Monday, November 14, 2011

WYOMING Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011

WYOMING Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011

News Release

Contact: Dennie Hammer


Cody Regional Office 2820 State Highway 120 Cody, WY 82414 Phone: (307) 527-7322 ext. *817 ? Fax: (307) 587-5430


CODY- Chronic wasting disease (CWD), a fatal neurological disease of deer, elk, and moose has been discovered in deer hunt area 165, bringing the known total CWD areas in the Big Horn Basin to fifteen out of thirty-nine.

A white-tailed deer taken on October 15, 2011 near the Greybull River has tested positive for the disease. Hunt area 165 borders deer CWD endemic hunt areas 122 to the north, 124 to the east, and 125 to the south. The disease is now known to occur in Big Horn Basin deer hunt areas 37, 39, 41, 42, 46, 47, 51, 119, 120, 122, 124, 125, 127, 164, and 165. After a review of available scientific data, the World Health Organization in December 1999 stated, "There is currently no evidence that CWD in cervidae (deer and elk) is transmitted to humans." In 2004, Dr. Ermias Belay of the Center for Disease Control said, "The lack of evidence of a link between CWD transmission and unusual cases of CJD, [Creutzfeldt-Jakob disease, a human prion disease] despite several epidemiological investigations, suggest that the risk, if any, of transmission of CWD to humans is low." Nonetheless to avoid risk, both organizations say parts or products from any animal that looks sick and/or tests positive for CWD should not be eaten. Cody region personnel continue to collect samples through hunter field checks, and at CWD sampling stations.

For more information on chronic wasting disease visit the Chronic Wasting Disease Alliance website at -WGFD-

Chronic Wasting Disease

Current scientific knowledge suggests that chronic wasting disease (CWD) prions are primarily found in tissues of the central nervous system (e.g. brain and spinal cord) and lymphatic system (e.g. tonsils, lymph nodes and spleen) of CWD – infected deer and elk. However, research has also identified other tissues with some, most likely small concentrations of the CWD prion. These tissues include heart tissue, eye tissue, skeletal muscle tissue, saliva and blood. Given the wide rage of tissues known to have the prion it is theoretically possible, and even likely, that the prion could be present throughout an infected animal.

Currently there is no scientific evidence to date that humans can acquire a prion related illness from eating meat from a CWD infected animal. However, there is much research to be done on the possibility that humans could acquire CWD. Therefore until more is known about the human health risk, individuals may want to consider the theoretical possibility that a yet-to-be determined human health risk may exist before consuming CWD infected animals. To minimize exposure to prions, both the US Centers for Disease Control and Prevention (CDC) and the World Health Organization (WHO) recommend that animals that have tested positive for CWD not be consumed by humans.

Public health officials recommend certain precautions for hunters to minimize their exposure to CWD prions. Individuals should avoid eating any animal that is obviously ill. Hunters should avoid contact or consumption of tissues of the central nervous system or lymphatic system where CWD prions are known to accumulate. Persons involved in field-dressing carcasses should wear gloves, bone out the meat from the animal and minimize handling of the brain and spinal cord tissues. Individuals may choose to get their animal tested for CWD before consuming its meat. For more information on CWD in Wyoming animals you may contact your Game and Fish representative or go to the Game and Fish website at

A retired biologist who worked at the National Elk Refuge near Jackson is calling for an end to the feeding program and putting forth an idea to train the elk their former migration route south. He is hoping to avoid the oncoming CWD outbreak which will undoubtably infect entire herds and soon enter Yellowstone National Park causing a wildlife disaster. The feeding grounds are like petri dishes for disease because of the high density of elk. Brucellosis thrives on the feeding grounds and continues to have wide ranging repercussions for elk and bison in places far removed from the feeding grounds. Also, CWD has a nasty habit of infecting soils for the long term leaving an area capable of infecting elk for years and years.

Where Elk Roam: Conservation and Biopolitics of Our National Elk Herd

Thursday, July 08, 2010

CWD Controversy still stalking elk feedgrounds in Wyoming 2010


This is very serious, please notice that one of the CWD clusters is only 45 miles from ELK feeding grounds in Wyoming, the second elk feeding ground is 98 miles from CWD cluster, and the third elk feeding ground is 130 miles from the CWD cluster. Common sense tells us we need to stop those feeding grounds, if you want your Elk to survive. There is no politics or plot against the hunters or elk about it. read the science please. ...TSS

chronic wasting disease proximity to elk feedgrounds in wyoming 2009-2010

Monday, December 13, 2010



Friday, November 12, 2010


Sunday, October 31, 2010


Wednesday, October 20, 2010


Wyoming Department of Health Reportable Diseases and Conditions

A report is required by law from both the attending healthcare provider/hospital and the laboratory performing diagnostic testing. Wyoming laboratories are responsible for reporting results when a reference laboratory is used. Mail reports to: Wyoming Department of Health, 6101 Yellowstone Road Suite 510, Cheyenne, WY 82002 OR Fax reports to our secure fax machine at (307) 777-5573 or phone (307) 777-3593 as indicated.

DISEASES IN RED: Immediate Notification at 1-888-996-9104

Diseases in Black: Reportable within 24 hours of diagnosis by fax or telephone

Diseases in Green: Reportable within 7 days of diagnosis by fax, phone, or mail

LAB: In addition to reporting, submit an isolate in accordance with IATA Dangerous Goods Regulations to: State Public Health Laboratory, Hathaway Building-Fifth Floor, 2300 Capitol Avenue, Cheyenne, WY 82002 (307) 777-7431

Creutzfeldt-Jacob Disease (including classic CJD and variant CJD

WYFDA-Wyoming Funeral Directors Association and CJD TSE Prion disease

Oral.40: Monitoring the Potential Transmission of Chronic Wasting Disease to Humans

Ermias D. Belay,1,† Joseph Abrams,1 Janell Kenfield,2 Kelly Weidenbach,3 Ryan A. Maddox,1 Elisabeth Lawaczeck2 and Lawrence B. Schonberger1

1 Centers for Disease Control and Prevention; Atlanta, GA USA; 2 Colorado Department of Public Health and Environment; Denver, CO USA; 3 Wyoming Department of Health; Cheyenne, WY USA†Presenting author; Email:

Introduction: Chronic wasting disease (CWD) has been occurring for several decades among wild cervids in Colorado and Wyoming. The increasing detection of CWD in an additional 12 US states and two Canadian provinces may have resulted in increased human exposure to CWD. Although studies have evaluated the possible transmission of CWD to humans in laboratory models, a reliable assessment requires conducting epidemiologic and laboratory studies designed to identify prion disease among humans exposed to CWD and generating scientific evidence causally linking the two illnesses.

Methods: In collaboration with the Centers for Disease Control and Prevention, the Wyoming Department of Health and the Colorado Department of Public Health and Environment established a long-term follow-up study of hunter data to monitor the potential CWD transmission to humans. Personal identifiers from deer or elk hunter database are cross-checked with mortality data to determine their mortality status and causes of death.

Results: In Colorado, the hunter data include about 4.9 million records of licenses purchased during 1995–2008, representing about 1.1 million hunters. Overall, 48% of hunters purchased a license to hunt in areas that included CWD positive game units and 47% to hunt anywhere in Colorado. In Wyoming, the data include about 1.2 million records of licenses purchased during 1996-2009, representing about 0.5 million hunters; 34% of hunters purchased a license to hunt in areas that included CWD positive game units and 28% to hunt anywhere in Wyoming. During the study period three Colorado hunters (expected number: 3-15 cases) and three Wyoming hunters (expected number: 0-6 cases) were identified to have died of Creutzfeldt-Jakob disease (CJD).

Conclusions: No evidence suggests that the CJD incidence is higher than expected among persons who hunted in Colorado or Wyoming. The hunter data are valuable for monitoring the potential transmission of CWD to humans. Ongoing assessment and long-term follow-up of the hunter population is necessary because human prion diseases are associated with long latency periods and the pathogenicity of CWD might change over time.

Saturday, November 12, 2011


Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease Fri, 22 Sep 2006 09:05:59 -0500

"We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD)."

"Additionally, routine surveillance has not shown any increase in the incidence of Creutzfeldt-Jakob disease in Colorado or Wyoming."


HOW RELIABLE IS REVIEWING DEATH CERTIFICATE DATA FOR SURVIELLANCE OF CJD, instead of making CJD and all Prion disease reportable Nationally ???

it's not reliable at all, that's the whole purpose of only _reviewing_ death certificate data for CJD TSE prion disease, and not making it reportable Nationally, Government officials do NOT want the public to know how many cases there are. IT's the same as with mad cow disease, if you don't look, you don't find. simple as that. ...tss



Tuesday, November 08, 2011

Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008 Vol. 37, No. 3-4, 2011

Original Paper

***Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.



One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...


Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R. Will



Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...

full text;

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.


Friday, November 04, 2011

Diagnostic accuracy of cerebrospinal fluid protein markers for sporadic Creutzfeldt-Jakob disease in Canada: a 6-year prospective study Research article


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.

NOR IS THE FDA recalling this CWD positive elk meat for the well being of the dead elk ;

Wednesday, March 18, 2009

Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS AND FIELD CORRECTIONS: FOODS CLASS II

Envt.06: Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates

Emmanuel Comoy,1,† Valérie Durand,1 Evelyne Correia,1 Aru Balachandran,2 Jürgen Richt,3 Vincent Beringue,4 Juan-Maria Torres,5 Paul Brown,1 Bob Hills6 and Jean-Philippe Deslys1

1Atomic Energy Commission; Fontenay-aux-Roses, France; 2Canadian Food Inspection Agency; Ottawa, ON Canada; 3Kansas State University; Manhattan, KS USA; 4INRA; Jouy-en-Josas, France; 5INIA; Madrid, Spain; 6Health Canada; Ottawa, ON Canada†Presenting author; Email:

The constant increase of chronic wasting disease (CWD) incidence in North America raises a question about their zoonotic potential. A recent publication showed their transmissibility to new-world monkeys, but no transmission to old-world monkeys, which are phylogenetically closer to humans, has so far been reported. Moreover, several studies have failed to transmit CWD to transgenic mice overexpressing human PrP.

Bovine spongiform encephalopathy (BSE) is the only animal prion disease for which a zoonotic potential has been proven. We described the transmission of the atypical BSE-L strain of BSE to cynomolgus monkeys, suggesting a weak cattle-to-primate species barrier. We observed the same phenomenon with a cattle-adapted strain of TME (Transmissible Mink Encephalopathy). Since cattle experimentally exposed to CWD strains have also developed spongiform encephalopathies, we inoculated brain tissue from CWD-infected cattle to three cynomolgus macaques as well as to transgenic mice overexpressing bovine or human PrP. Since CWD prion strains are highly lymphotropic, suggesting an adaptation of these agents after peripheral exposure, a parallel set of four monkeys was inoculated with CWD-infected cervid brains using the oral route.

Nearly four years post-exposure, monkeys exposed to CWD-related prion strains remain asymptomatic. In contrast, bovinized and humanized transgenic mice showed signs of infection, suggesting that CWD-related prion strains may be capable of crossing the cattle-to-primate species barrier. Comparisons with transmission results and incubation periods obtained after exposure to other cattle prion strains (c-BSE, BSE-L, BSE-H and cattle-adapted TME) will also be presented, in order to evaluate the respective risks of each strain.

link url not available, please see PRION 2011 ;

see more here on CWD and the Zoonotic Potential potential there from, and then the pass it forward mode through multiple routes and sources, i.e. iatrogenic CJD. ...tss

Monday, June 27, 2011

Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates

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 ;


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.

From: TSS (


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

From: "Belay, Ermias"


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

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


Dear Sir/Madam,

In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.

That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.

Ermias Belay, M.D. Centers for Disease Control and Prevention

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


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

To:;; ebb8@CDC.GOV


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

Wednesday, October 12, 2011

White-tailed deer are susceptible to the agent of sheep scrapie by intracerebral inoculation

Wednesday, July 06, 2011

Swine Are Susceptible to Chronic Wasting Disease by Intracerebral Inoculation


Journal of Wildlife Diseases, 47(1), 2011, pp. 78-93 © Wildlife Disease Association 2011

Wednesday, September 21, 2011

Evidence for distinct CWD strains in experimental CWD in ferrets

Tuesday, May 31, 2011

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

Chronic Wasting Disease Susceptibility of Four North American Rodents

Chad J. Johnson1*, Jay R. Schneider2, Christopher J. Johnson2, Natalie A. Mickelsen2, Julia A. Langenberg3, Philip N. Bochsler4, Delwyn P. Keane4, Daniel J. Barr4, and Dennis M. Heisey2 1University of Wisconsin School of Veterinary Medicine, Department of Comparative Biosciences, 1656 Linden Drive, Madison WI 53706, USA 2US Geological Survey, National Wildlife Health Center, 6006 Schroeder Road, Madison WI 53711, USA 3Wisconsin Department of Natural Resources, 101 South Webster Street, Madison WI 53703, USA 4Wisconsin Veterinary Diagnostic Lab, 445 Easterday Lane, Madison WI 53706, USA *Corresponding author email:

We intracerebrally challenged four species of native North American rodents that inhabit locations undergoing cervid chronic wasting disease (CWD) epidemics. The species were: deer mice (Peromyscus maniculatus), white-footed mice (P. leucopus), meadow voles (Microtus pennsylvanicus), and red-backed voles (Myodes gapperi). The inocula were prepared from the brains of hunter-harvested white-tailed deer from Wisconsin that tested positive for CWD. Meadow voles proved to be most susceptible, with a median incubation period of 272 days. Immunoblotting and immunohistochemistry confirmed the presence of PrPd in the brains of all challenged meadow voles. Subsequent passages in meadow voles lead to a significant reduction in incubation period. The disease progression in red-backed voles, which are very closely related to the European bank vole (M. glareolus) which have been demonstrated to be sensitive to a number of TSEs, was slower than in meadow voles with a median incubation period of 351 days. We sequenced the meadow vole and red-backed vole Prnp genes and found three amino acid (AA) differences outside of the signal and GPI anchor sequences. Of these differences (T56-, G90S, S170N; read-backed vole:meadow vole), S170N is particularly intriguing due its postulated involvement in “rigid loop” structure and CWD susceptibility. Deer mice did not exhibit disease signs until nearly 1.5 years post-inoculation, but appear to be exhibiting a high degree of disease penetrance. White-footed mice have an even longer incubation period but are also showing high penetrance. Second passage experiments show significant shortening of incubation periods. Meadow voles in particular appear to be interesting lab models for CWD. These rodents scavenge carrion, and are an important food source for many predator species. Furthermore, these rodents enter human and domestic livestock food chains by accidental inclusion in grain and forage. Further investigation of these species as potential hosts, bridge species, and reservoirs of CWD is required.

please see ;

Thursday, June 09, 2011

Detection of CWD prions in salivary, urinary, and intestinal tissues of deer: potential mechanisms of prion shedding and transmission


Wednesday, September 08, 2010


Wednesday, January 5, 2011



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



Oral.22: Transmission and Pathogenesis of Chronic Wasting Disease in Cervid and Non-Cervid Species

Edward Hoover,† Candace K. Mathiason, Nicholas J. Haley, Timothy D. Kurt, Davis M. Seelig, Amy V. Nalls, Mark D. Zabel, Glenn C. Telling

Department of Microbiology, Immunology, and Pathology; Colorado State University; Fort Collins, CO; Department of Microbiology, Immunology and Molecular Genetics and Neurology; University of Kentucky Medical Center; Lexington, KY USA†Presenting author

Now recognized in 18 states in the US, two Canadian provinces, and one Asian country, efficient horizontal transmission is a signature trait of chronic wasting disease (CWD) of cervids. The facile spread of CWD appears linked to the prion/host relationship facilitating efficient mucosal uptake, peripheral lymphoreticular amplification, and horizontal dissemination exploiting excretory tissues and their products. In addition, recent studies suggest the likelihood of early life mother to offspring transmission. Growing evidence from studies of cervid CWD exposure by natural routes indicate that the incubation period for overt infection detection and disease onset (if any) may be much longer than originally thought.

Whether non-cervid species (including humans) may be susceptible to CWD infection and/or act as reservoirs for infection in nature remains unknown. In vitro and in vivo studies of the CWD species barrier indicate the potential for a host range extending beyond cervid species, although no evidence for this has thus far been detected in nature. Interestingly, rodent and mustelid species sympatric with free ranging cervids have been shown susceptible to CWD prions and such trans-species infection broadens the host range/strain characteristics of CWD prions. While the origins of CWD remain unknown, the relationship between sheep scrapie and CWD and the existence of multiple CWD prion strains/quasispecies remain interesting and merit further investigation.

Oral.26: Minor Oral Lesions Facilitate CWD Infection

Nathaniel D. Denkers,1,† Glenn C. Telling2 and Edward A. Hoover1

1Colorado State University; Fort Collins, CO USA; 2University of Kentuckty; Lexington, KY USA†Presenting author; Email:

Purpose: While the exact mechanisms of chronic wasting disease (CWD) prion transmission, entry, and trafficking remain incompletely elucidated, transmission by exposure of the oral and/or nasal mucous membranes seems certain. As part of foraging, cervids likely experience minor lesions in the oral mucous membranes; these could have impact on susceptibility to prion entry and subsequent infection. To explore this potential co-factor, we used cervid PrP transgenic mice to assess whether or not micro-abrasions to the tongue may enhance susceptibility to oral CWD infection and whether or not infectious CWD PrPCWD could be detected immediately after exposure.

Methods: Two sets of FVB mice transgenically expressing the normal cervid PrPC protein [Tg(CerPrP-E226)5037+/-], with or without abrasions on the lingual mucosa, were inoculated orally with 10µl of a 10% w/v brain homogenate from either CWD-positive or negative deer. Abrasions were created by lightly scratching the dorsal lingual epithelium with a 27g needle. Cohorts were sacrificed at either early [0, 1, and 4 h post inoculation (pi)] or late [3, 12, and 24 months pi] time points or when signs of neurologic disease were observed. Tongue, lymphoid tissue, and the brain were assessed by western blotting and tyramide signal amplification (TSA) immunohistochemistry to detect the CWD abnormal prion protein (PrPCWD).

Results: Between 296 and 515 dpi, 9 of the 9 CWD-inoculated mice with lingual lesions developed clinical signs of neurologic dysfunction mandating euthanasia. Only the brain in all nine mice was positive for PrPCWD by western blot and TSA immunohistochemistry. Conversely, all mice without oral lesions remained asymptomatic for >700 dpi and no evidence of PrPCWD was detected in these mice terminally. Moreover, no evidence of PrPCWD could be detected when the micro-abrasion sites were examined at 0, 1, or 4 h after oral exposure or at any pre-terminal time point thereafter.

Conclusions: Micro-abrasions to the lingual surface substantially facilitated CWD transmission, suggesting that minor oral mucosal lesions may be a significant co-factor facilitating infection in foraging cervids or other species.

Oral.27: Identification of PrPCWD in the Salivary Gland Epithelium of White-Tailed Deer: Novel Insights Into Mechanisms of CWD Horizontal Transmission

Davis Seelig,1,† Gary Mason,1 Glenn Telling2 and Edward Hoover1

1Colorado State University; Fort Collins, CO USA; 2University of Kentucky; Lexington, KY USA†Presenting author; Email:

Background. Chronic wasting disease (CWD) of cervids is characterized by its efficient transmission among animals. Although bioassay and in vitro amplification studies have confirmed the infectious nature of saliva, urine, blood and feces, uncertainties remain regarding the mechanisms of this facile horizontal transmission. Notable among these is a specific understanding of the means by which prion infectivity is transferred to a body fluid or excretion.

Objectives. The chief objective of this work was to provide tissue-level insights into the process of prion shedding via the salivary glands by means of enhanced immunohistochemistry (IHC).

Methods. Formalin fixed, paraffin-embedded tissues from CWD-infected white-tailed deer (WTD) were evaluated for the presence of PrPCWD using sensitive amplified immunohistochemistry (IHC) methods employing, citrate buffer-based heat-induced epitope retrieval, tyramide signal amplification (TSA), and a polyclonal anti-prion protein antisera.

Results. Here we show that enhanced IHC techniques are capable of detecting pathogenic prion protein (PrPCWD) in the salivary glands of infected WTD. Utilizing optimized TSA we have detected granular to clumped, intra-cytoplasmic PrPCWD deposits in parotid and mandibular salivary gland ductular epithelial cells of WTD infected with CWD for 19 to 27 months. Salivary PrPCWD was not detected in sham-inoculated or naïve WTD. PrPCWD was not identified in any other salivary gland cell types.

Discussion. We present immunohistochemical evidence for PrPCWD accumulation in the salivary gland ductules, which provides a tissue level correlate to the infectivity present in cervid saliva and may explain the manner by which prions transit to saliva, and thereby facilitate the high degree of CWD horizontal transmission. These findings complement work by Haley et al. (this symposium) demonstrating the presence of CWD prions in salivary glands through the in vitro amplification assay PMCA. 12 Prion Volume 5 Supplement

Oral.28: Differential CWD Infection and Mortality of PRNP Genotypes in White-Tailed Deer: Implications for Genetic Selection, Disease Modeling and Management

Michael D. Samuel,1,† Stacie J. Robinson,1 Chad J. Johnson,1 Marie Adams1 and Debbie I. McKenzie2

1University of Wisconsin; Madison, WI USA; 2University of Alberta; Edmonton, AB Canada†Presenting author; Email:

Infectious diseases are increasingly recognized as an important force driving population dynamics, conservation biology and natural selection in wildlife populations. Infectious agents have been implicated in the decline of small or endangered populations and may act to constrain population size, distribution, or growth rates. Further, diseases may provide selective pressures that shape the genetic diversity of populations or species. Thus understanding disease dynamics and selective pressures from pathogens is crucial to understanding population processes, managing wildlife diseases, and conserving biological diversity. There is ample evidence that variation in the PRNP gene impacts host susceptibility to TSEs. Research in human TSEs and scrapie, as well as chronic wasting disease (CWD) has demonstrated that PRNP variation influences the susceptibility and progression of prion diseases. Still, little is known about how these genetic differences might influence natural selection within cervid populations or how genetic make-up might shape disease dynamics and the population response to CWD. Here we determine the links between genetic variation, CWD transmission, and susceptibility of white-tailed deer (Odocoileus virginianus) with implications for fitness and disease-driven genetic selection. We developed a single nucleotide polymorphism (SNP) assay to efficiently genotype deer at the 96th codon of the PRNP gene. We then used a Bayesian modeling approach to calculate genotype-specific infection and disease mortality rates. We found that the more susceptible (homozygous) genotype had over four times greater risk of CWD infection and, once infected, deer with the resistant (heterozygous) genotype survived 49% longer (8.25 more months). We used these epidemiological parameters in a multi-stage population matrix model to evaluate genotype-specific population growth. The differences in disease infection and mortality rates allowed genetically resistant deer to achieve higher population growth and obtain a long-term fitness advantage. This differential fitness produced a selection coefficient of over 1% favoring the heterozygous CWD-resistant genotype. Such strong selective pressure suggests that the resistant allele could become dominant in the population within just a few hundred years, extremely rapid on the evolutionary time scale. Our results have direct implications for the epidemiology, dynamics, and future trends in CWD transmission and spread.

Oral.29: Susceptibility of Domestic Cats to CWD Infection

Amy Nalls, Nicholas J. Haley, Jeanette Hayes-Klug, Kelly Anderson, Davis M. Seelig, Dan S. Bucy, Susan L. Kraft, Edward A. Hoover and Candace K. Mathiason†

Colorado State University; Fort Collins, CO USA†Presenting author; Email:

Domestic and non-domestic cats have been shown to be susceptible to one prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted through consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free ranging felids scavenge cervid carcasses, including those in CWD affected areas, we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between 40–43 months following IC inoculation, two cats developed mild but progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on the brain of one of these animals (vs. two age-matched controls) performed just before euthanasia revealed increased ventricular system volume, more prominent sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere and in cortical grey distributed through the brain, likely representing inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles were demonstrated in the brains of both animals by immunodetection assays. No clinical signs of TSE have been detected in the remaining primary passage cats after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5) of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC inoculated cats are demonstrating abnormal behavior including increasing aggressiveness, pacing, and hyper responsiveness. Two of these cats have developed rear limb ataxia. Although the limited data from this ongoing study must be considered preliminary, they raise the potential for cervid-to-feline transmission in nature.

Oral.44: Genetic Variability and Association with Prion Disease Susceptibility of the Prion Gene in the Mammalian Order Carnivora

Paula Stewart,1 Karen Griffin,8 Jon E. Swenson,2 Jens Persson,11 Olof Liberg,11 Jon M. Arnemo,3, 4 Thierry Baron,5 Martin Groschup,6 Danielle Gunn-Moore,9 Simon Girling,10 Michael W. Miller,8 Michael Tranulis7 and Wilfred Goldmann,1,†

1The Roslin Institute and Royal (Dick) School of Veterinary Studies, University of Edinburgh; Easter Bush, Midlothian, UK; 2Department of Ecology and Natural Resources Management, Norwegian University of Life Sciences; As, Norway; 3Department of Forestry and Wildlife Management, Hedmark University College; Campus Evenstad, Norway; 4Department of Wildlife, Fish and Environmental Studies, Faculty of Forest Sciences, Swedish University of Agricultural Sciences; Umea, Sweden; 5Agence Française de Sécurité Sanitaire des Aliments; Lyon, France; 6Friedrich Loeffler Institut; Riems, Germany; 7Department of Basic Sciences & Aquatic Medicine, Norwegian School of Veterinary Science; Oslo, Norway; 8Wildlife Research Center, Colorado Division of Wildlife; Fort Collins, CO USA; 9Small Animal Hospital, Royal (Dick) School of Veterinary Studies, University of Edinburgh; Edinburgh, UK; 10The Royal Zoological Society of Scotland, Edinburgh Zoo; Edinburgh, UK; 11Grimsö Wildlife Research Station, Department of Ecology, Swedish University of Agricultural Sciences ; Riddarhyttan, Sweden†Presenting author; Email:

Carnivores are exposed to significant levels of CWD in some regions of the US and Canada. Indeed it has been proposed recently that mountain lions prey selectively on prion–infected mule deer. It is likely that predators have also at least occasionally been exposed to other prion diseases, such as sheep scrapie in other countries. How susceptible are predators and scavengers to prion diseases? It is well known that the prion protein sequence is important as a major modulator of susceptibility and pathogenesis of prion disease. For example, prion disease susceptibility in sheep, goats and deer is modulated by at least 15 different polymorphisms of the PrP protein. PrP sequencing of carnivore species has not been done in great numbers and the degree of genetic variation of their PrP in wild and domesticated populations has not been addressed in any detail. However, to estimate the genetic risk of populations to diseases such as CWD one needs to understand the genetic variation of the target species.

We have analyzed the prion protein sequence of over 450 samples from over 20 species/subspecies of the suborders feliformia (cat-like) and caniformia (dog-like) representing ~320 samples from wild populations (US, Europe), ~110 samples from companion animals and ~25 samples from zoo collections. Within these samples were nine FSE cat cases, including the index case from the UK and six FSE cheetahs.

We established the PrP protein variants in our sample set and conclude that the number of PrP variants is small, with slightly more variability in caniformia than feliformia. All feline prion sequences have a characteristic alanine change in their repeat region that is not seen in any other species; all canine PrP encode aspartic acid in position 163, which is not present in any other species with the exception of wolverines. We hypothesis that these differences may explain some of the difference observed in prion disease susceptibility. The analysis of the FSE cases revealed no additional mutations therefore excluding the possibility of particularly susceptible PrP genotypes.

Although the general susceptibility of predators to CWD has not been established, we predict that it is unlikely that species

such as mountain lion and black bear will be protected by resistant alleles, whereas wolf and wolverine may have a slightly higher susceptibility threshold.

W.G. and P.S. supported by Institute Strategic Grant funding from the BBSRC, UK.

Oral.39: Crisis and Opportunity: Chronic Wasting Disease, Indigenous Peoples and Cross-Cultural Research and Communication Regarding Wildlife and Environmental Health

Stephane McLachlan,1,† Helen Cote-Quewezance,2 Stefan Epp,1 Carmen Fuentealba,3 Katie Peterson,1 Misty Potts-Sanderson,4 Dean Rennie,1 Manon Roy,1 Joyce Slater,1 Tamara Steffensen,1 Troy Stozek1 and Anna Weier1

1 University of Manitoba; Winnipeg, MB Canada; 2 Cote First Nation; Kamsack, SK Canada; 3 University of Calgary; Calcary, AB Canada; 4 Alexis Nakota Sioux Nation; Glenevis, AB Canada†Presenting author

Many Indigenous communities in western Canada are concerned about increases in wildlife disease and environmental contamination that threaten their longstanding use of moose, elk and deer. Over the last three years we have been exploring the implications of these changes in close collaboration with two First Nations in Alberta (Alexis Nakota Sioux Nation and Paul First Nation) and a third First Nation in Saskatchewan (Cote First Nation). The overall goal of our project has been to better understand and respond to chronic wasting disease and other potential threats to wildlife and environmental health that confront these communities. The project is holistic and cross-cultural in approach, bridging Western science with Traditional Knowledge, and has been shaped and controlled by the community partners at all stages.

The objective of the first phase of the project was to better understand the implications of CWD as well as the role of cervids (i.e. moose, elk, and deer) in the diets and culture of these communities. It focused on interviews and participatory mapping. The objective of the second phase was to identify any factors responsible for ongoing declines in the health of cervids, and to assess to what degree these declines have been associated with CWD and the adverse effects of surrounding oil and gas extraction, agriculture and deforestation. Hunters collected samples of moose, deer, and elk and these samples have been tested for CWD, parasites, heavy metals, and other contaminants.

The objective of third phase was to explore the role of risk communication regarding CWD as it affects Indigenous communities and other stakeholders. This was, in part, achieved by evaluating media coverage and governmental communication with Indigenous communities regarding CWD and more generally wildlife health. Results show there has been little effective communication with or inclusion of Indigenous Peoples in government risk communication strategies. What communication did exist was generally culturally inappropriate, inaccessible, and, often generated community concern and even fear. However, we have developed a number of effective approaches to knowledge exchange in this collaborative project. They include an interactive website (, plain-language newsletters, participatory video, and campouts on the land.

Outcomes of all three phases of this project have important implications for the understanding and communication of risks associated with CWD as they affect Indigenous communities. Yet, our findings are also relevant for risk communication with many stakeholders and a wide variety of threats associated with declining wildlife and environmental health.

Bio.048: PRNP Polymorphisms Generate Different PrPCWD Proteins in Orally Inoculated White-Tailed Deer (Odocoileus Virginianus)

Juan C. Duque Velasquez,1,† Allen Herbst,1 Chad Johnson,2 Judd Aiken1 and Debbie McKenzie1

1Centre For Prions and Protein Folding Diseases-University of Alberta; Edmonton, AB Canada; 2Department of Soil Science, University of Wisconsin; Madison, WI USA;†Presenting author; Email:

Prion strains have been identified in virtually every species affected by these transmissible neurological disorders. Prion strains can exhibit unique clinical symptoms, disease incubation period, biochemical characteristics of the prion protein, as well as species tropism. We have previously demonstrated that specific PRNP polymorphisms are linked to resistance to chronic wasting disease (CWD) infection in free-ranging white-tailed deer populations. In hunter-harvested CWD-positive deer, the "wild-type" alleles (with glutamine at 95 aa and glycine at 96 aa) were over-represented while the 95 (95H) and 96 (96S) polymorphisms were under-represented. Experimental oral infection of white-tailed deer with known PRNP genotypes (with inoculum from CWD-positive wt/wt deer) confirmed this link between prion protein primary sequence and incubation period. All orally infected animals became clinically positive for CWD. The wt/wt had the shortest incubation period (693 dpi); the animal heterozygous for 95H/96S the longest (1596 dpi). Analysis of the CWD isolates revealed biochemical and biophysical differences between PrPCWD from wt/wt deer and H95/S96 deer. These proteins can be distinguished by full-length PrP glycoform patterns, proteinase K resistance, molecular weight, sedimentation properties in N-Lauroylsarkosine and structural stability to chaotropes. The presence of these PrPCWD isoforms suggests that white-tailed deer can generate several different strains of CWD agent; potentially with different transmission properties.

==============tss=============== Prion

Sunday, July 27, 2008

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

-------- 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."


Greetings FDA,

i would kindly like to comment on;

Docket 03D-0186

FDA 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 healthy ones as well. it 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 the USA. 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 these TSE 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 disease PrPres in mule deer fawns (Odocoileus hemionus ) Christina J. Sigurdson1, Elizabeth S. Williams2, Michael W. Miller3, Terry R. Spraker1,4, Katherine I. O'Rourke5 and Edward A. Hoover1

Department of Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO 80523- 1671, USA1 Department of Veterinary Sciences, University of Wyoming, 1174 Snowy Range Road, University of Wyoming, Laramie, WY 82070, USA 2 Colorado Division of Wildlife, Wildlife Research Center, 317 West Prospect Road, Fort Collins, CO 80526-2097, USA3 Colorado State University Veterinary Diagnostic Laboratory, 300 West Drake Road, Fort Collins, CO 80523-1671, USA4 Animal Disease Research Unit, Agricultural Research Service, US Department of Agriculture, 337 Bustad Hall, Washington State University, Pullman, WA 99164-7030, USA5

Author for correspondence: Edward Hoover.Fax +1 970 491 0523. e-mail

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


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


snip...see full text ;

-------- 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."


Greetings FDA,

i would kindly like to comment on;

Docket 03D-0186

FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability


Experimental oral transmission of chronic wasting disease to red deer

(Cervus elaphus elaphus): Early detection and late stage distribution

of protease-resistant prion protein

Aru Balachandran, Noel P. Harrington, James Algire, Andrei Soutyrine, Terry R. Spraker,

Martin Jeffrey, Lorenzo González, Katherine I. O’Rourke

Abstract — Chronic wasting disease (CWD), an important emerging prion disease of cervids, is readily transmitted by intracerebral or oral inoculation from deer-to-deer and elk-to-elk, suggesting the latter is a natural route of exposure. Studies of host range susceptibility to oral infection, particularly of those species found in habitats where CWD currently exists are imperative. This report describes the experimental transmission of CWD to red deer following oral inoculation with infectious CWD material of elk origin. At 18 to 20 months post-inoculation, mild to moderate neurological signs and weight loss were observed and animals were euthanized and tested using 3 conventional immunological assays. The data indicate that red deer are susceptible to oral challenge and that tissues currently used for CWD diagnosis show strong abnormal prion (PrPCWD) accumulation. Widespread peripheral PrPCWD deposition involves lymphoreticular tissues, endocrine tissues, and cardiac muscle and suggests a potential source of prion infectivity, a means of horizontal transmission and carrier state.


There is a strong correlation between the presence of PrPTSE and infectivity in prion diseases. Although the epidemiologic evidence strongly suggests that CWD is not transmissible to humans, this study and others suggest caution in this regard. The finding of PrPCWD in various organs, albeit in clinical CWD, suggests that humans who consume or handle meat from CWD-infected red deer may be at risk of exposure to CWD prions. This study found that red deer tissues other than nervous and lymphoid tissue can support CWD prion replication and accumulation. As a result, the consumption or handling of meat from CWD-infected red deer will put humans at risk of exposure to CWD prions. In spite of a well-documented species barrier, a cautious approach would involve preventing such tissues from entering the animal and human food chains. Future studies will require sensitive and quantitative techniques such as bioassays in transgenic mice that assess tissue infectivity and quantitative immunoassays adapted to PrPCWD detection in peripheral tissues.


The exact mode of transmission of CWD in nature remains unclear but is believed to involve direct animal-to-animal contact or environmental contamination. As TSE agents are extremely resistant in the environment (39), oral exposure is the most plausible pathway by which the CWD prion may be introduced to deer in nature and represents a significant obstacle to eradication of CWD from either farmed or free-ranging cervid populations. The distribution of PrPCWD in gut-associated lymphoid tissues, salivary glands, and nasal mucosa in the red deer of this study suggests potential routes of PrPCWD shedding into the environment via fluids such as saliva or feces. However, this study did not identify the point at which an animal may become infectious during the course of infection. An improved understanding of the mechanisms of shedding and transmission will be important in the future management of CWD.


In summary, this study demonstrates the potential for oral transmission of CWD to red deer and describes the pattern of PrPCWD accumulation for this species. The current surveillance testing regime for cervids would be expected to identify CWD-infected red deer should it occur in North America. These results confirm the usefulness of rapid tests such as ELISA but with generally slightly lower sensitivity when compared with IHC when testing tissues with patchy or sporadic PrPCWD deposition. The finding of PrPCWD in several extraneural tissues including cardiac muscle and the endocrine system suggests that further investigation and monitoring of the potential transmissibility to other species including humans is warranted.


(Traduit par Isabelle Vallières)

Can Vet J 2010;51:169–178

Ottawa Laboratory — Fallowfield, Canadian Food Inspection Agency, Ottawa, Ontario (Balachandran, Harrington, Algire,

Soutyrine); Veterinary Diagnostic Laboratory, Colorado State University, Fort Collins, Colorado, USA (Spraker); Veterinary

Laboratory Agency, Department for the Environment, Food & Rural Affairs, Lasswade, Midlothian, Scotland, United Kingdom

(Jeffrey, González); Animal Disease Research Unit, Agricultural Research Service, United States Department of Agriculture, Pullman,

Washington, USA (O’Rourke).

Address all correspondence to Dr. Aru Balachandran; e-mail:

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)



Subject: NOR98-LIKE STRAIN OF SCRAPIE FOUND IN WYOMING Date: April 11, 2007 at 12:47 pm PST


March 16, 2007

Wyoming Livestock Board

2020 Carey Avenue 4th Floor

Cheyenne, Wyoming 82002

For more information contact: Dr. Walter Cook at (307) 631-2974 [weekend] or (307) 777-6443 [weekday]



CHEYENNE, Wyo. - On Friday, March 16, 2007, the Wyoming Livestock Board (WLSB) was notified by officials of the USDA Animal Plant Health Inspection Service (APHIS) that an adult female sheep had tested positive for a form of scrapie consistent with the Nor98 strain. The ewe was slaughtered in Michigan, where it was tested as part of USDA’s regulatory scrapie slaughter surveillance program and traced back to a flock in Wyoming. The results of this case are distinctly different from those seen for bovine spongiform encephalopathy (BSE) or classical scrapie.

Scrapie is a transmissible spongiform encephalopathy and falls into the same category of diseases as chronic wasting disease, found in deer and elk, and bovine spongiform encephalopathy, found in cattle. The disease is limited to sheep and goats and takes years to affect an animal after it has been infected. Scrapie causes sheep to itch and scratch (scrape) wool off, change their behavior and lose body condition; it ultimately ends in death.

Nor98-like scrapie differs from classical scrapie in the distribution of brain lesions and in the course of disease progression and epidemiology. Some sheep that are genetically resistant to the classic form of the disease may be susceptible to the Nor98-like strain. Oddly, Nor98-like scrapie is usually diagnosed during surveillance in animals without clinical signs. There are no known human health risks associated with either form of scrapie.

This is the first time a Nor98-like strain of scrapie has been documented in the United States. It gets the "Nor98-like" name because it is similar to a case first diagnosed in Norway in 1998. This strain of scrapie is a rare disease even in Europe. Since 1998, fewer than 300 cases have been diagnosed in all of Europe. It is usually seen in single animals and does not tend to become widespread in a flock. In contrast, in flocks infected by classical scrapie typically more than 10 percent of the genetically susceptible animals test positive.

"This provides evidence that the surveillance program is working," said Bryce Reece, executive director of the Wyoming Wool Growers Association. "It also indicates that the program is on the cutting-edge of science to detect such a rare disease during standard surveillance."

The Wyoming Livestock Board does not expect the Nor98-like strain of scrapie to become a major disease problem for the sheep industry in Wyoming. Risk is limited because diagnosis of Nor98-like scrapie is usually an incidental event, with even highly-exposed flock mates of the positive animal normally unaffected.

The infected ewe lambed in it in what is considered a low-risk, range-lambing environment. Nonetheless, the WLSB, APHIS and the Wyoming Wool Growers Association plan to assertively pursue this case to make sure that this strain of scrapie is extinguished and does not establish itself in the U.S.

The agencies continue to encourage producers to monitor their sheep for signs of scrapie and other diseases, and to notify their veterinarian if they discover anything unusual.

The positive ewe was purchased as an adult within the last several years and moved to a Wyoming flock near the Black Hills. The producer was notified and his flock quarantined as a precautionary measure. An epidemiologic investigation is ongoing and the producer has been cooperative. The case fits the pattern found in Europe - a single, older sheep that was not exhibiting clinical signs of scrapie.

The regulatory scrapie slaughter surveillance program is a targeted slaughter surveillance program for sheep and goats designed to identify infected animals and flocks. USDA is conducting this surveillance as part of a program to eradicate scrapie from the United States by the end of 2010. Reece said that the sheep industry supports this program and is committed to eliminating scrapie from the United States.



update Date: April 18, 2007 at 2:31 pm PST

Wyo. flock to be killed, tested after scrapie found By The Associated Press

CHEYENNE - Somewhere near Moorcroft, in an unincorporated area of northeastern Wyoming, a livestock owner will hand over his entire flock of sheep next week to the federal government for a mass execution. The rancher knows what will happen: his herd of roughly 300 sheep will be transported live out of state and taken to a slaughter plant where they will be euthanized, their brains and lymph node tissue harvested for testing.

He'll lose his herd because he owned the first U.S. sheep to test positive for a rare strain of scrapie _ a disease found in sheep and goats that's similar to mad cow disease in cattle and chronic wasting disease in sheep and elk.

Still, state statute prohibits officials from releasing the rancher's identity, and attempts by The Associated Press to reach him were unsuccessful.

Scrapie itself is rare in the United States. Out of more than 115,000 animals tested since 2003, only 300 have tested positive; federal officials hope to eliminate scrapie from U.S. herds by the end of 2010.

But the Wyoming rancher's case is even more rare: Fewer than 300 cases worldwide have been recorded of the "Nor98-like" strain of scrapie, so-named because it was first diagnosed in Norway in 1998.

"This is very unusual," Larry Cooper, regional spokesman in Fort Collins, Colo., for Animal Plant Health Inspection Service, or APHIS, said of the first discovery of a Nor98-like strain of scrapie in the U.S.

"It doesn't indicate that we're going to have mass outbreaks of this particular strain, it just indicates that one of these animals from Europe ended up in our system."

There are no known human health risks associated with scrapie. Cooper and Bryce Reece, executive vice president of the Wyoming Wool Growers Association, say consumers and livestock owners have nothing to fear from the diagnosis.

"From an industry standpoint, we're not at all concerned or alarmed by it," Reece said. "It's more of an interest to the researchers or the scientists than it is to the industry. I'm sure they're all questioning themselves as to how it got here."

Dr. Mark Hall, head of the special pathology section of the National Veterinary Services Laboratories in Ames, Iowa, agreed.

"It is something of interest and something I think we want to continue to look at," Hall said. "But this is certainly not a shocking revelation or anything like that. At this point, I don't think there's any evidence that there's any great need for concern."

Scrapie experts and Animal Plant Health Inspection Service officials say it's discovery is actually something to brag about.

"It means our surveillance system is working," Dr. Diane Sutton, National Scrapie Program coordinator, said. "We found it utilizing our current technologies to find scrapie cases in the United States."

APHIS notified the state last month that the sheep rancher's ewe tested positive for a form of scrapie consistent with the Nor98 strain. The ewe was slaughtered in Michigan last fall as part of the USDA's regular scrapie slaughter surveillance program and traced back to the Wyoming flock.

Dr. Walt Cook, acting state veterinarian, said the government would pay the livestock owner an indemnity fee based on fair market value for the sheep, before "depopulating" the herd.

"It's the best and the simplest thing to do," Cook said. "It's unfortunate to put down all those sheep, but it will allow us to test them and make sure no other animals are infected."

Cook and Sutton said the infected ewe's owner had one other option: to quarantine his flock, during which time he couldn't sell breeding animals for several years while the flock was monitored to ensure no other cases of the disease appeared.

"Economically it doesn't make much difference one way or the other," Cook said of the rancher's options. "Obviously, by depopulating, he has to go through the loss of his animals, which can be distressful."

Reece said when traditional scrapie is found in a flock, typically up to 10 percent of the rest of that herd can be infected with the disease. But with Nor98, when one case is found, to date it has been the only one found in the flock.

"I would bet we're not gonna see another one of these in Wyoming, and maybe not even in the United States, but who knows?" Reece said. "With 300 cases worldwide, it's not something out there that's highly transmittable."

He said that if an animal becomes infected with scrapie, it will die from it: "It's 100 percent fatal, and because it's 100 percent fatal it's a self-limiting disease."

"From a practical standpoint, scrapie is nothing," Reece said. "Pneumonia probably kills more sheep than anything else out there, but because of scrapie's similarities to mad cow disease, from a marketing and consumer standpoint it is important for the U.S. to eradicate the disease from the U.S."

Cooper said the effects of Nor98 are basically the same as those seen in classic scrapie _ the disease attacks the central nervous system of infected animals, causing behavioral changes such as tremors of the head and neck.

Classical scrapie is believed to be primarily transmitted through exposure to placenta and birth fluids from infected animals or from facilities in which infected animals have lambed.

The lamb and wool production business is a $50 million-a-year industry in Wyoming, according to Reece, and a $500 million-a-year industry nationally. According to Cooper, many sheep-importing countries require that the U.S. be free of scrapie for seven years before they'll import U.S. breeding sheep.

Wyoming Livestock Board Scrapie Prevention and Management Rules CHAPTER 13

Sunday, November 13, 2011

Atypical Scrapie Isolates Involve a Uniform Prion Species with a Complex Molecular Signature

PrPres peptides of low molecular mass have also been described in other types of prion disease, such as Gerstmann-Sträussler-Scheinker disease [32], [33] and Creutzfeldt-Jakob disease [34], [35] in humans as well as in H-BSE in cattle [36], [37]. Forthcoming directions of research are likely to focus on more precise comparative analyses of truncated PrPres peptides and their role in the biology of human and animal prion diseases...

Thursday, July 14, 2011

Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4)

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

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.

M.A. Tranulis (*)

Norwegian School of Veterinary Science, Oslo, Norway


S.L. Benestad

Norwegian Veterinary Institute, Oslo, Norway

T. Baron

Agence Nationale de Se´curite´ Sanitaire, ANSES, Lyon, France

H. Kretzschmar

Ludwig–Maximilians University of Munich, Munich, Germany

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

see full text and more here ;

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

Thursday, June 2, 2011

USDA scrapie report for April 2011 NEW ATYPICAL NOR-98 SCRAPIE CASES Pennsylvania AND California

Monday, June 20, 2011 2011

Annual Conference of the National Institute for Animal Agriculture ATYPICAL NOR-98 LIKE SCRAPIE UPDATE USA

Wednesday, October 12, 2011

White-tailed deer are susceptible to the agent of sheep scrapie by intracerebral inoculation

Saturday, June 25, 2011

Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque

"BSE-L in North America may have existed for decades"

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.


The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...

This is an interesting editorial about the Mad Cow Disease debacle, and it's ramifications that will continue to play out for decades to come ;

Monday, October 10, 2011

EFSA Journal 2011 The European Response to BSE: A Success Story


EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.


see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;

Thursday, August 4, 2011

Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis, Date aired: 27 Jun 2011 (SEE VIDEO)

Sunday, August 21, 2011

The British disease, or a disease gone global, The TSE Prion Disease (SEE VIDEO)

Saturday, March 5, 2011


9th Annual CJD Foundation Family Conference July 10, 2011

The Centers for Disease Control and Prevention Report: Prion Disease Activities at CDC

Ryan A. Maddox, MPH Epidemiologist CJD 2011 and the 9th Annual CJD Foundation Family Conference July 10, 2011

Surveillance mechanisms - Periodic review of national cause-of-death data

Ongoing review of clinical and pathologic records of CJD decedents aged < 55 years

Death certificate data review is effective as a surveillance tool for CJD:


Hunter study Goal: To determine whether chronic wasting disease (CWD), a prion disease of deer and elk, can cause disease in humans ćStudy: Follow-up of persons who hunted in Colorado and Wyoming, where CWD is found, and identifying those who died of prion disease

Results: Prion disease cases among this group within expected range so far


The lower incidence of CJD among Hispanics in the US may be at least partly due to: Underreporting of Hispanic ethnicity on death certificates relative to surveys and censuses.


Conclusion Collaboration with medical and public health personnel, NPDPSC, the CJD Foundation, and CDC is essential.

"Conclusion Collaboration with medical and public health personnel, NPDPSC, the CJD Foundation, and CDC is essential."

yada, yada, yada...same old song and dance $$$

YEP, it's ESSENTIAL for one thing, and one thing only, keeping all human TSE prion disease in the USA 'SPORADIC' CJD or 'SPORADIC' HUMAN TSE OF A NEW PHENOTYPE $$$

Isn't it amazing that in the above report from Ryan A. Maddox, MPH Epidemiologist CJD 2011 and the 9th Annual CJD Foundation Family Conference July 10, 2011, none of the information about what the rest of the world is worried about, this new threat from the atypical TSE's. USA Typical and Atypical BSE, USA Typical and Atypical Scrapie, and USA Typical and Atypical Chronic Wasting Disease as having zoonotic potential. The USDA, FDA, CDC et al are still relying on science that is almost 30 years old i.e. the UKBSEnvCJD only theory.

stupid is, as stupid does, and some times you just can't fix stupid $$$


DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)

The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a is a big fat sponge...the agent continues to eat the brain can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....

Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any is ALL gonna be sporadic!!!

And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...

Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"

again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.

You need to watch your back........but keep picking at a buzzard to the just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)


USDA ET AL said it long long, ago, and they meant it $

In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells

3. Prof. A Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. BSE was not reported in the USA.


Monday, September 26, 2011

L-BSE BASE prion and atypical sporadic CJD

Monday, June 27, 2011

Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates

Tuesday, November 08, 2011

Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008 Vol. 37, No. 3-4, 2011

Original Paper

***Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.

ALSO, Dr. Maddox states;

"make routine mortality surveillance a useful surrogate for ongoing CJD surveillance"

THIS has proven not very useful in the U.K.;



One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...


Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R. Will



Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...

full text;

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.


DR. Maddox states here;

In collaboration with appropriate local and state health departments and the National Prion Disease Pathology Surveillance Center, CDC is facilitating or conducting such surveillance and case- investigations, including related laboratory studies to characterize CJD and CWD prions.

HOWEVER in a recent article in the UPI out of Washington;

CJD screening may miss thousands of cases

By Steve Mitchell UPI Medical Correspondent Published 7/21/2003 3:00 PM


In addition, the NPDPSC sees less than half of all the CJD cases each year, so the CDC's investigational system not only is missing many of the misdiagnosed CJD cases, it also is not conducting autopsies on most of the detected cases.




[ProMED-mail thanks Terry S Singeltary Sr for drawing attention to this comprehensive document which provides a current evaluation of experimental work designed to explore the zoonotic potential of the various recently recognised TSEs of domestic and other animals. It is concluded that at present the only TSE agent demonstrated to be zoonotic is the classical BSE agent. Nor can it be entirely excluded at the present time that a small proportion of cases of sporadic CJD may be environmentally acquired. - Mod.CP]


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.



case; 5 Includes 13 cases in which the diagnosis is pending, and 18 inconclusive cases; 6 Includes 18 (15 from 2011) cases with type determination pending in which the diagnosis of vCJD has been excluded.

Saturday, March 5, 2011


Sunday, August 21, 2011

The British disease, or a disease gone global, The TSE Prion Disease

Thursday, August 4, 2011

Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis, Date aired: 27 Jun 2011

see video here ;


Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***

Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.

my comments to PLosone here ;

Sunday, August 09, 2009

CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009

Tuesday, August 18, 2009

BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009


BY Philip Yam

Yam Philip Yam News Editor Scientific American

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


Laying Odds

Are prion diseases more prevalent than we thought?

Researchers and government officials badly underestimated the threat that mad cow disease posed when it first appeared in Britain. They didn't think bovine spongiform encephalopathy was a zoonosis-an animal disease that can sicken people. The 1996 news that BSE could infect humans with a new form of Creutzfeldt-Jakob disease stunned the world. It also got some biomedical researchers wondering whether sporadic CJD may really be a manifestation of a zoonotic sickness. Might it be caused by the ingestion of prions, as variant CJD is?

Revisiting Sporadic CJD

It's not hard to get Terry Singeltary going. "I have my conspiracy theories," admitted the 49-year-old Texan.1 Singeltary is probably the nation's most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union's Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous www.madcow. org Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people's health.

Singeltary has similar inclinations. ...



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

June 2003

BY Philip Yam


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


The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003 doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI

Tracking spongiform encephalopathies in North America

Xavier Bosch

"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem." 49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)-the relative of mad cow disease seen among deer and elk in the USA. Although his feverish.

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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

Terry S. Singeltary, Sr Bacliff, Tex

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

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

26 March 2003

Terry S. Singeltary, retired (medically) CJD WATCH

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

2 January 2000

British Medical Journal

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

15 November 1999

British Medical Journal

vCJD in the USA * BSE in U.S.

14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

T. Singeltary

Bacliff, TX, USA


An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.


12 years independent research of available data


I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.


I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

Wednesday, August 24, 2011

There Is No Safe Dose of Prions

Wednesday, August 24, 2011

All Clinically-Relevant Blood Components Transmit Prion Disease following a Single Blood Transfusion: A Sheep Model of vCJD

Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING BY STATE 2011

(or the lack of reporting, due to flawed CJD Surveillance system of only periodically reviewing death certificates. ...tss)


Wednesday, November 09, 2011

Case report Sporadic fatal insomnia in a young woman: A diagnostic challenge: Case Report TEXAS


Case report Sporadic fatal insomnia in a young woman: A diagnostic challenge: Case Report

Karen M Moody , Lawrence B Schonberger , Ryan A Maddox , Wen-Quan Zou , Laura Cracco and Ignazio Cali

BMC Neurology 2011, 11:136doi:10.1186/1471-2377-11-136


31 October 2011


"Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle."



Wednesday, June 15, 2011

Galveston, Texas - Isle port moves through thousands of heifers headed to Russia, none from Texas, Alabama, or Washington, due to BSE risk factor

Creutzfeldt-Jakob Disease Surveillance in Texas

Sunday, July 11, 2010

CJD or prion disease 2 CASES McLennan County Texas population 230,213 both cases in their 40s

see the continuing rise of sporadic CJD in Texas here ;


Sunday, November 13, 2011

California BSE mad cow beef recall, QFC, CJD, and dead stock downer livestock


Sunday, November 13, 2011




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

No comments:

Post a Comment