Wednesday, January 24, 2018

Illinois Chronic Wasting Disease CWD TSE Prion cases mounting with 75 confirmed 2017 and 685 total to date

Illinois Chronic Wasting Disease: 2016-2017 Surveillance and Management Report (Project Period: July 1, 2016 - June 30, 2017) 

Paul Shelton and Patrick McDonald Forest Wildlife Program, Illinois Department of Natural Resources September 5, 2017 

Executive Summary

First CWD positive: A suspect adult female deer from northwest Boone County was diagnosed with CWD in November 2002.

Total samples through June 30, 2017: 105,836+

Total positives through June 30, 2017: 685

Number of counties affected through 6/30/2017: 17

(Boone, Carroll, DeKalb, DuPage, Grundy, JoDaviess, Kane, Kankakee, Kendall, Lake, LaSalle, Livingston, McHenry, Ogle, Stephenson, Will, Winnebago).

General distribution through 6/30/2017: Total affected area (determined by a minimum convex polygon that includes all positives) is now about 9,000 mi2 . While overall prevalence remains low in JoDaviess and Stephenson counties, disease is firmly established and appears to be worsening, accounting for 21/75 (28%) of all positives identified this year. Prevalence is somewhat higher in some eastern counties (Table 4) of the range, but deer populations in those areas are smaller and habitat much more fragmented.

CWD Surveillance Protocols During FY2017 (July 1, 2016-June 30, 2017) Testing: All CWD testing was conducted using immunohistochemistry (IHC) at the University of Illinois’ Veterinary Diagnostic Laboratory in Urbana, Illinois. Samples were initially screened using retropharyngeal lymph nodes (RPLN), followed by confirmatory testing of recut RPLN tissue and obex.

Sampling of hunter-harvested deer: Three sources were used to provide tissue samples from adult deer harvested by hunters: (1) mandatory firearm deer check stations in high-risk counties in northern Illinois; (2) designated voluntary drop-off testing locations in northern Illinois; and (3) cooperating meat lockers/taxidermists statewide who collected heads/sample tissues for IDNR.

Surveillance by other agencies/individuals authorized by special permits: Recipients of special permits from IDNR authorizing lethal deer removals were required to collect CWD samples when working in high-risk CWD areas or in areas needing additional surveillance. These permits included (1) Deer Population Control Permits (used by some agencies to control urban deer populations); (2) nuisance Deer Removal Permits (for crop depredation, etc.); and (3) Scientific Permits (various research projects).

Suspect (“target”) deer surveillance: Upon receiving reports from the public about sick deer, IDNR staff collected samples for CWD testing from deer that exhibited signs/symptoms consistent with chronic wasting disease. Surveillance from post-hunting season sharpshooting: Sharpshooting was conducted from mid-January through the end of March by trained IDNR staff. Sharpshooting was restricted to areas where CWD-infected deer had been identified (limited to lands within a 2-section buffer around known positive sections).

Executive Summary


CWD Surveillance Results FY2017

Total number of CWD samples collected statewide: 7,840 (7,839 WT deer, 1 elk). Figure 2 depicts the geographic distribution of sampling effort; Figure 3 compares annual sample numbers; Figure 4 presents a comparison of the number of deer sampled and the number of positives identified by source; and Appendix A summarizes the samples collected/positives identified by county.

Number of usable samples collected: 7,800 deer; 1 elk

Number of CWD-positive deer identified: 75. Table 1 presents a comparison of the number of positive deer found each year by county.

Number of counties with positive deer: 14 — Boone (7), Carroll (2), DeKalb (3), Grundy (7), JoDaviess (10), Kane (5), Kankakee (2), Kendall (6), LaSalle (4), Livingston (2), McHenry (8), Ogle (2), Stephenson (11), Winnebago (6). For distribution of positive sections, see Figure 5.

Number of new CWD counties: 1 (Carroll)

CWD prevalence information for the known CWD area (17 counties; adult deer from hunting sources only) —

Average CWD prevalence (all adult deer): 1.17% (43/3686)

Average CWD prevalence (adult males): 1.60% (31/1943)

Average CWD prevalence (adult females): 0.69% (12/1743)


Discussion: Illinois CWD in FY2017

Seventy-five CWD-positive deer were identified from 7,800 usable WT deer samples collected statewide.

Across the 17-county CWD range, the disease prevalence rate for all adult deer taken by hunters was
1.17%. The prevalence rate for hunter-harvested adult males (1.60%) was more than twice as high as for
adult females (0.69%). For comparison, deer taken by IDNR sharpshooters were more likely to be CWDpositive,
with an overall adult prevalence rate of 3.16%. Prevalence rate for adult males taken by sharpshooters
was 2.80%; for adult females taken by sharpshooters the rate was 3.41%. Prevalence rates have
remained low and changed little over time since discovery of CWD in 2002, but there is a slowly increasing
trend in recent years (Figure 7), most notably for adult males.

Surveillance data have identified a number of areas that present challenges for managing CWD, particularly
where heightened prevalence rates coincide with significant deer densities (Figure 8):

• Five of six positive deer found in Kendall County were from a relatively small area along the Fox River
south of Plano. As a result of this cluster of positives, Kendall County had the highest overall county
prevalence rate during 2017 at 9.26% (see Table 4), although sample size was low (n=54) and the confidence
interval was large.

• Estimated prevalence was >3.0% in the border area between JoDaviess and Stephenson counties, producing
a total of 16 positives from Blocks 0,-4 and 1,-4 (Figure 8). These included 2 positive deer identified
from the northern part of Carroll County, the first instances of CWD reported for that county. Deer
densities from those blocks have traditionally been very high, but weather conditions during the past
few winters have prevented the acquisition of current population data. Countywide prevalence rates in
Stephenson County appear to be on the rise (Figure 9), but in the absence of additional landowner cooperators
for DNR’s sharpshooting program, we are unlikely to change that trend.

• Prevalence rates along the Winnebago-Boone county line have remained above average in the years
since finding Illinois’ first positive there in 2002. Management has reduced deer densities and fewer
positives are being found as a result, but disease prevalence rates have not declined measurably in that
original focal area.

• The Marengo Ridge area in northern McHenry County (Fig. 8, Block 0,2) produced 7 positives from 97
samples. This area has been consistently high for the past four years.

• CWD-positive deer are being regularly identified each year along the Illinois River and its tributaries in
LaSalle and Grundy counties (Fig. 5 & Fig. 8). High deer densities along the Illinois River make control
efforts difficult, and emigration of deer from this area to non-CWD areas to the south poses a significant
risk for further CWD spread.

• CWD-positive deer are now identified routinely throughout northern Kane County (Fig. 8, Blocks 2,2
and 2,3). Much of the deer habitat is within subdivisions or owned by local units of government, so
opportunities for control of deer herds (and disease) via hunting are limited.

• The regularity with which we identify positive deer in spark areas such as Livingston and Kankakee
counties is disturbing. Deer habitat is limited in those counties but this may contribute to increased
mobility of deer across the landscape, with an increased probability for exploratory or migratory movements
and resultant increased risk of disease spread.


The spread of CWD into new areas and the establishment of new disease foci in those areas continues to be Illinois’ biggest management challenge. During 2002-2010, 80% of all CWD-positives identified in Illinois originated from either Boone or Winnebago County. In FY2017, only 17% of CWD-positives came from those counties. This shift in CWD distribution has posed significant challenges for management, because management must now be directed over a far larger area, spreading resources very thin. 

Disease management strategies in Illinois have been successful in controlling CWD prevalence rates at low levels, but in many management units the number of deer removed by sharpshooters is currently insufficient, whether because of limited access to property or because of our limited resources. Continuing our management program will slow increases in prevalence rates and also slow spread to the remainder of the state, but managers will require new tools in the future if we are to successfully fight this disease. 

IDNR staff must continue to work to educate Illinoisans about CWD and its potential to negatively impact our white-tailed deer herd. Only through an educated public can the Department continue to receive support for CWD management, even though our program is viewed as a model for disease management by professionals in many other states. 


Illinois six out of 19 deer samples tested positive for CWD in the Oswego zone of Kendall County

Illinois Loosing Ground to Chronic Wasting Disease CWD cases mounting with 71 confirmed in 2015 and 538 confirmed cases to date
Chronic Wasting Disease Page Content Update July 1, 2015: We now have a total of 538 cases of CWD
Note: Years are reported by fiscal year: 2015 is the period from July 1, 2014 through June 30, 2015, etc.
Total CWD Cases per year:
Year Cases ​

2015 ​71 

2014 59 

2013 36 

2012 36 

2011 42 

2010 37 

2009 30 

2008 38 

2007 42 

2006 51 

2005 31 

2004 51 

2003 14 

Total 538
 Monday, August 31, 2015
Illinois Loosing Ground to Chronic Wasting Disease CWD cases mounting with 71 confirmed in 2015 and 538 confirmed cases to date
see history of CWD in Illinois here
Saturday, February 08, 2014
Illinois CWD confirmed in Will County deer
Chronic Wasting Disease Illinois
Update July 1, 2013:
We now have a total of 408 cases of CWD.
Note: Years are reported by fiscal year: 2013 is the period from July 1, 2012 through June 30, 2013, etc.
Illinois CWD-Infected Sections - August 15, 2013
Monday, April 08, 2013
Evaluation of a wild white-tailed deer population management program for controlling chronic wasting disease in Illinois, 2003–2008
Wednesday, January 16, 2013
Illinois DuPage county deer found with Chronic Wasting Disease CWD
Tuesday, November 13, 2012 

Thursday, February 10, 2011
CWD ILLINOIS UPDATE FEBRUARY 2011 Locations of CWD-Positive Deer - Updated 2/07/2011
Thursday, January 28, 2010 CWD ILLINOIS UPDATE 2010
Saturday, March 08, 2008
CWD UPDATE ILLINOIS Stephenson County joins CWD list


Iowa Preliminary CWD TSE Prion Minimal Low Testing Reports 2 Confirmed With 5 Suspects To Date for 2017 Season


Contact tracing for the control of infectious disease epidemics: Chronic Wasting Disease in deer farms (Pennsylvania)

January 14, 2018

Ohio ODA confirms CWD TSE Prion in more captive deer


Wisconsin DNR has received confirmation wild deer tested positive for chronic wasting disease in the West Allis metropolitan area of Milwaukee County

January 14, 2018

Michigan’s Chronic Wasting Disease Working Group Recommendations Report to the Natural Resources Commission Prepared December 2017

57 cwd cases to date...

January 14, 2018

Arkansas AGFC Confirms 4 More CWD TSE Prion in WTD NW Counties 

January 14, 2018

Missouri MDC REPORTS 15 NEW CASES OF CWD TSE Prion in Deer 

January 14, 2018 

Texas TPWD CWD positive Panhandle Roadkill Whitetail 


Scientific opinion on chronic wasting disease (II) EFSA Panel on Biological Hazards (BIOHAZ)


Chronic Wasting Disease CWD TSE Prion (aka mad deer disease) Update USA December 14, 2017

What ever happened to 'LUCKY' the Wapiti cow elk thought to be immune from CWD with LL genotype MIA?


*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.
ALSO, one of the most, if not the most top TSE Prion God in Science today is Professor Adriano Aguzzi, and he recently commented on just this, on a cwd post on my facebook page August 20 at 1:44pm, quote;

''it pains me to no end to even comtemplate the possibility, but it seems entirely plausible that CWD originated from scientist-made spread of scrapie from sheep to deer in the colorado research facility. If true, a terrible burden for those involved.'' August 20 at 1:44pm ...end

*** After a natural route of exposure, 100% of WTD were susceptible to scrapie.

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

*** Infectious agent of sheep scrapie may persist in the environment for at least 16 years ***
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3
Classical scrapie is an environmentally transmissible disease because it has been reported in naïve, supposedly previously unexposed sheep placed in pastures formerly occupied by scrapie-infected sheep (4, 19, 20). Although the vector for disease transmission is not known, soil is likely to be an important reservoir for prions (2) where – based on studies in rodents – prions can adhere to minerals as a biologically active form (21) and remain infectious for more than 2 years (22). Similarly, chronic wasting disease (CWD) has re-occurred in mule deer housed in paddocks used by infected deer 2 years earlier, which was assumed to be through foraging and soil consumption (23).
Our study suggested that the risk of acquiring scrapie infection was greater through exposure to contaminated wooden, plastic, and metal surfaces via water or food troughs, fencing, and hurdles than through grazing. Drinking from a water trough used by the scrapie flock was sufficient to cause infection in sheep in a clean building. Exposure to fences and other objects used for rubbing also led to infection, which supported the hypothesis that skin may be a vector for disease transmission (9). The risk of these objects to cause infection was further demonstrated when 87% of 23 sheep presented with PrPSc in lymphoid tissue after grazing on one of the paddocks, which contained metal hurdles, a metal lamb creep and a water trough in contact with the scrapie flock up to 8 weeks earlier, whereas no infection had been demonstrated previously in sheep grazing on this paddock, when equipped with new fencing and field furniture. When the contaminated furniture and fencing were removed, the infection rate dropped significantly to 8% of 12 sheep, with soil of the paddock as the most likely source of infection caused by shedding of prions from the scrapie-infected sheep in this paddock up to a week earlier.
This study also indicated that the level of contamination of field furniture sufficient to cause infection was dependent on two factors: stage of incubation period and time of last use by scrapie-infected sheep. Drinking from a water trough that had been used by scrapie sheep in the predominantly pre-clinical phase did not appear to cause infection, whereas infection was shown in sheep drinking from the water trough used by scrapie sheep in the later stage of the disease. It is possible that contamination occurred through shedding of prions in saliva, which may have contaminated the surface of the water trough and subsequently the water when it was refilled. Contamination appeared to be sufficient to cause infection only if the trough was in contact with sheep that included clinical cases. Indeed, there is an increased risk of bodily fluid infectivity with disease progression in scrapie (24) and CWD (25) based on PrPSc detection by sPMCA. Although ultraviolet light and heat under natural conditions do not inactivate prions (26), furniture in contact with the scrapie flock, which was assumed to be sufficiently contaminated to cause infection, did not act as vector for disease if not used for 18 months, which suggest that the weathering process alone was sufficient to inactivate prions.
PrPSc detection by sPMCA is increasingly used as a surrogate for infectivity measurements by bioassay in sheep or mice. In this reported study, however, the levels of PrPSc present in the environment were below the limit of detection of the sPMCA method, yet were still sufficient to cause infection of in-contact animals. In the present study, the outdoor objects were removed from the infected flock 8 weeks prior to sampling and were positive by sPMCA at very low levels (2 out of 37 reactions). As this sPMCA assay also yielded 2 positive reactions out of 139 in samples from the scrapie-free farm, the sPMCA assay could not detect PrPSc on any of the objects above the background of the assay. False positive reactions with sPMCA at a low frequency associated with de novo formation of infectious prions have been reported (27, 28). This is in contrast to our previous study where we demonstrated that outdoor objects that had been in contact with the scrapie-infected flock up to 20 days prior to sampling harbored PrPSc that was detectable by sPMCA analysis [4 out of 15 reactions (12)] and was significantly more positive by the assay compared to analogous samples from the scrapie-free farm. This discrepancy could be due to the use of a different sPMCA substrate between the studies that may alter the efficiency of amplification of the environmental PrPSc. In addition, the present study had a longer timeframe between the objects being in contact with the infected flock and sampling, which may affect the levels of extractable PrPSc. Alternatively, there may be potentially patchy contamination of this furniture with PrPSc, which may have been missed by swabbing. The failure of sPMCA to detect CWD-associated PrP in saliva from clinically affected deer despite confirmation of infectivity in saliva-inoculated transgenic mice was associated with as yet unidentified inhibitors in saliva (29), and it is possible that the sensitivity of sPMCA is affected by other substances in the tested material. In addition, sampling of amplifiable PrPSc and subsequent detection by sPMCA may be more difficult from furniture exposed to weather, which is supported by the observation that PrPSc was detected by sPMCA more frequently in indoor than outdoor furniture (12). A recent experimental study has demonstrated that repeated cycles of drying and wetting of prion-contaminated soil, equivalent to what is expected under natural weathering conditions, could reduce PMCA amplification efficiency and extend the incubation period in hamsters inoculated with soil samples (30). This seems to apply also to this study even though the reduction in infectivity was more dramatic in the sPMCA assays than in the sheep model. Sheep were not kept until clinical end-point, which would have enabled us to compare incubation periods, but the lack of infection in sheep exposed to furniture that had not been in contact with scrapie sheep for a longer time period supports the hypothesis that prion degradation and subsequent loss of infectivity occurs even under natural conditions.
In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination. These results suggest that the VRQ/VRQ sheep model may be more sensitive than sPMCA for the detection of environmentally associated scrapie, and suggest that extremely low levels of scrapie contamination are able to cause infection in susceptible sheep genotypes.
Keywords: classical scrapie, prion, transmissible spongiform encephalopathy, sheep, field furniture, reservoir, serial protein misfolding cyclic amplification
Wednesday, December 16, 2015
*** Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission ***

Singeltary submission ;

Program Standards: Chronic Wasting Disease Herd Certification Program and Interstate Movement of Farmed or Captive Deer, Elk, and Moose

*** DOCUMENT ID: APHIS-2006-0118-0411


Subject: ***CDC Now Recommends Strongly consider having the deer or elk tested for CWD before you eat the meat

CDC Now Recommends Strongly consider having the deer or elk tested for CWD before you eat the meat 

Chronic Wasting Disease (CWD) 


If CWD could spread to people, it would most likely be through eating of infected deer and elk. In a 2006-2007 CDC survey of U.S. residents, nearly 20 percent of those surveyed said they had hunted deer or elk and more than two-thirds said they had eaten venison or elk meat. However, to date, no CWD infections have been reported in people. 

Hunters must consider many factors when determining whether to eat meat from deer and elk harvested from areas with CWD, including the level of risk they are willing to accept. Hunters harvesting wild deer and elk from areas with reported CWD should check state wildlife and public health guidance to see whether testing of animals is recommended or required in a given state or region. In areas where CWD is known to be present, CDC recommends that hunters strongly consider having those animals tested before eating the meat. 

Tests for CWD are monitoring tools that some state wildlife officials use to look at the rates of CWD in certain animal populations. Testing may not be available in every state, and states may use these tests in different ways. A negative test result does not guarantee that an individual animal is not infected with CWD, but it does make it considerably less likely and may reduce your risk of exposure to CWD. 

To be as safe as possible and decrease their potential risk of exposure to CWD, hunters should take the following steps when hunting in areas with CWD: 

Do not shoot, handle or eat meat from deer and elk that look sick or are acting strangely or are found dead (road-kill). When field-dressing a deer: Wear latex or rubber gloves when dressing the animal or handling the meat. Minimize how much you handle the organs of the animal, particularly the brain or spinal cord tissues. Do not use household knives or other kitchen utensils for field dressing. Check state wildlife and public health guidance to see whether testing of animals is recommended or required. Recommendations vary by state, but information about testing is available from many state wildlife agencies. Strongly consider having the deer or elk tested for CWD before you eat the meat. If you have your deer or elk commercially processed, consider asking that your animal be processed individually to avoid mixing meat from multiple animals. If your animal tests positive for CWD, do not eat meat from that animal. The U.S. Department of Agriculture’s Animal and Plant Health Inspection Service regulates commercially farmed deer and elk. The agency operates a national CWD herd certification program. As part of the voluntary program, states and individual herd owners agree to meet requirements meant to decrease the risk of CWD in their herds. Privately owned herds that do not participate in the herd certification program may be at increased risk for CWD. 

Page last reviewed: August 17, 2017 Page last updated: August 17, 2017 Content source: Centers for Disease Control and Prevention National Center for Emerging and Zoonotic Infectious Diseases (NCEZID) Division of High-Consequence Pathogens and Pathology (DHCPP) 

 > However, to date, no CWD infections have been reported in people. 

key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry 


*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).*** 

Molecular Barriers to Zoonotic Transmission of Prions 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein. 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype. 


CDC Now Recommends Strongly consider having the deer or elk tested for CWD before you eat the meat 

Prion 2017 Conference Abstracts CWD

First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress 

Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1 

University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen 

This is a progress report of a project which started in 2009. 21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves. 

Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice. 

At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation. 





 TUESDAY, JUNE 13, 2017


First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress

TUESDAY, JULY 04, 2017


TUESDAY, JUNE 13, 2017

PRION 2017 CONFERENCE ABSTRACT Chronic Wasting Disease in European moose is associated with PrPSc features different from North American CWD

Wednesday, May 24, 2017 

PRION2017 CONFERENCE VIDEO UPDATE 23 – 26 May 2017 Edinburgh UPDATE 1 

SATURDAY, JULY 29, 2017 

Risk Advisory Opinion: Potential Human Health Risks from Chronic Wasting Disease CFIA, PHAC, HC (HPFB and FNIHB), INAC, Parks Canada, ECCC and AAFC 

***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.*** 

SATURDAY, JULY 29, 2017 
Risk Advisory Opinion: Potential Human Health Risks from Chronic Wasting Disease CFIA, PHAC, HC (HPFB and FNIHB), INAC, Parks Canada, ECCC and AAFC 

Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿ 

Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations

 In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.

Prions in Skeletal Muscles of Deer with Chronic Wasting Disease 

Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.

*** WDA 2016 NEW YORK *** 

 We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species. We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions. 

 Wildlife Disease Risk Communication Research Contributes to Wildlife Trust Administration Exploring perceptions about chronic wasting disease risks among wildlife and agriculture professionals and stakeholders 

Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo Zoonotic Potential of CWD Prions: An Update 

Ignazio Cali1, Liuting Qing1, Jue Yuan1, Shenghai Huang2, Diane Kofskey1,3, Nicholas Maurer1, Debbie McKenzie4, Jiri Safar1,3,5, Wenquan Zou1,3,5,6, Pierluigi Gambetti1, Qingzhong Kong1,5,6 

1Department of Pathology, 3National Prion Disease Pathology Surveillance Center, 5Department of Neurology, 6National Center for Regenerative Medicine, Case Western Reserve University, Cleveland, OH 44106, USA. 

4Department of Biological Sciences and Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada, 

2Encore Health Resources, 1331 Lamar St, Houston, TX 77010 

Chronic wasting disease (CWD) is a widespread and highly transmissible prion disease in free-ranging and captive cervid species in North America. The zoonotic potential of CWD prions is a serious public health concern, but the susceptibility of human CNS and peripheral organs to CWD prions remains largely unresolved. We reported earlier that peripheral and CNS infections were detected in transgenic mice expressing human PrP129M or PrP129V. Here we will present an update on this project, including evidence for strain dependence and influence of cervid PrP polymorphisms on CWD zoonosis as well as the characteristics of experimental human CWD prions. 


In Conjunction with Asia Pacific Prion Symposium 2016 

PRION 2016 Tokyo 

Prion 2016 

Cervid to human prion transmission 

Kong, Qingzhong 

Case Western Reserve University, Cleveland, OH, United States 


Prion disease is transmissible and invariably fatal. Chronic wasting disease (CWD) is the prion disease affecting deer, elk and moose, and it is a widespread and expanding epidemic affecting 22 US States and 2 Canadian provinces so far. CWD poses the most serious zoonotic prion transmission risks in North America because of huge venison consumption (>6 million deer/elk hunted and consumed annually in the USA alone), significant prion infectivity in muscles and other tissues/fluids from CWD-affected cervids, and usually high levels of individual exposure to CWD resulting from consumption of the affected animal among often just family and friends. However, we still do not know whether CWD prions can infect humans in the brain or peripheral tissues or whether clinical/asymptomatic CWD zoonosis has already occurred, and we have no essays to reliably detect CWD infection in humans. We hypothesize that: 

(1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues; 

(2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence; 

(3) Reliable essays can be established to detect CWD infection in humans;and 

(4) CWD transmission to humans has already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches. 

Aim 1 will prove that the classical CWD strain may infect humans in brain or peripheral lymphoid tissues at low levels by conducting systemic bioassays in a set of "humanized" Tg mouse lines expressing common human PrP variants using a number of CWD isolates at varying doses and routes. Experimental "human CWD" samples will also be generated for Aim 3. 

Aim 2 will test the hypothesis that the cervid-to-human prion transmission barrier is dependent on prion strain and influenced by the host (human) PrP sequence by examining and comparing the transmission efficiency and phenotypes of several atypical/unusual CWD isolates/strains as well as a few prion strains from other species that have adapted to cervid PrP sequence, utilizing the same panel of humanized Tg mouse lines as in Aim 1. 

Aim 3 will establish reliable essays for detection and surveillance of CWD infection in humans by examining in details the clinical, pathological, biochemical and in vitro seeding properties of existing and future experimental "human CWD" samples generated from Aims 1-2 and compare them with those of common sporadic human Creutzfeldt-Jakob disease (sCJD) prions. 

Aim 4 will attempt to detect clinical CWD-affected human cases by examining a significant number of brain samples from prion-affected human subjects in the USA and Canada who have consumed venison from CWD-endemic areas utilizing the criteria and essays established in Aim 3. The findings from this proposal will greatly advance our understandings on the potential and characteristics of cervid prion transmission in humans, establish reliable essays for CWD zoonosis and potentially discover the first case(s) of CWD infection in humans. 

Public Health Relevance There are significant and increasing human exposure to cervid prions because chronic wasting disease (CWD, a widespread and highly infectious prion disease among deer and elk in North America) continues spreading and consumption of venison remains popular, but our understanding on cervid-to-human prion transmission is still very limited, raising public health concerns. This proposal aims to define the zoonotic risks of cervid prions and set up and apply essays to detect CWD zoonosis using mouse models and in vitro methods. The findings will greatly expand our knowledge on the potentials and characteristics of cervid prion transmission in humans, establish reliable essays for such infections and may discover the first case(s) of CWD infection in humans. 

Funding Agency Agency National Institute of Health (NIH) 

Institute National Institute of Neurological Disorders and Stroke (NINDS) 

Type Research Project (R01) 

Project # 1R01NS088604-01A1 

Application # 9037884 

Study Section Cellular and Molecular Biology of Neurodegeneration Study Section (CMND) 

Program Officer Wong, May 

Project Start 2015-09-30 

Project End 2019-07-31 

Budget Start 2015-09-30 

Budget End 2016-07-31 

Support Year 1 

Fiscal Year 2015 

Total Cost $337,507 

Indirect Cost $118,756 


Name Case Western Reserve University 

Department Pathology 

Type Schools of Medicine 

DUNS # 077758407 

City Cleveland 

State OH 

Country United States 

Zip Code 44106 

the tse prion aka mad cow type disease is not your normal pathogen. 

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

you cannot cook the TSE prion disease out of meat. 

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

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

the TSE prion agent also survives Simulated Wastewater Treatment Processes. 

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

you can bury it and it will not go away. 

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

it’s not your ordinary pathogen you can just cook it out and be done with. 

that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent.

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

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

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

Laboratory of Central Nervous System Studies, National Institute of 

Neurological Disorders and Stroke, National Institutes of Health, 

Bethesda, MD 20892. 

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

PMID: 8006664 [PubMed - indexed for MEDLINE] 

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 


Survival and Limited Spread of TSE Infectivity after Burial 


SUNDAY, JULY 16, 2017

*** Temporal patterns of chronic wasting disease prion excretion in three cervid species ***


Norwegian Food Safety Authority makes changes to measures to limit the spread of disease Skrantesjuke (CWD) in deer wildlife



*** Infectious agent of sheep scrapie may persist in the environment for at least 16 years *** 
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3 

 Using in vitro Prion replication for high sensitive detection of prions and prionlike proteins and for understanding mechanisms of transmission.
Claudio Soto
Mitchell Center for Alzheimer's diseases and related Brain disorders, Department of Neurology, University of Texas Medical School at Houston.
Prion and prion-like proteins are misfolded protein aggregates with the ability to selfpropagate to spread disease between cells, organs and in some cases across individuals. I n T r a n s m i s s i b l e s p o n g i f o r m encephalopathies (TSEs), prions are mostly composed by a misfolded form of the prion protein (PrPSc), which propagates by transmitting its misfolding to the normal prion protein (PrPC). The availability of a procedure to replicate prions in the laboratory may be important to study the mechanism of prion and prion-like spreading and to develop high sensitive detection of small quantities of misfolded proteins in biological fluids, tissues and environmental samples. Protein Misfolding Cyclic Amplification (PMCA) is a simple, fast and efficient methodology to mimic prion replication in the test tube. PMCA is a platform technology that may enable amplification of any prion-like misfolded protein aggregating through a seeding/nucleation process. In TSEs, PMCA is able to detect the equivalent of one single molecule of infectious PrPSc and propagate prions that maintain high infectivity, strain properties and species specificity. Using PMCA we have been able to detect PrPSc in blood and urine of experimentally infected animals and humans affected by vCJD with high sensitivity and specificity. Recently, we have expanded the principles of PMCA to amplify amyloid-beta (Aβ) and alphasynuclein (α-syn) aggregates implicated in Alzheimer's and Parkinson's diseases, respectively. Experiments are ongoing to study the utility of this technology to detect Aβ and α-syn aggregates in samples of CSF and blood from patients affected by these diseases.

***Recently, we have been using PMCA to study the role of environmental prion contamination on the horizontal spreading of TSEs. These experiments have focused on the study of the interaction of prions with plants and environmentally relevant surfaces. Our results show that plants (both leaves and roots) bind tightly to prions present in brain extracts and excreta (urine and feces) and retain even small quantities of PrPSc for long periods of time. Strikingly, ingestion of prioncontaminated leaves and roots produced disease with a 100% attack rate and an incubation period not substantially longer than feeding animals directly with scrapie brain homogenate. Furthermore, plants can uptake prions from contaminated soil and transport them to different parts of the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety of environmentally relevant surfaces, including stones, wood, metals, plastic, glass, cement, etc. Prion contaminated surfaces efficiently transmit prion disease when these materials were directly injected into the brain of animals and strikingly when the contaminated surfaces were just placed in the animal cage. These findings demonstrate that environmental materials can efficiently bind infectious prions and act as carriers of infectivity, suggesting that they may play an important role in the horizontal transmission of the disease.
Since its invention 13 years ago, PMCA has helped to answer fundamental questions of prion propagation and has broad applications in research areas including the food industry, blood bank safety and human and veterinary disease diagnosis. 
Friday, December 14, 2012

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


In the USA, under the Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law.

Animals considered at high risk for CWD include:

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

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

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

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

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

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


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

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

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


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


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


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


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

Back around 2000, 2001, or so, I was corresponding with officials abroad during the bse inquiry, passing info back and forth, and some officials from here inside USDA aphis FSIS et al. In fact helped me get into the USA 50 state emergency BSE conference call way back. That one was a doozy. But I always remember what “deep throat” I never knew who they were, but I never forgot;

Some unofficial information from a source on the inside looking out -


As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!

---end personal email---end...tss


*** Chronic Wasting Disease CWD TSE Prion (aka mad deer disease) Update USA December 14, 2017 ***

(zoonosis and environmental risk factors towards the bottom, after state by state reports)


Canada CFIA updating its national CWD TSE PRION efforts to eradicate disease farmed cervid NOT successful December 14, 2017


Bovine Spongiform Encephalopathy BSE TSE Prion (aka mad cow disease) Report December 14, 2017 2017






Creutzfeldt Jakob Disease CJD National Prion Disease Pathology Surveillance Center Cases Examined to December 14, 2017

Tuesday, December 12, 2017 

Neuropathology of iatrogenic Creutzfeldt–Jakob disease and immunoassay of French cadaver-sourced growth hormone batches suggest possible transmission of tauopathy and long incubation periods for the transmission of Abeta pathology

Illinois Creutzfeldt - Jakob Disease


Creutzfeldt-Jakob disease (CJD) belongs to the family of diseases known as transmissible spongiform encephalopathies (TSEs). TSEs are also known as prion-related diseases because they are thought to be caused by abnormal forms of a host protein, termed prion protein. There are three types of CJD in humans: sporadic or classic (85%), familial (15%), and acquired [iatrogenic and variant] (1%).1


CJD is the most common form of TSE in humans, with an estimated annual incidence of 1 case/1 million population worldwide. Persons ≥ 50 years old have a higher risk of CJD with an average annual rate of approximately 3.4 cases per million.1

CJD became reportable in Illinois effective March 3, 2008. From 2008 to 2015 the Chicago Department of Public Health (CDPH) received 75 CJD suspected case reports. Of these 75 reported cases, eight were confirmed and four were probable (Figure). As of 2014, only confirmed CJD cases are reportable to Illinois Department of Public Health. Confirmed cases have pathologic evidence of disease and probable cases have clinical, laboratory and radiographic features consistent with CJD. (

Of the 12 confirmed and probable cases, 7 (58%) cases were female. The age range was 51 -72 years, with a median age of 62 years. Regarding race/ethnicity, seven cases were white (58%), three cases (25%) were Hispanic, and one case each (8%) was black, and Asian/Pacific Islander. Based on pathology results reported from the National Prion Disease Pathology Surveillance Center of the eight confirmed cases, three were sporadic, two were familial, and three had prion protein present consistent with CJD, but type was undetermined.

Clinical Course

The incubation period for CJD ranges from years to decades.3

 Patients with sporadic CJD present with rapidly progressive dementia, visual or speech abnormalities or cerebellar dysfunction, including loss of muscle coordination and gait abnormalities. During the course of the disease most patients develop abnormal reflexes, spasticity, tremors, and rigidity. Patients may show behavioral changes including agitation, depression, or confusion.4 CJD is invariably fatal with a median illness duration of four months (mean of 7.6 months). Most CJD cases (85-90%) expire within 12 months of illness onset. There is no treatment for CJD.1

Infection-Control Guidelines

Investigations of potential CJD cases by CDPH have demonstrated a need for facilities to review policies and procedures pertaining to TSE. Published literature describes iatrogenic CJD exposures linked to use of cadaveric pituitary hormones, dura mater, corneal grafts and neurosurgical instruments.5

 Four of six cases linked to neurosurgical procedures from 1950s-1976 were related to contaminated neurosurgical instruments and two were related to implantable electrodes.5

 Conventional chemical and physical methods of decontamination are insufficient for reprocessing instruments potentially contaminated with TSEs. The World Health Organization (WHO) has developed TSE infection control guidelines that provide a framework to determine infectivity risk for patient tissues, secretions and excretions. Specific recommendations address patient care, occupational exposures, laboratory investigations, decontamination procedures, waste disposal, and precautions after death. No TSE patient should be denied admission to a health facility, kept in isolation, or deprived of an indicated procedure.7

In a review of management of neurosurgical instruments and patients exposed to CJD, over half of the procedures were likely performed as diagnostic work-up for the patients.3

 Infection preventionists should review infection control policies for intracranial diagnostic procedures. Providers evaluating patients with acute neurodegenerative illness of undetermined etiology should consider CJD in the differential diagnosis and plan ahead to use appropriate reprocessing measures for any instruments used during diagnostic procedures. Instruments should be kept moist and promptly cleaned and sterilized according to WHO guidance6 or quarantined until an alternative diagnosis is identified. Timely communication between physicians, the operating room staff, central sterilization and infection preventionists is critical to ensure appropriate instrument handling.3

 In addition, facilities should consider use of an instrument tracking procedure in order to trace potentially contaminated neurosurgical instruments linked to an identified case, should the need arise. Instruments from neurosurgical sets should not be mixed with those from general surgical sets.3


Creutzfeldt-Jakob Disease (CJD) is reportable within 7 days of identification. Clinicians and infection control practitioners should report suspect cases in Chicago by calling the CDPH Communicable Disease Program at (312) 746-5916. Laboratories should report patients who have a positive result on any laboratory test indicative of and specific for detecting CJD. Clinical specimens should be forwarded to the National Prion Disease Pathology Surveillance Center for disease confirmation.7,8 Chicago Department of Public Health 

Terry S. Singeltary Sr.


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