Thursday, April 02, 2015
Wasting disease spreads through more western Kansas deer 
By Michael Pearce - The Wichita Eagle 04/01/2015 6:17 PM | Updated: 
04/01/2015 6:31 PM 
Nine deer shot by hunters last fall tested positive for chronic wasting 
disease in western Kansas, including first-time cases in six southwest counties. 
Nine deer shot by hunters last fall tested positive for chronic wasting 
disease in western Kansas, including first-time cases in six southwest counties. 
Michael Pearce / The Wichita Eagle 
Chronic wasting disease, an illness that’s 100 percent fatal in deer and 
elk, has spread to six new counties in southwest Kansas. To date, the disease 
has never been passed to humans or livestock, though it is related to mad cow 
disease and some other illnesses that can be fatal to both.
Shane Hesting, Kansas Department of Wildlife, Parks and Tourism, wildlife 
disease coordinator, said of about 600 deer tested, nine carried the disease. 
Most were shot by hunters during deer seasons. New counties with the disease are 
Gray, Hodgeman, Kearny, Pawnee, Meade and Scott counties, with one deer each. 
Decatur, Norton and Rawlins counties in northwest Kansas each had one deer test 
positive from last fall’s samples. All three have had multiple deer test CWD 
positive in past years. Hesting said hunters who killed the animals are being 
notified of the results, and urged not to eat the venison from those animals as 
a precaution. The disease now nearly stretches from Kansas’ borders with 
Nebraska and Oklahoma. Biologists in Oklahoma have been notified the disease was 
found about 30 miles north of the state line.
Hesting said the six southwest Kansas deer that tested positive came from a 
test sample of 213 deer.
“It’s a small sample size so the prevalence is probably higher than we 
expected in that part of the state,” said Hesting, who added that none of the 
338 deer recently checked from south-central Kansas tested positive for CWD. He 
said the state focused its testing last fall on southwest and south-central 
Kansas. The three deer from northwest Kansas were tested because the hunters who 
killed them suspected the animal was ill because of actions or appearances. 
Chronic wasting disease, a contagious neurological disease, was first 
discovered along the Wyoming/Colorado border in the 1960s, impacting deer and 
elk. The disease spread slowly on its own but appears to have had some help as 
infected animals from game farms in that region were shipped across the country. 
CWD has now shown up as far away as New York, southern New Mexico and parts of 
Saskatchewan.
It’s believed the disease is passed from animal to animal through things 
like saliva and feces, though it’s been known to contaminate an area for years 
in the soil. It is mostly contained in the central nervous system and bones of 
infected deer. 
Some states no longer allow hunters to bring the complete skulls and bones 
of deer and elk they’ve shot from states with CWD, like Kansas, into their home 
state. Hunters in many states are now advised to avoid contact with the brains, 
glands and to avoid cutting or breaking bones when they’re cleaning deer, elk 
and moose they’ve killed.
As the disease gradually spread into the Dakotas and Nebraska, Wildlife and 
Parks began testing deer in northwest Kansas for CWD in 1996. The state’s first 
positive in a wild deer was in 2005 in Cheyenne County, in extreme northwest 
Kansas. A captive elk transplanted from Colorado tested positive for the disease 
in Harper County in 2001.
Since 2005, 73 deer have tested positive in Kansas. Hesting said about 
24,800 have been tested through the years. A loss of federal funding several 
years ago means the agency must focus it’s testing on one or two areas of the 
state annually. Four years ago testing in southwest Kansas showed now signs of 
the disease. 
 Trained technicians, often taxidermists or veterinarians, remove the 
glands or tissue needed to test for the disease for the department. Testing is 
done at Colorado State University and Kansas State University. Hunters can also 
pay to have samples taken and tested.
Hesting said two of the positive bucks were mule deer, of which only 51 
were tested last fall. The rest were whitetails. All were bucks at least 3 1/2 
years old when they were shot. All seven of the southwest Kansas bucks appeared 
healthy to the hunter and the technician who took the sample tissue or 
glands.
Lloyd Fox, Wildlife and Parks big game program coordinator, said initially 
most animals found with the disease in northwest Kansas appeared healthy, too. 
More and more are being found showing weakness, poor physical conditions or 
wandering aimlessly in that region because of the disease. Also, some localized 
areas have produced CWD deer for several years.
So far the disease hasn’t had much of an impact on the deer populations in 
Kansas. Fox said that could change.
“The first few years we see little impact but most of us think it will, in 
decades, have to have a population effect as they environment becomes more 
contaminated,” he said. “When that happens, populations won’t jump back quickly 
from this. It’s a terrible disease.”
 Saturday, March 01, 2014 
KANSAS Tests confirm 5 new CWD cases 
 Sunday, March 10, 2013 
Kansas Four more deer test positive for chronic wasting disease
KANSAS
Chronic Wasting Disease
The first case of CWD was found in a captive elk in Harper County in 2001. 
Since that time, CWD has been detected in 49 wild, free-ranging white-tailed and 
1 mule deer in Deer Management Units (DMU) 1, 2, 3, 4, 5, 17. 
In 2010-2011 the first positive mule deer was detected in Decatur County. 
Currently, the total number of positives since surveillance started in 1996 is 
51 (1 captive elk, 1 mule deer, and 49 white-tailed deer). Hunters and other 
wildlife enthusiasts can avoid the human-assisted spread of CWD by not 
transporting a live or dead deer or elk from areas where CWD occurs to those 
areas which are CWD-free. There is currently no known treatment or eradication 
method for CWD, so preventing the introduction of the the disease into new areas 
is of utmost importance to the health of local deer herds. Baiting and feeding 
deer tend to concentrate deer at small point on the landscape, often with the 
trails leading to the feeding sites resembling the wheel spokes of a bicycle. 
Anytime animals are concentrated at the "hub," the likelihood of disease 
transmission increases in a deer herd. More alarming, CWD is not the only 
serious disease of concern. Diseases such as bovine tuberculosis and a host of 
detrimental parasites such as exotic lice, meningeal worms, flukes, and stomach 
worms are transmitted more efficiently when deer are concentrated in a small 
area. 
Another major concern is the potential for spread of CWD from captive 
cervid farms into the wild cervid population. Once a disease gets into a wild 
population, it is virtually impossible eradicate. The only thing that can be 
done is control the spread of the disease at great expense. KDWPT recommends 
that every captive cervid rancher enroll in the voluntary CWD monitoring program 
administered by the Kansas Animal Health Department. The sooner diseases such as 
CWD can be detected in captives, the sooner control efforts can begin and 
possibly prevent the spread of disease to wild populations of the state. CWD is 
only one of many diseases that could go undetected in an unmonitored captive 
cervid herd. Bovine tuberculosis, for example, is a serious disease that could 
seriously damage not only populations of deer and an annual 350 million-dollar 
hunting economy, but could also threaten the 4 billion-dollar Kansas cattle 
industry via quarantines and loss of accreditation. 
2012-2013 CWD CONFIRMED POSITIVES by County (Surveillance Reduced to 
Northcentral Zone Due to Funding Cuts) 
Thursday, July 19, 2012 
NINE DEER TEST POSITIVE FOR CHRONIC WASTING DISEASE 
Thursday, February 09, 2012 
THREE KANSAS DEER CONFIRMED POSITIVE IN EARLY STAGES OF CWD TESTING 
Thursday, March 31, 2011 
TEN KANSAS DEER CONFIRMED POSITIVE IN CWD TESTS 
Thursday, January 06, 2011 
KANSAS FIRST CASE OF CHRONIC WASTING IN 2010 DEER SEASON CONFIRMED 
Thursday, January 21, 2010 
Kansas has more CWD cases 
Thursday, April 02, 2015 
OHIO CONFIRMS SECOND POSTIVE CHRONIC WASTING DISEASE CWD on Yoder's 
properties near Millersburg 
Wednesday, March 18, 2015 
Chronic Wasting Disease CWD Confirmed Texas Trans Pecos March 18, 
2015
Wednesday, March 25, 2015 
Chronic Wasting Disease CWD Cases Confirmed In New Mexico 2013 and 2014 
UPDATE 2015
Tuesday, October 21, 2014 
*** Pennsylvania Department of Agriculture Tenth Pennsylvania Captive Deer 
Tests Positive for Chronic Wasting Disease CWD TSE PRION DISEASE 
”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. 
Sunday, January 06, 2013 
USDA TO PGC ONCE CAPTIVES ESCAPE 
*** "it‘s no longer its business.” 
Sunday, July 13, 2014 
Louisiana deer mystery unleashes litigation 6 does still missing from CWD 
index herd in Pennsylvania Great Escape 
Saturday, June 29, 2013 
PENNSYLVANIA CAPTIVE CWD INDEX HERD MATE YELLOW *47 STILL RUNNING LOOSE IN 
INDIANA, YELLOW NUMBER 2 STILL MISSING, AND OTHERS ON THE RUN STILL IN LOUISIANA 
Tuesday, June 11, 2013 
*** CWD GONE WILD, More cervid escapees from more shooting pens on the 
loose in Pennsylvania 
Wednesday, September 04, 2013 
***cwd - cervid captive livestock escapes, loose and on the run in the 
wild... 
Tuesday, October 07, 2014 
*** Wisconsin white-tailed deer tested positive for CWD on a Richland 
County breeding farm, and a case of CWD has been discovered on a Marathon County 
hunting preserve 
Thursday, October 02, 2014 
*** IOWA TEST RESULTS FROM CAPTIVE DEER HERD WITH CHRONIC WASTING DISEASE 
RELEASED 79.8 percent of the deer tested positive for the disease 
Tuesday, December 20, 2011
CHRONIC WASTING DISEASE CWD WISCONSIN Almond Deer (Buckhorn Flats) 
FarmUpdate DECEMBER 2011The CWD infection rate was nearly 80%, the highest ever 
in a North American captive herd. RECOMMENDATION: That the Board approve the 
purchase of 80acres of land for $465,000 for the Statewide Wildlife Habitat 
Program inPortage County and approve the restrictions on public use of the 
site.SUMMARY:
For Immediate Release Thursday, October 2, 2014 
Dustin Vande Hoef 515/281-3375 or 515/326-1616 (cell) or 
Dustin.VandeHoef@IowaAgriculture.gov
TEST RESULTS FROM CAPTIVE DEER HERD WITH CHRONIC WASTING DISEASE RELEASED 
79.8 percent of the deer tested positive for the disease
DES MOINES – The Iowa Department of Agriculture and Land Stewardship today 
announced that the test results from the depopulation of a quarantined captive 
deer herd in north-central Iowa showed that 284 of the 356 deer, or 79.8% of the 
herd, tested positive for Chronic Wasting Disease (CWD). The owners of the 
quarantined herd have entered into a fence maintenance agreement with the Iowa 
Department of Agriculture and Land Stewardship,which requires the owners to 
maintain the 8’ foot perimeter fence around the herd premises for five years 
after the depopulation was complete and the premises had been cleaned and 
disinfected CWD is a progressive, fatal, degenerative neurological disease of 
farmed and free-ranging deer, elk, and moose. There is no known treatment or 
vaccine for CWD. CWD is not a disease that affects humans.On July 18, 2012, USDA 
Animal and Plant Health Inspection Service’s (APHIS)National Veterinary Services 
Lab in Ames, IA confirmed that a male whitetail deer harvested from a hunting 
preserve in southeast IA was positive for CWD. An investigation revealed that 
this animal had just been introduced into the hunting preserve from the 
above-referenced captive deer herd in north-central Iowa.The captive deer herd 
was immediately quarantined to prevent the spread of CWD. The herd has remained 
in quarantine until its depopulation on August 25 to 27, 2014.The Iowa 
Department of Agriculture and Land Stewardship participated in a joint operation 
to depopulate the infected herd with USDA Veterinary Services, which was the 
lead agency, and USDA Wildlife Services.Federal indemnity funding became 
available in 2014. USDA APHIS appraised the captive deer herd of 376 animals at 
that time, which was before depopulation and testing, at $1,354,250. At that 
time a herd plan was developed with the owners and officials from USDA and the 
Iowa Department of Agriculture and Land Stewardship.Once the depopulation was 
complete and the premises had been cleaned and disinfected, indemnity of 
$917,100.00 from the USDA has been or will be paid to the owners as compensation 
for the 356 captive deer depopulated.The Iowa Department of Agriculture and Land 
Stewardship operates a voluntary CWD program for farms that sell live animals. 
Currently 145 Iowa farms participate in the voluntary program. The 
above-referenced captive deer facility left the voluntary CWD program prior to 
the discovery of the disease as they had stopped selling live animals. All deer 
harvested in a hunting preserve must be tested for CWD. -30-
*** see history of this CWD blunder here ; 
On June 5, 2013, DNR conducted a fence inspection, after gaining approval 
from surrounding landowners, and confirmed that the fenced had beencut or 
removed in at least four separate locations; that the fence had degraded and was 
failing to maintain the enclosure around the Quarantined Premises in at least 
one area; that at least three gates had been opened;and that deer tracks were 
visible in and around one of the open areas in the sand on both sides of the 
fence, evidencing movement of deer into the Quarantined Premises.
Tuesday, January 06, 2015 
APHIS Provides Additional Information on Chronic Wasting Disease (CWD) 
Indemnity Requests January 5, 2015 05:26 PM EST
***please read this*** 
98 | Veterinary Record | January 24, 2015
EDITORIAL
Scrapie: a particularly persistent pathogen
Cristina Acín
Resistant prions in the environment have been the sword of Damocles for 
scrapie control and eradication. Attempts to establish which physical and 
chemical agents could be applied to inactivate or moderate scrapie infectivity 
were initiated in the 1960s and 1970s,with the first study of this type focusing 
on the effect of heat treatment in reducing prion infectivity (Hunter and 
Millson 1964). Nowadays, most of the chemical procedures that aim to inactivate 
the prion protein are based on the method developed by Kimberlin and 
collaborators (1983). This procedure consists of treatment with 20,000 parts per 
million free chlorine solution, for a minimum of one hour, of all surfaces that 
need to be sterilised (in laboratories, lambing pens, slaughterhouses, and so 
on). Despite this, veterinarians and farmers may still ask a range of questions, 
such as ‘Is there an official procedure published somewhere?’ and ‘Is there an 
international organisation which recommends and defines the exact method of 
scrapie decontamination that must be applied?’
From a European perspective, it is difficult to find a treatment that could 
be applied, especially in relation to the disinfection of surfaces in lambing 
pens of affected flocks. A 999/2001 EU regulation on controlling spongiform 
encephalopathies (European Parliament and Council 2001) did not specify a 
particular decontamination measure to be used when an outbreak of scrapie is 
diagnosed. There is only a brief recommendation in Annex VII concerning the 
control and eradication of transmissible spongiform encephalopathies (TSE 
s).
Chapter B of the regulation explains the measures that must be applied if 
new caprine animals are to be introduced to a holding where a scrapie outbreak 
has previously been diagnosed. In that case, the statement indicates that 
caprine animals can be introduced ‘provided that a cleaning and disinfection of 
all animal housing on the premises has been carried out following 
destocking’.
Issues around cleaning and disinfection are common in prion prevention 
recommendations, but relevant authorities, veterinarians and farmers may have 
difficulties in finding the specific protocol which applies. The European Food 
and Safety Authority (EFSA ) published a detailed report about the efficacy of 
certain biocides, such as sodium hydroxide, sodium hypochlorite, guanidine and 
even a formulation of copper or iron metal ions in combination with hydrogen 
peroxide, against prions (EFSA 2009). The report was based on scientific 
evidence (Fichet and others 2004, Lemmer and others 2004, Gao and others 2006, 
Solassol and others 2006) but unfortunately the decontamination measures were 
not assessed under outbreak conditions.
The EFSA Panel on Biological Hazards recently published its conclusions on 
the scrapie situation in the EU after 10 years of monitoring and control of the 
disease in sheep and goats (EFSA 2014), and one of the most interesting findings 
was the Icelandic experience regarding the effect of disinfection in scrapie 
control. The Icelandic plan consisted of: culling scrapie-affected sheep or the 
whole flock in newly diagnosed outbreaks; deep cleaning and disinfection of 
stables, sheds, barns and equipment with high pressure washing followed by 
cleaning with 500 parts per million of hypochlorite; drying and treatment with 
300 ppm of iodophor; and restocking was not permitted for at least two years. 
Even when all of these measures were implemented, scrapie recurred on several 
farms, indicating that the infectious agent survived for years in the 
environment, even as many as 16 years after restocking (Georgsson and others 
2006).
In the rest of the countries considered in the EFSA (2014) report, 
recommendations for disinfection measures were not specifically defined at the 
government level. In the report, the only recommendation that is made for sheep 
is repopulation with sheep with scrapie-resistant genotypes. This reduces the 
risk of scrapie recurrence but it is difficult to know its effect on the 
infection.
Until the EFSA was established (in May 2003), scientific opinions about TSE 
s were provided by the Scientific Steering Committee (SSC) of the EC, whose 
advice regarding inactivation procedures focused on treating animal waste at 
high temperatures (150°C for three hours) and high pressure alkaline hydrolysis 
(SSC 2003). At the same time, the TSE Risk Management Subgroup of the Advisory 
Committee on Dangerous Pathogens (ACDP) in the UK published guidance on safe 
working and the prevention of TSE infection. Annex C of the ACDP report 
established that sodium hypochlorite was considered to be effective, but only if 
20,000 ppm of available chlorine was present for at least one hour, which has 
practical limitations such as the release of chlorine gas, corrosion, 
incompatibility with formaldehyde, alcohols and acids, rapid inactivation of its 
active chemicals and the stability of dilutions (ACDP 2009).
In an international context, the World Organisation for Animal Health (OIE) 
does not recommend a specific disinfection protocol for prion agents in its 
Terrestrial Code or Manual. Chapter 4.13 of the Terrestrial Code, General 
recommendations on disinfection and disinsection (OIE 2014), focuses on 
foot-and-mouth disease virus, mycobacteria and Bacillus anthracis, but not on 
prion disinfection. Nevertheless, the last update published by the OIE on bovine 
spongiform encephalopathy (OIE 2012) indicates that few effective 
decontamination techniques are available to inactivate the agent on surfaces, 
and recommends the removal of all organic material and the use of sodium 
hydroxide, or a sodium hypochlorite solution containing 2 per cent available 
chlorine, for more than one hour at 20ºC.
The World Health Organization outlines guidelines for the control of TSE s, 
and also emphasises the importance of mechanically cleaning surfaces before 
disinfection with sodium hydroxide or sodium hypochlorite for one hour (WHO 
1999).
Finally, the relevant agencies in both Canada and the USA suggest that the 
best treatments for surfaces potentially contaminated with prions are sodium 
hydroxide or sodium hypochlorite at 20,000 ppm. This is a 2 per cent solution, 
while most commercial household bleaches contain 5.25 per cent sodium 
hypochlorite. It is therefore recommended to dilute one part 5.25 per cent 
bleach with 1.5 parts water (CDC 2009, Canadian Food Inspection Agency 
2013).
So what should we do about disinfection against prions? First, it is 
suggested that a single protocol be created by international authorities to 
homogenise inactivation procedures and enable their application in all 
scrapie-affected countries. Sodium hypochlorite with 20,000 ppm of available 
chlorine seems to be the procedure used in most countries, as noted in a paper 
summarised on p 99 of this issue of Veterinary Record (Hawkins and others 2015). 
But are we totally sure of its effectiveness as a preventive measure in a 
scrapie outbreak? Would an in-depth study of the recurrence of scrapie disease 
be needed?
What we can conclude is that, if we want to fight prion diseases, and 
specifically classical scrapie, we must focus on the accuracy of diagnosis, 
monitoring and surveillance; appropriate animal identification and control of 
movements; and, in the end, have homogeneous and suitable protocols to 
decontaminate and disinfect lambing barns, sheds and equipment available to 
veterinarians and farmers. Finally, further investigations into the resistance 
of prion proteins in the diversity of environmental surfaces are required.
References
snip...
98 | Veterinary Record | January 24, 2015
Persistence of ovine scrapie infectivity in a farm environment following 
cleaning and decontamination 
Steve A. C. Hawkins, MIBiol, Pathology Department1, Hugh A. Simmons, BVSc 
MRCVS, MBA, MA Animal Services Unit1, Kevin C. Gough, BSc, PhD2 and Ben C. 
Maddison, BSc, PhD3 + Author Affiliations
1Animal and Plant Health Agency, Woodham Lane, New Haw, Addlestone, Surrey 
KT15 3NB, UK 2School of Veterinary Medicine and Science, The University of 
Nottingham, Sutton Bonington, Loughborough, Leicestershire LE12 5RD, UK 3ADAS 
UK, School of Veterinary Medicine and Science, The University of Nottingham, 
Sutton Bonington, Loughborough, Leicestershire LE12 5RD, UK E-mail for 
correspondence: ben.maddison@adas.co.uk Abstract Scrapie of sheep/goats and 
chronic wasting disease of deer/elk are contagious prion diseases where 
environmental reservoirs are directly implicated in the transmission of disease. 
In this study, the effectiveness of recommended scrapie farm decontamination 
regimens was evaluated by a sheep bioassay using buildings naturally 
contaminated with scrapie. Pens within a farm building were treated with either 
20,000 parts per million free chorine solution for one hour or were treated with 
the same but were followed by painting and full re-galvanisation or replacement 
of metalwork within the pen. Scrapie susceptible lambs of the PRNP genotype 
VRQ/VRQ were reared within these pens and their scrapie status was monitored by 
recto-anal mucosa-associated lymphoid tissue. All animals became infected over 
an 18-month period, even in the pen that had been subject to the most stringent 
decontamination process. These data suggest that recommended current guidelines 
for the decontamination of farm buildings following outbreaks of scrapie do 
little to reduce the titre of infectious scrapie material and that environmental 
recontamination could also be an issue associated with these premises. 
SNIP...
Discussion
Thorough pressure washing of a pen had no effect on the amount of 
bioavailable scrapie infectivity (pen B). The routine removal of prions from 
surfaces within a laboratory setting is treatment for a minimum of one hour with 
20,000 ppm free chlorine, a method originally based on the use of brain 
macerates from infected rodents to evaluate the effectiveness of decontamination 
(Kimberlin and others 1983). Further studies have also investigated the 
effectiveness of hypochlorite disinfection of metal surfaces to simulate the 
decontamination of surgical devices within a hospital setting. Such treatments 
with hypochlorite solution were able to reduce infectivity by 5.5 logs to lower 
than the sensitivity of the bioassay used (Lemmer and others 2004). Analogous 
treatment of the pen surfaces did not effectively remove the levels of scrapie 
infectivity over that of the control pens, indicating that this method of 
decontamination is not effective within a farm setting. This may be due to the 
high level of biological matrix that is present upon surfaces within the farm 
environment, which may reduce the amount of free chlorine available to 
inactivate any infectious prion. Remarkably 1/5 sheep introduced into pen D had 
also became scrapie positive within nine months, with all animals in this pen 
being RAMALT positive by 18 months of age. Pen D was no further away from the 
control pen (pen A) than any of the other pens within this barn. Localised hot 
spots of infectivity may be present within scrapie-contaminated environments, 
but it is unlikely that pen D area had an amount of scrapie contamination that 
was significantly different than the other areas within this building. 
Similarly, there were no differences in how the biosecurity of pen D was 
maintained, or how this pen was ventilated compared with the other pens. This 
observation, perhaps, indicates the slower kinetics of disease uptake within 
this pen and is consistent with a more thorough prion removal and 
recontamination. These observations may also account for the presence of 
inadvertent scrapie cases within other studies, where despite stringent 
biosecurity, control animals have become scrapie positive during challenge 
studies using barns that also housed scrapie-affected animals (Ryder and others 
2009). The bioassay data indicate that the exposure of the sheep to a farm 
environment after decontamination efforts thought to be effective in removing 
scrapie is sufficient for the animals to become infected with scrapie. The main 
exposure routes within this scenario are likely to be via the oral route, during 
feeding and drinking, and respiratory and conjunctival routes. It has been 
demonstrated that scrapie infectivity can be efficiently transmitted via the 
nasal route in sheep (Hamir and others 2008), as is the case for CWD in both 
murine models and in white-tailed deer (Denkers and others 2010, 2013). 
Recently, it has also been demonstrated that CWD prions presented as dust when 
bound to the soil mineral montmorillonite can be infectious via the nasal route 
(Nichols and others 2013). When considering pens C and D, the actual source of 
the infectious agent in the pens is not known, it is possible that biologically 
relevant levels of prion survive on surfaces during the decontamination regimen 
(pen C). With the use of galvanising and painting (pen D) covering and sealing 
the surface of the pen, it is possible that scrapie material recontaminated the 
pens by the movement of infectious prions contained within dusts originating 
from other parts of the barn that were not decontaminated or from other areas of 
the farm.
Given that scrapie prions are widespread on the surfaces of affected farms 
(Maddison and others 2010a), irrespective of the source of the infectious prions 
in the pens, this study clearly highlights the difficulties that are faced with 
the effective removal of environmentally associated scrapie infectivity. This is 
likely to be paralleled in CWD which shows strong similarities to scrapie in 
terms of both the dissemination of prions into the environment and the facile 
mode of disease transmission. These data further contribute to the understanding 
that prion diseases can be highly transmissible between susceptible individuals 
not just by direct contact but through highly stable environmental reservoirs 
that are refractory to decontamination.
The presence of these environmentally associated prions in farm buildings 
make the control of these diseases a considerable challenge, especially in 
animal species such as goats where there is lack of genetic resistance to 
scrapie and, therefore, no scope to re-stock farms with animals that are 
resistant to scrapie.
Scrapie Sheep Goats Transmissible spongiform encephalopathies (TSE) 
Accepted October 12, 2014. Published Online First 31 October 2014 
Tuesday, December 16, 2014 
Evidence for zoonotic potential of ovine scrapie prions 
Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves 
Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle 
Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia 
Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, 
Affiliations Contributions Corresponding author Journal name: Nature 
Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 
Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 
Article tools Citation Reprints Rights & permissions Article metrics 
Abstract 
Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant 
Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie 
prions remains unknown. Mice genetically engineered to overexpress the human 
prion protein (tgHu) have emerged as highly relevant models for gauging the 
capacity of prions to transmit to humans. These models can propagate human 
prions without any apparent transmission barrier and have been used used to 
confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie 
prions transmit to several tgHu mice models with an efficiency comparable to 
that of cattle BSE. The serial transmission of different scrapie isolates in 
these mice led to the propagation of prions that are phenotypically identical to 
those causing sporadic CJD (sCJD) in humans. These results demonstrate that 
scrapie prions have a zoonotic potential and raise new questions about the 
possible link between animal and human prions.
Subject terms: Biological sciences• Medical research At a glance
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 
Friday, January 30, 2015
Scrapie: a particularly persistent pathogen
Monday, November 3, 2014 
Persistence of ovine scrapie infectivity in a farm environment following 
cleaning and decontamination
PPo3-22:
Detection of Environmentally Associated PrPSc on a Farm with Endemic 
Scrapie
Ben C. Maddison,1 Claire A. Baker,1 Helen C. Rees,1 Linda A. Terry,2 Leigh 
Thorne,2 Susan J. Belworthy2 and Kevin C. Gough3 1ADAS-UK LTD; Department of 
Biology; University of Leicester; Leicester, UK; 2Veterinary Laboratories 
Agency; Surry, KT UK; 3Department of Veterinary Medicine and Science; University 
of Nottingham; Sutton Bonington, Loughborough UK
Key words: scrapie, evironmental persistence, sPMCA
Ovine scrapie shows considerable horizontal transmission, yet the routes of 
transmission and specifically the role of fomites in transmission remain poorly 
defined. Here we present biochemical data demonstrating that on a 
scrapie-affected sheep farm, scrapie prion contamination is widespread. It was 
anticipated at the outset that if prions contaminate the environment that they 
would be there at extremely low levels, as such the most sensitive method 
available for the detection of PrPSc, serial Protein Misfolding Cyclic 
Amplification (sPMCA), was used in this study. We investigated the distribution 
of environmental scrapie prions by applying ovine sPMCA to samples taken from a 
range of surfaces that were accessible to animals and could be collected by use 
of a wetted foam swab. Prion was amplified by sPMCA from a number of these 
environmental swab samples including those taken from metal, plastic and wooden 
surfaces, both in the indoor and outdoor environment. At the time of sampling 
there had been no sheep contact with these areas for at least 20 days prior to 
sampling indicating that prions persist for at least this duration in the 
environment. These data implicate inanimate objects as environmental reservoirs 
of prion infectivity which are likely to contribute to disease transmission. 
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. 
*** We conclude that TSE infectivity is likely to survive burial for long 
time periods with minimal loss of infectivity and limited movement from the 
original burial site. However PMCA results have shown that there is the 
potential for rainwater to elute TSE related material from soil which could lead 
to the contamination of a wider area. These experiments reinforce the importance 
of risk assessment when disposing of TSE risk materials. 
*** The results show that even highly diluted PrPSc can bind efficiently to 
polypropylene, stainless steel, glass, wood and stone and propagate the 
conversion of normal prion protein. For in vivo experiments, hamsters were ic 
injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, 
inoculated with 263K-contaminated implants of all groups, developed typical 
signs of prion disease, whereas control animals inoculated with non-contaminated 
materials did not.
PRION 2014 CONFERENCE
CHRONIC WASTING DISEASE CWD 
A FEW FINDINGS ; 
Conclusions. To our knowledge, this is the first established experimental 
model of CWD in TgSB3985. We found evidence for co-existence or divergence of 
two CWD strains adapted to Tga20 mice and their replication in TgSB3985 mice. 
Finally, we observed phenotypic differences between cervid-derived CWD and 
CWD/Tg20 strains upon propagation in TgSB3985 mice. Further studies are underway 
to characterize these strains. 
We conclude that TSE infectivity is likely to survive burial for long time 
periods with minimal loss of infectivity and limited movement from the original 
burial site. However PMCA results have shown that there is the potential for 
rainwater to elute TSE related material from soil which could lead to the 
contamination of a wider area. These experiments reinforce the importance of 
risk assessment when disposing of TSE risk materials. 
The results show that even highly diluted PrPSc can bind efficiently to 
polypropylene, stainless steel, glass, wood and stone and propagate the 
conversion of normal prion protein. For in vivo experiments, hamsters were ic 
injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, 
inoculated with 263K-contaminated implants of all groups, developed typical 
signs of prion disease, whereas control animals inoculated with non-contaminated 
materials did not.
Our data establish that meadow voles are permissive to CWD via peripheral 
exposure route, suggesting they could serve as an environmental reservoir for 
CWD. Additionally, our data are consistent with the hypothesis that at least two 
strains of CWD circulate in naturally-infected cervid populations and provide 
evidence that meadow voles are a useful tool for CWD strain typing. 
Conclusion. CWD prions are shed in saliva and urine of infected deer as 
early as 3 months post infection and throughout the subsequent >1.5 year 
course of infection. In current work we are examining the relationship of 
prionemia to excretion and the impact of excreted prion binding to surfaces and 
particulates in the environment.
Conclusion. CWD prions (as inferred by prion seeding activity by RT-QuIC) 
are shed in urine of infected deer as early as 6 months post inoculation and 
throughout the subsequent disease course. Further studies are in progress 
refining the real-time urinary prion assay sensitivity and we are examining more 
closely the excretion time frame, magnitude, and sample variables in 
relationship to inoculation route and prionemia in naturally and experimentally 
CWD-infected cervids.
Conclusions. Our results suggested that the odds of infection for CWD is 
likely controlled by areas that congregate deer thus increasing direct 
transmission (deer-to-deer interactions) or indirect transmission 
(deer-to-environment) by sharing or depositing infectious prion proteins in 
these preferred habitats. Epidemiology of CWD in the eastern U.S. is likely 
controlled by separate factors than found in the Midwestern and endemic areas 
for CWD and can assist in performing more efficient surveillance efforts for the 
region.
Conclusions. During the pre-symptomatic stage of CWD infection and 
throughout the course of disease deer may be shedding multiple LD50 doses per 
day in their saliva. CWD prion shedding through saliva and excreta may account 
for the unprecedented spread of this prion disease in nature. 
see full text and more ; 
Monday, June 23, 2014 
*** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD 
*** Infectious agent of sheep scrapie may persist in the environment for at 
least 16 years*** 
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3 
New studies on the heat resistance of hamster-adapted scrapie agent: 
Threshold survival after ashing at 600°C suggests an inorganic template of 
replication 
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel 
Production 
Detection of protease-resistant cervid prion protein in water from a 
CWD-endemic area 
A Quantitative Assessment of the Amount of Prion Diverted to Category 1 
Materials and Wastewater During Processing 
Rapid assessment of bovine spongiform encephalopathy prion inactivation by 
heat treatment in yellow grease produced in the industrial manufacturing process 
of meat and bone meals 
Sunday, December 21, 2014 
Mucosal immunization with an attenuated Salmonella vaccine partially 
protects white-tailed deer from chronic wasting disease 
Friday, December 19, 2014 
Pan-Provincial Vaccine Enterprise Inc. (PREVENT) Conducting a Chronic 
Wasting Disease (CWD) Vaccine Efficacy Trial in Elk 
CHRONIC WASTING DISEASE CWD TSE PRION, how much does it pay to find CWD 
$$$
CWD, spreading it around...
for the game farm industry, and their constituents, to continue to believe 
that they are _NOT_, and or insinuate that they have _NEVER_ been part of the 
problem, will only continue to help spread cwd. the game farming industry, from 
the shooting pens, to the urine mills, the antler mills, the sperm mills, velvet 
mills, shooting pens, to large ranches, are not the only problem, but it is 
painfully obvious that they have been part of the problem for decades and 
decades, just spreading it around, as with transportation and or exportation and 
or importation of cervids from game farming industry, and have been proven to 
spread cwd. no one need to look any further than South Korea blunder ; 
=========================================== 
spreading cwd around...
Between 1996 and 2002, chronic wasting disease was diagnosed in 39 herds of 
farmed elk in Saskatchewan in a single epidemic. All of these herds were 
depopulated as part of the Canadian Food Inspection Agency’s (CFIA) disease 
eradication program. Animals, primarily over 12 mo of age, were tested for the 
presence CWD prions following euthanasia. Twenty-one of the herds were linked 
through movements of live animals with latent CWD from a single infected source 
herd in Saskatchewan, 17 through movements of animals from 7 of the secondarily 
infected herds. 
***The source herd is believed to have become infected via importation of 
animals from a game farm in South Dakota where CWD was subsequently diagnosed 
(7,4). A wide range in herd prevalence of CWD at the time of herd depopulation 
of these herds was observed. Within-herd transmission was observed on some 
farms, while the disease remained confined to the introduced animals on other 
farms. 
spreading cwd around...
Friday, May 13, 2011 
Chronic Wasting Disease (CWD) outbreaks and surveillance program in the 
Republic of Korea 
Chronic Wasting Disease (CWD) outbreaks and surveillance program in the 
Republic of Korea 
Hyun-Joo Sohn, Yoon-Hee Lee, Min-jeong Kim, Eun-Im Yun, Hyo-Jin Kim, 
Won-Yong Lee, Dong-Seob Tark, In- Soo Cho, Foreign Animal Disease Research 
Division, National Veterinary Research and Quarantine Service, Republic of Korea 
Chronic wasting disease (CWD) has been recognized as an important prion 
disease in native North America deer and Rocky mountain elks. The disease is a 
unique member of the transmissible spongiform encephalopathies (TSEs), which 
naturally affects only a few species. CWD had been limited to USA and Canada 
until 2000. 
On 28 December 2000, information from the Canadian government showed that a 
total of 95 elk had been exported from farms with CWD to Korea. These consisted 
of 23 elk in 1994 originating from the so-called “source farm” in Canada, and 72 
elk in 1997, which had been held in pre export quarantine at the “source 
farm”.Based on export information of CWD suspected elk from Canada to Korea, CWD 
surveillance program was initiated by the Ministry of Agriculture and Forestry 
(MAF) in 2001. 
All elks imported in 1997 were traced back, however elks imported in 1994 
were impossible to identify. CWD control measures included stamping out of all 
animals in the affected farm, and thorough cleaning and disinfection of the 
premises. In addition, nationwide clinical surveillance of Korean native 
cervids, and improved measures to ensure reporting of CWD suspect cases were 
implemented. 
Total of 9 elks were found to be affected. CWD was designated as a 
notifiable disease under the Act for Prevention of Livestock Epidemics in 2002. 
Additional CWD cases - 12 elks and 2 elks - were diagnosed in 2004 and 
2005. 
Since February of 2005, when slaughtered elks were found to be positive, 
all slaughtered cervid for human consumption at abattoirs were designated as 
target of the CWD surveillance program. Currently, CWD laboratory testing is 
only conducted by National Reference Laboratory on CWD, which is the Foreign 
Animal Disease Division (FADD) of National Veterinary Research and Quarantine 
Service (NVRQS). 
In July 2010, one out of 3 elks from Farm 1 which were slaughtered for the 
human consumption was confirmed as positive. Consequently, all cervid – 54 elks, 
41 Sika deer and 5 Albino deer – were culled and one elk was found to be 
positive. Epidemiological investigations were conducted by Veterinary 
Epidemiology Division (VED) of NVRQS in collaboration with provincial veterinary 
services. 
Epidemiologically related farms were found as 3 farms and all cervid at 
these farms were culled and subjected to CWD diagnosis. Three elks and 5 
crossbreeds (Red deer and Sika deer) were confirmed as positive at farm 2. 
All cervids at Farm 3 and Farm 4 – 15 elks and 47 elks – were culled and 
confirmed as negative. 
Further epidemiological investigations showed that these CWD outbreaks were 
linked to the importation of elks from Canada in 1994 based on circumstantial 
evidences. 
In December 2010, one elk was confirmed as positive at Farm 5. 
Consequently, all cervid – 3 elks, 11 Manchurian Sika deer and 20 Sika deer – 
were culled and one Manchurian Sika deer and seven Sika deer were found to be 
positive. This is the first report of CWD in these sub-species of deer. 
Epidemiological investigations found that the owner of the Farm 2 in CWD 
outbreaks in July 2010 had co-owned the Farm 5. 
In addition, it was newly revealed that one positive elk was introduced 
from Farm 6 of Jinju-si Gyeongsang Namdo. All cervid – 19 elks, 15 crossbreed 
(species unknown) and 64 Sika deer – of Farm 6 were culled, but all confirmed as 
negative. 
Tuesday, February 10, 2015 
Alberta Canada First case of chronic wasting disease found in farm elk 
since 2002 
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... 
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 
BSE RUMINANT FEED BAN FOR CERVIDS AND PETS IN THE USA ?
in short, there is none, and never has been. 
I am concerned with pets as well.
I strongly, strenuously, urge the FDA et al and scientist (minus the 
industry, politicians, and lobbyist there from on all issues), to revisit the 
foolish voluntary ban on ruminant feed to cervids, and adopt an immediate 
measure to make mandatory the ban of all ruminant feed to all cervids and pets. 
... TSS 
FDA WARNING LETTER (14-ATL-04) adulterated under Section 402(a)(4) [21 
U.S.C. 342(a)(4)] of the Act, protein derived from mammalian tissues to feeds 
that may be used for ruminants [21 C.F.R. 589.2000(e)(1)(iii)(B)] 
2013
Sunday, December 15, 2013 
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED 
VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
Tuesday, December 23, 2014 
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED 
VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION 
DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer 
and Elk in Animal Feed; Availability Date: Fri, 16 May 2003 11:47:37 -0500 EMC 1 
Terry S. Singeltary Sr. Vol #: 1 http://www.fda.gov/ohrms/dockets/dailys/03/Jun03/060903/060903.htm 
PLEASE SEE FULL TEXT SUBMISSION ; 
Discussion: The C, L and H type BSE cases in Canada exhibit molecular 
characteristics similar to those described for classical and atypical BSE cases 
from Europe and Japan. *** This supports the theory that the importation of BSE 
contaminated feedstuff is the source of C-type BSE in Canada. *** It also 
suggests a similar cause or source for atypical BSE in these countries. *** 
see page 176 of 201 pages...tss 
*** Singeltary reply ; 
Molecular, Biochemical and Genetic Characteristics of BSE in Canada 
Susceptibility of European Red Deer (Cervus elaphus elaphus) to Alimentary 
Challenge with Bovine Spongiform Encephalopathy 
Mark P. Dagleish , * E-mail: mark.dagleish@moredun.ac.uk
Affiliation: Moredun Research Institute, Pentlands Science Park, Bush Loan, 
Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Stuart Martin, Affiliation: Animal Health & Veterinary Laboratories 
Agency Lasswade, Pentlands Science Park, Bush Loan, Penicuik, Near Edinburgh 
EH26 0PZ, United Kingdom 
⨯ Philip Steele, Affiliation: Moredun Research Institute, Pentlands Science 
Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Jeanie Finlayson, Affiliation: Moredun Research Institute, Pentlands 
Science Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Samantha L. Eaton, Affiliation: Neurobiology Division, The Roslin 
Institute at, Royal (Dick) School of Veterinary Studies, University of 
Edinburgh, Easter Bush Campus, Midlothian, EH25 9RG, United Kingdom 
⨯ Sílvia Sisó, Affiliation: Animal Health & Veterinary Laboratories 
Agency Lasswade, Pentlands Science Park, Bush Loan, Penicuik, Near Edinburgh 
EH26 0PZ, United Kingdom 
⨯ Paula Stewart, Affiliation: Neurobiology Division, The Roslin Institute 
at, Royal (Dick) School of Veterinary Studies, University of Edinburgh, Easter 
Bush Campus, Midlothian, EH25 9RG, United Kingdom 
⨯ Natalia Fernández-Borges, Affiliation: CIC bioGUNE, Parque tecnológico de 
Bizkaia, Derio 48160, Spain 
⨯ Scott Hamilton, Affiliation: Moredun Research Institute, Pentlands 
Science Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Yvonne Pang, Affiliation: Moredun Research Institute, Pentlands Science 
Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Francesca Chianini, Affiliation: Moredun Research Institute, Pentlands 
Science Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Hugh W. Reid, Affiliation: Moredun Research Institute, Pentlands Science 
Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Wilfred Goldmann, Affiliation: Neurobiology Division, The Roslin 
Institute at, Royal (Dick) School of Veterinary Studies, University of 
Edinburgh, Easter Bush Campus, Midlothian, EH25 9RG, United Kingdom 
⨯ Lorenzo González, Affiliation: Animal Health & Veterinary 
Laboratories Agency Lasswade, Pentlands Science Park, Bush Loan, Penicuik, Near 
Edinburgh EH26 0PZ, United Kingdom 
⨯ Joaquín Castilla, Affiliations: CIC bioGUNE, Parque tecnológico de 
Bizkaia, Derio 48160, Spain, IKERBASQUE, Basque Foundation for Science, Bilbao 
48013, Bizkaia, Spain 
⨯ [ ... ], Martin Jeffrey Affiliation: Animal Health & Veterinary 
Laboratories Agency Lasswade, Pentlands Science Park, Bush Loan, Penicuik, Near 
Edinburgh EH26 0PZ, United Kingdom 
⨯ [ view all ] [ view less ] Susceptibility of European Red Deer (Cervus 
elaphus elaphus) to Alimentary Challenge with Bovine Spongiform Encephalopathy 
Mark P. Dagleish, Stuart Martin, Philip Steele, Jeanie Finlayson, Samantha L. 
Eaton, Sílvia Sisó, Paula Stewart, Natalia Fernández-Borges, … Scott Hamilton, 
Yvonne Pang PLOS x Published: January 23, 2015 DOI: 10.1371/journal.pone.0116094 
Abstract
European red deer (Cervus elaphus elaphus) are susceptible to the agent of 
bovine spongiform encephalopathy, one of the transmissible spongiform 
encephalopathies, when challenged intracerebrally but their susceptibility to 
alimentary challenge, the presumed natural route of transmission, is unknown. To 
determine this, eighteen deer were challenged via stomach tube with a large dose 
of the bovine spongiform encephalopathy agent and clinical signs, gross and 
histological lesions, presence and distribution of abnormal prion protein and 
the attack rate recorded. Only a single animal developed clinical disease, and 
this was acute with both neurological and respiratory signs, at 1726 days post 
challenge although there was significant (27.6%) weight loss in the preceding 
141 days. The clinically affected animal had histological lesions of vacuolation 
in the neuronal perikaryon and neuropil, typical of transmissible spongiform 
encephalopathies. Abnormal prion protein, the diagnostic marker of transmissible 
encephalopathies, was primarily restricted to the central and peripheral nervous 
systems although a very small amount was present in tingible body macrophages in 
the lymphoid patches of the caecum and colon. Serial protein misfolding cyclical 
amplification, an in vitro ultra-sensitive diagnostic technique, was positive 
for neurological tissue from the single clinically diseased deer. All other 
alimentary challenged deer failed to develop clinical disease and were negative 
for all other investigations. These findings show that transmission of bovine 
spongiform encephalopathy to European red deer via the alimentary route is 
possible but the transmission rate is low. Additionally, when deer carcases are 
subjected to the same regulations that ruminants in Europe with respect to the 
removal of specified offal from the human food chain, the zoonotic risk of 
bovine spongiform encephalopathy, the cause of variant Creutzfeldt-Jakob 
disease, from consumption of venison is probably very low. 
snip...
Discussion This investigation resulted in the first and only known case, to 
date, of clinical disease or accumulation of abnormal PrPd in any cervid species 
due to oral challenge with BSE. The increase in incubation period compared to 
European red deer challenged with BSE intra-cerebrally (1060 days) [33] compared 
to oral challenge (1727 days) is approximately 60% and similar to the 
differences observed in incubation periods for sheep or goats when challenged 
with TSE agents by these two routes [40,41]. The neurological clinical signs 
observed could be broadly related to the spongiform encephalopathy and the 
accumulation of PrPd in that the restlessness, stereotypic head movements and 
pacing may be due to compromise of the nucleus accumbens [42], found in the 
striatum, and the laboured breathing due to the lesions in the medulla, where 
the respiratory centre is located [43]. Alternatively, the laboured and audible 
mouth breathing may have been due to, or contributed to by, compromise of either 
of the recurrent laryngeal nerves resulting in some degree of laryngeal 
paralysis but we were unable to determine this. Apart from the gradual loss of 
body weight, the speed of onset of clinical signs and progression was very rapid 
but animal welfare requirements precluded any further longitudinal study of 
these. The clinical signs described for this animal are broadly similar to those 
reported for clinical BSE in European red deer challenged via the intracerebral 
route [33], clinical cases of CWD in deer [44] and clinical cases of BSE in 
cattle [45].
snip...see full text ; 
*** Singeltary reply ; 
ruminant feed ban for cervids in the United States ? 
31 Jan 2015 at 20:14 GMT 
*** The potential impact of prion diseases on human health was greatly 
magnified by the recognition that interspecies transfer of BSE to humans by beef 
ingestion resulted in vCJD. While changes in animal feed constituents and 
slaughter practices appear to have curtailed vCJD, there is concern that CWD of 
free-ranging deer and elk in the U.S. might also cross the species barrier. 
Thus, consuming venison could be a source of human prion disease. Whether BSE 
and CWD represent interspecies scrapie transfer or are newly arisen prion 
diseases is unknown. Therefore, the possibility of transmission of prion disease 
through other food animals cannot be ruled out. There is evidence that vCJD can 
be transmitted through blood transfusion. There is likely a pool of unknown size 
of asymptomatic individuals infected with vCJD, and there may be asymptomatic 
individuals infected with the CWD equivalent. These circumstances represent a 
potential threat to blood, blood products, and plasma supplies. 
when an industry is catering to the public, with products which can risk 
human and animal health, in my opinion, you should have NO property rights. you 
should not be able to hide behind property rights when you are clearly risking 
human and animal health from your product, or the way you handle that product. 
if you are going to raise, grow, produce a product for the consumer, you have an 
obligation NOT to risk the public domain, public property, and or the wild 
animal populations. just my opinion, I still have that right in 2015. ... 
CHRONIC WASTING DISEASE CWD TSE PRION, how much does it pay to find CWD 
$$$
CWD, spreading it around...
Tuesday, January 06, 2015 
APHIS Provides Additional Information on Chronic Wasting Disease (CWD) 
Indemnity Requests January 5, 2015 05:26 PM EST
Sunday, December 28, 2014
CHRONIC WASTING DISEASE CWD TSE PRION DISEASE AKA MAD DEER DISIEASE 
USDAUSAHA INC DECEMBER 28, 2014
Thursday, March 20, 2014
CHRONIC WASTING DISEASE CWD TSE PRION OF CERVID AND THE POTENTIAL FOR HUMAN 
TRANSMISSION THEREFROM 2014
Tuesday, July 01, 2014
*** CHRONIC WASTING DISEASE CWD TSE PRION DISEASE, GAME FARMS, AND 
POTENTIAL RISK FACTORS THERE FROM ***
Thursday, July 03, 2014
*** How Chronic Wasting Disease is affecting deer population and what’s the 
risk to humans and pets? ***
Thursday
CWD TO HUMANS, AND RISK FACTORS THERE FROM (see latest science) 
Tuesday, November 04, 2014 
*** Six-year follow-up of a point-source exposure to CWD contaminated 
venison in an Upstate New York community: risk behaviours and health outcomes 
2005–2011 
TSS
    OHIO CONFIRMS SECOND POSTIVE CHRONIC WASTING DISEASE CWD on Yoder's properties near Millersburg
Second Ohio white-tailed deer tests positive for deadly brain disease 
 Print Email D'Arcy Egan, The Plain Dealer By D'Arcy Egan, The Plain Dealer 
Email the author | Follow on Twitter on April 01, 2015 at 2:10 PM, updated April 
01, 2015 at 3:09 PM 
 Ohio Dept. of Agriculture.jpg CLEVELAND, Ohio -- As state officials try to 
legally force Holmes County deer farmer Daniel Yoder to euthanize a herd of 
expensive white-tailed deer, a second deer has tested positive for chronic 
wasting disease (CWD) on Yoder's properties near Millersburg.
The first positive test ever for CWD in Ohio was from a deer killed Oct. 22 
at Yoder's hunting preserve, World Class Whitetails. The second positive result 
came a few days ago while testing another Yoder deer that had died.
The National Veterinary Services Laboratories in Ames, Iowa confirmed the 
results.
"It has always been a case of not if, but when another deer would test 
positive on those properties," said Communications Director Erica Hawkins of the 
Ohio Department of Agriculture. "This wasn't unexpected. We expected to find 
positives at his facilities and, with de-population, we expect to find 
more."
Yoder's two breeding farms and hunting preserve were quarantined a year ago 
after it was discovered an infected Pennsylvania deer had been shipped there. It 
has become common for the breeding and hunting operations to ship deer to other 
states.
In Ohio, the ODA manages captive deer herds. The Ohio Division of Wildlife 
manages the wild deer herd.
Breeding large-antlered deer to supply the fenced deer-hunting operations 
has become a booming business. The price for a deer sporting trophy antlers can 
range from a few thousands dollars to more than $50,000.
Because the disease is highly contagious and almost impossible to 
eradicate, Yoder's breeding farms and fenced deer hunting operation was shut 
down and all deer 12 months and older that die on the properties were required 
to be tested for CWD, regardless of the circumstances, said Hawkins.
While CWD has become a major problem in Wisconsin and many western states, 
Yoder's captive deer have been the only Ohio animals to test positive for the 
always-fatal brain disease. CWD is found in the wild, but spreads far more 
quickly among crowded herds of captive deer or elk.
The ODOW constantly tests road-killed deer and deer killed by hunters for 
the disease. Because of the positive test in Holmes County, wildlife officials 
focused on deer harvested from that area during the recent hunting 
seasons.
The disease is related to mad cow disease, but has never been contracted by 
humans, according to the Centers for Disease Control and Prevention.
The ODOW reported last fall at least two deer had escaped from Yoder's 
farms and were killed by sport hunters. There have also been other instances of 
deer escaping from fenced hunting operations around state.
Hawkins said discussions are still underway to determine the best way to 
eliminate the herd because of the high cost of destruction and disposal of the 
deer.
"A lot of questions still need to be answered," Hawkins said. "This is the 
first situation like this in Ohio."
Yoder was charged in Holmes County Common Pleas Court on Feb. 25 with two 
counts of tampering with evidence, reported the Wooster Daily Record. Yoder had 
given a customer packages of venison obtained from a doe after the trophy buck 
he had killed appeared to be ill. The customer received the antlers, but Yoder 
disposed of the buck's head and carcass without performing the required tests, 
charged ODA enforcement agent William Lesho.
Three West Virginia hunters had paid to kill three deer on the preserve on 
another date and the required samples of the deer were not collected. In order 
to determine if a deer has CWD, the brain or brain stem must be tested 
Thursday, October 23, 2014 
*** FIRST CASE OF CHRONIC WASTING DISEASE CONFIRMED IN OHIO ON PRIVATE 
PRESERVE 
Wednesday, February 11, 2015 
World Class Whitetails quarantined CWD deer Daniel M. Yoder charged with 
two counts of tampering with evidence 
Table 34. Other Animals and Animal Products – Inventory: 2012 and 2007 [For 
meaning of abbreviations and symbols, see introductory text.]
Deer in captivity TOTAL 2012 USA 
4,042 Farms
231,431 Number
Elk in captivity TOTAL 2012 USA
1,199 Farms
38,061 Number 
OHIO 
Deer in captivity 2012 TOTAL
234 Farms
5,911 Number
Elk in captivity 2012 TOTAL
25 Farms
401 Number 
see other Ohio figures here ;
Ohio currently has 684 deer propagation farms and 29 hunting preserves that 
include whitetails. 
2010 
ECONOMIC IMPACT OF OHIO DEER FARMING INDUSTRY
snip...
Ohio – Industry Leader 
• 15,084 deer kept on farms in Ohio 
• 695 deer farms in Ohio in 2009 
• 440 commercial deer farms in 2009 
• 14,209 deer kept on commercial farms with farms of up to 390 deer in 
size 
• State of Ohio has 9% of commercial deer farms within the U.S. in 2009 
• Deer farms located within 82 of 88 Ohio counties as of 2009 
see more here ;
Monday, June 11, 2012
*** OHIO Captive deer escapees and non-reporting ***
*** Ohio Division of Wildlife officers have killed five deer in the Holmes 
County area in recent months that had ear tags, most likely escapees from deer 
farms or high fence hunting preserves. Two of those deer were traced to the 
World Class Whitetails Hunting Preserve. None of the five deer tested positive 
for CWD.
http://www.cleveland.com/outdoors/index.ssf/2014/12/holmes_county_deer_hunting_pre.html#incart_river 
On the last Saturday of Ohio’s shotgun season, 17-year-old Alex Wright 
killed a 30-point monster that had escaped from a nearby high-fence hunting 
outfitter. Wright had heard rumors about the escape, and after seeing a trail 
camera image of this buck wandering the property he hunts, the Ulrichsville teen 
took down the non-typical that would have cost him more than $19,900 to shoot 
behind Stillwater Trophy Outfitters’ fence. 
Seven trophy whitetailed deer being raised by Byler meandered out of their 
suddenly not-so-fenced-in pen on April 26. It may prove to be a fatal escape. 
State wildlife officials intend to shoot and kill any runaways that Byler fails 
to round up within the next few days. 
Three remained on the lam as of Monday afternoon. Byler managed to 
recapture the other big money bucks last week with the help of friends. 
"All we need is more time," Byler said. 
He won't get much. 
The concern is chronic wasting disease entering Ohio, said Dan Kramer, a 
state wildlife management supervisor in Northeast Ohio.
Tuesday, October 21, 2014 
*** Pennsylvania Department of Agriculture Tenth Pennsylvania Captive Deer 
Tests Positive for Chronic Wasting Disease CWD TSE PRION DISEASE 
”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. 
Sunday, January 06, 2013 
USDA TO PGC ONCE CAPTIVES ESCAPE 
*** "it‘s no longer its business.” 
Sunday, July 13, 2014 
Louisiana deer mystery unleashes litigation 6 does still missing from CWD 
index herd in Pennsylvania Great Escape 
Saturday, June 29, 2013 
PENNSYLVANIA CAPTIVE CWD INDEX HERD MATE YELLOW *47 STILL RUNNING LOOSE IN 
INDIANA, YELLOW NUMBER 2 STILL MISSING, AND OTHERS ON THE RUN STILL IN LOUISIANA 
Tuesday, June 11, 2013 
*** CWD GONE WILD, More cervid escapees from more shooting pens on the 
loose in Pennsylvania 
Wednesday, September 04, 2013 
***cwd - cervid captive livestock escapes, loose and on the run in the 
wild... 
Tuesday, October 07, 2014 
*** Wisconsin white-tailed deer tested positive for CWD on a Richland 
County breeding farm, and a case of CWD has been discovered on a Marathon County 
hunting preserve 
Thursday, October 02, 2014 
*** IOWA TEST RESULTS FROM CAPTIVE DEER HERD WITH CHRONIC WASTING DISEASE 
RELEASED 79.8 percent of the deer tested positive for the disease 
Tuesday, December 20, 2011
CHRONIC WASTING DISEASE CWD WISCONSIN Almond Deer (Buckhorn Flats) 
FarmUpdate DECEMBER 2011The CWD infection rate was nearly 80%, the highest ever 
in a North American captive herd. RECOMMENDATION: That the Board approve the 
purchase of 80acres of land for $465,000 for the Statewide Wildlife Habitat 
Program inPortage County and approve the restrictions on public use of the 
site.SUMMARY:
For Immediate Release Thursday, October 2, 2014 
Dustin Vande Hoef 515/281-3375 or 515/326-1616 (cell) or 
Dustin.VandeHoef@IowaAgriculture.gov
TEST RESULTS FROM CAPTIVE DEER HERD WITH CHRONIC WASTING DISEASE RELEASED 
79.8 percent of the deer tested positive for the disease
DES MOINES – The Iowa Department of Agriculture and Land Stewardship today 
announced that the test results from the depopulation of a quarantined captive 
deer herd in north-central Iowa showed that 284 of the 356 deer, or 79.8% of the 
herd, tested positive for Chronic Wasting Disease (CWD). The owners of the 
quarantined herd have entered into a fence maintenance agreement with the Iowa 
Department of Agriculture and Land Stewardship,which requires the owners to 
maintain the 8’ foot perimeter fence around the herd premises for five years 
after the depopulation was complete and the premises had been cleaned and 
disinfected CWD is a progressive, fatal, degenerative neurological disease of 
farmed and free-ranging deer, elk, and moose. There is no known treatment or 
vaccine for CWD. CWD is not a disease that affects humans.On July 18, 2012, USDA 
Animal and Plant Health Inspection Service’s (APHIS)National Veterinary Services 
Lab in Ames, IA confirmed that a male whitetail deer harvested from a hunting 
preserve in southeast IA was positive for CWD. An investigation revealed that 
this animal had just been introduced into the hunting preserve from the 
above-referenced captive deer herd in north-central Iowa.The captive deer herd 
was immediately quarantined to prevent the spread of CWD. The herd has remained 
in quarantine until its depopulation on August 25 to 27, 2014.The Iowa 
Department of Agriculture and Land Stewardship participated in a joint operation 
to depopulate the infected herd with USDA Veterinary Services, which was the 
lead agency, and USDA Wildlife Services.Federal indemnity funding became 
available in 2014. USDA APHIS appraised the captive deer herd of 376 animals at 
that time, which was before depopulation and testing, at $1,354,250. At that 
time a herd plan was developed with the owners and officials from USDA and the 
Iowa Department of Agriculture and Land Stewardship.Once the depopulation was 
complete and the premises had been cleaned and disinfected, indemnity of 
$917,100.00 from the USDA has been or will be paid to the owners as compensation 
for the 356 captive deer depopulated.The Iowa Department of Agriculture and Land 
Stewardship operates a voluntary CWD program for farms that sell live animals. 
Currently 145 Iowa farms participate in the voluntary program. The 
above-referenced captive deer facility left the voluntary CWD program prior to 
the discovery of the disease as they had stopped selling live animals. All deer 
harvested in a hunting preserve must be tested for CWD. -30-
*** see history of this CWD blunder here ; 
On June 5, 2013, DNR conducted a fence inspection, after gaining approval 
from surrounding landowners, and confirmed that the fenced had beencut or 
removed in at least four separate locations; that the fence had degraded and was 
failing to maintain the enclosure around the Quarantined Premises in at least 
one area; that at least three gates had been opened;and that deer tracks were 
visible in and around one of the open areas in the sand on both sides of the 
fence, evidencing movement of deer into the Quarantined Premises.
Tuesday, January 06, 2015 
APHIS Provides Additional Information on Chronic Wasting Disease (CWD) 
Indemnity Requests January 5, 2015 05:26 PM EST
***please read this*** 
98 | Veterinary Record | January 24, 2015
EDITORIAL
Scrapie: a particularly persistent pathogen
Cristina Acín
Resistant prions in the environment have been the sword of Damocles for 
scrapie control and eradication. Attempts to establish which physical and 
chemical agents could be applied to inactivate or moderate scrapie infectivity 
were initiated in the 1960s and 1970s,with the first study of this type focusing 
on the effect of heat treatment in reducing prion infectivity (Hunter and 
Millson 1964). Nowadays, most of the chemical procedures that aim to inactivate 
the prion protein are based on the method developed by Kimberlin and 
collaborators (1983). This procedure consists of treatment with 20,000 parts per 
million free chlorine solution, for a minimum of one hour, of all surfaces that 
need to be sterilised (in laboratories, lambing pens, slaughterhouses, and so 
on). Despite this, veterinarians and farmers may still ask a range of questions, 
such as ‘Is there an official procedure published somewhere?’ and ‘Is there an 
international organisation which recommends and defines the exact method of 
scrapie decontamination that must be applied?’
From a European perspective, it is difficult to find a treatment that could 
be applied, especially in relation to the disinfection of surfaces in lambing 
pens of affected flocks. A 999/2001 EU regulation on controlling spongiform 
encephalopathies (European Parliament and Council 2001) did not specify a 
particular decontamination measure to be used when an outbreak of scrapie is 
diagnosed. There is only a brief recommendation in Annex VII concerning the 
control and eradication of transmissible spongiform encephalopathies (TSE 
s).
Chapter B of the regulation explains the measures that must be applied if 
new caprine animals are to be introduced to a holding where a scrapie outbreak 
has previously been diagnosed. In that case, the statement indicates that 
caprine animals can be introduced ‘provided that a cleaning and disinfection of 
all animal housing on the premises has been carried out following 
destocking’.
Issues around cleaning and disinfection are common in prion prevention 
recommendations, but relevant authorities, veterinarians and farmers may have 
difficulties in finding the specific protocol which applies. The European Food 
and Safety Authority (EFSA ) published a detailed report about the efficacy of 
certain biocides, such as sodium hydroxide, sodium hypochlorite, guanidine and 
even a formulation of copper or iron metal ions in combination with hydrogen 
peroxide, against prions (EFSA 2009). The report was based on scientific 
evidence (Fichet and others 2004, Lemmer and others 2004, Gao and others 2006, 
Solassol and others 2006) but unfortunately the decontamination measures were 
not assessed under outbreak conditions.
The EFSA Panel on Biological Hazards recently published its conclusions on 
the scrapie situation in the EU after 10 years of monitoring and control of the 
disease in sheep and goats (EFSA 2014), and one of the most interesting findings 
was the Icelandic experience regarding the effect of disinfection in scrapie 
control. The Icelandic plan consisted of: culling scrapie-affected sheep or the 
whole flock in newly diagnosed outbreaks; deep cleaning and disinfection of 
stables, sheds, barns and equipment with high pressure washing followed by 
cleaning with 500 parts per million of hypochlorite; drying and treatment with 
300 ppm of iodophor; and restocking was not permitted for at least two years. 
Even when all of these measures were implemented, scrapie recurred on several 
farms, indicating that the infectious agent survived for years in the 
environment, even as many as 16 years after restocking (Georgsson and others 
2006).
In the rest of the countries considered in the EFSA (2014) report, 
recommendations for disinfection measures were not specifically defined at the 
government level. In the report, the only recommendation that is made for sheep 
is repopulation with sheep with scrapie-resistant genotypes. This reduces the 
risk of scrapie recurrence but it is difficult to know its effect on the 
infection.
Until the EFSA was established (in May 2003), scientific opinions about TSE 
s were provided by the Scientific Steering Committee (SSC) of the EC, whose 
advice regarding inactivation procedures focused on treating animal waste at 
high temperatures (150°C for three hours) and high pressure alkaline hydrolysis 
(SSC 2003). At the same time, the TSE Risk Management Subgroup of the Advisory 
Committee on Dangerous Pathogens (ACDP) in the UK published guidance on safe 
working and the prevention of TSE infection. Annex C of the ACDP report 
established that sodium hypochlorite was considered to be effective, but only if 
20,000 ppm of available chlorine was present for at least one hour, which has 
practical limitations such as the release of chlorine gas, corrosion, 
incompatibility with formaldehyde, alcohols and acids, rapid inactivation of its 
active chemicals and the stability of dilutions (ACDP 2009).
In an international context, the World Organisation for Animal Health (OIE) 
does not recommend a specific disinfection protocol for prion agents in its 
Terrestrial Code or Manual. Chapter 4.13 of the Terrestrial Code, General 
recommendations on disinfection and disinsection (OIE 2014), focuses on 
foot-and-mouth disease virus, mycobacteria and Bacillus anthracis, but not on 
prion disinfection. Nevertheless, the last update published by the OIE on bovine 
spongiform encephalopathy (OIE 2012) indicates that few effective 
decontamination techniques are available to inactivate the agent on surfaces, 
and recommends the removal of all organic material and the use of sodium 
hydroxide, or a sodium hypochlorite solution containing 2 per cent available 
chlorine, for more than one hour at 20ºC.
The World Health Organization outlines guidelines for the control of TSE s, 
and also emphasises the importance of mechanically cleaning surfaces before 
disinfection with sodium hydroxide or sodium hypochlorite for one hour (WHO 
1999).
Finally, the relevant agencies in both Canada and the USA suggest that the 
best treatments for surfaces potentially contaminated with prions are sodium 
hydroxide or sodium hypochlorite at 20,000 ppm. This is a 2 per cent solution, 
while most commercial household bleaches contain 5.25 per cent sodium 
hypochlorite. It is therefore recommended to dilute one part 5.25 per cent 
bleach with 1.5 parts water (CDC 2009, Canadian Food Inspection Agency 
2013).
So what should we do about disinfection against prions? First, it is 
suggested that a single protocol be created by international authorities to 
homogenise inactivation procedures and enable their application in all 
scrapie-affected countries. Sodium hypochlorite with 20,000 ppm of available 
chlorine seems to be the procedure used in most countries, as noted in a paper 
summarised on p 99 of this issue of Veterinary Record (Hawkins and others 2015). 
But are we totally sure of its effectiveness as a preventive measure in a 
scrapie outbreak? Would an in-depth study of the recurrence of scrapie disease 
be needed?
What we can conclude is that, if we want to fight prion diseases, and 
specifically classical scrapie, we must focus on the accuracy of diagnosis, 
monitoring and surveillance; appropriate animal identification and control of 
movements; and, in the end, have homogeneous and suitable protocols to 
decontaminate and disinfect lambing barns, sheds and equipment available to 
veterinarians and farmers. Finally, further investigations into the resistance 
of prion proteins in the diversity of environmental surfaces are required.
References
snip...
98 | Veterinary Record | January 24, 2015
Persistence of ovine scrapie infectivity in a farm environment following 
cleaning and decontamination 
Steve A. C. Hawkins, MIBiol, Pathology Department1, Hugh A. Simmons, BVSc 
MRCVS, MBA, MA Animal Services Unit1, Kevin C. Gough, BSc, PhD2 and Ben C. 
Maddison, BSc, PhD3 + Author Affiliations
1Animal and Plant Health Agency, Woodham Lane, New Haw, Addlestone, Surrey 
KT15 3NB, UK 2School of Veterinary Medicine and Science, The University of 
Nottingham, Sutton Bonington, Loughborough, Leicestershire LE12 5RD, UK 3ADAS 
UK, School of Veterinary Medicine and Science, The University of Nottingham, 
Sutton Bonington, Loughborough, Leicestershire LE12 5RD, UK E-mail for 
correspondence: ben.maddison@adas.co.uk Abstract Scrapie of sheep/goats and 
chronic wasting disease of deer/elk are contagious prion diseases where 
environmental reservoirs are directly implicated in the transmission of disease. 
In this study, the effectiveness of recommended scrapie farm decontamination 
regimens was evaluated by a sheep bioassay using buildings naturally 
contaminated with scrapie. Pens within a farm building were treated with either 
20,000 parts per million free chorine solution for one hour or were treated with 
the same but were followed by painting and full re-galvanisation or replacement 
of metalwork within the pen. Scrapie susceptible lambs of the PRNP genotype 
VRQ/VRQ were reared within these pens and their scrapie status was monitored by 
recto-anal mucosa-associated lymphoid tissue. All animals became infected over 
an 18-month period, even in the pen that had been subject to the most stringent 
decontamination process. These data suggest that recommended current guidelines 
for the decontamination of farm buildings following outbreaks of scrapie do 
little to reduce the titre of infectious scrapie material and that environmental 
recontamination could also be an issue associated with these premises. 
SNIP...
Discussion
Thorough pressure washing of a pen had no effect on the amount of 
bioavailable scrapie infectivity (pen B). The routine removal of prions from 
surfaces within a laboratory setting is treatment for a minimum of one hour with 
20,000 ppm free chlorine, a method originally based on the use of brain 
macerates from infected rodents to evaluate the effectiveness of decontamination 
(Kimberlin and others 1983). Further studies have also investigated the 
effectiveness of hypochlorite disinfection of metal surfaces to simulate the 
decontamination of surgical devices within a hospital setting. Such treatments 
with hypochlorite solution were able to reduce infectivity by 5.5 logs to lower 
than the sensitivity of the bioassay used (Lemmer and others 2004). Analogous 
treatment of the pen surfaces did not effectively remove the levels of scrapie 
infectivity over that of the control pens, indicating that this method of 
decontamination is not effective within a farm setting. This may be due to the 
high level of biological matrix that is present upon surfaces within the farm 
environment, which may reduce the amount of free chlorine available to 
inactivate any infectious prion. Remarkably 1/5 sheep introduced into pen D had 
also became scrapie positive within nine months, with all animals in this pen 
being RAMALT positive by 18 months of age. Pen D was no further away from the 
control pen (pen A) than any of the other pens within this barn. Localised hot 
spots of infectivity may be present within scrapie-contaminated environments, 
but it is unlikely that pen D area had an amount of scrapie contamination that 
was significantly different than the other areas within this building. 
Similarly, there were no differences in how the biosecurity of pen D was 
maintained, or how this pen was ventilated compared with the other pens. This 
observation, perhaps, indicates the slower kinetics of disease uptake within 
this pen and is consistent with a more thorough prion removal and 
recontamination. These observations may also account for the presence of 
inadvertent scrapie cases within other studies, where despite stringent 
biosecurity, control animals have become scrapie positive during challenge 
studies using barns that also housed scrapie-affected animals (Ryder and others 
2009). The bioassay data indicate that the exposure of the sheep to a farm 
environment after decontamination efforts thought to be effective in removing 
scrapie is sufficient for the animals to become infected with scrapie. The main 
exposure routes within this scenario are likely to be via the oral route, during 
feeding and drinking, and respiratory and conjunctival routes. It has been 
demonstrated that scrapie infectivity can be efficiently transmitted via the 
nasal route in sheep (Hamir and others 2008), as is the case for CWD in both 
murine models and in white-tailed deer (Denkers and others 2010, 2013). 
Recently, it has also been demonstrated that CWD prions presented as dust when 
bound to the soil mineral montmorillonite can be infectious via the nasal route 
(Nichols and others 2013). When considering pens C and D, the actual source of 
the infectious agent in the pens is not known, it is possible that biologically 
relevant levels of prion survive on surfaces during the decontamination regimen 
(pen C). With the use of galvanising and painting (pen D) covering and sealing 
the surface of the pen, it is possible that scrapie material recontaminated the 
pens by the movement of infectious prions contained within dusts originating 
from other parts of the barn that were not decontaminated or from other areas of 
the farm.
Given that scrapie prions are widespread on the surfaces of affected farms 
(Maddison and others 2010a), irrespective of the source of the infectious prions 
in the pens, this study clearly highlights the difficulties that are faced with 
the effective removal of environmentally associated scrapie infectivity. This is 
likely to be paralleled in CWD which shows strong similarities to scrapie in 
terms of both the dissemination of prions into the environment and the facile 
mode of disease transmission. These data further contribute to the understanding 
that prion diseases can be highly transmissible between susceptible individuals 
not just by direct contact but through highly stable environmental reservoirs 
that are refractory to decontamination.
The presence of these environmentally associated prions in farm buildings 
make the control of these diseases a considerable challenge, especially in 
animal species such as goats where there is lack of genetic resistance to 
scrapie and, therefore, no scope to re-stock farms with animals that are 
resistant to scrapie.
Scrapie Sheep Goats Transmissible spongiform encephalopathies (TSE) 
Accepted October 12, 2014. Published Online First 31 October 2014 
Tuesday, December 16, 2014 
Evidence for zoonotic potential of ovine scrapie prions 
Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves 
Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle 
Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia 
Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, 
Affiliations Contributions Corresponding author Journal name: Nature 
Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 
Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 
Article tools Citation Reprints Rights & permissions Article metrics 
Abstract 
Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant 
Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie 
prions remains unknown. Mice genetically engineered to overexpress the human 
prion protein (tgHu) have emerged as highly relevant models for gauging the 
capacity of prions to transmit to humans. These models can propagate human 
prions without any apparent transmission barrier and have been used used to 
confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie 
prions transmit to several tgHu mice models with an efficiency comparable to 
that of cattle BSE. The serial transmission of different scrapie isolates in 
these mice led to the propagation of prions that are phenotypically identical to 
those causing sporadic CJD (sCJD) in humans. These results demonstrate that 
scrapie prions have a zoonotic potential and raise new questions about the 
possible link between animal and human prions.
Subject terms: Biological sciences• Medical research At a glance
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 
Friday, January 30, 2015
Scrapie: a particularly persistent pathogen
Monday, November 3, 2014 
Persistence of ovine scrapie infectivity in a farm environment following 
cleaning and decontamination
PPo3-22:
Detection of Environmentally Associated PrPSc on a Farm with Endemic 
Scrapie
Ben C. Maddison,1 Claire A. Baker,1 Helen C. Rees,1 Linda A. Terry,2 Leigh 
Thorne,2 Susan J. Belworthy2 and Kevin C. Gough3 1ADAS-UK LTD; Department of 
Biology; University of Leicester; Leicester, UK; 2Veterinary Laboratories 
Agency; Surry, KT UK; 3Department of Veterinary Medicine and Science; University 
of Nottingham; Sutton Bonington, Loughborough UK
Key words: scrapie, evironmental persistence, sPMCA
Ovine scrapie shows considerable horizontal transmission, yet the routes of 
transmission and specifically the role of fomites in transmission remain poorly 
defined. Here we present biochemical data demonstrating that on a 
scrapie-affected sheep farm, scrapie prion contamination is widespread. It was 
anticipated at the outset that if prions contaminate the environment that they 
would be there at extremely low levels, as such the most sensitive method 
available for the detection of PrPSc, serial Protein Misfolding Cyclic 
Amplification (sPMCA), was used in this study. We investigated the distribution 
of environmental scrapie prions by applying ovine sPMCA to samples taken from a 
range of surfaces that were accessible to animals and could be collected by use 
of a wetted foam swab. Prion was amplified by sPMCA from a number of these 
environmental swab samples including those taken from metal, plastic and wooden 
surfaces, both in the indoor and outdoor environment. At the time of sampling 
there had been no sheep contact with these areas for at least 20 days prior to 
sampling indicating that prions persist for at least this duration in the 
environment. These data implicate inanimate objects as environmental reservoirs 
of prion infectivity which are likely to contribute to disease transmission. 
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. 
*** We conclude that TSE infectivity is likely to survive burial for long 
time periods with minimal loss of infectivity and limited movement from the 
original burial site. However PMCA results have shown that there is the 
potential for rainwater to elute TSE related material from soil which could lead 
to the contamination of a wider area. These experiments reinforce the importance 
of risk assessment when disposing of TSE risk materials. 
*** The results show that even highly diluted PrPSc can bind efficiently to 
polypropylene, stainless steel, glass, wood and stone and propagate the 
conversion of normal prion protein. For in vivo experiments, hamsters were ic 
injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, 
inoculated with 263K-contaminated implants of all groups, developed typical 
signs of prion disease, whereas control animals inoculated with non-contaminated 
materials did not.
PRION 2014 CONFERENCE
CHRONIC WASTING DISEASE CWD 
A FEW FINDINGS ; 
Conclusions. To our knowledge, this is the first established experimental 
model of CWD in TgSB3985. We found evidence for co-existence or divergence of 
two CWD strains adapted to Tga20 mice and their replication in TgSB3985 mice. 
Finally, we observed phenotypic differences between cervid-derived CWD and 
CWD/Tg20 strains upon propagation in TgSB3985 mice. Further studies are underway 
to characterize these strains. 
We conclude that TSE infectivity is likely to survive burial for long time 
periods with minimal loss of infectivity and limited movement from the original 
burial site. However PMCA results have shown that there is the potential for 
rainwater to elute TSE related material from soil which could lead to the 
contamination of a wider area. These experiments reinforce the importance of 
risk assessment when disposing of TSE risk materials. 
The results show that even highly diluted PrPSc can bind efficiently to 
polypropylene, stainless steel, glass, wood and stone and propagate the 
conversion of normal prion protein. For in vivo experiments, hamsters were ic 
injected with implants incubated in 1% 263K-infected brain homogenate. Hamsters, 
inoculated with 263K-contaminated implants of all groups, developed typical 
signs of prion disease, whereas control animals inoculated with non-contaminated 
materials did not.
Our data establish that meadow voles are permissive to CWD via peripheral 
exposure route, suggesting they could serve as an environmental reservoir for 
CWD. Additionally, our data are consistent with the hypothesis that at least two 
strains of CWD circulate in naturally-infected cervid populations and provide 
evidence that meadow voles are a useful tool for CWD strain typing. 
Conclusion. CWD prions are shed in saliva and urine of infected deer as 
early as 3 months post infection and throughout the subsequent >1.5 year 
course of infection. In current work we are examining the relationship of 
prionemia to excretion and the impact of excreted prion binding to surfaces and 
particulates in the environment.
Conclusion. CWD prions (as inferred by prion seeding activity by RT-QuIC) 
are shed in urine of infected deer as early as 6 months post inoculation and 
throughout the subsequent disease course. Further studies are in progress 
refining the real-time urinary prion assay sensitivity and we are examining more 
closely the excretion time frame, magnitude, and sample variables in 
relationship to inoculation route and prionemia in naturally and experimentally 
CWD-infected cervids.
Conclusions. Our results suggested that the odds of infection for CWD is 
likely controlled by areas that congregate deer thus increasing direct 
transmission (deer-to-deer interactions) or indirect transmission 
(deer-to-environment) by sharing or depositing infectious prion proteins in 
these preferred habitats. Epidemiology of CWD in the eastern U.S. is likely 
controlled by separate factors than found in the Midwestern and endemic areas 
for CWD and can assist in performing more efficient surveillance efforts for the 
region.
Conclusions. During the pre-symptomatic stage of CWD infection and 
throughout the course of disease deer may be shedding multiple LD50 doses per 
day in their saliva. CWD prion shedding through saliva and excreta may account 
for the unprecedented spread of this prion disease in nature. 
see full text and more ; 
Monday, June 23, 2014 
*** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD 
*** Infectious agent of sheep scrapie may persist in the environment for at 
least 16 years*** 
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3 
New studies on the heat resistance of hamster-adapted scrapie agent: 
Threshold survival after ashing at 600°C suggests an inorganic template of 
replication 
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel 
Production 
Detection of protease-resistant cervid prion protein in water from a 
CWD-endemic area 
A Quantitative Assessment of the Amount of Prion Diverted to Category 1 
Materials and Wastewater During Processing 
Rapid assessment of bovine spongiform encephalopathy prion inactivation by 
heat treatment in yellow grease produced in the industrial manufacturing process 
of meat and bone meals 
Sunday, December 21, 2014 
Mucosal immunization with an attenuated Salmonella vaccine partially 
protects white-tailed deer from chronic wasting disease 
Friday, December 19, 2014 
Pan-Provincial Vaccine Enterprise Inc. (PREVENT) Conducting a Chronic 
Wasting Disease (CWD) Vaccine Efficacy Trial in Elk 
CHRONIC WASTING DISEASE CWD TSE PRION, how much does it pay to find CWD 
$$$
CWD, spreading it around...
for the game farm industry, and their constituents, to continue to believe 
that they are _NOT_, and or insinuate that they have _NEVER_ been part of the 
problem, will only continue to help spread cwd. the game farming industry, from 
the shooting pens, to the urine mills, the antler mills, the sperm mills, velvet 
mills, shooting pens, to large ranches, are not the only problem, but it is 
painfully obvious that they have been part of the problem for decades and 
decades, just spreading it around, as with transportation and or exportation and 
or importation of cervids from game farming industry, and have been proven to 
spread cwd. no one need to look any further than South Korea blunder ; 
=========================================== 
spreading cwd around...
Between 1996 and 2002, chronic wasting disease was diagnosed in 39 herds of 
farmed elk in Saskatchewan in a single epidemic. All of these herds were 
depopulated as part of the Canadian Food Inspection Agency’s (CFIA) disease 
eradication program. Animals, primarily over 12 mo of age, were tested for the 
presence CWD prions following euthanasia. Twenty-one of the herds were linked 
through movements of live animals with latent CWD from a single infected source 
herd in Saskatchewan, 17 through movements of animals from 7 of the secondarily 
infected herds. 
***The source herd is believed to have become infected via importation of 
animals from a game farm in South Dakota where CWD was subsequently diagnosed 
(7,4). A wide range in herd prevalence of CWD at the time of herd depopulation 
of these herds was observed. Within-herd transmission was observed on some 
farms, while the disease remained confined to the introduced animals on other 
farms. 
spreading cwd around...
Friday, May 13, 2011 
Chronic Wasting Disease (CWD) outbreaks and surveillance program in the 
Republic of Korea 
Chronic Wasting Disease (CWD) outbreaks and surveillance program in the 
Republic of Korea 
Hyun-Joo Sohn, Yoon-Hee Lee, Min-jeong Kim, Eun-Im Yun, Hyo-Jin Kim, 
Won-Yong Lee, Dong-Seob Tark, In- Soo Cho, Foreign Animal Disease Research 
Division, National Veterinary Research and Quarantine Service, Republic of Korea 
Chronic wasting disease (CWD) has been recognized as an important prion 
disease in native North America deer and Rocky mountain elks. The disease is a 
unique member of the transmissible spongiform encephalopathies (TSEs), which 
naturally affects only a few species. CWD had been limited to USA and Canada 
until 2000. 
On 28 December 2000, information from the Canadian government showed that a 
total of 95 elk had been exported from farms with CWD to Korea. These consisted 
of 23 elk in 1994 originating from the so-called “source farm” in Canada, and 72 
elk in 1997, which had been held in pre export quarantine at the “source 
farm”.Based on export information of CWD suspected elk from Canada to Korea, CWD 
surveillance program was initiated by the Ministry of Agriculture and Forestry 
(MAF) in 2001. 
All elks imported in 1997 were traced back, however elks imported in 1994 
were impossible to identify. CWD control measures included stamping out of all 
animals in the affected farm, and thorough cleaning and disinfection of the 
premises. In addition, nationwide clinical surveillance of Korean native 
cervids, and improved measures to ensure reporting of CWD suspect cases were 
implemented. 
Total of 9 elks were found to be affected. CWD was designated as a 
notifiable disease under the Act for Prevention of Livestock Epidemics in 2002. 
Additional CWD cases - 12 elks and 2 elks - were diagnosed in 2004 and 
2005. 
Since February of 2005, when slaughtered elks were found to be positive, 
all slaughtered cervid for human consumption at abattoirs were designated as 
target of the CWD surveillance program. Currently, CWD laboratory testing is 
only conducted by National Reference Laboratory on CWD, which is the Foreign 
Animal Disease Division (FADD) of National Veterinary Research and Quarantine 
Service (NVRQS). 
In July 2010, one out of 3 elks from Farm 1 which were slaughtered for the 
human consumption was confirmed as positive. Consequently, all cervid – 54 elks, 
41 Sika deer and 5 Albino deer – were culled and one elk was found to be 
positive. Epidemiological investigations were conducted by Veterinary 
Epidemiology Division (VED) of NVRQS in collaboration with provincial veterinary 
services. 
Epidemiologically related farms were found as 3 farms and all cervid at 
these farms were culled and subjected to CWD diagnosis. Three elks and 5 
crossbreeds (Red deer and Sika deer) were confirmed as positive at farm 2. 
All cervids at Farm 3 and Farm 4 – 15 elks and 47 elks – were culled and 
confirmed as negative. 
Further epidemiological investigations showed that these CWD outbreaks were 
linked to the importation of elks from Canada in 1994 based on circumstantial 
evidences. 
In December 2010, one elk was confirmed as positive at Farm 5. 
Consequently, all cervid – 3 elks, 11 Manchurian Sika deer and 20 Sika deer – 
were culled and one Manchurian Sika deer and seven Sika deer were found to be 
positive. This is the first report of CWD in these sub-species of deer. 
Epidemiological investigations found that the owner of the Farm 2 in CWD 
outbreaks in July 2010 had co-owned the Farm 5. 
In addition, it was newly revealed that one positive elk was introduced 
from Farm 6 of Jinju-si Gyeongsang Namdo. All cervid – 19 elks, 15 crossbreed 
(species unknown) and 64 Sika deer – of Farm 6 were culled, but all confirmed as 
negative. 
Tuesday, February 10, 2015 
Alberta Canada First case of chronic wasting disease found in farm elk 
since 2002 
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... 
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 
BSE RUMINANT FEED BAN FOR CERVIDS AND PETS IN THE USA ?
in short, there is none, and never has been. 
I am concerned with pets as well.
I strongly, strenuously, urge the FDA et al and scientist (minus the 
industry, politicians, and lobbyist there from on all issues), to revisit the 
foolish voluntary ban on ruminant feed to cervids, and adopt an immediate 
measure to make mandatory the ban of all ruminant feed to all cervids and pets. 
... TSS 
FDA WARNING LETTER (14-ATL-04) adulterated under Section 402(a)(4) [21 
U.S.C. 342(a)(4)] of the Act, protein derived from mammalian tissues to feeds 
that may be used for ruminants [21 C.F.R. 589.2000(e)(1)(iii)(B)] 
2013
Sunday, December 15, 2013 
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED 
VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
Tuesday, December 23, 2014 
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED 
VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION 
DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer 
and Elk in Animal Feed; Availability Date: Fri, 16 May 2003 11:47:37 -0500 EMC 1 
Terry S. Singeltary Sr. Vol #: 1 http://www.fda.gov/ohrms/dockets/dailys/03/Jun03/060903/060903.htm 
PLEASE SEE FULL TEXT SUBMISSION ; 
Discussion: The C, L and H type BSE cases in Canada exhibit molecular 
characteristics similar to those described for classical and atypical BSE cases 
from Europe and Japan. *** This supports the theory that the importation of BSE 
contaminated feedstuff is the source of C-type BSE in Canada. *** It also 
suggests a similar cause or source for atypical BSE in these countries. *** 
see page 176 of 201 pages...tss 
*** Singeltary reply ; 
Molecular, Biochemical and Genetic Characteristics of BSE in Canada 
Susceptibility of European Red Deer (Cervus elaphus elaphus) to Alimentary 
Challenge with Bovine Spongiform Encephalopathy 
Mark P. Dagleish , * E-mail: mark.dagleish@moredun.ac.uk
Affiliation: Moredun Research Institute, Pentlands Science Park, Bush Loan, 
Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Stuart Martin, Affiliation: Animal Health & Veterinary Laboratories 
Agency Lasswade, Pentlands Science Park, Bush Loan, Penicuik, Near Edinburgh 
EH26 0PZ, United Kingdom 
⨯ Philip Steele, Affiliation: Moredun Research Institute, Pentlands Science 
Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Jeanie Finlayson, Affiliation: Moredun Research Institute, Pentlands 
Science Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Samantha L. Eaton, Affiliation: Neurobiology Division, The Roslin 
Institute at, Royal (Dick) School of Veterinary Studies, University of 
Edinburgh, Easter Bush Campus, Midlothian, EH25 9RG, United Kingdom 
⨯ Sílvia Sisó, Affiliation: Animal Health & Veterinary Laboratories 
Agency Lasswade, Pentlands Science Park, Bush Loan, Penicuik, Near Edinburgh 
EH26 0PZ, United Kingdom 
⨯ Paula Stewart, Affiliation: Neurobiology Division, The Roslin Institute 
at, Royal (Dick) School of Veterinary Studies, University of Edinburgh, Easter 
Bush Campus, Midlothian, EH25 9RG, United Kingdom 
⨯ Natalia Fernández-Borges, Affiliation: CIC bioGUNE, Parque tecnológico de 
Bizkaia, Derio 48160, Spain 
⨯ Scott Hamilton, Affiliation: Moredun Research Institute, Pentlands 
Science Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Yvonne Pang, Affiliation: Moredun Research Institute, Pentlands Science 
Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Francesca Chianini, Affiliation: Moredun Research Institute, Pentlands 
Science Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Hugh W. Reid, Affiliation: Moredun Research Institute, Pentlands Science 
Park, Bush Loan, Penicuik, Near Edinburgh EH26 0PZ, United Kingdom 
⨯ Wilfred Goldmann, Affiliation: Neurobiology Division, The Roslin 
Institute at, Royal (Dick) School of Veterinary Studies, University of 
Edinburgh, Easter Bush Campus, Midlothian, EH25 9RG, United Kingdom 
⨯ Lorenzo González, Affiliation: Animal Health & Veterinary 
Laboratories Agency Lasswade, Pentlands Science Park, Bush Loan, Penicuik, Near 
Edinburgh EH26 0PZ, United Kingdom 
⨯ Joaquín Castilla, Affiliations: CIC bioGUNE, Parque tecnológico de 
Bizkaia, Derio 48160, Spain, IKERBASQUE, Basque Foundation for Science, Bilbao 
48013, Bizkaia, Spain 
⨯ [ ... ], Martin Jeffrey Affiliation: Animal Health & Veterinary 
Laboratories Agency Lasswade, Pentlands Science Park, Bush Loan, Penicuik, Near 
Edinburgh EH26 0PZ, United Kingdom 
⨯ [ view all ] [ view less ] Susceptibility of European Red Deer (Cervus 
elaphus elaphus) to Alimentary Challenge with Bovine Spongiform Encephalopathy 
Mark P. Dagleish, Stuart Martin, Philip Steele, Jeanie Finlayson, Samantha L. 
Eaton, Sílvia Sisó, Paula Stewart, Natalia Fernández-Borges, … Scott Hamilton, 
Yvonne Pang PLOS x Published: January 23, 2015 DOI: 10.1371/journal.pone.0116094 
Abstract
European red deer (Cervus elaphus elaphus) are susceptible to the agent of 
bovine spongiform encephalopathy, one of the transmissible spongiform 
encephalopathies, when challenged intracerebrally but their susceptibility to 
alimentary challenge, the presumed natural route of transmission, is unknown. To 
determine this, eighteen deer were challenged via stomach tube with a large dose 
of the bovine spongiform encephalopathy agent and clinical signs, gross and 
histological lesions, presence and distribution of abnormal prion protein and 
the attack rate recorded. Only a single animal developed clinical disease, and 
this was acute with both neurological and respiratory signs, at 1726 days post 
challenge although there was significant (27.6%) weight loss in the preceding 
141 days. The clinically affected animal had histological lesions of vacuolation 
in the neuronal perikaryon and neuropil, typical of transmissible spongiform 
encephalopathies. Abnormal prion protein, the diagnostic marker of transmissible 
encephalopathies, was primarily restricted to the central and peripheral nervous 
systems although a very small amount was present in tingible body macrophages in 
the lymphoid patches of the caecum and colon. Serial protein misfolding cyclical 
amplification, an in vitro ultra-sensitive diagnostic technique, was positive 
for neurological tissue from the single clinically diseased deer. All other 
alimentary challenged deer failed to develop clinical disease and were negative 
for all other investigations. These findings show that transmission of bovine 
spongiform encephalopathy to European red deer via the alimentary route is 
possible but the transmission rate is low. Additionally, when deer carcases are 
subjected to the same regulations that ruminants in Europe with respect to the 
removal of specified offal from the human food chain, the zoonotic risk of 
bovine spongiform encephalopathy, the cause of variant Creutzfeldt-Jakob 
disease, from consumption of venison is probably very low. 
snip...
Discussion This investigation resulted in the first and only known case, to 
date, of clinical disease or accumulation of abnormal PrPd in any cervid species 
due to oral challenge with BSE. The increase in incubation period compared to 
European red deer challenged with BSE intra-cerebrally (1060 days) [33] compared 
to oral challenge (1727 days) is approximately 60% and similar to the 
differences observed in incubation periods for sheep or goats when challenged 
with TSE agents by these two routes [40,41]. The neurological clinical signs 
observed could be broadly related to the spongiform encephalopathy and the 
accumulation of PrPd in that the restlessness, stereotypic head movements and 
pacing may be due to compromise of the nucleus accumbens [42], found in the 
striatum, and the laboured breathing due to the lesions in the medulla, where 
the respiratory centre is located [43]. Alternatively, the laboured and audible 
mouth breathing may have been due to, or contributed to by, compromise of either 
of the recurrent laryngeal nerves resulting in some degree of laryngeal 
paralysis but we were unable to determine this. Apart from the gradual loss of 
body weight, the speed of onset of clinical signs and progression was very rapid 
but animal welfare requirements precluded any further longitudinal study of 
these. The clinical signs described for this animal are broadly similar to those 
reported for clinical BSE in European red deer challenged via the intracerebral 
route [33], clinical cases of CWD in deer [44] and clinical cases of BSE in 
cattle [45].
snip...see full text ; 
*** Singeltary reply ; 
ruminant feed ban for cervids in the United States ? 
31 Jan 2015 at 20:14 GMT 
*** The potential impact of prion diseases on human health was greatly 
magnified by the recognition that interspecies transfer of BSE to humans by beef 
ingestion resulted in vCJD. While changes in animal feed constituents and 
slaughter practices appear to have curtailed vCJD, there is concern that CWD of 
free-ranging deer and elk in the U.S. might also cross the species barrier. 
Thus, consuming venison could be a source of human prion disease. Whether BSE 
and CWD represent interspecies scrapie transfer or are newly arisen prion 
diseases is unknown. Therefore, the possibility of transmission of prion disease 
through other food animals cannot be ruled out. There is evidence that vCJD can 
be transmitted through blood transfusion. There is likely a pool of unknown size 
of asymptomatic individuals infected with vCJD, and there may be asymptomatic 
individuals infected with the CWD equivalent. These circumstances represent a 
potential threat to blood, blood products, and plasma supplies. 
when an industry is catering to the public, with products which can risk 
human and animal health, in my opinion, you should have NO property rights. you 
should not be able to hide behind property rights when you are clearly risking 
human and animal health from your product, or the way you handle that product. 
if you are going to raise, grow, produce a product for the consumer, you have an 
obligation NOT to risk the public domain, public property, and or the wild 
animal populations. just my opinion, I still have that right in 2015. ... 
CHRONIC WASTING DISEASE CWD TSE PRION, how much does it pay to find CWD 
$$$
CWD, spreading it around...
Tuesday, January 06, 2015 
APHIS Provides Additional Information on Chronic Wasting Disease (CWD) 
Indemnity Requests January 5, 2015 05:26 PM EST
Sunday, December 28, 2014
CHRONIC WASTING DISEASE CWD TSE PRION DISEASE AKA MAD DEER DISIEASE 
USDAUSAHA INC DECEMBER 28, 2014
Thursday, March 20, 2014
CHRONIC WASTING DISEASE CWD TSE PRION OF CERVID AND THE POTENTIAL FOR HUMAN 
TRANSMISSION THEREFROM 2014
Tuesday, July 01, 2014
*** CHRONIC WASTING DISEASE CWD TSE PRION DISEASE, GAME FARMS, AND 
POTENTIAL RISK FACTORS THERE FROM ***
Thursday, July 03, 2014
*** How Chronic Wasting Disease is affecting deer population and what’s the 
risk to humans and pets? ***
Thursday
CWD TO HUMANS, AND RISK FACTORS THERE FROM (see latest science) 
Tuesday, November 04, 2014 
*** Six-year follow-up of a point-source exposure to CWD contaminated 
venison in an Upstate New York community: risk behaviours and health outcomes 
2005–2011 
TSS 


