_161

Atypical Prion Diseases in Humans and Animals

Michael A. Tranulis, Sylvie L. Benestad, Thierry Baron and Hans Kretzschmar


Although prion diseases, such as Creutzfeldt–Jakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the “scrapie form” (PrPSc), are used as disease markers, and for taxonomic purposes, in correlation with clinical, pathological, and genetic data. In humans, prion diseases can arise sporadically (sCJD) or genetically (gCJD and others), caused by mutations in the PrP-gene (PRNP), or as a foodborne infection, with the agent of bovine spongiform encephalopathy (BSE) causing variant CJD (vCJD). Person-to-person spread of human prion disease has only been known to occur following cannibalism (kuru disease in Papua New Guinea) or through medical or surgical treatment (iatrogenic CJD, iCJD). In contrast, scrapie in small ruminants and chronic wasting disease (CWD) in cervids behave as infectious diseases within these species. Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents. This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans.

Keywords Animal - Atypical - Atypical/Nor98 scrapie - BSE-H - BSE-L - Human - Prion disease - Prion strain - Prion type

Monday, May 23, 2011

Atypical Prion Diseases in Humans and Animals 2011

Top Curr Chem (2011)

DOI: 10.1007/128_2011_161

# Springer-Verlag Berlin Heidelberg 2011

Sunday, May 01, 2011

STUDY OF ATYPICAL BSE 2010 Annual Report May 2011

Wednesday, August 11, 2010


Thursday, August 19, 2010


Thursday, February 10, 2011

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31

Friday, March 4, 2011

Alberta dairy cow found with mad cow disease

i wonder if CFIA Canada uses the same OBEX ONLY diagnostic criteria as the USDA ? they could not have found a mad cow if they were standing beside the stumbling and staggering bovine $$$, using the diagnostic criteria they were using, AND TOLD SO, but they kept on doing it anyway. ...

Tuesday, November 02, 2010

BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992


Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

Emmanuel E. Comoy1*, Cristina Casalone2, Nathalie Lescoutra-Etchegaray1, Gianluigi Zanusso3, Sophie Freire1, Dominique Marcé1, Frédéric Auvré1, Marie-Magdeleine Ruchoux1, Sergio Ferrari3, Salvatore Monaco3, Nicole Salès4, Maria Caramelli2, Philippe Leboulch1,5, Paul Brown1, Corinne I. Lasmézas4, Jean-Philippe Deslys1

1 Institute of Emerging Diseases and Innovative Therapies, CEA, Fontenay-aux-Roses, France, 2 Istituto Zooprofilattico Sperimentale del Piemonte, Turin, Italy, 3 Policlinico G.B. Rossi, Verona, Italy, 4 Scripps Florida, Jupiter, Florida, United States of America, 5 Genetics Division, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts, United States of America

Abstract Top Background Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform Encephalopathy (cBSE). Atypical forms of BSE, which remain mostly asymptomatic in aging cattle, were recently identified at slaughterhouses throughout Europe and North America, raising a question about human susceptibility to these new prion strains.

Methodology/Principal Findings Brain homogenates from cattle with classical BSE and atypical (BASE) infections were inoculated intracerebrally into cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously demonstrated to be susceptible to the original strain of cBSE. The resulting diseases were compared in terms of clinical signs, histology and biochemistry of the abnormal prion protein (PrPres). The single monkey infected with BASE had a shorter survival, and a different clinical evolution, histopathology, and prion protein (PrPres) pattern than was observed for either classical BSE or vCJD-inoculated animals. Also, the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine.

Conclusion/Significance Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products.

Citation: Comoy EE, Casalone C, Lescoutra-Etchegaray N, Zanusso G, Freire S, et al. (2008) Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate. PLoS ONE 3(8): e3017. doi:10.1371/journal.pone.0003017

Editor: Neil Mabbott, University of Edinburgh, United Kingdom

Received: April 24, 2008; Accepted: August 1, 2008; Published: August 20, 2008

Copyright: © 2008 Comoy et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Funding: This work has been supported by the Network of Excellence NeuroPrion.

Competing interests: CEA owns a patent covering the BSE diagnostic tests commercialized by the company Bio-Rad.

* E-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000110/!


BASE Transmitted to Primates and MV2 sCJD Subtype Share PrP27-30 and PrPSc C-terminal Truncated Fragments

Zanusso, G1; Commoy, E2; Fasoli, E3; Fiorini, M3; Lescoutra, N4; Ruchoux, MM4; Casalone, C5; Caramelli, M5; Ferrari, S3; Lasmezas, C6; Deslys, J-P4; Monaco, S3 1University of Verona, of Neurological and Visual Sciences, Italy; 2CEA, IMETI/SEPIA, France; 3University of Verona, Neurological and Visual Sciences, Italy; 4IMETI/SEPIA, France; 5IZSPLVA, Italy; 6The Scripps Research Insitute, USA

The etiology of sporadic Creutzfeldt-Jakob disease (sCJD), the most frequent human prion disease, remains still unknown. The marked disease phenotype heterogeneity observed in sCJD is thought to be influenced by the type of proteinase K-resistant prion protein, or PrPSc (type 1 or type 2 according to the electrophoretic mobility of the unglycosylated backbone), and by the host polymorphic Methionine/Valine (M/V) codon 129 of the PRNP. By using a two-dimensional gel electrophoresis (2D-PAGE) and imunoblotting we previously showed that in sCJD, in addition to the PrPSc type, distinct PrPSc C-terminal truncated fragments (CTFs) correlated with different sCJD subtypes. Based on the combination of CTFs and PrPSc type, we distinguished three PrPSc patterns: (i) the first was observed in sCJD with PrPSc type 1 of all genotypes,; (ii) the second was found in M/M-2 (cortical form); (iii) the third in amyloidogenic M/V- 2 and V/V-2 subtypes (Zanusso et al., JBC 2004) . Recently, we showed that sCJD subtype M/V-2 shared molecular and pathological features with an atypical form of BSE, named BASE, thus suggesting a potential link between the two conditions. This connection was further confirmed after 2D-PAGE analysis, which showed an identical PrPSc signature, including the biochemical pattern of CTFs. To pursue this issue, we obtained brain homogenates from Cynomolgus macaques intracerebrally inoculated with brain homogenates from BASE. Samples were separated by using a twodimensional electrophoresis (2D-PAGE) followed by immunoblotting. ***We here show that the PrPSc pattern obtained in infected primates is identical to BASE and sCJD MV-2 subtype. These data strongly support the link, or at least a common ancestry, between a sCJD subtype and BASE. This work was supported by Neuroprion (FOOD-CT-2004-506579)


Transmission of Italian BSE and BASE Isolates in Cattle Results into a Typical BSE Phenotype and a Muscle Wasting Disease

Zanusso, G1; Lombardi, G2; Casalone, C3; D’Angelo, A4; Gelmetti, D2; Torcoli, G2; Barbieri, I2; Corona, C3; Fasoli, E1; Farinazzo, A1; Fiorini, M1; Gelati, M1; Iulini, B3; Tagliavini, F5; Ferrari, S1; Monaco, S1; Caramelli, M3; Capucci, L2 1University of Verona, Neurological and Visual Sciences, Italy; 2IZSLER, Italy; 3IZSPLVA, Italy; 4University of Turin, Animal Pathology, Italy; 5Isituto Carlo Besta, Italy

The clinical phenotype of bovine spongiform encephalopathy has been extensively reported in early accounts of the disorder. Following the introduction of statutory active surveillance, almost all BSE cases have been diagnosed on a pathological/molecular basis, in a pre-symptomatic clinical stage. In recent years, the active surveillance system has uncovered atypical BSE cases, which are characterized by distinct conformers of the PrPSc, named high-type (BSE-H) and low-type (BSE-L), whose clinicopathological phenotypes remain unknown. We recently reported two Italian atypical cases with a PrPSc type similar to BSE-L, pathologically characterized by PrP amyloid plaques. Experimental transmission to TgBov mice has recently disclosed that BASE is caused by a distinct prion strain which is extremely virulent. A major limitation of transmission studies to mice is the lack of reliable information on clinical phenotype of BASE in its natural host. In the present study, we experimentally infected Fresian/Holstein and Alpine/Brown cattle with Italian BSE and BASE isolates by i.c. route. BASE infected cattle showed survival times significantly shorter than BSE, a finding more readily evident in Fresian/Holstein, and in keeping with previous observations in TgBov mice. Clinically, BSE-infected cattle developed a disease phenotype highly comparable with that described in field BSE cases and in experimentally challenged cattle. On the contrary, BASE-inoculated cattle developed an amyotrophic disorder accompanied by mental dullness. The molecular and neuropathological profiles, including PrP deposition pattern, closely matched those observed in the original cases. ***This study further confirms that BASE is caused by a distinct prion isolate and discloses a novel disease phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated cattle and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease.


Molecular Features of the Protease-resistant Prion Protein (PrPres) in H-type BSE

Biacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron, TGM1 1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, Sweden

Western blot analyses of PrPres accumulating in the brain of BSE-infected cattle have demonstrated 3 different molecular phenotypes regarding to the apparent molecular masses and glycoform ratios of PrPres bands. We initially described isolates (H-type BSE) essentially characterized by higher PrPres molecular mass and decreased levels of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type is also distinct from another BSE phenotype named L-type BSE, or also BASE (for Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low representation of the diglycosylated PrPres band as well as a lower PrPres molecular mass. Retrospective molecular studies in France of all available BSE cases older than 8 years old and of part of the other cases identified since the beginning of the exhaustive surveillance of the disease in 20001 allowed to identify 7 H-type BSE cases, among 594 BSE cases that could be classified as classical, L- or H-type BSE. By Western blot analysis of H-type PrPres, we described a remarkable specific feature with antibodies raised against the C-terminal region of PrP that demonstrated the existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in cattle seems to by higher compared to the PrPres#1. Furthermore another PK–resistant fragment at about 7 kDa was detected by some more N-terminal antibodies and presumed to be the result of cleavages of both N- and C-terminal parts of PrP. These singular features were maintained after transmission of the disease to C57Bl/6 mice. ***The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band) reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;

please see full text ;


Transmission of atypical BSE in humanized mouse models

Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University (Previously at USDA National Animal Disease Center), USA

Background: Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Atypical BSE cases have been discovered in three continents since 2004; they include the L-type (also named BASE), the H-type, and the first reported case of naturally occurring BSE with mutated bovine PRNP (termed BSE-M). The public health risks posed by atypical BSE were largely undefined.

Objectives: To investigate these atypical BSE types in terms of their transmissibility and phenotypes in humanized mice. Methods: Transgenic mice expressing human PrP were inoculated with several classical (C-type) and atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation time, characteristics and distribution of PrPSc, symptoms, and histopathology were or will be examined and compared.

Results: Sixty percent of BASE-inoculated humanized mice became infected with minimal spongiosis and an average incubation time of 20-22 months, whereas only one of the C-type BSE-inoculated mice developed prion disease after more than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse brains was biochemically different from bovine BASE or sCJD. PrPSc was also detected in the spleen of 22% of BASE-infected humanized mice, but not in those infected with sCJD. Secondary transmission of BASE in the humanized mice led to a small reduction in incubation time.*** The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice, but no BSE-M transmission has been observed so far.

Discussion: Our results demonstrate that BASE is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg mice. The possibility of more than two atypical BSE strains will be discussed.

Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.


Analysis of Bovine Prion Protein Gene Sequence Variation in Animals with Classical and Atypical BSE

Polak, MP; Larska, M; Rola, J; Zmudzinski, JF National Veterinary Research Institute, Department of Virology, Poland B.

Variation within prion protein gene sequence have major impact on the susceptibility to prion diseases in humans and sheep. However no major differences between healthy cattle and bovine spongiform encephalopathy (BSE) affected individuals were identified. Recent studies indicate that susceptibility to bovine spongiform encephalopathy is associated with 23-base pair (bp) and 12-bp indel sequences. Identification of atypical BSE in older cattle in several countries pointed at the possibility of spontaneous origin of this new form of prion disease due to possible mutations within prion gene (PRNP) sequence. A./O. Therefore the aim of the study was to analyze and to compare prion protein gene sequences in animals showing classical and atypical BSE for any genetic traits differentating both forms of the disease. M. Analysis included: octapeptide-repeat polymorphism; sequence analysis of exon 3 region; deletion/insertion polymorphism within the promoter sequence (23-bp), intron 1 (12-bp) and 3’untranslated region - UTR (14-bp) of PRNP gene. R. No major differences were found as for the octapeptide-repeats. Most dominant genotype in both classical and atypical BSE involved 6/6 homozygous animals. Sequence comparison within exon 3 region also showed no differences. Results from indel sequence analysis within three regions of PRNP gene were also quite uniform between both forms of BSE. D. Therefore no genetic traits explaining the appearance of atypical BSE could be found. However, it is too early to reject the hypothesis that genetic makeup is not involved in atypical BSE. Further and more detailed studies including more cases of atypical BSE would be more reliable to draw such a conclusion.


Biological typing of sporadic Creutzfeldt- Jakob disease isolates and comparison with animal prion isolates

Romolo Nonno1, Michele Di Bari1, Laura Pirisinu1, Stefano Marcon1, Claudia D’Agostino1, Elena Esposito1, Paola Fazzi1, Shimon Simson1, Paolo Frassanito1, Cristina Casalone3, Franco Cardone2, Maurizio Pocchiari2, Gabriele Vaccari1, Umberto Agrimi1 1Dept. SPVSA, Istituto Superiore di Sanità, Italy; 2Dept. BCN, Istituto Superiore di Sanità, Italy; 3Istituto Zooprofilattico del Piemonte, Liguria e Valle D’Aosta, Italy

Background: Our incomplete understanding of the nature of TSE agents, along with the current technical limitations in the analysis of PrPSc structure, prevent the direct typing of prion isolates. The characterization of prion strains still relies upon bioassay in rodents. Bank vole (Myodes glareolus), being susceptible to a wide range of prion sources, offers the opportunity to investigate the biological properties of prion isolates from different species in a single model.

Objectives: To study the biological properties of sCJD subtypes and compare them with animal TSEs. Methods: We analysed the phenotype of transmission of MM1, MV1, MM2, MV2, and VV2 sCJD subtypes to voles, in comparison with BSE, BASE and classical scrapie isolates from different EU countries. Molecular analysis of PrPSc from the original isolates preceded voles inoculation. Survival time and attack rate were calculated upon primary transmissions and subsequent passages. The brain of voles were analysed by WB for PrPSc type, by Gnd- HCl denaturation for PrPSc conformational stability, by immunohistochemistry and PET-blot for PrPSc deposition pattern and by E&E for lesion profile.

Results: This study demonstrated that prion diseases induce in voles a variety of molecular and pathological phenotypes. CJD isolates were grouped into 4 categories: i) MM1/MV1 (n=3), ii) MM2 (n=1), iii) MV2 (n=2) and iv) VV2 (n=1). Scrapie isolates were categorised in at least 4 groups, with no overlapping with sCJD isolates. BSE was distinct from scrapie and sCJD phenotypes. Finally, BASE gave a phenotype distinct from BSE and scrapie but indistinguishable from VV2 sCJD.

Discussion: Overall, the biological classification of sCJD subtypes concurs with their clinico-pathological classification.*** Similarities in the transmission pattern of prion isolates from different host species were very rare, with the notable exception of BASE and VV2 sCJD. Herein, the meaning of such similarities is discussed in the context of current knowledge on strains and of available tools for their typing.


Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University, Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous address: USDA National Animal Disease Center, Ames, IA 50010, USA

Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have been discovered in three continents since 2004. The first case of naturally occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006 in the USA. The transmissibility and phenotypes of these atypical BSE strains/isolates in humans were unknown. We have inoculated humanized transgenic mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M isolate. We have found that the atypical BSE-L strain is much more virulent than the classical BSE-C.*** The atypical BSE-H strain is also transmissible in the humanized transgenic mice with distinct phenotype, but no transmission has been observed for the BSE-M isolate so far.


I ask Professor Kong ;

Thursday, December 04, 2008 3:37 PM Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment

''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....''

Professor Kong reply ;


''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete. Thanks for your interest.''

Best regards, Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA


BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein

Emmanuel A. Asante, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew F. Hill, Sarah E. Lloyd, Jonathan D.F. Wadsworth, and John Collinge1 MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK 1Corresponding author e-mail: August 1, 2002; Revised September 24, 2002; Accepted October 17, 2002.

The EMBO Journal (2002) 21, 6358 - 6366 doi:10.1093/emboj/cdf653

BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein

Emmanuel A. Asante1, Jacqueline M. Linehan1, Melanie Desbruslais1, Susan Joiner1, Ian Gowland1, Andrew L. Wood1, Julie Welch1, Andrew F. Hill1, Sarah E. Lloyd1, Jonathan D.F. Wadsworth1 and John Collinge1

1.MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK Correspondence to:

John Collinge, E-mail:

Received 1 August 2002; Accepted 17 October 2002; Revised 24 September 2002



Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2. These data suggest that more than one BSE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.

Keywords:BSE, Creutzfeldt–Jakob disease, prion, transgenic

The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.

Sunday, March 27, 2011


Wednesday, February 16, 2011




Sunday, April 18, 2010


Monday, April 25, 2011

Experimental Oral Transmission of Atypical Scrapie to Sheep

Volume 17, Number 5-May 2011

Monday, November 30, 2009


I strenuously urge the USDA and the OIE et al to revoke the exemption of the legal global trading of atypical Nor-98 scrapie TSE. ...TSS

Friday, February 11, 2011

Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues


This is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........

Thursday, December 23, 2010

Molecular Typing of Protease-Resistant Prion Protein in Transmissible Spongiform Encephalopathies of Small Ruminants, France, 2002-2009 Volume 17, Number 1 January 2011

Thursday, November 18, 2010

Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep

Sunday, October 3, 2010

Scrapie, Nor-98 atypical Scrapie, and BSE in sheep and goats North America, who's looking ?

Saturday, November 6, 2010

TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU Berne, 2010 TAFS


Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR> Prion disease update 2010 (11)

PRION DISEASE UPDATE 2010 (11),F2400_P1001_PUB_MAIL_ID:1000,86129

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

Subject: DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability

Date: Fri, 16 May 2003 11:47:37 -0500

From: "Terry S. Singeltary Sr." To:

Greetings FDA,

i would kindly like to comment on; Docket 03D-0186FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability Several factors on this apparent voluntary proposal disturbs me greatly, please allow me to point them out;

1. MY first point is the failure of the partial ruminant-to-ruminant feed ban of 8/4/97. this partial and voluntary feed ban of some ruminant materials being fed back to cattle is terribly flawed. without the_total_ and _mandatory_ ban of all ruminant materials being fed back to ruminants including cattle, sheep, goat, deer, elk and mink, chickens, fish (all farmed animals for human/animal consumption), this half ass measure will fail terribly, as in the past decades...

2. WHAT about sub-clinical TSE in deer and elk? with the recent findings of deer fawns being infected with CWD, how many could possibly be sub-clinically infected. until we have a rapid TSE test to assure us that all deer/elk are free of disease (clinical and sub-clinical), we must ban not only documented CWD infected deer/elk, but healthyones as well. if this is not done, they system will fail...

3. WE must ban not only CNS (SRMs specified risk materials), but ALL tissues. recent new and old findings support infectivity in the rump or ass muscle. wether it be low or high, accumulation will play a crucial role in TSEs.

4. THERE are and have been for some time many TSEs in theUSA. TME in mink, Scrapie in Sheep and Goats, and unidentified TSE in USA cattle. all this has been proven, but the TSE in USA cattle has been totally ignored for decades. i will document this data below in my references.

5. UNTIL we ban all ruminant by-products from being fed back to ALL ruminants, until we rapid TSE test (not only deer/elk) but cattle in sufficient numbers to find (1 million rapid TSE test in USA cattle annually for 5 years), any partial measures such as the ones proposed while ignoring sub-clinical TSEs and not rapid TSE testing cattle, not closing down feed mills that continue to violate the FDA's BSE feed regulation (21 CFR 589.2000) and not making freely available those violations, will only continue to spread theseTSE mad cow agents in the USA.

I am curious what we will call a phenotype in a species that is mixed with who knows how many strains of scrapie, who knows what strain or how many strains of TSE in USA cattle, and the CWD in deer and elk (no telling how many strains there), but all of this has been rendered for animal feeds in the USA for decades. it will get interesting once someone starts looking in all species, including humans here in the USA, but this has yet to happen...

6. IT is paramount that CJD be made reportable in every state (especially ''sporadic'' cjd), and that a CJD Questionnaire must be issued to every family of a victim of TSE. only checking death certificates will not be sufficient. this has been proven as well (see below HISTORY OF CJD -- CJD QUESTIONNAIRE)

7. WE must learn from our past mistakes, not continue to make the same mistakes...



Oral transmission and early lymphoid tropism of chronic wasting diseasePrPres 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. Hoover1Department of Pathology, College of Veterinary Medicine and BiomedicalSciences, Colorado State University, Fort Collins, CO 80523- 1671, USA1Department of Veterinary Sciences, University of Wyoming, 1174 SnowyRange Road, University of Wyoming, Laramie, WY 82070, USA 2Colorado Division of Wildlife, Wildlife Research Center, 317 WestProspect Road, Fort Collins, CO 80526-2097, USA3Colorado State University Veterinary Diagnostic Laboratory, 300 WestDrake Road, Fort Collins, CO 80523-1671, USA4Animal Disease Research Unit, Agricultural Research Service, USDepartment of Agriculture, 337 Bustad Hall, Washington State University,Pullman, WA 99164-7030, USA5Author for correspondence: Edward Hoover.Fax +1 970 491 0523.

Mule deer fawns (Odocoileus hemionus) were inoculated orally with abrain 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 lymphnode, 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 monoclonalantibody F89/160.1.5. These results indicate that CWD PrPres 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 PrPres after oral exposure to an inoculum containing CWD prions. In the earliest post-exposure period, CWD PrPres was traced to the lymphoidtissues draining the oral and intestinal mucosa (i.e. there tropharyngeal 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, ileumand 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 supportoral exposure as a natural route of CWD infection in deer and supportoral inoculation as a reasonable exposure route for experimental studies of CWD.



now, just what is in that deer feed? _ANIMAL PROTEIN_


Date: Sat, 25 May 2002 18:41:46 -0700 From: "Terry S. Singeltary Sr." Reply-To: BSE-LTo: BSE-L

8420-20.5% Antler DeveloperFor Deer and Game in the wildGuaranteed Analysis Ingredients / Products Feeding Directions


_animal protein_



_animal protein_

IngredientsGrain Products, Plant Protein Products, Processed Grain By-Products,Forage Products, Roughage Products 15%, Molasses Products,

__Animal Protein Products__,

Monocalcium Phosphate, Dicalcium Pyosphate, Salt,Calcium Carbonate, Vitamin A Acetate with D-activated Animal Sterol(source of Vitamin D3), Vitamin E Supplement, Vitamin B12 Supplement,Riboflavin Supplement, Niacin Supplement, Calcium Panothenate, CholineChloride, Folic Acid, Menadione Soduim Bisulfite Complex, PyridoxineHydorchloride, Thiamine Mononitrate, d-Biotin, Manganous Oxide, ZincOxide, Ferrous Carbonate, Calcium Iodate, Cobalt Carbonate, DriedSacchoromyces Berevisiae Fermentation Solubles, Cellulose gum,Artificial Flavors added.



Bode's #1 Game PelletsA RATION FOR DEERF3153GUARANTEED ANALYSISCrude Protein (Min) 16%Crude Fat (Min) 2.0%Crude Fiber (Max) 19%Calcium (Ca) (Min) 1.25%Calcium (Ca) (Max) 1.75%Phosphorus (P) (Min) 1.0%Salt (Min) .30%Salt (Max) .70%IngredientsGrain Products, Plant Protein Products, Processed Grain By-Products,Forage Products, Roughage Products, 15% Molasses Products,

__Animal Protein Products__,

Monocalcium Phosphate, Dicalcium Phosphate, Salt,Calcium Carbonate, Vitamin A Acetate with D-activated Animal Sterol(source of Vitamin D3) Vitamin E Supplement, Vitamin B12 Supplement,Roboflavin Supplement, Niacin Supplement, Calcium Pantothenate, CholineChloride, Folic Acid, Menadione Sodium Bisulfite Complex, PyridoxineHydrochloride, Thiamine Mononitrate, e - Biotin, Manganous Oxide, ZincOxide, Ferrous Carbonate, Calcium Iodate, Cobalt Carbonate, DriedSaccharyomyces Cerevisiae Fermentation Solubles, Cellulose gum,Artificial Flavors added.FEEDING DIRECTIONSFeed as Creep Feed with Normal Diet


Grain Products, Roughage Products (not more than 35%), Processed GrainBy-Products, Plant Protein Products, Forage Products,

__Animal Protein Products__,

L-Lysine, Calcium Carbonate, Salt, Monocalcium/DicalciumPhosphate, Yeast Culture, Magnesium Oxide, Cobalt Carbonate, BasicCopper Chloride, Manganese Sulfate, Manganous Oxide, Sodium Selenite,Zinc Sulfate, Zinc Oxide, Sodium Selenite, Potassium Iodide,Ethylenediamine Dihydriodide, Vitamin E Supplement, Vitamin ASupplement, Vitamin D3 Supplement, Mineral Oil, Mold Inhibitor, CalciumLignin Sulfonate, Vitamin B12 Supplement, Menadione Sodium BisulfiteComplex, Calcium Pantothenate, Riboflavin, Niacin, Biotin, Folic Acid,Pyridoxine Hydrochloride, Mineral Oil, Chromium Tripicolinate


Deer Builder Pellets is designed to be fed to deer under rangeconditions or deer that require higher levels of protein. Feed to deerduring gestation, fawning, lactation, antler growth and pre-rut, allphases which require a higher level of nutrition. Provide adequateamounts of good quality roughage and fresh water at all times.



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


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 cattleor 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 withthe law. We have enclosed a copy of FDA's Small Entity Compliance Guideto assist you with complying with the regulation... blah, blah, blah...


snip...end...full text ;

2003D-0186 Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

EMC 1 Terry S. Singeltary Sr. Vol #: 1

2003D-0186 Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

EMC 7 Terry S. Singeltary Sr. Vol #: 1

2003D-0186 Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

EMC 7 Terry S. Singeltary Sr. Vol #: 1

01N-0423 Substances Prohibited from use in animal food/Feed Ruminant

APE 5 National Renderers Association, Inc. Vol#: 2

APE 6 Animal Protein Producers Industry Vol#: 2

APE 7 Darling International Inc. Vol#: 2

EMC 1 Terry S. Singeltary Sr. Vol#: 3

Saturday, May 16, 2009

Chronic Wasting Disease Herd Certification Program Document ID APHIS-2006-0118-0096

Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

Thursday, April 9, 2009

Docket No. FDA2002N0031 (formerly Docket No. 2002N0273) RIN 0910AF46 Substances Prohibited From Use in Animal Food or Feed; Final Rule: Proposed Thursday, April 09, 2009

Docket No. FDA2002N0031 (formerly Docket No. 2002N0273) RIN 0910AF46 Substances Prohibited From Use in Animal Food or Feed; Final Rule: Proposed

Greetings FDA et al,

I kindly wish to comment on the following ;

[Docket No. FDA-2002-N-0031] (formerly Docket No. 2002N-0273) RIN 0910-AF46

[Federal Register: April 9, 2009 (Volume 74, Number 67)] [Proposed Rules] [Page 16160-16161] From the Federal Register Online via GPO Access [] [DOCID:fr09ap09-18]


Food and Drug Administration

21 CFR Part 589

[Docket No. FDA-2002-N-0031] (formerly Docket No. 2002N-0273) RIN 0910-AF46

Substances Prohibited From Use in Animal Food or Feed; Final Rule: Proposed Delay of Effective Date

AGENCY: Food and Drug Administration, HHS.

ACTION: Notice of proposed delay of effective date.


I find it deeply disturbing, that with the science to date, especially with the science to date, transmission studies, the more virulent atypical strains of the BSE i.e. h-BSE and l-BSE, both of which have now been documented in North America, that we are even still discussing this most important topic. The industry involved has beat this mad cow feed ban to death, and still refuse to comply. IF they would have adhered to policy, rules and regulations put forth August 4th, 1997, when the partial, and voluntary ruminant to ruminant feed ban was first put in place, they would not still be crying the same tune. WE need not only to enforce the present ban, but strengthen it, especially to include blood in the ban. WE (the consumer), was promised this would happen years ago. For Pete's sake, this will be the third president to have to address these same questions, and I pray that this one has the guts to finally do something. We need NOT discuss this for one more second. We had 8 years that President Bush literally covered up mad cow disease, and let literally millions and millions of pounds of mad cow feed into commerce to be fed out. IN one feed ban recall alone in 2007, 10 MILLION PLUS POUNDS was fed out into commerce. and under this same President, we now millions of kids across our Nation that have been needlessly exposed to the mad cow agent via the infamous USDA CERTIFIED DOWNER COW DEAD STOCK SCHOOL LUNCH PROGRAM. if you think for one moment that the largest meat recall in the history of the USA was because a few animals were filmed being abused, your only kidding yourself. that meat was recalled because dead stock downer cows are at the highest risk to carry mad cow disease, and they had been feeding our children this stuff for years. AND then had the nerve to lie to us about THE GREAT BSE FIREWALL IN THE USA THAT WOULD PROTECT THE CONSUMER I.E. THE BSE FEED BAN, that never was nothing more than ink on paper. who will monitor these children in the years and decades to come for a human form of Transmissible Spongiform Encephalopathy? who can with a CJD/TSE surveillance system and CJD Questionnaire set up the way it is now? you can't.


I strenuously urge President Obama to NOT discuss this for one more moment, actions must be put forth now, and enforce such actions.

I strenuously urge President Obama to ENHANCE the feed ban to include blood, and enforce said regulations, based on sound science.

I strenuously urge President Obama to ban the use of "poultry litter" and the use of all mammalian and poultry protein in ruminant feed,as a feed ingredient for ruminant animals, and enforce said regulations, based on sound science.

I strenuously urge President Obama to ban the use of "plate waste" as a feed ingredient for ruminants, and enforce said regulations, based on sound science.

I strenuously urge President Obama to ban from human food (including dietary supplements please see latest May 2009 CDC warning on these type supplements, CWD, and Elk Antler Velvet), and cosmetics a wide range of bovine-derived material so that the same safeguards that protect Americans from exposure to the agent of BSE through meat products regulated by USDA also apply to food products that FDA regulates, and enforce such actions, based on sound science.

I strenuously urge President Obama to further minimize the possibility of cross-contamination of ruminant and non-ruminant animal feed by requiring equipment, facilities or production lines to be dedicated to non-ruminant animal feeds if they use protein that is prohibited in ruminant feed. Currently, some equipment, facilities and production lines process or handle prohibited and non-prohibited materials and make both ruminant and non-ruminant feed -- a practice which could lead to cross-contamination, and enforce said regulations, based on sound science.

LET's take a look back at past promises and discussions on this issue, and then for a breath of fresh air, let's look at some sound science, and why no further discussion is warranted, and why action is needed ASAP ;

Friday, April 25, 2008

Substances Prohibited From Use in Animal Food or Feed [Docket No. 2002N-0273] (Formerly Docket No. 02N-0273) RIN 0910-AF46


Date: January 9, 2007 at 9:08 am PST

Docket Management Docket: 02N-0273 - Substances Prohibited From Use in

Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed

Comment Number: EC -10

Accepted - Volume 2


Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE SAFEGUARDS (comment submission)

Animal Proteins Prohibited in Ruminant Feed Docket ID: FDA-2002-N-0031Agency: FDARIN: 0910-AF46!docketDetail;rpp=10;po=480;D=FDA-2002-N-0031

Sunday, July 27, 2008

Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE SAFEGUARDS (comment submission)

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

Subject: Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE SAFEGUARDS (comment submission)

Date: Sun, 11 Jul 2004 21:34:22 -0500 From: "Terry S. Singeltary Sr."

To: CC:,


No. 04-047-l

No. 04-021ANPR

No. 2004N-0264


Federal Measures to Mitigate BSE Risks: Considerations for Further Action

Greetings FDA, USDA and APHIS et al,

I would kindly like to comment on the continued delay of the regulations that have been proposed for years to reduce the risk of BSE/TSE in the USA. Each day that is wasted debating this issue allows this agent to spread, and many many more humans and animals become needlessly exposed to this agent via a multitude of potential routes and sources right here in the USA. TO continue to ignore the new findings from several scientists about the fact that BSE is not the only strain of TSE in cattle, the fact that new atypical strains of TSE are showing up in not only cattle, but sheep and the fact that the new strain of TSE in cattle seems to be more similar to sporadic CJD as opposed to the nv/v CJD, to continue to ignore these findings will only further spread this agent.

CWD and Scrapie have been running rampant in the USA for decades. BOTH of which have been rendered and fed back to animals for human/animal consumption for decades. All of which transmits to primates by the natural and non-forced oral consumption of TSE scrapie, CJD, Kuru agent (and CWD by inoculation).

Strong Scientific evidence discovered back in the 80s support the fact that a TSE has been prevalent in the USA bovine for decades, either undetected or ignored. IF you consider the recent stumbling and staggering TEXAS cow that was showing all signs of a CNS/TSE disorder that was ordered to be rendered without BSE/TSE test, brains, spinal cord, head and all (as to no possible evidence left of TSE), I would think the 'ignored' or 'covered up' to be the better terminology. Then you have the Downer in Washington state that was actually a good walker and then all the banned Canadian products that some how found it's way across the border into the USA, considering all this, it is very difficult for me to believe that the FDA/USDA/APHIS et al are doing everything possible to protect the 'consumer'. Hardly the case;

Congressman Henry Waxmans Letter to the Honorable Ann Veneman

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

Subject: Re: Congressman Henry Waxmans Letter to the Honorable Ann Veneman on failure by USDA/APHIS TO TEST TEXAS MAD COW Date: Wed, 9 Jun 2004 16:48:31 -0500

From: "Terry S. Singeltary Sr."

Reply-To: Bovine Spongiform Encephalopathy

To: References: <>

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


October 1998

BSE Red Book 2.1-36 Laboratory Coordination--The Laboratory Coordination Officer will advise the READE(3 Director concerning laboratory capabilities and appropriate laboratory examinations to be conducted to provide needed results as rapidly as possible. This individual will assist with interpretation of results.

seems that if the 'enhanced BSE/TSE testing program' is to test some 400,000+ animals in 1 1/2 years, they better hurry up, times a wasting.

BSE Red Book 2.1-39

7.6 Depopulation Procedures

Under no circumstances may BSE suspects be sent fo slaughhter or rendering.


BSE Red Book 2.1-40

7.7 Disposal Under no circumstances may BSE suspects be sent to slaughter or rendering. Notify FDA, CVM if you suspect that the carcass of a BSE-confirmed animal has moved to rendering or animal feed manufacturing. Field personel should arrange for the carcass to be transported to and examined by a qualified veterinary pathologist or field veterinary medical officer. After the pathologic examination has been completed and the necessary diagnostic specimens have been obtained, field personnel should arrange for disposal of the carcass. Before a method of disposal is selected, there are many factors that must be considered, and often other State and Federal agencies must be consulted. The environmental and legal impacts of the operation must be considered. Upon recommendation of the State or Federal agencies, VS may consider other disposal methods.


7.7.3 Rendering Because BSE is spread by rendered animal protein, BSE-suspect and confirmed carcasses must not be rendered, unless the rendered material is incinerated. Notify FDA, CVM if you suspect that dead BSE animals or carcasses have moved to rendering or animal feed manufacturing.


7.10.11 Prevention--Suspects and animals confirmed to have BSE must not be rendered. Producers, feed mills, and rendering establishments should adhere to U.S. State and local rendering policies and FDA regulations concerning the feeding of rendered animal protein to ruminants.


Terry S. Singeltary Sr. wrote:


[Federal Register: January 9, 2007 (Volume 72, Number 5)] [Proposed Rules] [Page 1101-1129] From the Federal Register Online via GPO Access [] [DOCID:fr09ja07-21]

Attachment to Singletary comment Document ID: APHIS-2006-0041-0028.1 This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Docket ID: APHIS-2006-0041

SEE DOC ;!documentDetail;D=APHIS-2006-0041-0028.1

From: Terry S. Singeltary Sr.


Cc: ;

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...

APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15, 2006

(this starts out in part III, then part II, and part I and the beginning is at the bottom. ...tss)

[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

suppressed peer review of Harvard study October 31, 2002

Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005


The United States Department of Agriculture’s Food Safety and Inspection Service (FSIS) held a public meeting on July 25, 2006 in Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 (report and model located on the FSIS website:

Comments on technical aspects of the risk assessment were then submitted to FSIS. Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.

This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:

[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle 9/13/2005

Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION]

From: Terry S. Singeltary Sr. []

Sent: Tuesday, July 29, 2003 1:03 PM


Cc:;; BSE-L

Subject: Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION TO DOCKET 2003N-0312]

Greetings FDA,

my name is Terry S. Singeltary Sr., i lost my mother to hvCJD (Heidenhain Variant Creutzfeldt Jakob Disease).

i would kindly like to comment on the proposed HACCP method of detecting and or preventing TSEs in the human/animal feed supply.

it seems to me by implementing something that was designed for Astronauts instead of cattle, something that the GAO has already stated is terribly flawed (HACCP), i find it very disturbing to continue to insist on refusing to use rapid TSE TESTING in sufficient numbers to find TSEs, as with other Countries that they too once thought they were BSE free. for example, it took Italy 1 MILLION rapid TSE tests since 2001 to find 102 cases of BSE. THE USA has only tested 48,000 cattle in the 14 years of surveillance. there is documented proof that indeed the USA cattle have been infected with a TSE for decades, but the FDA/USDA and other USA Gov. agencies continue to conveniently ignore these findings. YOU must not ignore what Richard Marsh found. Plus, you must not ignore Asante/Collinge new findings 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. The USA has been feeding ruminant by-products back to cattle, deer, elk and sheep for decades, and TSEs in these species have been recycled for feed for decades in the USA. The rendering process here in the USA will not kill this agent. to implement any HACCP over massive rapid TSE testing is only prolonging the inevitable, and will only allow the agent to spread further. it is simply a band-aid approach to something that needs a tourniquet...

Dockets Entered On October 2, 2003 Table of Contents, Docket #, Title, 1978N-0301,

OTC External Analgesic Drug Products, ... EMC 7, Terry S. Singeltary Sr. Vol #: 1, ...

Daily Dockets Entered on 02/05/03

DOCKETS ENTERED on 2/5/03. ... EMC 4 Terry S. Singeltary Sr. Vol#: 2. ... Vol#: 1.

03N-0009 Federal Preemption of State & Local Medical Device Requireme. ...

Docket Management

Docket: 02N-0370 - Neurological Devices; Classification of Human Dura Mater

Comment Number: EC -1

Accepted - Volume 1

Daily Dockets - 04/10/03

... 00D-1662 Use of Xenotransplantation Products in Humans. EMC 98 Terry S. Singeltary Sr. Vol#: 3. 01F ...  - 05-20-2003 - Cached

# Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of 2002; [TSS SUBMISSION ON POTENTIAL FOR BSE/TSE & FMD 'SUITCASE BOMBS'] - TSS 1/27/03 (0)

Docket Management

Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food Facilities, Section 305 Comment Number: EC-254 [TSS SUBMISSION]


File Format: PDF/Adobe Acrobat -

Page 1. J Freas, William From:

Sent: To:

Subject: Terry S. Singeltary Sr. []

Monday, January 08,200l 3:03 PM freas ...

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

From: Terry S. Singeltary Sr. []

Sent: Tuesday, February 18, 2003 12:45 PM

To: Freas, William

Cc: Langford, Sheila

Subject: Re: re-vCJD/blood and meeting of Feb. 20, 2003

Greetings FDA,

Asante/Collinge et al, that BSE transmission to the 129-methionine

genotype can lead to an alternate phenotype that is indistinguishable

from type 2 PrPSc, the commonest _sporadic_ CJD;

Tuesday, February 8, 2011


Monday, May 30, 2011

CEPs for gelatin and impact of the revised EU Note for Guidance on the TSE risk EMEA/410/01 Rev.3) will come into force in July 2011

Friday, May 13,

2011 EFSA Joint Scientific Opinion on any possible epidemiological or molecular association between TSEs in animals and humans

Tuesday, May 24, 2011 2:24 PM

O.I.E. Terrestrial Animal Health Standards Commission and prion (TSE) disease reporting 2011

Thursday, August 12, 2010

Seven main threats for the future linked to prions

First threat

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

Second threat


Rural and Regional Affairs and Transport References Committee

The possible impacts and consequences for public health, trade and agriculture of the Government’s decision to relax import restrictions on beef Final report June 2010

2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49

2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo—has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50

Tuesday, April 26, 2011

sporadic CJD RISING Text and figures of the latest annual report of the NCJDRSU covering the period 1990-2009 (published 11th March 2011)

Saturday, March 5, 2011


Wednesday, May 18, 2011

Variably Protease-Sensitive Prionopathy: a Novel Disease of the Prion Protein 17 May 2011

Journal of Molecular Neuroscience DOI: 10.1007/s12031-011-9543-1


Sunday, April 3, 2011


Tuesday, April 5, 2011

Action Plan National Program 103 Animal Health 2012-2017 PRIONS AND TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY

Action Plan National Program 103 Animal Health 2012-2017


WITH more and more atypical Transmissible Spongiform Encephalopathy cases showing up in more and more species here in North America, and the enormous monumental amount of banned mad cow protein in commerce since the infamous partial and voluntary mad cow feed ban inked on paper, with tons and tons crossing back and forth between the USA, Canada, and Mexico, it just does not surprise me of all these "PENDING CLASSIFICATIONS" of human TSE in Canada, and the USA. UK c-BSE transmitted to humans became nvCJD. WE now have atypical strains of BSE in cattle. Mission Texas experiments long ago showed that transmitted USA sheep scrapie to USA bovine, produced a TSE much different than the UK typical c-BSE. SO why would human TSE in the USA look like UK human TSE ? The corruption is mind boggling. The UK saw a suspicious TSE in humans, and science linked it to cattle. North America is awash with human and animal TSE, CJD is rising in young and old, with the same pathology and same symptoms, and none of it is related to the other. isn't that nice. who, what, bestowed such miracles upon North America $

Archive Number 20100405.1091 Published Date 05-APR-2010

Subject PRO/AH/EDR> Prion disease update 1010 (04)


[Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed


The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?",F2400_P1001_PUB_MAIL_ID:1000,82101


CJD Deaths Reported by CJDSS1, 1994-20112 As of January 31, 2011

3. Final classification of 49 cases from 2009, 2010, 2011 is pending.


USA 2011


National Prion Disease Pathology Surveillance Center

Cases Examined1

(November 1, 2010)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 & earlier 51 33 28 5 0 0

1997 114 68 59 9 0 0

1998 87 51 43 7 1 0

1999 121 73 65 8 0 0

2000 146 103 89 14 0 0

2001 209 119 109 10 0 0

2002 248 149 125 22 2 0

2003 274 176 137 39 0 0

2004 325 186 164 21 0 13

2005 344 194 157 36 1 0

2006 383 197 166 29 0 24

2007 377 214 187 27 0 0

2008 394 231 205 25 0 0

2009 425 258 215 43 0 0

2010 333 213 158 33 0 0

TOTAL 38315 22656 1907 328 4 3

1 Listed based on the year of death or, if not available, on year of referral;

2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;

3 Disease acquired in the United Kingdom;

4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;

5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

Please notice where sporadic CJD cases in 1996 went from 28 cases, to 215 cases in 2009, the highest recorded year to date. sporadic CJD is on a steady rise, and has been since 1996.

I also urge you to again notice these disturbing factors in lines 5 and 6 ;

5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.


Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)


Friday, November 30, 2007


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.


AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.


OR nvCJD. ......could look like any one or all of the sporadic CJD's. ...TSS


WHAT about funding in the USA for 2010 on prion disease. a big fat zero dollars $ 0.0 $



"which includes the ___elimination___ of Prion activities ($5,473,000),"

All Other Emerging and Zoonotic Infectious Diseases CDC‘s FY 2012 request of $52,658,000 for all other emerging and zoonotic infectious disease activities is a decrease of $13,607,000 below the FY 2010 level, which includes the elimination of Prion activities ($5,473,000), a reduction for other cross-cutting infectious disease activities, and administrative savings. These funds support a range of critical emerging and zoonotic infectious disease programs such Lyme Disease, Chronic Fatigue Syndrome, and Special Pathogens, as well as other activities described below.

Friday, April 15, 2011


Wednesday, March 9, 2011

27 U.S. Senators want to force feed Japan Highly Potential North America Mad Cow Beef TSE PRION CJD March 8, 2011

President Barack Obama The White House

1600 Pennsylvania Avenue, W Washington, DC 20500

Dear President Obama:

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



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