Monday, June 30, 2008

Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease

Monday, June 30, 2008

Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease

Environmental Health 2008, 7:31 doi:10.1186/1476-069X-7-31

Ralph M Garruto (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Chris Reiber (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Marta P Alfonso (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Heidi Gastrich (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Kelsey Needham (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Sarah Sunderman (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Sarah Walker (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Jennifer Weeks (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Nicholas DeRosa (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Eric Faisst (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! John Dunn (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Kenneth Fanelli (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! Kenneth Shilkret (mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! ISSN 1476-069X Article type Research Submission date 17 March 2008 Acceptance date 24 June 2008 Publication date 24 June 2008 Article URL This peer-reviewed article was published immediately upon acceptance. It can be downloaded, printed and distributed freely for any purposes (see copyright notice below). Articles in Environmental Health are listed in PubMed and archived at PubMed Central. For information about publishing your research in Environmental Health or any BioMed Central journal, go to Environmental Health © 2008 Garruto et al., licensee BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. For information about other BioMed Central publications go to Environmental Health © 2008 Garruto et al., licensee BioMed Central Ltd. This is an open access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Risk behaviors in a rural community with a known point-source exposure to chronic wasting disease

Ralph M. Garruto1,2, Chris Reiber2, Marta P. Alfonso2 , Heidi Gastrich2, Kelsey Needham2, Sarah Sunderman2, Sarah Walker2, Jennifer Weeks2,3, Nicholas DeRosa4, Eric Faisst3,4, John Dunn4, Kenneth Fanelli4, Kenneth Shilkret4 1Laboratory of Biomedical Anthropology and Neurosciences, State University of New York at Binghamton, PO Box 6000, Binghamton, New York, 13902-6000 USA 2 Graduate Program in Biomedical Anthropology, State University of New York at Binghamton, PO Box 6000, Binghamton, New York, 13902-6000 USA 3 Madison County Health Department, Wampsville, New York, 13163 USA 4 Oneida County Health Department, Utica, New York, 13501 USA

Corresponding author: Ralph M. Garruto, PhD Graduate Program in Biomedical Anthropology State University of New York at Binghamton P.O. Box 6000 Binghamton, NY 13902-6000 Phone (607) 777-6562

Email addresses: RMG: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! CR: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! MPA: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! HG: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! KN: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! SS: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! SW: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! JW: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! ND: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! EF: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! JD: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! KF: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/! KS: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/!


Background The emergence and continuing spread of Chronic Wasting Disease (CWD) in cervids has now reached 14 U.S. states, two Canadian provinces, and South Korea, producing a potential for transmission of CWD prions to humans and other animals globally. In 2005, CWD spread for the first time from the Midwest to more densely populated regions of the East Coast. As a result, a large cohort of individuals attending a wild game feast in upstate New York were exposed to a deer that was subsequently confirmed positive for CWD.

Methods Eighty-one participants who ingested or otherwise were exposed to a deer with chronic wasting disease at a local New York State sportsman’s feast were recruited for this study. Participants were administered an exposure questionnaire and agreed to follow-up health evaluations longitudinally over the next six years.

Results Our results indicate two types of risks for those who attended the feast, a Feast Risk and a General Risk. The larger the number of risk factors, the greater the risk to human health if CWD is transmissible to humans. Long-term surveillance of feast participants exposed to CWD is ongoing.


The risk data from this study provide a relative scale for cumulative exposure to CWD-infected tissues and surfaces, and those in the upper tiers of cumulative risk may be most at risk if CWD is transmissible to humans.

Background Chronic Wasting Disease (CWD) was first observed in the United States in the late 1960s, though its origins are still unclear [1,2]. The term “chronic wasting disease” was first used in 1967 to describe clinical symptoms in captive Colorado mule deer [1,2]. In 1978, the disease was diagnosed as a spongiform encephalopathy [1,3]. By 1980, CWD had been described in captive elk and mule deer herds in both Colorado and Wyoming [1,2]. Subsequently in 1985, CWD was found in free-ranging elk in Wyoming, and recognized in free-ranging mule deer and whitetail deer in both states by 1990 [3]. CWD has since been described in 14 states across the US and in 2 provinces in Canada (Figure 1).

In April of 2002, the New York State Department of Environmental Conservation (NYSDEC) began a statewide surveillance program for CWD in whitetail deer [4]. The first positive samples (five in total) were confirmed in April 2005 from animals on two domestic deer farms in Oneida County, NY. The second deer farm, located very near to the first, received two deer that tested positive from the first deer farm. In response, the NYSDEC implemented an intensive mandatory surveillance of CWD, primarily in Oneida and Madison counties [4]. In order to monitor the wild deer herd, a CWD containment area was created encompassing approximately a 10 mile radius around the location of the CWD positive domestic deer farms in Oneida and Madison counties (Figure 1) [4]. During the initial phase of intensive monitoring (through April 30, 2005), 317 wild deer were collected and tested from this containment area as well as from the Town of Arietta in Hamilton County. Wild deer (two in total), in close proximity to the domestic deer farms, were found to be positive for CWD. During the second phase of the intensive monitoring program, all deer that died or were killed within this area were subject to mandatory testing

(Figure 2). Additionally, the NYSDEC expanded its testing program statewide [4], and now the annual testing of deer for CWD includes 800-1000 animals within the containment area (Figure 1) and approximately 5000 animals statewide [4]. No additional positive deer, wild or domestic, have been found since April 2005. Details of the distribution of the deer tested across New York State for CWD can be found on the DEC website [4].

Traditions of hunting deer, elk, moose, and other cervids, and management of domestic cervid preserves bring humans into contact with animals that could have CWD. Currently, it is unclear whether CWD prions can be naturally transmitted from infected cervids to humans or to noncervids. The question of cross-species transmission of CWD has been raised in the past [5-13]. In 2002 an unusual cluster of Creutzfeldt-Jakob Disease (CJD) – a human prion disease – developed in individuals who were avid lifelong Idaho deer hunters [14]; however, this and other reports investigating clusters of CJD have failed to establish a link with exposure to cervids through hunting or consuming the animals, or through other associated behaviors [12,14-18]. Although still unclear for CWD, epidemiological and laboratory findings have established an unequivocal link between Bovine Spongiform Encephalopathy (BSE) and vCJD [11,19-26].

On March 13, 2005, a local fire company in Oneida County, New York, hosted approximately 200-250 individuals at their annual Sportsmen’s Feast, during which local wild game, including venison (deer meat), was prepared, cooked in various ways, served, and consumed by individuals primarily from Oneida and neighboring counties. Shortly thereafter, laboratory tests indicated that one of the deer served was positive for CWD [27]. Since 2002, the New York State Department of Environmental Conservation has required the mandatory testing of deer for CWD

harvested from a domestic deer farm. However, there is no requirement that the meat not be consumed before the results are made available and thus reaction to the finding that the deer tested positive ranged from unconcerned to anger. Feast attendees were exposed to the contaminated meat through a variety of activities, including butchering and processing, cooking, consumption of venison, and/or through contact with contaminated surfaces. This incident represents the only known large scale point-source exposure of humans to an animal with confirmed CWD.

In response to this known point-source exposure, the Oneida County Health Department and the State University of New York at Binghamton (SUNY) proposed and launched the Oneida County Chronic Wasting Disease Surveillance Project, a cohort study designed to examine a natural experimental model of human exposure to CWD. The objective of the study are to determine the potential human health risks associated with exposure to CWD-contaminated cervids by following the events and health outcomes of attendees at the Sportsmen’s Feast. Study participants are being followed for a minimum period of six years from the time of exposure. This is based on the minimum incubation period and earliest age at onset for known human prion diseases including vCJD [11] and kuru [28-31]. The current report describes the initial results of a risk analysis based on behaviors associated with the wild game feast in upstate New York.

Methods The exposed cohort, whose names are held confidentially by the Oneida County Health Department, were contacted by mail about an informational meeting held in Oneida County in November, 2005. The project was approved by the Institutional Review Board at SUNY

Binghamton. Questionnaires and informational packets were distributed either by mail or in person to those who expressed an interest in participating in the surveillance project. From January to June 2006, SUNY Binghamton research assistants conducted additional follow-up mailings and telephone calls to those who indicated an interest in participating. The information from the project’s 81 recruited participants was entered into an Excel database, using standard double-entry, cross-check and proof-reading techniques to ensure accuracy. Statistical Package for the Social Sciences (SPSS) version 13.0 was used to analyze the data from the participant questionnaires.

The epidemiological questionnaire consisted of 54 questions, divided into four sections: demographic information, participation in feast activities, participation in general activities (including hunting and occupations), and current health information. The questionnaire was designed to obtain information to allow risk assessment at two levels: participation in feast activities termed Feast Risk, and other activities outside of the feast, termed General Risk.

Eleven variables were chosen as potential risk factors in the analysis of Feast Risk. These variables were selected based on known transmission routes from studies of other transmissible spongiform encephalopathies [11,19-26,28,32,33]. Feast Risk included eleven different activities that took place in preparation of, or at the Sportsmen’s Feast. They include: venison consumption, deer butchering, sustaining wounds while butchering, not wearing gloves while butchering, handling raw meat, eating raw meat, eating chops, eating tongue, cooking, not wearing gloves while cooking, and sustaining injuries while cooking.

In addition to the risk factors at the feast, seven behaviors were considered risk factors for analyzing General Risk. These included: hunting deer, hunting in the containment area, eating venison “often” (by subjective self-report), butchering deer, field dressing deer, removing antlers, and not wearing gloves while butchering.

Results General Characteristics of Study Participants The feast participant sample is composed of 86.4% males and 13.6% females (N=81). Based on self-report, the majority of the participants identified themselves as white (89.8%), with 1.3% identifying as Native Hawaiian, and 1.3% as Native American. Most participants attended the Feast (96.3%) and of those attending most were accompanied by friends or family (78.9%). A total of nine different counties of residence in New York and two outside the state were reported, but the majority live in two New York counties: 53 in Oneida County (65%), and 17 in Madison County (21%). Ages ranged from 10 to 82 years, with a mean of 48 years.

A total of 13.6% of participants reported being diabetic (Type II). Only 2.5% had been diagnosed previously with a neurological disease, but 19.8% reported a family history of neurological disease, with Alzheimer’s disease being the most common condition (13.6%).

Feast Risk Figure 3 shows the percentage of the 81 participants who sustained each individual risk factor at the Feast, with 87.7% of the attendees eating venison. Table 1 shows that most of the 81 participants had only one Feast Risk factor (69.1%), most commonly eating venison, while 9.9%

had none. Participants who reported venison consumption reported that they ate it prepared in different ways, most commonly as chili and steak (Figure 4). The only organ meat consumed was deer liver (1.3%). No other organ meat such as tongue, heart or kidney was reported to have been consumed.

Seventeen individuals (21%) had multiple Feast Risk factors, having both eaten venison and participated in some other way(s) in the butchering and/or preparation of the venison. Among participants who attended the feast, only one (1.3%) participated in field dressing deer served at the feast, and no one recalled field dressing the contaminated animal. Individuals involved in the preparation and processing of the deer reported they: butchered (n=8; 9.9%), handled raw meat (n=12; 15%), and cooked venison (n=8; 9.9%). No one reported sustaining a cut or any other injury while butchering, but only one of the eight who butchered wore gloves. However, two individuals stated that they had cuts on their hands at the time of the feast (2.5%), while 32.5% do not recall whether they did. Of the eight participants involved in cooking venison, one reported sustaining an injury while cooking, and one also indicated that they wore gloves while cooking.

General Risk Results regarding General Risk factors are presented in Figure 5. In relation to deer hunting practices, of the 81 participants, 69.1% hunted deer, and of those who hunted, 80.4% harvested a deer between the years 2000-2005. Of those who hunted, 25.0% reported hunting only in Oneida County. Furthermore, a total of 32.1% of the participants indicated that they hunted in the Oneida County CWD containment area, although not exclusively. Of those who hunted, 96.4%

field dressed, 75.5% removed antlers and 70.9% butchered harvested deer. However, only 26.4% of them wore gloves while butchering deer. Of those who hunted, 92.7% consumed the deer they killed, and 96.3% of all feast participants reported eating venison outside of the Sportsmen’s Feast. Of the 81 participants, 24.6% also reported eating venison from other states. In addition to hunting, the 81 participants reported general contact with animals, including deer (39.5%), cattle (13.6%), sheep (1.2%), alpacas (1.2%), and other animals (9.9%).

Discussion The purpose of this report is to describe the events surrounding the exposure of a large cohort of individuals to a CWD-infected animal, and present information on risk behaviors for activities at the feast and general hunting behaviors outside the feast. This study focuses on two levels of risk, that specific to activities at the feast, Feast Risk, and activities outside the feast, General Risk. Because CWD has not been definitively linked to the development of a prion disease in humans, the risk behaviors evaluated reflect those associated with known prion disease transmission routes [11,19-26,28-33].

Studies have shown that CWD can be experimentally transmitted to voles [13], non-human primates [7], cattle [5,8], and sheep [9]. Since transmissible spongiform encephalopathies have the ability to cross species barriers and are resistant to degradation [5-10,13,16,18,28,29,32-34], food chain transmission of prion diseases is a growing human and animal health concern. Infected prions are found in the blood, skeletal muscle, and saliva as well as the central nervous system of infected animals [5,10,33,35]. Contact with these tissues is a likely mode of transmission of CWD from animal to animal, and could potentially present a risk to humans. It is

currently considered unlikely that CWD can cross the species barrier to humans [12,14,18]. However, if it can, those with multiple risk factors may be most vulnerable. The information presented above provides a relative scale for cumulative exposures to CWD-infected tissues and surfaces.

Since CWD has spread from regions of the Midwest with generally low human population densities to high-density regions of the Eastern U.S. (New York and West Virginia), direct contact between infected cervids and humans has increased. Additionally, the potential for crossspecies transmission of CWD from deer to cattle to humans may be increased as a result of increasing contact between large herds of potentially infected cervids and cattle in pastures on smaller Eastern U.S. farms.

Conclusions The Oneida County CWD Surveillance Project introduced here, and the data reported in this paper, provides the first step in developing a natural experimental model of possible transmission of CWD prions from deer to humans [36,37] with a known point-source exposure. Surveillance of the cohort will continue for a minimum of six years (and likely extended) through annual follow-up questionnaires including self-report health information. Since human prion diseases are reportable in New York State, the Oneida County Health Department and health departments in other neighboring counties will be especially vigilant in this surveillance effort. This prospective cohort study will provide new information previously unavailable from retrospective studies where the CWD status of cervids was unknown and hunting behaviors only evaluated retrospectively many years after onset of cases of CJD.

Abbreviations Creutzfeldt-Jacob Disease (CJD), Chronic Wasting Disease (CWD), New York State Department of Environmental Conservation (NYSDEC), State University of New York (SUNY), Variant Creutzfeldt-Jacob Disease (vCJD)

Competing interests The authors declare that they have no competing interests.

Authors’ contributions RMG conceived, designed and coordinated the study and prepared the manuscript. CR coordinated the data analysis, analyzed the data and prepared the manuscript. JW coordinated the field research, and HG and SS followed-up with the participants and conducted the analysis. MA, KN, SS, and SW conducted follow-up of the participants, data analysis and preparation of the manuscript. ND, EF, JD, KF and KS coordinated the town hall meeting, distribution of recruitment letters, development and dissemination of the questionnaires to the participants, and preparation of the manuscript. All authors read and approved the final manuscript.

Acknowledgements This Project was approved by the Institutional Review Board of Binghamton University and is in compliance with regulations regarding human participants in biomedical research. The Project received initial seed funding from both the Research Foundation, Binghamton University and the Oneida County Health Department and is supported by the Oneida County Executive. We would

like to thank Steve Heerkens, wildlife biologist in charge of the New York State Department of Environmental Conservation’s CWD Surveillance Program in the Oneida County Containment area, for providing an overview of the program. We would also like to thank Abby Milberg of Binghamton University for her assistance with the manuscript graphics.



full text ;

greetings xxxx,

xxxx wrote ;

My history is my best friend and brother in law, the same person, died last year July 9th from CJD that is what the death certificate says. We gave permission to CJD foundation for autopsy but have not heard anything since. Our Neuologist in Colorado suggested that he could have consumed Elk from the rifle area of Colorado, which he did, and that vCJD may be jumping to humans, yet noone mentions this anywhere.What do you know?<<<>


IN my opinion, Gambetti et al at the CWRU CJD Foundation have their hands tied and bound by higher ups $$$

NOW, for my long-winded version, as follows ;

IN MY opinion, the reason you have not gotten an answer yet, the CJD Foundation INC., (CWRU), is backed by the CDC, and the NIH, these are the same groups that wanted to destroy our loved ones brain samples that we so painfully donated, this about the same time the atypical BSE mad cows were accidently being found in the USA. ONLY an act of congress documented the mad cow in TEXAS, and only an act of congress saved those brain tissue samples that had been collect since the 60s i believe, some primate samples older than that. thats another story i will try and find a link and post at bottom.

BUT, YOU ask about the CWD and potential link there, and there is some very disturbing data there as well. a recent study, now you have to realize, as far as a link to humans, most of these folks (excuse my slang), would not say shit if they had a mouth full of it. it's there job to keep any public hysteria from exploding, so they string the science out year after year, decade after decade, when by their own experimental findings in rodents and primates, they seem to ignore as any potential threat for humans, even with there so cold humanized mice, those 20,000 dollar each white mice. so here goes, please excuse any of my rants, and just read the science and the sources of the science, compared to the old 'no threat to humans' BSe. ...


8. Human susceptibility to CWD

Millions of North Americans hunt deer and elk (U.S. Department of the Interior, Census Bureau), and there is no doubt that people have been exposed to CWD through venison consumption, particularly in light of recent data showing CWD prions in muscle [2]. Human susceptibility to CWD or to other newly emerging animal TSE [9, 14] is still unclear, although we can be somewhat reassured in that there have been no large scale outbreaks of human TSE cases in Colorado and Wyoming, where CWD has existed for decades [51]. Up until approximately 10 years ago, autopsies were not performed on suspect human TSE cases in many states due to biosafety concerns, therefore the diagnosis of potential new TSE strains has been hampered. This indicates that clinical TSE diagnoses in humans were not confirmed, nor was any strain typing done to look for the appearance of potentially subtle or unusual pathological or biochemical phenotypes of a new TSE strain. Fortunately, the autopsy rate for suspect cases is improving. At the National Prion Disease Pathology Surveillance Center at Case Western Reserve University (Cleveland, Ohio), Creutzfeldt-Jakob disease (CJD) suspect cases are studied and classified by CJD subtype. Thus far,


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

however there have been no unusual or novel prion subtypes that might indicate the appearance of a new prion strain [7, 41]. Other indirect studies of human susceptibility to CWD also suggest that the risk is low. In biochemical conversion studies, Raymond et al. [68] showed that the efficiency of CWD to convert recombinant human PrP into amyloid fibrils was low, but similar to that of both BSE and scrapie fibrils to do the same. These results suggest that there is a molecular incompatibility in the conversion of human PrPC by CWD, sheep scrapie, or BSE, and that cross species infections in humans may be rare events. To determine whether common PrPSc strain features may link CWD and CJD, histopathology and the PrPSc biochemical characteristics from deer and elk were compared with that of humans with sporadic CJD (sCJD) cases that are methionine homozygous at codon 129 of the Prnp gene by Xie et al. [96], although strain features including histologic profile, target organs, and glycoform patterns will not necessarily remain the same upon crossing species barriers [6, 5, 8, 57]. The PrPSc form is cleaved by proteinase-K (PK) at different sites depending on the conformation of the protein and may aid determination of whether the PrPSc conformation is similar. By western blot (SDS-PAGE) of elk CWD, the unglycosylated PK-resistant PrPSc migrated at 21 kDa, similar to sCJD (MM1 subtype) and the PK cleavage site was the same, occurring at residues 78 and 82 as assessed by N-terminal sequencing. Conformational stability was evaluated by measuring the PrPSc stability under partially denaturing conditions and also showed no significant difference between elk CWD and sCJD MM1 PrPSc. However, elk CWD and human sCJD MM1 strains exhibited distinct glycoform patterns by two dimensional gel electrophoresis, suggesting that the strains differed. Future studies may utilize luminescent conjugated polymers, which were recently shown to distinguish naturally- and experimentally-derived prion strains [79]. To study elk-human prion species barriers, Kong et al. inoculated elk CWD into transgenic mice expressing either human PrP or elk PrP. Whereas the elk PrP expressing mice developed disease after only 118-142 days post-inoculation, human PrP expressing mice (129M) did not develop any features of TSE after more than 657 or more than 756 days [41]. In accordance with these results, Tamgüney et al. also reported that human PrP overexpressing mice were not susceptible to 9 CWD isolates from mule deer, white-tailed deer, and elk [84]. However, mice have a limited lifespan and further passages may be necessary to detect low levels of prion infectivity that may be present subclinically. Although indi rect evidence is accumulating that there may be a robust species barrier for CWD transmission to humans, one report indicates nonhuman primate susceptibility to CWD. Intracerebral inoculation of squirrel monkeys (Saimiri sciureus) demonstrated a positive CWD transmission [49]. Among non-human primates, however, the Prnp sequence of the new world monkeys are the most distant from humans [72], and therefore may not indicate that human prion conversion would occur by CWD.


11. Disease control challenges posed by CWD

Evidence is building that indicates efficient horizontal transmission occurs in CWD, indeed a complicating aspect in disease control [91]. Potential transmission mechanisms range from spread via direct contact among animals to environmental exposure through grazing in areas contaminated by prion-infected secretions, excretions (saliva, urine, feces), tissues (placenta), or decomposed carcasses. Recently, in a breakthrough finding, saliva from CWD infected deer was shown to transmit prion disease [50]. An additional experiment by Miller and colleagues showed that CWD-infected carcasses allowed to decay naturally in confined pastures can lead to CWD infections in captive deer, demonstrating the potential for environmental contamination to spread infection [55]. Modelling studies have provided further


support that environmental contamination is likely playing a significant role in transmitting CWD [56, 53]. Additionally, infectious prions have been demonstrated to bind soil particles and remain infectious to animals by both intracerebral and oral exposure routes [38, 37]. Prion infectivity has been recovered from soil more than two years after experimental exposure to prions, suggesting the soil may serve as a reservoir for CWD prions [75]. Taken together, these results indicate that there may even be multiple sources for CWD exposure, perhaps through direct contact and environmental routes. Significant challenges to CWD eradication exist in free-ranging cervids. Infected deer and elk range over a broad geographic region, and even previously surmised geographic barriers such as the Continental Divide have proven passable by infected animals. Ridding the environment of CWD-contaminated soil or even CWD-infected carcasses is not possible. Moreover, the available ante-mortem diagnostic tests for surveillance are laborious and impractical for large numbers of free-ranging animals [74, 88, 95]. Therefore for a wildlife manager, this disease is costly to survey and difficult to control.

12. Conclusion

***CWD in cervids is efficiently transmitted, likely more than any other TSE in animals or humans. Therefore, it is unlikely that this TSE can be eradicated, but perhaps through an improved understanding of transmission routes, biological factors influencing pathogenesis, and the molecular basis of CWD prion conversion, a targeted strategy for interrupting disease spread may be developed.


I thank Drs. Michael Miller, Jason Bartz and Mathias Heikenwalder for critical review of the manuscript.

snip...see full text 19 pages ;

Subject: Species barriers for chronic wasting disease by in vitro conversion of prion protein Date: November 3, 2007 at 10:57 am PST

Species barriers for chronic wasting disease by in vitro conversion of prion protein

Li Li a, Michael B. Coulthart b, Aru Balachandran c, Avi Chakrabartty d, Neil R. Cashman a,* a Brain Research Centre, Division of Neurology, Department of Medicine, University of British Columbia and Vancouver Coastal Health, UBC Hospital, 2211 Wesbrook Mall, Vancouver, BC, Canada V6T 2B5 b Prion Diseases Program, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg, Man., Canada R3E 3R2 Q1 c National Reference Laboratory for Scrapie and CWD, Animal Diseases Research Institute, Canadian Food Inspection Agency, 3851 Fallowfield Road, Nepean, Ont., Canada K2H 8P9 d University Health Network, Department of Medical Biophysics, University of Toronto, Toronto, Ont., Canada M5G 1L7 Received 6 October 2007


Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy that can affect North American cervids (deer, elk, and moose). Using a novel in vitro conversion system based on incubation of prions with normal brain homogenates, we now report that PrPCWD of elk can readily induce the conversion of normal cervid PrP (PrPC) molecules to a protease-resistant form, but is less efficient in converting the PrPC of other species, such as human, bovine, hamster, and mouse. However, when substrate brain homogenates are partially denatured by acidic conditions (pH 3.5), PrPCWD-induced conversion can be greatly enhanced in all species. Our results dem- onstrate that PrPC from cervids (including moose) can be efficiently converted to a protease-resistant form by incubation with elk CWD prions, presumably due to sequence and structural similarities between these species. Moreover, partial denaturation of substrate PrPC can apparently overcome the structural barriers between more distant species.


Although Syrian hamsters were initially deemed resistant to CWD [19], a recent publication demonstrates that CWD can be transmitted and adapted to hamster [20].


Substrate denaturation and human health

We confirm with multiple species that acid/GdnHCl- treated brain PrPC is a superior substrate for in vitro con- version than untreated PrPC, possibly by overcoming con- formational barriers in partial denaturation of substrate PrPC. PrP conversion in scrapie-infected neuroblastoma cells is believed to occur in endosomes, a low-pH and reducing environment [26]. The non-ruminant stomach possesses a low pH lumen, and PrPC is expressed in this organ [27]. Such acidic (denaturing) organ or cellular organellar environments might also promote CWD trans- mission to non-cervid species, including humans.


This work was supported by the Canadian Institutes of Health Research (Institute of Infection and Immunity, Safe Food and Water program) and PrioNet Canada.

[20] G.J. Raymond, L.D. Raymond, K.D. Meade-White, A.G. Hughson, C. Favara, D. Gardner, E.S. Williams, M.W. Miller, R.E. Race, B. Caughey, Transmission and adaptation of chronic wasting disease to hamsters and transgenic mice: evidence for strains, J. Virol. 81 (2007) 4305?4314.

2007 Elsevier Inc. All rights reserved.

Please cite this article in press as: L. Li et al., Species barriers for chronic wasting disease by in vitro..., Biochem. Biophys. Res. Commun. (2007), doi:10.1016/j.bbrc.2007.10.087

Transmission and adaptation of chronic wasting disease to hamsters and transgenic mice: evidence for strains

Gregory J. Raymond1, Lynne D. Raymond1, Kimberly D. Meade-White1, Andrew G. Hughson1, Cynthia Favara1, Donald Gardner2, Elizabeth S. Williams3§, Michael W. Miller4, Richard E. Race1*, and Byron Caughey1*

Running title: CWD transmission to rodent species

Laboratory of Persistent Viral Diseases1, and Rocky Mountain Veterinary Branch2, NIAID, NIH, Rocky Mountain Laboratories, Hamilton, MT 59840; Department of Veterinary Sciences, University of Wyoming, Laramie, WY 820703; Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526-20974. §deceased *corresponding authors: Byron Caughey, Rocky Mountain Labs, 903 S. 4th St, Hamilton, MT 59840, USA; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/!; Tel: (406) 363-9264; FAX: (406) 363-9286 Richard Race, Rocky Mountain Labs, 903 S. 4th St, Hamilton, MT 59840, USA; mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/!; Tel: (406) 363-9358; FAX: (406) 363-9286

In vitro screening using the cell-free prion protein conversion system indicated that certain rodents may be susceptible to chronic wasting disease (CWD). Therefore, CWD isolates from mule deer, white-tailed deer and elk were inoculated intracerebrally into various rodent species to assess their susceptibility and to develop new rodent models of CWD. The species inoculated were Syrian golden, Djungarian, Chinese, Siberian, and Armenian hamsters; transgenic mice expressing the Syrian golden hamster prion protein; and, RML Swiss and C57 BL10 wild-type mice. The transgenic mice and the Syrian golden, Chinese, Siberian and Armenian hamsters had limited susceptibility to certain of the CWD inocula as evidenced by incomplete attack rates and long incubation periods. With serial passages of CWD isolates in Syrian golden hamsters, incubation periods rapidly stabilized as isolates with either short (85-89 days) or long (408-544 days) mean incubation periods and distinct neuropathological patterns. In contrast, wild-type mouse strains and Djungarian hamsters were not susceptible to CWD. These results show that CWD can be transmitted and adapted to some species of rodents and suggest that the cervid-derived CWD inocula may have contained, or diverged into, at least two distinct transmissible spongiform encephalopathy strains.


Differences in PrP-res glycoform patterns analyzed from several CWD- affected deer and elk have also suggested that CWD in mule deer may be more heterogeneous than in elk (19). Curiously, however, this apparent strain difference was not manifested when the identical mule deer CWD inoculum was serially passaged through only one recipient species. Serial passage in Sg hamsters yielded only the fast isolate (Table 1 and Figure 3), while passage first through the Tg (haPrP) mice then into Sg hamsters yielded only the slow isolate (Table 2 and Figure 3). With this in mind, it is important to consider other possible explanations for these results. One possibility is that CWD might be able to undergo a stochastic change into a more rapid and aggressive strain in Sg hamsters, and that this happened to occur after the mule deer CWD inoculations. A similar emergence of both fast and slow strains has been observed upon inoculation of TME into Sg hamsters (5). These strains developed even when a clonal isolate of the TME inoculum was used, suggesting that they arose in the recipient Sg hamsters rather than in the mink source (1). Finally, although extensive precautions were taken, we cannot formally prove that inadvertent contamination of the mule deer CWD inoculum with hamster-derived 263K strain did not occur which potentially could yield short- incubation-period passages in Sg hamsters (Table 1). However, the incubation period observed with the CWD passages (85-89 d) were significantly longer than 263K incubation periods observed in our lab (70-75 d) and no mock-infected

controls became sick during their lifespan. Also, we saw no 263K-like infectivity develop in the highly susceptible Tg (haPrP) mice even though we used the identical primary inoculum for both recipient species. Interestingly, the similarity of the Sg hamster-adapted CWD fast isolate and 263K might be due to a common origin since there is circumstantial evidence that CWD arose from cervid exposure to sheep scrapie, which was also the origin of the 263K strain in hamsters (14). Furthermore, the Hyper strain derived from TME inoculations has 263K-like strain characteristics in Sg hamsters (5). Thus, it would appear that both CWD and TME transmissions into Sg hamsters can result in divergent fast and slow strains.


ALSO, i have a link somewhere about CJD surveillance of CWD hunters in Colorado that is new and ongoing, but again, these folks would not say anything about any public health threat now, from a disease that will not kill you for years, if not a decade later, but what they fail to miss, is that those that have been exposed, will go on and expose many more via the medical, dental, and surgical arena's i.e. FRIENDLY FIRE. ...

anyway, that cwd cjd study in colorado was at this url in a sub-url somewhere. my filing system is crude to say the least, but it's there somewhere ;

check out links and sub-strings at bottom of this thread, more links to studies there ;

hmmm, no luck there for me, it's there somewhere, so lets go to my google filing system. ah yes, here it is ;

Colorado Surveillance Program for Chronic Wasting Disease Transmission to Humans (TWO SUSPECT CASES)

Subject: Re: Colorado Surveillance Program for Chronic Wasting Disease Transmission to Humans (TWO SUSPECT CASES) From: "Terry S. Singeltary Sr." <[log in to unmask]> Reply-To: Sustainable Agriculture Network Discussion Group <[log in to unmask]> Date: Wed, 4 Apr 2007 16:22:22 -0500 Content-Type: multipart/alternative Parts/Attachments: text/plain (3340 lines) , text/html (2782 lines)

Content-Type: text/html

FURTHER into this case study, Colorado Surveillance Program for Chronic Wasting Disease Transmission to Humans (TWO SUSPECT CASES) a look at case 1 and case 2 ;


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 proteaseresistant 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.


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-Stra¨ussler-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 proteaseresistant prion protein by Western blot analysis. Genetic evaluation revealed the P102L mutation in the prion protein gene with methionine/valine heterozygosity at codon 129.


I can't understand how they can keep claiming 'low, or no occupational transmission of CJD' ??? when there have been many cases that should have raised awareness, and in some cases they did, only to be swept under the rug as the infamous sporadic CJD, or some other TSE other than the nvCJD of the ukbsenvcjd only theory. it's a blown theory no one will accept too. lets look at a few occupational cases. ...TSS

now, some things to ponder ;


1. Do neuritic plaques and tau-positive neurofibrillary tangles indicate definite AD? Aren't these also found in GSS? What about concurrent AD and TSE?

2. Are the NPDPSC results conclusive? Do WB results depend on the part of the brain sampled?

3. Doesn't it seem unlikely the woman would flat-out lie about working with CWD tissues? (I'm working on this locally.)

4. What about cross-contamination? The lab gets large numbers of scrapie-infected sheep and CWD-infected deer and elk. I assume the necropsy area is contaminated with TSEs.

Approved-By: tom Message-ID: Date: Mon, 19 Jul 1999 11:21:25 -0800 Reply-To: Bovine Spongiform Encephalopathy Sender: Bovine Spongiform Encephalopathy From: tom Subject: iatrogenic scrapie from sheep dura mater? Someone kindly sent me the full text of a very curious 1993 Lancet article. This is available from the Ovid service -- Lancet itself ironically does not offer an electronic version this far back.

An orthopaedic surgeon employed by the lyodura company [Braun Melsungen] extracted dura mater from sheep and human cadavers and came down with fast CJD 22 years later. The ratio of sheep to human dura mater he collected was150 sheep to 12 humans. Apparently the surgeon and the sheep were German. Scrapie has long been present in Germany but reported levels are very low, about a flock a year has to be destroyed. I am not aware of any high sensitivity tests or random screening every being used in Germany to assess the levels of preclinical animals.

This raises the question, what did the lyodura company do with so much dura mater from sheep? The market for specialty surgical products was overwelmingly in humans in 1968. The Lancet article only says it was for research -- but in what species? Perhaps dura mater gives an immune response across species after processing, ruling out its use in humans. But as far as I know, blood type or other genetic differences do not matter within humans, ie, there is no tissue matching with dura mater.

I always wondered how CJD could show up from a handful of human dura mater donations with sporadic CJD supposedly so rare -- on the other hand, there would be no surprise at all if a case of subclinical scrapie showed up in 150 sheep.

This raises the question, have dura mater recipients or the surgeon subsequently been strain-typed? This might give a very different outcome than other forms of iatrogenic CJD or simply co-classify with pituitary growth hormone if route of infection (injected, oral, hereditary, etc.) is more important than strain source.

Otherwise, iatrogenic scrapie (like cwdCJD) is somwhat unpredictable in its gel pattern (though strains of scrapie in other primate species might be re-examined). The original scrapie strain is not be identifiable directly because material was not likely retained. Strain-typing was not available at the time of the article -- but Collinge was one of the authors.

There is little doubt that scrapie could be transfered to humans by intracerebral injection (based on lack of species barrier in primates) and that processed pooled (human?) dura mater can carry sufficient infectivity to cause CJD. I am not aware of animal experiments that specifically used sheep dura mater as experimental dose source.



Transmission of Creutzfeldt-Jakob disease by handling of dura mater. The Lancet Volume 341(8837) January 9, 1993 pp 123-124 Weber, Thomas; Tumani, Hayrettin; Holdorff, Bernd; Collinge, John; Palmer, Mark; Kretzschmar, Hans A.; Felgenhauer, Klaus

Sir,- Creutzfeldt-Jakob disease (CJD) can be transmitted iatrogenically by human pituitary growth hormone, corneal transplants, and dura mater grafts (1). Possible accidental transmission has been reported in only four people-a neurosurgeon (2), a pathologist (3), and two laboratory technicians (4,5) . We have encountered an unusually rapid case of CJD probably acquired through handling of sheep and human dura mater. In May, 1992, a 55-year-old orthopaedic surgeon developed paraesthesia of the left arm. A few days later he had spatial disorientation, apraxia, and gait ataxia. In June he was admitted and a neurologist suspected CJD on the basis of the clinical signs, typical electroencephalogram (EEG) pattern, and history. An EEG in June revealed a typical pattern of periodic biphasic and triphasic sharp wave complexes. We saw the patient in July, 1992. He was awake and oriented, with dyscalculia, dysgraphia, disturbed vision, apraxia mainly of the left side, rigidity of wrists, spasticity of all muscles, myoclonus of the left arm, increased tendon reflexes, ataxia of limbs and trunk, and incoordination of left arm. Within 3 weeks he had impaired consciousness and attention, mildly impaired memory, and threatening visual hallucinations with restless turning. He had periodic states with movements of his head and eye-bulbs resembling tonic adversive seizures. During sleep these motor disturbances stopped. 2 1/2 months later the patient died.

This patient had worked with sheep and human dura mater from 1968 to 1972. He handled about 150 specimens of ovine origin and at least a dozen human preparations for research. Handling involved opening skulls with a band saw, removing dura, and testing them either fresh (usually), preserved, or lyophilised for mechanical qualities. These specimens were sent to a company that has sold dura mater preparations by which CJD was transmitted in six instances. No information was available from the company about a possible connection with this patient's disease and the earlier cases of transmitted CJD. Brain biopsy was consistent with diagnosis of CJD. Cerebrospinal fluid obtained in July showed neuron-specific enolase (NSE) at 82.0 ng/mL, compared with 16.7 ng/mL in serum of other cases (6). Proton magnetic resonance spectroscopy of parieto-occipital and temporal grey matter, parietal white matter, and thalamus revealed a 20-30% reduction of N-acetylaspartate, as described (7). DNA was genotyped with allele-specific oligonucleotides (8) and was homozygous for methionine at the polymorphic codon 129. Subsequent direct DNA sequencing for the PrP gene open-reading frame demonstrated normal sequence on both alleles, excluding known or novel pathogenic PrP mutations.

It is tempting to speculate that prions were transmitted to this patient from sheep or human dura mater through small lacerations of his skin, but the patient and his wife did not remember any significant injury during his four years of working with these samples. It cannot be excluded that this was a case of sporadic CJD although this assumption is unlikely in view of the clinical course which was similar to iatrogenic CJD transmitted by peripheral inoculation, such as with human pituitary growth hormone or gonadotropin or to kuru (1). Iatrogenic cases resulting from intracerebral inoculation with the transmissible agent, for instance following dura mater grafts (2-5), present with a dementing picture, as is usual in sporadic CJD, rather than with ataxia as in this case.

1. Brown P, Preece MA, Will RG. "Friendly fire" in medicine: hormones, homografts, and Creutzfeldt-Jakob disease. Lancet 1992; 340: 24-27. [Medline Link] [Context Link]

2. Schoene WC, Masters CL, Gibbs CJ Jr, et al. Transmissible spongiform encephalopathy (Creutzfeldt-Jakob Disease): atypical clinical and pathological findings. Arch Neurol 1981; 38: 473-77. [Medline Link] [Context Link]

3. Gorman DG, Benson DF, Vogel DG, Vinters HV. Creutzfeldt-Jakob disease in a pathologist. Neurology 1992; 42: 463. [Medline Link] [Context Link]

4. Miller DC. Creutzfeldt-Jakob disease in histopathology technicians. N Engl J Med 1988; 318: 853-54. [Medline Link] [Context Link]

5. Sitwell L, Lach B, Atack E, Atack D, Izukawa D. Creutzfeldt-Jakob disease in histopathology technicians. N Engl J Med 1988; 318: 854. [Medline Link] [Context Link]

6. Wakayama Y, Shibuya S, Kawase J, Sagawa F, Hashizume Y. High neuron-specific enolase level of cerebrospinal fluid in the early stage of Creutzfeldt-Jakob disease. Klin Wochenschr 1987; 65: 798-801. [Medline Link] [Context Link]

7. Bruhn H, Weber T, Thorwirth V, Frahm J. In-vivo monitoring of neuronal loss in Creutzfeldt-Jakob disease by proton magnetic resonance spectroscopy. Lancet 1991; 337: 1610-11. [Medline Link] [Context Link]

8. Collinge J, Palmer MS, Dryden AJ. Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease. Lancet 1991; 337: 1444-42. [Medlin


Dormont*, and Jean-Philippe Deslys* et al, that The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate;

NOT to forget all the farmers, and one wife of a farmer of a BSE herd, that contracted CJD i.e. sporadic. ...tss

----- Original Message ----- From: "Terry S. Singeltary Sr." <[log in to unmask]">[log in to unmask]> To: <[log in to unmask]">[log in to unmask]> Sent: Thursday, March 29, 2007 11:21 AM Subject: ANOTHER FARMER DIES OF MAD COW DISEASE i.e. sporadic CJD

Subject: ANOTHER FARMER DIES OF MAD COW DISEASE i.e. sporadic CJD Date: March 29, 2007 at 8:00 am PST


March 29, 2007 02:15am

A YORKE Peninsula man who had worked as a farmer has died from a rare illness linked with mad cow disease.

The man, aged in his late 50s, died in the Royal Adelaide Hospital on Sunday, after becoming ill in late January. Communicable Diseases branch director Dr Ann Koehler said an autopsy had confirmed the man died from Creutzfeldt-Jakob Disease, a variant of which is called mad cow disease in other countries.

The death was the first in the state attributed to the disease for at least two years.,00.html

----- Original Message ----- From: XXXXXXXX XXXXXXXXXXXX(SEAC) To: [log in to unmask]">[log in to unmask] Sent: Friday, February 23, 2007 8:13 AM Subject: RE: SEAC ANNUAL REPORT 2006

Dear Mr Singeltary

Thank you for your email about a possible relationship between BASE and sCJD.

SEAC is continually informed about and considers the emerging science on both animal and human TSEs. The committee regularly receives updates from the UK's National CJD Surveillance Centre Unit on the epidemiology of vCJD and sCJD and is also aware of emerging research on BASE. It is envisaged that SEAC will conduct a detailed consideration of the available science and the possible animal and human health implications of BASE at its meeting on 10th May 2007.

Yours sincerely


---------------------------------------------------------------------------- ---- From: Terry S. Singeltary Sr. [mailto:[log in to unmask]] Sent: 02 February 2007 16:44 To: Bovine Spongiform Encephalopathy Cc: SEACsecretariat (AHEG); [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask]; Sustainable Agriculture Network Discussion Group Subject: SEAC ANNUAL REPORT 2006

Date: February 2, 2007 at 8:25 am PST SEAC ANNUAL REPORT 2006 (ignoring the obvious)

i guess the sporadic CJD cases have all be resolved, and the route and cause classified. to this day, i cannot figure this out. if BSE farmers died from sporadic CJD, and now another study seems to point that BSE and BASE may be the same thing, and that BASE does not produce vCJD like pathology, but pathology resembling that of sporadic CJD, then why does SEAC still seem to put sporadic CJD on the back burners ??? IN fact, SEAC could not even speak about it in there 2006 report. would it not seem prudent to mention these findings in this 2006 SEAC report ;

Other work presented suggested that BSE and bovine amyloidotic spongiform encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A MUTATION FOUND IN CASES OF SPORADIC CJD.


***These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.***

6:30 Close of Day One

64. A member noted that at the recent Neuroprion meeting, a study was presented showing that in transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE.

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

genotype can lead to an alternate phenotype that is indistinguishable

from type 2 PrPSc, the commonest _sporadic_ CJD;

I STILL think to ignore these findings below is not only rediculous, but indeed very suspicious ;


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?

4th farmer, and 1st teenager

2. snip...

Over a 5 year period, which is the time period on which the advice from Professor Smith and Dr. Gore was based, and assuming a population of 120,000 dairy farm workers, and an annual incidence of 1 per million cases of CJD in the general population, a DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN an individual in the general population to develop CJD. Using the actual current annual incidence of CJD in the UK of 0.7 per million, this figure becomes 7.5 TIMES.

3. You will recall that the advice provided by Professor Smith in 1993 and by Dr. Gore this month used the sub-population of dairy farm workers who had had a case of BSE on their farms - 63,000, which is approximately half the number of dairy farm workers - as a denominator. If the above sums are repeated using this denominator population, taking an annual incidence in the general population of 1 per million the observed rate in this sub-population is 10 TIMES, and taking an annual incidence of 0.7 per million, IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than that in the general population...


in the USA, a 16 year old in 1978;


(20 year old died from sCJD in USA in 1980 and a 16 year old in 1981. see second url below)

in France, a 19 year old in 1982;

in Canada, a 14 year old of UK origin in 1988;

in Poland, cases in people aged 19, 23, and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;

Creutzfeldt's first patient in 1923 was aged 23.

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....

FOR SEAC TO continue to flounder with this ukbsenvcjd only theory, when we know that sporadic CJD's were proven long ago to transmit via the medical and surgical arena, will only continue to spread this agent around the globe via a multitude of proven routes and sources.

IN my opinion, for SEAC to continue to go down this same road, year after year, and continue to ignore sporadic CJD's, and continue to say that "BSE in the UK sheep population is most likely to be zero, or very low if present at all." "Consequently, any impact of the schemes on human health from removing BSE from sheep is likely to be negligible." when in fact they do not know. and in fact science is showing that in "transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE." IN my opinion this continued neglect of other TSEs in human and animals, the continued belief in this ukbsenvcjd only theory, and the risk thereof, is reckless, dangerous, and in my mind, criminal. SEAC has failed the public terribly in this regard. ...TSS

----- Original Message ----- From: "Terry S. Singeltary Sr." To: Sent: Friday, February 02, 2007 10:43 AM Subject: SEAC ANNUAL REPORT 2006

Date: February 2, 2007 at 8:25 am PST SEAC ANNUAL REPORT 2006 (ignoring the obvious)

i guess the sporadic CJD cases have all be resolved, and the route and cause classified. to this day, i cannot figure this out. if BSE farmers died from sporadic CJD, and now another study seems to point that BSE and BASE may be the same thing, and that BASE does not produce vCJD like pathology, but pathology resembling that of sporadic CJD, then why does SEAC still seem to put sporadic CJD on the back burners ??? IN fact, SEAC could not even speak about it in there 2006 report. would it not seem prudent to mention these findings in this 2006 SEAC report ; ........SNIP.........END............TSS

-------- Original Message -------- Subject: ANOTHER FARMER KILLED BY CJD !!! Date: Wed, 23 Apr 2003 10:45:34 -0500 From: "Terry S. Singeltary Sr." To: Bovine Spongiform Encephalopathy CC: CJDvoice , bloodcjd


10:30 - 22 April 2003

A father-of-two from Lincolnshire has died from suspected Variant Creutzfeldt- Jakob Disease.

Farmer David Fussey (34), who owned Manor Farm, Middle Rasen, near Market Rasen, was diagnosed with the condition in October 2002.

He died last Tuesday with his wife Jess (28) at his side. He left two children Tom (four) and Laura (two).

Today his wife paid tribute to her husband, who spent the last seven months being cared for at Blenheim House Nursing Home, in Hemswell Cliff, near Market Rasen.

She said: "David was a strong man who was dedicated to his family.

"He was very proud to be a farmer and was keen to work with the land and protect the environment.

"He was also at the heart of the community and represented the village football and dart teams."

Doctors do not know how Mr Fussey contracted the disease.

Mrs Fussey said she first noticed a change in her husband's behaviour in December 2001.

"It was Christmas and usually David would be out having a few drinks but instead he was withdrawn and tired," she said.

"He had recently started a second job building barns to supplement our main income as arable farmers, so I just thought he was worn out."

Mr Fussey's condition continued to worsen and by July 2002 Mrs Fussey started to notice signs that something was seriously wrong.

She said her husband had started to have hallucinations.

"One day he said he had spoken to staff who had not worked with us for years," she said.

"Another time he was looking for a tractor we had sold some time ago."

Mr Fussey was admitted to Lincoln County Hospital on September 23. In October the doctors told Mrs Fussey the news she had feared.

She said: "I was deeply upset and thought this shouldn't be happening to me and my family.

"But in a strange way it was comforting because I had the answer to why my husband had deteriorated."

Mrs Fussey said she was not looking for anybody to blame.

"It is hard to know how to feel," she said. "I think what has happened is cruel and unfair but the disease does not discriminate.

"Nobody knows exactly how the condition is caught so until someone tells me the definitive answer I am not prepared to be bitter."

Variant CJD was first reported internationally in 1996 and since then 127 people in Britain have died from the disease. Currently there are seven British people thought to be infected.

The UK Creutzfeldt-Jakob Disease Surveillance Unit at the University of Edinburgh is researching the illness.

The unit's Professor James Ironside said: "Variant CJD is thought to be contracted by the same agent that causes BSE in cattle.

"The most likely way humans would come into contact with the agent is through the food chain. Fortunately the disease is very rare."

Mr Fussey's funeral will be held at 1pm on Friday at the St Peter and St Paul Church, in North Street, Middle Rasen. Mr Fussey will be taken to his funeral in a coffin on a trailer pulled by his favourite green John Deere tractor.

La Medicina del Lavoro Med Lav 2003; 94,4:353-363

Occupational risk factors for the sporadic form of Creutzfeldt-Jakob disease


Dipardmento di Sanita Pubblica, Sezione di Medicina del Lavoro, Universita di Cagliari * Isdtuto di Medicina del Lavoro, Facolta di Medicina e Chirurgia A. Gemelli, Universita Cattolica del Sacro Cuore, Roma


All the observed cases were livestock farmers, four of whom (0.6 expected) occurred in farms where BSE cases had been reported. No cases were observed among veterinarians (0.03 expected), or butchers. (0.15 expected). A matched case-control study of 206 sCJD cases and controls did not iden- tify any association with a priori suspected occupa- tions (38). However, 21 cases (10%) e 14 controls (7%) occurred in subjects with occupational contact with animal products (p=0.17). The authors did not explore this finding in detail. We conducted a crude calculation based on the published data, and we found instead an Odds Ratio of 1.6 (95% confi- dence interval 1.18,2.15).



The 24 US states death certificates database we used consists of several million coded death certfi- cates from 24 US states, covering the years 1984- 95. The 24 states are: Colorado, Georgia, Idaho (from 1988), Indiana (from 1986), Kansas, Ken- tucky, Missouri (in 1984-86), Maine, Nebraska (in



1984-85), Nevada, New Hampshire, New Jersey (from 1988), New Mexico (from 1986), North Carolina (from 1987), Ohio (from 1985), Okla- homa, Rhode Island, South Carolina, Tennessee (in 1985-88), Utah (from 1985), Vermont, West Virginia (from 1988), Washington (from 1989), and Wisconsin. In addition to standardized coding procedures, information on usual occupation and kind of business or industry, reported in the death certificate for each decedent, was included in the database provided to the National Center for Health Statistics (NCHS) (7). The information on occupation and industry was coded according to the 1980 US Bureau of the Census classification (6). The underlying cause of death was coded ac- cording to the International Classification of Dis- eases - 9th revision. No further details, such as du- ration of employment or concurrent diseases, are available from this database.



In this death certificate based case-control study, we observed a statistically significant association of CJD with work as a butcher and with employment in the office of a physician, occupation and indus- try categories for which previous literature reports suggested potential exposure to a TSE agent. However, other occupations and industries for which the same hypothesis was raised, such as work on a livestock farm, were unassociated with CJD. The positive associations with the occupation


of butcher and employment in physician's offices cannot be conclusively interpreted because of the small numbers and lack of information on the type and extent of exposure to potentially infectious material. Also, other generally smaller studies of CJD in Europe have not found specific associa- tions with livestock farming or other specific occu- pations, including health care workers (1,42).


An important limitation in our study is that it was based on the one occupation and industry combination on the death certificate of study sub- jects, and no further details, such as duration of employment, were available. ...snip...end

THE LAST PARAGRAPH SAYS IT ALL, just imagine what would happen IF CJD was reportable Nationally and Internationally, can you just imagine what they would find. maybe thats why it is not reportable nationally and internationally ;


MARCH 26, 2003

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

disease in the United States

Email Terry S. Singeltary:

[log in to unmask]">[log in to unmask]

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

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

IN the reply from Dr. Maddox to Terry S. Singeltary Sr., he states;


If BSE causes a sporadic CJD-like illness in humans, an increase in sporadic CJD cases would be expected to first occur in the United Kingdom, where the vast majority of vCJD cases have been reported. In the United Kingdom during 1997 through 2002, however, the overall average annual mortality rate for sporadic CJD was not elevated; it was about 1 case per million population per year. In addition, during this most recent 6-year period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of UK sporadic CJD deaths.[3, 5] Furthermore, surveillance in the UK has shown no increase in the proportion of sporadic CJD cases that are homozygous for methionine (Will RG, National CJD Surveillance Unit, United Kingdom, 2003; personal communication)...


THERE seems to be some difference of opinion;

Mouse model sheds new light on human prion disease


Professor John Collinge said “We are not saying that all or even most cases of sporadic CJD are as a result of BSE exposure, but some more recent cases may be – the incidence of sporadic CJD has shown an upward trend in the UK over the last decade. While most of this apparent increase may be because doctors are now more aware of CJD and better at diagnosing it, serious consideration should be given to a proportion of this rise being BSE-related. Switzerland, which has had a substantial BSE epidemic, has noted a sharp recent increase in sporadic CJD.

snip... news_archive/public-news-archive_nov_dec_02/public- bse_and_sporadic_cjd.htm

ALSO, Dr. Maddox states;

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

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



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


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


DR. Maddox states here;

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

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

CJD screening may miss thousands of cases

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


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


snip...CONTINUED...TSS Full Edit Quick Edit


View Member Profile Add as Friend Send Message Find Member's Topics Find Member's Posts Today, 08:58 AM Post #3

Group: Members Posts: 73 Joined: 31-March 08 From: Texas Member No.: 2,521

ALSO in Philip Yams book 'The Pathological Protein';

''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''....

THERE has been a _documented_ case of nv/v CJD in a 74 year old, so the errors Schonberger speaks of (above) would be of significant importance, if one believes in the nv/v CJD 'only' theory.

NOW we have _documented_ cases of very young CJD victims in the USA continuing to appear in several different states.

HOW does Dr. Maddox explain this, and does he still believe that a National CJD surveillance program with a CJD questionnaire to every victims family is still not warranted?

WITH CWD and Scrapie running rampant in the USA, with BSE now _documented_ in North America, with the feeding of ruminant-to-ruminant animal protein still happening in the USA in 2003 even though there has been a partial voluntary ban on ruminant feeding since 8/4/97, with only 48,000 BSE/TSE tests done on USA cattle in some 14 years of surveillance, when in any given year there are 100 million cattle in the USA, with all this, i think refusing to make CJD/TSEs reportable Nationally in the USA is not ONLY a grave mistake, but in my opinion, should be looked at with great suspicion...

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

The clinical phenotype of patients with dura mater-associated CJD is similar to that seen in the classical sporadic form of CJD: rapidly progressive dementia, myoclonus, and in the majority of patients a characteristic EEG. However, there are some differences: dura-associated cases tend to have more prominent early cerebellar symptoms and a somewhat more prolonged clinical course. In sporadic CJD the median illness duration is 4 1/2 months and this is doubled on average in dura mater cases. The age at onset is about 10-15 years younger on average than we see with sporadic CJD.

I think the youngest dura mater case documented was 16 or 17. The average incubation period from the time the patient received their graft to onset of their illness is approximately 6 years, but ranges from 16 months to 16 years. Although most of these cases have arisen through the use of dura mater for cranial surgery, there are some cases which have been known to have resulted from ear, nose and throat surgery or from spinal surgery. Two cases from France, as mentioned, followed embolism procedures, in one case the patient had a nasopharyngeal tumor and dura mater was cut up into lots of little pieces and injected into the external carotid artery to embolise this, and in the other case the dura mater was inoculated into an artery in the chest to embolise an area of infection.

I would like to just go back and show you the countries which are known to have had dura mater cases of CJD. Most of the reports come from Japan, and we were rather surprised at the WHO consultation last year in Geneva, to hear reports from Dr Tateishi of a recent study conducted in Japan which had shown the presence of 43 cases of dura mater CJD.

I think you will agree looking at the slide that the other cases have been reported really quite widely throughout the world. Virtually all of these patients received one particular form of dura mater graft that was commercially manufactured by a single German company. The product was called Lyodura, and most of the patients had received grafts that had been manufactured during a 4 year period between 1983 and 1987. Lyodura was pooled during this time, so there was a potential for cross contamination and the sterilization procedures used involved 10% hydrogen peroxide for 24 hours and ionizing radiation. Subsequent animal experiments have shown that this is not an adequate form of sterilization.

An important question is whether any of the dura mater cases were recipients of grafts that were treated with more thorough and adequate sterilisation. By this, I mean treatment with 1N sodium hydroxide, which is in the standard step which was introduced in the treatment of Lyodura after 1987. There are four cases out of this series of 83 which perhaps I'll talk about in a little bit more detail. Two cases were clearly not Lyodura. These were locally procured grafts, one from Italy and the other from the United Kingdom - these were used between 1969 and 1981.

Furthermore, there was the a recently reported case from America which we heard about yesterday. Perhaps the case of most interest is one from the Japanese series. This was a lady in her mid-60s who received a graft in 1991 and later developed clinically probable CJD, but this was not histologically confirmed. The hospital records did not note whether or not her graft was Lyodura or the other form of dura used in that hospital at that time. It was concluded in the report of the Japanese cases that it was unlikely that this patient had received Lyodura produced before 1987.

So the possibility exists that this patient had received a form of dura which was considered to be adequately sterilised. It is important to note two points, first, as this case did not undergo histological examination the diagnosis of CJD is not 100% certain, and second, we can not be absolutely sure that in this or some of the of other 82 cases that the history of receiving a dural mater graft is coincidental. In none of these cases is there data which tells if the graft donor had CJD.

Following the announcement of the first case here in the United States, doctors in the United Kingdom, Australia and New Zealand decided that they were going to use alternatives to cadaveric dura homografts, and here in the States I believe there was an importation ban on Lyodura.

So what alternatives are there to cadaveric-derived dura? There are several - I'll just run through these. One of the most popular is fascia lata, this is the fibrous covering of the lateral thigh muscle. The removal of this can add about 30 minutes to the length of the operation and of course leaves a wound which, as with all wounds, can potentially become infected or have other complications.

Other alternatives include pericranium (the covering of the skull bone), temporalis fascia (the membrane which surrounds the temporalis muscle at the side of the head) and synthetic materials - a number of such materials have been tried over the years including gold foils, cellophane, and dacron grafts. However, there has been some concern about the safety of synthetic materials and neurosurgeons have felt that these were rather inferior, although I think with the newer materials that's not so clearly the case. I should note that there is no controlled trial that has ever been conducted to answer the question as to whether or not these substitutes are better or worse than cadaveric-derived dura.

I think there are two key questions that need to be addressed, first, are there situations where cadaveric dura is better than available alternatives? If the answer is no then we need to question why we are using cadaveric dural grafts at all. If the answer is yes, then the next question is how can dura be made as safe as possible? I'd like to show you some of the report from the WHO meeting over a year ago. I'll read it to you.

"Because over 50 cases of CJD have resulted from cadaveric dura mater grafts, the group strongly recommended that dura mater no longer be used, especially in the case of neurosurgery, unless no alternative is available. If dura mater is to be used, only material which is from non-pooled sources originating from carefully screened donors subjected to validated inactivated treatment should be considered."

Following this recommendation the Japanese authorities decided that they were no longer going to issue a license for the use of dura mater and the TSE Advisory Committee here in the States met again to discuss the issue of dura mater. I just want to run through their recommendations, there were some differences from WHO's: although they also discouraged use of dura mater, the final decision on its usage was left up to the individual physicians, but certain additional safeguards were put into place.

For instance, it was felt mandatory that for every donor a full brain autopsy should be performed and examined histologically and with immunocytochemistry, which is probably the most sensitive method that we have, other than transmission studies. It was further recommended that a sample of the dura and the brain should be kept for further testing as needed.

Additionally, standard protocols for determining donors eligibility and tissue procurement were recommended, and dura should be collected before the brain at autopsy - which obviously makes sense to avoid contamination of the graft. Furthermore, decontamination with 1N sodium hydroxide for one hour should be used. This had previously been confirmed by Paul Brown and colleagues to be an effective decontamination procedure. There should be no pooling of grafts, to prevent cross-contamination and there should be documentation to allow tracking from the donor to the recipient and from the recipients to the donor. I think there can be little doubt that if these recommendations are adopted, then the safety of dura mater grafts will be dramatically improved.

However, I would like to just play the devil's advocate here and to mention a few cautions. We know from animal experiments that infectivity can predate any pathological changes and this includes immunocytological changes as well. We also know that standard decontamination procedures using sodium hydroxide, as David Taylor mentioned yesterday, may not completely be effective. I think we have to remember that dura is a potentially high-risk material, and that studies also performed by David Taylor have shown that dura mater can have 106 ID50 per gram. Perhaps through the use of current decontamination procedures we will produce grafts which are much safer than those previously used, with but with low-level residual infectivity which may lead to disease with a potentially long incubation period. (For TSE agents it is known that dose administered is inversely proportional to incubation period)...

Subject: Re: Transmission and adaptation of chronic wasting disease to hamsters and transgenic mice: evidence for strains Date: April 1, 2007 at 7:30 pm PST

Nonetheless, the rodent-adapted CWD models we have developed may be useful to experimentally analyze TSE species and strain differences. Despite the low initial attack rate on the first passage of CWD into Sg hamsters, CWD derived initially from elk and mule deer readily adapted to hamsters as evidenced by the 100% infection rate on second and third passages. The average incubation periods were similar for the second and third passages, but considerably shorter than the first passage in the Sg hamsters, suggesting that any species barrier to infection (formally, the shortening of incubation period between the first and subsequent passages in a new species) was overcome quickly.<<<>

---------------------------------------------------------------------------- ----

Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions.



The lack of evidence of a link between CWD transmission and unusual cases of CJD, despite several epidemiologic investigations, and the absence of an increase in CJD incidence in Colorado and Wyoming suggest that the risk, if any, of transmission of CWD to humans is low. Although the in vitro studies indicating inefficient conversion of human prion protein by CWD-associated prions raise the possibility of low-level transmission of CWD to humans, no human cases of prion disease with strong evidence of a link with CWD have been identified. However, the transmission of BSE to humans and the resulting vCJD indicate that, provided sufficient exposure, the species barrier may not completely protect humans from animal prion diseases. Because CWD has occurred in a limited geographic area for decades, an adequate number of people may not have been exposed to the CWD agent to result in a clinically recognizable human disease. The level and frequency of human exposure to the CWD agent may increase with the spread of CWD in the United States. Because the number of studies seeking evidence for CWD transmission to humans is limited, more epidemiologic and laboratory studies should be conducted to monitor the possibility of such transmissions. Studies involving transgenic mice expressing human and cervid prion protein are in progress to further assess the potential for the CWD agent to cause human disease. Epidemiologic studies have also been initiated to identify human cases of prion disease among persons with an increased risk for exposure to potentially CWD-infected deer or elk meat (47). If such cases are identified, laboratory data showing similarities of the etiologic agent to that of the CWD agent would strengthen the conclusion for a causal link. Surveillance for human prion diseases, particularly in areas where CWD has been detected, remains important to effectively monitor the possible transmission of CWD to humans. Because of the long incubation period associated with prion diseases, convincing negative results from epidemiologic and experimental laboratory studies would likely require years of follow-up. In the meantime, to minimize the risk for exposure to the CWD agent, hunters should consult with their state wildlife agencies to identify areas where CWD occurs and continue to follow advice provided by public health and wildlife agencies. Hunters should avoid eating meat from deer and elk that look sick or test positive for CWD. They should wear gloves when field-dressing carcasses, bone-out the meat from the animal, and minimize handling of brain and spinal cord tissues. As a precaution, hunters should avoid eating deer and elk tissues known to harbor the CWD agent (e.g., brain, spinal cord, eyes, spleen, tonsils, lymph nodes) from areas where CWD has been identified.

From: TSS ( Subject: CWD aka MAD DEER/ELK TO HUMANS ??? Date: September 30, 2002 at 7:06 am PST

From: "Belay, Ermias" To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" Sent: Monday, September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

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----- From: Sent: Sunday, September 29, 2002 10:15 AM To: [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask]; [log in to unmask]">[log in to unmask] Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

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


A. Aguzzi - Chronic Wasting Disease (CWD) also needs to be addressed. Most serious because of rapid horizontal spread and higher prevalence than BSE in UK, up to 15% in some populations. Also may be a risk to humans - evidence that it is not dangerous to humans is thin.

JOURNAL OURNAL OF VIROLOGY IROLOGY, Nov. 2005, p. 13794–13796 Vol. 79, No. 21 0022-538X/05/$08.00 !0 doi:10.1128/JVI.79.21.13794–13796.2005 Copyright © 2005, American Society for Microbiology. All Rights Reserved.


Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel Monkeys (Saimiri sciureus sciureus)

Richard F. Marsh, 1† Anthony nthony E. Kincaid, 2 Richard A. Bessen, 3 and Jason C. Bartz Bartz4* Department of Animal Health and Biomedical Sciences, University of Wisconsin, Madison 53706 537061; Department of Physical Therapy Therapy2 and Department of Medical Microbiology and Immunology, 4 Creighton University, Omaha, Nebraska 68178; and Department of Veterinary Molecular Biology, Montana State University, Bozeman, Montana 59718 597183 Received 3 May 2005/Accepted 10 August 2005

Chronic wasting disease (CWD) is an emerging prion disease of deer and elk. The risk of CWD transmission to humans following exposure to CWD-infected tissues is unknown. To assess the susceptibility of nonhuman primates to CWD, two squirrel monkeys were inoculated with brain tissue from a CWD-infected mule deer. The CWD-inoculated squirrel monkeys developed a progressive neurodegenerative disease and were euthanized at 31 and 34 months postinfection. Brain tissue from the CWD-infected squirrel monkeys contained the abnormal isoform of the prion protein, PrP-res, and displayed spongiform degeneration. This is the first reported transmission of CWD to primates. ...snip...end

Full Text

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

vCJD questionnaire


why is a cjd questionnaire not listed on the cjd foundation 'forms' site with the rest of their forms ???

i thought they had one now going out to all families of victims of a human TSE???

this is very important that a _written_ cjd questionnaire, asking extensive questions pertaining to any potential route and source of the TSE agent be submitted to every family of a victim of a human TSE. this is key to finding cause. so why is this still so difficult?

are the families of CJD victims getting these written cjd questionnaires??? i hope so....


The statistical incidence of CJD cases in the United States has been revised to reflect that there is_one case per 9000 in adults age 55 and older_.

Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

2007 USA, we now have documented not only BSE mad cow, but also BASE mad cow, of which is more virulent to humans and BSE, of which is also tied to sporadic CJD. There is much science now pointing to more than one strain of CWD in deer and elk, of which, Despite the low initial attack rate on the first passage of CWD into Sg hamsters, CWD derived initially from elk and mule deer readily adapted to hamsters as evidenced by the 100% infection rate on second and third passages, a very disturbing factor considering iatrogenic CJD. this BSe that scrapie does not transmit to humans is another false myth. there is more science pointing to just the opposite, that in fact scrapie is just as capable of transmitting to humans as is BSE, and with the very likelyhood that BSE has been in sheep/goat for some time, and the fact of atypical scrapie, i.e. NOR98 of which was just documented in the USA, to say there is no risk to humans from sheep or goat is simply a false myth. AS well with blood issue and TSE, the fact that for years it could not happen, would not happen, and now it has. They have floundered to long with this. ...TSS




7. Which tissues were involved?

Please tick Please circle or underline where appropriate Any notes/details?


CNS / Spinal cord

Posterior eye / anterior eye / cornea

Olfactory epithelium

Tonsil / appendix / spleen / thymus

Other lymphoid tissue

Peripheral nerve

Dental pulp / gingival tissue / Blood / bone marrow / CSF / Placenta / Urine

Other or not know (please give available details):

8. How many instruments were used, and what were they?

(Please continue overleaf or attach details if necessary)

9. What type of decontamination procedures are used for these instruments?

10. How many times have the instruments been used and decontaminated since this procedure?

10. Can you trace the instruments?

(e.g. all / some / none / disposable / don’t know)

11. Where are the instruments now?*

(e.g. all are quarantined / some are quarantined / none are quarantined / not applicable/ don’t know)

12. How many people might have been exposed to the instruments (or pool of instruments)?

13. Other comments (Please continue overleaf or attach if necessary)


Subject: CJD: update for dental staff Date: November 12, 2006 at 3:25 pm PST 1: Dent Update. 2006 Oct;33(8):454-6, 458-60.

CJD: update for dental staff.

Scully C, Smith AJ, Bagg J. Eastman Dental Institute, University of London.

It is almost a decade since the recognition of the emergence of a new infectious disease termed variant Creutzfeldt-Jakob disease (vCJD) caused by prions (PrPTSE), abnormal variants of a normal human cell surface protein (PrP).This disease has a number of similarities to other forms of CJD--lethal disorders characterized by a prolonged incubation period, and progressive mental deterioration. In relation to oral tissues, PrPTSE have been found in neural, gingival, pulpal, lingual, lymphoreticular and salivary gland tissue in animal models. In both sporadic and variant CJD, PrPTSE is detectable in the trigeminal ganglion and, in vCJD, in lymphoreticular tissues, but infectivity has not been tested in other human oral tissues. CLINICAL RELEVANCE: PrPTSE is much more resistant to the common methods of inactivation than conventional pathogens, and it adheres avidly to steel whilst retaining its infectivity. Particular attention must be paid to cleaning and sterilizing re-usable dental instruments. Single-use devices, such as endodontic files and matrix bands, must never be re-used. Advice on the reprocessing of dental instruments used on known CJD patients must be obtained from local infection control teams. Research into effective methods of prion inactivation appears promising, although further work on the applicability to general dental practice is required.

PMID: 17087448 [PubMed - in process]

Sent: Thursday, December 28, 2006 10:23 AM Subject: Ophthalmic Surgery in Prion Diseases

Evidence For CJD/TSE Transmission Via Endoscopes From Terry S. Singletary, Sr [log in to unmask]">[log in to unmask]


1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8

Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.

Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

PMID: 8006664 [PubMed - indexed for MEDLINE]

Possible occupational risks from TSEs

Although CJD has been documented in a neurosurgeon, two neuropathology technicians, an

orthopaedic surgeon and a pathologist, it is reassuring to note that in none of these individuals

was there a history of a definite infective event. The orthopaedic surgeon had however worked

with human and ovine dura mater 20 years prior to his illness. Case-control studies do not

suggest that individuals potentially exposed to the TSE agent in the health care setting are at an

increased risk of developing CJD. However, the possibility that cases of CJD have rarely

occurred in such circumstances cannot be confidently dismissed.


If CJD were transmitted in pooled blood products or saliva, clusters would be detected. Most

clusters have usually been attributed to familial disease (Masters, 1979 & Reingold 1996).

Surveillance systems have found cases of CJD among persons who have received blood

transfusions but none have been linked to blood transmission.

It must be remembered, however, that surveillance systems may not detect cases when unique

epidemiological or clinical features are present.


A number of factors must be taken into consideration when assessing the risk of transmission of

nvCJD during dental treatment. Dental patients are at risk of infection from a number of sources,

the most significant being the consumption of infected animal products during the 1980s. A

small minority of patients will also be at increased risk in their place of work, such as

neuropathologists, neurosurgeons and laboratory technicians.

The most likely route of infection is via ineffectively sterilised instruments. If a patient is

suspected of having or has been diagnosed with nv-CJD further precautions can be taken. The

patient is likely to be showing clinical signs, which are likely to make dental treatment difficult,

therefore only emergency treatment is going to be appropriate. Where possible, a treatment

option that involves the least cross infection risk should be undertaken. . To reduce this risk, all

instruments that have been used on a patient with nvCJD should be disposable or discarded. This

includes oral surgery equipment, root planing hand instruments and ultrasonic tips in addition to

needles and blades. In the case of accidental inoculation it is unlikely that sufficient infective

material will be involved to transmit the disease. As discussed previously, the peripheral route of

infection is ineffective compared with intracerebral inoculation, and blood or saliva contains

little infectivity compared to central nervous tissue. All patients should be treated using universal

precautions, which should be employed in all dental practices as a matter of routine, providing

the maximum protection equally to clinical staff and patients. This includes the use of gloves,

masks and eye protection at all times. After each patient all instruments should be autoclaved and

disposable alternatives should be used where appropriate.

The number of patients likely to be incubating nv-CJD is impossible to predict at present. Much

depends on the average incubation time, the longer the time, the higher the figure is likely to be.

At present the average incubation time can not be calculated nor is it possible to estimate the

dose required to infect a human. With the possibility of a nationwide epidemic investigation

must be carried out to determine the risk from cross infection. Research has yet to prove that

there is a risk, however, until all possibility of this can be discounted caution must prevail.

The resistance of the TSE agent to standard medical sterilisation procedures is noteworthy.

Experimental evidence demonstrates that the agent shows resistance to the following: exposure

to boiling, freezing, ethanol, H2O2, permanganate, iodine, ethylene oxide vapour, detergents,

organic solvents, formaldehyde, UV and gamma irradiation, and standard autoclaving.

Since conventional methods of sterilisation and disinfection do not decontaminate the CJD

infectious agent, specific measures must be used, however, many of these are impractical in the

dental practice.

Although the TSE agent is known to be infectious it is not contagious in the usual sense.

Individuals exposed to patients with CJD: their spouses, nurses and doctors, do not appear to

have an increased risk of developing the disease. Furthermore, professionals who might be

considered 'high risk' in relation to exposure to TSE agents: e.g. pathologists, neurosurgeons,

butchers etc. also do not appear to be at an increased risk of developing CJD. No proven instance

of CJD contracted occupationally has yet been identified. However, over 170 cases of iatrogenic

CJD contracted through inoculation of contaminated CNS tissue or corneal transplantation serve

to remind those of us involved in the management of all CJD patients of the importance of safety

procedures in relation to the TSE agents.



Subject: MASTER DENTIST FALLS VICTIM TO CJD Date: March 31, 2007 at 1:27 pm PST

'In the hands of God' Thursday, March 29, 2007 By CRYSTAL HARMON TIMES WRITER Dr. Gregory J. Bever, 54, died Tuesday morning, at his home on Linwood Beach, surrounded by his family. His death was the result of a rare neurological malady called Creutzfeldt-Jakob disease. It struck suddenly and progressed rapidly. ...snip...end

Subject: SEAC Position statement vCJD and Endodontic dentistry Date: May 8, 2006 at 6:45 am PST CJD WATCH MESSAGE BOARD TSS 2005 SEAC Position statement vCJD and Endodontic dentistry Mon May 8, 2006 09:08

SEAC Position Statement


Position statement vCJD and Endodontic dentistry Issue

1. The Department of Health (DH) asked SEAC to advise on the findings and implications of a preliminary risk assessment of potential vCJD transmission via endodontic procedures (dental procedures involved in the maintenance of dental pulp and the treatment of the pulp cavity) 1. This is particularly pertinent because of the large number of endodontic procedures undertaken in the UK.

Background ...snip...end

Page updated: 8th May 2006


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

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

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

suppressed peer review of Harvard study October 31, 2002

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

03-025IFA 03-025IFA-2



By Terry S Singeltary

Bacliff, Texas USA Jan 24, 07


File Format: PDF/Adobe Acrobat -

Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary

Sr. [[log in to unmask]] Monday, January 08,200l 3:03 PM freas ...

----- Original Message ----- From: Terry S. Singeltary Sr. To: Terry S. Singeltary Sr. ; [log in to unmask] Cc: [log in to unmask] ; [log in to unmask] Sent: Thursday, November 30, 2006 1:47 PM Subject: Re: TSE advisory committee for the meeting December 15, 2006 [TSS SUBMISSION PART II]


full text ;

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


Mad Cow Cover-Up: NIH Plans to Destroy Brain Samples of Humans Afflicted with Mad Cow-Like CJD

Steve Mitchell of UPI has been providing excellent, ongoing coverage of mad cow and its threats in the US. Here is his latest piece. One more strong piece of evidence of the cover-up ‹ it now deserves that label ‹ of mad cow risks here in the US under an administration beholden to big beef.

John Stauber -

NIH may destroy human brain collection

Washington Times - Washington,DC,USA NIH may destroy human brain collection

By Steve Mitchell Medical Correspondent

Washington, DC, Mar. 24 (UPI) -- The National Institutes of Health may discard part or all of a rare collection that includes hundreds of human brain samples from patients that suffered from a disorder similar to mad cow disease -- unless another researcher or institution takes them on, United Press International has learned.

Several scientists said the collection, which is held by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- and includes brains and other tissue samples from people afflicted with the brain-wasting illness Creutzfeldt Jakob disease -- is irreplaceable and could even provide insight into treatments for the fatal disorder.

Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.

However, NIH officials in control of the collection's fate told UPI the remaining samples are of little scientific value and may be disposed of if researchers outside the agency do not claim it. That position stands in sharp contrast with CJD experts who thought the collection should be preserved.

"It's invaluable," said Dr. Paul Brown, former medical director of the NIH's Laboratory for Central Nervous System Studies, whose expertise is in CJD and mad cow disease (also known as bovine spongiform encephalopathy, or BSE).

The collection is badly in need of organization and no one is certain how many brains or other tissue samples it contains, said Brown, who worked with the collection since its inception in the 1960's until his retirement last year. There could be brains, blood, spinal fluid and various other tissues from 1,000 people or more, he said. Some of the specimens would be of scientific use today, he said.

"This collection has the unique value of stretching back to the beginning of when these diseases were discovered," Brown told UPI, noting that the first samples were obtained in 1963. "It would be as though you had in your hands the possibility of finding out when AIDS started."

Bruce Johnson, a former technician at the CNSS lab who worked extensively with the collection before he retired in 2003, told UPI he was told "in two years they (NIH officials)are going to destroy it, if nobody wants it."

Eugene Major, acting director of the basic neuroscience program at the NIH, said no specific timeframe had been established.

"We have not set a firm deadline date," Major told UPI. "We are working very hard with investigators that we know in order to be able to make sure that whatever we deem is valuable is potentially kept here." Some samples already have been determined not to have any research value and have been "removed and disposed of," he said.

Others samples have been given out to Dr. David Asher at the Food and Drug Administration and Pierluigi Gambetti at the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio.

Major maintained the remaining collection was not particularly valuable for research. "Whatever had been collected here that has not already been distributed to responsible investigators who could use them really has very little remaining value," he said.

Neither Asher nor Gambetti returned phone calls from UPI, but Brown said he thought Asher had received only a dozen or two samples at most and Gambetti had not received much at all.

Neil Cashman, a brain-disease researcher at the University of Toronto's Center for Research in Neurodegenerative Diseases -- who has tried to obtain the collection from the NIH -- said it was priceless.

"It would be like destroying an art museum," Cashman told UPI. "There's all this information and insight that's locked up in these tissues and if it's destroyed it will be lost forever."

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 university and institute researchers from the United States, Canada, United Kingdom and France, also thinks the brain collection is invaluable.

"It is the opinion of the Board of Directors ... of The MIND Inc., that the ... brain bank should not be broken up nor destroyed," said Harry E. Peery, MIND's executive director, in a letter to UPI. "We believe that this collection is of inestimable research value and should be kept intact."

The institute, at the University of Saskatchewan in Saskatoon, applied for possession of the collection in early 2004, but received a letter from the NINDS indicating the fate of the collection had not yet been determined.

"We have heard nothing further since that time" and continue to be interested in acquiring the complete collection, Peery said.

CJD belongs to a group of rare, brain-wasting disorders that are little understood, incurable and fatal. This includes mad cow disease in cows, chronic wasting disease in deer and elk. The most infamous of these illnesses in humans is variant CJD, which people can contract from eating beef products infected with the mad-cow pathogen.

Although vCJD has infected more than 154 people worldwide, only one case has ever been detected in the United States -- in a Florida woman who is thought to have contracted the disease while living in the United Kingdom. However, the NIH brain samples have never been screened for vCJD -- something Johnson thinks is critically important.

"No one has ever looked to see if any American (in the collection) in the past had variant CJD," Johnson said. "You think it would be required that they do that. You think it would be a Congressional mandate that they test these brains: 'Let's see if we've got this disease in our country.'"

Johnson noted at least one brain in the collection he personally had examined -- from a French woman collected in 1971 -- showed evidence of possible vCJD infection, but the sample needed further study to be sure.

Other samples in the collection include the brains of patients who were only 16 years old when they were diagnosed with CJD. This would be unusual for sporadic CJD, because generally it strikes those over age 60. Variant CJD, on the other hand, typically occurs in patients in their 20s or younger.

"I thought it was absolutely vital (to test these brains)," Johnson said. "Maybe there's a dozen cases in there of variant CJD."

Major disagreed. "There's really no reason to do that," he said. "The effort it would take to screen those samples ... would not give us any new insights into variant CJD beyond what it is we already know."

Johnson said he was frustrated with the NIH administration's lack of interest in preserving the collection or testing for vCJD. "They don't understand," he said, "they honest-to-god don't understand what it's all about."

Patient advocates also objected to the possible destruction of the brains.

Terry Singeltary, whose mother died of a type of CJD called Heidenhain variant in 1997, said he is outraged and families of other CJD victims probably will be, too.

"A lot of these families went through a lot of heartache and a lot of trouble to get these brain samples to the NIH," Singeltary told UPI. "Now they're just going to discard them because they're not of scientific use? That's just asinine. That stuff is valuable information."

Graham Steel, vice-chair of the Human BSE Foundation in the United Kingdom, told UPI, "The potential loss of such important tissue samples would be a massive blow for TSE (the group of diseases that includes CJD and BSE) research in the United States. This should not be allowed to happen."

Singeltary noted there currently is no cure for these diseases. "If you don't have any answers yet, why would you throw these specimens away?" he asked.

He added that more sensitive tests are just becoming available and could help determine the origin of some of the CJD cases. "We've all been sitting around waiting for more sensitive tests to get validated because we want answers," he said.

"You know, it must be an embarrassment," Johnson said. "Some Senator is going to eventually say 'What is NIH doing about mad cow disease?' And people are going to scratch their heads and say 'not much'." He added, "What's going to happen (is) one of these senators or their wife is going to develop spontaneous CJD one day and ... there's going to be hell raised and they're going to ask, 'Why isn't NIH working on this?'"


E-mail mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/!

NIH sends mixed signals on CJD brains

By Steve Mitchell Medical Correspondent

Washington, DC, Apr. 7 (UPI) -- A National Institutes of Health official who told United Press International the agency might destroy its collection of brains from human patients afflicted with a condition similar to mad cow disease reportedly has told the head of a patient-advocate group the collection would be preserved.

The official, Eugene Major, acting director of the basic neuroscience program at the NIH, has not responded to e-mail or a phone call from UPI seeking clarification of his remarks, and the official status of the collection remains unknown.

As reported by UPI on March 24, the collection is stored in freezers by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. It contains brains and other tissue samples from hundreds of people who died from the brain-wasting illness Creutzfeldt Jakob disease, as well as tissues from an untold number of experimental animals.

The consensus of scientists in this field is the collection, which dates back to 1963, is invaluable for research and could even provide insight into treatments for the fatal disorder. Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.

Florence Kranitz, president of the non-profit advocacy group CJD Foundation, told UPI she had "a very long conversation" with Major, in which he told her the remaining tissues in the collection would not be destroyed.

"He reassured me in no uncertain terms," Kranitz said, noting constituents of the foundation and other CJD advocacy groups had been expressing concerns to her the tissues would be destroyed.

Kranitz, who has personal reasons for wanting the collection preserved -- her husband died of CJD in 2000 -- said she plans to meet with Major at the end of April to discuss the issue further.

CJD belongs to a group of diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep. All TSEs are incurable and fatal.

Major previously told UPI some samples already have been destroyed and others have been given to researchers at the Food and Drug Administration and the National Prion Disease Pathology Surveillance Center in Cleveland.

Major said the remaining collection "has very little remaining value" and could be destroyed if another entity does not claim them.

Bruce Johnson, a former NIH scientist who retired at the end of 2003, said he had been told the collection would be destroyed in two years if no one took the samples from the NIH.

In response to hearing that Major had failed to confirm to UPI the brain collection would not be destroyed, Patricia Ewanitz, who lives in Port Jefferson Station, N.Y., and is founder of the advocacy group CJD Voice, said, "The brain tissue might not be indispensable to the National Institutes of Health but it is absolutely necessary to the families who thought enough of science to donate the brains, brain tissue and blood in hopes of someday finding an answer to why their loved one died."

Ewanitz, whose husband died of CJD in 1997, added, "It now seems like such a joke."

Terry Singeltary, whose mother passed away from a type of CJD in 1997, said the NIH should use the samples for scientific research, not just store them in freezers.

Both Singeltary and Ewanitz said they would feel more reassured if Major verified in writing the collection will not be destroyed.

"I would go further and ask Major what he plans to do with them," Singeltary said. "If the samples are just going to sit up there and go bad, then they should give them out to researchers looking for cause and cure."

The revelation the NIH might destroy part or all of the collection sparked an outcry from patient advocates, consumer groups and scientists.

Advocates have been contacting their members of Congress, urging them to investigate and prevent the NIH from destroying the brains. Consumer groups also have gotten involved and scientists have taken steps to obtain the collection or have urged Major not to destroy the samples.

Felicia Nestor, who serves as a consultant to Public Citizen, told UPI she had contacted certain legislators and at least one was considering looking into the situation. Nestor asked the legislator's name be withheld.

Kranitz said Major also told her he plans "to advertise in professional neurological journals and by whatever means necessary to make it known" to researchers in the field the tissues are available.

Major previously said, however, that efforts to inform researchers of the availability of the collection were already underway and included informing NIH grantees. He added he had personally notified researchers at scientific meetings, but no TSE researcher contacted by UPI was aware of this.

"I was never informed," said Laura Manuelidis, an expert on these diseases and section chief of surgery in the neuropathology department at Yale University. She said the first she had heard of the situation was in UPI's March 24 report.

Manuelidis also said she contacted Major, expressing interest in the specimens, but so far has not received a response.

"I sent a letter to (Major) on (March 25) about our interest in these specimens, but he has not replied," she told UPI in an e-mail.

Neil Cashman, a TSE expert at the University of Toronto, who said he was not aware the samples might be destroyed, has lobbied colleagues at the University of British Columbia -- where Cashman is scheduled to move to this summer -- to help draft a letter requesting the collection.

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 university and institute researchers from the United States, Canada, the United Kingdom and France, requested the collection in January, 2004. So far, the institute has not been informed of a decision by the NIH.

Asked if Major had told him whether the collection would be preserved, MIND Executive Director Harry Peery said, "We have heard nothing further from Eugene Major or anyone else at the NIH regarding the brain collection."


E-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/!

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

NIH says it will preserve CJD brains By STEVE MITCHELL

WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.

An NIH official had told United Press International previously that the brain collection, which consists of samples from hundreds of people who died from the brain-wasting illness called Creutzfeldt Jakob disease, could be discarded if another entity does not claim them.

That sparked an outcry from patient-advocacy groups, consumer watchdogs and scientists, and the agency now appears to have backed away from that course.

"All the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved," Story Landis, director of the National Institute of Neurological Disorders and Stroke, which oversees the brain collection, wrote in a May 10 letter to Sen. John Cornyn, R-Texas.

Cornyn had inquired about the status of the collection in April.

Last March, Eugene Major, acting director of the basic neuroscience program at the NIH, told UPI the useful portions of the collection had been doled out to scientists and the remaining samples had "very little remaining value" and could be destroyed.

Landis could not be reached for comment Tuesday. NINDS spokesman Paul Girolami told UPI he had been unable to locate her.

Scientists think the collection, which dates back to 1963, is invaluable for research on CJD and similar diseases and could even provide insight into treatments. There is no cure for CJD and patients typically die within a year after symptoms begin.

"Absolutely, the collection is worth keeping," Bruce Johnson, a former NIH scientist who said he had been told the collection would be destroyed in two years if no one took the samples from the agency, told UPI.

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 researchers from several countries, offered to take the collection off of NIH's hands more than a year ago and so far has not heard anything from the agency, Harry Peery, MIND's executive director, told UPI.

CJD belongs to a group of incurable and fatal diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep.

Variant CJD, or vCJD, is a relatively new TSE, which people can contract from consuming beef products infected with the mad cow pathogen.

Despite Landis' assurance the collection will be preserved, some family members of the patients who donated their brains to the NIH are still skeptical. This is because the wording Landis used in the letter leaves open the possibility that some brain samples are being destroyed.

"The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification," Landis wrote.

"Which ones" are being destroyed? asked Terry Singeltary, who is involved with several CJD patient groups.

"With a system like this, they could destroy whatever and whenever they wanted, for whatever reason they wanted," Singeltary, whose mother died of CJD in 1997, told UPI.

"It's a perfect excuse to discard some suspicious tissue resembling vCJD or some atypical TSE related to animal TSEs in the USA," he added.

Although the collection includes samples from CJD patients as young as 16 that could make them candidates for possible vCJD, the brains have never been screened for evidence of the disease. The only confirmed vCJD case in the United States occurred in a Florida woman who is thought to have contracted the disease in England.

Johnson said he along with renowned CJD expert Paul Brown were in the process of sorting through the samples to match them up with patient identification documents until they both retired. Some of the samples may prove impossible to identify, he said, but he and Brown are the only ones familiar enough with the collection to organize it and neither has been asked back by the agency to aid in the identification process.

Steve Mitchell is UPI's Medical Correspondent. E-mail: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/!

Copyright 2005 by United Press International. All Rights Reserved.


JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 April 26,2005 Mr. Terry SingeltaryP.O. Box 42Bacliff, Texas 77518 Dear Mr. Singeltary: In response to your recent request for my assistance, I have contacted the National Institutes ofHealth. I will write you again as soon as I receive a reply. I appreciate having the opportunity to represent you in the United States Senate and to be ofservice in this matter. Sincerely,

JOHN CORNYN United States Senator JC:djl


JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 May 18,2005 Mr. Terry SingeltaryP.O. Box 42Bacliff, Texas 77518 Dear Mr. Singeltary: Enclosed is the reply I received from the Department of Health and Human Services in response to my earlier inquiry on your behalf. I hope this will be useful to you. I appreciate having the opportunity to represent you in the United States Senate. Thank you for taking time to contact me. Sincerely,

JOHN CORNYN United States Senate JC:djl Enclosure

DEPARTMENT OF HEALTH & HUMAN SERVICES National Institutes of HealthNational Institute of NeurologicalDisorders and Stroke NINDS Building 31, Room 8A52 31 Center Dr., MSC 2540 Bethesda, Maryland 20892-2540 Phone: 301-496-9746 Fax: 301-496-0296 Email: mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000200/!

May 10, 2005

The Honorable John CornynUnited States SenatorOccidental Tower5005 LBJ Freeway, Suite 1150Dallas, Texas 75244-6199

Dear Senator Cornyn:

Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about thepreservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by theNational Institute of Neurological Disorders and Stroke (NINDS) Intramural Research programfor many years. I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand hisdesire that any tissues that could help investigators unravel the puzzle of this deadly disease arepreserved. I hope he will be pleased to learn that all the brains and other tissues with potential tohelp scientists learn about CJD are, and will continue to be, conserved. (The tissues that arediscarded are those that have either decayed to an extent that renders them no longer appropriatefor research or those for which we do not have sufficient identification.) The purpose of gathering these brains and tissues is to help scientists learn about CJD. To that end, some of the NINDS-held samples are distributed to investigators who can demonstrate thatthey have a compelling research or public health need for such materials. For example, sampleshave been transferred to NIH grantee Dr. Pierluigi Gambetti, who heads the National PrionDiseases Pathology Surveillance Center at Case Western Reserve University in Ohio and workswith the Centers for Disease Control and Prevention to monitor all cases of CJD in the UnitedStates. Dr. Gambetti studies the tissues to learn about the formation, physical and chemicalproperties, and pathogenic mechanisms of prion proteins, which are believed to be involved inthe cause of CJD. Samples have also been transferred to Dr. David Asher, at the U.S. Food andDrug Administration, for use in assessing a potential diagnostic test for CJD.

Page 2 - The Honorable John Cornyn

in closing, we know that donating organs and tissue from loved ones is a very difficult andpersonal choice that must often be made at the most stressful of times. We at the NINDS aregrateful to those stalwart family members who make this choice in the selfless hope that it willhelp others afflicted with CJD. We also know the invaluable contribution such donations maketo the advancement of medical science, and we are dedicated to the preservation of all of thetissue samples that can help in our efforts to overcome CJD.

I hope this information is helpful to you in responding to Mr. Singeltary. Sincerely,

Story C. Landis, Ph.D. Director, National Institute ofNeurological Disorders and Stroke ==================================


a.. see re-NIH to destroy brain samples and tissues


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Dina, this may help explain all the TSE in the USA, and any potential link thereof to humans ;

A novel human disease with abnormal prion protein sensitive to protease (prionopathy) JUNE 2008

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

Monday, June 23, 2008


Thursday, June 26, 2008

Texas Firm Recalls Cattle Heads That Contain Prohibited Materials

Tuesday, May 27, 2008

FDA BSE/Ruminant Feed Inspections Firms Inventory Report Texas Legend Ranch OAI 05/10/2008

In 2007, in one weekly enforcement report, the fda recalled 10,000,000+ pounds of BANNED MAD COW FEED, 'in commerce', and i can tell you that most of it was fed out ;


Date: March 21, 2007 at 2:27 pm PST REASON Blood meal used to make cattle feed was recalled because it was cross-contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement. VOLUME OF PRODUCT IN COMMERCE 42,090 lbs. DISTRIBUTION WI

REASON Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement. VOLUME OF PRODUCT IN COMMERCE 9,997,976 lbs. DISTRIBUTION ID and NV


Subject: MAD COW FEED RECALL USA SEPT 6, 2006 1961.72 TONS IN COMMERCE AL, TN, AND WV Date: September 6, 2006 at 7:58 am PST

snip... see listings and references of enormous amounts of banned mad cow protein 'in commerce' in 2006 and 2005 ;

see full text ;

Friday, April 25, 2008

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



Thursday, June 26, 2008

Texas Firm Recalls Cattle Heads That Contain Prohibited Materials

STILL DISGUSTED IN SUNNY AND HOT BACLIFF, TEXAS, where we still feed cows to cows in 2008 for pete's sake, and where we feed our children, dead stock downer cows all across our Nation. ...


Wednesday, June 25, 2008 Abuse of downer cattle continues, Rep. Rosa DeLauro, D-Conn., "potentially contaminated meat into the school lunch program" Group: Abuse of downer cattle continues

Tuesday, June 3, 2008


hoped this helped,

P.S. IF you would like me to speed up your loved ones autopsy, i could forward this email through the CJD-Voice list, one that the CJD Foundation hates, but monitors closely. it might help? so please let me know if you would like me to forward this through to the CJD-Voice list. ...

with kindest regards,


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


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