Sunday, July 21, 2013

As Chronic Wasting Disease CWD rises in deer herd, what about risk for humans?

Patricia Randolph's Madravenspeak: As CWD rises in deer herd, what about risk for humans?


"The ever-increasing number of CWD-infected deer on the landscape ... and the accompanying exponential increase of environmental contamination with CWD prion will result in increased interspecies, including human, exposure.” — Dr. David Clausen, veterinarian, recently retired Natural Resources Board chairman


Scientists around the world are concerned that prion diseases may be zoonotic — communicated from animal to human. According to Wikipedia, “Many modern diseases, even epidemic diseases, started out as zoonotic diseases.” It is suspected that AIDS was transmitted to humans from African primates eaten as meat.


The leaders at the Wisconsin Department of Natural Resources simply follow the ideological agenda of its hunter clientele. Since killing deer is the primary attraction for hunters, the department does not want to threaten the deer hunt. But they are doing just that. Testing for chronic wasting disease has been drastically reduced as CWD rates in our state rise. In 2002, just after CWD was recognized here, 40,000 deer were tested and the rate of infection was 0.51 precent. In 2012, 5.13 percent of 6,611 deer tested showed infection — a 10-fold increase in 10 years.


According to a PR Watch article in June, “about one out of every three male deer aged 2.5 years and older carries CWD in north-central Iowa County, as does one out of every six yearling male deer (1.5 years old)” and the rate is increasing 10 percent each year. Bucks are twice as likely to be infected but the disease is expanding faster in does.


Experts told outdoor writer Pat Durkin this rate of increase is "unprecedented," "frightening" and "disturbing." According to Durkin, “That resembles annual infection rates of mule deer in southeastern Wyoming’s Converse area, where 40 to 50 percent of the herd is infected.”


I was just ending my three-year term as an elected Dane County delegate to the so-called Conservation Congress when the DNR recognized CWD in our deer herd. In 2002, I attended the “expert” panel on CWD presented in Mount Horeb by the DNR. The auditorium was filled with frightened deer hunters, fearing the loss of their main hunt.


There is no cure for prion diseases. Death is imminent after disorientation and body wasting. Learn about a Utah hunter whose death may be attributed to CWD infection.


Diseased elk confined on a farm in Colorado were all killed. The land was poisoned, burned, and left fallow for three years. Then a fresh herd of ungulates was put on the land. They reinfected. Prions live in soil and infect healthy animals. 


So what increases the chances of deer-to-human transmission of CWD? One prime factor is high animal density. Since the DNR under cheerleader Secretary Cathy Stepp favors “growing the herd,” and deer farmers aiming for 175,000 more deer each year, that is a given.


It has been discovered that Wisconsin has a different strain of CWD than the Western states. Transmission of prions to transgenic mice expressing human genes suggests that the species barrier from deer to human is prion-strain dependent and that humans can be more vulnerable to novel prion strains.


Hunters are delivering deer to food pantries for the poor, and much of that is deer sausage. One infected animal could potentially spread disease to many people. Processing plant equipment could be contaminated. 


Back in 2002, Tryg Solberg, chair of the Natural Resources Board said, “There is a lot more risk in game farms than baiting and feeding. … Maybe we should get rid of them. We should do something." Then acting state veterinarian Dr. Bob Ehlenfeldt supported a total ban on baiting and feeding to limit possible spread of brucellosis and bovine tuberculosis as well as CWD.


Ironically, a Journal of Wildlife Diseases article in 2011 said the wolf could provide protection from CWD: “We suggest that as CWD distribution and wolf range overlap in the future, wolf predation may suppress disease emergence or limit prevalence.” However, dogs can be infected with prions — and that makes wolves susceptible.


The Center for Media and Democracy founder John Stauber says, "Every dead deer in the state should be tested, and no deer should be butchered, processed or eaten unless it has been tested free of the disease.”


Human health is dependent on healthy ecosystems. Killing wildlife is potentially deadly to all of us now.


Patricia Randolph of Portage is a longtime activist for wildlife. or






nice article Patricia et al. for everyone’s records, some science to show said risk factors for Chronic Wasting Disease CWD transmission to humans and other species as follows. ...



kind regards,



The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.




The chances of a person or domestic animal contracting CWD are “extremely remote,” Richards said. The possibility can’t be ruled out, however. “One could look at it like a game of chance,” he explained. “The odds (of infection) increase over time because of repeated exposure. That’s one of the downsides of having CWD in free-ranging herds: We’ve got this infectious agent out there that we can never say never to in terms of (infecting) people and domestic livestock.”











Chad Johnson1, Judd Aiken2,3,4 and Debbie McKenzie4,5 1 Department of Comparative Biosciences, University of Wisconsin, Madison WI, USA 53706 2 Department of Agriculture, Food and Nutritional Sciences, 3 Alberta Veterinary Research Institute, 4.Center for Prions and Protein Folding Diseases, 5 Department of Biological Sciences, University of Alberta, Edmonton AB, Canada T6G 2P5


The identification and characterization of prion strains is increasingly important for the diagnosis and biological definition of these infectious pathogens. Although well-established in scrapie and, more recently, in BSE, comparatively little is known about the possibility of prion strains in chronic wasting disease (CWD), a disease affecting free ranging and captive cervids, primarily in North America. We have identified prion protein variants in the white-tailed deer population and demonstrated that Prnp genotype affects the susceptibility/disease progression of white-tailed deer to CWD agent. The existence of cervid prion protein variants raises the likelihood of distinct CWD strains. Small rodent models are a useful means of identifying prion strains. We intracerebrally inoculated hamsters with brain homogenates and phosphotungstate concentrated preparations from CWD positive hunter-harvested (Wisconsin CWD endemic area) and experimentally infected deer of known Prnp genotypes. These transmission studies resulted in clinical presentation in primary passage of concentrated CWD prions. Subclinical infection was established with the other primary passages based on the detection of PrPCWD in the brains of hamsters and the successful disease transmission upon second passage. Second and third passage data, when compared to transmission studies using different CWD inocula (Raymond et al., 2007) indicate that the CWD agent present in the Wisconsin white-tailed deer population is different than the strain(s) present in elk, mule-deer and white-tailed deer from the western United States endemic region.







Prion Transmission from Cervids to Humans is Strain-dependent


Qingzhong Kong, Shenghai Huang,*Fusong Chen, Michael Payne, Pierluigi Gambetti and Liuting Qing Department of Pathology; Case western Reserve University; Cleveland, OH USA *Current address: Nursing Informatics; Memorial Sloan-Kettering Cancer Center; New York, NY USA


Key words: CWD, strain, human transmission


Chronic wasting disease (CWD) is a widespread prion disease in cervids (deer and elk) in North America where significant human exposure to CWD is likely and zoonotic transmission of CWD is a concern. Current evidence indicates a strong barrier for transmission of the classical CWD strain to humans with the PrP-129MM genotype. A few recent reports suggest the presence of two or more CWD strains. What remain unknown is whether individuals with the PrP-129VV/MV genotypes are also resistant to the classical CWD strain and whether humans are resistant to all natural or adapted cervid prion strains. Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP, indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains. Preliminary results on CWD transmission in transgenic mice expressing human PrP-129V will also be discussed.


Acknowledgement Supported by NINDS NS052319 and NIA AG14359.






Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions


Marcelo A. Barria,1 Glenn C. Telling,2 Pierluigi Gambetti,3 James A. Mastrianni4 and Claudio Soto1 1Mitchell Center for Alzheimer's disease and related Brain disorders; Dept of Neurology; University of Texas Houston Medical School; Houston, TX USA; 2Dept of Microbiology, Immunology & Molecular Genetics and Neurology; Sanders Brown Center on Aging; University of Kentucky Medical Center; Lexington, KY USA; 3Institute of Pathology; Case western Reserve University; Cleveland, OH USA; 4Dept of Neurology; University of Chicago; Chicago, IL USA


Prion diseases are infectious neurodegenerative disorders affecting humans and animals that result from the conversion of normal prion protein (PrPC) into the misfolded and infectious prion (PrPSc). Chronic wasting disease (CWD) of cervids is a prion disorder of increasing prevalence within the United States that affects a large population of wild and captive deer and elk. CWD is highly contagious and its origin, mechanism of transmission and exact prevalence are currently unclear. The risk of transmission of CWD to humans is unknown. Defining that risk is of utmost importance, considering that people have been infected by animal prions, resulting in new fatal diseases. To study the possibility that human PrPC can be converted into the infectious form by CWD PrPSc we performed experiments using the Protein Misfolding Cyclic Amplification (PMCA) technique, which mimic in vitro the process of prion replication. Our results show that cervid PrPSc can induce the pathological conversion of human PrPC, but only after the CWD prion strain has been stabilized by successive passages in vitro or in vivo. Interestingly, this newly generated human PrPSc exhibits a distinct biochemical pattern that differs from any of the currently known forms of human PrPSc, indicating that it corresponds to a novel human prion strain. Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids.






Biochemical and Biophysical Characterization of Different CWD Isolates


Martin L. Daus and Michael Beekes Robert Koch Institute; Berlin, Germany


Key words: CWD, strains, FT-IR, AFM


Chronic wasting disease (CWD) is one of three naturally occurring forms of prion disease. The other two are Creutzfeldt-Jakob disease in humans and scrapie in sheep. CWD is contagious and affects captive as well as free ranging cervids. As long as there is no definite answer of whether CWD can breach the species barrier to humans precautionary measures especially for the protection of consumers need to be considered. In principle, different strains of CWD may be associated with different risks of transmission to humans. Sophisticated strain differentiation as accomplished for other prion diseases has not yet been established for CWD. However, several different findings indicate that there exists more than one strain of CWD agent in cervids. We have analysed a set of CWD isolates from white-tailed deer and could detect at least two biochemically different forms of disease-associated prion protein PrPTSE. Limited proteolysis with different concentrations of proteinase K and/or after exposure of PrPTSE to different pH-values or concentrations of Guanidinium hydrochloride resulted in distinct isolate-specific digestion patterns. Our CWD isolates were also examined in protein misfolding cyclic amplification studies. This showed different conversion activities for those isolates that had displayed significantly different sensitivities to limited proteolysis by PK in the biochemical experiments described above. We further applied Fourier transform infrared spectroscopy in combination with atomic force microscopy. This confirmed structural differences in the PrPTSE of at least two disinct CWD isolates. The data presented here substantiate and expand previous reports on the existence of different CWD strains.









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 cattleadapted 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.






Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease


Martin L. Daus,1,† Johanna Breyer,2 Katjs Wagenfuehr,1 Wiebke Wemheuer,2 Achim Thomzig,1 Walter Schulz-Schaeffer2 and Michael Beekes1 1Robert Koch Institut; P24 TSE; Berlin, Germany; 2Department of Neuropathology, Prion and Dementia Research Unit, University Medical Center Göttingen; Göttingen, Germany †Presenting author; Email:


Chronic wasting disease (CWD) is a contagious, rapidly spreading transmissible spongiform encephalopathy (TSE) occurring in cervids in North America. Despite efficient horizontal transmission of CWD among cervids natural transmission of the disease to other species has not yet been observed. Here, we report a direct biochemical demonstration of pathological prion protein PrPTSE and of PrPTSE-associated seeding activity in skeletal muscles of CWD-infected cervids. The presence of PrPTSE was detected by Western- and postfixed frozen tissue blotting, while the seeding activity of PrPTSE was revealed by protein misfolding cyclic amplification (PMCA). The concentration of PrPTSE in skeletal muscles of CWD-infected WTD was estimated to be approximately 2000- to 10000-fold lower than in brain tissue. Tissue-blot-analyses revealed that PrPTSE was located in muscle- associated nerve fascicles but not, in detectable amounts, in myocytes. The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.





Reasons for Caution There are several reasons for caution with respect to zoonotic and interspecies CWD transmission. First, there is strong evidence that distinct CWD strains exist (36). Prion strains are distinguished by varied incubation periods, clinical symptoms, PrPSc conformations, and CNS PrPSc depositions (3,32). Strains have been identified in other natural prion diseases, including scrapie, BSE, and CJD (3). Intraspecies and interspecies transmission of prions from CWD-positive deer and elk isolates resulted in identification of >2 strains of CWD in rodent models (36), indicating that CWD strains likely exist in cervids. However, nothing is currently known about natural distribution and prevalence of CWD strains. Currently, host range and pathogenicity vary with prion strain (28,37). Therefore, zoonotic potential of CWD may also vary with CWD strain. In addition, diversity in host (cervid) and target (e.g., human) genotypes further complicates definitive findings of zoonotic and interspecies transmission potentials of CWD.


Intraspecies and interspecies passage of the CWD agent may also increase the risk for zoonotic CWD transmission. The CWD prion agent is undergoing serial passage naturally as the disease continues to emerge. In vitro and in vivo intraspecies transmission of the CWD agent yields PrPSc with an increased capacity to convert human PrPc to PrPSc (30). Interspecies prion transmission can alter CWD host range (38) and yield multiple novel prion strains (3,28). The potential for interspecies CWD transmission (by cohabitating mammals) will only increase as the disease spreads and CWD prions continue to be shed into the environment. This environmental passage itself may alter CWD prions or exert selective pressures on CWD strain mixtures by interactions with soil, which are known to vary with prion strain (25), or exposure to environmental or gut degradation.


Given that prion disease in humans can be difficult to diagnose and the asymptomatic incubation period can last decades, continued research, epidemiologic surveillance, and caution in handling risky material remain prudent as CWD continues to spread and the opportunity for interspecies transmission increases. Otherwise, similar to what occurred in the United Kingdom after detection of variant CJD and its subsequent link to BSE, years of prevention could be lost if zoonotic transmission of CWD is subsequently identified, CWD will likely continue to emerge in North America. ...












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


Our results have far-reaching implications for human health, since they indicate that cervid PrPSc can trigger the conversion of human PrPC into PrPSc, suggesting that CWD might be infectious to humans. Interestingly our findings suggest that unstable strains from CWD affected animals might not be a problem for humans, but upon strain stabilization by successive passages in the wild, this disease might become progressively more transmissible to man.




Our results also have profound implications for understanding the mechanisms of the prion species barrier and indicate that the transmission barrier is a dynamic process that depends on the strain and moreover the degree of adaptation of the strain. If our findings are corroborated by infectivity assays, they will imply that CWD prions have the potential to infect humans and that this ability progressively increases with CWD spreading.






Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain



Date: August 25, 2007 at 12:42 pm PST


our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions.








Wednesday, September 08, 2010











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.






now, let’s see what the authors said about this casual link, personal communications years ago. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ????




“Our conclusion stating that we found no strong evidence of CWD transmission to humans”




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


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 ;








CWD ongoing experiment on humans, long term $$$

Monday, November 14, 2011

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


Wednesday, November 16, 2011

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


Observation| March 2007

Colorado Surveillance Program for Chronic Wasting Disease Transmission to HumansLessons From 2 Highly Suspicious but Negative CasesFREE

C. Alan Anderson, MD; Patrick Bosque, MD; Christopher M. Filley, MD; David B. Arciniegas, MD; B. K. Kleinschmidt-DeMasters, MD; W. John Pape, BS; Kenneth L. Tyler, MD

[+] Author Affiliations

Arch Neurol. 2007;64(3):439-441. doi:10.1001/archneur.64.3.439.

Objective To describe 2 patients with rapidly progressive dementia and risk factors for exposure to chronic wasting disease (CWD) in whom extensive testing negated the possible transmission of CWD.

Design/Methods We describe the evaluation of 2 young adults with initial exposure histories and clinical presentations that suggested the possibility of CWD transmission to humans.

Patients A 52-year-old woman with possible laboratory exposure to CWD and a 25-year-old man who had consumed meat from a CWD endemic area.

Interventions Clinical evaluation, neuropathological examination, and genetic testing.

Results Neuropathological and genetic assessment in the 2 patients proved the diagnoses of early-onset Alzheimer disease and a rare genetic prion disease.

Conclusion No convincing cases of CWD transmission to humans have been detected in our surveillance program.


CASE 1 .

A 52-year-old right-handed woman presented with a 1-year history of progressive memory loss, language impairment, visuospatial disturbance, and myoclonus. She related that she had been a histology technician in a laboratory that processed tissue specimens from deer and elk with CWD and had handled specimens without wearing gloves. Both she and her family expressed significant concerns about the possibility of transdermal transmission of CWD. Her family history was negative for dementia and other neurologic disorders. Brain magnetic resonance imaging showed mild diffuse volume loss, and electroencephalography demonstrated mild diffuse slowing. Other laboratory studies were unremarkable. Cerebrospinal fluid findings were unremarkable except for a weakly immunostaining 14-3-3 protein band, an indeterminate finding for the diagnosis of prion disease. Genetic testing of the prion protein gene was normal, revealing methionine homozygosity at codon 129. Brain biopsy results were negative for the presence of protease-resistant prion protein but showed definite Alzheimer disease with numerous neuritic plaques and tau-positive neurofibrillary tangles (Figure). Further analysis of brain tissue at the National Prion Disease Pathology Surveillance Center was negative for prion disease by Western blot analysis. Subsequent investigation by the state department of health revealed the patient had worked in an area of the laboratory that conducted necropsies on domestic animals and had never been assigned to the CWD testing laboratory. The Colorado Department of Public Health and Environment could not confirm that the technician had ever worked with deer and elk tissues.

CASE 2 .

This 25-year-old right-handed man had a 4-month history of progressive gait disturbance, myoclonus, hallucinations, slowed cognition, impaired attention, and memory loss. He had hunted deer and elk in a CWD endemic area of southern Wyoming and cooked and ate the field-dressed meat. His family history was significant in that his mother had died of a dementing disease at age 40 years, although there was neither a clinical diagnosis nor an autopsy. Brain magnetic resonance imaging findings were unremarkable, and electroencephalography demonstrated 1-Hz high-amplitude periodic sharp wave complexes. Other laboratory studies had negative results. Testing for the 14-3-3 protein had positive results, but the cerebrospinal fluid was otherwise unremarkable. The diagnosis of Gerstmann-Sträussler-Scheinker syndrome, a familial prion disease, was confirmed with a detailed autopsy examination and referral of the brain to the National Prion Disease Pathology Surveillance Center. Autopsy brain tissue showed the presence of protease-resistant prion protein by Western blot analysis. Genetic evaluation revealed the P102L mutation in the prion protein gene with methionine/valine heterozygosity at codon 129.


Saturday, August 01, 2009

Cases of Early-Onset Sporadic Creutzfeld-Jakob Disease in Michigan Chronic Wasting Disease and Potential Transmission to Humans

Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,† Michael W. Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger*


In 2001, two additional CJD patients 26 and 28 years of age were reported from a single state (Table 2) (34). The patients grew up in adjacent counties and had illness onset within several months of each other. As a result of this fact and their unusually young age, a possible environmental source of infection, including exposure to CWD-infected venison, was considered. One of the patients died after an illness lasting 5–6 months that was characterized by progressive aphasia, memory loss, social withdrawal, vision disturbances, and seizure activity leading to status epilepticus and induced coma. Histopathologic, immunohistochemical, and Western blot testing of brain biopsy and autopsy samples confirmed a CJD diagnosis. The patient’s disease phenotype corresponded to the MM2 sporadic CJD subtype reported by Parchi et al. (35). This patient did not hunt, and family members provided no history of regularly eating venison. The patient may have occasionally eaten venison originating from the Upper Peninsula of Michigan while away from home during his college years. However, ongoing surveillance has not detected CWD in Michigan deer (36).

The second patient died from an illness lasting ˜16 months. The patient’s illness began with behavioral changes, including unusual outbursts of anger and depression. Confusion, memory loss, gait disturbances, incontinence, headaches, and photophobia also developed. Western blot analysis of frozen brain biopsy tissue confirmed a prion disease diagnosis. Immunohistochemical analysis of brain tissue obtained after the patient’s death

showed prion deposition consistent with GSS. Aprion protein gene analysis could not be performed because appropriate samples were lacking. However, prion protein gene analysis of a blood sample from one of the patient’s parents indicated a GSS P102L mutation. The patient did not hunt but may have eaten venison from Michigan once when he was 1–2 years old. The GSS diagnosis greatly reduced the likelihood that the two patients reported from adjacent counties had disease with a common origin. Recently, rare neurologic disorders resulting in the deaths of three men who participated in “wild game feasts” in a cabin owned by one of the decedents created concern about the possible relationship of their illnesses with CWD (Table 2) (37). Two of the patients reportedly died of CJD, and the third died from Pick’s disease. More than 50 persons were identified as possibly participating in these feasts; the three patients were the only participants reported to have died of a degenerative neurologic disorder. Reanalysis of autopsy brain tissues from the three patients at the National Prion Disease Pathology Surveillance Center indicated that two of them had no evidence of a prion disease by immunohistochemical analysis. CJD was confirmed in the third patient, who had clinicopathologic, codon 129, and prion characteristics similar to the most common sporadic CJD subtype (MM1/MV1) (35). This patient participated in the feasts only once, perhaps in the mid-1980s. In addition, the investigation found no evidence that the deer and elk meat served during the feasts originated from the known CWD-endemic areas of Colorado and Wyoming.

In 2003, CJD in two deer and elk hunters (54 and 66 years of age) was reported (38). The report implied that the patients had striking neuropathologic similarities and that their illness may represent a new entity in the spectrum of prion diseases. A third patient (63 years of age), who was also purported to have been a big game hunter, was subsequently reported from the same area. However, none of the three patients were reported to have eaten venison from the CWD-endemic areas of the western United States. The 66- year-old patient hunted most of his life in Washington State. Although information about the 54-year-old patient was limited, there was no evidence that he hunted in CWD-endemic areas. The third patient was not a hunter but ate venison harvested from Pennsylvania and Washington. The neuropathologic changes, Western blot profile, and genotype at codon 129 of the three patients each fit the MM1, VV1, or VV2 sporadic CJD subtype, indicating absence of phenotypic similarity among the cases or atypical neuropathologic features (35). To date, only two nonfamilial CJD cases with a positive history of exposure to venison obtained from the known CWD-endemic areas have been reported. One of the patients was a 61-year-old woman who grew up in an area where this disease is known to be endemic, and she ate venison harvested locally. She died in 2000, and analysis of autopsy brain specimens confirmed that the patient’s CJD phenotype fit the MM1 subtype, with no atypical neuropathologic features. The second patient was a 66-yearold man who was reported to have eaten venison from two deer harvested in a CWD-endemic area. Both deer tested negative for CWD, and the patient’s illness was consistent with the MM1 CJD phenotype.


September 29, 2004

Grand Forks man dies from rare disease

State health officials say lab results are not expected for several weeks on a Grand Forks man who is believed to have died from Creutzfeldt-jakob (croitz-feld juh-COHB') disease, or C-J-D.

It's a rare neurological disease with symptoms similar to mad cow disease.

State Epidemiologist Larry Shireley says North Dakota has seen only a couple of cases of CJD in recent years. Scientists say about 250 people are diagnosed with it in the United States each year.

Shireley says a Cleveland lab that specializes in CJD wants to test tissue samples to make sure 48-old Michael Jose did not have new variant CJD, also known as mad cow disease. Both diseases have some similar symptoms, but no human mad cow cases have been reported in this country.

The testing will be done at the National Prion Disease Pathology Surveillance Center at Case Western Reserve University.

Family members said Jose was never hospitalized and lived at home until the time of his death last Saturday.

Doctor James Hargreaves is an infectious disease specialist at Altru Hospital in Grand Forks. He says it's difficult to know how long Jose had the disease.


February 21, 2003 / 52(07);125-127

Fatal Degenerative Neurologic Illnesses in Men Who Participated in Wild Game Feasts --- Wisconsin, 2002

Creutzfeldt-Jakob disease (CJD) is a fatal neurologic disorder in humans. CJD is one of a group of conditions known as transmissible spongiform encephalopathies (TSEs), or prion diseases, that are believed to be caused by abnormally configured, host-encoded prion proteins that accumulate in the central nervous tissue (1). CJD has an annual incidence of approximately 1 case per million population in the United States (1) and occurs in three forms: sporadic, genetically determined, and acquired by infection. In the latter form, the incubation period is measured typically in years. Recent evidence that prion infection can cross the species barrier between humans and cattle has raised increasing public health concerns about the possible transmission to humans of a TSE among deer and elk known as chronic wasting disease (CWD) (2). During 1993--1999, three men who participated in wild game feasts in northern Wisconsin died of degenerative neurologic illnesses. This report documents the investigation of these deaths, which was initiated in August 2002 and which confirmed the death of only one person from CJD. Although no association between CWD and CJD was found, continued surveillance of both diseases remains important to assess the possible risk for CWD transmission to humans.

Case Reports

Case 1. In December 1992, a Wisconsin man aged 66 years with a history of seizures since 1969 sought treatment for recurring seizures, increasing forgetfulness, and worsening hand tremors. Electroencephalographic (EEG) examination demonstrated focal epileptiform activity and nonspecific diffuse abnormalities, but no specific diagnosis was made. In February 1993, he was hospitalized for increasing confusion, ataxia, and movement tremors of his extremities. A magnetic resonance image (MRI) demonstrated mild, nonspecific enhancement along the inferior parasagittal occipital lobe. A repeat EEG showed bifrontal intermittent, short-interval, periodic sharp waves, suggesting a progressive encephalopathy; a diagnosis of CJD was suspected. The man died later that month; neuropathologic examination of brain tissue during autopsy indicated subacute spongiform encephalopathy, compatible with CJD.

The man was a lifelong hunter who ate venison frequently. He hunted primarily in northern Wisconsin but also at least once in Montana. He hosted wild game feasts at his cabin in northern Wisconsin from 1976 until shortly before his death. Fixed brain tissue obtained during the autopsy was sent for analysis to the National Prion Disease Pathology Surveillance Center (NPDPSC) and reexamined at the institution where the autopsy was conducted. Histopathologic examination did not substantiate the diagnosis of prion disease. In addition, 27 brain tissue sections were negative for prions by immunostaining despite positive antibody reactions against other proteins (controls), which indicated that other epitopes in the tissue samples were preserved.

Case 2. In May 1999, a Minnesota man aged 55 years with no previous history of a neurologic disease sought evaluation and treatment following a 3-month history of progressive difficulty in writing and unsteadiness of gait. A computerized tomography (CT) scan and MRI examination of his head did not indicate any abnormality. In June 1999, he was hospitalized following onset of dementia, speech abnormalities, and myoclonic jerking. An EEG indicated left-hemispheric periodic sharp waves and moderate generalized background slowing; CJD was diagnosed clinically. In July 1999, following worsening symptoms and development of right upper extremity dystonia, the patient died. Neuropathologic evaluation of brain tissue during autopsy demonstrated widespread subcortical spongiform lesions, consistent with CJD.

The man was not a hunter but had a history of eating venison. He made an estimated 12 visits to the cabin where the wild game feasts were held, but he participated in only one feast during the mid-1980s. Sections of fixed and frozen brain tissue obtained during autopsy were analyzed at NPDPSC, and prion disease was confirmed by immunohistochemical and Western blot testing. The Western blot characteristics and prion disease phenotype in this patient were consistent with the most common form of sporadic CJD, classified as M/M (M/V) 1 (3). Subsequent genetic typing confirmed the presence of methionine homozygosity (M/M) at codon 129 of the patient's prion protein gene.

Case 3. In June 1992, a Wisconsin man aged 65 years sought treatment for progressive slowing of speech, worsening memory, and personality changes. By January 1993, his speech was reduced to one-word utterances. Neurologic examination showed a flat affect, decreased reflexes, and apraxia. A CT head scan showed mild atrophy, and an EEG was normal. Pick's disease was diagnosed. By May, he was unable to perform any daily living activities; he died in August 1993. Neuropathologic evaluation of brain tissue during autopsy showed symmetrical frontal lobe cerebral cortical atrophy and mild temporal lobe atrophy. No Pick's bodies or spongiform lesions were observed.

The man had a history of eating venison and participated regularly in wild game feasts held at the cabin owned by patient 1. He was a lifelong hunter and hunted mostly in Wisconsin but also in Wyoming and British Columbia. No game was brought to the wild game feasts from his hunting trips outside of Wisconsin. Examination of fixed brain tissue sent to NPDPSC demonstrated no lesions indicative of CJD, and immunohistochemical testing with antibody to the prion protein did not demonstrate the granular deposits seen in prion diseases.

Epidemiologic Investigation

Wild game feasts consisting of elk, deer, antelope, and other game that occurred at a cabin in northern Wisconsin owned by patient 1 began in 1976 and continued through 2002. These feasts typically involved 10--15 participants and usually occurred on weekends before or during hunting seasons in the fall and occasionally in the spring. Wild game brought to these feasts usually were harvested in Wisconsin, but three men who attended these feasts reported hunting in the western United States and bringing game back to Wisconsin. These activities took place in Colorado (near the towns of Cortez, Trinidad, Collbran, Durango, and Meeker), Wyoming (near the towns of Gilette and Cody), and Montana (near the town of Malta). CWD was not known to be endemic in these areas at the time that these hunting activities took place.

Information was obtained for 45 (85%) of 53 persons who were identified as possibly participating in the wild game feasts; all were male. Information was obtained by direct interview or from family members of decedents. Of the 45 persons, for whom information was obtained, 34 were reported to have attended wild game feasts. Seven of the 34 feast attendees were deceased, including the three patients. None of the four other decedents had a cause of death attributed to or associated with a degenerative neurologic disorder. None of the living participants had any signs or symptoms consistent with a degenerative neurologic disorder.

Reported by: JP Davis, MD, J Kazmierczak, DVM, M Wegner, MD, R Wierzba, Div of Public Health, State of Wisconsin Dept of Health and Family Svcs. P Gambetti, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio. L Schonberger, MD, R Maddox, MPH, E Belay, MD, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases; V Hsu, MD, EIS Officer, CDC.

Editorial Note:

CWD was first described in the United States in the 1960s and classified as a TSE in 1978. Previously localized to a contiguous endemic area in northeastern Colorado and southeast Wyoming, since 2000, CWD has been found in free-ranging deer or elk in Illinois, Nebraska, New Mexico, South Dakota, Wisconsin, and outside the previously known endemic areas of Colorado and Wyoming. CWD has been identified also in captive deer or elk in Colorado, Kansas, Minnesota, Montana, Nebraska, Oklahoma, South Dakota, and Wisconsin (4). Because a variant form of CJD, with specific neuropathologic and molecular characteristics that distinguish it from sporadic CJD, has been associated with eating cattle products infected with a prion that causes bovine spongiform encephalopathy (5), concern has been raised about the possibility that the prion associated with CWD might be transmitted to humans in a similar way.

In this investigation, because only one of the three cases in Wisconsin had neuropathologic confirmation of a prion disease, no association could be made between case participation in the wild game feasts and the development of CJD. Although patient 2 had confirmed CJD, he was unlikely to have eaten CWD-infected venison at these feasts because venison and other game from outside Wisconsin that was served at these feasts did not originate from known CWD-endemic areas, and the man participated in the feasts only once. In addition, the prion disease in this case was consistent with the most common form of sporadic CJD, without apparent unusual neuropathologic or molecular characteristics that might occur if the prion related to CWD had been responsible for the disease.

The findings in this report are subject to at least two limitations. First, not all members participating in wild game feasts could be identified, and not all persons listed as participating could be contacted for interviews. Second, interviews that were conducted required recall of events that occurred up to 25 years ago, limiting the detail or accuracy of events. However, the similar responses obtained from different sources support the accuracy of the investigation findings.

A previous investigation of unusually young CJD patients in whom the transmission of CWD was suspected also did not provide convincing evidence for a causal relationship between CWD and CJD (2). However, limited epidemiologic investigations cannot rule out the possibility that CWD might play a role in causing human illness. Ongoing surveillance of CJD, particularly in states with CWD, is important to assess the risk, if any, for CWD transmission to humans. Because the confirmation of CJD and the detection of a new prion disease require neuropathologic study of brain tissue, physicians are encouraged to contact NPDPSC (; telephone, 216-368-0587) to confirm diagnoses of CJD and to distinguish its various subtypes. Because of the known severity of TSEs in humans and the possibility that the CWD prion can affect humans, animals with evidence of CWD should be excluded from the human food or animal feed chains. Hunters and wild venison consumers should follow precautionary guidelines available from the Wisconsin Department of Agriculture, Trade, and Consumer Protection ( to prevent potential exposures to the CWD agent.


Belay E. Transmissible spongiform encephalopathies in humans. Annu Rev Microbiol 1999;53:283--314. Belay E, Gambetti P, Schonberger L, et al. Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Arch Neurol 2001;58:1673--8. Parchi P, Giese A, Capellari S, et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999;46:224--33. U.S. Department of Agriculture. Positive CWD cases: cumulative through Dec 2002 (including farm herds already depopulated). Available at

Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996;347:921--5.


CMAJ. 2003 September 2; 169(5): 443.

PMCID: PMC183301

Public Health

Wild game feasts and fatal degenerative neurologic illness

John Hoey

Author information ► Copyright and License information ►

This article has been cited by other articles in PMC.

Background and epidemiology: Creutzfeldt–Jakob disease (CJD) is one of a group of conditions known as prion diseases that are caused by abnormally configured host-encoded prion proteins that accumulate in central nervous system tissue. There are 3 forms: sporadic, genetically determined and, more recently, variant CJD, acquired by infection when the disease crosses the species barrier between humans and cattle.1 A similar prion disease in deer and elk known as chronic wasting disease (CWD) has raised concerns that this prion could also cross species barriers in people who eat wild game.

A report was recently published describing 3 cases of CWD in men who had attended wild game feasts.2 In the first case, a 66-year-old man who had been a lifelong hunter and who ate venison frequently had hosted wild game feasts at his cabin in Montana for 26 years before he died in 1992 of an autopsy-confirmed subacute spongiform encephalopathy compatible with CJD. He had a history of seizures since the age of 43 and had sought treatment for recurring seizures, increasing forgetfulness and worsening hand tremors about a month before he died.

The 2 other cases involved men aged 55 and 65 who died of neurologic illness. These men had histories of eating venison and participating in 1 or more of the first patient's wild game feasts. The younger man presented in 1999 with a 3-month history of difficulty in writing and unsteadiness of gait, followed by dementia, speech abnormalities and myoclonic jerking. Pathologic examination of the brain at autopsy 3 months later revealed widespread subcortical spongiform lesions consistent with CJD. He had visited the first patient's cabin 12 times and had participated in 1 wild game feast.

The older man sought treatment in 1992 for progressive slowing of speech, worsening memory and personality changes. His speech became reduced to single-word utterances, and he died 10 months after initial presentation. He had been a lifelong hunter and had hunted mostly in Wisconsin but also in Wyoming and British Columbia. He had participated regularly in the first patient's wild game feasts. No game had been brought to the wild game feasts from his hunting trips outside of Wisconsin.

Testing of fixed brain tissue from all 3 men revealed no evidence on immunostaining for prions using antibodies to the prion protein in the first and third patients. In the second patient prion disease was confirmed by means of immunohistochemical and Western blot testing. The blot characteristics and prion disease phenotype were consistent with the common form of sporadic CJD, classified as M/M (M/V) 1. Subsequent genetic typing revealed methionine homozygosity (M/M) at codon 129 of the patient's prion protein gene, making it likely that the patient had the most common form of sporadic CJD.

Information obtained for 45 people of 53 identified as possibly participating in the wild game feasts revealed that only 34 had actually attended them. Of the 34, 7 were dead, including the 3 described here; none of the other 4 had died of a degenerative neurologic disorder. None of the living participants had any signs or symptoms consistent with a degenerative neurologic disorder.

Clinical management: Because of the continuing possibility that CWD in deer and elk might be transmissible to humans, physicians should attempt to confirm cases of illness compatible with a transmissible spongiform encephalopathy through pathological examination of brain tissue and by alerting the Canadian CJD surveillance unit (; tel 888 489-2999) or the US National Prion Disease Pathology Surveillance Center ( There is no known treatment for the condition.

Prevention: CWD was first identified in the United States in 1967. In Canada, only 8 cases of CWD have been reported in wild deer, all in Saskatchewan. In the United States, CWD has been found in free-ranging deer and elk in Illinois, Nebraska, New Mexico, South Dakota, Wisconsin, Colorado and Wyoming. Limited epidemiologic investigations to date cannot exclude the possibility that CWD may cause human disease. Because of the severity of the spongiform encephalopathies in humans and the absence of treatment, animals with evidence of CWD should be excluded from human and animal food chains.

John Hoey CMAJ


Doctors study brain tissue of elk hunter / Montana man had symptoms similar to mad cow disease David Perlman

Chronicle Science Editor Published 4:00 a.m., Saturday, September 7, 2002


Researchers at UC San Francisco are analyzing the brain tissue of a Montana elk hunter who died last month with symptoms of a rare human disorder similar to mad cow disease in cattle and the chronic wasting disease that has felled elk and deer in the Midwest and Rocky mountains.

Last October, 50-year-old Gary Padgham of Bozeman first showed the stumbling gait, the dementia and the impaired vision and memory loss that are typical of Creutzfeldt-Jacob disease, known also as CJD. He was taken to a hospital in Seattle where doctors first diagnosed his illness as Huntington's disease, and then as CJD.

Padgham was later moved to Monterey where his family lives, and after he died in August his body was taken to UCSF for a specialized autopsy.

The institution is the nation's primary center for research in the varied brain diseases of humans and animals that are all believed to be caused and transmitted by malformed brain proteins called prions.

CJD is known to be one of the prion diseases, and researchers at UCSF study as many as 20 cases of suspected prion brain diseases a year in an effort to learn more about the invariably fatal malady.

The prions were discovered 20 years ago by Dr. Stanley B. Prusiner, a UCSF neurologist and virologist at UCSF who had been studying several strange brain diseases for a decade and won the 1997 Nobel prize in physiology and medicine for his prion work. Prusiner coined the term prion from the words "proteinaceous infectious particles."

Officials at the UCSF medical center would not discuss Padgham's case specifically Friday because of privacy rules, but a spokeswoman did note that although the disease-causing prions can be transmitted, there has never been a known case of humans contracting CJD from infected elk or deer.

Specialists at the California state health department said the chances are "slim to none" that Padgham could have contracted his disease from eating the meat of the elk he hunted in Montana.

According to the Federal Centers for Disease Control and Prevention, chronic wasting disease has hit captive deer and elk herds in several Midwestern and mountain states, and some wild deer in Wisconsin -- a development that has caused widespread fears among hunters.

In California, the Department of Fish and Game has asked hunters to stop bringing deer and elk parts back from states where the wasting disease has been found, and will seek a regulation banning the import of all high-risk parts, such as brains.

The CDC and other state agencies are also investigating the deaths of Padgham and three other hunters in Montana and Wisconsin. Two of the three died of Creutzfeldt-Jacob disease and one of a different brain disorder called Pick's Disease, where prions are not known to be involved.

Read more:



Observation| October 2001

Creutzfeldt-Jakob Disease in Unusually Young Patients Who Consumed VenisonFREE

Ermias D. Belay, MD; Pierluigi Gambetti, MD; Lawrence B. Schonberger, MD; Piero Parchi, MD; Douglas R. Lyon, MD; Sabina Capellari, MD; Jennifer H. McQuiston, DVM; Kristy Bradley, DVM; Gerrie Dowdle, MSPH; J. Michael Crutcher, MD; Craig R. Nichols, MPA

[+] Author Affiliations

Arch Neurol. 2001;58(10):1673-1678. doi:10-1001/pubs.Arch Neurol.-ISSN-0003-9942-58-10-nob10126.


In early 1997, a 28-year-old woman was examined several times in an emergency department for abnormal mental status, weakness, and unsteady gait. She also developed ataxia, dyskinesia, and marked dysarthria. The patient's condition gradually deteriorated, and she was admitted to a community hospital in March 1997. On admission, the patient had lethargia, athetoid and choreoform movements, constant lip smacking, possible hallucination, and increased muscle tone. After hospital admission, she developed primitive frontal release signs and episodic focal seizures. The electroencephalogram showed a severely abnormal tracing with diffuse, slow triphasic waves. The computed tomographic scan and magnetic resonance image of the brain did not reveal any abnormality. A brain biopsy performed to evaluate a suspected CJD diagnosis showed only gliosis. The patient died in June 1997, almost 4 months after the onset of illness. .

Histologic examination of brain tissue samples obtained at autopsy showed widespread spongiform degeneration involving the cerebral cortex. The spongiosis was associated with astrogliosis and moderate loss of neurons. The lesions seemed to be more prominent in the frontal lobe and in the entorhinal cortex. The basal ganglia; the thalamus, especially the mediodorsal nucleus; the tectum of the midbrain and pons; the substantia nigra; and the molecular layer of the cerebellar cortex all showed spongiosis and astrogliosis. Immunohistochemical examination for prion protein residues demonstrated a strong and consistent "synaptic" or "punctate" pattern of immunostaining in the cerebral cortex. Rarely, the immunostaining showed a preferential perineuronal distribution that involved both cell body and processes. Analysis of the PRNP indicated a Met/Met homozygosity at the polymorphic codon 129 and absence of genetic mutations. .

The patient's mother indicated that the patient had worked as a cashier at different department stores and a fast food restaurant. She had undergone tonsillar surgery at age 5 years. Her regular diet included consumption of beef, pork, and chicken several times a week. She might have consumed lamb or mutton once every several years. In addition, the patient consumed deer meat between ages 1 and 6 years. The deer were harvested mostly from Maine by the patient's father but occasionally from New Jersey by other family members. The deer carcasses were usually prepared by a custom processor. The patient primarily consumed the deer meat as steaks and ground meat mixed into sauce. At about 6 years of age, the patient had also consumed elk meat provided by a family friend as a gift on 2 different occasions. Although the origin of the elk could not be clearly ascertained, a family member reported that it was likely harvested in Wyoming. The patient was also reported to have occasionally consumed meat from squirrel, bear, and rabbits. No consumption of brain or organ meat from domesticated or game animals was reported. .


In September 1998, a 29-year-old man was examined at a university hospital for progressive cognitive difficulties. His illness began in May 1998, when he experienced difficulty completing his travel expenses after a routine business trip. The patient became increasingly forgetful, with inability to recall his wife's name, his own home telephone number, and names of long-time family friends. In August 1998 the patient resigned from his job because of the cognitive problems. Subsequently, he developed behavioral problems and difficulties with speech, writing, naming objects, and dressing without assistance. The patient was ambulatory without imbalance but had dysmetria, tremors, and occasional myoclonus. Findings from the initial 2 electroencephalograms, the computed tomographic scan, and cerebral angiographic studies were normal. The magnetic resonance image showed cerebral atrophy without any other abnormalities. Single-photon emission computed tomographic imaging of the brain revealed a nonspecific, asymmetric, diminished perfusion over the left parietal lobe. Results of initial cerebrospinal fluid analysis for 14-3-3 protein were negative in November 1998. A brain biopsy performed in November 1998 showed diffuse spongiform encephalopathy consistent with CJD. The patient died at age 30 years in March 1999, almost 10 months after the onset of illness. .

Histologic examination of the brain tissue samples obtained at autopsy showed prominent spongiform degeneration and gliosis with possible neuronal loss in the cerebral cortex and basal ganglia. In contrast, the cerebellum showed virtually no pathologic changes. The spongiosis often displayed a laminar distribution that affected the deep cortical regions. Neurons that were moderately ballooned were occasionally seen in the cerebral cortex. The pattern of prion protein immunostaining was exclusively "synaptic" in the cerebral cortex and basal ganglia, whereas the cerebellum was virtually unstained. Analysis of the PRNP indicated that the patient was homozygous for valine at the polymorphic codon 129. No PRNP mutation was detected. Immunoblot analysis showed that the PrP-res fragment migrated to 21 kd, corresponding to the prion protein type 1. .

His past occupations included working as a stock boy in a grocery store and recently as a salesperson. The patient had undergone a hernia repair during infancy and tonsillar surgery at approximately 10 years of age. His regular diet included consumption of beef many times a week and pork several times a week. He occasionally ate mutton and cow brain, approximately once every several years. The patient was described as a regular hunter, hunting almost every year since 1985. He was reported to have hunted deer and elk in many areas, almost always in Utah. He did, however, hunt an elk in the southwestern part of Wyoming in 1995 and was part of a team that hunted deer around Williams Lake, British Columbia, in 1989. The patient usually field dressed the carcasses himself and took them to a particular plant for custom processing. In addition, the family has many times received gifts of deer and elk meat from the patient's brother, who regularly hunted in Utah. The family usually ate the deer and elk meat as steak, ground meat, and jerky almost once a month. Moreover, the patient regularly ate liver from deer and elk but not other organ meat, including brain, from any game animals. .


In December 1998, a 27-year-old man began experiencing difficulty finding his hometown and lapses in memory while performing his duties as a truck driver. During the next month, the patient became increasingly forgetful, constantly asking for directions and instructions on how to operate the truck. He started to exhibit impulsive and impatient behavior, the inability to dress properly, difficulty finding words, and confusion. He also developed myoclonus and sleep disturbances. The electroencephalographic tracing was abnormal but nondiagnostic. The magnetic resonance image of the brain showed diffuse cortical hyperintensity with an unusual but nonspecific pattern. Findings from the cerebrospinal fluid 14-3-3 immunoassay were positive. Histopathologic examination of the cerebral cortical tissue samples obtained at biopsy revealed widespread spongiform degeneration associated with astrogliosis and a possible loss of neurons. Analysis of the PRNP indicated a Met/Val heterozygosity at codon 129 and absence of PRNP mutations. Immunoblot analysis showed that the PrP-res fragment migrated to 21 kd, in the polyacrylamide gel corresponding to the prion protein type 1. The patient died in April 2000 at age 28 years, almost 15 months after the onset of illness. No autopsy was performed. .

His occupations included driving a truck locally and spreading fertilizers. Since 1993 he had also been assisting his father-in-law in raising beef cattle. The patient had no history of surgical procedures. His regular diet included consumption of beef, pork, and chicken several times a week. No consumption of lamb or mutton was reported. The patient was described as an "avid hunter," hunting deer regularly since age 13 years, and frequently harvested at least 1 deer annually. Almost all his deer hunting took place exclusively in 2 very localized areas within 2 counties close to his hometown. The patient usually field dressed the deer carcasses himself and took them to a particular plant for custom processing. During the previous 6 years, the patient and his family had reportedly consumed only ground venison almost once a month. During his childhood, the patient also consumed deer meat harvested by his father. The patient's wife reported no consumption of deer or elk meat originating from either Colorado or Wyoming. However, the custom processing plant where the patient regularly took the deer carcasses for processing also processed approximately 20 elk from Colorado every year. The exact geographic origin in Colorado of these elk could not be ascertained. No consumption of brain or other organs from domesticated or game animals was reported. On the basis of hunter survey data obtained from the local Department of Wildlife Conservation, the patient's hunting practices were typical of other game animal hunters in the area. Analysis of the 1998 hunter licensing data estimated that approximately 24% of families and approximately 17% of households in the state might include a licensed hunter.


Tuesday, 1 May, 2001, 12:28 GMT 13:28 UK

Mad deer disease in Colorado


John Pape from Colorado's Health Department said: "At this point, the answers to these questions are unknown. Because of the BSE experience, it is theoretically possible, and based on that there are some precautions that hunters may want to take."

A farm in the mountains of Nevada may hold a key to the puzzle of Mad Deer disease.

The farm's owner, a cowboy called Jim Koepke, died suddenly from Creutzfeldt Jakob Disease (CJD), an illness very similar to both BSE and the Mad Deer variant.

Brenda, Jim's widow, admits that he loved to hunt. It is possible that he may have died from eating infected deer meat. "I couldn't comprehend that a cowboy in the middle of farming Nevada could get such a horrendous disease," she said. "So I dragged the poor man to many other doctors looking for something that made an ounce of sense."

Just coincidence?

Jim Koepke was the third deer hunter to die in the last two years from CJD. So far the evidence suggests that it is just coincidence.

But in Colorado, where as many as one in five deer and elk now has the disease, the authorities are urging caution.


Family fears another blood-borne disaster Saturday 27 March 1999 Mark Kennedy The Ottawa Citizen

Doug McEwen is dying of Creutzfeldt-Jakob disease, but plasma products from his blood have been cleared for distribution. Mark Kennedy reports. SALT LAKE CITY - Tracie McEwen reaches over to the dying man in the hospital bed that crowds her small living room. His eyes are closed and he has rarely opened them in recent days. As he moans softly, she strokes his arm and kisses his forehead. "It's OK. Doug, it's OK."

Tracie married Doug exactly four years ago on this date in late March. She marked their anniversary by pouring sparkling cider into cups, making a toast, and lovingly dropping some into Doug's mouth. It is celebration enough that he has survived this long, but it is a bittersweet experience. For he is just a shell of the man he was a few short months ago.

Mr. McEwen is dying of Creutzfeldt-Jakob Disease (CJD), a rare and always fatal neurological disorder that has many different forms. The horrifying condition creeps up on its victims, eating away at the brain, giving it the appearance of a sponge.

Experts say only one in a million people gets the most common strain, known as "classical" CJD, which causes death within a year of symptoms first appearing. The World Health Organization is so concerned about the potential growth of CJD that it is holding an international meeting to discuss how to deal with it.

Doug McEwen's case has spawned an international debate over whether a new, more virulent strain -- dubbed "mad deer disease" -- has appeared in North America.

Governments say Mr. McEwen, 30, has the classical strain, but some scientists question that diagnosis. They say it's possible the Utah man, who was an occasional hunter, is the first known victim of a newer strain contracted by eating deer and elk -- much the same way some in Britain contracted "mad cow disease" from eating infected beef in the 1980s.

In the nearby states of Colorado and Wyoming, there are herds of deer and elk suffering from chronic wasting disease (CWD), a neurological disease in animals associated with CJD. Consumer groups say governments are ignoring what could be the beginning of a worldwide epidemic, but other scientists say there is no basis for such a prediction, which they view as irresponsible and alarmist.

Complicating the situation is the fact that Mr. McEwen has been a blood plasma donor for nearly two years, starting in late 1996. His plasma was sent to the pharmaceutical company Bayer, which fractionated it into various blood products at its plant in Clayton, North Carolina. The products were then shipped to 46 countries around the world, including Canada.

When Mr. McEwen was diagnosed with CJD late last year, Health Canada ordered a temporary quarantine on products containing his plasma. Regulators were alarmed by his age -- classical CJD victims usually get the disease in their 50s and 60s. Only one per cent of them fall into Mr. McEwen's age bracket. Could this be like mad cow disease, regulators asked. After consulting outside experts, the U.S. Food and Drug Administration and Centers for Disease Control, it was determined that Mr. McEwen has the classical form. On Christmas Eve, blood products created with his plasma were cleared for redistribution.

At her apartment in the small town of Kaysville, 50 kilometres north of Salt Lake City, Tracie McEwen has been swamped with calls and visits from U.S. regulators and government scientists.

She believes governments in the U.S. and Canada have made a terrible error by underestimating the potential seriousness of her husband's disease and by prematurely lifting the ban on blood products linked to his plasma.

"I'm not a scientist but I think it's a serious threat," says the 28-year-old, who teaches geography to Grade 9 students. "It's a horrible disease and until they know exactly how it's spread, I think that all the governments are fools to ignore it the way they have. They're fools because they still can't tell me how he got it."

She says her husband, a devout Mormon, was "devastated" at the thought that somehow his disease might be spread through his plasma donations. "Of course, he never would have donated, had we had known," says Tracie, tears welling up in her eyes. "It made me sick when I found out they had put it back on the market. They had it. They had it. They didn't have to use it. That would have been how many less people getting it? And they still put it out. And they still don't know. It's wrong."

Last summer, Doug was a big, strapping man who lived life to its fullest. He fished and hiked, and rode his bicycle in the stunning Utah mountains. He devoted himself to Tracie and their daughters, Sharon, 8, and Rilee, 3. Since then, his weight has dropped from 230 pounds to 130 pounds. He has been trapped in a body that has steadily given up on him.

It started slowly. First, there was the memory loss and the inability to do simple math, then the light tremors. Eventually, came violent seizures as well as the unexplainable outbursts of emotion -- hysterical laughter, sometimes followed by uncontrollable crying. By late January, he could no longer speak in sentences. Three weeks ago, he stopped walking.

Now, he is near death. Tracie, who has cared for all his needs and slept next to him every night on a floor mattress, can only wait for the inevitable.

"This is the worst thing I have seen," she says. "I wouldn't wish it on my worst enemy."


snip...see full text and more ;


Sunday, November 11, 2012

Susceptibilities of Nonhuman Primates to Chronic Wasting Disease November 2012




The Center For FOOD SAFETY


 8 May 2000


 Commissioner Jane Henney Food and Drug Administration Parklawn Building, Room 1471 5600 Fishers Lane Rockville, MD 20857


 CC: Docket No. 99P-0033 FDA Dockets Management Branch HFA - 305 5630 Fishers Lane, Room 1061 Rockville, MD 20852


 Dear Commissioner Henney:


 Pursuant to the Administrative Procedure Act ("APA"), 5 U.S.C. S 553 (e), and the FDA implementing regulations, the Humane Farming Association, the Center for Food Safety and others petitioned your office in January 7, 1999, to take action regarding, inter alia, the potential human and animal health impacts, including transmissible spongiform encephalopathies (TSEs) associated with the current FDA animal feed regulations found at 21 C.F.R. 589.2000. See FDA Docket No. 99P-0033. More specifically, the agency has been requested to initiate new rulemaking to close loopholes in current animal feed regulations that create risks of TSE transmission in animals and pose a significant health threat to the public.


 Since the filing of the petition well over a year ago, your office has failed to take any action concerning the issues presented by the petitioners. Unfortunately, the issues presented in the petition are more salient than ever. A fatal TSE disease called 'chronic wasting disease' or CWD is occurring at epidemic levels in deer and elk in Western states and on game farms. This TSE may already be claiming human lives as suggested by the alarming appearance of unusually young victims of Creutzfeldt-Jakob disease or CJD, a human form of TSE. Some scientists suspect that CWD emerged when a strain of scrapie, a TSE widespread in US sheep, transmitted to deer and elk. Now, CWD may have transmitted from deer and elk into humans as CJD.


 - 2 -


 Our original petition cited the case of Doug McEwen of Utah, who is now dead of CJD, as an example of the potential human health threat posed by the continued lax FDA animal feeding regulations. Since the filing of our petition news reports have mentioned other young, confirmed and suspected victims. For example: Jay Dee Whitlock II, age 28, of Miami, Oklahoma died of CJD less than a month ago on April 7, 2000. Mr. Whitlock was an avid deer hunter and consumer of venison. Jim Koepke, age 39, died of CJD on February 6, 1999, and was also a consumer of deer and elk.


 We can look at Great Britain to see the massive human and animal health, economic and environmental disaster caused by the British TSE dubbed 'mad cow disease,' a bovine spongiform encephalopathy or BSE. The current human death toll from BSE is 53 and rising. In the decades ahead some scientists suspect it could kill hundreds of thousands of people.


 It was the emergence of a "new variant CJD" or nvCJD in unusually young victims that led to the current scientific consensus that mad cow disease is killing humans. Thus, the appearance of unusually young CJD victims in the US is a very disconcerting development in light of the widespread occurrence of US sheep scrapie and the emergence of CWD at epidemic levels in deer and elk.


 Furthermore, given the situation in Great Britain, it may be just months or a couple years before cases of nvCJD appear in the US among people who lived in or visited Britain in the 1980s. This event, no matter how it is downplayed by industry interests, will emphasize the inadequacies of current US regulations and our own dilemmas with chronic wasting disease and sheep scrapie.


 On August 5, 1999, petitioners reiterated the request that the agency answer their legal petition. In correspondence, FDA Director of the Center for Veterinary Medicine Stephen Sundlof stated that the agency required more time to respond. The time afforded the agency since Dr. Sundlof's response has been more than adequate to facilitate the agency's answer to the petition. And, as of November 1999, well over 250 members of the public have written in support of the petition's request.


 Given the mounting evidence in favor of taking the most precautionary stance toward our own animal feeding regulations, in refusing to act the FDA continues to deny petitioners and these members of the public relief at the agency level and is a constructive denial of the petitioner's request. As such, petitioners intend to pursue other avenues, including judicial review, in order to assure that the agency responds to the issues raised by the petitioners.


 - 3 -


 Indeed, the agency inaction in this matter is subject to judicial review. Under the APA "agency action" is defined to include "the whole or part of an agency rule, order, license, sanction, relief, or the equivalent denial thereof, or failure to act" and gives courts the power to "compel agency action unlawfully withheld or unreasonably delayed." Thus, the APA authorizes courts to review agency decisions to refrain from taking action.


 When administrative inaction has precisely the same impact on the rights of the parties as denial of relief, an agency cannot preclude judicial review by casting its decision in the form of inaction rather than in the form of an order denying relief.


 In addition, the agency's inaction is violative of established agency regulations. The FDA has established regulations in which a reasonable period for agency response to citizen petitions can be no more than 180 days. Regulations which are promulgated by an administrative agency in carrying out its statutory mandate can also provide standards for judicial review of agency action. Such self-imposed constraints may supply the "law to apply" to overcome the judicial presumption against reviewing administrative inaction. Thus, the agency must act in a "prompt" manner or be subject to further action. The agency's delay in answering the current petition amounts to a refusal to act, with sufficient finality and ripeness to permit judicial review.


 On behalf of the petitioners,


 Joseph Mendelson, III Legal Director







The webmaster has learned that the Schmerr blood tests on humans have been ongoing for 18 months; it is possible that the English had some positive tonsilectomies where blood samples could be taken and that these have since gone clinical. Reportedly, some, but not all, US sporadic CJD cases have tested positive, some perhaps blood donors. Further, Schmerr is said to be most unhappy with the decision in Dec 98 to reverse the blood recall on Doug McEwen. Million of doses of medicine subsequently were injected worldwide. (There is no information on whether a test was done there, or if one could still be done.) At her talk at the Tubingen meeting, Schmerr reported lambs in scrapie environment can become prp-res positive in blood at 4 weeks of age (even one at 2 weeks!). Test works on elk, md, sheep, hamster. This gives rise to hopes that provided facilities are not too contaminated with persistent agent, livestock producers could test their way out of the disease. The August 1999 MJ Schmerr article in J Chrom. provides some technical aspects of the assay but does not answer many questions about where things are as of now. Dr.Mary Jo Schmerr email address is Her title is Research Chemist, National Animal Disease Center, Respiratory & Neurologic Disease Research Ames, Iowa tel (515) 239-8287 X287 fax (515) 239-8458 web







Subject: deer hunters and farmers and TSEs (a comparison) & a small survey of sCJD/CWD?


Date: Mon, 23 Sep 2002 22:03:13 –0700


From: "Terry S. Singeltary Sr."


Reply-To: Bovine Spongiform Encephalopathy




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



 Greetings BSE-L members,


 a comparison of sporadic CJD's in Farmers and Hunters. and below this, a small survey done on CJD Voice list. i asked about consumption of deer/elk or beef and any organs that might have been consumed...TSS




 In 1995, there were 34 deaths from CJD (29 sporadic cases and 4 iatrogenic cases - see Table []). The number of sporadic cases represents a significant decrease on the 1994 figure of 59 cases although this remains provisional until the 1995 data finalised. The make up of cases recorded included the __fourth__ farmer and the first teenager to have died from CJD in the UK. A second case of CJD in a teenager was confirmed in 1995, but the patient remains alive. These cases are unusual but not outside the bounds of probability...





DFA 16






Farmer 4



71 On 28 September 1995 Dr Wight minuted Dr CalmanâEUR^(TM)s private secretary about a probable fourth case of CJD in a beef farmer who had had a case of BSE in his herd four years earlier.[95] She copied the minute to Dr Metters, Dr Rubery, Dr Skinner, Mr Lister, Ms French (ID) and Mr Eddy (MAFF). Dr Wight noted:



If he is confirmed as suffering from CJD, this brings the total number of cases in farmers in the UK to four. CMO will recall that earlier advice the SEAC had from Professor Peter SmithâEUR¦ was that the occurrence of a fourth case (in a dairy farmer) would be of serious concern...



The CJD Unit hope to visit tomorrow more details will be available then.



There is some urgency in dealing with this as the case is already in the public domain âEUR" BBC Wales are making a programme which refers to this case. In addition, the Lancet articles on the third UK farmer and on the European situation are to be published tomorrow. Together, these factors are very likely to provoke wider interest.[96]



72 In a minute the same day, Mr Eddy informed the MAFF Ministers about the fourth farmer.[97] He said that DH would âEUR~take the lead in handling any media enquiriesâEUR^(TM).[98] He noted that the local media were aware of the case and we understand will be running an item on it today. This could stimulate further media interest).[99]



73 Mr Eddys minute recalled the (July) 1993 advice from Professor Peter Smith that if four cases of CJD occurred in farmers over a five year period then the possibility that the association was not due to chance had to be given very serious consideration.[100]



74 Dr Calman met DH staff the next day (29 September) to discuss the latest findings in research and any further safety measures which should be taken to minimise the risk to.[101] An emergency meeting of SEAC was scheduled for the next week, on 4 October.[102]



75 On 29 September 1995 Mr Lister minuted Baroness Cumberleges private secretary, and copied the minute to the private secretaries of Mr Packer and Dr Calman, and to Drs Metters, Rubery, Skinner, Wight, and Callaghan, Mr Jobson, Mrs French (ID), Mr C K McIntosh (SHHD), Ms Liz Jones (WO), Mr T Robinson (DHSS NI) and Dr Render (MAFF). He invited Baroness Cumberlege to agree on a publication date for the CJD Surveillance Units Annual Report, and he advised her of a fourth case of BSE in a farmer whose herd had suffered from BSE. He said that a special SEAC meeting had been called to discuss the case, and that the line to take in the meantime was We are aware of the case. The necessary investigations are underway, and we cannot comment further at present.[103]



76 On 29 September 1995 the press reported the third case of CJD in a dairy farmer.[104] This information was from a letter which had been published in the Lancet, dated 30 September 1995. The letter said:



The occurrence of CJD in another dairy farmer with a potential occupational exposure to BSE is clearly a matter of concern. Statistical analysis indicates that the probability of discovering three or more dairy farmers with CJD by chance since 1990 in England and Wales ranges from 0.09 to 0.0002 [105]



77 In the same issue of the Lancet, there was a publication of the European statistics for CJD in farmers. It concluded that there is no differential increase in the risk of CJD to farmers in the UK through potential occupational contact with cases of BSE.[106] Dr Wight explained to the BSE Inquiry that on the continent there was also a slight excess of CJD cases arising in farmers.[107] Dr Will said it indicated that in the UK, CJD in farmers had probably not been transmitted from BSE.[108]



78 Dr Skinner (DH) told the BSE Inquiry that when he was advised at the end of September of the possible fourth case of CJD in a farmer, this information together with the slowly emerging information concerning breaches of the SBO ban, caused considerable unease.[109]



October 1995



79 On 3 October 1995 there was a meeting between MAFF and DH Permanent Secretaries. Present from MAFF were Mr Packer, Mr Carden, Mr Meldrum and Mr Dickinson, and from DH, Mr Hart, Dr Calman, Dr Skinner and Mr Lister.[110]



80 The DH files show some notes about this meeting. The notes identified two teen-aged cases of CJD, in a 19- and a 17-year-old, the second of which was not public and should be treated as confidential. It was noted that CJD had been described previously in two adolescents in the USA which is free of BSE and one adolescent in France at a time when it too was free of BSE.[111]



81 minutes of the meeting record that DH and MAFF



noted a suspected new case of CJD in a cattle farmer who had had BSE in his herd. There was no conclusive evidence that recent cases of CJD were linked to BSE, but it was agreed that it was important to keep the issue under close review, particularly in view of the long incubation period of CJD.[112]



82 Mr Packers private secretary wrote to DH on 24 October 1995 suggesting some changes to these minutes, including deletion of the word conclusive (see 24 October below).[113]



SEAC Meeting 21



83 SEAC emergency meeting to discuss the fourth farmer was held on 4 October 1995.



84 SEAC reached the following main conclusions at the meeting:



there had been a worrying number of cases in farmers exposed to cattle with BSE. However, if there were an occupational link, there would be other occupations that might be expected to be at greater risk, and there was no evidence for this;



the stage was being reached where it was difficult to explain the cases as a chance phenomenon. However, although significant, the absolute risk remained extremely low;



the evidence in the current case was exposure to BSE for a short period. There was also the likely presence of meat and bonemeal fed to poultry before 1990;



it was unclear whether the potential risk factor might be association with animals with BSE or the food given to them (it was known that some farmers ate small amounts of calf and cattle feed but not whether they also ate pig and poultry feed). It was suggested that there might be a problem with dust from feed but that this should be more of a problem with dust compounders;



given that there was a problem relating the case to a causal link, the transmission studies were particularly important;



it was still necessary to make a final diagnosis of CJD in the possible 4th case.[114]



85 At the meeting,



Dr Will summarised recent cases of sporadic CJD in young people. There were currently two cases in teenagers a 19 year old and a 17 year old. The case in the 17 year old had unusual pathology, although this could be related to age. There were also other cases in their 30s and 40s, which was unusual. However, although this was a change from previous experience, it was difficult to relate to BSE.[115]



86 Also at the meeting, Dr Wight invited members to make a fairly clear statement on how they viewed the significance of a fourth case. She also invited the Committee to consider whether they were satisfied that nothing else needed to be done in terms of practical measures.[116] Dr Wight told the BSE Inquiry I do not think that SEAC any more than anybody else had any idea of how to make sense of this at that stage.[117]



87 In response to Dr Wight invitation, SEAC agreed to draw up a statement which the Department of Health could issue in response to media enquiries.[118]



88 The statement, attached to the minutes, said that SEAC had not altered their advice to Government on the precautions necessary to protect either the public health [sic], including farmers, or animal health.[119]



89 The statement said that SEAC had



...also noted that surveillance of CJD elsewhere in Europe has shown a similar incidence of CJD in farmers, including dairy farmers, in countries with no or very few cases of BSE. They [SEAC] therefore felt that it was important to undertake further epidemiological studies to detect any particular risk factors which might be involved, and reiterated their advice that the UK cases of CJD in cattle farmers and the strain of the agent recovered from them should be studied in detail.[120]



90 This was Dr Tyrrells last SEAC meeting because he was retiring. Professor Pattison was taking over the SEAC Chair.



91 That day (4 October), after the meeting, Mr Eddy minuted the MAFF Ministers to alert them of the outcome of SEACs meeting. He copied his minute to a number of officials, including Mr Bradley (CVL), Mr Lister (DH) and Mr Huws (Welsh Office). He said that the CJD Surveillance Unit had



downgraded the case from a probable to a possible which reflects some uncertainty about the diagnosis now that the symptoms have been examined in more detail by the experts but final confirmation will depend on further tests and ultimately the post mortem.



SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD would have had BSE cases on their farms. It was difficult to calculate accurately the likelihood of this being due to a series of random events; but looking at all male farmers and farm workers in England and Wales the chance of four CJD cases occurring randomly since 1990 was around 5/100; the chances of four cases of CJD occurring randomly in farmers with BSE in their herds was very much lower, around 3/10,000. The Committee therefore concluded that it was difficult to explain the incidence as a chance phenomenon. This is a change to the Committees position; it had said that the most likely explanation of the three previous cases of CJD in dairy farm workers was that they were chance phenomena...[121]



92 Mr Eddy advised that SEAC had not recommended any changes to the measures currently in place to protect human and animal health, including that of farmers and others handling cattle and BSE suspects.[122]



93 The same day (4 October), Mr Eddy minuted Dr Danny Matthews (MAFF, technical adviser to SEAC) about discussions during the SEAC meeting. He copied the minute to Mr Keith Meldrum (CVO) and others. At the SEAC meeting, Dr William Watson, Director of the Central Veterinary Laboratory (CVL), had briefly listed four possible routes of infection although neither he nor anyone else felt any of them were particularly plausible.[123] Mr Eddy listed them:



(i.) that cattle were excreting the agent in some form there is no evidence for this least of all from the epidemiology of BSE in cattle;



(ii.) that farmers may be exposed through meat and bonemeal in cattle feed again it was felt that if feed, rather than the cattle themselves, were a problem then there should be a similar excess of cases in pig and poultry farmers where more ruminant material would be in feed;



(iii.) from normal food as for the rest of the population but it was unclear why this should discriminate in favour of cattle farmers;



(iv.) through contact with animals possibly animals killed on the farm and possibly with BSE suspects.[124]



1. The last point entailed discussion of the slaughter procedures for BSE-suspect cattle and Mr Eddy suggested putting together a short note for SEAC to cover this point.[125]



2. Dr Matthews wrote a paper for presentation at SEACs next meeting on 23 November.[126]



94 In a press release dated 5 October 1995, DH announced the Fourth Annual CJD Surveillance Unit report.[127] The report showed an increase in the number of deaths from CJD in 1994. The press release quoted Dr Calman saying:



I continue to be satisfied that there is currently no evidence of a link between meat eating and development of CJD and that beef and other meats are safe to eat. However, in view of the long incubation period of CJD, it is important that the Unit continues its careful surveillance of CJD for some years to come.[128]








































young USA victims ?



20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. A 19 year old died from sCJD in France in 1985. There is no evidence of an iatrogenic cause for those cases....






cover-up of 4th farm worker ???














now story changes from;



SEAC concluded that, if the fourth case were confirmed, it would be worrying, especially as all four farmers with CJD would have had BSE cases on their farms.








This is not unexpected...



was another farmer expected?









Deer Hunters














CONCLUSIONS: Although the occurrence of 3 unusually young patients with CJD who consumed venison suggested a possible relationship with CWD, our follow-up investigation found no strong evidence for a causal link. Ongoing CJD surveillance remains important for continuing to assess the risk, if any, of CWD transmission to humans.






Article Last Updated: Sunday, September 22, 2002 - 3:37:40 AM MST



'Mad cow' tainted blood a risk






All those pleas that blood banks make when supplies dwindle spurred Doug McEwen to roll up his sleeves not once, not twice


 but more than a dozen times to donate blood.


 McEwen, a rugged guy who enjoyed hunting deer and eating game, had a good job in Salt Lake City and a solid future. He was 29 years old with a wife, two young children and a new house


 the couple's first


 under construction. He felt blessed with a good life. And partly because of his Mormon upbringing, he tried to help others by donating blood.


 He's dead now. And his generosity haunts his surviving relatives. That's because they fear that through a transfusion someone else might contract what killed McEwen


 Creutzfeldt-Jakob disease, an always-fatal condition caused by mutated prions. It's the same protein linked with "mad cow" disease and its human form in Great Britain, a new variant of Creutzfeldt-Jakob disease.


 "This might be the 21st century's own AIDS epidemic," says John Stauber, co-author of Mad Cow USA. "That's how massive an epidemic we face."


 It seems that "more could have been done" says Kay McEwen, his mother, from her home in Salt Lake City. "I don't think they knew what they were doing with him," she says of the neurologists and disease detectives who have studied slivers of her son's brain since his death in 1999. Part of their aim was to determine whether McEwen consumed venison infected with chronic wasting disease, a neurologic prion disorder in deer and elk, similar to mad cow, that has spread in some parts of Colorado, Wyoming, Michigan and Wisconsin.


 "Now I think they are trying to ignore it," as if it didn't exist and isn't any problem, McEwen says, her voice cracking. "I'm sorry but it's gotten so I can't talk about it."


 Voluntary recall


 Eventually all those pints of McEwen's blood were pooled with donations from as many as 60,000 other people. Then it was divvied up into countless plasma products that Bayer corporation, with facilities in West Haven, Conn., and Raleigh, N.C., shipped before the U.S. Food & Drug Administration suggested a "voluntary recall" of McEwen's plasma.


 "We treated it as if all the plasma we had [on hand], all the inventory and products could be implicated. Everything was put on hold," says Stephen R. Petteway Jr., vice president of research and pathogen safety at Bayer's biological products division. "We stopped everything that we were doing with it."


 Bayer began a rigorous self-examination of its plasma purification process


 convincing both itself and the FDA that its processing and in-house testing were capable of filtering out prions, lowering the chances of infection by 100,000-fold


 or more than 99.99 percent.


 Chances remote


 But what of those other products the pharmaceutical company generated from McEwen's blood that already were distributed? Are prions quietly circulating in somebody else's blood undetected?


 Michael Hansen, a scientist with Consumers Union, says Bayer informed McEwen's family that his blood potentially was already in 120 separate plasma products by the time McEwen became aware of his condition.






full text;






Rec 16 May 2002; Acc 9 July 2002 DOI: 10.1099/vir.0.18580-0



Transmission of prion diseases by blood transfusion



Nora Hunter,1 James Foster,1 Angela Chong,1 Sandra McCutcheon,2 David Parnham,1 Samantha Eaton,1 Calum MacKenzie1 and Fiona Houston2



see full text;






Subject: CDC looks at deaths of 3 for possible chronic wasting link ($$$all sporadic $$$)


Date: August 13, 2002 at 8:50 am PST




CDC looks at deaths of 3 for possible chronic wasting link




6:20 PM 8/12/02 Ron Seely Science reporter


 An investigator with the Centers for Disease Control and Prevention in Atlanta has been in Wisconsin for two weeks looking into the deaths of three men who died of neurological diseases and who may have eaten venison at the same game feed.


 State epidemiologist Jim Kazmierczak said Monday he and other state health officials are assisting the CDC investigator, who spent last week interviewing relatives of the three men. Two of the men died of Creutzfeldt-Jakob disease and the third died of Pick's disease, another more common neurological illness.


 The cases have attracted attention because of concerns that chronic wasting disease, found in the Wisconsin deer herd in February, could eventually show up in humans in the form of CJD, a fatal brain illness that is in the same class of diseases as CWD.


 Although there has been no scientifically proven link between CWD and the human illness CJD, both are versions of a family of diseases called spongiform encephalopathies. Health officials are also concerned because mad cow disease, the version of the illness that affects cattle, did jump the species barrier in Europe and has killed 125 people.


 Ermias Belay, a medical epidemiologist with the CDC confirmed in an interview from Atlanta that the agency is looking for links between CWD and the deaths of the three men.


 "We are in the early stages of the investigation," said Belay. "We're interviewing relatives and friends as well as fellow hunters. We're here to gather as much information as we can."


 But Belay also warned against jumping to conclusions. He said a similar case studied in Michigan showed no connection between the consumption of venison and the deaths of two men there from CJD.


 The investigation is focusing on the deaths of Wayne Waterhouse, of Chetek, and Roger Marten, of Mondovi. Both men died in 1993 at the age of 66. The third man, James Botts, 55, of Blaine, Minn., died in 1999.


 Waterhouse and Botts died of Creutzfeldt-Jakob while Marten died of Pick's disease. But what interests investigators most is that all three men knew one another and ate at wild game feasts that Waterhouse hosted at his cabin near Superior.


 Kazmierczak said the investigation has focused not only on the deaths of the three men but also on who else might have attended the game feeds and whether any other guests have died of neurological illnesses. He said at least 50 people attended the dinners and added that investigators now have names and addresses for 40 of them. So far, 20 of those people have been interviewed and no other evidence of illness has surfaced.


 "We want to make sure we don't have another case that someone hasn't told us about," Kazmierczak said.


 In addition, tissues collected from the three men during their autopsies are being sent to a national lab that specializes in studying such diseases. One important part of the investigation, Kazmierczak said, will be to see if there are similarities in the strains of the disease that killed each of the men. In Europe, he added, the version of CJD that people contracted eating tainted beef was similar in each victim.


 Belay also expressed interested in another CJD death, reported last week in the Janesville Gazette. The newspaper reported that Larry Brown, of Edgerton, died of the disease in 1998 at the age of 59. Brown, according to the report, hunted and ate deer from both Wisconsin and Colorado.


 Increasing interest in possible connections between CWD and human illness is also prompting the state to make Creutzfeldt-Jakob a reportable disease, according to Kazmierczak. Belay said the disease is not reportable nationwide but individual states can decide on their own to require that all cases be reported.


 Currently, Kazmierczak said, the only information available is from death records. He said a review of those records between 1991 and 2000 shows the following number of CJD cases: 5 in 1991, 5 in 1992, 7 in 1993, 4 in 1994, 3 in 1995, 3 in 1996, 6 in 1997, 6 in 1998, 3 in 1999, and 1 in 2000. Those numbers, he added, show the disease is no more frequent here than elsewhere.







2001 ADDITIONAL CASE INVESTIGATIONS (3 more young cases)


 * Investigated a 25-year-old patient who consumed venison originating from Southeastern Wyoming.


 * He was later shown to have GSS 102 mutation with valine at codon 129 in the mutant allele of the prion protein gene.


 * Investigated 2 patients (26 and 28 years of age) who lived in adjacent counties, and had illness and had illness onset within a few months of each other.


 * Although venison consumption was reported for the 28-year-old patient at 1.5 years of age, this history was questionable; the immunohistochemical analysis was consistant with GSS.


 * No venison consumption history was reported for the 26-year-old.





 Continued surveillance is critical to monitor the possible occurance of BSE and vCJD in the United States as well as monitor the risk, if any, of CWD transmission to humans.




 Greetings List,


 from a conference of Anatomy of Emerging Infectious Diseases. not two, but _three_ more under 30 CJD victims in 2001 in USA. please note where Dr. Belay stipulates about the fact that the suspect CJD victims in the USA autopsies are "very low". so i definitely would not make CJD/Human TSEs reportable? or why bother testing for TSEs in cattle in numbers sufficient to find a TSE, since CWD is spreading amongst deer/elk?





Anatomy of Emerging Infectious Diseases Panel Session (Wednesday, March 27)


 Impact of the BSE/vCJD Outbreak: U.S. Concerns for TSEs





The Agent: Prions as Emerging Infectious Particles





The Reservoir: Bovine Spongiform Encephalopathy and What's Been Done About It





The Unfolding Variant (vCJD) Epidemic Robert Will, Western General Hospital, Edinburgh, United Kingdom





Diagnosis and Reporting of Creutzfeldt-Jakob Disease T. S. Singeltary, Sr; D. E. Kraemer; R. V. Gibbons, R. C. Holman, E. D. Belay, L. B. Schonberger





sporadic CJDs do have a cause and a route, potentially many of both. many possible routes and sources exist in the USA. there are now, 6 different strains of sporadic CJDs;





 also, i have recently done a small survey on the CJD-Voice-List asking for anyone of the families whos loved ones consumed deer/elk or beef and or organs. some of the submissions may surprise you. a few examples;


 Terry: You probably have my info. already, but just in case, there are 3 CJD cases that I am very familiar with -- my dad, his good friend of 30+ years and a husband of a former coworker of mine. Here's what I know:


 Dad - regular, yearly deer hunter for at least 50 years. He probably ate squirrel brains as a young teen, but never after he married my mom when he was 19. He died at age 74.


 Dad's friend - regular, yearly deer hunter for at least 30 years. He and my dad participated in many church suppers where all the men brought various potluck deer meat meals. He was 66 when he died.


 My friend's husband - never ate deer at least after he married her when he was 30. He served overseas in the military. He was 69 when he died.


 All 3 men were autopsied and diagnosed "sporadic" CJD.






different submission;



terry. My husband was a hunter and ate deer meat all the time. He started making sausage for about the last 5 years. I'm not sure what part of the deer goes in to that. no deer supplements and the biggest part of his diet was beef. he was only sick for 31 days from the first day to his death.






different submission;



However, when she was growing up (during the depression), her mother used to make sheep's head soup, in which the head was used in much the same way as a ham bone would be, and the brains were creamed and added to the soup. She used to rave about how good that soup was! Also, before moving to the U.S. from Scotland, our whole family occasionally used to eat calves' liver and frequently ate tripe (one of my favorite dishes as a child).






different submission;



I believe thats how my husband got it. He went hunting in Colorado in 1987 and died of CJD in 1996. About the right timing!!!






different submission;



Hi Terry, My mom consumed venison on a regular basis her entire life. My parents even fed a white tail deer herd ,of about 30 in there back yard throughout the winters, then grew a vegtable garden in the same area in the summer. My parents had a deer experimental station very close to thier home and several years ago had a couple of escapees from there come into there yard, 5 deer in all. They said they were some of the sickest deer they had ever seen. Extremely thin,stumbly and appeared to not have a clue as to what they were doing. The DNR guys came looking for them and asked if my parents had seen them. When asked what was wrong with them the DNR would say nothing about the sick deer, only that it was very important that they get them as soon as possible...






*** Friday, December 14, 2012 ***




DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012




In the USA, under the Food and Drug Administration’s BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients.



*** For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.



Animals considered at high risk for CWD include:


1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and


2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.


Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.


The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES. It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.


Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.


There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.




36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011).


The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE).


Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison.




The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008).




In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion.




In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible. For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.




Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.






see full text report here ;






see much more here ;




Friday, December 14, 2012


DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012






Friday, November 09, 2012


*** Chronic Wasting Disease CWD in cervidae and transmission to other species





Sunday, November 11, 2012


*** Susceptibilities of Nonhuman Primates to Chronic Wasting Disease November 2012





Friday, December 14, 2012


Susceptibility Chronic Wasting Disease (CWD) in wild cervids to Humans 2005 – December 14, 2012





Tuesday, April 16, 2013


Cervid Industry Unites To Set Direction for CWD Reform and seem to ignore their ignorance and denial in their role in spreading Chronic Wasting Disease




Monday, June 24, 2013


The Effects of Chronic Wasting Disease on the Pennsylvania Cervid Industry Following its Discovery





Sunday, July 07, 2013


Could avian scavengers translocate infectious prions to disease-free areas initiating new foci of chronic wasting disease?


Prion. 2013 Jul 3;7(4). [Epub ahead of print]






Thursday, June 20, 2013


atypical, BSE, CWD, Scrapie, Captive Farmed shooting pens (livestock), Wild Cervids, Rectal Mucosa Biopsy 2012 USAHA Proceedings, and CJD TSE prion Update





Friday, February 08, 2013


*** Behavior of Prions in the Environment: Implications for Prion Biology





Wednesday, April 24, 2013

Dissociation between Transmissible Spongiform Encephalopathy (TSE) Infectivity and Proteinase K-Resistant PrPSc Levels in Peripheral Tissue from a Murine Transgenic Model of TSE Disease



Thursday, May 02, 2013


Chronic Wasting Disease (CWD) Texas Important Update on OBEX ONLY TEXTING





Wednesday, May 15, 2013


Intranasal Inoculation of White-Tailed Deer (Odocoileus virginianus) with Lyophilized Chronic Wasting Disease Prion Particulate Complexed to Montmorillonite Clay


Research Article






Tuesday, June 11, 2013


Weld County Bi-Products dba Fort Morgan Pet Foods 6/1/12 significant deviations from requirements in FDA regulations that are intended to reduce the risk of bovine spongiform encephalopathy (BSE) within the United States







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."











Saturday, June 29, 2013









Tuesday, May 28, 2013



Chronic Wasting Disease CWD quarantine Louisiana via CWD index herd Pennsylvania Update May 28, 2013



6 doe from Pennsylvania CWD index herd still on the loose in Louisiana, quarantine began on October 18, 2012, still ongoing, Lake Charles premises.






Tuesday, June 11, 2013


CWD GONE WILD, More cervid escapees from more shooting pens on the loose in Pennsylvania







Wednesday, June 12, 2013


CWD now waltzing into Texas from Pennsylvania CWD index herd, via Louisiana, or Missouri now ?







Thursday, June 27, 2013


Introducing a Rigid Loop Structure from Deer into Mouse Prion Protein Increases Its Propensity for Misfolding In Vitro







Tuesday, July 2, 2013


APHIS USDA Administrator Message to Stakeholders: Agency Vision and Goals Eliminating ALL remaining BSE barriers to export market






Saturday, July 6, 2013


Small Ruminant Nor98 Prions Share Biochemical Features with Human Gerstmann-Sträussler-Scheinker Disease and Variably Protease-Sensitive Prionopathy


Research Article






Tuesday, June 11, 2013


Weld County Bi-Products dba Fort Morgan Pet Foods 6/1/12 significant deviations from requirements in FDA regulations that are intended to reduce the risk of bovine spongiform encephalopathy (BSE) within the United States






Thursday, June 6, 2013









since our fine federal friends have decided not to give out any more reports on the USA breaches of the feed ban and surveillance etc. for the BSE TSE prion mad cow type disease in the USDA livestock, I thought I might attempt it. I swear, I just don’t understand the logic of the SSS policy, and that includes all of it. I assure you, it would be much easier, and probably better for the FDA and the USDA INC., if they would simply put some kind of report out for Pete’s sake, instead of me doing it after I get mad, because I am going to put it all out there. the truth.



PLEASE BE ADVISED, any breach of any of the above classifications OAI, VAI, RTS, CAN lead to breaches into the feed BSE TSE prion protocols, and CAN lead to the eventual suspect tainted feed reaching livestock. please, if any USDA official out there disputes this, please explain then how they could not. paperwork errors can eventually lead to breaches of the BSE TSE prion mad cow feed ban reaching livestock, or contamination and exposure there from, as well.



I would sure like to see the full reports of just these ;




4018 CHI-DO 3007091297 Rancho Cantera 2866 N Sunnyside Rd Kent IL 61044-9605 OPR FR, OF HP 11/26/2012 OAI Y



9367 3008575486 Rocky Ford Pet Foods 21693 Highway 50 East Rocky Ford CO 81067 OPR RE, TH HP 2/27/2013 OAI N



9446 DEN-DO 1713202 Weld County Bi Products, Inc. 1138 N 11th Ave Greeley CO 80631-9501 OPR RE, TH HP 10/12/2012 OAI N



9447 DEN-DO 3002857110 Weld County Bi-Products dba Fort Morgan Pet Foods 13553 County Road 19 Fort Morgan CO 80701-7506 OPR RE HP 12/7/2011 OAI N





see full list of the fda mad cow bse feed follies, toward the bottom, after a short brief update on the mad cow bse follies, and our good friend Lester Crawford that was at the FDA.



ALSO, I would kindly like to comment on this FDA BSE/Ruminant Feed Inspections Firms Inventory (excel format)4 format, for reporting these breaches of BSE TSE prion protocols, from the extensive mad cow feed ban warning letters the fda use to put out for each violations. simply put, this excel format sucks, and the FDA et al intentionally made it this difficult to follow the usda fda mad cow follies. this is an intentional format to make it as difficult as possible to follow these breaches of the mad cow TSE prion safety feed protocols. to have absolutely no chronological or numerical order, and to format such violations in a way that they are almost impossible to find, says a lot about just how far the FDA and our fine federal friends will go through to hide these continued violations of the BSE TSE prion mad cow feed ban, and any breaches of protocols there from. once again, the wolf guarding the henhouse $$$














Inspections conducted by State and FDA investigators are classified to reflect the compliance status at the time of the inspection, based upon whether objectionable conditions were documented. Based on the conditions found, inspection results are recorded in one of three classifications:


OAI (Official Action Indicated) when inspectors find significant objectionable conditions or practices and believe that regulatory sanctions are warranted to address the establishment’s lack of compliance with the regulation. An example of an OAI classification would be findings of manufacturing procedures insufficient to ensure that ruminant feed is not contaminated with prohibited material. Inspectors will promptly re-inspect facilities classified OAI after regulatory sanctions have been applied to determine whether the corrective actions are adequate to address the objectionable conditions.


VAI (Voluntary Action Indicated) when inspectors find objectionable conditions or practices that do not meet the threshold of regulatory significance, but warrant an advisory to inform the establishment that inspectors found conditions or practices that should be voluntarily corrected. VAI violations are typically technical violations of the 1997 BSE Feed Rule. These violations include minor recordkeeping lapses or conditions involving non-ruminant feeds.


NAI (No Action Indicated) when inspectors find no objectionable conditions or practices or, if they find objectionable conditions, those conditions are of a minor nature and do not justify further actions.








when sound science was bought off by junk science, in regards to the BSE TSE prion mad cow type disease, by the USDA, CFIA, WHO, OIE, et al. $$$


when the infamous, and fraudulently USDA, FSIS, APHIS, FDA, gold card was taken away that infamous day in December of 2003, all cards were off the table, it was time to change the science, and change they did. ...tss




snip. ...please see full text ;




Thursday, June 6, 2013













(Adopted by the International Committee of the OIE on 23 May 2006)


11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,







Thursday, May 30, 2013


World Organization for Animal Health (OIE) has upgraded the United States' risk classification for mad cow disease to "negligible" from "controlled", and risk further exposing the globe to the TSE prion mad cow type disease


U.S. gets top mad-cow rating from international group and risk further exposing the globe to the TSE prion mad cow type disease









Chronic Wasting Disease CWD, and other TSE prion disease, these TSE prions know no borders.



these TSE prions know no age restrictions.



The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit.



you cannot cook the TSE prion disease out of meat.



you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE.



Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well.



the TSE prion agent also survives Simulated Wastewater Treatment Processes.



IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades.



you can bury it and it will not go away.



The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area.



it’s not your ordinary pathogen you can just cook it out and be done with. that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent.



I go from state to state trying to warn of the CWD and other TSE prion disease in other species, I just made a promise to mom. back then, there was no information.



so, I submit this to you all in good faith, and hope that you take the time to read my research of the _sound_, peer review science, not the junk science that goes with the politics $$$



right or left or teaparty or independent, you cannot escape the TSE prion disease.



there is a lot of science here to digest, but better digesting this _sound_ science, instead of the junk political science you will hear from the shooting pen industry.



I don’t care what you eat, whom you eat, or what party you are affiliated with, my problem is, when you consume these TSE prions, and then go enter the medical, surgical, dental, blood and tissue arena, then you risk exposing _me or MY_ family to the TSE prion disease via friendly fire, the pass it forward mode of transmission mission, or what they call iatrogenic CJD. all iatrogenic CJD is, is sporadic CJD, until the route and source of the TSE prion agent is proven.



I am NOT anti-hunter, I am or was a hunter (disabled with neck injury and other medical problems), I am a meat eater.



I just don’t care for stupid, and sometimes you just can’t fix stupid, Lord knows I have tried.



I do NOT advertise on these blogs, they are there for educational use. ...




please see full test submission below ;




Sunday, June 09, 2013


Missouri House forms 13-member Interim Committee on the Cause and Spread of Chronic Wasting Disease CWD











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