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Well-known member
Sep 3, 2005
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##################### Bovine Spongiform Encephalopathy #####################

Date: January 23, 2006 at 8:51 am PST

Latest Information (as of January 23, 2006 - 10:00 EST)
The Canadian Food Inspection Agency (CFIA) today confirmed bovine spongiform encephalopathy (BSE) in an approximately six-year-old cross-bred cow born and raised in Alberta. No part of the animal entered the human food or animal feed systems.
The CFIA, working collaboratively with the producer and the Province of Alberta, has launched a comprehensive investigation into the feeding regime and storage practices employed on the farm, as well as the production and source of feeds delivered to the farm. Consistent with international standards, the CFIA will identify cattle born on the farm within 12 months before and after the affected animal, as well as offspring of the affected animal born during the last two years. Any live animals found from these groups will be segregated and tested.
This detection is consistent with a low level of disease and does not indicate an increased risk of BSE in Canada. Based on the guidelines and certification recommendations of the World Organization for Animal Health, this finding should not affect Canada’s ability to export live animals, beef and beef products.
Case 4 - Confirmed January 22, 2006


OTTAWA, January 23, 2006 - The Canadian Food Inspection Agency (CFIA) today confirmed bovine spongiform encephalopathy (BSE) in an approximately six-year-old cross-bred cow born and raised in Alberta. No part of the animal entered the human food or animal feed systems.

This finding is not unexpected and was identified through Canada’s national surveillance program, which targets cattle at highest risk of being infected with BSE. The program has tested more than 87,000 animals since Canada’s first BSE case in 2003.

The geographic location and age of this animal are consistent with the three domestic cases previously detected through the national BSE surveillance program and the current understanding of BSE in Canada. The clustering of these cases is examined in the epidemiological report, Canada’s Assessment of the North American BSE Cases Diagnosed from 2003 to 2005 (Part II), which is available on the CFIA’s Website.

Food safety remains protected through the removal of specified risk material (SRM) from all cattle slaughtered for human food in Canada. SRM are tissues that, in infected cattle, contain the BSE agent. This measure is internationally recognized as the most effective means to protect public health from BSE.

The CFIA, working collaboratively with the producer and the Province of Alberta, has launched a comprehensive investigation into the feeding regime and storage practices employed on the farm, as well as the production and source of feeds delivered to the farm. Consistent with international standards, the CFIA will identify cattle born on the farm within 12 months before and after the affected animal, as well as offspring of the affected animal born during the last two years. Any live animals found from these groups will be segregated and tested.

Definitive conclusions regarding the source of infectivity cannot be made until the investigation is complete; however, it is probable that the source is contaminated feed. This scenario is consistent with Canada’s previous experience and that of the international community. Although the first evidence of BSE in the Canadian herd was in May 2003 and this recent animal would have become infected with the disease prior to that time, this case does support the need for Canada to continue to move towards enhancing the current feed ban. In December 2004, there was a formal call for comments on the draft regulations through Canada Gazette, which have been the subject of extensive consultations with the provinces and industry. The CFIA has completed its analysis and is prepared to provide advice to the government on next steps.

Canada has a suite of safeguards that work together to systematically limit the risks to animal and public health associated with BSE. These measures include import controls, surveillance of the national cattle herd and the removal of potentially harmful tissues from all animals slaughtered for human consumption. Of principal importance from an animal health perspective is Canada’s feed ban, which is designed to limit BSE spread and eradicate the disease over time. Analysis of previous Canadian investigations and surveillance results to date continue to indicate that Canada’s feed ban is working as intended to effectively reduce BSE risks to animal health through a robust compliance and enforcement strategy. Our experiences related to the feed ban and finding cases born after the feed ban are in keeping with other countries.

This detection is consistent with a low level of disease and does not indicate an increased risk of BSE in Canada. Based on the guidelines and certification recommendations of the World Organization for Animal Health, this finding should not affect Canada’s ability to export live animals, beef and beef products. Canada has notified its key trading partners, including the United States. Since May 2003, Canada has clearly stated the possibility of finding a small number of additional BSE cases. This international dialogue will continue as Canadian officials work closely with their international counterparts to ensure the facts and supporting science of this case are shared in an open and timely manner.

As the investigation progresses, the CFIA will provide Canadians with regular updates. Information will be posted to the CFIA’s Website as it becomes available.

- 30 -

For information:

Canadian Food Inspection Agency
Media Relations: (613) 228-6682



#################### https://lists.aegee.org/bse-l.html ####################


Well-known member
Feb 11, 2005
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Home on the Range, Alberta
Why don't we as producers and consumers, demand that the brain of this BSE diseased animal be tested for TOTAL metal content at the University of Saskatchewan's Light Synchrotron?

This must include, radio-active ions, like uranium.

Copper, zinc, iron, selenium, phosphorous, calcium..............

Compare these results to a healthy cow of the same sex and age.


Well-known member
Sep 3, 2005
Reaction score
Greetings Ranchers and board members,

Kathy wrote in another thread, but yet never documented;

> didn't say that OPs caused prion disease, but that they contributed greatly
> to the UK experience,

and again, could your please reference this materials with scientific data, instead
of hearsay please ???

you can argue the origin of TSE until all the mad cows on earth come home to roost.

i.e. ;

Phosmet induces up-regulation of surface levels of the cellular prion protein.
Neuroreport. 9(7):1391-1395, May 11, 1998.
Gordon, Irit 1; Abdulla, Elizabeth M. 1; Campbell, Iain C. 1; Whatley, Stephen A. 1,2
CHRONIC (2 day) exposure of human neuroblastoma cells to the organophosphate pesticide phosmet induced a marked concentration-dependent increase in the levels of PrP present on the cell surface as assessed by biotin labelling and immunoprecipitation. Levels of both phospholipase C (PIPLC)-releasable and non-releasable forms of PrP were increased on the plasma membrane. These increases appear to be due to post-transcriptional mechanisms, since PrP mRNA levels as assessed by Northern blotting were unaffected by phosmet treatment. These data raise the possibility that phosmet exposure could increase the susceptibility to the prion agent by altering the levels of accessible PrP.

(C) Lippincott-Raven Publishers.


BUT, amplification and transmission is the most important factor in stopping the

spread of the TSE agent. WE have known for decades what factors involve the amplification

and transmission, yet the industries involved CHOSE to ignore this science until the incubation

period in humans and animals started to catch up with society.

would it not have been more easy and very much less expensive for MAFF/USDA et al to jump on the OP
bandwagon and save the industry, if OP theory had any credence to it at all? and why did they not?

transmission studies DO NOT LIE !

show me the data that ops are in all the primate/mouse transmission studies?

Scientific Steering Committee
In its opinion on possible links between BSE and Organophosphates adopted on 25-26 June
1998 and in its opinion on Hypotheses on the origin and transmission of BSE adopted on 29-
30 November 2001 the SSC concluded that there is no scientific evidence in support of the
hypothesis of an OP origin of BSE.
The issue of organophosphate poisoning has not been dealt with by the SSC so far. The
concerns expressed in the enquiries cover mainly intoxication by occupational exposure of
shepherds and farmers to OPs upon use against ecto-parasites, especially in sheep dipping and
treatment of cattle against Warble Fly infestation. Risks from residues are addressed to a
lesser extend.
In early 2003, a large number of additional enquiries on the issue have been addressed to
European Commission’s Health and Consumer Directorate General. Four of these with
substantial enclosures were by one person. Most of them are addressing both issues: chronic
organophosphate (OP) poisoning and the origin of BSE.
Information provided with the enquiries
In addition to numerous newspaper and magazine articles the enclosures to the enquiries
provide the Material Safety Data Sheet on diazinon, the OHSA Occupational Safety and
Health Guideline for Tetraethylpyrophosphate (TEPP), an US agency Hazardous Substances
Fact Sheet on crufomate, company safety information sheets, some correspondence with UK
authorities including their activities to improve safe use of these chemicals. The information
regarding claimed OP chronic poisoning of cases presented does not provide evidence, neither
for OPs being the cause for diseases nor for their exclusion (i.e., “very low” bloodcholinesterase
levels, provided without data or comparison with the normal distribution of
values; successful treatment of a patient for OP clearance without giving any OP data). It
C:\WINNT\Temporary Internet Files\SSC_Last_OP_Final.doc 2
seems however, that due to insufficient, non-prudent use of the safety requirements undue
exposures of shepherds and farmers have occurred.
There is no additional information on the claimed involvement of OPs in the origin of BSE.
This applies for both, the hypotheses on the direct effect of OPs as well as on their
hypothetical role for Cu-deficiency to be involved in the origin of BSE (Cu binding of prion
protein is known). New publications are mentioned in one enquiry but they have not yet been
provided. In an Internet search no recent scientifically valid publications were traceable. The
SSC had been informed that research would be launched on this hypothesis, but no
information has been provided so far on its status or on results.
a) As regards the involvement of organophosphates in the origin of BSE, no new scientific
information providing evidence or supporting the hypothesis by valid data became
available after the adoption of the last opinion of the SSC on this issue. Consequently
there is no reason for modifying the existing opinions.
b) Regarding the possibility of OP poisoning, the European legislation for registration of
plant protection products and veterinary medicines – addressed in the enquiries – provide
the basis for safe use of registered compounds and their formulations. Regarding the
alleged intoxication cases reported and OP exposure it must be concluded that safety
measures may not have been strictly followed.
Brown, D.R., Qin, K., Herms, J.W., Madlung, A., Manson, J., Strome, R., Fraser, P.E., Kruck, T., von
Bohlen, A., Schulz- Schaeffer, W., Giese, A., Westaway, D. and Kretzschmar, H. (1997) The Cellular
Prion Protein Binds Copper In Vivo, Nature, 390, 684-7.
Purdey, M. (2000) Ecosystems Supporting Clusters of Sporadic TSEs Demonstrate Excesses of the Radical-
Generating Divalent Cation Manganese and Deficiencies of Antioxidant Co-Factors Cu, Se, Fe, Zn Medical
Hypotheses, 54, 278-306.
Scientific Steering Committee, 1998. Opinion on possible links between BSE and Organophosphates. Adopted
on 25-26 June 1998
Scientific Steering Committee, 2001. Opinion on Hypotheses on the origin and transmission of BSE. Adopted
on 29-30 November 2001.


Studies on the Putative Interactions between the Organophosphorus Insecticide Phosmet and Recombinant Mouse PrP and its Implication in the BSE Epidemic
I. Shaw1, C. Berry2, E. Lane1, P. Fitzmaurice1, D. Clarke3 and A. Holden1

(1) Centre for Toxicology, University of Central Lancashire, Preston, UK
(2) Department of Morbid Anatomy, The Royal London Hospital, London, UK
(3) CLRC Daresbury Laboratory, Warrington, UK

Abstract It has been suggested that exposure of cattle to the ectoparasiticide Phosmet in the 1980s caused a conformational change in the cellular prion protein (PrPC) to form the BSE prion (PrPSC), which initiated the epidemic of bovine spongiform encephalopathy (BSE).
Recombinant mouse cellular prion (r[mouse]PrPC) was exposed to the organophosphorus pesticide Phosmet in vitro and the conformation of the prion before and after exposure was monitored using circular dichroism (CD) spectroscopy, utilizing synchrotron radiation at the Council for the Central Laboratory of the Research Councils (CLRC) facilities at Daresbury, UK. Metabolites of Phosmet, generated in situ by rat microsomes, were investigated in the same way, to determine whether they might initiate the conformational change due to their high chemical reactivity.
Our studies showed that exposure of r[mouse]PrPC to Phosmet or microsomes-generated metabolites of Phosmet did not result in the conformational change in the protein from -helix to -pleated sheet that is characteristic of the PrPC to PrPSC conversion and, therefore, Phosmet is very unlikely to have initiated the BSE epidemic by a simple direct mechanism of conformational change in the prion protein.
bovine spongiform encephalopathy - circular dichroism spectroscopy - insecticide - organophosphate - Phosmet - prion - protein conformation


transmission studies do not lie, amplification and transmission!

i see NO properties of high levels of Manganese in the diet, combined with low levels of copper, in any of these primate
transmission studies.

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to
nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of
sheep and goats were transmitted to squirrel monkeys (Saimiri
sciureus) that were exposed to the infectious agents only by their
nonforced consumption of known infectious tissues. The asymptomatic
incubation period in the one monkey exposed to the virus of kuru was
36 months; that in the two monkeys exposed to the virus of
Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and
that in the two monkeys exposed to the virus of scrapie was 25 and
32 months, respectively. Careful physical examination of the buccal
cavities of all of the monkeys failed to reveal signs or oral
lesions. One additional monkey similarly exposed to kuru has
remained asymptomatic during the 39 months that it has been under

PMID: 6997404


Oral Transmission And Early Lymphoid Tropism Of Chronic Wasting Disease
Prpres In Mule Deer Fawns

Publication: Journal Of General Virology
Publication Request Approval Date: June 25, 1999

Interpretive Summary: Chronic wasting disease is a transmissible
spongiform encephalopathy or prion disease of deer and elk. CWD is a
member of the family of diseases that includes sheep scrapie and bovine
spongiform encephalopathy (BSE). The natural route of transmission of
these diseases in ruminant animals is unknown but oral exposure to
contaminated feeds, bedding, or tissues is presumed to be a major source
of infection. In this study, mule deer fawns were orally fed an
infectious homogenate and sacrificed at intervals to examine the
lymphoid tissue of the alimentary tract for signs of infection. Prion
protein was detected as early as 42 days and was evident in all fawns
after 53 days. This paper provides an improved procedure for detecting
prions in early infection, establishes a protocol for accelerated study
of transmission routes, and supports the hypothesis that oral exposure
may reflect the initial pathway of CWD infection in deer.

Technical Abstract: Mule deer fawns were inoculated orally with a brain
homogenate prepared from mule deer with naturally occurring chronic
wasting disease (CWD), a prion induced transmissible spongiform
encephalopathy. Fawns were necropsies and examined for PrP-res, a
protein marker for infection, at 10, 42, 53, 77, 78 and 80 days post
inoculation using an immunohistochemistry assay modified to enhance
sensitivity. PrP-res was detected in alimentary-tract- associated
lymphoid tissues as early as 42 days post inoculation and in all fawns
after 53 days. These results indicated that CWD PrP-res can be detected
at least 16 months before clinical signs would be expected to appear and
may reflect the initial pathway of CWD infection in deer.


Establishing the transmission of BSE to mink

44. Transmissible mink encephalopathy ("TME") is a rare disease of ranch
reared mink, first recognised in the USA. It had been assumed to be
scrapie in mink and, like BSE, outbreaks have epidemiological
features consistent with a foodborne infection, but it has never been
possible to demonstrate that scrapie infected sheep brain tissue is
pathogenic to mink by oral exposure. In an incident of TME in
Stetsonville, Wisconsin, USA in 1985 Dr Richard Marsh observed that
although the rancher fed 'dead stock', mainly in the form of cattle
carcasses, sheep tissues had never been fed. Studies in the USA of
this incident showed not only that cattle inoculated intracerebrally
with the mink brain developed a fatal spongiform encephalopathy, but
also that the cattle passaged agent remained pathogenic for
mink by either intracerebral inoculation or feeding. In the absence of
reports of a clinical disease homologous to BSE in domestic cattle,
these findings prompted the suggestion that a rare or occult
form of such a disease might exist in the USA. Comparison of the
biological properties of the BSE
pathogen with those of the Stetsonville isolate was therefore of
considerable interest in relation to hypotheses concerning possible
origins of BSE and potential for subclinical infection in cattle.
45. Proposals to carry out studies with mink in the USA were developed
in collaboration with, the United States Department of Agriculture
("USDA") Agricultural Research Service ("ARS") and the
Department of Veterinary Science, University of Wisconsin, Madison,
Wisconsin, USA. On 30th October, 1990 I attended a CVL/NPU BSE R&D
meeting at the NPU in Edinburgh (YB90/10.30/1.1). I reported that brain
material from BSE affected cows and a control cow (not fed meat and
bonemeal) had been sent coded to Mr Mark Robinson (USDA) for
transmission studies in mink. The studies were conducted from February
1991 under the control and principal funding of USDA-ARS. The results,
discussed at the tenth CVL/NPU BSE R&D meeting on 27th April, 1993
(YB93/4.27/1.1) indicated that mink were indeed susceptible to BSE and,
in contrast to previous attempts to transmit scrapie to the species,
were susceptible by the oral route of inoculation. The collaboration
resulted in the publication of a paper: Robinson, M.M. et al (1994)
Experimental infection of mink with bovine spongiform encephalopathy.
Journal of General Virology 75, 2151-2155 (J/JVIR /75/2151).





1: Proc Natl Acad Sci U S A 2001 Mar 27;98(7):4142-7

Adaptation of the bovine spongiform encephalopathy agent to primates and
comparison with Creutzfeldt-- Jakob disease: implications for human health.

Lasmezas CI, Fournier JG, Nouvel V, Boe H, Marce D, Lamoury F, Kopp N,
Hauw JJ, Ironside J, Bruce M, Dormont D, Deslys JP.

Commissariat a l'Energie Atomique, Service de Neurovirologie, Direction
des Sciences du Vivant/Departement de Recherche Medicale, Centre de
Recherches du Service de Sante des Armees 60-68, Fontenay-aux-Roses,
France. [email protected]

There is substantial scientific evidence to support the notion that
bovine spongiform encephalopathy (BSE) has contaminated human beings,
causing variant Creutzfeldt-Jakob disease (vCJD). This disease has
raised concerns about the possibility of an iatrogenic secondary
transmission to humans, because the biological properties of the
primate-adapted BSE agent are unknown. We show that (i) BSE can be
transmitted from primate to primate by intravenous route in 25 months,
and (ii) an iatrogenic transmission of vCJD to humans could be readily
recognized pathologically, whether it occurs by the central or
peripheral route. Strain typing in mice demonstrates that the BSE agent
adapts to macaques in the same way as it does to humans and confirms
that the BSE agent is responsible for vCJD not only in the United
Kingdom but also in France. 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. These data will be key in identifying the origin of
human cases of prion disease, including accidental vCJD transmission,
and could provide bases for vCJD risk assessment.


this next one frightens me the most, you might want to read
it twice, and really think about it...TSS

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.


Proc Natl Acad Sci U S A 1999 Mar 30;96(7):4046-51

Natural and experimental oral infection of nonhuman primates by bovine
spongiform encephalopathy agents.

Bons N, Mestre-Frances N, Belli P, Cathala F, Gajdusek DC, Brown P.

Ecole Pratique des Hautes Etudes, Laboratoire de Neuromorphologie
Fonctionnelle, Universite Montpellier II, 34095-Montpellier cedex 5, France.

Experimental lemurs either were infected orally with the agent of bovine
spongiform encephalopathy (BSE) or were maintained as uninfected control
animals. Immunohistochemical examination for proteinase-resistant
protein (prion protein or PrP) was performed on tissues from two
infected but still asymptomatic lemurs, killed 5 months after infection,
and from three uninfected control lemurs. Control tissues showed no
staining, whereas PrP was detected in the infected animals in tonsil,
gastrointestinal tract and associated lymphatic tissues, and spleen. In
addition, PrP was detected in ventral and dorsal roots of the cervical
spinal cord, and within the spinal cord PrP could be traced in nerve
tracts as far as the cerebral cortex. Similar patterns of PrP
immunoreactivity were seen in two symptomatic and 18 apparently healthy
lemurs in three different French primate centers, all of which had been
fed diets supplemented with a beef protein product manufactured by a
British company that has since ceased to include beef in its veterinary
nutritional products. This study of BSE-infected lemurs early in their
incubation period extends previous pathogenesis studies of the
distribution of infectivity and PrP in natural and experimental scrapie.
The similarity of neuropathology and PrP immunostaining patterns in
experimentally infected animals to those observed in both symptomatic
and asymptomatic animals in primate centers suggests that BSE
contamination of zoo animals may have been more widespread than is
generally appreciated.


1: Vet Rec 1993 Apr 17;132(16):403-6

Experimental transmission of BSE and scrapie to the common marmoset.

Baker HF, Ridley RM, Wells GA.

Division of Psychiatry, Clinical Research Centre, Harrow, Middlesex.

Two young male common marmosets (Callithrix jacchus) were injected
intracerebrally and intraperitoneally with a crude brain homogenate
prepared from a cow with bovine spongiform encephalopathy (BSE). Two
other marmosets were similarly injected with brain homogenate from a
sheep with natural scrapie. The two animals injected with scrapie
material developed neurological signs 38 and 42 months after injection
and the two animals injected with BSE material developed neurological
signs after 46 and 47 months. Post mortem examination of the brains
revealed spongiform encephalopathy especially in the basal nuclei and
diencephalon of all the animals and, in addition, involvement of the
cerebral cortex of the marmosets injected with scrapie material. The
experiment extends the host range of experimental BSE to include a
primate species.


1: J Gen Virol 1991 Mar;72 ( Pt 3):589-94

Epidemiological and experimental studies on a new incident of
transmissible mink encephalopathy.

Marsh RF, Bessen RA, Lehmann S, Hartsough GR.

Department of Veterinary Science, University of Wisconsin-Madison 53706.

Epidemiological investigation of a new incident of transmissible mink
encephalopathy (TME) in Stetsonville, Wisconsin, U.S.A. in 1985 revealed
that the mink rancher had never fed sheep products to his mink but did
feed them large amounts of products from fallen or sick dairy cattle. To
investigate the possibility that this occurrence of TME may have
resulted from exposure to infected cattle, two Holstein bull calves were
injected intracerebrally with mink brain from the Stetsonville ranch.
Each bull developed a fatal spongiform encephalopathy 18 and 19 months
after inoculation, respectively, and both bovine brains passaged back
into mink were highly pathogenic by either intracerebral or oral
inoculation. These results suggest the presence of a previously
unrecognized scrapie-like infection in cattle in the United States.


1: Ital J Neurol Sci 1983 Apr;4(1):61-4

Creutzfeld-Jakob disease in the province of Siena: two cases transmitted
to monkeys.

Fieschi C, Orzi F, Pocchiari M, Nardini M, Rocchi F, Asher D, Gibbs C,
Gajdusek D.

Two cases of histopathologically documented Creutzfeldt-Jakob disease
were observed in the same area of the province of Siena in 1974-1975.
The transmission of the disease was obtained through brain homogenates
and lymphnodes in one of the two cases. This confirms that the agent is
present in other tissues besides the brain and underlines further the
analogies between Creutzfeld-Jakob disease and scrapie.


1: Dev Biol Stand 1993;80:9-13

Transmission of human spongiform encephalopathies to experimental
animals: comparison of the chimpanzee and squirrel monkey.

Asher DM, Gibbs CJ Jr, Sulima MP, Bacote A, Amyx H, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of
Neurological Disorders and Stroke, NIH, Bethesda, MD 20992.

The agents of kuru and Creutzfeldt-Jakob disease have been consistently
transmitted from patients with those diseases to chimpanzees and
squirrel monkeys, as well as to other new-world primates, with average
incubation periods of two or three years. No other animals have been
found so consistently susceptible to the agents in human tissues. More
rapid and convenient assays for the infectious agents would greatly
facilitate research on the spongiform encephalopathies of humans.


1: J Vet Diagn Invest 2001 Jan;13(1):91-6

Preliminary findings on the experimental transmission of chronic wasting
disease agent of mule deer to cattle.

Hamir AN, Cutlip RC, Miller JM, Williams ES, Stack MJ, Miller MW,
O'Rourke KI, Chaplin MJ.

National Animal Disease Center, ARS, USDA, Ames, IA 50010, USA.

To determine the transmissibility of chronic wasting disease (CWD) to
cattle and to provide information about clinical course, lesions, and
suitability of currently used diagnostic procedures for detection of CWD
in cattle, 13 calves were inoculated intracerebrally with brain
suspension from mule deer naturally affected with CWD. Between 24 and 27
months postinoculation, 3 animals became recumbent and were euthanized.
Gross necropsies revealed emaciation in 2 animals and a large pulmonary
abscess in the third. Brains were examined for protease-resistant prion
protein (PrP(res)) by immunohistochemistry and Western blotting and for
scrapie-associated fibrils (SAFs) by negative-stain electron microscopy.
Microscopic lesions in the brain were subtle in 2 animals and absent in
the third case. However, all 3 animals were positive for PrP(res) by
immunohistochemistry and Western blot, and SAFs were detected in 2 of
the animals. An uninoculated control animal euthanized during the same
period did not have PrP(res) in its brain. These are preliminary
observations from a currently in-progress experiment. Three years after
the CWD challenge, the 10 remaining inoculated cattle are alive and
apparently healthy. These preliminary findings demonstrate that
diagnostic techniques currently used for bovine spongiform
encephalopathy (BSE) surveillance would also detect CWD in cattle should
it occur naturally.


P.S. the study above confirmed 5 cows and 1 goat transmission of CWD to cattle,
of the final stages of the study. ...tss

Published online before print March 20, 2001, 10.1073/pnas.041490898
Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt- Jakob disease: Implications for human health
Corinne Ida Lasmézas*,dagger, Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas KoppDagger , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce||, Dominique Dormont*, and Jean-Philippe Deslys*



One aim of this study was to determine the risk of secondary transmission to humans of vCJD, which is caused not by a primarily human strain of TSE agent but by the BSE strain having passed the species barrier to humans. This risk is tightly linked to the capacity of the BSE agent to adapt to primates and harbor enhanced virulence (i.e., induce disease after a short incubation period and provoke disease even if highly diluted) and to its pathogenicity after inoculation by the peripheral route. With respect to the latter, there are huge variations between different TSE agent strains and hosts. For example, the BSE agent is pathogenic to pigs after i.c. inoculation but not after oral administration (23). Thus, we wanted to know to what extent the BSE/vCJD agent is pathogenic to humans by the i.c. and i.v. routes. To achieve this, we used the macaque model. To monitor the evolution of the BSE agent in primates, but also to verify the identity of French vCJD, we conducted parallel transmission to C57BL/6 mice, allowing strain-typing. The experimental scheme is depicted in Fig. 1.

Characterization of the BSE Agent in Primates. The identity of the lesion profiles obtained from the brains of the French patient with vCJD, two British patients with vCJD, and nonhuman primates infected with BSE provides experimental demonstration of the fact that the BSE agent strain has been transmitted to humans both in the U.K. and in France. Further, it lends support to the validity of the macaque model as a powerful tool for the study of vCJD. As far as the evolution of the BSE agent in primates is concerned, we observed an interesting phenomenon: at first passage of BSE in macaques and with vCJD, there was a polymorphism of the lesion profile in mice in the hippocampal region, with about half of them harboring much more severe vacuolation than the mice inoculated with cattle BSE. At second passage, the polymorphism tended to disappear, with all mice showing higher vacuolation scores in the hippocampus than cattle BSE mice. This observation suggests the appearance of a variant of the BSE agent at first passage in primates and its clonal selection during second passage in primates. The lesion profiles showed that it was still the BSE agent, but the progressive appearance of a "hippocampal signature" hallmarked the evolution toward a variant by essence more virulent to primates.

Characterization of the CJD and Scrapie Strains. Controls were set up by transmitting one French and one U.S. scrapie isolate from ruminants as well as French sCJD and iCJD cases from humans. None of these revealed a lesion profile or transmission characteristics similar or close to those of BSE or vCJD, respectively, thus extending to the present French scrapie isolate the previous observation that the BSE agent was different from all known natural scrapie strains (4, 24).
The lesion profiles of sCJD and iCJD differed only slightly in severity of the lesions, but not in shape of the profile, revealing the identity of the causative agents. One of us reported the absence of similarity between sCJD (six cases) and U.K. scrapie (eight cases) in transmission characteristics in mice (4). Herein, we made the striking observation that the French natural scrapie strain (but not the U.S. scrapie strain) has the same lesion profile and transmission times in C57BL/6 mice as do the two human TSE strains studied. This strain "affiliation" was confirmed biochemically. There is no epidemiological evidence for a link between sheep scrapie and the occurrence of CJD in humans (25). However, such a link, if it is not a general rule, would be extremely difficult to establish because of the very low incidence of CJD as well as the existence of different isolates in humans and multiple strains in scrapie. Moreover, scrapie is transmissible to nonhuman primates (26). Thus, there is still a possibility that in some instances TSE strains infecting humans do share a common origin with scrapie, as pointed out by our findings.

Transmission of vCJD and BSE to Nonhuman Primates. vCJD transmitted readily to the cynomolgus macaque after 2 years of incubation, which was comparable to the transmission obtained from first-passaged macaque BSE and much shorter than the interspecies transmission of BSE. Starting with 100 mg of BSE-macaque brain material, dilutions up to 4 µg still provoked disease. These data suggest that the BSE agent rapidly adapts to primates accompanied by enhanced virulence.
Examination of macaque brain inoculated with vCJD revealed a similar pathology to that with second-passage BSE. The distribution of vacuolation and gliosis, as well as the pattern of PrP deposition, including the dense, sometimes florid plaques, were similar to the human vCJD and the BSE hallmarks of the first passage (1, 2). These data show that the phenotype of BSE in primates is conserved over two passages. Moreover, they confirm that the BSE agent behaves similarly in humans and macaques, a precious finding that will prove useful in the near future for the design of pathogenesis or therapeutic studies. Because of the number of macaques examined in this study, we can now reliably state that the pathology, in particular the PrP deposition pattern provoked by BSE, is similar in older and very young animals. However, plaque deposition is greater, and mature florid plaques were more numerous, in the young, which may be correlated with a longer duration of the clinical phase observed in this animal (2). This is important with regard to the fact that vCJD has been diagnosed mainly in teenagers and young adults, which raises the concern that older patients may have been misdiagnosed because of an alternative phenotype of the disease. One should bear in mind, however, that cynomolgus macaques are all homozygotes for methionine at codon 129 of the PrP gene. Thus, our observations may not be relevant to humans carrying one or both valine alleles; however, all patients with vCJD reported to date have been M/M at this position (27).

Intravenous Transmissions to Nonhuman Primates. Brain pathology was identical in macaques inoculated i.c. and i.v. The i.v. route proved to be very efficient for the transmission of BSE, as shown by the 2-year survival of the animals, which is only 5 months longer than that obtained after inoculating the same amount of agent i.c. As the i.v. injection of the infectious agent implies per se a delayed neuroinvasion compared with a direct inoculation in the brain, this slight lengthening of the incubation period cannot, at this stage, be interpreted as a lower efficiency of infection as regards the i.c. route.
These data should be taken into account in the risk assessment of iatrogenic vCJD transmission by i.v. administration of biological products of human origin. They also constitute an incentive for a complete i.v. titration.

From BSE and vCJD transmissions in nonhuman primates, a number of conclusions can be drawn that are of major importance for human health: (i) human-adapted BSE appears to be a variant of the BSE agent that is more virulent for humans than cattle BSE and is efficiently transmitted by the peripheral route; (ii) the detection of vCJD in unusually young patients is probably not because of a lack of diagnosis of cases in older patients, thus raising the question of the source of human contamination with BSE early in life; and (iii) iatrogenic transmissions from patients with vCJD would be readily recognized by using the same diagnostic criteria as those applied to vCJD [clinical and pathological criteria (27) comprising neuronal loss and gliosis in the thalamus correlated with high MRI signal (28, 29)], whether such contaminations had occurred by the central or i.v. route. Primary and iatrogenic cases of vCJD could be distinguished on the basis of the patient's clinical history.

The risk assessment of biological products of human origin, notably those derived from blood, has been deeply modified by the appearance of vCJD. We confirm that the BSE agent has contaminated humans not only in the U.K. and the Republic of Ireland but also in France, and we show that its pathogenic properties for primates are being enhanced by a primary passage in humans. Considering the flow of potentially contaminated bovine-derived products between 1980 and 1996, it is obvious that further vCJD cases may occur outside the U.K. Thus, and in the light of the present study, it is necessary to sustain worldwide CJD surveillance regardless of national BSE incidence and to take all precautionary measures to avoid iatrogenic transmissions from vCJD.



MRC-43-00 [ ] [Text only version of this site] [Print this page]
Issued: Monday, 28 August 2000

A team of researchers led by Professor John Collinge at the Medical Research Council Prion Unit1 report today in the Proceedings of the National Academy of Sciences, on new evidence for the existence of a ?sub-clinical? form of BSE in mice which was unknown until now.

The scientists took a closer look at what is known as the ?species barrier? - the main protective factor which limits the ability of prions2 to jump from one species to infect another. They found the mice had a ?sub-clinical? form of disease where they carried high levels of infectivity but did not develop the clinical disease during their normal lifespan. The idea that individuals can carry a disease and show no clinical symptoms is not new. It is commonly seen in conventional infectious diseases.

Researchers tried to infect laboratory mice with hamster prions3 called Sc237 and found that the mice showed no apparent signs of disease. However, on closer inspection they found that the mice had high levels of mouse prions in their brains. This was surprising because it has always been assumed that hamster prions could not cause the disease in mice, even when injected directly into the brain.

In addition the researchers showed that this new sub-clinical infection could be easily passed on when injected into healthy mice and hamsters.

The height of the species barrier varies widely between different combinations of animals and also varies with the type or strain of prions. While some barriers are quite small (for instance BSE easily infects mice), other combinations of strain and species show a seemingly impenetrable barrier. Traditionally, the particular barrier studied here was assumed to be robust.

Professor John Collinge said: "These results have a number of important implications. They suggest that we should re-think how we measure species barriers in the laboratory, and that we should not assume that just because one species appears resistant to a strain of prions they have been exposed to, that they do not silently carry the infection. This research raises the possibility, which has been mentioned before, that apparently healthy cattle could harbour, but never show signs of, BSE.

"This is a timely and unexpected result, increasing what we know about prion disease. These new findings have important implications for those researching prion disease, those responsible for preventing infected material getting into the food chain and for those considering how best to safeguard health and reduce the risk that theoretically, prion disease could be contracted through medical and surgical procedures."




Professor Collinge is a consultant neurologist and Director of the newly formed MRC Prion Unit based at The Imperial College School of Medicine at St Mary?s Hospital. He is also a member of the UK Government?s Spongiform Encephalopathy Advisory Committee (SEAC). The MRC prion unit is was set up in 1999, and its work includes molecular genetic studies of human prion disease and transgenic modelling of human prion diseases.

Prions are unique infectious agents that cause fatal brain diseases such as Creutzfeldt-Jakob disease (CJD) in humans and scrapie and BSE (mad cow disease) in animals. In some circumstances prions from one species of animals can infect another and it is clear that BSE has done this to cause the disease variant CJD in the UK and France. It remains unclear how large an epidemic of variant CJD will occur over the years ahead.

The strain of prion used here to infect the mice is the Sc237 strain (also known as 263K) which infects hamsters, and until now was assumed not to infect mice.

This research was funded by the Medical Research Council and Wellcome Trust.

The Medical Research Council (MRC) is a national organisation funded by the UK tax-payer. Its business is medical research aimed at improving human health; everyone stands to benefit from the outputs. The research it supports and the scientists it trains meet the needs of the health services, the pharmaceutical and other health-related industries and the academic world. MRC has funded work which has led to some of the most significant discoveries and achievements in medicine in the UK. About half of the MRC?s expenditure of £345 million is invested in over 50 of its Institutes and Units, where it employs its own research staff. The remaining half goes in the form of grant support and training awards to individuals and teams in universities and medical schools.

The Wellcome Trust is the world's largest medical research charity with a spend of some £600 million in the current financial year 1999/2000. The Wellcome Trust supports more than 5,000 researchers, at 400 locations, in 42 different countries to promote and foster research with the aim of improving human and animal health. As well as funding major initiatives in the public understanding of science, the Wellcome Trust is the country's leading supporter of research into the history of medicine.

©2002 Medical Research Council


and for Gods sake, if someone is smearing this [email protected] all over there kids
heads for lice and did not come up with a TSE, i would say this is good case study;

1) None of our animals that contracted BSE were treated with OP's, even
in utero.
2) My kids were treated with OP's as infants to control head lice. This
seems to be endemic as infection waves in UK primary schools (and
possibly elsewhere).
3) One might argue if the continued use of british beef in the UK was
ethical, none the less it happened. We have a duty to learn from it, not
least a duty to learn on behalf of those people who died so horribly....

NOPE, greed and money is the name of the game, they have known for decades;



To minimise the risk of farmers' claims for compensation from feed

To minimise the potential damage to compound feed markets through adverse publicity.

To maximise freedom of action for feed compounders, notably by
maintaining the availability of meat and bone meal as a raw
material in animal feeds, and ensuring time is available to make any
changes which may be required.




MAFF remains under pressure in Brussels and is not skilled at
handling potentially explosive issues.

5. Tests _may_ show that ruminant feeds have been sold which
contain illegal traces of ruminant protein. More likely, a few positive
test results will turn up but proof that a particular feed mill knowingly
supplied it to a particular farm will be difficult if not impossible.

6. The threat remains real and it will be some years before feed
compounders are free of it. The longer we can avoid any direct
linkage between feed milling _practices_ and actual BSE cases,
the more likely it is that serious damage can be avoided. ...

SEE full text ;


BUT, i applaud ranchers that understand the TSE science and not the hype that surrounds it such as 'reader the second' ;

>>>Look, Mark Purdey, I applaud you for your perserverance, your bravery in taking on the establishment, and your creativity. It may well be that you were instrumental in bringing the familial and other genetic clusters in Slovenia to the attention of science.

I acknowledge that you suffered but your suffering sir is NOTHING compared to the suffering of the families afflicted with CJD and variant CJD and to the extent that your writings are used to ignore public health threats and to avoid taking public health measures that may well protect humans from CJD, then your writings are being ill used. You should acknowledge that and if you do not, I will pay no further attention to you.

Your supporters use your writings to (1) deny that CJD is transmissable from person to person, e.g., via contaminated surgical instruments or tissue or blood donations; (2) reject totally the strong hypothesis that variant CJD is due to the BSE epidemic in the UK being "pushed" over the species barrier. They claim that all cases of human CJD are due to metal contamination. It is they who are perverting your theories and therefore subjecting you to ridicule. Unless you deny these things as well and in which case, you should be ignored since in the case of (1) you deny fact and in the case of (2) you reject a hypothesis that must be accepted for the time being and must lead our public health measures given its strength.

NO ONE has denied that the ultimate cause of TSEs is unknown. However TSEs ARE transmissable between species, albeit not easily, and they are certainly transmissable among the same species. The suffering of the individual and his/her family who contracts CJD or variant CJD is hideous beyond description. If TSEs are your life work, you had better be taking that into consideration.<<<

>>>This is a danger to ranchers and to all humans<<<

INDEED there is;




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


cover-up of 4th farm worker ???





now story changes from;

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


This is not unexpected...

was another farmer expected?


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
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,
that in the general population...


and for those that believe this was a spontaneous event or a happen-stance of bad luck from a spontatneous misfolding PrP, well, dream on. ...TSS

TSS submissions to federal dockets and peer review journals
Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION







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




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


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

Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed

Comment Number: EC -10

Accepted - Volume 2





File Format: PDF/Adobe Acrobat -

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

Sr. [[email protected]] Monday, January 08,200l 3:03 PM freas ...


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;


Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice
in Manufacturing, Packing, or Holding Dietary Ingredients a
Comment Number: EC -2
Accepted - Volume 7


[PDF] Appendices to PL107-9 Inter-agency Working Group Final Report 1-1
File Format: PDF/Adobe Acrobat - View as HTML
Agent, Weapons of Mass Destruction Operations Unit Federal Bureau of
those who provided comments in response to Docket No. ...
Meager 8/18/01 Terry S. Singeltary Sr ...


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


# Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of
TSS 1/27/03 (0)

Docket Management

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


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

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


Daily Dockets Entered on 02/05/03

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

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


Docket Management

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

Comment Number: EC -1

Accepted - Volume 1




Daily Dockets - 04/10/03

... 00D-1662 Use of Xenotransplantation Products in Humans.
EMC 98 Terry S. Singeltary Sr. Vol#: 3. 01F ...
http://www.fda.gov/ohrms/dockets/dailys/03/Apr03/041003/041003.htm - 05-20-2003
- Cached

Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

Terry S. Singeltary Sr.
Vol #:


Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

Terry S. Singeltary Sr.
Vol #:

Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

Terry S. Singeltary Sr.
Vol #:


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

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

APE 6 Animal Protein Producers Industry Vol#: 2

APE 7 Darling International Inc. Vol#: 2

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


Send Post-Publication Peer Review to journal:

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

disease in the United States

Email Terry S. Singeltary:

[email protected]

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?



Volume 3, Number 8 01 August 2003


Tracking spongiform encephalopathies in North America

Xavier Bosch

My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost

my mom to hvCJD (Heidenhain variant CJD) and have been searching for

answers ever since. What I have found is that we have not been told the

truth. CWD in deer and elk is a small portion of a much bigger problem.

49-year-old Singeltary is one of a number of people who have remained

largely unsatisfied after being told that a close relative died from a

rapidly progressive dementia compatible with spontaneous

Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of

documents on transmissible spongiform encephalopathies (TSE) and

realised that if Britons could get variant CJD from bovine spongiform

encephalopathy (BSE), Americans might get a similar disorder from

chronic wasting disease (CWD)the relative of mad cow disease seen among

deer and elk in the USA. Although his feverish search did not lead him

to the smoking gun linking CWD to a similar disease in North American

people, it did uncover a largely disappointing situation.

Singeltary was greatly demoralised at the few attempts to monitor the

occurrence of CJD and CWD in the USA. Only a few states have made CJD

reportable. Human and animal TSEs should be reportable nationwide and

internationally, he complained in a letter to the Journal of the

American Medical Association (JAMA 2003; 285: 733). I hope that the CDC

does not continue to expect us to still believe that the 85% plus of all

CJD cases which are sporadic are all spontaneous, without route or source.

Until recently, CWD was thought to be confined to the wild in a small

region in Colorado. But since early 2002, it has been reported in other

areas, including Wisconsin, South Dakota, and the Canadian province of

Saskatchewan. Indeed, the occurrence of CWD in states that were not

endemic previously increased concern about a widespread outbreak and

possible transmission to people and cattle.

To date, experimental studies have proven that the CWD agent can be

transmitted to cattle by intracerebral inoculation and that it can cross

the mucous membranes of the digestive tract to initiate infection in

lymphoid tissue before invasion of the central nervous system. Yet the

plausibility of CWD spreading to people has remained elusive.

Part of the problem seems to stem from the US surveillance system. CJD

is only reported in those areas known to be endemic foci of CWD.

Moreover, US authorities have been criticised for not having performed

enough prionic tests in farm deer and elk.

Although in November last year the US Food and Drug Administration

issued a directive to state public-health and agriculture officials

prohibiting material from CWD-positive animals from being used as an

ingredient in feed for any animal species, epidemiological control and

research in the USA has been quite different from the situation in the

UK and Europe regarding BSE.

Getting data on TSEs in the USA from the government is like pulling

teeth, Singeltary argues. You get it when they want you to have it,

and only what they want you to have.

Norman Foster, director of the Cognitive Disorders Clinic at the

University of Michigan (Ann Arbor, MI, USA), says that current

surveillance of prion disease in people in the USA is inadequate to

detect whether CWD is occurring in human beings; adding that, the

cases that we know about are reassuring, because they do not suggest the

appearance of a new variant of CJD in the USA or atypical features in

patients that might be exposed to CWD. However, until we establish a

system that identifies and analyses a high proportion of suspected prion

disease cases we will not know for sure. The USA should develop a

system modelled on that established in the UK, he points out.

Ali Samii, a neurologist at Seattle VA Medical Center who recently

reported the cases of three hunterstwo of whom were friendswho died

from pathologically confirmed CJD, says that at present there are

insufficient data to claim transmission of CWD into humans; adding that

[only] by asking [the questions of venison consumption and deer/elk

hunting] in every case can we collect suspect cases and look into the

plausibility of transmission further. Samii argues that by making both

doctors and hunters more aware of the possibility of prions spreading

through eating venison, doctors treating hunters with dementia can

consider a possible prion disease, and doctors treating CJD patients

will know to ask whether they ate venison.

CDC spokesman Ermias Belay says that the CDC will not be investigating

the [Samii] cases because there is no evidence that the men ate

CWD-infected meat. He notes that although the likelihood of CWD

jumping the species barrier to infect humans cannot be ruled out 100%

and that [we] cannot be 100% sure that CWD does not exist in humans&

the data seeking evidence of CWD transmission to humans have been very



he complained in a letter to the Journal of the American Medical

Association (JAMA 2003; 285: 733). I hope that the CDC does not

continue to expect us to still believe that the 85% plus of all CJD

cases which are sporadic are all spontaneous, without route or source.<<<

actually, that quote was from a more recent article in the Journal of

Neurology (see below), not the JAMA article...

Full Text

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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









BY Philip Yam

Yam Philip Yam News Editor Scientific American www.sciam.com

IN light of Asante/Collinge et al findings that BSE transmission to the
129-methionine genotype can lead to an alternate phenotype that is
indistinguishable from type 2 PrPSc, the commonest _sporadic_ CJD;

-------- Original Message -------- Subject: re-BSE prions propagate as

either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43

-0000 From: "Asante, Emmanuel A" To:
"'[email protected]'"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer's version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best wishes.

Emmanuel Asante

<> ____________________________________

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:

[email protected] (until 9/12/02)

New e-mail: [email protected] (active from now)



full text ;


AND the new findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic

Creutzfeldt-Jakob disease


Adaptation of the bovine spongiform encephalopathy agent to primates
and comparison with Creutzfeldt- Jakob disease: Implications for
human health

THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*,
Virginie Nouvel*,

Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger

] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique

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;


Characterization of two distinct prion strains
derived from bovine spongiform encephalopathy
transmissions to inbred mice


ALL animals for human/animal consumption must be tested for TSE.

ALL human TSEs must be made reportable Nationally and Internationally, OF ALL AGES...TSS

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