National Scrapie Eradication Program February 2014 Monthly Report Fiscal 
Year 2014
RSSS started April 1, 2003. It is a targeted slaughter surveillance program 
which is designed to identify infected flocks. Samples have been collected from 
403,213 animals since April 1, 2003. There have been 470 NVSL confirmed positive 
animals** (462 classical cases and 8 Nor98-like cases) since the beginning of 
RSSS. As of November 30, 2013, 5,301 samples have been collected in FY 2014, 
1,335 of which were from goats. As of February 28, 2014, 1 white-faced sheep has 
tested positive for scrapie in FY 2014. The percentage of samples that have 
tested positive for each face color from FY 2003 through FY 2014 is depicted in 
Chart 3. In November 2013, administrative units within APHIS Veterinary Services 
reorganized from 2 Regions to 6 Districts (Figure 1). Cumulative district sample 
collection numbers are shown in Chart 4 and are based upon the State in which 
the animal was tagged. The number of animals collected for FY 2014 by month and 
by district where collected is shown in Chart 5. A monthly comparison of RSSS 
collections by fiscal year is displayed in Chart 6. Chart 7 is a retrospective 
6-month rolling average of the percent positive, black-faced sheep sampled at 
RSSS collection sites.
*RSSS and On-farm surveillance data are not available for December through 
February due to migration of these data to a new database.
**RSSS positives are reported based on collection date and may have been 
confirmed after February 28, 2014. 
Testing of animals in the field is an essential part of scrapie 
surveillance, and it includes both regulatory field cases and live-animal 
testing. As the National Scrapie Eradication Program moves closer towards 
meeting the goal of identifying the last remaining cases of classical scrapie, 
finding and testing all sheep and goats meeting targeted sampling criteria is 
even more important. As of November 30, 2013, 439 sheep and 63 goats have been 
tested on-farm for FY 2014. As of February 28, 2014, 15 sheep and 7 goats have 
tested positive. The number of animals tested on-farm by month and by species 
for FY 2014 is shown in Chart 8. 
• 5,301 RSSS samples and 502 on-farm samples [includes regulatory testing 
(necropsy and live-animal) and on-farm surveillance] (Chart 9);
• Of which 4,405 were sheep and 1,398 were goats. 
Distribution of sampling by type (RSSS or on-farm) and by species is shown 
in Chart 10. 
*RSSS and On-farm surveillance data are not available for December through 
February due to migration of these data to a new database. 
One positive white-faced sheep tested through RSSS has been reported in FY 
2014.* Fifteen additional sheep (Finn sheep) from the flock of origin of the 
RSSS positive also tested positive for scrapie (Table 1 and Figure 2).
The number of confirmed positive cases in goats since FY 2002 is 41. The 
most recent cases were reported in February 2014; all animals were from the same 
goat herd and were commingled with sheep in a previously identified infected 
flock. (Table 1 and Figure 3).
As of February 28, 2014, there were 4 flocks with an open infected or 
source status
(Figure 4). Three new infected flocks and 1 new source flock have been 
designated in 2014 (Figure 5). Six flocks have completed flock plans since the 
beginning of FY 2014 (Figure 6). New infected and source statuses from FY 1997 
to FY 2014 are depicted in Chart 2.
* Samples collected between October 1, 2013 and February 28, 2014 and 
confirmed by March 17, 2014.
snip...
* Through November 30, 2013—Adjusted to exclude multiple positive animals 
from the same flock. Does not include Nor98-like scrapie cases found through 
RSSS (2 in FY 2007, 1 in FY 2008, 4 in FY 2010, 1 in FY 2011). The increase in 
FY 2014 is not statistically meaningful due to small sample size.
snip...
Iowa progress: Until this year, Iowa’s last case of Scrapie was found in 
July 2010. This fall Iowa identified 1 new Source and 4 new infected flocks in 
NW Iowa. The 4 Infected flocks occurred as a result of sales of breeding sheep 
out of the Source flock to other sheep producers. Flock cleanup is ongoing in 
these flocks. There have been a total of 82 sheep flocks in Iowa that have been 
found to be infected with Scrapie since the accelerated National Scrapie 
Eradication Program (NSEP) started in November 2001. In Fiscal Year 2005, Iowa 
had a high of 15 newly found Source or Infected flocks.
Bovine spongiform encephalopathy in goats could be misdiagnosed as scrapie 
in the absence of appropriate discriminatory tests, and such misidentification 
occurred at least once before such tests were developed, according to a report 
released in December. 
The article, "Isolation of prion with BSE properties from farmed goat" 
(Emerging Infectious Diseases 2011;17:2253-2261), indicates BSE can affect small 
ruminants under natural conditions and that the condition can be misdiagnosed. 
The agent that causes scrapie is not known to infect humans, but consumption of 
beef contaminated with the prions that cause BSE is connected with variant 
Creutzfeldt-Jakob disease, a neurodegenerative disorder in humans. 
 The report calls for continued extensive surveillance and breeding plans 
to prevent BSE outbreaks among small ruminants. Such outbreaks could harm public 
health. 
The authors stated in the text that the misdiagnosis occurred in 1990 in 
the United Kingdom. The case had been identified as suspected BSE in 2006 
because differential immunohistochemical analysis of fixed brain tissue produced 
a signature indistinguishable from BSE. The authors of the recent report used a 
bioassay to confirm the BSE diagnosis. 
The sample collected in 1990 was among 26 historic samples collected from 
1984-2002, the report states. 
The report indicates the U.K. goat and a goat in France found to have BSE 
in 2005 both likely became infected through contaminated food supplements. 
While BSE lesions are contained mainly within nervous tissue in cattle, the 
report states "in small ruminants the BSE agent is widely distributed in 
peripheral tissues and can be transmitted horizontally." Feed ban measures alone 
would be insufficient for controlling a BSE outbreak in small ruminants, 
according to the report. 
"Also, it would be impossible to prevent BSE from entering the human food 
chain through consumption of food products derived from small ruminants," the 
report states. 
John Spiropoulos , Richard Lockey, Rosemary E. Sallis, Linda A. Terry, 
Leigh Thorne, Thomas M. Holder, Katy E. Beck, and Marion M. Simmons 
Author affiliations: Animal Health and Veterinary Laboratories Agency, 
Weybridge, Surrey, UK 
Transmissible spongiform encephalopathies are fatal neurodegenerative 
diseases that include variant Creutzfeldt-Jakob disease in humans, scrapie in 
small ruminants, and bovine spongiform encephalopathy (BSE) in cattle. Scrapie 
is not considered a public health risk, but BSE has been linked to variant 
Creutzfeldt-Jakob disease. Small ruminants are susceptible to BSE, and in 2005 
BSE was identified in a farmed goat in France. We confirm another BSE case in a 
goat in which scrapie was originally diagnosed and retrospectively identified as 
suspected BSE. The prion strain in this case was further characterized by mouse 
bioassay after extraction from formaldehyde-fixed brain tissue embedded in 
paraffin blocks. Our data show that BSE can infect small ruminants under natural 
conditions and could be misdiagnosed as scrapie. Surveillance should continue so 
that another outbreak of this zoonotic transmissible spongiform encephalopathy 
can be prevented and public health safeguarded. 
snip... 
We confirmed that the agent responsible for TSE in a UK goat, which was 
initially reported as scrapie in 1990 and subsequently as suspected BSE in 2006 
(16), was a BSE agent. This conclusion was based on bioassay of nervous tissue 
in mice demonstrating similarities of histopathologic lesions, PrPSc mapping in 
the brain, and WB of PrPSc with those of mice inoculated with BSE from various 
ovine, caprine, and bovine sources. 
From a method perspective, the data suggest that AR, IP, and LP are not 
optimal bioassay parameters for differentiating TSE sources during first passage 
because they represent mean values derived from a group of animals that have 
been inoculated with a specific source. Therefore, a substantial number of 
animals must die of clinical TSE for these parameters to be meaningful. This 
finding is a limiting factor in instances in which TSE is diagnosed in only a 
few animals because of low titer, restricted permissiveness of specific TSE 
strains in certain laboratory animals, or both. These limitations can be 
overcome by application of IHC and WB to differentiate BSE from scrapie 
confidently in individual mice on first passage. Use of IHC has shown that 
different PrPSc deposits can be identified, and the distribution of each deposit 
in the brain can be mapped (22,28,32). This approach generates high-resolution 
data that appear to be specific to individual TSE strains. 
The data show that the TSE agents in this study were not altered by the 
adverse conditions applied to them during histologic procedures. However, titer 
may decrease, suggesting that the effect of histologic processing is 
quantitative not qualitative. Therefore, bioassay is a valid approach for 
identifying BSE in archived histologic material when other techniques are not 
applicable, as in the current study. Regarding the suitability of different 
mouse lines for confirming BSE, our data show that any mouse line in which the 
agent can propagate sufficiently is suitable. An additional requirement at a 
practical level is the ability to characterize the agent on first passage. In 
this respect, use of PrP-a mice is preferable because in addition to AR, IP, 
histopathologic analysis, and PrPSc patterning, WB can also be applied to 
diagnose BSE. In contrast, its application in PrP-b mice is less informative 
(33). 
These methods can also be applied to analyze bioassay data derived from 
validated transgenic mouse lines that offer the advantage of higher AR and 
decreased IP, provided that appropriate transgenic lines are selected and the 
TSE source and the donor species under investigation are taken into 
consideration. In this particular instance, our first choices would have been 
the use of a mouse line overexpressing a bovine transgene in combination with 1 
that overexpresses a caprine transgene. At initiation of the study, an 
established bovinised line was not available to us, and the data generated from 
the wild-type mice were considered sufficient to identify unequivocally the 
agent strain. Caprine transgenic mouse lines are still under development and not 
characterized or widely available. Instead, we used tg338 mice although they 
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The 2 cases of naturally occurring BSE in small ruminants—the 1 reported 
here and the 1 identified in France (15)—occurred in different countries, during 
different time periods, and before strict BSE control measures were fully 
implemented. Therefore, the most likely origin of these 2 cases would be 
exposure to BSE-contaminated food supplements. Although in France goats 
constitute 14.3% of the small ruminant population, in the United Kingdom they 
account for only 0.3% of small ruminants. It is intriguing, therefore, that the 
only naturally occurring BSE cases in small ruminants in France and particularly 
in the United Kingdom were detected in goats and not in sheep, although they 
have also been exposed to contaminated food supplements. A possible explanation 
could be that goats are generally managed more intensively than sheep and thus 
might have been exposed to higher doses of the infectious agent because of the 
more frequent use of concentrates in intensive dairy farming. Similar 
observations have been reported in cattle, in which the incidence of BSE was 
significantly higher in dairy herds and in which management is much more 
intensive than in beef herds (34). In the United Kingdom, most of the commercial 
goat herds are kept for milk production in a typically intensive production 
system, similar to dairy cattle. 
 
The BSE case we have confirmed was 1 of 26 historic goat samples examined 
in the United Kingdom collected during 1984–2002 (16,17). Since 1993, scrapie in 
goats has been a notifiable disease in the United Kingdom, and since 2005, 
samples from all suspected cases of TSE in small ruminants are required to be 
tested for BSE-like features by using WB (19). No BSE cases have been 
identified, although an intermediate case in a goat was reported and is under 
investigation by bioassay for final resolution (35,36). This screening of brain 
samples from all small ruminant cases offers reassurance that BSE is not present 
in the contemporary small ruminant population. However, application of WB to 
sheep experimentally co-infected with BSE and scrapie detected only the scrapie 
agent (37). Also, in contrast to BSE, where infectivity is mainly confined to 
the nervous system, in small ruminants the BSE agent is widely distributed in 
peripheral tissues and can be transmitted horizontally (11,38). Therefore, feed 
ban measures alone would be inadequate to control a BSE outbreak in small 
ruminants. Also, it would be impossible to prevent BSE from entering the human 
food chain through consumption of food products derived from small ruminants. 
 
Because TSEs in goats are still a problem, particularly in Mediterranean 
countries, our data suggest that extensive surveillance and breeding schemes 
must remain in place to prevent a BSE outbreak in small ruminants and to 
safeguard public health. This report also highlights several issues regarding 
the use of mouse bioassay to identify TSE strains. As governing bodies seek 
confirmation of equivocal cases that are identified worldwide, they must be 
aware of the limitations, cost, and timescale demands of confirming such cases. 
 
Dr Spiropoulos is a veterinary researcher at Veterinary Laboratories Agency 
with a particular interest in animal pathology. He is the head of the Mouse 
Bioassay Team that specializes in pathology of experimental animals. His 
research interests include neurodegenerative disorders and animal diseases of 
policy relevance, particularly zoonoses. 
 
Acknowledgments 
 
We thank John Sheehan for tissue retrieval from wax-impregnated tissue 
blocks; Angel Ortiz-Pelaez for epidemiologic assistance; histopathology 
employees at Veterinary Laboratories Agency for expert technical support in 
histopathology and immunohistochemistry; and Animal Services Unit employees at 
Veterinary Laboratories Agency for expert support with animal procedures and 
care. 
 
This work was supported by a Department of Environment, Food and Rural 
Affairs grant (project SE1849). 
 
 
 
Saturday, December 3, 2011 
 
Isolation of Prion with BSE Properties from Farmed Goat Volume 17, Number 
 
12—December 2011 
 
 
snip... 
 
Scrapie Nor-98 like case in California FY 2011 AS of December 31, 2010. 
 
Scrapie cases in goats FY 2002 - 2011 AS of December 31, 2010 Total goat 
cases = 21 Scrapie cases, 0 Nor-98 like Scrapie cases (21 field cases, 0 RSSS 
cases) 
 
Last herd with infected goats disignated in FY 2008 Michigan 8 cases 
 
 
UPDATE PLEASE NOTE ; 
 
AS of June 30, 2011, 
 
snip... 
 
INCLUDING 10 POSITIVE GOATS FROM THE SAME HERD (FIGURE 7). 
 
snip... 
 
see updated APHIS scrapie report ; 
 
 
Tuesday, February 01, 2011 
 
Sparse PrP-Sc accumulation in the placentas of goats with naturally 
acquired scrapie 
 
Research article 
 
snip... 
 
Date: Tuesday, February 01, 2011 5:03 PM 
 
To: Mr Terry Singeltary 
 
Subject: Your comment on BMC Veterinary Research 2011, 7:7 
 
Dear Mr Singeltary 
 
Thank you for contributing to the discussion of BMC Veterinary Research 
2011, 7:7 . 
 
Your comment will be posted within 2 working days, as long as it 
contributes to the topic under discussion and does not breach patients' 
confidentiality or libel anyone. You will receive a further notification by 
email when the posting appears on the site or if it is rejected by the 
moderator. 
 
Your posting will read: 
 
Mr Terry Singeltary, retired Scrapie cases Goats from same herd USA 
Michigan 
 
Comment: " In spite of the poorly defined effects of PRNP genetics, scrapie 
strain, dose, route and source of infection, the caprine placenta may represent 
a source of infection to progeny and herd mates as well as a source of 
persistent environmental contamination. " 
 
Could this route of infection be the cause of the many cases of Goat 
scrapie from the same herd in Michigan USA ? 
 
Has this been investigated ? 
 
(Figure 6) including five goat cases in FY 2008 that originated from the 
same herd in Michigan. This is highly unusual for goats, and I strenuously urge 
that there should be an independent investigation into finding the common 
denominator for these 5 goats in the same herd in Michigan with Scrapie. ... 
 
Kind Regards, Terry 
 
Thursday, January 07, 2010 
 
Scrapie and Nor-98 Scrapie November 2009 Monthly Report Fiscal Year 2010 
and FISCAL YEAR 2008 
 
 
In FY 2010, 72 cases of classical Scrapie and 5 cases of Nor-98 like 
Scrapie were confirmed... 
 
 
Scrapie Nor-98 like case in California FY 2011 AS of December 31, 2010. 
 
Scrapie cases in goats FY 2002 - 2011 AS of December 31, 2010 Total goat 
cases = 21 Scrapie cases, 0 Nor-98 like Scrapie cases (21 field cases, 0 RSSS 
cases) 
 
Last herd with infected goats disignated in FY 2008 Michigan 8 cases 
 
 
Thursday, November 18, 2010 
 
Increased susceptibility of human-PrP transgenic mice to bovine spongiform 
encephalopathy following passage in sheep 
 
 
Monday, November 30, 2009 
 
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH 
CODE 
 
 
atypical scrapie just MAY be contagious, and MAY, IN FACT, NOT be a 
spontaneous degenerative condition of older sheep, AND with science transmission 
studies to date, there is more evidence that typical scrapie MAY transmit to 
man. and to imagine that the USDA and the OIE now base their scientific human 
and animal risk factors on MAY FACTORS, is really unbelieveable, unacceptable, 
and shows just how corrupt this global TSE livestock food system is, thanks to 
the OIE and the USDA. ...TSS 
 
P03.141 
 
Aspects of the Cerebellar Neuropathology in Nor98 
 
Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National 
Veterinary Insitute, Sweden; 2National Veterinary Institute, 
 
Norway Nor98 is a prion disease of old sheep and goats. This atypical form 
of scrapie was first described in Norway in 1998. Several features of Nor98 were 
shown to be different from classical scrapie including the distribution of 
disease associated prion protein (PrPd) accumulation in the brain. The 
cerebellum is generally the most affected brain area in Nor98. The study here 
presented aimed at adding information on the neuropathology in the cerebellum of 
Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A 
panel of histochemical and immunohistochemical (IHC) stainings such as IHC for 
PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers 
for phagocytic cells were conducted. The type of histological lesions and tissue 
reactions were evaluated. The types of PrPd deposition were characterized. The 
cerebellar cortex was regularly affected, even though there was a variation in 
the severity of the lesions from case to case. Neuropil vacuolation was more 
marked in the molecular layer, but affected also the granular cell layer. There 
was a loss of granule cells. Punctate deposition of PrPd was characteristic. It 
was morphologically and in distribution identical with that of synaptophysin, 
suggesting that PrPd accumulates in the synaptic structures. PrPd was also 
observed in the granule cell layer and in the white matter.  The pathology 
features of Nor98 in the cerebellum of the affected sheep showed similarities 
with those of sporadic Creutzfeldt-Jakob disease in humans. 
 
***The pathology features of Nor98 in the cerebellum of the affected sheep 
showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans. 
 
 
PR-26 
 
 NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS 
 
 R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B. 
Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto 
Superiore di Sanità, Department of Food Safety and Veterinary Public Health, 
Rome, Italy (romolo.nonno@iss.it); 2 Istituto Zooprofilattico della Sardegna, 
Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo, 
Norway 
 
 Molecular variants of PrPSc are being increasingly investigated in sheep 
scrapie and are generally referred to as "atypical" scrapie, as opposed to 
"classical scrapie". Among the atypical group, Nor98 seems to be the best 
identified. We studied the molecular properties of Italian and Norwegian Nor98 
samples by WB analysis of brain homogenates, either untreated, digested with 
different concentrations of proteinase K, or subjected to enzymatic 
deglycosylation. The identity of PrP fragments was inferred by means of 
antibodies spanning the full PrP sequence. We found that undigested brain 
homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11), 
truncated at both the C-terminus and the N-terminus, and not N-glycosylated. 
After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and 
N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11. 
Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are 
mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at 
the highest concentrations, similarly to PrP27-30 associated with classical 
scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment 
of 17 kDa with the same properties of PrP11, that was tentatively identified as 
a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in 
2% sodium laurylsorcosine and is mainly produced from detergentsoluble, 
full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a 
sample with molecular and pathological properties consistent with Nor98 showed 
plaque-like deposits of PrPSc in the thalamus when the brain was analysed by 
PrPSc immunohistochemistry. Taken together, our results show that the 
distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids 
~ 90-155. This fragment is produced by successive N-terminal and C-terminal 
cleavages from a full-length and largely detergent-soluble PrPSc, is produced in 
vivo and is extremely resistant to PK digestion. 
 
 *** Intriguingly, these conclusions suggest that some pathological 
features of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease. 
 
 119 
 
 
A newly identified type of scrapie agent can naturally infect sheep with 
resistant PrP genotypes 
 
Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne 
Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?, 
Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,? +Author Affiliations 
 
*Virologie Immunologie Moléculaires and ?Génétique Biochimique et 
Cytogénétique, Institut National de la Recherche Agronomique, 78350 
Jouy-en-Josas, France; ?Unité Mixte de Recherche, Institut National de la 
Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions Hôte 
Agent Pathogène, 31066 Toulouse, France; §Agence Française de Sécurité Sanitaire 
des Aliments, Unité Agents Transmissibles Non Conventionnels, 69364 Lyon, 
France; **Pathologie Infectieuse et Immunologie, Institut National de la 
Recherche Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, 
National Veterinary Institute, 0033 Oslo, Norway 
 
 ***Edited by Stanley B. Prusiner, University of California, San Francisco, 
CA (received for review March 21, 2005) 
 
 Abstract Scrapie in small ruminants belongs to transmissible spongiform 
encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative 
disorders that affect humans and animals and can transmit within and between 
species by ingestion or inoculation. Conversion of the host-encoded prion 
protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP 
(PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified 
surveillance of scrapie in the European Union, together with the improvement of 
PrPSc detection techniques, has led to the discovery of a growing number of 
so-called atypical scrapie cases. These include clinical Nor98 cases first 
identified in Norwegian sheep on the basis of unusual pathological and PrPSc 
molecular features and "cases" that produced discordant responses in the rapid 
tests currently applied to the large-scale random screening of slaughtered or 
fallen animals. Worryingly, a substantial proportion of such cases involved 
sheep with PrP genotypes known until now to confer natural resistance to 
conventional scrapie. Here we report that both Nor98 and discordant cases, 
including three sheep homozygous for the resistant PrPARR allele (A136R154R171), 
efficiently transmitted the disease to transgenic mice expressing ovine PrP, and 
that they shared unique biological and biochemical features upon propagation in 
mice. 
 
*** These observations support the view that a truly infectious TSE agent, 
unrecognized until recently, infects sheep and goat flocks and may have 
important implications in terms of scrapie control and public health. 
 
 
Monday, December 1, 2008 
 
When Atypical Scrapie cross species barriers 
 
Authors 
 
Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon 
S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J. 
M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France; 
ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex, 
France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway, 
INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France. 
 
Content 
 
Atypical scrapie is a TSE occurring in small ruminants and harbouring 
peculiar clinical, epidemiological and biochemical properties. Currently this 
form of disease is identified in a large number of countries. In this study we 
report the transmission of an atypical scrapie isolate through different species 
barriers as modeled by transgenic mice (Tg) expressing different species PRP 
sequence. 
 
 The donor isolate was collected in 1995 in a French commercial sheep 
flock. inoculation into AHQ/AHQ sheep induced a disease which had all 
neuro-pathological and biochemical characteristics of atypical scrapie. 
Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate 
retained all the described characteristics of atypical scrapie. 
 
Surprisingly the TSE agent characteristics were dramatically different 
v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and 
biochemical characteristics similar to those of atypical BSE L in the same mouse 
model. Moreover, whereas no other TSE agent than BSE were shown to transmit into 
Tg porcine mice, atypical scrapie was able to develop into this model, albeit 
with low attack rate on first passage. 
 
Furthermore, after adaptation in the porcine mouse model this prion showed 
similar biological and biochemical characteristics than BSE adapted to this 
porcine mouse model. Altogether these data indicate. 
 
*** (i) the unsuspected potential abilities of atypical scrapie to cross 
species barriers 
 
*** (ii) the possible capacity of this agent to acquire new characteristics 
when crossing species barrier 
 
These findings raise some interrogation on the concept of TSE strain and on 
the origin of the diversity of the TSE agents and could have consequences on 
field TSE control measures. 
 
 
 
why do we not want to do TSE transmission studies on chimpanzees $ 
 
5. A positive result from a chimpanzee challenged severly would likely 
create alarm in some circles even if the result could not be interpreted for 
man. I have a view that all these agents could be transmitted provided a large 
enough dose by appropriate routes was given and the animals kept long enough. 
Until the mechanisms of the species barrier are more clearly understood it might 
be best to retain that hypothesis. 
 
snip... 
 
R. BRADLEY 
 
 
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 
observation. 
 
snip... 
 
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie 
by natural feeding to squirrel monkeys that we have reported provides further 
grounds for concern that scrapie-infected meat may occasionally give rise in 
humans to Creutzfeldt-Jakob disease. 
 
PMID: 6997404 
 
 
Recently the question has again been brought up as to whether scrapie is 
transmissible to man. This has followed reports that the disease has been 
transmitted to primates. One particularly lurid speculation (Gajdusek 1977) 
conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and 
transmissible encephalopathy of mink are varieties of a single "virus". The U.S. 
Department of Agriculture concluded that it could "no longer justify or permit 
scrapie-blood line and scrapie-exposed sheep and goats to be processed for human 
or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is 
emphasised by the finding that some strains of scrapie produce lesions identical 
to the once which characterise the human dementias" 
 
Whether true or not. the hypothesis that these agents might be 
transmissible to man raises two considerations. First, the safety of laboratory 
personnel requires prompt attention. Second, action such as the "scorched meat" 
policy of USDA makes the solution of the acrapie problem urgent if the sheep 
industry is not to suffer grievously. 
 
snip... 
 
76/10.12/4.6 
 
 
Nature. 1972 Mar 10;236(5341):73-4. 
 
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). 
 
Gibbs CJ Jr, Gajdusek DC. 
 
Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0 
 
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) 
 
C. J. GIBBS jun. & D. C. GAJDUSEK 
 
National Institute of Neurological Diseases and Stroke, National Institutes 
of Health, Bethesda, Maryland 
 
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey 
(Macaca fascicularis) with an incubation period of more than 5 yr from the time 
of intracerebral inoculation of scrapie-infected mouse brain. The animal 
developed a chronic central nervous system degeneration, with ataxia, tremor and 
myoclonus with associated severe scrapie-like pathology of intensive astroglial 
hypertrophy and proliferation, neuronal vacuolation and status spongiosus of 
grey matter. The strain of scrapie virus used was the eighth passage in Swiss 
mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral 
passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, 
Berkshire). 
 
 
 
Wednesday, February 16, 2011 
 
IN CONFIDENCE 
 
SCRAPIE TRANSMISSION TO CHIMPANZEES 
 
IN CONFIDENCE 
 
 
Sunday, December 12, 2010 
 
EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 
December 2010 
 
 
Sunday, April 18, 2010 
 
SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010 
 
 
Thursday, December 23, 2010 
 
Molecular Typing of Protease-Resistant Prion Protein in Transmissible 
Spongiform Encephalopathies of Small Ruminants, France, 2002-2009 
 
Volume 17, Number 1 January 2011 
 
 
Thursday, November 18, 2010 
 
Increased susceptibility of human-PrP transgenic mice to bovine spongiform 
encephalopathy following passage in sheep 
 
 
Monday, April 25, 2011 
 
Experimental Oral Transmission of Atypical Scrapie to Sheep 
 
Volume 17, Number 5-May 2011 
 
 
Friday, February 11, 2011 
 
Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues 
 
 
Thursday, March 29, 2012 
 
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012 
 
NIAA Annual Conference April 11-14, 2011San Antonio, Texas 
 
 
Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine 
(reticular) deposits, see also ; All of the Heidenhain variants were of the 
methionine/ methionine type 1 molecular subtype. 
 
 
Tuesday, July 29, 2008 
 
Heidenhain Variant Creutzfeldt Jakob Disease Case Report 
 
snip... 
 
Heidenhain Variant Creutzfeldt Jakob Disease autopsy case report 
 
'MOM' DIVISION OF NEUROPATHOLOGY University of Texas Medical Branch 114 
McCullough Bldg. Galveston, Texas 77555-0785 FAX COVER SHEET DATE: 4-23-98 TO: 
Mr. Terry Singeltary @ 
 
-------
 
 FROM: Gerald Campbell FAX: (409) 772-5315 PHONE: (409) 772-2881 Number of 
Pages (including cover sheet): Message: *CONFIDENTIALITY NOTICE* This document 
accompanying this transmission contains confidential information belonging to 
the sender that is legally privileged. This information is intended only for the 
use of the individual or entry names above. If you are not the intended 
recipient, you are hereby notified that any disclosure, copying distribution, or 
the taking of any action in reliances on the contents of this telefaxed 
information is strictly prohibited. If you received this telefax in error, 
please notify us by telephone immediately to arrange for return of the original 
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----------- 
 
Patient Account: 90000014-518 Med. Rec. No.: (0160)118511Q Patient Name: 
POULTER, BARBARA Age: 63 YRS DOB: 10/17/34 Sex: F Admitting Race: C Attending 
Dr.: Date / Time Admitted : 12/14/97 1228 Copies to: UTMB University of Texas 
Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 
Pathology Report FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858 Autopsy 
NO.: AU-97-00435 AUTOPSY INFORMATION: Occupation: Unknown Birthplace: Unknown 
Residence: Crystal Beach Date/Time of Death: 12/14/97 13:30 Date/Time of 
Autopsy: 12/15/97 15:00 Pathologist/Resident: Pencil/Fernandez Service: Private 
Restriction: Brain only FINAL AUTOPSY DIAGNOSIS I. Brain: Creutzfeldt-Jakob 
disease, Heidenhain variant. 
 
snip...see full text ; 
 
 
P.5.21 Parallels between different forms of sheep scrapie and types of 
Creutzfeldt-Jakob disease (CJD) 
 
Wiebke M. Wemheuer1, Sylvie L. Benestad2, Arne Wrede1, Wilhelm E. 
Wemheuer3, Tatjana Pfander1, Bjørn Bratberg2, Bertram Brenig3,Walter J. 
Schulz-Schaeffer1 1University Medical Center Goettingen, Germany; 2Institute of 
Veterinary Medicine Oslo, Norway; 3Institute of Veterinary Medicine Goettingen, 
Germany 
 
Background: Scrapie in sheep and goats is often regarded as the archetype 
of prion diseases. In 1998, a new form of scrapie - atypical/Nor98 scrapie - was 
described that differed from classical scrapie in terms of epidemiology, Western 
blot profile, the distribution of pathological prion protein (PrPSc) in the body 
and its stability against proteinase K. In a similar way, distinct disease types 
exist in sporadic Creutzfeldt-Jakob disease (CJD). They differ with regard to 
their clinical outcome, Western blot profile and PrPSc deposition pattern in the 
central nervous system (CNS). 
 
Objectives: The comparison of PrPSc deposits in sheep scrapie and human 
sporadic CJD. Methods: Tissues of the CNS of sheep with classical scrapie, sheep 
with atypical/Nor98 scrapie and 20 patients with sporadic CJD were examined 
using the sensitive Paraffin Embedded Tissue (PET) blot method. The results were 
compared with those obtained by immunohistochemistry. With the objective of 
gaining information on the protein conformation, the PrPSc of classical and 
atypical/Nor98 sheep scrapie and sporadic CJD was tested for its stability 
against denaturation with guanidine hydrochloride (GdnHCl) using a Membrane 
Adsorption Assay. 
 
Results: The PrPSc of atypical/Nor98 scrapie cases and of CJD prion type 1 
patients exhibits a mainly reticular/synaptic deposition pattern in the brain 
and is relatively sensitive to denaturation with GdnHCl. In contrast classical 
scrapie cases and CJD prion type 2 patients have a more complex PrPSc deposition 
pattern in common that consists of larger PrPSc aggregates and the PrPSc itself 
is comparatively stable against denaturation. 
 
Discussion: The similarity between CJD types and scrapie types indicates 
that at least two comparable forms of the misfolded prion protein exist beyond 
species barriers and can elicit prion diseases. It seems therefore reasonable to 
classify classical and atypical/Nor98 scrapie - in analogy to the existing CJD 
types - as different scrapie types. 
 
 
Monday, December 1, 2008 
 
When Atypical Scrapie cross species barriers 
 
 
Thursday, December 20, 2012 
 
OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED, WISHES TO 
CONTINUE SPREADING IT AROUND THE GLOBE 
 
 
Monday, November 30, 2009 
 
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH 
CODE, DOES NOT SURPRISE ME $
 
 
Wednesday, December 4, 2013 
 
Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine 
Products; Final Rule Federal Register / Vol. 78 , No. 233 / Wednesday, December 
4, 2013 TO ALL IMPORTING COUNTRIES THAT IMPORTS FROM THE USA, BE WARNED, NEW MAD 
COW BSE REGULATIONS USDA, AND OIE, not worth the paper the regulations were 
wrote on, kind of like the mad cow feed ban of August 1997, nothing but ink on 
paper $$$ 
 
full text ; 
 
 
IN A NUT SHELL ; (Adopted by the International Committee of the OIE on 23 
May 2006) 11. Information published by the OIE is derived from appropriate 
declarations made by the official Veterinary Services of Member Countries. The 
OIE is not responsible for inaccurate publication of country disease status 
based on inaccurate information or changes in epidemiological status or other 
significant events that were not promptly reported to the Central Bureau, 
 
 
Thursday, May 30, 2013 
 
World Organization for Animal Health (OIE) has upgraded the United States' 
risk classification for mad cow disease to "negligible" from "controlled", and 
risk further exposing the globe to the TSE prion mad cow type disease U.S. gets 
top mad-cow rating from international group and risk further exposing the globe 
to the TSE prion mad cow type disease 
 
 
 
Wednesday, January 18, 2012 
 
BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE
 
 
*** Spraker suggested an interesting explanation for the occurrence of CWD. 
The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. 
Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at 
this site. When deer were introduced to the pens they occupied ground that had 
previously been occupied by sheep. ...
 
also, see where even decades back, the USDA had the same thought as they do 
today with CWD, not their problem...see page 27 below as well, where USDA stated 
back then, the same thing they stated in the state of Pennsylvania, not their 
damn business, once they escape, and they said the same thing about CWD in 
general back then ; 
 
”The occurrence of CWD must be viewed against the contest of the locations 
in which it occurred. It was an incidental and unwelcome complication of the 
respective wildlife research programmes. Despite it’s subsequent recognition as 
a new disease of cervids, therefore justifying direct investigation, no specific 
research funding was forthcoming. The USDA veiwed it as a wildlife problem and 
consequently not their province!” ...page 26. 
 
 
”The occurrence of CWD must be viewed against the contest of the locations 
in which it occurred. It was an incidental and unwelcome complication of the 
respective wildlife research programmes. Despite it’s subsequent recognition as 
a new disease of cervids, therefore justifying direct investigation, no specific 
research funding was forthcoming. The USDA veiwed it as a wildlife problem and 
consequently not their province!” ...page 26. 
 
sound familiar $$$ 
 
Sunday, January 06, 2013 
 
USDA TO PGC ONCE CAPTIVES ESCAPE 
 
*** "it‘s no longer its business.” 
 
 
now, decades later ; 
 
2012 
 
PO-039: A comparison of scrapie and chronic wasting disease in white-tailed 
deer 
 
Justin Greenlee, Jodi Smith, Eric Nicholson US Dept. Agriculture; 
Agricultural Research Service, National Animal Disease Center; Ames, IA USA 
 
snip...
 
The results of this study suggest that there are many similarities in the 
manifestation of CWD and scrapie in WTD after IC inoculation including early and 
widespread presence of PrPSc in lymphoid tissues, clinical signs of depression 
and weight loss progressing to wasting, and an incubation time of 21-23 months. 
Moreover, western blots (WB) done on brain material from the obex region have a 
molecular profile similar to CWD and distinct from tissues of the cerebrum or 
the scrapie inoculum. However, results of microscopic and IHC examination 
indicate that there are differences between the lesions expected in CWD and 
those that occur in deer with scrapie: amyloid plaques were not noted in any 
sections of brain examined from these deer and the pattern of immunoreactivity 
by IHC was diffuse rather than plaque-like. 
 
*** After a natural route of exposure, 100% of WTD were susceptible to 
scrapie. 
 
Deer developed clinical signs of wasting and mental depression and were 
necropsied from 28 to 33 months PI. Tissues from these deer were positive for 
PrPSc by IHC and WB. Similar to IC inoculated deer, samples from these deer 
exhibited two different molecular profiles: samples from obex resembled CWD 
whereas those from cerebrum were similar to the original scrapie inoculum. On 
further examination by WB using a panel of antibodies, the tissues from deer 
with scrapie exhibit properties differing from tissues either from sheep with 
scrapie or WTD with CWD. Samples from WTD with CWD or sheep with scrapie are 
strongly immunoreactive when probed with mAb P4, however, samples from WTD with 
scrapie are only weakly immunoreactive. In contrast, when probed with mAb’s 6H4 
or SAF 84, samples from sheep with scrapie and WTD with CWD are weakly 
immunoreactive and samples from WTD with scrapie are strongly positive. This 
work demonstrates that WTD are highly susceptible to sheep scrapie, but on first 
passage, scrapie in WTD is differentiable from CWD. 
 
 
2011 
 
*** After a natural route of exposure, 100% of white-tailed deer were 
susceptible to scrapie. 
 
 
Scrapie in Deer: Comparisons and Contrasts to Chronic Wasting Disease (CWD) 
 
Justin J. Greenlee of the Virus and Prion Diseases Research Unit, National 
Animal Disease Center, ARS, USDA, Ames, IA 
 
snip...
 
This highlights the facts that 
 
1) prior to the onset of clinical signs PrPSc is widely distributed in the 
CNS and lymphoid tissues and 
 
2) currently used diagnostic methods are sufficient to detect PrPSc prior 
to the onset of clinical signs. 
 
The results of this study suggest that there are many similarities in the 
manifestation of CWD and scrapie in white-tailed deer after IC inoculation 
including early and widespread presence of PrPSc in lymphoid tissues, clinical 
signs of depression and weight loss progressing to wasting, and an incubation 
time of 21-23 months. Moreover, western blots (WB) done on brain material from 
the obex region have a molecular profile consistent with CWD and distinct from 
tissues of the cerebrum or the scrapie inoculum. However, results of microscopic 
and IHC examination indicate that there are differences between the lesions 
expected in CWD and those that occur in deer with scrapie: amyloid plaques were 
not noted in any sections of brain examined from these deer and the pattern of 
immunoreactivity by IHC was diffuse rather than plaque-like. After a natural 
route of exposure, 100% of white-tailed deer were susceptible to scrapie. Deer 
developed clinical signs of wasting and mental depression and were necropsied 
from 28 to 33 months PI. Tissues from these deer were positive for scrapie by 
IHC and WB. Tissues with PrPSc immunoreactivity included brain, tonsil, 
retropharyngeal and mesenteric lymph nodes, hemal node, Peyer’s patches, and 
spleen. While two WB patterns have been detected in brain regions of deer 
inoculated by the natural route, unlike the IC inoculated deer, the pattern 
similar to the scrapie inoculum predominates. 
 
 
2011 Annual Report 
 
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF 
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Location: Virus and Prion Research 
Unit 
 
2011 Annual Report 
 
In Objective 1, Assess cross-species transmissibility of transmissible 
spongiform encephalopathies (TSEs) in livestock and wildlife, numerous 
experiments assessing the susceptibility of various TSEs in different host 
species were conducted. Most notable is deer inoculated with scrapie, which 
exhibits similarities to chronic wasting disease (CWD) in deer suggestive of 
sheep scrapie as an origin of CWD. 
 
snip... 
 
4. Accomplishments 
 
1. Deer inoculated with domestic isolates of sheep scrapie. 
Scrapie-affected deer exhibit 2 different patterns of disease associated prion 
protein. In some regions of the brain the pattern is much like that observed for 
scrapie, while in others it is more like chronic wasting disease (CWD), the 
transmissible spongiform encephalopathy typically associated with deer. 
 
his work conducted by ARS scientists at the National Animal Disease Center, 
Ames, IA suggests that an interspecies transmission of sheep scrapie to deer may 
have been the origin of CWD. This is important for husbandry practices with both 
captive deer, elk and sheep for farmers and ranchers attempting to keep their 
herds and flocks free of CWD and scrapie. 
 
 
White-tailed Deer are Susceptible to Scrapie by Natural Route of Infection 
 
Jodi D. Smith, Justin J. Greenlee, and Robert A. Kunkle; Virus and Prion 
Research Unit, National Animal Disease Center, USDA-ARS 
 
snip...
 
This work demonstrates for the first time that white-tailed deer are 
susceptible to sheep scrapie by potential natural routes of inoculation. 
In-depth analysis of tissues will be done to determine similarities between 
scrapie in deer after intracranial and oral/intranasal inoculation and chronic 
wasting disease resulting from similar routes of inoculation. 
 
see full text ; 
 
 
Thursday, March 20, 2014 
 
*** CHRONIC WASTING DISEASE CWD TSE PRION OF CERVID AND THE POTENTIAL FOR 
HUMAN TRANSMISSION THEREFROM 2014 
 
 
Saturday, March 15, 2014 
 
*** Potential role of soil properties in the spread of CWD in western 
Canada 
 
 
Friday, February 08, 2013
 
*** Behavior of Prions in the Environment: Implications for Prion Biology 
 
 
Friday, December 14, 2012 
 
DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced 
into Great Britain? A Qualitative Risk Assessment October 2012 
 
snip... 
 
In the USA, under the Food and Drug Administration’s BSE Feed Regulation 
(21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) 
from deer and elk is prohibited for use in feed for ruminant animals. With 
regards to feed for non-ruminant animals, under FDA law, CWD positive deer may 
not be used for any animal feed or feed ingredients. For elk and deer considered 
at high risk for CWD, the FDA recommends that these animals do not enter the 
animal feed system. However, this recommendation is guidance and not a 
requirement by law. 
 
Animals considered at high risk for CWD include: 
 
1) animals from areas declared to be endemic for CWD and/or to be CWD 
eradication zones and 
 
2) deer and elk that at some time during the 60-month period prior to 
slaughter were in a captive herd that contained a CWD-positive animal. 
 
Therefore, in the USA, materials from cervids other than CWD positive 
animals may be used in animal feed and feed ingredients for non-ruminants. 
 
The amount of animal PAP that is of deer and/or elk origin imported from 
the USA to GB can not be determined, however, as it is not specified in TRACES. 
It may constitute a small percentage of the 8412 kilos of non-fish origin 
processed animal proteins that were imported from US into GB in 2011. 
 
Overall, therefore, it is considered there is a __greater than negligible 
risk___ that (nonruminant) animal feed and pet food containing deer and/or elk 
protein is imported into GB. 
 
There is uncertainty associated with this estimate given the lack of data 
on the amount of deer and/or elk protein possibly being imported in these 
products. 
 
snip... 
 
36% in 2007 (Almberg et al., 2011). In such areas, population declines of 
deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of 
Colorado, the prevalence can be as high as 30% (EFSA, 2011). 
 
The clinical signs of CWD in affected adults are weight loss and 
behavioural changes that can span weeks or months (Williams, 2005). In addition, 
signs might include excessive salivation, behavioural alterations including a 
fixed stare and changes in interaction with other animals in the herd, and an 
altered stance (Williams, 2005). These signs are indistinguishable from cervids 
experimentally infected with bovine spongiform encephalopathy (BSE). 
 
Given this, if CWD was to be introduced into countries with BSE such as GB, 
for example, infected deer populations would need to be tested to differentiate 
if they were infected with CWD or BSE to minimise the risk of BSE entering the 
human food-chain via affected venison. 
 
snip... 
 
The rate of transmission of CWD has been reported to be as high as 30% and 
can approach 100% among captive animals in endemic areas (Safar et al., 2008). 
 
snip... 
 
In summary, in endemic areas, there is a medium probability that the soil 
and surrounding environment is contaminated with CWD prions and in a 
bioavailable form. In rural areas where CWD has not been reported and deer are 
present, there is a greater than negligible risk the soil is contaminated with 
CWD prion. 
 
snip... 
 
In summary, given the volume of tourists, hunters and servicemen moving 
between GB and North America, the probability of at least one person travelling 
to/from a CWD affected area and, in doing so, contaminating their clothing, 
footwear and/or equipment prior to arriving in GB is greater than negligible. 
For deer hunters, specifically, the risk is likely to be greater given the 
increased contact with deer and their environment. However, there is significant 
uncertainty associated with these estimates. 
 
snip... 
 
Therefore, it is considered that farmed and park deer may have a higher 
probability of exposure to CWD transferred to the environment than wild deer 
given the restricted habitat range and higher frequency of contact with tourists 
and returning GB residents. 
 
snip... 
 
 
 
 
Saturday, November 2, 2013 
 
APHIS Finalizes Bovine Import Regulations in Line with International Animal 
Health Standards while enhancing the spread of BSE TSE prion mad cow type 
disease around the Globe 
 
 
Tuesday, March 5, 2013 
 
Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening 
of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION) 
 
FDA believes current regulation protects the public from BSE but reopens 
comment period due to new studies 
 
 
Tuesday, March 11, 2014 
 
Science and Technology Committee Oral evidence: Blood, tissue and organ 
screening, HC 990 Wednesday 5 March 2014 SPORADIC CJD 
 
Actually, it is nearer 2 per million per year of the population will 
develop sporadic CJD, but your lifetime risk of developing sporadic CJD is about 
1 in 30,000. So that has not really changed. When people talk about 1 per 
million, often they interpret that as thinking it is incredibly rare. They think 
they have a 1-in-a-million chance of developing this disease. You haven’t. 
You’ve got about a 1-in-30,000 chance of developing it. 
 
 
*** Because typical clinical signs of BSE cannot always be observed in 
nonambulatory disabled cattle, and because evidence has indicated these cattle 
are more likely to have BSE than apparently healthy cattle, FDA is designating 
material from nonambulatory disabled cattle as prohibited cattle materials. 
 
 
 
 
 
*** And Terry, I promised the editor you would respond so thanks for 
backing my prediction up. I have read your tripe before so did not reread the 
whole thing. but your point about the age of the cattle takes on the scientific 
regulatory bodies of every country but one that exports US beef. They all, but 
one, agree that meat from cattle under 30 months of age carries zero risk of BSE 
prions. 1 △ ▽ • Reply • Share › 
 
Terry S. Singeltary Sr. > doc raymond • a month ago 
 
Dr. Richard Raymond Sir, I only reply when you are scientifically wrong. I 
commented today, because again, you were scientifically wrong, and I proved it 
again, with scientific facts to back it up. sorry if that upsets you. you can 
fool some of the folks some of the time, but not all of us all the time. you 
either blatantly lied in your editorial, or you are grossly uninformed, time and 
time again. I think the public can take their pick on that, and in both cases, 
and they would be correct in both cases, in my opinion. you have a nice day sir. 
...kind regards, terry
 
kind regards, terry 
 
What is a Downer Calf? 
 
By Dr. Richard Raymond | February 21, 2014
 
 
see full text Dr. Richard Raymond vs Terry S. Singeltary Sr.
 
 
Monday, March 10, 2014 
 
Investigators study silent variant of mad cow disease Galveston Daily News 
March 4, 2014 
 
 
Thursday, February 20, 2014 
 
*** Unnecessary precautions BSE MAD COW DISEASE Dr. William James FSIS VS 
Dr. Linda Detwiler 2014 
 
 
Owens, Julie 
 
From: Terry S. Singeltary Sr. [flounder9@verizon.net] 
 
Sent: Monday, July 24, 2006 1:09 PM 
 
To: FSIS RegulationsComments 
 
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine 
Spongiform Encephalopathy (BSE) Page 1 of 98 
 
 
FSIS, USDA, REPLY TO SINGELTARY 
 
 
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 
2009
 
 
Tuesday, August 18, 2009
 
* BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009
 
 
WHAT about the sporadic CJD TSE proteins ? 
 
WE now know that some cases of sporadic CJD are linked to atypical BSE and 
atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all 
it’s sub-types $$$ 
 
Creutzfeldt-Jakob Disease CJD cases rising North America updated report 
August 2013 
 
*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada 
seeing an extreme increase of 48% between 2008 and 2010 *** 
 
 
Sunday, October 13, 2013 
 
*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012 
 
 
Sunday, March 09, 2014 
 
A Creutzfeldt-Jakob Disease (CJD) Lookback Study: Assessing the Risk of 
Blood Borne Transmission of Classic Forms of Creutzfeldt-Jakob Disease 
 
FDA TSEAC CIRCUS AND TRAVELING ROAD SHOW FOR THE TSE PRION DISEASES
 
 
*** Because typical clinical signs of BSE cannot always be observed in 
nonambulatory disabled cattle, and because evidence has indicated these cattle 
are more likely to have BSE than apparently healthy cattle, FDA is designating 
material from nonambulatory disabled cattle as prohibited cattle materials. 
 
 
 
 
 
Friday, March 21, 2014 
 
Rancho Dead Stock Cancer Downers Recall Explained FSIS March 20 2014 
?
 
“As of March 20, 2014, FSIS has completed all checks (effectiveness checks 
and disposition verification checks) for recalls 002-2014 and 013-2014 regarding 
Rancho Feeding Corporation. FSIS has determined that based on the number of 
successful checks (see Directive 8080.1, Attachment 1, Table 3) where businesses 
were notified of the recall and removed affected products from commerce that the 
recall activities were effective.”
 
huh ???
 
 
Thursday, March 20, 2014 
 
JACK IN THE BOX NOW CAUGHT UP IN MASSIVE RANCHO DEAD STOCK DOWNER CANCER 
COW RECALL 
 
 
Thursday, March 6, 2014 
 
TEXAS RECALL LIST MASSIVE FROM DEAD STOCK DOWNER CANCER COWS OFFAL from 
Class I Recall 002-2014 and 013-2014 Health Risk: High Jan 13, 2014 and Feb 8, 
2014 shipped to Texas, Florida, and Illinois UPDATE FEBRUARY 14, 2014 
 
 
Monday, March 3, 2014 
 
*** Gov. C.L. "Butch" Otter of Idaho signs bill that will force consumers 
to eat dead stock downers and whatever else the industry decides 
 
 
Thursday, February 13, 2014 
 
*** HSUS VS USDA ET AL BAN DOWNER CALVES FOR HUMAN CONSUMPTION (*veal) and 
potential BSE risk factor there from 
 
 
*** Because typical clinical signs of BSE cannot always be observed in 
nonambulatory disabled cattle, and because evidence has indicated these cattle 
are more likely to have BSE than apparently healthy cattle, FDA is designating 
material from nonambulatory disabled cattle as prohibited cattle materials. 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
TSS
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