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

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

 

K.M. Olszowy x K.M. Olszowy Search for articles by this author Affiliations Department of Anthropology, SUNY Binghamton, Binghamton, NY, USA Laboratory of Biomedical Anthropology and Neuroscience, SUNY Binghamton, Binghamton, NY, USA , J. Lavelle x J. Lavelle Search for articles by this author Affiliations Department of Anthropology, SUNY Binghamton, Binghamton, NY, USA Laboratory of Biomedical Anthropology and Neuroscience, SUNY Binghamton, Binghamton, NY, USA , K. Rachfal x K. Rachfal Search for articles by this author Affiliations Department of Anthropology, SUNY Binghamton, Binghamton, NY, USA Laboratory of Biomedical Anthropology and Neuroscience, SUNY Binghamton, Binghamton, NY, USA , S. Hempstead x S. Hempstead Search for articles by this author Affiliations Department of Anthropology, SUNY Binghamton, Binghamton, NY, USA Laboratory of Biomedical Anthropology and Neuroscience, SUNY Binghamton, Binghamton, NY, USA , K. Drouin x K. Drouin Search for articles by this author Affiliations Department of Anthropology, SUNY Binghamton, Binghamton, NY, USA Laboratory of Biomedical Anthropology and Neuroscience, SUNY Binghamton, Binghamton, NY, USA Biospecimen Archive Facility, SUNY Binghamton, Binghamton, NY, USA , J.M. Darcy x J.M. Darcy Search for articles by this author Affiliations Department of Anthropology, SUNY Binghamton, Binghamton, NY, USA Laboratory of Biomedical Anthropology and Neuroscience, SUNY Binghamton, Binghamton, NY, USA II , C. Reiber x C. Reiber Search for articles by this author Affiliations Department of Anthropology, SUNY Binghamton, Binghamton, NY, USA , R.M. Garruto x R.M. Garruto Search for articles by this author Affiliations Department of Anthropology, SUNY Binghamton, Binghamton, NY, USA Laboratory of Biomedical Anthropology and Neuroscience, SUNY Binghamton, Binghamton, NY, USA Biospecimen Archive Facility, SUNY Binghamton, Binghamton, NY, USA Correspondence Corresponding author. Graduate Program in Biomedical Anthropology, State University of New York (SUNY) Binghamton, PO Box 6000, Binghamton, NY 13902-6000, USA. Tel.: +1 607 777 6562 (office); fax: +1 607 777 2477. email Received: October 22, 2013; Received in revised form: June 2, 2014; Accepted: June 11, 2014; Published Online: September 14, 2014 DOI: http://dx.doi.org/10.1016/j.puhe.2014.06.012 Abstract Full Text Images Images/Data References Related Articles To view the full text, please login as a subscribed user or purchase a subscription. Click here to view the full text on ScienceDirect.

 

Figures

 

Fig. 1

 

Spatial distribution of CWD in the United States and Canada, 2000 and 2013. The CWD map is overlaid on a demographic map of the US population. The insert map of New York State shows the Madison/Oneida County Containment Area where the outbreak of CWD occurred.7,8

 

Fig. 2

 

Percent of participants reporting hunting and eating venison from both within and outside the Oneida/Madison county containment area. Venison consumption is predicted by hunting across all years (p < 0.001).

 

Abstract

 

Objectives

 

It is currently unknown whether chronic wasting disease (CWD), a transmissible spongiform encephalopathy of cervids, is transmissible to humans. Reported on here are the behavioural risk factors and health conditions associated with a six-year follow-up of a known point-source exposure to a CWD infected deer in an Upstate New York community.

 

Study design

 

Longitudinal.

 

Methods

 

The Oneida County Chronic Wasting Disease Surveillance Project was launched in 2005 in response to a point-source exposure to a CWD infected deer at a March 2005 Sportsmen's feast in Upstate New York. Eighty-one exposed individuals participated in the 2005 baseline data collection, and were sent follow-up questionnaires following each deer hunting season between 2005 and 2011.

 

Results

 

Over a six year period, participants reported a reduction in overall venison consumption. Participants reported no significant changes in health conditions, although several conditions (vision loss, heart disease, type 2 diabetes, weight changes, hypertension, and arthritis), were significantly associated with age.

 

Conclusions

 

To this day, this incident remains the only known large-scale point-source exposure to a CWD infected deer. Prion diseases can incubate for multiple decades before the manifestation of clinical symptoms; thus, continued surveillance of this exposed study population represents a unique opportunity to assess the risk of CWD transmission to humans. This project is uniquely situated to provide the first epidemiological evidence of CWD transmission to humans, should it occur.

 

Keywords: Wasting disease, chronic, Prion disease, Public health surveillance, Community health

 


 

WITH AN INCUBATION PERIOD OF ANYWHERE FROM 30 TO 50 YEARS, ONLY TIME WILL TELL...TSS

 

-------- Original Message --------

Subject: No medical studies planned for CWD Feast in New York, 'a missed opportunity'

Date: Sun, 17 Apr 2005 19:08:28 –0500

From: "Terry S. Singeltary Sr." flounder@WT.NET

Reply-To: Bovine Spongiform Encephalopathy BSE-L@LISTS.UNI-KARLSRUHE.DE

To: BSE-L@LISTS.UNI-KARLSRUHE.DE

 

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

 

AP New York ------------------------------------------------------------------------ Health officials to wait and watch humans exposed to deer disease

 

By WILLIAM KATES Associated Press

 

Writer April 17, 2005, 11:22 AM EDT

 

SYRACUSE, N.Y. -- After 350 people at a sportsman's dinner ate venison from sick deer, a scientist says now is the time to launch a study to determine if the fatal chronic wasting disease could spread to humans who ingest infected meat. Chronic wasting disease _ CWD _ was detected earlier this month in two private captive deer herds in central New York's Oneida County, the first time it was found outside the Midwest or Rocky Mountains. Scientists say they're still learning about CWD and can't say for sure if it could be transmitted to humans, but state and local health officials say they have no plans to study the people who ate the meat last month. That's a missed opportunity, said an animal disease expert with the International Society for Infectious Diseases. "Currently, the disease and the speculation surrounding the disease far out reaches any real science about the disease," said Tam Garland, a professor of veterinary medicine at Texas A&M University. "New York has the opportunity to do an epidemiological study ... Seldom are we presented with such an opportunity to study humans," Garland said. One of the infected deer from Oneida County was served at an annual banquet on March 13 at the Verona Fire Department. The Oneida County Health Department made a list of those who attended the dinner and sent them letters to give them accurate information about CWD and reassure them it does not pose a health risk to humans, said Ken Fanelli, a department spokesman. About 70 people called the county health department after getting the letter, Fanelli said. "No one was particularly concerned or fearful," he said. "Most just wanted more information." The venison was served in steak, chili, stew, sausage and meat patties, health officials said. No organs or bone product from the deer was served, the parts scientists test when looking for signs of CWD. State health department spokesman Robert Kenny said although no medical studies are planned, the list prepared by the county health department will allow officials to quickly locate and contact the people if the need arises. In 2004, scientists at the federal Centers for Disease Control in Atlanta issued a study on chronic wasting disease that stressed the absence of any evidence linking CWD to humans. Authors, though, acknowledged the study was limited in geography and sample size and so it couldn't draw a conclusion about the risk to humans. They recommended more study. Dr. Ermias Belay was the report's principal author but he said New York and Oneida County officials are following the proper course by not launching a study. "There's really nothing to monitor presently. No one's sick," Belay said, noting the disease's incubation period in deer and elk is measured in years. "This was one carcass, one meal. It was an animal without symptoms. If it becomes an issue, if other studies suggest there is a risk, we have a list to go back to." Belay noted that the CDC also is involved in long-term human medical studies in Colorado and Wyoming, where the disease has been endemic for more than two decades. He said they don't know definitively if people there have eaten meat from infected deer. "If people are going to get CWD, these people would be among the first because of their earlier and longer exposure," Belay said. In New York, authorities have so far confirmed five infected captive deer. The state Department of Environmental Conservation is testing the wild deer population in Oneida and Hamilton counties to determine if the disease has spread beyond the two domestic herds. They are killing about 450 wild deer in central and northern New York to test for the disease. One of the key questions to answer is how the disease leapfrogged from Illinois _ which had been the easternmost state to detect CWD _ to New York. DEC spokesman Michael Fraser said it did not appear to be the result of natural animal migration. "The leading theory is that it was somehow imported. But how? That's what we need to find out," Fraser said. Although it appears the disease is passed either through direct animal-to-animal contact or indirect exposure, including feed and contaminated water sources, scientists admit they don't fully understand how it's transmitted. ___

 

On the Net: Centers for Disease

 


 

International Society for Infectious Diseases: www.isid.org

 


 

Greetings,

 

> That's a missed opportunity, said an animal disease expert with the

 

> International Society for Infectious Diseases.

 

> > "Currently, the disease and the speculation surrounding the disease '

 

> far out reaches any real science about the disease," said Tam Garland,

 

> a professor of veterinary medicine at Texas A&M University.

 

> > "New York has the opportunity to do an epidemiological study ...

 

> Seldom are we presented with such an opportunity to study humans," > Garland said.

 

excellent point! sadly Tam, they don't want to know. i cannot think of any other reason for not doing a study. but it's like CJD and making it reportable Nationally, in every state, and most importantly, of ALL AGES with a CJD questionnaire asking questions pertaining to route and source of agent. they just dont want to know... kindest regards, terry

 


 

HERE IS ANOTHER CWD FEAST ;

 

From: TSS (216-119-162-44.ipset44.wt.net)

Subject: Re: CWD--THE FEAST--Investigators find no common source in hunters' deaths $$$ (not cjd, now it's not picks, what the hell is it$$$)

Date: November 22, 2002 at 1:34 pm PST

 

In Reply to: Re: CWD--THE FEAST--Investigators find no common source in hunters' deaths $$$ (or did they???) posted by TSS on November 22, 2002 at 12:14 pm:

 

not picks, not cjd ???

 

CWD and neurological disease cluster link investigated

 

Mary Quirk

 

No link to chronic wasting disease (CWD) is suggested from the re-examination of one case in a cluster of degenerative neurological diseases involving three men who participated in wild game feasts, according to public health officials.

 

Figure. CWD incidence: Red--wild deer and elk. Blue--farmed elk. Purple--farmed deer. Courtesy of US Department of Agriculture

 

Of one group of 75 people who regularly attended these annual meals in the US midwest since the late 1970s, 43 have been contacted and interviewed. "We are not aware of any other related health events in this group", Jeffrey Davis (Wisconsin Division of Public Health, Madison, WI, USA) told TLID.

 

Brain autopsy samples from the three men were sent to the National Prion Disease Pathology Surveillance Center (NPDPSC) in Cleveland, OH, USA. The centre examines an estimated 50-60% of Creutzfeldt-Jakob disease (CJD) cases in the USA, according to Shu G Chen, director of their Prion Protein Analysis Laboratory. Archived tissues may be re-examined to confirm prion disease, and to conduct up-to-date immunohistochemistry and prion protein genetic testing.

 

Until now findings have been reported on one case. NPDPSC pathologists have concluded that, although they did not concur with the previous diagnosis of Pick's disease, a form of dementia, the autopsy tissue from 1993 showed no evidence of a prion-related disorder. Information is still pending on the other two, both suspected cases of CJD; one man died in 1993 and the other in 1999.

 

Davis has participated in CWD information meetings for the public in Wisconsin, where he focuses on current knowledge about CWD, recommending that residents heed WHO precautions, and directs hunters to advice on the internet (http://datcp.state.wi.us/ah/agriculture/animals/disease/chronic/pdf/venison_safety_2side.pdf ).

 

At the feasts, people shared game that they had harvested throughout the USA. "We are not aware of any meat having come from known CWD-endemic areas", says Davis.

 

Previously, researchers have reviewed CJD cases from CWD-endemic areas to look for differences in clinical symptoms, pathology, or the characteristics of the prion protein. "So far, we haven't found a link", Chen told TLID. "For us to be able to tell whether these cases would have any relationship to CWD, they need to have distinguishable characteristics."

 

John Pape (Disease Control and Environmental Epidemiology Division, Colorado Department of Public Health and Environment, Denver, CO, USA) told TLID that he and colleagues are taking a broad- brush look at deaths due to neurological diseases in Colorado. "We are examining the death certificates of all Colorado residents for a 32-year period." Pape hopes to get sufficient numbers to compare rates of death from various neurological disorders, including CJD, in CWD-endemic and non-endemic areas of Colorado.

 


 

greetings list members,

 

=================== "Until now findings have been reported on one case. NPDPSC pathologists have concluded that, although they

 

__did not concur with the previous diagnosis of Pick's disease__,

 

a form of dementia, the autopsy tissue from 1993 showed no evidence of a prion-related disorder."

 

=========================

 

if CDC/NPDPSC says it's not CJD, NPDPSC/CDC says it's not Picks, then who do we call, ghostbusters???

 

what the heck is it then?

 

"For us to be able to tell whether these cases would have any relationship to CWD, they need to have distinguishable characteristics."

 

what if sCJD does _not_ have distinguishable characteristics, do we sit on our butts and just continue to say everything is o.k. it's all sporadic/spontaneous CJDs, even though we are getting more variants? plus, will these distinguishable characteristics look the same after lying around for 10 years? what if these distinguishable characteristics that they use to distinguish between various TSE have nothing to do with actually distinguishing anything other than a single TSE, but the titre of infectivity, the route, and the source is what plays the role in the different distinguishable characterisics they speak of?

 

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

 


 


 

and i would _assume_ (sorry Roland;-), that this would apply with other TSEs as well...

 

oh Lord, thank you for the weekend, i could not stand anymore of this BSeee going around...

 

still disgusted in Bacliff, Texas USA

 

Terry S. Singeltary Sr. wrote:

 

Date: Fri, 22 Nov 2002 14:14:12 –0600

Reply-To: Bovine Spongiform Encephalopathy

Sender: Bovine Spongiform Encephalopathy

From: "Terry S. Singeltary Sr."

Subject: Re: Investigators find no common source in hunters' deaths $$$ (or did they???)

 

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

 

greetings list members,

 

"jimmeny cricket" !!!

 

how about a statement from Gambetti et al, instead of a bunch of alleged second hand statements.

 

does state epidemiologist or even mayo really have access to a specialized panel of _proven_ antibodies and know how with prion ihc??? probably not.

 

only gambetti and prusiner have the necessary reference collections if i am not mistaken $$$

 

who validated it, false positive, false negatives?

 

what's going on here???

 

just in time too to avert a public panic and at the opening weekend of deer hunting season at that, how convenient and excellent timing$$$

 

unpublished, unpeer-reviewed = unreliable

 

and just more BSeee.

 

and what did deep throat tell me many moons ago;

 

..."I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at....."

 

snip...

 

Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!

 

And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...

 

i sure would be interested to know more about this new test they are using now, and why it has not been used in the past, or were they just waiting for the opening weekend of deer season? so many things to ponder$

 

all these new test (cjd now, CWD, Scrapie), but absolutely nothing on any sort of rapid testing TSE 1 M cattle annually in the USA$$$ (more convienience)

 

i have serious doubts about the _new_ CJD Foundation and their intentions. you know the one, the one that _refuses_ to make up a CJD Quesionnaire asking questions to seek answers as to the route and source of sporadic CJDs in the USA, the same one funded by the CDC??? i have one email from a family member of a very young CJD victim in the USA (sporadic of course) that wrote me questioning the fact that the _new_ CJD Foundation/CDC questionnaire was useless. the family wrote telling them more info on there own, cause they said there were not many questions being asked. see comments snip;

 

I also sent xxxx a follow-up email with more info because I didn't think the questionnaire asked enough, or perhaps I just needed to write about it. I sent it on xxxx. See next...

 

snip...

 

also;

 

i am seeing more and more confirmed sporadic CJDs, that are being negated by the _new_ CDC funded CJD Foundation? see ref;

 

Subject: HUMAN TSEs WITH INSOMNIA & _new_ CJD Foundation Questionnaire ??? Date: Sun, 17 Nov 2002 16:57:58 -0600 From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy To: BSE-L

 

(this was the one where they changed the diagnosis of CJD to just dementia, from after death analysis of only a small diamond shape piece from back of brain. the victim could not sleep for 3 to 4 days at a time etc. the family told me that the original diagnosis was made from MRI and spinal, before being changed by CJD Foundation/CDC to only dementia.)

 

now i am back to here;

 

Thursday's report contradicts the findings of a Mayo Clinic doctor, who in a May 17, 1993, letter to Waterhouse's family said a postmortem exam of Waterhouse confirmed that he died of Creutzfeldt-Jakob disease.

 

In fact, when reached at home on Thursday night, Joseph Parisi, a Mayo pathologist, said the new tests on Waterhouse were "inconclusive" and could not confirm that he died of the disease.

 

That differs from a statement issued Thursday by Davis' office, which says that Waterhouse did not have Creutzfeldt-Jakob disease. Davis also said the latest analysis was confirmed by pathologists at the National Prion Disease Pathology Surveillance Center in Cleveland.

 

The center receives federal funding to monitor prion diseases,

 

snip...

 

what's going on here??? heck, just more of my conspiracy theories i suppose...

 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease T. S. Singeltary, Sr; D. E. Kraemer; R. V. Gibbons, R. C. Holman, E. D. Belay, L. B. Schonberger

 


 

CJD WATCH

 


 

CJD Watch message board

 


 

TSS

 

 human risk factors from cwd ;

 

>>> According to the Centers for Disease Control and Prevention (CDC) and the World Health Organization, there is no evidence CWD can be transmitted to humans, but it is still wise to not consume any part of an animal that shows signs of CWD.

 

NOW, what is the latest on human risk factors to CWD strains ???

 

*** PPo3-7: Prion Transmission from Cervids to Humans is Strain-dependent

 

*** Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP,

 

*** indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains.

 

PPo2-27:

 

Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions

 

*** Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids.

 

PPo2-7:

 

Biochemical and Biophysical Characterization of Different CWD Isolates

 

*** The data presented here substantiate and expand previous reports on the existence of different CWD strains.

 


 

Envt.07:

 

Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease

 

***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.

 


 

>>>CHRONIC WASTING DISEASE , THERE WAS NO ABSOLUTE BARRIER TO CONVERSION OF THE HUMAN PRION PROTEIN<<<

 

*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***

 

Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014

 

Wednesday, January 01, 2014

 

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.

 


 


 

PRION2013 CONGRESSIONAL ABSTRACTS CWD

 

Sunday, August 25, 2013

 

HD.13: CWD infection in the spleen of humanized transgenic mice

 

***These results indicate that the CWD prion may have the potential to infect human peripheral lymphoid tissues.

 

Oral.15: Molecular barriers to zoonotic prion transmission: Comparison of the ability of sheep, cattle and deer prion disease isolates to convert normal human prion protein to its pathological isoform in a cell-free system ***However, they also show that there is no absolute barrier to conversion of human prion protein in the case of chronic wasting disease.

 

PRION2013 CONGRESSIONAL ABSTRACTS CWD

 

Sunday, August 25, 2013

 

***Chronic Wasting Disease CWD risk factors, humans, domestic cats, blood, and mother to offspring transmission

 


 

there is in fact evidence that the potential for cwd transmission to humans can NOT be ruled out.

 

I thought your readers and hunters and those that consume the venison, should have all the scientific facts, personally, I don’t care what you eat, but if it effects me and my family down the road, it should then concern everyone, and the potential of iatrogenic transmission of the TSE prion is real i.e. ‘friendly fire’, medical, surgical, dental, blood, tissue, and or products there from...like deer antler velvet and TSE prions and nutritional supplements there from, all a potential risk factor that should not be ignored or silenced. ...

 

the prion gods at the cdc state that there is ;

 

''no strong evidence''

 

but let's see exactly what the authors of this cwd to human at the cdc state ;

 

now, let’s see what the authors said about this casual link, personal communications years ago. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ????

 

“Our conclusion stating that we found no strong evidence of CWD transmission to humans”

 

From: TSS (216-119-163-189.ipset45.wt.net)

 

Subject: CWD aka MAD DEER/ELK TO HUMANS ???

 

Date: September 30, 2002 at 7:06 am PST

 

From: "Belay, Ermias"

 

To:

 

Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"

 

Sent: Monday, September 30, 2002 9:22 AM

 

Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

 

Dear Sir/Madam,

 

In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.

 

That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.

 

Ermias Belay, M.D. Centers for Disease Control and Prevention

 

-----Original Message-----

 

From:

 

Sent: Sunday, September 29, 2002 10:15 AM

 

To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV

 

Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

 

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

 

Thursday, April 03, 2008

 

A prion disease of cervids: Chronic wasting disease

 

2008 1: Vet Res. 2008 Apr 3;39(4):41

 

A prion disease of cervids: Chronic wasting disease

 

Sigurdson CJ.

 

snip...

 

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

 

snip...

 

full text ;

 


 


 


 

***********CJD REPORT 1994 increased risk for consumption of veal and venison and lamb***********

 

CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994

 

Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss)

 

These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...

 

Table 9 presents the results of an analysis of these data.

 

There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).

 

Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.

 

There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).

 

The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).

 

There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).

 

The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).

 

snip...

 

It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).

 

snip...

 

In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...

 

snip...

 

In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)

 

snip...see full report ;

 


 

Thursday, October 10, 2013

 

*************CJD REPORT 1994 increased risk for consumption of veal and venison and lamb**************

 


 

CJD9/10022

 

October 1994

 

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ

 

Dear Mr Elmhirst,

 

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

 

Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

 

The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

 

The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.

 

The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.

 

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

 


 

*** our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions. ***

 


 

snip...see full text ;

 


 

Thursday, January 2, 2014

 

*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***

 


 

*** We hypothesize that both BSE prions and CWD prions passaged through felines will seed human recPrP more efficiently than BSE or CWD from the original hosts, evidence that the new host will dampen the species barrier between humans and BSE or CWD. The new host effect is particularly relevant as we investigate potential means of trans-species transmission of prion disease.

 


 

Monday, August 8, 2011

 

*** Susceptibility of Domestic Cats to CWD Infection ***

 

Oral.29: Susceptibility of Domestic Cats to CWD Infection

 

Amy Nalls, Nicholas J. Haley, Jeanette Hayes-Klug, Kelly Anderson, Davis M. Seelig, Dan S. Bucy, Susan L. Kraft, Edward A. Hoover and Candace K. Mathiason†

 

Colorado State University; Fort Collins, CO USA†Presenting author; Email: ckm@lamar.colostate.edu

 

Domestic and non-domestic cats have been shown to be susceptible to one prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted through consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free ranging felids scavenge cervid carcasses, including those in CWD affected areas, we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between 40–43 months following IC inoculation, two cats developed mild but progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on the brain of one of these animals (vs. two age-matched controls) performed just before euthanasia revealed increased ventricular system volume, more prominent sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere and in cortical grey distributed through the brain, likely representing inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles were demonstrated in the brains of both animals by immunodetection assays. No clinical signs of TSE have been detected in the remaining primary passage cats after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5) of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC inoculated cats are demonstrating abnormal behavior including increasing aggressiveness, pacing, and hyper responsiveness.

 

*** Two of these cats have developed rear limb ataxia. Although the limited data from this ongoing study must be considered preliminary, they raise the potential for cervid-to-feline transmission in nature.

 


 


 

AD.63:

 

Susceptibility of domestic cats to chronic wasting disease

 

Amy V.Nalls,1 Candace Mathiason,1 Davis Seelig,2 Susan Kraft,1 Kevin Carnes,1 Kelly Anderson,1 Jeanette Hayes-Klug1 and Edward A. Hoover1 1Colorado State University; Fort Collins, CO USA; 2University of Minnesota; Saint Paul, MN USA

 

Domestic and nondomestic cats have been shown to be susceptible to feline spongiform encephalopathy (FSE), almost certainly caused by consumption of bovine spongiform encephalopathy (BSE)-contaminated meat. Because domestic and free-ranging nondomestic felids scavenge cervid carcasses, including those in areas affected by chronic wasting disease (CWD), we evaluated the susceptibility of the domestic cat (Felis catus) to CWD infection experimentally. Cohorts of 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD-infected deer brain. At 40 and 42 mo post-inoculation, two IC-inoculated cats developed signs consistent with prion disease, including a stilted gait, weight loss, anorexia, polydipsia, patterned motor behaviors, head and tail tremors, and ataxia, and progressed to terminal disease within 5 mo. Brains from these two cats were pooled and inoculated into cohorts of cats by IC, PO, and intraperitoneal and subcutaneous (IP/SC) routes. Upon subpassage, feline-adapted CWD (FelCWD) was transmitted to all IC-inoculated cats with a decreased incubation period of 23 to 27 mo. FelCWD was detected in the brains of all the symptomatic cats by western blotting and immunohistochemistry and abnormalities were seen in magnetic resonance imaging, including multifocal T2 fluid attenuated inversion recovery (FLAIR) signal hyper-intensities, ventricular size increases, prominent sulci, and white matter tract cavitation. Currently, 3 of 4 IP/SQ and 2 of 4 PO inoculared cats have developed abnormal behavior patterns consistent with the early stage of feline CWD.

 

*** These results demonstrate that CWD can be transmitted and adapted to the domestic cat, thus raising the issue of potential cervid-to- feline transmission in nature.

 


 

www.landesbioscience.com

 

PO-081: Chronic wasting disease in the cat— Similarities to feline spongiform encephalopathy (FSE)

 


 


 

FELINE SPONGIFORM ENCEPHALOPATHY FSE

 


 


 

PRION CONFERENCE 2014 HELD IN ITALY RECENTLY CWD BSE TSE UPDATE

 

> First transmission of CWD to transgenic mice over-expressing bovine prion protein gene (TgSB3985)

 

PRION 2014 - PRIONS: EPIGENETICS and NEURODEGENERATIVE DISEASES – Shaping up the future of prion research

 

Animal TSE Workshop 10.40 – 11.05 Talk Dr. L. Cervenakova First transmission of CWD to transgenic mice over-expressing bovine prion protein gene (TgSB3985)

 


 

P.126: Successful transmission of chronic wasting disease (CWD) into mice over-expressing bovine prion protein (TgSB3985)

 

Larisa Cervenakova,1 Christina J Sigurdson,2 Pedro Piccardo,3 Oksana Yakovleva,1 Irina Vasilyeva,1 Jorge de Castro,1 Paula Saá,1 and Anton Cervenak1 1American Red Cross, Holland Laboratory; Rockville, MD USA; 2University of California; San Diego, CA USA; 3Lab TSE/OBRR /CBER/FDA; Rockville, MD USA

 

Keywords: chronic wasting disease, transmission, transgenic mouse, bovine prion protein

 

Background. CWD is a disease affecting wild and farmraised cervids in North America. Epidemiological studies provide no evidence of CWD transmission to humans. Multiple attempts have failed to infect transgenic mice expressing human PRNP gene with CWD. The extremely low efficiency of PrPCWD to convert normal human PrPC in vitro provides additional evidence that transmission of CWD to humans cannot be easily achieved. However, a concern about the risk of CWD transmission to humans still exists. This study aimed to establish and characterize an experimental model of CWD in TgSB3985 mice with the following attempt of transmission to TgHu mice.

 

Materials and Methods. TgSB3985 mice and wild-type FVB/ NCrl mice were intracranially injected with 1% brain homogenate from a CWD-infected Tga20 mouse (CWD/Tga20). TgSB3985 and TgRM (over-expressing human PrP) were similarly injected with 5% brain homogenates from CWD-infected white-tailed deer (CWD/WTD) or elk (CWD/Elk). Animals were observed for clinical signs of neurological disease and were euthanized when moribund. Brains and spleens were removed from all mice for PrPCWD detection by Western blotting (WB). A histological analysis of brains from selected animals was performed: brains were scored for the severity of spongiform change, astrogliosis, and PrPCWD deposition in ten brain regions.

 

Results. Clinical presentation was consistent with TSE. More than 90% of TgSB3985 and wild-type mice infected with CWD/Tga20, tested positive for PrPres in the brain but only mice in the latter group carried PrPCWD in their spleens. We found evidence for co-existence or divergence of two CWD/ Tga20 strains based on biochemical and histological profiles. In TgSB3985 mice infected with CWD-elk or CWD-WTD, no animals tested positive for PrPCWD in the brain or in the spleen by WB. However, on neuropathological examination we found presence of amyloid plaques that stained positive for PrPCWD in three CWD/WTD- and two CWD/Elk-infected TgSB3985 mice. The neuropathologic profiles in CWD/WTD- and CWD/Elkinfected mice were similar but unique as compared to profiles of BSE, BSE-H or CWD/Tg20 agents propagated in TgSB3985 mice. None of CWD-infected TgRM mice tested positive for PrPCWD by WB or by immunohistochemical detection.

 

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.

 

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.

 

P.28: Modeling prion species barriers and the new host effect using RT-QuIC

 

Kristen A Davenport, Davin M Henderson, Candace K Mathiason, and Edward A Hoover Prion Research Center; Colorado State University; Fort Collins, CO USA

 

The propensity for trans-species prion transmission is related to the structural characteristics of the enciphering and heterologous PrP, but the exact mechanism remains mostly mysterious.

 

Studies of the effects of primary or tertiary prion protein www.landesbioscience.com Prion 37 structures on trans-species prion transmission have relied upon animal bioassays, making the influence of prion protein structure vs. host co-factors (e.g. cellular constituents, trafficking, and innate immune interactions) difficult to dissect.

 

As an alternative strategy, we are using real-time quaking-induced conversion (RT-QuIC) to investigate the propensity for and the kinetics of trans-species prion conversion. RT-QuIC has the advantage of providing more defined conditions of seeded conversion to study the specific role of native PrP:PrPRES interactions as a component of the species barrier.

 

We are comparing chronic wasting disease (CWD) and bovine spongiform encephalopathy (BSE) prions by seeding each prion into its native host recPrP (full-length bovine recPrP, or white tail deer recPrP) vs. into the heterologous species.

 

Upon establishing the characteristics of intra-species and inter-species prion seeding for CWD and BSE prions, we will evaluate the seeding kinetics and cross-species seeding efficiencies of BSE and CWD passaged into a common new host—feline—shown to be a permissive host for both CWD and BSE.

 

*** We hypothesize that both BSE prions and CWD prions passaged through felines will seed human recPrP more efficiently than BSE or CWD from the original hosts, evidence that the new host will dampen the species barrier between humans and BSE or CWD. The new host effect is particularly relevant as we investigate potential means of trans-species transmission of prion disease.

 


 

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

 

***Singeltary submission

 

Docket No. 00-108-10 Chronic Wasting Disease Herd Certification Program and Interstate Movement of Farmed or Captive Deer, Elk, and Moose; Program Standards

 

>>>The CWD herd certification program is a voluntary, cooperative program that establishes minimum requirements for the interstate movement of farmed or captive cervids, provisions for participating States to administer Approved State CWD Herd Certification Programs, and provisions for participating herds to become certified as having a low risk of being infected with CWD<<<

 

Greetings USDA/APHIS et al,

 

I kindly would like to comment on Docket No. 00-108-10 Chronic Wasting Disease Herd Certification Program and Interstate Movement of Farmed or Captive Deer, Elk, and Moose; Program Standards.

 

I believe, and in my opinion, and this has been proven by scientific facts, that without a validated and certified test for chronic wasting disease cwd, that is 100% sensitive, and in use, any voluntary effort will be futile. the voluntary ban on mad cow feed and SRMs have failed terribly, the bse mad cow surveillance program has failed terribly, as well as the testing for bse tse prion in cattle, this too has failed terrible. all this has been proven time and time again via OIG reports and GOA reports.

 

I believe that until this happens, 100% cwd testing with validated test, ALL MOVEMENT OF CERVIDS BETWEEN STATES MUST BE BANNED, AND THE BORDERS CLOSED TO INTERSTATE MOVEMENT OF CERVIDS. there is simply to much at risk.

 

In my opinion, and the opinions of many scientists and DNR officials, that these so called game farms are the cause of the spreading of chronic wasting disease cwd through much negligence. the game farms in my opinion are not the only cause, but a big factor. I kindly wish to submit the following to show what these factors are, and why interstate movement of cervids must be banned. ...

 

snip...see full text and PDF ATTACHMENT HERE ;

 


 


 

Sunday, June 23, 2013

 

National Animal Health Laboratory Network Reorganization Concept Paper (Document ID APHIS-2012-0105-0001)

 

***Terry S. Singeltary Sr. submission

 


 

Friday, November 22, 2013

 

Wasting disease is threat to the entire UK deer population CWD TSE PRION disease in cervids

 

***SINGELTARY SUBMISSION

 

The Scottish Parliament’s Rural Affairs, Climate Change and Environment Committee has been looking into deer management, as you can see from the following press release,

 

***and your email has been forwarded to the committee for information:

 


 


 

Friday, November 22, 2013

 

Wasting disease is threat to the entire UK deer population

 


 

Sunday, July 21, 2013

 

Welsh Government and Food Standards Agency Wales Joint Public Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013

 

*** Singeltary Submission WG18417

 


 

Saturday, October 18, 2014

 

Chronic wasting disease threatens Canadian agriculture, Alberta MLA says

 


 

Thursday, October 23, 2014

 

FIRST CASE OF CHRONIC WASTING DISEASE CONFIRMED IN OHIO ON PRIVATE PRESERVE

 


 

Tuesday, October 21, 2014

 

Pennsylvania Department of Agriculture Tenth Pennsylvania Captive Deer Tests Positive for Chronic Wasting Disease CWD TSE PRION DISEASE

 


 

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

 


 

Thursday, July 03, 2014

 

*** How Chronic Wasting Disease is affecting deer population and what’s the risk to humans and pets?

 


 

Tuesday, July 01, 2014

 

*** CHRONIC WASTING DISEASE CWD TSE PRION DISEASE, GAME FARMS, AND POTENTIAL RISK FACTORS THERE FROM

 


 

Saturday, October 25, 2014

 

118th USAHA Annual Meeting CWD and Captive Cerivds

 


 

*** In some cases, the incubation period may be as long as 50 years

 


 

 At a hearing in Parliament last Wednesday, the Science and Technology Committee was told that vCJD continued to pose a “significant” risk to UK public health and that more than one in every 2000 people could be silent carriers of the disease. *** vCJD can have an incubation period of over 30 years.

 

Monday, February 03, 2014

 

CREUTZFELDT-JAKOB DISEASE T.S.E. PRION U.K. UPDATE As at 3rd February 2014

 


 

 *** 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.

 


 

cwd exposure, and iatrogenic CJD, what if ???

 

*** our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions. ***

 


 

snip...see full text ;

 


 

Thursday, January 2, 2014

 

*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***

 


 

*** We hypothesize that both BSE prions and CWD prions passaged through felines will seed human recPrP more efficiently than BSE or CWD from the original hosts, evidence that the new host will dampen the species barrier between humans and BSE or CWD. The new host effect is particularly relevant as we investigate potential means of trans-species transmission of prion disease.

 


 

Tuesday, November 04, 2014

 

Towards an Age-Dependent Transmission Model of Acquired and Sporadic Creutzfeldt-Jakob Disease

 


 

 

 

Terry S. Singeltary Sr.

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