Wednesday, July 07, 2021

Wyoming Upper Powder River Mule Deer Initiative Research Project raising red flags CWD TSE PRION

Wyoming Upper Powder River Mule Deer Initiative Research Project raising red flags CWD TSE PRION 

Mule deer study raises red flags

Elsa Freise Buffalo Bulletin Via Wyoming News Exchange Jul 3, 2021 Updated Jul 3, 2021

Elsa Freise Buffalo Bulletin Via Wyoming News Exchange

BUFFALO — There are still six months remaining in the three-year study to better understand why the population of the Upper Powder River mule deer herd is in decline, but already biologists have identified concerning trends.

Statewide, mule deer populations have been on the decline. Biologists have identified the Upper Powder River herd, which ranges in Hunt Areas 30, 32, 33, 163 and 169 south and west of Buffalo, as one of the high concerns.

“We just have way less deer than we used to,” said Cheyenne Stewart, the Sheridan Region wildlife coordinator for the Wyoming Game and Fish Department. “We were trying to look at what we are missing that could explain why the population isn’t rebounding.”

The Upper Powder River Mule Deer Initiative is also looking at history and strategies. Stewart said the initiative is pursuing answers to such questions as, “Are there differences in population metrics like survival and fawn recruitment for deer that migrate versus don’t migrate?” and “What is the relation to agricultural areas versus native habitat?

Two years deep into the study, Stewart has identified some red flags.

“Overall poor body condition. When you compare these deer to the famous Wyoming-range deer that migrate record miles, our deer coming into winter (pre-winter) are comparable to their deer after they have been starving, at the end of winter (post-winter),” Stewart said.

The energy required to lactate can contribute to poor body condition, but Stewart said that not enough does are lactating in December to explain the number of deer who enter winter in poor body condition. Stewart said there is some concern that low lactation rates among the herd’s does could mean that fawns are at higher risk for winter mortality because of the lack of addition al nutrition.

Stewart has also identified high mortality rates primary causes of deaths including chronic wasting disease and mountain lion mortality and a high CWD prevalence.

“Even though it’s a small sample size to make that calculation, (CWD prevalence) is higher than we would expect. Based on the data we have now, we are sitting at the mid-teens (15% to 17%) for prevalence in harvested adult bucks. But this time next year, that can be changed a little bit,” Stewart said.

What’s interesting about this project is that the doe prevalence for CWD is around 20%, higher than the adult buck prevalence an unusual occurrence. Game and Fish is curious to see if the adult buck deer prevalence increases, will the doe prevalence decrease, Stewart said.

Game and Fish has implemented several strategies aimed at boosting herd population: generating liberal licenses for animals that prey on deer, reducing doe harvest, treating the habitat to become more resilient to climate change to ensure that important mule deer habitats persist long-term, monitoring for CWD and other diseases and monitoring fawn survival (which has not been alarmingly low). Yet the trends have been consistent.

“Nothing can really explain what was really going on,” Stewart said.

In phase one of the study, each deer selected for the three-year study was fitted with a GPS neck collar and various body measurements were taken. There are 70 running collars. If a deer dies over the course of the year, a new deer will be collared. The study has collared around 110 deer. Blood samples

were collected and will be analyzed for genetics. In addition, samples were collected to test for parasites, and a small sample of rectal tissue was collected to test for CWD. An ultrasound was also performed to assess body condition, Stewart said.

Every December, biologists catch the collared deer to take

the same body measurements. The Upper Powder River Mule Deer Initiative will end in December, with a final capture, the removal of all collars, recording measurements and additional CWD work, Stewart said.

This story is supported by a grant through Wyoming EPSCoR and the National Science Foundation. 



''Stewart has also identified high mortality rates primary causes of deaths including chronic wasting disease and mountain lion mortality and a high CWD prevalence.''

“Even though it’s a small sample size to make that calculation, (CWD prevalence) is higher than we would expect. Based on the data we have now, we are sitting at the mid-teens (15% to 17%) for prevalence in harvested adult bucks. But this time next year, that can be changed a little bit,” Stewart said.''

''What’s interesting about this project is that the doe prevalence for CWD is around 20%, higher than the adult buck prevalence an unusual occurrence. Game and Fish is curious to see if the adult buck deer prevalence increases, will the doe prevalence decrease, Stewart said.''

SEE ALSO; 

CWD prevalence in North Bighorns elk herd unit

April 07, 2021

SHERIDAN -

In 2019, the Game and Fish Department’s chronic wasting disease surveillance program shifted from monitoring distribution and spread of the disease to concentrated focus on selected deer and elk herds in each administrative region of the state each year. Efforts are made by regional personnel to collect a minimum of 200 tissue samples from harvested animals in each selected herd. This minimum sample size produces a reliable estimate of prevalence, rather than simply detecting presence of the disease in an area.

The North Bighorns Elk herd, consisting of elk hunt areas 35 through 40, was originally scheduled for priority CWD sampling in 2021. However, enough hunter-harvested samples were collected during the 2018 to 2020 hunting seasons to obtain an adequate sample size (n=206).

Test results identified seven positive elk in two of the hunt areas, Areas 35 and 37, for a prevalence rate estimate of 3.4 percent. Both elk hunt areas overlap deer hunt areas with documented CWD in mule deer and white-tailed deer. Distribution of sampling was not uniform between hunt areas, with Hunt Area 37 accounting for 52 percent of the sampling effort and only five samples collected from Hunt Area 39.

“We plan to prioritize this herd for sampling again in 2027,” said Sheridan Region Wildlife Biologist Tim Thomas. “At that time, we will implement protocols to improve equitable sampling across all hunt areas.”

No CWD management actions have been implemented for this herd. Click here to learn more about CWD and read the department’s CWD management plan. - WGFD -


Managing Deer for Tomorrow: Upper Powder River Mule Deer Initiative Research Project

In 2014, during WGFD Mule Deer Initiative public meetings, the public voiced concerns about the population of the Upper Powder River mule deer herd. The population has been below the objective of 18,000 animals since the early 2000s.

Game and Fish is currently implementing several management strategies in response. These include nearly complete elimination of doe/fawn licenses, very conservative general license deer harvest, the liberalization of mountain lion, black bear, white-tailed deer and elk seasons and the initiation of habitat improvement projects. In addition, research has begun to assess mule deer survival, nutritional status, seasonal movement patterns, fawn recruitment and habitat use patterns.

Phase 1 Captured 70 adult doe mule deer in December 2018. Collected biological samples & affixed GPS radio-collars. Radio-collars will record deer locations every 2 hours for 3 years.

Phase 2 Re-capture radio-collared deer to measure body condition annually (pending additional funding).

Phase 3 Data analysis will begin in 2022, after all GPS collar location data has been collected. We plan to assess: 1) the main causes & rates of adult doe mortality, 2) the relationship between annual doe nutrition and population dynamics and habitat use, 3) seasonal travel patterns and potential migration routes, 4) fawning locations, habitats, and recruitment, and 5) habitat use patterns.

Phase 4 Use the new information gained to inform our management strategies for the Upper Powder River mule deer herd.

 Managing Deer for Tomorrow: Upper Powder River Mule Deer Initiative Research Project

Thank you to our funding and cooperating partners:

Wyoming Game and Fish Mule Deer Initiative, Buffalo Bureau of Land Management, Wyoming Sportsman Group and multiple private landowners in the Kaycee and Buffalo area.

Updated 9/2019

Wyoming Game and Fish Department

 Phase 1 Update - Fall 2019

For more information on this and other Mule Deer Initiatives, please visit

wgfd.wyo.gov/Habitat/Statewide-Mule-Deer-Initiatives

What comes next…

Game and Fish personnel will search for radio-collared deer in November 2019 to determine which does recruited fawns onto winter range.

Radio-collared does will be recaptured in December 2019 to take biological and disease samples and measure body condition going into winter.

Travel patterns

Twenty-five of the deer showed spring migratory movements along an elevational gradient. One deer traveled an impressive 30 miles before settling onto her summer range. We are eager to track fall migrations as these deer make their way back to winter range. The rest of the deer were mostly resident deer, where their home range did not have a major seasonal shift. In the map, the pink dots represent all of the GPS locations for all of the migratory deer and the blue dots represent all of the GPS locations for all of the resident deer.

Nutrition

Deer forage on nutritionally rich foods in the spring and summer, which sustain them while lactating and holds them over through the winter when only lower quality food is available. We therefore expect doe deer to come into winter in good body condition, which is why we were surprised when the captured deer were in fairly poor condition in December. This is why it is so important for us to re-capture these deer each year in December; so we can find out if the 2018 results were an anomaly or if it is normal for these deer to be in poor condition before winter.

Mortality

As of September 2019, 18 radio-collared deer have died. When a radio-collared deer dies, we get an email notification so that we can locate the carcass as soon as possible to investigate the cause of death. To date, chronic wasting disease (CWD) is the leading cause of death for radio-collared deer, with 8 confirmed positives. Five mortalities have unknown causes because they were heavily scavenged or degraded when we located them or because we are awaiting results from tissue samples that were submitted for analysis. Two deer were likely killed by mountain lions and three, including a CWD positive deer, were likely killed by coyotes. One deer fell off of a short, steep cliff and died from trauma.

Habitat preferences

We are using deer locations to target our habitat assessment and treatment efforts. We are also looking for opportunities to modify fences to allow easier wildlife passage while maintaining their intended purpose for livestock management.

To date, we have captured and radio-collared 76 adult doe mule deer in the Upper Powder River mule deer herd unit. Captures were focused around 8 staging areas distributed throughout the herd unit. We have already learned some new and unexpected things about this mule deer herd. 



FRIDAY, JANUARY 24, 2020

Wyoming Game & Fish Discovers CWD-Positive Mule Deer in Pinedale, Discourages Feeding of Wildlife ''As of September 2019, CWD has been identified in 31 of 37 (84%) Wyoming mule deer herds, nine of 36 (25%)elk herds, and generally wherever white-tailed deer occur. 

Increasing prevalence and distribution of CWD has the potential to cause widespread and long-term negative impacts to Wyoming’s cervid populations. 

Prevalence of this disease in chronically infected Wyoming deer herds has exceeded 40%, with one elk herd exhibiting nearly 15% prevalence.'' 

''for the first time, there is clear evidence that CWD is adversely affecting the overall health and viability of some herds.''



Wyoming Game and Fish Department 2018/2019 Chronic Wasting Disease Surveillance 

Report May 2020


2012

In the endemic area of Wyoming, for example, the prevalence of CWD in mule deer has increased from approximately 11% in 1997 to 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). 


re-Wyoming Legislature Strips Science From WGFC Likely Dooming Elk To CWD Epidemic In The Future

April 27, 2021 

Terry Singeltary flounder9@verizon.net 

Dear Mr. Singeltary: 

Governor Gordon received your April 1, 2021 email titled, “Wyoming Legislature Strips Science From WGFC Likely Dooming Elk to CWD Epidemic in the Future” and he asked that I update you on the actions the Wyoming Game and Fish Department (Department) is taking in regards to chronic wasting disease (CWD) and elk feedground management within Wyoming. First, thank you for your thoughts, input and CWD reference information. 

In July 2020, the Wyoming Game and Fish Commission approved the Department’s CWD Management Plan (Plan), which guides the surveillance/monitoring of CWD and a suite of potential strategies wildlife managers may implement in an attempt to manage the prevalence and distribution of the disease in Wyoming’s deer, elk and moose herds. The Plan includes specific details on how the Department will address CWD on elk feedgrounds. Many of the concerns you raise are covered within the Plan. The document is available at: https://wgfd.wyo.gov/WGFD/media/content/PDF/Vet%20Services/Approved-CWD-Mgmt-PlanJuly-16-2020.pdf ;

One of the critical Plan components of CWD management on elk feedgrounds is the initiation of a collaborative process that will direct the long-term management of elk feedgrounds, which will include CWD and other diseases. The Department completed Phase I in January 2021. Phase I provided the public and stakeholders with a comprehensive understanding of elk feedground operation and sought feedback to help shape the public process for Phase II. Phase II is in its initial steps and will begin public engagement during the summer of 2021. The feedground legislation you reference will not impact the Department in moving forward with developing and implementing a long-term feedground management plan. I encourage you to be actively engaged in the Phase II process and beyond. For more information on the public elk feedground collaborative process, please go to: https://wgfd.wyo.gov/Get-Involved/elk-feedgrounds ;

The Department remains committed to utilizing the best available science in managing the state’s cherished ungulate populations. I appreciate your comments, thoughts and concern and look forward to your continued engagement in our collaborative process. 

Sincerely, Brian R. Nesvik Director cc: Governor’s Office Rick King, Chief, Wildlife Division Scott Edberg, Deputy Chief, Wildlife Division

=====end=====

Wyoming WGFD CWD seven positive elk in two of the hunt areas, Areas 35 and 37, for a prevalence rate estimate of 3.4 percent

CWD prevalence in North Bighorns elk herd unit

April 07, 2021

SHERIDAN -


Wyoming Legislature Strips Science From WGFC Likely Dooming Elk To CWD Epidemic In The Future

Greetings Honorable Director Nesvik, Governor, WGFC, Wyofiles, Legislatures, Hunters et al,

This is what happens when you let a bunch of legislatures that are oblivious to cwd tse prion science congregate together and remove sound science policy makers from the table, and then dictate junk science from legislators and Government to the people, and they love to do it with Chronic Wasting Disease CWD TSE Prion, and that's why CWD continues to spread, industry running the show from the sidelines. 

SCIENCE SHOWS THAT CONTINUED CONGREGATION OF CERVID IN A GIVEN AREA WILL LOAD THE ENVIRONMENT UP WITH CWD TSE PRIONS, AND SCIENCE SHOWS THAT ENVIRONMENT CAN BECOME CONTAMINATED WITH TSE PRIONS FROM 16 TO 21 YEARS!

same thing is happening in Texas with CWD TSE Prion. you let a bunch of deer farmers, breeders, dictate science, and we all lose, but that money keeps flowing, and it does not matter that what they did keeps the cwd tse prion flowing as well. 

you keep letting these elk congregate year after year after year in the same areas, with CWD growing exponentially across Wyoming, your playing with fire. 

just look at some of these game farms that had upward to 80% CWD infection rate. is that what you want these feed grounds to become?

let's review the science, shall we. 

first, let's see what the sixty-sixth Wyoming legislatures did, then the science they refuse to acknowledge, and this could cost the great state of Wyoming dearly. 

Furthermore, you cattle ranchers better start paying close attention to these cwd regulations and what these legeslators plan on doing with cwd, and how that might affect you as a cattle rancher, or a pig farmer. you don't want a deer farmer or breeder siding up close to your land, i can assure you. 

wait, there's more, CWD has now transmitted to pigs by oral routes, and that's puts a big bulls eye on the feed industry, especially since the BSE 589.2001 FEED REGULATIONS for cervid is only VOLUNTARY. the price of TSE PRION POKER has gone up, are you all in $$$ 

i can only pray that the Governor of Wyoming will shoot this down, and let the scientist do their job.

TSE PRIONS HAVE NO PLACE IN A POLITICIANS HANDS, this has been proven time and time again.

with kindest regards, 

i am sincerely,

Terry S. Singeltary Sr.

THURSDAY, APRIL 01, 2021 

Wyoming Legislature Strips Science From WGFC Likely Dooming Elk To CWD Epidemic In The Future


''Stewart has also identified high mortality rates primary causes of deaths including chronic wasting disease and mountain lion mortality and a high CWD prevalence.''

“Even though it’s a small sample size to make that calculation, (CWD prevalence) is higher than we would expect. Based on the data we have now, we are sitting at the mid-teens (15% to 17%) for prevalence in harvested adult bucks. But this time next year, that can be changed a little bit,” Stewart said.''

''What’s interesting about this project is that the doe prevalence for CWD is around 20%, higher than the adult buck prevalence an unusual occurrence. Game and Fish is curious to see if the adult buck deer prevalence increases, will the doe prevalence decrease, Stewart said.''


THIS CONCERNS ME for various reasons, but what about CWD TSE Prion transmission to Mountain Lions, and or other big cats here in the wild in North America, or what about domestic cats, what if?

we know TSE Prion can transmit to domestic cats and big zoo cats, so let's study this for a minute.

HOW MANY MOUNTAIN LIONS ARE BEING TESTED FOR CWD TSE PRION?

I BRING THIS UP FOR GREAT CONCERN, with a new outbreak of a new TSE Prion disease in a new livestock species i.e. CAMEL IN AFRICA, a rather large outbreak, and NEW STUDIES OF TRANSMISSION OF CWD AND SCRAPIE TSE PRION TRANSMITTING TO PIGS BY ORAL ROUTES, should bring great concern to everyone, especially BSE 589.2001 FEED REGULATIONS, they must be updated ASAP IMMEDIATELY!...terry

SPONGIFORM ENCEPHALOPATHY IN A CAPTIVE PUMA

an article in yesterday's Times (attached) which suggested that the puma concerned had never ''eaten any part of a cow or sheep which, in the opinion of Government Scientists, could transmit the species to a different species''.

3. You explained to me that this was INCORRECT. The position was as set out in the briefing for Prime Minister's questions attached to Mr Taylor's note. The puma had probably been fed low quality beef meat in the form of split carcasses. ...



Sun, Dec 20, 2020 4:54 pm

Subject: TSE in exotic ruminants

TSE in exotic ruminants


NUMBER OF CONFIRMED CASES OF FSE IN DOMESTIC CATS BY YEAR Year Reported No. of cases Year of Onset No. of cases

1988 0 1988 0

1989 0 1989 1

1990 12 1990 16

1991 12 1991 11

1992 10 1992 14

1993 11 1993 10

1994 16 1994 14

1995 8 1995 4

1996 6 1996 7

1997 6 1997 8

1998 4 1998 1

1999 2 1999 1

2000 1 2000 1

2001 1 2001 1

2002 0 2002 0

2003 0 2003 0

2004 0 2004 0

2005 0 2005 0

2006 0 2006 0

2007 0 2007 0

2008 0 2008 0

2009 0 2009 0

2010 0 2010 0

2011 0 2011 0

2012 0 2012 0

2013 0 2013 0

2014 0 2014 0

2015 0 2015 0

2016 0 2016 0

2017 0 2017 0

2018 0 2018 0

2019 0 2019 0

2020 0 2020 0

Total 89 Total 89 Data valid to 30 November 2020 Includes one case from Guernsey 

Published 11 February 2015 Last updated 21 December 2020 - hide all updates







SE DIAGNOSES IN EXOTIC SPECIES

KUDU 6

GEMSBOK 1

NYALA 1

ORYX 2

ELAND 6

CHEETAH 4*

PUMA 3

TIGER 1

OCELOT 2

BISON (bison bison) 1

ANKOLE COW 2

* Excludes one cheetah in Australia and one in ROI - litter mates born in GB, and another in France also born in G.B. [figures to 1 January 1998]

FELINE SPONGIFORM ENCEPHALOPATHY

TOTAL TO DATE 81 (Plus 1 in N Ireland, 1 in Norway, 1 in Lichtenstein )

YEAR Cases 

1990 12

1991 12

1992 10

1993 11

1994 16

1995 8

1996 6

1997 6


Exotic species
Species Number of cases Dates affected
Ankole Cow 2 1991, 95
Bison 1 1996
Asian Leopard Cat (1) 1 2005
Cheetah 5 1992, 98
Eland 6 1989, 95
Gemsbok 1 1987
Kudu 6 1989, 92
Lion 4 1998, 2001
Nyala 1 1986
Ocelot 3 1994, 99
Oryx 2 1989, 92
Puma 3 1992, 95
Tiger 3 1995, 99
As at 12 January 2006.
A total of 38 cases of spongiform encephalopathy have been confirmed in exotic species, the last one in 2005.
(1) Felis (Prionailurus) bengalensis.
BSE TSE PRION STATISTICS
ZOO ANIMALS AND TSE PRION DISEASE
The 82 zoo animals with BSE:
Id TSE Genus Species Subsp Birth Origin Death Place of Death
654 x Microcebus murinus - 1997 U.Montpellier 1998 U.Montpellier
656 x Microcebus murinus - 1997 U.Montpellier 1998 U.Montpellier
481 + Eulemur fulvus mayottensis 1974 Madagascar 1992 Montpellier zoo
474 + Eulemur fulvus mayottensis 1974 Madagascar 1990 Montpellier zoo
584 - Eulemur fulvus mayottensis 1984 Montpellier 1991 Montpellier zoo
455 + Eulemur fulvus mayottensis 1983 Montpellier 1989 Montpellier zoo
 - + Eulemur fulvus mayottensis 1988 Montpellier 1992 Montpellier zoo
 - + Eulemur fulvus mayottensis 1995 Montpellier 1996 Montpellier zoo
 - + Eulemur fulvus albifrons 1988 Paris 1992 Montpellier zoo
 - + Eulemur fulvus albifrons 1988 Paris 1990 Montpellier zoo
 - + Eulemur fulvus albifrons 1988 Paris 1992 Montpellier zoo
456 + Eulemur fulvus albifrons 1988 Paris 1990 Montpellier zoo
586 + Eulemur mongoz - 1979 Madagascar 1998 Montpellier zoo
 - p Eulemur mongoz - 1989 Mulhouse 1991 Montpellier zoo
 - p Eulemur mongoz - 1989 Mulhouse 1990 Montpellier zoo
 - p Eulemur macaco - 1986 Montpellier 1996 Montpellier zoo
 - p Lemur catta - 1976 Montpellier 1994 Montpellier zoo
 - p Varecia variegata variegata 1985 Mulhouse 1990 Montpellier zoo
 - p Varecia variegata variegata 1993 xxx 1994 Montpellier zoo
455 + Macaca mulatta - 1986 Ravensden UK 1992 Montpellier zoo
 - p Macaca mulatta - 1986 Ravensden UK 1993 Montpellier zoo
 - p Macaca mulatta - 1988 Ravensden UK 1991 Montpellier zoo
 - p Saimiri sciureus - 1987 Frejus France 1990 Frejus zoo
700 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo
701 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo
702 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo
703 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo
704 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo
705 pc eulemur hybrid - - Besancon zoo 1998 Besancon zoo
706 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
707 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
708 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
709 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
710 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
711 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
712 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
713 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
714 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
715 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
716 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
717 pc eulemur hybrid - - Strasbourg zoo 1998 Strasbourg zoo
 x p genus species - - Lille zoo 1996 Lille zoo
 y p genus species - - Lille zoo 1996 Lille zoo
 z p genus species - - Lille zoo 1996 Lille zoo 
1 + Actinonyx jubatus cheetah 1986 Marwell zoo 1991 Pearle Coast AU
Duke + Actinonyx jubatus cheetah 1984 Marwell zoo 1992 Colchester zoo? UK
Saki + Actinonyx jubatus cheetah 1986 Marwell zoo 1993 unknown UK
Mich + Actinonyx jubatus cheetah 1986 Whipsnade 1993 Whipsnade UK
Fr1 + Actinonyx jubatus cheetah 1987 Whipsnade 1997 Safari de Peaugres FR
Fr2 + Actinonyx jubatus cheetah 1991 Marwell zoo 1997 Safari de Peaugres Fr
xx + Actinonyx jubatus cheetah 19xx xxx zoo 199x Fota zoo IR
yy + Actinonyx jubatus cheetah 19xx yyy zoo 1996+ yyyy zoo UK
zz + Actinonyx jubatus cheetah 19xx zzz zoo 1996+ yyyy zoo UK
aaa + Felis concolor puma 1986 Chester zoo 1991 Chester zoo UK
yy + Felis concolor puma 1980 yyy zoo 1995 yyyy zoo UK
zz + Felis concolor puma 1978 zzz zoo 1995 zzzz zoo UK
xxx + Felis pardalis ocelot 1987 xxx 1994 Chester zoo UK
zzz + Felis pardalis ocelot 1980 zzz 1995 zzzz zoo UK
85 + Felis catus cat 1990+ various 1999+ various UK LI NO 
19 + Canis familia. dog 1992+ various 1999+ various UK 
Fota + Panthera tigris tiger 1981 xxx zoo 1995 xxxx zoo UK
yy + Panthera tigris tiger 1983 yyy zoo 1998 yyyy zoo UK
Lump + Panthera leo lion 1986 Woburn SP 1998 Edinburgh zoo UK [since 1994]
1 + Taurotragus oryx eland 1987 Port Lympne 1989 Port Lympne zoo UK
Moll + Taurotragus oryx eland 1989 xx UK 1991 not Port Lympne UK
Nedd + Taurotragus oryx eland 1989 xx UK 1991 not Port Lympne UK
Elec + Taurotragus oryx eland 1990 xx UK 1992 not Port Lympne Uk
Daph p Taurotragus oryx eland 1988 xx UK 1990 not Port Lympne UK
zzz + Taurotragus oryx eland 1991 zz UK 1994 zzz UK 
yyy + Taurotragus oryx eland 1993 yy UK 1995 yyy UK 
Fran p Tragelaphus strepsi. kudu 1985 London zoo 1987 London zoo UK
Lind + Tragelaphus strepsi. kudu 1987 London zoo 1989 London zoo UK
Karl + Tragelaphus strepsi. kudu 1988 London zoo 1990 London zoo UK
Kaz + Tragelaphus strepsi. kudu 1988 London zoo 1991 London zoo UK
Bamb pc Tragelaphus strepsi. kudu 1988 London zoo 1991 London zoo UK
Step - Tragelaphus strepsi. kudu 1984 London zoo 1991 London zoo UK
346 pc Tragelaphus strepsi. kudu 1990 London zoo 1992 London zoo UK
324 + Tragelaphus strepsi. kudu 1989 Marwell zoo 1992 London zoo UK
xxx + Tragelaphus angasi nyala 1983 Marwell zoo 1986 Marwell zoo UK
yy + Oryx gazella gemsbok 1983 Marwell zoo 1986 Marwell zoo UK
zz + Oryx gazella gemsbok 1994+ zzz zoo 1996+ zzzz zoo UK
xx + Oryx dammah scim oryx 1990 xxxx zoo 1993 Chester zoo UK
yy + Oryx leucoryx arab oryx 1986 Zurich zoo 1991 London zoo UK
yy + Bos taurus ankole cow 1987 yyy zoo 1995 yyyy zoo UK
zz + Bos taurus ankole cow 1986 zzz zoo 1991 zzzz zoo UK
xx + Bison bison Eu bison 1989 xxx zoo 1996 xxxx zoo UK
Vet Rec 1997 Sep 13;141(11):270-1 Baron-T, Belli-P Madec-J-Y Moutou-F Vitaud-C Savey-M Spongiform encephalopathy in an imported cheetah in France. CNEVA-Lyon, Laboratoire de Pathologie Bovine, France.

Proc Soc Exp Biol Med 1996 Apr;211(4):306-22 Narang H Origin and implications of bovine spongiform encephalopathy. [tiger]

Vet Rec. 1994 Nov 12;135(20):488. Benbow G. Spongiform encephalopathies in zoo animals. comment

Vet Rec 1994 Oct 29;135(18):440 Swainston J. comment

Vet Rec 1994 Sep 24;135(13):296-303 Kirkwood JK, Cunningham AA Epidemiological observations on spongiform encephalopathies

Vet Rec 1994 Feb 12;134(7):167-8 Kirkwood JK, Cunningham AA, Austin AR, Wells GA, Sainsbury AW Spongiform encephalopathy in a greater kudu

Vet Rec. 1993 Oct 9;133(15):360-4. Kirkwood JK, et al. Spongiform encephalopathy in a herd of greater kudu

Vet Rec. 1993 Jan 16;132(3):68. Cunningham AA, et al. Transmissible spongiform encephalopathy in greater kudu

Vet Rec. 1992 Nov 7;131(19):431-4. Willoughby K, et al. Spongiform encephalopathy in a captive puma

Aust Vet J 1992 Jul;69(7):171 Peet RL, Curran JM Spongiform encephalopathy in an imported cheetah

Vet Rec 1992 Apr 25;130(17):365-7 Kirkwood JK, Wells GA, Cunningham AA, Jackson SI, Scott AC, Dawson M, Wilesmith JW Scrapie-like encephalopathy in a greater kudu

Acta Neuropathol (Berl) 1992;84(5):559-69 Jeffrey M, Scott JR, Williams A, Fraser H Ultrastructural features of spongiform encephalopathy

Vet Rec. 1991 Oct 5;129(14):320 Synge BA, et al. Spongiform encephalopathy in a Scottish cat.

Vet Rec 1991 Sep 14;129(11):233-6 Wyatt JM, Pearson GR, Naturally occurring scrapie-like s

Vet Rec. 1991 Jun 1;128(22):532. Pearson GR, et al. Feline spongiform encephalopathy.

Vet Rec. 1991 Mar 30;128(13):311. Kock R. Spongiform encephalopathies in ungulates.

Vet Rec. 1991 Feb 2;128(5):115. Gibson PH. Spongiform encephalopathies in ungulates. comment

Vet Rec 1990 Dec 15;127(24):586-8 Leggett MM, Dukes J, Pirie HM A spongiform encephalopathy in a cat.

Done JT. Vet Rec. 1990 Nov 10;127(19):484. Spongiform encephalopathy in pigs.

Vet Rec. 1990 Oct 27;127(17):418-20. Kirkwood JK, et al. Spongiform encephalopathy in an arabian oryx (Oryx leucoryx) and a greater kudu.

Vet Rec. 1990 Sep 29;127(13):338. Dawson M, et al. Primary parenteral transmission of bovine spongiform encephalopathy to the pig.

Vet Rec. 1990 May 19;126(20):513 no authors listed Spongiform encephalopathy in a cat.

Vet Rec 1990 May 12;126(19):489-90 Gibson PH Spongiform encephalopathy in an eland.

Nature. 1990 Mar 15;344(6263):183 Aldhous P. Antelopes die of "mad cow" disease.

Vet Rec 1990 Apr 21;126(16):408-9 Fleetwood AJ, Furley CW Spongiform encephalopathy in an eland.

Vet Pathol. 1988 Sep;25(5):398-9 Jeffrey M, Wells GA Spongiform encephalopathy in a nyala (Tragelaphus angasi) Lasswade Veterinary Laboratory, Midlothian



The BSE Inquiry / Statement No 324

Dr James Kirkwood

(not scheduled to give oral evidence)

Statement to the BSE Inquiry

James K Kirkwood BVSc PhD FIBiol MRCVS

[This witness has not been asked to give oral evidence in Phase 1 of the Inquiry] 1. I became involved in the field of TSEs through my work as Head of the Veterinary Science Group at the Zoological Society of London’s Institute of Zoology. I held this post from November 1984 until June 1996, when I took up my present post at UFAW. During this time, concurrent with the BSE epidemic, cases of scrapie-like spongiform encephalopathies occurred in animals at the Zoological Society of London’s collections at Regent’s Park and Whipsnade and in other zoos. It was appropriate to investigate the epidemiology of these cases in order to try to determine the possible impact on zoo animals and breeding programmes, and to consider how the disease in zoo animals might be controlled.

2. Throughout the period from 1985 to March 1996, I worked at the Institute of Zoology (IoZ). I was Head of the Veterinary Science Group of the IoZ and Senior Veterinary Officer of the Zoological Society of London (ZSL). I was responsible for the provision of the veterinary service for the ZSL collections.

3. During the period from 1985 to March 1996, scrapie-like spongiform encephalopathies were diagnosed in the following animals which died, or were euthanased, at London Zoo and Whipsnade:

Animal Sex Date of Death Age (mos)

Arabian Oryx Oryx leucoryx F 24.3.89 38

Greater kudu Tragelaphus strepsiceros (Linda) F 18.8.89 30

Greater kudu (Karla) F 13.11.90 19

Greater kudu (Kaz) M 6.6.91 37

Greater kudu (Bambi) M 24.10.91 36

Greater kudu (346/90) M 26.2.92 18

Greater kudu (324/90) F 22.11.92 38

Cheetah Acinonyx jubatus (Michelle) F 22.12.93 91

All these cases were described in papers published in the scientific literature (as cited below).

4. All the animals listed above were bred in captivity. The greater kudu were from a highlyinbred group whose founders were Koo (imported from West Africa in 1967), Doo (imported from a Danish Zoo in 1969) and Chester (transferred to London from Chester Zoo in 1982). The family tree of the group is shown in: Kirkwood, J.K., Cunningham, A.A., Wells, G.A.H., Wilesmith, J.W. & Barnett, J.E.F. (1993) Spongiform encephalopathy in a herd of Greater kudu Tragelaphus strepsiceros: epidemiological observations. Veterinary Record 133, 360-364: (J/VR/133/360)

5. The first case diagnosed among the ZSL’s animals was in the greater kudu ‘Linda’, which died in August 1989. Retrospective examination of the brain of the Arabian oryx that had died 5 months earlier, revealed that this animal also had brain lesions characteristic of a scrapielike spongiform encephalopathy. Diagnostic histopathology of these (and of all the other cases that occurred at London and Whipsnade) was undertaken by the Central Veterinary Laboratory. The clinical features, diagnosis and possible aetiology of these first two ZSL cases was discussed in a paper published in 1990 (Kirkwood, J.K., Wells, G.A.H., Wilesmith, J.W., Cunningham, A.A. & Jackson, S.I. (1990) Spongiform encephalopathy in an Arabian oryx Oryx leucoryx and a greater kudu Tragelaphus strepsiceros. Veterinary Record 127, 418-420).(J/VR/127/418). We noted, in this paper, that it seemed probable that these cases had a common aetiology with BSE.

6. A greater kudu ‘Frances’ which had died some 18 months earlier (17.11.87) had shown clinical signs which, in retrospect, could have been due to SE but CNS tissue had not been saved for examination so this could not be checked (Kirkwood, J.K., Cunningham, A.A., Wells, G.A.H., Wilesmith, J.W. & Barnett, J.E.F. (1993) Spongiform encephalopathy in a herd of Greater kudu Tragelaphus strepsiceros: epidemiological observations. Veterinary Record 133, 360-364)(J/VR/133/360).

7. During the following 3 years, SE was diagnosed in 5 further greater kudu in the ZSL collections (see list in point 3 above). The second confirmed case in a greater kudu occurred in the 19-month old calf (Karla) born to the first confirmed case (Linda). This case gave us concern since the calf was born after the July 1988 ban on inclusion of ruminant derived protein in ruminant feeds and it was considered to be extremely unlikely that this animal could have been exposed to contaminated feeds (the kudu diet prior to February 1987 had included a cattle pellet but pelleted diets fed from then on were thought not to contain RDP). We speculated that maternal transmission may have occurred (Kirkwood, J.K., Wells, G.A.H., Cunningham, A.A., Jackson, S.I., Scott, A.C., Dawson, M. & Wilesmith, J.W. (1992). Scrapie-like encephalopathy in greater kudu (Tragelaphus strepsiceros) which had not been fed on ruminant-derived protein. Veterinary Record 130, 365-367:J/VR/130/365).

8. Since the next three animals in which the disease was confirmed (Kaz, Bambi and 346/90) were not thought to have been exposed to contaminated feeds, and were not born to dams who had been clinical cases (Cunningham, A.A., Wells, G.A.H., Scott, A.C., Kirkwood, J.K. & Barnett, J.E.F. (1993) Transmissible spongiform encephalopathy in greater kudu (Tragelaphus strepsiceros). Veterinary Record 132, 68), we considered the possibility that horizontal transmission may have occurred (Kirkwood, J.K., Cunningham, A.A., Wells, G.A.H., Wilesmith, J.W. & Barnett, J.E.F. (1993) Spongiform encephalopathy in a herd of Greater kudu Tragelaphus strepsiceros: epidemiological observations. Veterinary Record 133, 360-364: J/VR/132/68). The occurrence of SE in a greater kudu (324/90), that had been born in another zoo and was not thought to have been exposed to feeds contaminated with RDP, 27 months after being introduced to the group at Regent’s Park, was further cause for concern that transmission may have occurred between animals (Kirkwood, J.K., Cunningham, A.A., Austin, A.R.,Wells, G.A.H & Sainsbury, A.W. (1994) Spongiform encephalopathy in a Greater kudu Tragelaphus strepsiceros introduced into an affected group. Veterinary Record 134, 167-168: J/VR/134/167).

9. At that time, around 1993, the most likely explanation of the pattern of events seemed to be that the disease in kudu was the same as that in cattle: that it had originally entered the group in contaminated feed but that thereafter transmission may have occurred between individuals (Cunningham, A.A., Wells, G.A.H., Scott, A.C., Kirkwood, J.K. & Barnett, J.E.F. (1993) Transmissible spongiform encephalopathy in greater kudu (Tragelaphus strepsiceros).Veterinary Record 132, 68). In view of this and the likelihood that individuals of a wide range of species of zoo animals had been exposed we recommended (Kirkwood, J.K., Cunningham, A.A., Wells, G.A.H., Wilesmith, J.W. & Barnett, J.E.F. (1993) Spongiform encephalopathy in a herd of Greater kudu Tragelaphus strepsiceros: epidemiological observations. Veterinary Record 133, 360-364 ) that all zoo animals that may have been exposed to contaminated feeds should be observed closely and that because of the potentially serious implications for captive breeding programmes, well-described by my colleague Mr Andrew Cunningham (Cunningham, A.A. (1991) Bovine spongiform encephalopathy and British Zoos. Journal of Zoo and Wildlife Medicine 11, 605-634: J/ZWM/11/605), there was a need for caution about exporting such animals. In addition the kudu were kept isolated from other zoo animals. A very cautious approach was taken and the keeper staff used separate tools for cleaning out the kudu dens and paddocks, changed overalls and boots, used latex gloves, and, for a period until it seemed (for reasons mentioned below) less likely that the situation in kudu differed from that in cattle, collected wastes for incineration. Management practices were reviewed again in March 1996 when it was announced that cases of new variant CJD had occurred which might be related to the BSE agent. In order to pre-empt any public concern that might follow this announcement, the walkways past the kudu paddock were closed to the public. No cases have occurred in the kudu group since 1992.

10. The case of SE in a cheetah that occurred during the period, involved a 7 year-old female which had been born and lived all her life at Whipsnade (except for the final stages when she was moved to the Animal Hospital at Regent’s Park for diagnosis and treatment). This animal, which died in December 1993, had been fed on cuts of meat and bone from carcases of cattle unfit for human consumption and it was thought likely that she had been exposed to spinal cord (Kirkwood, J.K., Cunningham, A.A., Flach, E.J., Thornton, S.M. & Wells, G.A.H. (1995) Spongiform encephalopathy in another captive cheetah (Acinonyx jubatus): evidence for variation in susceptibility or incubation periods between species. Journal of Zoo and Wildlife Medicine 26, 577-582: J/ZWM/26/577).

11. During the period we also collated information on cases of SE that occurred in wild animals at or from other zoos in the British Isles. The total number of cases of which I was aware in June 1996, when I presented a review on occurrence of spongiform encephalopathies in zoo animals (at the Royal College of Pathologists’ Symposium on Transmitting prions: BSE, CJD, and other TSEs, The Royal Society, London, 4th July 1996), was 25, involving 10 species. The animals involved were all from the families Bovidae and Felidae, and comprised: 1 Nyala Tragelaphus angasi, 5 Eland Taurotragus oryx, 6 greater kudu Tragelaphus strepsiceros, 1 Gemsbok Oryx gazella, 1 Arabian oryx Oryx leucoryx, 1 Scimitar-horned oryx Oryx dammah, 4 Cheetah Acinonyx jubatus, 3 Puma Felis concolor 2 Ocelot Felis pardalis, and 1 Tiger Panthera tigris. (A spongiform encephalopathy, which was thought probably to have a different aetiology, had also been reported in 3 ostriches Struthio camelus in Germany). This list did not include cases of BSE in domesticated species in zoos (ie BSE in Ankole or other cattle, or SEs, assumed to be scrapie, in mouflon sheep Ovis musimon).

12. Since the time the above statistics were published, a few further cases have occurred in animals at or from zoos in the British Isles. The total number of cases in cheetah that have now been documented has, as far as I am aware, risen to seven (Vitaud, C., Flach, E.J., Thornton, S.M. & Capello, R. (1998) Clinical observations on four cases of feline spongiform encephalopathy in cheetahs (Acinonyx jubatus). Proceedings of the European Association of Zoo and Wildlife Veterinarians, Chester, UK, 21st-24th May 1998. Pp 133-138). There has also been a case in a bison.

13. Epidemiological aspects of the majority of these cases (those diagnosed up to the end of 1993) were considered in paper published in 1994 (Kirkwood, J.K. & Cunningham, A.A. (1994) Epidemiological observations on spongiform encephalopathies in captive wild animals in the British Isles. Veterinary Record 135, 296-303:J/VR/135/296.) This paper was based on a paper presented at the Consultation on BSE with the Scientific Veterinary Committee of the Commission of the European Communities held in Brussels, 14-15th September 1993 (Kirkwood, J.K. & Cunningham, A.A. (1993) Spongiform encephalopathy in captive wild animals in Britain: epidemiological observations. In R. Bradley & B Marchant (Eds) Transmissible spongiform encephalopathies. Proceedings of a Consultation on BSE with the Scientific Veterinary Committee of the Commission of the European Communities, 14-15 September 1993, Brussels. European Commission. Pp 29-47:M9 tab 46). It was thought likely that at least some, and probably all, of the cases in zoo animals were caused by the BSE agent. Strong support for this hypothesis came from the findings of Bruce and others (1994) ( Bruce, M.E., Chree, A., McConnell, I., Foster, J., Pearson, G. & Fraser, H. (1994) Transmission of bovine spongiform encephalopathy and scrapie to mice: strain variation and species barrier. Philosophical Transactions of the Royal Society B 343, 405-411: J/PTRSL/343/405), who demonstrated that the pattern of variation in incubation period and lesion profile in six strains of mice inoculated with brain homogenates from an affected kudu and the nyala, was similar to that seen when this panel of mouse strains was inoculated with brain from cattle with BSE. The affected zoo bovids were all from herds that were exposed to feeds that were likely to have contained contaminated ruminant-derived protein and the zoo felids had been exposed, if only occasionally in some cases, to tissues from cattle unfit for human consumption.

14. Among the affected bovids were others (including scimitar horned oryx and eland) which, like some of the kudu, were born some considerable time after the July 1988 ban on inclusion of RDP in ruminant feeds (Kirkwood, J.K. & Cunningham, A.A. (1994) Epidemiological observations on spongiform encephalopathies in captive wild animals in the British Isles. Veterinary Record 135, 296-303:J/VR/135/296). The source of infection to these animals was puzzling. However, as it emerged that many cases of BSE were continuing to occur in domestic cattle born after the July 1988 ban on inclusion of RDP in ruminant feeds, it was clear that the ban had not been immediately effective, and it was therefore possible (or, at least, impossible to rule out) that the late cases in zoo ungulates were also due to exposure to contaminated feeds.

15. We drew attention to the fact that, from a taxonomic perspective, the incidence of cases was strikingly patchy (Kirkwood, J.K. & Cunningham, A.A. (1994) Epidemiological observations on spongiform encephalopathies in captive wild animals in the British Isles. Veterinary Record 135, 296-303. Also Kirkwood, J.K., Cunningham, A.A., Flach, E.J., Thornton, S.M. & Wells, G.A.H. (1995) Spongiform encephalopathy in another captive cheetah (Acinonyx jubatus): evidence for variation in susceptibility or incubation periods between species? Journal of Zoo and Wildlife Medicine 26, 577-582) Compared with many other species of exotic ruminants, few kudu were present in the UK but there had been 6 cases of SE among them. The picture seemed similar in the felids. Compared with other species of exotic felids (eg lions in which no cases had occurred), there were relatively small numbers of puma and cheetah in the UK but (at that time) there had been 3 and 4 cases among these respectively. Almost certainly a wider range of species were exposed to contaminated feeds than those in which cases have occurred or been detected. However, we were cautious about drawing firm conclusions about variation in susceptibility between species because (i) incubation periods vary between species and we thought other cases may emerge and (ii) because the variation might be related to differences in intensity of exposure.


TSEs in Exotic Ruminants

TSEs have been detected in exotic ruminants in UK zoos since 1986. These include antelopes (Eland, Gemsbok, Arabian and Scimitar oryx, Nyala and Kudu), Ankole cattle and Bison. With hindsight the 1986 case in a Nyala was diagnosed before the first case of BSE was identified. The TSE cases in exotic ruminants had a younger onset age and a shorter clinical duration compared to that in cattle with BSE. All the cases appear to be linked to the BSE epidemic via the consumption of feed contaminated with the BSE agent. The epidemic has declined as a result of tight controls on feeding mammalian meat and bone meal to susceptible animals, particularly from August 1996.


American Society for Microbiology

Journal of VirologyVolume 87, Issue 4, 15 February 2013, Pages 1947-1956


Prions

Susceptibility of Domestic Cats to Chronic Wasting Disease

Candace K. Mathiasona, Amy V. Nallsa, Davis M. Seeliga, Susan L. Kraftb, Kevin Carnesb, Kelly R. Andersona, Jeanette Hayes-Kluga, and Edward A. Hoovera

aDepartment of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, USA

bDepartment of Environmental and Radiological Health Sciences, Colorado State University, Fort Collins, Colorado, USA

ABSTRACT

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 intracerebrally (i.c.) or orally (p.o.) with CWD-infected deer brain. At 40 and 42 months postinoculation, two i.c.-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 the cats progressed to terminal disease within 5 months. Brains from these two cats were pooled and inoculated into cohorts of cats by the i.c., p.o., and intraperitoneal and subcutaneous (i.p./s.c.) routes. Upon subpassage, feline CWD was transmitted to all i.c.-inoculated cats with a decreased incubation period of 23 to 27 months. Feline-adapted CWD (FelCWD) was demonstrated in the brains of all of the affected cats by Western blotting and immunohistochemical analysis. Magnetic resonance imaging revealed abnormalities in clinically ill cats, which included multifocal T2 fluid attenuated inversion recovery (FLAIR) signal hyperintensities, ventricular size increases, prominent sulci, and white matter tract cavitation. Currently, 3 of 4 i.p./s.c.- and 2 of 4 p.o. secondary passage-inoculated 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.

SNIP...

Implications associated with trans-species transmission of CWD prions.

It has been determined that, once a prion strain has been adapted to a new host species, the prions from this new host species propagate more efficiently in a third host. Specifically, the passage of cow BSE prions in sheep or goats markedly increased the transmission efficiency into human transgenic mice (70). Although a substantial species barrier appears to exist between deer and cats, barring an invasive route of inoculation, we must consider the epidemiologic and ecologic implications associated with CWD transmission to felids, which could potentially result in the generation of prion strains adapted for natural (mucosal) routes of transmission to felids and/or other noncervid species. The high genetic identity homology observed between domestic and nondomestic cats compared to 5 other mammalian species sharing a home range with CWD-infected cervids, while warranting further analysis of protein structure and protein-protein interactions, suggests that nondomestic cats may be a susceptible reservoir species in nature (Fig. 5). If CWD has the ability to infect and establish alternate host reservoirs in nature or to initiate the adaptations necessary for trans-species transmission, this will impact not only wildlife, but also domestic species, which can lead to serious consequences for human health. Here, we have demonstrated that one scavenger/predator species (the domestic cat) that cohabitates with the natural hosts of CWD could become an intermediate host for this prion disease in nature.

Received 21 September 2012; Accepted 4 December 2012; Published online 23 January 2013. Copyright © 2013, American Society for Microbiology. All Rights Reserved. Address correspondence to Candace K. Mathiason, candace.mathiason@colostate.edu. C.K.M. and A.V.N. contributed equally to this article as first authors.


***> READ THIS VERY, VERY, CAREFULLY, AUGUST 1997 MAD COW FEED BAN WAS A SHAM, AS I HAVE STATED SINCE 1997! 3 FAILSAFES THE FDA ET AL PREACHED AS IF IT WERE THE GOSPEL, IN TERMS OF MAD COW BSE DISEASE IN USA, AND WHY IT IS/WAS/NOT A PROBLEM FOR THE USA, and those are; 

BSE TESTING (failed terribly and proven to be a sham) 

BSE SURVEILLANCE (failed terribly and proven to be a sham) 

BSE 589.2001 FEED REGULATIONS (another colossal failure, and proven to be a sham) 

these are facts folks. trump et al just admitted it with the feed ban. 

see; 

FDA Reports on VFD Compliance 

John Maday 

August 30, 2019 09:46 AM VFD-Form 007 (640x427) 

Before and after the current Veterinary Feed Directive rules took full effect in January, 2017, the FDA focused primarily on education and outreach. ( John Maday ) Before and after the current Veterinary Feed Directive (VFD) rules took full effect in January, 2017, the FDA focused primarily on education and outreach to help feed mills, veterinarians and producers understand and comply with the requirements. Since then, FDA has gradually increased the number of VFD inspections and initiated enforcement actions when necessary. On August 29, FDA released its first report on inspection and compliance activities. The report, titled “Summary Assessment of Veterinary Feed Directive Compliance Activities Conducted in Fiscal Years 2016 – 2018,” is available online.


SUNDAY, SEPTEMBER 1, 2019 

***> FDA Reports on VFD Compliance 


TUESDAY, APRIL 18, 2017 

*** EXTREME USA FDA PART 589 TSE PRION FEED LOOP HOLE STILL EXIST, AND PRICE OF POKER GOES UP *** 

THURSDAY, SEPTEMBER 26, 2019 

Veterinary Biologics Guideline 3.32E: Guideline for minimising the risk of introducing transmissible spongiform encephalopathy prions and other infectious agents through veterinary biologics


U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001

Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001

Date: Tue, 9 Jan 2001 16:49:00 -0800

From: "Terry S. Singeltary Sr."

Reply-To: Bovine Spongiform Encephalopathy


snip...

[host Richard Barns] and now a question from Terry S. Singeltary of CJD Watch.

[TSS] yes, thank you, U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?

[no answer, you could hear in the back ground, mumbling and 'we can't. have him ask the question again.]

[host Richard] could you repeat the question?

[TSS] U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?

[not sure whom ask this] what group are you with?

[TSS] CJD Watch, my Mom died from hvCJD and we are tracking CJD world-wide.

[not sure who is speaking] could you please disconnect Mr. Singeltary

[TSS] you are not going to answer my question?

[not sure whom speaking] NO

snip...see full archive and more of this;


3.2.1.2 Non‐cervid domestic species

The remarkably high rate of natural CWD transmission in the ongoing NA epidemics raises the question of the risk to livestock grazing on CWD‐contaminated shared rangeland and subsequently developing a novel CWD‐related prion disease. This issue has been investigated by transmitting CWD via experimental challenge to cattle, sheep and pigs and to tg mouse lines expressing the relevant species PrP.

For cattle challenged with CWD, PrPSc was detected in approximately 40% of intracerebrally inoculated animals (Hamir et al., 2005, 2006a, 2007). Tg mice expressing bovine PrP have also been challenged with CWD and while published studies have negative outcomes (Tamguney et al., 2009b), unpublished data provided for the purposes of this Opinion indicate that some transmission of individual isolates to bovinised mice is possible (Table 1).

In small ruminant recipients, a low rate of transmission was reported between 35 and 72 months post‐infection (mpi) in ARQ/ARQ and ARQ/VRQ sheep intracerebrally challenged with mule deer CWD (Hamir et al., 2006b), while two out of two ARQ/ARQ sheep intracerebrally inoculated with elk CWD developed clinical disease after 28 mpi (Madsen‐Bouterse et al., 2016). However, tg mice expressing ARQ sheep PrP were resistant (Tamguney et al., 2006) and tg mice expressing the VRQ PrP allele were poorly susceptible to clinical disease (Beringue et al., 2012; Madsen‐Bouterse et al., 2016). In contrast, tg mice expressing VRQ sheep PrP challenged with CWD have resulted in highly efficient, life‐long asymptomatic replication of these prions in the spleen tissue (Beringue et al., 2012).

A recent study investigated the potential for swine to serve as hosts of the CWD agent(s) by intracerebral or oral challenge of crossbred piglets (Moore et al., 2016b, 2017). Pigs sacrificed at 6 mpi, approximately the age at which pigs reach market weight, were clinically healthy and negative by diagnostic tests, although low‐level CWD agent replication could be detected in the CNS by bioassay in tg cervinised mice. Among pigs that were incubated for up to 73 mpi, some gave diagnostic evidence of CWD replication in the brain between 42 and 72 mpi. Importantly, this was observed also in one orally challenged pig at 64 mpi and the presence of low‐level CWD replication was confirmed by mouse bioassay. The authors of this study argued that pigs can support low‐level amplification of CWD prions, although the species barrier to CWD infection is relatively high and that the detection of infectivity in orally inoculated pigs with a mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity.




MONDAY, NOVEMBER 16, 2020 

North America coyotes or pumas can serve as a vehicle for prions contributing to the spread of the infectious agent in the environment


THURSDAY, DECEMBER 19, 2019

TSE surveillance statistics exotic species and domestic cats Update December 2019


CWD AND SCRAPIE TRANSMISSION TO PIGS BY ORAL ROUTES, OH MY!

2021 Transmissible Spongiform Encephalopathy TSE Prion End of Year Report 2020

CJD FOUNDATION VIRTUAL CONFERENCE CJD Foundation Research Grant Recipient Reports Panel 2 Nov 3, 2020

zoonotic potential of PMCA-adapted CWD PrP 96SS inoculum


4 different CWD strains, and these 4 strains have different potential to induce any folding of the human prion protein. 


***> PIGS, WILD BOAR, CWD <***

***> POPULATIONS OF WILD BOARS IN THE UNITED STATES INCREASING SUPSTANTUALLY AND IN MANY AREAS WE CAN SEE  A HIGH DENSITY OF WILD BOARS AND HIGH INCIDENT OF CHRONIC WASTING DISEASE

HYPOTHOSIS AND SPECIFIC AIMS

HYPOTHOSIS 

BSE, SCRAPIE, AND CWD, EXPOSED DOMESTIC PIGS ACCUMULATE DIFFERENT QUANTITIES AND STRAINS OF PRIONS IN PERIPHERAL TISSUES, EACH ONE OF THEM WITH PARTICULAR ZOONOTIC POTENTIALS


Final Report – CJD Foundation Grant Program A. 

Project Title: Systematic evaluation of the zoonotic potential of different CWD isolates. Principal Investigator: Rodrigo Morales, PhD.


Systematic evaluation of the zoonotic potential of different CWD isolates. Rodrigo Morales, PhD Assistant Professor Protein Misfolding Disorders lab Mitchell Center for Alzheimer’s disease and Related Brain Disorders Department of Neurology University of Texas Health Science Center at Houston Washington DC. July 14th, 2018

Conclusions and Future Directions • We have developed a highly sensitive and specific CWD-PMCA platform to be used as a diagnostic tool. • Current PMCA set up allow us to mimic relevant prion inter-species transmission events. • Polymorphic changes at position 96 of the prion protein apparently alter strain properties and, consequently, the zoonotic potential of CWD isolates. • Inter-species and inter-polymorphic PrPC → PrPSc conversions further increase the spectrum of CWD isolates possibly present in nature. • CWD prions generated in 96SS PrPC substrate apparently have greater inter-species transmission potentials. • Future experiments will explore the zoonotic potential of CWD prions along different adaptation scenarios, including inter-species and inter-polymorphic.



Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Location: Virus and Prion Research

Title: Disease-associated prion protein detected in lymphoid tissues from pigs challenged with the agent of chronic wasting disease 

Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.



Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research

Title: The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP 

Author item MOORE, S - Orise Fellow item Kokemuller, Robyn item WEST-GREENLEE, M - Iowa State University item BALKEMA-BUSCHMANN, ANNE - Friedrich-Loeffler-institut item GROSCHUP, MARTIN - Friedrich-Loeffler-institut item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 5/10/2018 Publication Date: 5/22/2018 Citation: Moore, S.J., Kokemuller, R.D., West-Greenlee, M.H., Balkema-Buschmann, A., Groschup, M.H., Greenlee, J.J. 2018. The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP. Prion 2018, Santiago de Compostela, Spain, May 22-25, 2018. Paper No. WA15, page 44.

Interpretive Summary:

 The successful transmission of pig-passaged CWD to Tg40 mice reported here suggests that passage of the CWD agent through pigs results in a change of the transmission characteristics which reduces the transmission barrier of Tg40 mice to the CWD agent. If this biological behavior is recapitulated in the original host species, passage of the CWD agent through pigs could potentially lead to increased pathogenicity of the CWD agent in humans.


cwd scrapie pigs oral routes 

***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <*** 

>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <*** 

***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%). 

***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains. 




Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.


CONFIDENTIAL

EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY

LINE TO TAKE

3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:- 

 "There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.

DO Hagger RM 1533 MT Ext 3201


While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...


we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.


May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...


3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...


But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...


Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....


Tuesday, April 27, 2021 

Working Document on Camel Prion Disease (CPrD) 14/09/2020


Subject: TEXAS CWD TSE PRION 

TEXAS CHRONIC WASTIND DISEASE CWD TSE PRION CASES JUMPS TO 228 CONFIRMED

In Texas, the disease was first discovered in 2012 in free-ranging mule deer along a remote area of the Hueco Mountains near the Texas-New Mexico border and has since been detected in 228 captive or free-ranging cervids, including white-tailed deer, mule deer, red deer and elk in 13 Texas counties. For more information on previous detections visit the CWD page on the TPWD website.


TEXAS CWD, see the latest positives;

2021-04-27 Breeder Deer Mason Facility #10 White-tailed Deer M 2.482191781

2021-04-27 Breeder Deer Uvalde Facility #7 White-tailed Deer M 1.5

2021-04-27 Breeder Deer Uvalde Facility #7 White-tailed Deer M 1.5

2021-04-20 Breeder Deer Matagorda Facility #9 White-tailed Deer F 1.5

2021-03-29 Breeder Deer Uvalde Facility #7 White-tailed Deer F 3.536986301

2021-03-29 Breeder Deer Uvalde Facility #7 White-tailed Deer M 2.178082192

2021-03-29 Breeder Deer Uvalde Facility #7 White-tailed Deer M 3.5

2021-03-29 Breeder Deer Uvalde Facility #7 White-tailed Deer M 1.545205479

2021-03-29 Breeder Deer Uvalde Facility #7 White-tailed Deer M 2.482191781

2021-03-29 Breeder Deer Hunt Facility #8 White-tailed Deer F 2.482191781

expand to see all;


Texas Kimble County Farm Chronic Wasting Disease CWD TSE Prion Approximate Herd Prevalence 12% 

Texas Kimble County Farm Chronic Wasting Disease CWD TSE Prion Approximate Herd Prevalence 12%

SUMMARY MINUTES OF THE 407th COMMISSION MEETING Texas Animal Health Commission

September 22, 2020 

Chronic Wasting Disease (CWD):

A new CWD positive breeding herd was disclosed in February 2020 in Kimble County. This herd depopulation was completed in July 2020. Including the two index positive deer, an additional eight more positive deer were disclosed (approximate herd prevalence 12%). Since July 2015 and prior to this discovery, five positive captive breeder herds have been disclosed and four of those are in Medina County. One herd in Lavaca and three herds in Medina County were depopulated leaving one large herd in Medina County that is managed on a herd plan. A new zone was established in Val Verde County in December 2019 as a result of a positive free-ranging White-tailed Deer (WTD). A second positive WTD was also disclosed in February 2020 in the same area. 


“Regrettably, the gravity of this situation continues to mount with these new CWD positive discoveries, as well as with the full understanding of just how many other facilities and release sites across Texas were connected to the CWD positive sites in Uvalde and Hunt Counties,” said Carter Smith, Executive Director of TPWD.



“Regrettably, the gravity of this situation continues to mount with these new CWD positive discoveries, as well as with the full understanding of just how many other facilities and release sites across Texas were connected to the CWD positive sites in Uvalde and Hunt Counties,” said Carter Smith, Executive Director of TPWD.

For Immediate Release

May 14, 2021

Chronic Wasting Disease Discovered at Deer Breeding Facilities in Matagorda and Mason Counties

AUSTIN, TX – Chronic Wasting Disease (CWD) has been discovered in deer breeding facilities in both Matagorda and Mason counties. This marks the first positive detection of the disease in each county. 

An epidemiological investigation found that both deer breeding facilities had received deer from the Uvalde County premises confirmed positive with CWD on March 29, 2021. Postmortem tissue samples were submitted by the permitted deer breeders to assist Texas Parks and Wildlife Department (TPWD) and Texas Animal Health Commission (TAHC) with the epidemiological investigation. The National Veterinary Services Laboratory (NVSL) in Ames, Iowa, has since confirmed CWD in those tissue samples.

TPWD and TAHC officials have taken immediate action to secure all cervids at the Matagorda County and Mason County deer breeding facilities and plan to conduct additional investigations for CWD. In addition, other breeding facilities and release sites that have received deer from these facilities or shipped deer to these facilities during the last five years have been contacted by TPWD and cannot move or release deer at this time.

On March 31, 2021, TPWD and TAHC reported two CWD confirmations at breeding facilities in both Hunt and Uvalde counties. The Hunt facility underwent further DNA testing to confirm animal identification and origin, and on May 12 the DNA test results confirmed the deer’s connection to the premises.

TPWD and TAHC continue to work together to determine the extent of the disease within all the affected facilities and evaluate risks to Texas’ free ranging deer populations. Quick detection of CWD can help mitigate the disease’s spread. 

“Regrettably, the gravity of this situation continues to mount with these new CWD positive discoveries, as well as with the full understanding of just how many other facilities and release sites across Texas were connected to the CWD positive sites in Uvalde and Hunt Counties,” said Carter Smith, Executive Director of TPWD. “Along with our partners at the Texas Animal Health Commission, we will continue to exercise great diligence and urgency with this ongoing investigation. Accelerating the testing at other exposed facilities will be critical in ensuring we are doing all we can to arrest the further spread of this disease, which poses great risks to our native deer populations, both captive and free-ranging alike.”

CWD was first recognized in the U.S. in 1967 and has since been documented in captive and/or free-ranging deer in 26 states and 3 Canadian provinces. 

In Texas, the disease was first discovered in 2012 in free-ranging mule deer along a remote area of the Hueco Mountains near the Texas-New Mexico border and has since been detected in 228 captive or free-ranging cervids, including white-tailed deer, mule deer, red deer and elk in 13 Texas counties. For more information on previous detections visit the CWD page on the TPWD website. CWD is a fatal neurological disease found in certain cervids, including deer, elk, moose and other members of the deer family. CWD is a slow and progressive disease. Due to a long incubation, cervids infected with CWD may not produce any visible signs for a number of years after becoming infected. As the disease progresses, animals with CWD show changes in behavior and appearance. Clinical signs may include, progressive weight loss, stumbling or tremors with a lack of coordination, excessive thirst, salivation or urination, loss of appetite, teeth grinding, abnormal head posture, and/or drooping ears. To date there is no evidence that CWD poses a risk to humans or non-cervids. However, as a precaution, the U.S. Centers for Disease Control and the World Health Organization recommend not to consume meat from infected animals. For more information about CWD, visit the TPWD web site or the TAHC web site.

###


“Regrettably, the gravity of this situation continues to mount with these new CWD positive discoveries, as well as with the full understanding of just how many other facilities and release sites across Texas were connected to the CWD positive sites in Uvalde and Hunt Counties,” said Carter Smith, Executive Director of TPWD.

TEXAS ANIMAL HEALTH COMMISSION 409 TH COMMISSION MEETING AGENDA June 29, 2021 8:30 A.M.


TEXAS CHRONIC WASTIND DISEASE CWD TSE PRION CASES JUMPS TO 228 CONFIRMED

In Texas, the disease was first discovered in 2012 in free-ranging mule deer along a remote area of the Hueco Mountains near the Texas-New Mexico border and has since been detected in 228 captive or free-ranging cervids, including white-tailed deer, mule deer, red deer and elk in 13 Texas counties. For more information on previous detections visit the CWD page on the TPWD website.


Texas Emergency Order Issued to Restrict Movement of Deer from Breeding Facilities Where CWD has Been Detected

PRICE OF CWD TSE PRION TEXAS HOLDEM POKER GOES UP $$$

Emergency Order Issued to Restrict Movement of Deer from Breeding Facilities Where CWD has Been Detected 

June 22, 2021 Media Contact: TPWD News, Business Hours, 512-389-8030 News Image Share on Facebook Share Release URL

AUSTIN – Texas Parks and Wildlife Department (TPWD) has issued an “emergency order” to impose additional movement and testing restrictions on deer breeding facilities that are affiliated with six deer breeding facilities where Chronic Wasting Disease (CWD) has been positively detected. Existing rules already restrict the movement of deer from 264 sites in 95 counties that are directly linked to these CWD-positive facilities, but further measures are necessary given the gravity of this situation.

TPWD and Texas Animal Health Commission (TAHC) are addressing risks and improving management strategies to protect big game resources from CWD in captive or free-ranging cervid populations. Both agencies recognize the need for full cooperation and partnership among government agencies, deer breeders, private landowners, hunters, conservation organizations and the general public in managing CWD in Texas.

“This is a terribly unfortunate development that we are committed to addressing as proactively, comprehensively, and expeditiously as possible,” said Carter Smith, TPWD Executive Director. “The health of our state’s free-ranging and captive deer herds, as well as affiliated hunting, wildlife, and rural based economies, are vitally important to Texas hunters, communities, and landowners. As such, our primary objectives are to enhance testing at sites that received deer from affected facilities and avoid the unintentional release of CWD-positive deer. Along with our partners at TAHC, we will continue to exercise great diligence and urgency with this ongoing investigation.”

Officials have taken action to secure all cervids at the CWD-positive facilities with plans to conduct additional investigations for CWD. In addition, those breeding facilities that received deer or shipped deer to those facilities during the last five years are under movement restrictions and cannot move or release cervids until cleared by a herd plan. The additional measures included in this emergency order include enhanced testing requirements for facilities with close epidemiological ties to the CWD-positive facilities and antemortem testing of deer from all movement qualified deer breeding facilities prior to transfer to a release site. These requirements are necessary to further minimize risk of CWD spreading into Texas’ free-ranging white-tailed deer herd, and to protect the captive deer breeding industry.

“The TAHC is committed to working with TPWD and affected stakeholders and landowners to address this latest development in Texas’ CWD history,” said Dr. Andy Schwartz, TAHC Executive Director and State Veterinarian. “The TAHC will continue to use its veterinary and epidemiological expertise to facilitate and contribute to the state’s CWD herd management and surveillance strategies.”

“The Texas Parks and Wildlife Commission and TPWD are deeply concerned about the gravity and the urgency of the CWD challenges now confronting us,” said Arch H. “Beaver” Aplin, III Chairman TPW Commission. “Please also know that the engagement and input from everyone interested in deer management will be important as we work together along with TAHC to try and arrest the spread of this insidious disease.”

“I am proud of the partnership between TAHC and TPWD and the dedication of the two agencies to address Chronic Wasting Disease in this state,” said Coleman Locke, TAHC Chairman. “Because of this collaboration, Texas has led the nation in CWD management techniques and will continue to improve the overall understanding of the disease.”

As the state veterinary diagnostic laboratory, the Texas A&M Veterinary Medical Diagnostic Laboratory (TVMDL), uses surveillance testing to help wildlife producers and wildlife/animal health agencies to ensure the health of species susceptible to CWD. With the expected increase in CWD testing, TVMDL, a state agency within the Texas A&M System, has committed additional resources to ramp up testing capacity.

“Working with TPWD, the Texas A&M System will use all of its resources to perform these tests quickly and efficiently,” said Texas A&M University System Chancellor John Sharp.

CWD was first recognized in the United States in 1967 and has since been documented in captive and/or free-ranging deer in 26 states and three Canadian provinces.

In Texas, the disease was first discovered in 2012 in free-ranging mule deer along a remote area of the Hueco Mountains near the Texas-New Mexico border and has since been detected in 228 captive or free-ranging cervids, including white-tailed deer, mule deer, red deer and elk in 13 Texas counties. For more information on previous detections visit the CWD page on the TPWD website.

CWD is a fatal neurological disease found in certain cervids, including deer, elk, moose and other members of the deer family. CWD is a slow and progressive disease. Due to a long incubation, cervids infected with CWD may not produce any visible signs for a number of years after becoming infected. As the disease progresses, animals with CWD show changes in behavior and appearance. Clinical signs may include, progressive weight loss, stumbling or tremors with a lack of coordination, excessive thirst, salivation or urination, loss of appetite, teeth grinding, abnormal head posture, and/or drooping ears.

To date there is no evidence that CWD poses a risk to humans or non-cervids. However, as a precaution, the U.S. Centers for Disease Control and the World Health Organization recommend not to consume meat from infected animals.

For more information about CWD, visit the TPWD Chronic Wasting Disease page or the TAHC web site.

A full list of TVMDL’s test offering are available on the TVMDL website and laboratory experts are available to answer any questions by calling 979-845-3414.


Emergency Order Issued to Restrict Movement of Deer from Breeding Facilities Where CWD has Been Detected 

June 22, 2021 


Official Update from the TPWD Wildlife Permit Office 

Dear Texas Deer Breeder, 

Emergency rules have been adopted in response to the discovery of CWD positive deer in 6 facilities since March 23, 2021.

As of June 21, 2021, the following regulations have been enacted and apply to all permitted deer breeding facilities:

All deer of eligible age (9 months and older) must have a “Not Detected” rectal or tonsil biopsy test result within the six month period prior to transfer to a release site.

Transferring deer to a release site without a valid ante mortem test as required is a violation that will result in immediate loss of Movement Qualified status and subject to the CWD testing requirements required by Parks and Wildlife Code §65.94(f) and may result in subsequent permit denial. All escaped deer required to be reported to TPWD under §65.605 of this title (relating to Holding Facility Standards and Care of Deer) will be considered mortalities for the purposes of this subchapter until lawfully recaptured. A deer that is not recaptured will be treated as a mortality that occurred within the facility from which the escape is required to be reported. Deer that according to department records should be present in a breeding facility but cannot be accounted for to the satisfaction of TPWD will be considered mortalities.

Additional information will follow for facilities who have been identified as Trace or Tier 1 facilities. 

If you have any questions please contact the Wildlife Permitting Office at 512-389-4585 or deer.breeder@tpwd.texas.gov. 

=====end=====

COMPREHENSIVE CWD MANAGEMENT RULES ADOPTION PREAMBLE

1. Introduction.

The Texas Parks and Wildlife Commission (Commission) in a duly noticed meeting on June 20, 2016 adopted the repeal of §§65.90-65.94 and new §§65.90-65.99 concerning Chronic Wasting Disease - Movement of Deer. New §§65.90-65.92 and 65.94-65.98 are adopted with changes to the proposed text as published in the April 22, 2016, issue of the Texas Register (41 TexReg 2853). The repeals, new §65.93, and new §65.99 are adopted without changes and will not be republished.

Change to Definitions

The change to §65.90, concerning Definitions, adds a definition for “Interim Breeder Rules.” The rules as adopted, include provisions predicated upon compliance with previous rules. Therefore, a definition of “Interim Breeder Rules” was added to provide a shorthand reference for those rules.

Changes to General Provisions

The change to §65.91, concerning General Provisions, alters subsection (a). As proposed, the subsection provided that to the extent any provision of the proposed new rules conflicted with any other provision of Chapter 65, the new rules would control; however, Chapter 65, Subchapter B, Division 1 contains provisions regarding Chronic Wasting Disease (CWD) management zones that are intended to function on their own terms. Therefore, it is necessary to clarify that fact.

The change to §65.91 also adds new subsection (d) to clarify that a deer breeding facility is prohibited from moving deer out of the breeding facility if such movement is prohibited under a hold order or quarantine imposed on the breeding facility by the Texas Animal Health Commission (TAHC). Under the rules as proposed, and as adopted, the lawful movement of breeder deer is predicated on a facility’s designation as Movement Qualified (MQ). Although the provisions of §65.99 as adopted, concerning Violations and Penalties, provide that a person who possesses or receives white-tailed deer or mule deer pursuant to a Triple T permit, DMP or 

2

a deer breeder permit is subject to the provisions of TAHC regulations regarding Chronic Wasting Disease, subsection (d) was clarified to state that if a facility is prohibited from moving deer under a hold order or quarantine issued by TAHC, movement of deer under those circumstances is prohibited.

In addition, a change was made to §65.91(e) (which was §65.91(d) as proposed) to modify the cross-reference to the subsection regarding receipt of deer by a release site. The rules as adopted adjust the time period for release site testing. As a result, it is more appropriate to reference the entirety of §65.95(c) regarding release sites, rather than the more specific §65.95(c)(1)(D).

Changes to CWD Testing

The change to §65.92, concerning CWD Testing, clarifies who may collect the tissue upon which ante-mortem tests are to be conducted and provides additional detail to ensure that a valid sample is collected. As proposed, §65.92(b) required ante-mortem test samples be collected “by or under the supervision of a qualified licensed veterinarian.” However, to ensure compliance with statutory requirements regarding the practice of veterinary medicine, as well as regulatory requirements of TAHC and the Texas Board of Veterinary Medical Examiners regarding the collection of ante-mortem samples, the change to §65.92(b) provides that antemortem samples must be collected by a “licensed veterinarian authorized pursuant to statutes and regulations governing the practice of veterinary medicine in Texas and regulations of the TAHC.” In addition, to ensure that samples are sufficient to accommodate ante-mortem testing, §95.92(b) was modified to require that at least six lymphoid follicles be collected.

The change to §65.92(b) also eliminates the 16-month residency requirement for antemortem testing of breeder deer imposed by proposed subsection (b)(2). The intent of proposed subsection (b)(2) was to ensure that animals subjected to ante-mortem testing had been in a facility long enough to have contracted CWD if it were present. However, while a deer with a residency in a facility of less than 16 months may not have had sufficient incubation time to detect CWD if it was contracted in that facility, a test of that deer would provide information about any previous facility in which the deer was held. Therefore, the residency requirement was eliminated.

snip...see full text;


TUESDAY, MARCH 02, 2021
Texas Confirms CWD TSE Prion in 213 white-tailed deer, mule deer, red deer and elk to date, 148 connected to deer breeding facilities and release sites

FRIDAY, JANUARY 22, 2021 

Wisconsin DNR CONFIRMS CWD IN WOOD COUNTY WILD DEER; RENEWS BAITING AND FEEDING BANS


THURSDAY, DECEMBER 17, 2020 

Wisconsin DNR CONFIRMS CWD DETECTED IN WASHINGTON COUNTY; NEW BAITING AND FEEDING BAN NOW FOR OZAUKEE COUNTY 


SUNDAY, SEPTEMBER 20, 2020 

Wisconsin Sinks Further Into the Abyss With CWD TSE Prion 2020


TUESDAY, JUNE 09, 2020 

Wisconsin Trempealeau County Deer Farm Tests Positive for CWD ​Release Date: June 9, 2020


MONDAY, JUNE 01, 2020 

Wisconsin CWD TSE Prion Continues to Spiral Out of Control, 6585 Cases Confirmed to Date in Wild, and it's anyone's guess for captive


WEDNESDAY, FEBRUARY 05, 2020

Wisconsin CWD TSE Prion 2019 to date wild deer 1317 positive and Captive Farmed Livestock Cervid CWD update

Minnesota CWD TSE Prion

Temporary ban on movement of farmed deer after CWD discovered in a deer at Beltrami County farm

On June 1, the DNR issued an emergency rule temporarily prohibiting the movement of all farmed white-tailed deer within the state, with the exception of deer being transported to slaughter.
The rule, which ends July 31, is a temporary, emergency action to reduce further spread of chronic wasting disease and protect Minnesota’s wild deer. The action is in response to concerning developments following the discovery of CWD in white-tailed deer at a farm in Beltrami County in northern Minnesota. The emergency rule will provide time to examine and respond to connections between this farm and other potentially exposed farms throughout the state.
The Minnesota Board of Animal Health confirmed on May 25 that a total of 13 deer tested positive for CWD in a herd of 55 deer. The initial detection was confirmed on April 7, 2021. Deer remains from the farm were also discovered on adjacent, county-managed land; testing conducted by the University of Minnesota’s Center for Prion Research and Outreach indicated the presence of CWD-causing prions in at least one of the bones found there.
Due to the detection, the DNR will conduct precautionary surveillance in the area surrounding the Beltrami County farm to determine if nearby wild deer are infected with CWD. Surveillance will begin with this fall’s hunting season and last a minimum of three consecutive years.
The DNR encourages Beltrami County residents to report observations of sick wild deer to their local wildlife office by calling 218-732-8452. A deer feeding ban will also be put in place in Beltrami and surrounding counties.
WEDNESDAY, MAY 19, 2021 

***> U of M testing finds presence of CWD prions at Beltrami County carcass dump site <***


FRIDAY, JUNE 25, 2021 

Minnesota Legislature a Threat For Wild Cervid, Fumbles Football Again With Farmed CWD TSE Prion


FRIDAY, JUNE 11, 2021 

Minnesota Deer farming drives predicament over CWD-infested dump site on public land


TUESDAY, JUNE 01, 2021 

Minnesota DNR to protect wild deer health through temporary ban on movement of farmed deer


TUESDAY, MAY 25, 2021 

Minnesota Twelve additional white-tailed deer tested positive for Chronic Wasting Disease (CWD) in the infected Beltrami County farmed deer herd


MONDAY, FEBRUARY 22, 2021 

Minnesota Nine more deer added to tally of CWD positive whitetails at Houston County farm


WEDNESDAY, APRIL 07, 2021 

Minnesota 3-year-old white-tailed doe at a Beltrami County farm has been confirmed CWD positive


MONDAY, FEBRUARY 01, 2021 

Minnesota 2020 hunting season and early 2021 special hunts confirmed CWD TSE Prion in 22 wild deer

Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004 Singeltary Submission
Greetings APHIS et al, i would kindly like to comment on Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004.
Greetings APHIS et al, i would kindly like to comment on Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004.
***> 1st and foremost your biggest problem is 'VOLUNTARY'! AS with the BSE 589.2001 FEED REGULATIONS, especially since it is still voluntary with cervid, knowing full well that cwd and scrapie will transmit to pigs by oral route. VOLUNTARY DOES NOT WORK! all animal products should be banned and be made mandatory, and the herd certification program should be mandatory, or you don't move cervid. IF THE CWD HERD CERTIFICATION IS NOT MANDATORY, it will be another colossal tse prion failure from the start. 
***> 2nd USA should declare a Declaration of Extraordinary Emergency due to CWD, and all exports of cervid and cervid products must be stopped internationally, and there should be a ban of interstate movement of cervid, until a live cwd test is available. 
***> 3rd Captive Farmed cervid ESCAPEES should be made mandatory to report immediately, and strict regulations for those suspect cwd deer that just happen to disappear. IF a cervid escapes and is not found, that farm should be indefinitely shut down, all movement, until aid MIA cervid is found, and if not ever found, that farm shut down permanently. 
***> 4th Captive Farmed Cervid, INDEMNITY, NO MORE Federal indemnity program, or what i call, ENTITLEMENT PROGRAM for game farm industry. NO MORE BAIL OUTS FROM TAX PAYERS. if the captive industry can't buy insurance to protect not only themselves, but also their customers, and especially the STATE, from Chronic Wasting Disease CWD TSE Prion or what some call mad deer disease and harm therefrom, IF they can't afford to buy that insurance that will cover all of it, then they DO NOT GET A PERMIT to have a game farm for anything. This CWD TSE Prion can/could/has caused property values to fall from some reports in some places. roll the dice, how much is a state willing to lose?
***> 5th QUARANTINE OF ALL FARMED CAPTIVE, BREEDERS, URINE, ANTLER, VELVET, SPERM, OR ANY FACILITY, AND THEIR PRODUCTS, that has been confirmed to have Chronic Wasting Disease CWD TSE Prion, the QUARANTINE should be for 21 years due to science showing what scrapie can do. 5 years is NOT near long enough. see; Infectious agent of sheep scrapie may persist in the environment for at least 16 to 21 years.
***> 6th America BSE 589.2001 FEED REGULATIONS CWD TSE Prion
***> 7TH TRUCKING TRANSPORTING CERVID CHRONIC WASTING DISEASE TSE PRION VIOLATING THE LACEY ACT
***> 8TH ALL CAPTIVE FARMING CERVID OPERATIONS MUST BE INSURED TO PAY FOR ANY CLEAN UP OF CWD AND QUARANTINE THERE FROM FOR THE STATE, NO MORE ENTITLEMENT PROGRAM FOR CERVID GAME FARMING PAY TO PLAY FOR CWD TSE PRION OFF THE TAX PAYERS BACK.
***> 9TH ANY STATE WITH DOCUMENTED CWD, INTERSTATE, NATIONAL, AND INTERNATIONAL MOVEMENT OF ALL CERVID, AND ALL CERVID PRODUCTS MUST BE HALTED!
***> 10TH BAN THE SALE OF STRAW BRED BUCKS AND ALL CERVID SEMEN AND URINE PRODUCTS
***> 11th ALL CAPTIVE FARMED CERVID AND THEIR PRODUCTS MUST BE CWD TSE PRION TESTED ANNUALLY AND BEFORE SALE FOR CWD TSE PRION
Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004 Singeltary Submission
Comment ID
APHIS-2021-0004-0002
Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004 Singeltary Submission
Subject: Chronic Wasting Disease Outbreak in Texas Deer Breeding Facilities Could Be a Major Issue for All Deer Hunters

SAD DAY for Texas, sad day indeed...we knew it was coming...i call this ;

CWD TSE PRION TEXAS HOLDEM, PRICE OF POKER COMES BACK TO HAUNT US, just plain stupidity and greed, just follow the money$$$

can't say i did not try...terry

Colorado Chronic Wasting Disease Response Plan December 2018

I. Executive Summary Mule deer, white-tailed deer, elk and moose are highly valued species in North America. Some of Colorado’s herds of these species are increasingly becoming infected with chronic wasting disease (CWD). As of July 2018, at least 31 of Colorado's 54 deer herds (57%), 16 of 43 elk herds (37%), and 2 of 9 moose herds (22%) are known to be infected with CWD. Four of Colorado's 5 largest deer herds and 2 of the state’s 5 largest elk herds are infected. Deer herds tend to be more heavily infected than elk and moose herds living in the same geographic area. Not only are the number of infected herds increasing, the past 15 years of disease trends generally show an increase in the proportion of infected animals within herds as well. Of most concern, greater than a 10-fold increase in CWD prevalence has been estimated in some mule deer herds since the early 2000s; CWD is now adversely affecting the performance of these herds.

snip...

(the map on page 71, cwd marked in red, is shocking...tss)


CWD Advisory Group


SATURDAY, FEBRUARY 01, 2020

Colorado confirmed CWD TSE Prion in 24 game management units in the state where it previously hadn’t been found

Feb 1, 2020 Feb 1, 2020 46 min ago 

snip...see full text;


Chronic Wasting Disease Drives Population Decline of White-Tailed Deer 

David R. Edmunds ,Matthew J. Kauffman,Brant A. Schumaker,Frederick G. Lindzey,Walter E. Cook,Terry J. Kreeger,Ronald G. Grogan,Todd E. Cornish Published: August 30, 2016 https://doi.org/10.1371/journal.pone.0161127

This population highlights the potential long-term negative outcome of endemic CWD to population sustainability and stresses the importance of preventing CWD from becoming endemic in a population, rather than attempting to manage it after the fact. Therefore, as previously suggested [43], the best management strategy remains minimizing movement of CWD to new areas.


PLoS One. 2017; 12(10): e0186512. Published online 2017 Oct 19. doi: 10.1371/journal.pone.0186512 PMCID: PMC5648191 PMID: 29049389

Endemic chronic wasting disease causes mule deer population decline in Wyoming


Implications of farmed-cervid movements on the transmission of chronic wasting disease Cite


ABSTRACT

Chronic wasting disease is a transmissible spongiform encephalopathy that affects cervids with a clinical picture of muscle wasting in infected animals. The objective of this study was to quantify movement patterns of farmed cervids in the state of Minnesota as a model for identifying potential disease mitigation points. Time aggregated network analysis was performed on data consisting of 1221 intra-state cervid movements from farms located within Minnesota (n = 432 farms). Intra-state movements accounted for 48.2 % of all documented movements (2578) in Minnesota from 2013 to 2018; the remaining movements were inter-state. Annual networks were sparse in nature with low graph densities (6.9 × 10−4 - 1.4 × 10-3) and transitivity (0.06−0.12). Frequency of movements increased significantly (p < 0.05) in the months of September and October before decreasing in November, which coincided with the breeding and hunting seasons. Some of these contacts were as far as 500 km apart. The median length of infection chains for CWD positive farms was estimated to be 5.0 and 6.0 farms in-and out-going infection chains, respectively. A k-test analysis demonstrated that the observed median number of infected farms directly connected to other infected farms was 2.0, which was significantly higher than a fortuitous event (p = 0.002). Movements of cervids between farms were largely unpredictable with very low edge overlap (mean 0.02 %) from year to year, suggesting that persistent commercial relationships among farms were rare. 

In conclusion, long distance trade movements present a risk for spread of chronic wasting disease in Minnesota. The sparse networks and unpredictable farm contacts could be because cervid production is not as vertically integrated as other species-differentiated and established industries, such as swine or poultry. Our analytical approach can be used to understand chronic wasting disease in other states in the U.S. and North America in general. 


***> This is very likely to have parallels with control efforts for CWD in cervids.

Paper

Rapid recontamination of a farm building occurs after attempted prion removal

Kevin Christopher Gough BSc (Hons), PhD Claire Alison Baker BSc (Hons) Steve Hawkins MIBiol Hugh Simmons BVSc, MRCVS, MBA, MA Timm Konold DrMedVet, PhD, MRCVS … See all authors 

First published: 19 January 2019 https://doi.org/10.1136/vr.105054

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This study clearly demonstrates the difficulty in removing scrapie infectivity from the farm environment. Practical and effective prion decontamination methods are still urgently required for decontamination of scrapie infectivity from farms that have had cases of scrapie and this is particularly relevant for scrapiepositive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.

https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054

***>This is very likely to have parallels with control efforts for CWD in cervids.





***> Infectious agent of sheep scrapie may persist in the environment for at least 16 years

***> Nine of these recurrences occurred 14–21 years after culling, apparently as the result of environmental contamination, but outside entry could not always be absolutely excluded. 

JOURNAL OF GENERAL VIROLOGY Volume 87, Issue 12

Infectious agent of sheep scrapie may persist in the environment for at least 16 years Free

Gudmundur Georgsson1, Sigurdur Sigurdarson2, Paul Brown3

http://www.microbiologyresearch.org/docserver/fulltext/jgv/87/12/3737.pdf?expires=1540908280&id=id&accname=guest&checksum=ED0572E1E5B272C100A32212A3E3761A

Circulation of prions within dust on a scrapie affected farm

Kevin C Gough1 , Claire A Baker2 , Hugh A Simmons3 , Steve A Hawkins3 and Ben C Maddison2*

The sources of dust borne prions are unknown but it seems reasonable to assume that faecal, urine, skin, parturient material and saliva-derived prions may contribute to this mobile environmental reservoir of infectivity. This work highlights a possible transmission route for scrapie within the farm environment, and this is likely to be paralleled in CWD which shows strong similarities with scrapie in terms of prion dissemination and disease transmission. The data indicate that the presence of scrapie prions in dust is likely to make the control of these diseases a considerable challenge.


Texas Kimble County Farm Chronic Wasting Disease CWD TSE Prion Approximate Herd Prevalence 12%

SUMMARY MINUTES OF THE 407th COMMISSION MEETING Texas Animal Health Commission

September 22, 2020 

Chronic Wasting Disease (CWD):

A new CWD positive breeding herd was disclosed in February 2020 in Kimble County. This herd depopulation was completed in July 2020. Including the two index positive deer, an additional eight more positive deer were disclosed (approximate herd prevalence 12%). Since July 2015 and prior to this discovery, five positive captive breeder herds have been disclosed and four of those are in Medina County. One herd in Lavaca and three herds in Medina County were depopulated leaving one large herd in Medina County that is managed on a herd plan. A new zone was established in Val Verde County in December 2019 as a result of a positive free-ranging White-tailed Deer (WTD). A second positive WTD was also disclosed in February 2020 in the same area. 


For Immediate Release 

Thursday, October 2, 2014 

Dustin Vande Hoef 515/281-3375 or 515/326-1616 (cell) or Dustin.VandeHoef@IowaAgriculture.gov 

TEST RESULTS FROM CAPTIVE DEER HERD WITH CHRONIC WASTING DISEASE RELEASED 79.8 percent of the deer tested positive for the disease

DES MOINES – The Iowa Department of Agriculture and Land Stewardship today announced that the test results from the depopulation of a quarantined captive deer herd in north-central Iowa showed that 284 of the 356 deer, or 79.8% of the herd, tested positive for Chronic Wasting Disease (CWD). The owners of the quarantined herd have entered into a fence maintenance agreement with the Iowa Department of Agriculture and Land Stewardship, which requires the owners to maintain the 8’ foot perimeter fence around the herd premises for five years after the depopulation was complete and the premises had been cleaned and disinfected

CWD is a progressive, fatal, degenerative neurological disease of farmed and free-ranging deer, elk, and moose. There is no known treatment or vaccine for CWD. CWD is not a disease that affects humans.

On July 18, 2012, USDA Animal and Plant Health Inspection Service’s (APHIS) National Veterinary Services Lab in Ames, IA confirmed that a male white tail deer harvested from a hunting preserve in southeast IA was positive for CWD. An investigation revealed that this animal had just been introduced into the hunting preserve from the above-referenced captive deer herd in north-central Iowa.

The captive deer herd was immediately quarantined to prevent the spread of CWD. The herd has remained in quarantine until its depopulation on August 25 to 27, 2014.

The Iowa Department of Agriculture and Land Stewardship participated in a joint operation to depopulate the infected herd with USDA Veterinary Services, which was the lead agency, and USDA Wildlife Services.

Federal indemnity funding became available in 2014. USDA APHIS appraised the captive deer herd of 376 animals at that time, which was before depopulation and testing, at $1,354,250. At that time a herd plan was developed with the owners and officials from USDA and the Iowa Department of Agriculture and Land Stewardship.

Once the depopulation was complete and the premises had been cleaned and disinfected, indemnity of $917,100.00 from the USDA has been or will be paid to the owners as compensation for the 356 captive deer depopulated.

The Iowa Department of Agriculture and Land Stewardship operates a voluntary CWD program for farms that sell live animals. Currently 145 Iowa farms participate in the voluntary program. The above-referenced captive deer facility left the voluntary CWD program prior to the discovery of the disease as they had stopped selling live animals. All deer harvested in a hunting preserve must be tested for CWD.

-30-


Wisconsin Buckhorn Flats CWD

SUBJECT: Almond Deer Farm Update

The first case of Chronic Wasting Disease (CWD) among Wisconsin's farm-raised deer occurred in a white-tailed deer buck shot by a hunter at the property (formerly known as Buckhorn Flats) in September 2002. This situation prompted the eventual depopulation of the entire farm. 

The deer, a mix of does and yearlings, were destroyed on January 17, 2006- 4 years later- by U.S. Department of Agriculture shooters under a USDA agreement with the farm owner. 

Sixty of the 76 animals tested positive for CWD. The 76 deer constituted the breeding herd in the breeding facility on the farm. The property also had a hunting preserve until 2005. Four deer, two does and two fawns, the only deer remaining in the former preserve, were killed and tested as well. CWD was not detected in those animals. 

The total number of deer to test positive from this farm from the initial discovery to final depopulation is 82. The nearly 80% prevalence rate discovered on Buckhorn Flats is the highest prevalence recorded in any captive cervid operation in North America.

Tuesday, December 20, 2011

Chronic Wasting Disease CWD WISCONSIN Almond Deer

(Buckhorn Flats) Farm Update DECEMBER 2011 The CWD infection rate was nearly 80%, the highest ever in a North American captive herd. RECOMMENDATION: That the Board approves the purchase of 80 acres of land for $465,000 for the Statewide Wildlife Habitat Program in Portage County and approve the restrictions on public use of the site.

Form 1100-001 (R 2/11) NATURAL RESOURCES BOARD AGENDA ITEM SUBJECT: Information Item: Almond Deer Farm Update FOR:

DECEMBER 2011 BOARD MEETING

TUESDAY TO BE PRESENTED BY TITLE: Tami Ryan, Wildlife Health Section Chief SUMMARY:





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 

http://www.pnas.org/content/97/7/3418.full

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

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/

MONDAY, APRIL 19, 2021

Evaluation of the application for new alternative biodiesel production process for rendered fat including Category 1 animal by-products (BDI-RepCat® process, AT) ???

https://transmissiblespongiformencephalopathy.blogspot.com/2021/04/evaluation-of-application-for-new.html

Detection of protease-resistant cervid prion protein in water from a CWD-endemic area 

https://www.ncbi...nlm.nih.gov/pmc/articles/PMC2802782/pdf/prion0303_0171.pdf

A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing 

http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract

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 

https://bmcvetres.biomedcentral.com/track/pdf/10.1186/1746-6148-9-134.pdf

THURSDAY, FEBRUARY 28, 2019 

BSE infectivity survives burial for five years with only limited spread


***> CONGRESSIONAL ABSTRACTS PRION CONFERENCE 2018

P69 Experimental transmission of CWD from white-tailed deer to co-housed reindeer 

Mitchell G (1), Walther I (1), Staskevicius A (1), Soutyrine A (1), Balachandran A (1) 

(1) National & OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario, Canada. 

Chronic wasting disease (CWD) continues to be detected in wild and farmed cervid populations of North America, affecting predominantly white-tailed deer, mule deer and elk. Extensive herds of wild caribou exist in northern regions of Canada, although surveillance has not detected the presence of CWD in this population. Oral experimental transmission has demonstrated that reindeer, a species closely related to caribou, are susceptible to CWD. Recently, CWD was detected for the first time in Europe, in wild Norwegian reindeer, advancing the possibility that caribou in North America could also become infected. Given the potential overlap in habitat between wild CWD-infected cervids and wild caribou herds in Canada, we sought to investigate the horizontal transmissibility of CWD from white-tailed deer to reindeer. 

Two white-tailed deer were orally inoculated with a brain homogenate prepared from a farmed Canadian white-tailed deer previously diagnosed with CWD. Two reindeer, with no history of exposure to CWD, were housed in the same enclosure as the white-tailed deer, 3.5 months after the deer were orally inoculated. The white-tailed deer developed clinical signs consistent with CWD beginning at 15.2 and 21 months post-inoculation (mpi), and were euthanized at 18.7 and 23.1 mpi, respectively. Confirmatory testing by immunohistochemistry (IHC) and western blot demonstrated widespread aggregates of pathological prion protein (PrPCWD) in the central nervous system and lymphoid tissues of both inoculated white-tailed deer. Both reindeer were subjected to recto-anal mucosal associated lymphoid tissue (RAMALT) biopsy at 20 months post-exposure (mpe) to the white-tailed deer. The biopsy from one reindeer contained PrPCWD confirmed by IHC. This reindeer displayed only subtle clinical evidence of disease prior to a rapid decline in condition requiring euthanasia at 22.5 mpe. Analysis of tissues from this reindeer by IHC revealed widespread PrPCWD deposition, predominantly in central nervous system and lymphoreticular tissues. Western blot molecular profiles were similar between both orally inoculated white-tailed deer and the CWD positive reindeer. Despite sharing the same enclosure, the other reindeer was RAMALT negative at 20 mpe, and PrPCWD was not detected in brainstem and lymphoid tissues following necropsy at 35 mpe. Sequencing of the prion protein gene from both reindeer revealed differences at several codons, which may have influenced susceptibility to infection. 

Natural transmission of CWD occurs relatively efficiently amongst cervids, supporting the expanding geographic distribution of disease and the potential for transmission to previously naive populations. The efficient horizontal transmission of CWD from white-tailed deer to reindeer observed here highlights the potential for reindeer to become infected if exposed to other cervids or environments infected with CWD. 

SOURCE REFERENCE 2018 PRION CONFERENCE ABSTRACT

Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Location: Virus and Prion Research

Title: Horizontal transmission of chronic wasting disease in reindeer

Author

item MOORE, SARAH - ORISE FELLOW item KUNKLE, ROBERT item WEST GREENLEE, MARY - IOWA STATE UNIVERSITY item Nicholson, Eric item RICHT, JUERGEN item HAMIR, AMIRALI item WATERS, WADE item Greenlee, Justin

Submitted to: Emerging Infectious Diseases

Publication Type: Peer Reviewed Journal

Publication Acceptance Date: 8/29/2016

Publication Date: 12/1/2016

Citation: Moore, S., Kunkle, R., Greenlee, M., Nicholson, E., Richt, J., Hamir, A., Waters, W., Greenlee, J. 2016. Horizontal transmission of chronic wasting disease in reindeer. Emerging Infectious Diseases. 22(12):2142-2145. doi:10.3201/eid2212.160635.

Interpretive Summary: Chronic wasting disease (CWD) is a fatal neurodegenerative disease that occurs in farmed and wild cervids (deer and elk) of North America and was recently diagnosed in a single free-ranging reindeer (Rangifer tarandus tarandus) in Norway. CWD is a transmissible spongiform encephalopathy (TSE) that is caused by infectious proteins called prions that are resistant to various methods of decontamination and environmental degradation. Little is known about the susceptibility of or potential for transmission amongst reindeer. In this experiment, we tested the susceptibility of reindeer to CWD from various sources (elk, mule deer, or white-tailed deer) after intracranial inoculation and tested the potential for infected reindeer to transmit to non-inoculated animals by co-housing or housing in adjacent pens. Reindeer were susceptible to CWD from elk, mule deer, or white-tailed deer sources after experimental inoculation. Most importantly, non-inoculated reindeer that were co-housed with infected reindeer or housed in pens adjacent to infected reindeer but without the potential for nose-to-nose contact also developed evidence of CWD infection. This is a major new finding that may have a great impact on the recently diagnosed case of CWD in the only remaining free-ranging reindeer population in Europe as our findings imply that horizontal transmission to other reindeer within that herd has already occurred. Further, this information will help regulatory and wildlife officials developing plans to reduce or eliminate CWD and cervid farmers that want to ensure that their herd remains CWD-free, but were previously unsure of the potential for reindeer to transmit CWD.

Technical Abstract: Chronic wasting disease (CWD) is a naturally-occurring, fatal prion disease of cervids. Reindeer (Rangifer tarandus tarandus) are susceptible to CWD following oral challenge, and CWD was recently reported in a free-ranging reindeer of Norway. Potential contact between CWD-affected cervids and Rangifer species that are free-ranging or co-housed on farms presents a potential risk of CWD transmission. The aims of this study were to 1) investigate the transmission of CWD from white-tailed deer (Odocoileus virginianus; CWDwtd), mule deer (Odocoileus hemionus; CWDmd), or elk (Cervus elaphus nelsoni; CWDelk) to reindeer via the intracranial route, and 2) to assess for direct and indirect horizontal transmission to non-inoculated sentinels. Three groups of 5 reindeer fawns were challenged intracranially with CWDwtd, CWDmd, or CWDelk. Two years after challenge of inoculated reindeer, non-inoculated negative control reindeer were introduced into the same pen as the CWDwtd inoculated reindeer (direct contact; n=4) or into a pen adjacent to the CWDmd inoculated reindeer (indirect contact; n=2). Experimentally inoculated reindeer were allowed to develop clinical disease. At death/euthanasia a complete necropsy examination was performed, including immunohistochemical testing of tissues for disease-associated CWD prion protein (PrPcwd). Intracranially challenged reindeer developed clinical disease from 21 months post-inoculation (months PI). PrPcwd was detected in 5 out of 6 sentinel reindeer although only 2 out of 6 developed clinical disease during the study period (< 57 months PI). We have shown that reindeer are susceptible to CWD from various cervid sources and can transmit CWD to naïve reindeer both directly and indirectly.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=328261

Infectivity surviving ashing to 600*C is (in my opinion) degradable but infective. based on Bown & Gajdusek, (1991), landfill and burial may be assumed to have a reduction factor of 98% (i.e. a factor of 50) over 3 years. CJD-infected brain-tissue remained infectious after storing at room-temperature for 22 months (Tateishi et al, 1988). Scrapie agent is known to remain viable after at least 30 months of desiccation (Wilson et al, 1950). and pastures that had been grazed by scrapie-infected sheep still appeared to be contaminated with scrapie agent three years after they were last occupied by sheep (Palsson, 1979).

http://europa.eu.int/comm/food/fs/sc/ssc/out58_en.pdf

Dr. Paul Brown Scrapie Soil Test BSE Inquiry Document

https://web.archive.org/web/20090505211734/http://www.bseinquiry.gov.uk/files/sc/Seac07/tab03.pdf

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. 

source reference Prion Conference 2015 abstract book

Grass Plants Bind, Retain, Uptake, and Transport Infectious Prions

Sandra Pritzkow,1 Rodrigo Morales,1 Fabio Moda,1,3 Uffaf Khan,1 Glenn C. Telling,2 Edward Hoover,2 and Claudio Soto1, * 1Mitchell Center for Alzheimer’s Disease and Related Brain Disorders, Department of Neurology, University of Texas Medical School at Houston, 6431 Fannin Street, Houston, TX 77030, USA

2Prion Research Center, Department of Microbiology, Immunology, and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO 80523, USA

3Present address: IRCCS Foundation Carlo Besta Neurological Institute, 20133 Milan, Italy *Correspondence: claudio.soto@uth.tmc.edu http://dx.doi.org/10.1016/j.celrep.2015.04.036

SUMMARY

Prions are the protein-based infectious agents responsible for prion diseases. Environmental prion contamination has been implicated in disease transmission. Here, we analyzed the binding and retention of infectious prion protein (PrPSc) to plants. Small quantities of PrPSc contained in diluted brain homogenate or in excretory materials (urine and feces) can bind to wheat grass roots and leaves. Wild-type hamsters were efficiently infected by ingestion of prion-contaminated plants. The prion-plant interaction occurs with prions from diverse origins, including chronic wasting disease. Furthermore, leaves contaminated by spraying with a prion-containing preparation retained PrPSc for several weeks in the living plant. Finally, plants can uptake prions from contaminated soil and transport them to aerial parts of the plant (stem and leaves). These findings demonstrate that plants can efficiently bind infectious prions and act as carriers of infectivity, suggesting a possible role of environmental prion contamination in the horizontal transmission of the disease.

INTRODUCTION

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DISCUSSION

This study shows that plants can efficiently bind prions contained in brain extracts from diverse prion infected animals, including CWD-affected cervids. PrPSc attached to leaves and roots from wheat grass plants remains capable of seeding prion replication in vitro. Surprisingly, the small quantity of PrPSc naturally excreted in urine and feces from sick hamster or cervids was enough to efficiently contaminate plant tissue. Indeed, our results suggest that the majority of excreted PrPSc is efficiently captured by plants’ leaves and roots. Moreover, leaves can be contaminated by spraying them with a prion-containing extract, and PrPSc remains detectable in living plants for as long as the study was performed (several weeks). Remarkably, prion contaminated plants transmit prion disease to animals upon ingestion, producing a 100% attack rate and incubation periods not substantially longer than direct oral administration of sick brain homogenates.

Finally, an unexpected but exciting result was that plants were able to uptake prions from contaminated soil and transport them to aerial parts of the plant tissue. Although it may seem farfetched that plants can uptake proteins from the soil and transport it to the parts above the ground, there are already published reports of this phenomenon (McLaren et al., 1960; Jensen and McLaren, 1960;Paungfoo-Lonhienne et al., 2008). The high resistance of prions to degradation and their ability to efficiently cross biological barriers may play a role in this process. The mechanism by which plants bind, retain, uptake, and transport prions is unknown. We are currently studying the way in which prions interact with plants using purified, radioactively labeled PrPSc to determine specificity of the interaction, association constant, reversibility, saturation, movement, etc.

Epidemiological studies have shown numerous instances of scrapie or CWD recurrence upon reintroduction of animals on pastures previously exposed to prion-infected animals. Indeed, reappearance of scrapie has been documented following fallow periods of up to 16 years (Georgsson et al., 2006), and pastures were shown to retain infectious CWD prions for at least 2 years after exposure (Miller et al., 2004). It is likely that the environmentally mediated transmission of prion diseases depends upon the interaction of prions with diverse elements, including soil, water, environmental surfaces, various invertebrate animals, and plants.

However, since plants are such an important component of the environment and also a major source of food for many animal species, including humans, our results may have far-reaching implications for animal and human health. Currently, the perception of the riskfor animal-to-human prion transmission has beenmostly limited to consumption or exposure to contaminated meat; our results indicate that plants might also be an important vector of transmission that needs to be considered in risk assessment. 

https://www.cell.com/cell-reports/pdf/S2211-1247%2815%2900437-4.pdf

RIGINAL RESEARCH ARTICLE

Front. Vet. Sci., 14 September 2015 | https://doi.org/10.3389/fvets.2015.00032

Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission

imageTimm Konold1*, imageStephen A. C. Hawkins2, imageLisa C. Thurston3, imageBen C. Maddison4, imageKevin C. Gough5, imageAnthony Duarte1 and imageHugh A. Simmons1

1Animal Sciences Unit, Animal and Plant Health Agency Weybridge, Addlestone, UK

2Pathology Department, Animal and Plant Health Agency Weybridge, Addlestone, UK

3Surveillance and Laboratory Services, Animal and Plant Health Agency Penrith, Penrith, UK

4ADAS UK, School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK

5School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK

Classical scrapie is an environmentally transmissible prion disease of sheep and goats. Prions can persist and remain potentially infectious in the environment for many years and thus pose a risk of infecting animals after re-stocking. In vitro studies using serial protein misfolding cyclic amplification (sPMCA) have suggested that objects on a scrapie-affected sheep farm could contribute to disease transmission. This in vivo study aimed to determine the role of field furniture (water troughs, feeding troughs, fencing, and other objects that sheep may rub against) used by a scrapie-infected sheep flock as a vector for disease transmission to scrapie-free lambs with the prion protein genotype VRQ/VRQ, which is associated with high susceptibility to classical scrapie. When the field furniture was placed in clean accommodation, sheep became infected when exposed to either a water trough (four out of five) or to objects used for rubbing (four out of seven). This field furniture had been used by the scrapie-infected flock 8 weeks earlier and had previously been shown to harbor scrapie prions by sPMCA. Sheep also became infected (20 out of 23) through exposure to contaminated field furniture placed within pasture not used by scrapie-infected sheep for 40 months, even though swabs from this furniture tested negative by PMCA. This infection rate decreased (1 out of 12) on the same paddock after replacement with clean field furniture. Twelve grazing sheep exposed to field furniture not in contact with scrapie-infected sheep for 18 months remained scrapie free. The findings of this study highlight the role of field furniture used by scrapie-infected sheep to act as a reservoir for disease re-introduction although infectivity declines considerably if the field furniture has not been in contact with scrapie-infected sheep for several months. PMCA may not be as sensitive as VRQ/VRQ sheep to test for environmental contamination.

snip...

Discussion 

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 

http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full

FRIDAY, APRIL 30, 2021 

Should Property Evaluations Contain Scrapie, CWD, TSE PRION Environmental Contamination of the land?

***> Confidential!!!!

***> 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


WEDNESDAY, DECEMBER 04, 2013 

Chronic Wasting Disease CWD and Land Value concerns? 


Chemical Inactivation of Prions Is Altered by Binding to the Soil Mineral Montmorillonite

Clarissa J. Booth, Stuart Siegfried Lichtenberg, Richard J. Chappell, and Joel A. Pedersen* Cite this: ACS Infect. Dis. 2021, XXXX, XXX, XXX-XXX Publication Date:March 31, 2021 https://doi.org/10.1021/acsinfecdis.0c00860 © 2021 American Chemical Society

Abstract

Environmental routes of transmission contribute to the spread of the prion diseases chronic wasting disease of deer and elk and scrapie of sheep and goats. Prions can persist in soils and other environmental matrices and remain infectious for years. Prions bind avidly to the common soil mineral montmorillonite, and such binding can dramatically increase oral disease transmission. Decontamination of soil in captive facilities and natural habitats requires inactivation agents that are effective when prions are bound to soil microparticles. Here, we investigate the inactivation of free and montmorillonite-bound prions with sodium hydroxide, acidic pH, Environ LpH, and sodium hypochlorite. Immunoblotting and bioassays confirm that sodium hydroxide and sodium hypochlorite are effective for prion deactivation, although montmorillonite appears to reduce the efficacy of hypochlorite. Acidic conditions slightly reduce prion infectivity, and the acidic phenolic disinfectant Environ LpH produces slight reductions in infectivity and immunoreactivity. The extent to which the association with montmorillonite protects prions from chemical inactivation appears influenced by the effect of chemical agents on the clay structure and surface pH. When clay morphology remains relatively unaltered, as when exposed to hypochlorite, montmorillonite-bound prions appear to be protected from inactivation. In contrast, when the clay structure is substantially transformed, as when exposed to high concentrations of sodium hydroxide, the attachment to montmorillonite does not slow degradation. A reduction in surface pH appears to cause slight disruptions in clay structure, which enhances degradation under these conditions. We expect our findings will aid the development of remediation approaches for successful decontamination of prion-contaminated sites.


Front. Vet. Sci., 04 March 2021 | https://doi.org/10.3389/fvets.2021.643754

Real-Time Quaking-Induced Conversion Detection of PrPSc in Fecal Samples From Chronic Wasting Disease Infected White-Tailed Deer Using Bank Vole Substrate

Soyoun Hwang, Justin J. Greenlee and Eric M. Nicholson*

Virus and Prion Research Unit, National Animal Disease Center, United States Department of Agriculture, Agricultural Research Service, Ames, IA, United States

Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy (TSE) that is fatal to free-range and captive cervids. CWD has been reported in the United States, Canada, South Korea, Norway, Finland, and Sweden, and the case numbers in both wild and farmed cervids are increasing rapidly. Studies indicate that lateral transmission of cervids likely occurs through the shedding of infectious prions in saliva, feces, urine, and blood into the environment. Therefore, the detection of CWD early in the incubation time is advantageous for disease management. In this study, we adapt real-time quacking-induced conversion (RT-QuIC) assays to detect the seeding activity of CWD prions in feces samples from clinical and preclinical white-tailed deer. By optimizing reaction conditions for temperature as well as the salt and salt concentration, prion seeding activity from both clinical and preclinical animals were detected by RT-QuIC. More specifically, all fecal samples collected from 6 to 30 months post inoculation showed seeding activity under the conditions of study. The combination of a highly sensitive detection tool paired with a sample type that may be collected non-invasively allows a useful tool to support CWD surveillance in wild and captive cervids.

snip...

Altogether, we confirm again that RT-QuIC is a powerful tool to detect infectious fecal prions from CWD infected white-tailed deer. Use of feces is a non-invasive and non-stressing approach to sampling of animals, of particular importance for non-domesticated animals that may be less tolerant to the handling required for sampling by other means. This is of importance to the management of both wild and farmed cervids and is also of use in experimental settings where repeated sampling of an individual animal would be otherwise difficult. Ultimately, fecal sampling may prove useful in the determination of disease prevalence in a geographic region or within a herd.


***> 2021 CWD TSE PRION ZOONOSIS ZOONOTIC UPDATE <***

TUESDAY, MAY 11, 2021 

A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet

Conclusion

We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.

Supplemental data including molecular tissue sample analysis and autopsy findings could yield further supporting evidence. Given this patient’s clinical resemblance to CBD and the known histological similarities of CBD with CJD, clinicians should consider both diseases in the differential diagnosis of patients with a similarly esoteric presentation. Regardless of the origin of this patient’s disease, it is clear that the potential for prion transmission from cervids to humans should be further investigated by the academic community with considerable urgency. https://thescipub.com/pdf/ajidsp.2021.43.48.pdf

please see recent data from Professor Kong on cwd zoonosis, IN CONFIDENCE, below, after your report here;...snip...end...tss

CONFIDENTIAL

IN CONFIDENCE

i figure your as about as official as it gets, and i think this science is extremely important for you to know and to converse about with your officials. it's about to burn a whole in my pocket. this is about as close as it will ever get for cwd zoonosis to be proven in my time, this and what Canada Czub et al found with the Macaques, plus an old study from cjd surveillance unit back that showed cjd and a 9% increase in risk from folks that eat venison, i will post all this below for your files Sir. i remember back in the BSE nvCJD days, from when the first BSE case in bovine was confirmed around 1984 maybe 83, i forget the good vets named that screwed it up first, Carol something... CAROL RICHARDSON, but from 83ish to 95 96 when nvCJD was linked to humans from BSE in cattle, so that took 10 to 15 years. hell, at that rate, especially with Texas and cwd zoonsis, hell, i'll be dead before it's official, if ever, so here ya go Sir. there was a grant study on cwd zoonosis that had been going on for some time, i followed it over the years, then the grant date for said study had expired, so, i thought i would write the good Professor about said study i.e. Professor Kong, CWRU et al. i will post the grant study abstract first, and then after that, what reply i got back, about said study that i was told not to post/publish, has not been peer reviewed yet...

i was told;

''At this point, please do not post any of the points publicly yet, but you can refer to points 1-3 in private discussions and all 5 points when discussing with relevant public officials to highlight the long-term risks of CWD zoonosis.'' 

CWD ZOONOSIS GRANT FIRST;

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

Cervid to human prion transmission

Kong, Qingzhong 

Case Western Reserve University, Cleveland, OH, United States

 Abstract 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

snip... 


Professor Kongs reply to me just this month about above grant study that has NOT been published in peer review yet...

=====IN CONFIDENCE=====

snip...tss


On Sat, Apr 3, 2021 at 12:19 PM Terry Singeltary <flounder9@verizon.net> wrote:

snip...

end...tss

==============END TSS============


CWD ZOONOSIS ZOONOTIC THE FULL MONTY TO DATE

International Conference on Emerging Diseases, Outbreaks & Case Studies & 16th Annual Meeting on Influenza March 28-29, 2018 | Orlando, USA

Qingzhong Kong

Case Western Reserve University School of Medicine, USA

Zoonotic potential of chronic wasting disease prions from cervids

Chronic wasting disease (CWD) is the prion disease in cervids (mule deer, white-tailed deer, American elk, moose, and reindeer). It has become an epidemic in North America, and it has been detected in the Europe (Norway) since 2016. The widespread CWD and popular hunting and consumption of cervid meat and other products raise serious public health concerns, but questions remain on human susceptibility to CWD prions, especially on the potential difference in zoonotic potential among the various CWD prion strains. We have been working to address this critical question for well over a decade. We used CWD samples from various cervid species to inoculate transgenic mice expressing human or elk prion protein (PrP). We found infectious prions in the spleen or brain in a small fraction of CWD-inoculated transgenic mice expressing human PrP, indicating that humans are not completely resistant to CWD prions; this finding has significant ramifications on the public health impact of CWD prions. The influence of cervid PrP polymorphisms, the prion strain dependence of CWD-to-human transmission barrier, and the characterization of experimental human CWD prions will be discussed.

Speaker Biography Qingzhong Kong has completed his PhD from the University of Massachusetts at Amherst and Post-doctoral studies at Yale University. He is currently an Associate Professor of Pathology, Neurology and Regenerative Medicine. He has published over 50 original research papers in reputable journals (including Science Translational Medicine, JCI, PNAS and Cell Reports) and has been serving as an Editorial Board Member on seven scientific journals. He has multiple research interests, including public health risks of animal prions (CWD of cervids and atypical BSE of cattle), animal modeling of human prion diseases, mechanisms of prion replication and pathogenesis, etiology of sporadic Creutzfeldt-Jacob disease (CJD) in humans, normal cellular PrP in the biology and pathology of multiple brain and peripheral diseases, proteins responsible for the α-cleavage of cellular PrP, as well as gene therapy and DNA vaccination.






Prion Conference 2018 Abstracts

BSE aka MAD COW DISEASE, was first discovered in 1984, and it took until 1995 to finally admit that BSE was causing nvCJD, the rest there is history, but that science is still evolving i.e. science now shows that indeed atypical L-type BSE, atypical Nor-98 Scrapie, and typical Scrapie are all zoonosis, zoonotic for humans, there from. 

HOW long are we going to wait for Chronic Wasting Disease, CWD TSE Prion of Cervid, and zoonosis, zoonotic tranmission to humans there from?

Studies have shown since 1994 that humans are susceptible to CWD TSE Prion, so, what's the hold up with making CWD a zoonotic zoonosis disease, the iatrogenic transmissions there from is not waiting for someone to make a decision.

Prion Conference 2018 Abstracts

P190 Human prion disease mortality rates by occurrence of chronic wasting disease in freeranging cervids, United States

Abrams JY (1), Maddox RA (1), Schonberger LB (1), Person MK (1), Appleby BS (2), Belay ED (1)

(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.

Background

Chronic wasting disease (CWD) is a prion disease of deer and elk that has been identified in freeranging cervids in 23 US states. While there is currently no epidemiological evidence for zoonotic transmission through the consumption of contaminated venison, studies suggest the CWD agent can cross the species barrier in experimental models designed to closely mimic humans. We compared rates of human prion disease in states with and without CWD to examine the possibility of undetermined zoonotic transmission.

Methods

Death records from the National Center for Health Statistics, case records from the National Prion Disease Pathology Surveillance Center, and additional state case reports were combined to create a database of human prion disease cases from 2003-2015. Identification of CWD in each state was determined through reports of positive CWD tests by state wildlife agencies. Age- and race-adjusted mortality rates for human prion disease, excluding cases with known etiology, were determined for four categories of states based on CWD occurrence: highly endemic (>16 counties with CWD identified in free-ranging cervids); moderately endemic (3-10 counties with CWD); low endemic (1-2 counties with CWD); and no CWD states. States were counted as having no CWD until the year CWD was first identified. Analyses stratified by age, sex, and time period were also conducted to focus on subgroups for which zoonotic transmission would be more likely to be detected: cases <55 years old, male sex, and the latter half of the study (2010-2015).

Results

Highly endemic states had a higher rate of prion disease mortality compared to non-CWD states (rate ratio [RR]: 1.12, 95% confidence interval [CI] = 1.01 - 1.23), as did low endemic states (RR: 1.15, 95% CI = 1.04 - 1.27). Moderately endemic states did not have an elevated mortality rate (RR: 1.05, 95% CI = 0.93 - 1.17). In age-stratified analyses, prion disease mortality rates among the <55 year old population were elevated for moderately endemic states (RR: 1.57, 95% CI = 1.10 – 2.24) while mortality rates were elevated among those ≥55 for highly endemic states (RR: 1.13, 95% CI = 1.02 - 1.26) and low endemic states (RR: 1.16, 95% CI = 1.04 - 1.29). In other stratified analyses, prion disease mortality rates for males were only elevated for low endemic states (RR: 1.27, 95% CI = 1.10 - 1.48), and none of the categories of CWD-endemic states had elevated mortality rates for the latter time period (2010-2015).

Conclusions

While higher prion disease mortality rates in certain categories of states with CWD in free-ranging cervids were noted, additional stratified analyses did not reveal markedly elevated rates for potentially sensitive subgroups that would be suggestive of zoonotic transmission. Unknown confounding factors or other biases may explain state-by-state differences in prion disease mortality.

=====

P172 Peripheral Neuropathy in Patients with Prion Disease

Wang H(1), Cohen M(1), Appleby BS(1,2)

(1) University Hospitals Cleveland Medical Center, Cleveland, Ohio (2) National Prion Disease Pathology Surveillance Center, Cleveland, Ohio.

Prion disease is a fatal progressive neurodegenerative disease due to deposition of an abnormal protease-resistant isoform of prion protein. Typical symptoms include rapidly progressive dementia, myoclonus, visual disturbance and hallucinations. Interestingly, in patients with prion disease, the abnormal protein canould also be found in the peripheral nervous system. Case reports of prion deposition in peripheral nerves have been reported. Peripheral nerve involvement is thought to be uncommon; however, little is known about the exact prevalence and features of peripheral neuropathy in patients with prion disease.

We reviewed autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017. We collected information regarding prion protein diagnosis, demographics, comorbidities, clinical symptoms, physical exam, neuropathology, molecular subtype, genetics lab, brain MRI, image and EMG reports. Our study included 104 patients. Thirteen (12.5%) patients had either subjective symptoms or objective signs of peripheral neuropathy. Among these 13 patients, 3 had other known potential etiologies of peripheral neuropathy such as vitamin B12 deficiency or prior chemotherapy. Among 10 patients that had no other clear etiology, 3 (30%) had familial CJD. The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%). The Majority of cases wasere male (60%). Half of them had exposure to wild game. The most common subjective symptoms were tingling and/or numbness of distal extremities. The most common objective finding was diminished vibratory sensation in the feet. Half of them had an EMG with the findings ranging from fasciculations to axonal polyneuropathy or demyelinating polyneuropathy.

Our study provides an overview of the pattern of peripheral neuropathy in patients with prion disease. Among patients with peripheral neuropathy symptoms or signs, majority has polyneuropathy. It is important to document the baseline frequency of peripheral neuropathy in prion diseases as these symptoms may become important when conducting surveillance for potential novel zoonotic prion diseases.

=====

P177 PrP plaques in methionine homozygous Creutzfeldt-Jakob disease patients as a potential marker of iatrogenic transmission

Abrams JY (1), Schonberger LB (1), Cali I (2), Cohen Y (2), Blevins JE (2), Maddox RA (1), Belay ED (1), Appleby BS (2), Cohen ML (2)

(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.

Background

Sporadic Creutzfeldt-Jakob disease (CJD) is widely believed to originate from de novo spontaneous conversion of normal prion protein (PrP) to its pathogenic form, but concern remains that some reported sporadic CJD cases may actually be caused by disease transmission via iatrogenic processes. For cases with methionine homozygosity (CJD-MM) at codon 129 of the PRNP gene, recent research has pointed to plaque-like PrP deposition as a potential marker of iatrogenic transmission for a subset of cases. This phenotype is theorized to originate from specific iatrogenic source CJD types that comprise roughly a quarter of known CJD cases.

Methods

We reviewed scientific literature for studies which described PrP plaques among CJD patients with known epidemiological links to iatrogenic transmission (receipt of cadaveric human grown hormone or dura mater), as well as in cases of reported sporadic CJD. The presence and description of plaques, along with CJD classification type and other contextual factors, were used to summarize the current evidence regarding plaques as a potential marker of iatrogenic transmission. In addition, 523 cases of reported sporadic CJD cases in the US from January 2013 through September 2017 were assessed for presence of PrP plaques.

Results

We identified four studies describing 52 total cases of CJD-MM among either dura mater recipients or growth hormone recipients, of which 30 were identified as having PrP plaques. While sporadic cases were not generally described as having plaques, we did identify case reports which described plaques among sporadic MM2 cases as well as case reports of plaques exclusively in white matter among sporadic MM1 cases. Among the 523 reported sporadic CJD cases, 0 of 366 MM1 cases had plaques, 2 of 48 MM2 cases had kuru plaques, and 4 of 109 MM1+2 cases had either kuru plaques or both kuru and florid plaques. Medical chart review of the six reported sporadic CJD cases with plaques did not reveal clinical histories suggestive of potential iatrogenic transmission.

Conclusions

PrP plaques occur much more frequently for iatrogenic CJD-MM cases compared to sporadic CJDMM cases. Plaques may indicate iatrogenic transmission for CJD-MM cases without a type 2 Western blot fragment. The study results suggest the absence of significant misclassifications of iatrogenic CJD as sporadic. To our knowledge, this study is the first to describe grey matter kuru plaques in apparently sporadic CJD-MM patients with a type 2 Western blot fragment.

=====

P180 Clinico-pathological analysis of human prion diseases in a brain bank series

Ximelis T (1), Aldecoa I (1,2), Molina-Porcel L (1,3), Grau-Rivera O (4), Ferrer I (5), Nos C (6), Gelpi E (1,7), Sánchez-Valle R (1,4)

(1) Neurological Tissue Bank of the Biobanc-Hospital ClÃnic-IDIBAPS, Barcelona, Spain (2) Pathological Service of Hospital ClÃnic de Barcelona, Barcelona, Spain (3) EAIA Trastorns Cognitius, Centre Emili Mira, Parc de Salut Mar, Barcelona, Spain (4) Department of Neurology of Hospital ClÃnic de Barcelona, Barcelona, Spain (5) Institute of Neuropathology, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Barcelona (6) General subdirectorate of Surveillance and Response to Emergencies in Public Health, Department of Public Health in Catalonia, Barcelona, Spain (7) Institute of Neurology, Medical University of Vienna, Vienna, Austria.

Background and objective:

The Neurological Tissue Bank (NTB) of the Hospital Clínic-Institut d‘Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain is the reference center in Catalonia for the neuropathological study of prion diseases in the region since 2001. The aim of this study is to analyse the characteristics of the confirmed prion diseases registered at the NTB during the last 15 years.

Methods:

We reviewed retrospectively all neuropathologically confirmed cases registered during the period January 2001 to December 2016.

Results:

176 cases (54,3% female, mean age: 67,5 years and age range: 25-86 years) of neuropathological confirmed prion diseases have been studied at the NTB. 152 cases corresponded to sporadic Creutzfeldt-Jakob disease (sCJD), 10 to genetic CJD, 10 to Fatal Familial Insomnia, 2 to GerstmannSträussler-Scheinker disease, and 2 cases to variably protease-sensitive prionopathy (VPSPr). Within sCJD subtypes the MM1 subtype was the most frequent, followed by the VV2 histotype.

Clinical and neuropathological diagnoses agreed in 166 cases (94%). The clinical diagnosis was not accurate in 10 patients with definite prion disease: 1 had a clinical diagnosis of Fronto-temporal dementia (FTD), 1 Niemann-Pick‘s disease, 1 Lewy Body‘s Disease, 2 Alzheimer‘s disease, 1 Cortico-basal syndrome and 2 undetermined dementia. Among patients with VPSPr, 1 had a clinical diagnosis of Amyotrophic lateral sclerosis (ALS) and the other one with FTD.

Concomitant pathologies are frequent in older age groups, mainly AD neuropathological changes were observed in these subjects.

Discussion:

A wide spectrum of human prion diseases have been identified in the NTB being the relative frequencies and main characteristics like other published series. There is a high rate of agreement between clinical and neuropathological diagnoses with prion diseases. These findings show the importance that public health has given to prion diseases during the past 15 years. Continuous surveillance of human prion disease allows identification of new emerging phenotypes. Brain tissue samples from these donors are available to the scientific community. For more information please visit:


=====

P192 Prion amplification techniques for the rapid evaluation of surface decontamination procedures

Bruyere-Ostells L (1), Mayran C (1), Belondrade M (1), Boublik Y (2), Haïk S (3), Fournier-Wirth C (1), Nicot S (1), Bougard D (1)

(1) Pathogenesis and control of chronic infections, Etablissement Français du Sang, Inserm, Université de Montpellier, Montpellier, France. (2) Centre de Recherche en Biologie cellulaire de Montpellier, CNRS, Université de Montpellier, Montpellier, France. (3) Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Université Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, Paris, France.

Aims:

Transmissible Spongiform Encephalopathies (TSE) or prion diseases are a group of incurable and always fatal neurodegenerative disorders including Creutzfeldt-Jakob diseases (CJD) in humans. These pathologies include sporadic (sCJD), genetic and acquired (variant CJD) forms. By the past, sCJD and vCJD were transmitted by different prion contaminated biological materials to patients resulting in more than 400 iatrogenic cases (iCJD). The atypical nature and the biochemical properties of the infectious agent, formed by abnormal prion protein or PrPTSE, make it particularly resistant to conventional decontamination procedures. In addition, PrPTSE is widely distributed throughout the organism before clinical onset in vCJD and can also be detected in some peripheral tissues in sporadic CJD. Risk of iatrogenic transmission of CJD by contaminated medical device remains thus a concern for healthcare facilities. Bioassay is the gold standard method to evaluate the efficacy of prion decontamination procedures but is time-consuming and expensive. Here, we propose to compare in vitro prion amplification techniques: Protein Misfolding Cyclic Amplification (PMCA) and Real-Time Quaking Induced Conversion (RT-QuIC) for the detection of residual prions on surface after decontamination.

Methods:

Stainless steel wires, by mimicking the surface of surgical instruments, were proposed as a carrier model of prions for inactivation studies. To determine the sensitivity of the two amplification techniques on wires (Surf-PMCA and Surf-QuIC), steel wires were therefore contaminated with serial dilutions of brain homogenates (BH) from a 263k infected hamster and from a patient with sCJD (MM1 subtype). We then compared the different standard decontamination procedures including partially and fully efficient treatments by detecting the residual seeding activity on 263K and sCJD contaminated wires. We completed our study by the evaluation of marketed reagents endorsed for prion decontamination.

Results:

The two amplification techniques can detect minute quantities of PrPTSE adsorbed onto a single wire. 8/8 wires contaminated with a 10-6 dilution of 263k BH and 1/6 with the 10-8 dilution are positive with Surf-PMCA. Similar performances were obtained with Surf-QuIC on 263K: 10/16 wires contaminated with 10-6 dilution and 1/8 wires contaminated with 10-8 dilution are positive. Regarding the human sCJD-MM1 prion, Surf-QuIC allows us to detect 16/16 wires contaminated with 10-6 dilutions and 14/16 with 10-7 . Results obtained after decontamination treatments are very similar between 263K and sCJD prions. Efficiency of marketed treatments to remove prions is lower than expected.

Conclusions:

Surf-PMCA and Surf-QuIC are very sensitive methods for the detection of prions on wires and could be applied to prion decontamination studies for rapid evaluation of new treatments. Sodium hypochlorite is the only product to efficiently remove seeding activity of both 263K and sCJD prions.

=====

WA2 Oral transmission of CWD into Cynomolgus macaques: signs of atypical disease, prion conversion and infectivity in macaques and bio-assayed transgenic mice

Schatzl HM (1, 2), Hannaoui S (1, 2), Cheng Y-C (1, 2), Gilch S (1, 2), Beekes M (3), SchulzSchaeffer W (4), Stahl-Hennig C (5) and Czub S (2, 6)

(1) University of Calgary, Calgary Prion Research Unit, Calgary, Canada (2) University of Calgary, Faculty of Veterinary Medicine, Calgary, Canada, (3) Robert Koch Institute, Berlin, Germany, (4) University of Homburg/Saar, Homburg, Germany, (5) German Primate Center, Goettingen, Germany, (6) Canadian Food Inspection Agency (CFIA), Lethbridge, Canada.

To date, BSE is the only example of interspecies transmission of an animal prion disease into humans. The potential zoonotic transmission of CWD is an alarming issue and was addressed by many groups using a variety of in vitro and in vivo experimental systems. Evidence from these studies indicated a substantial, if not absolute, species barrier, aligning with the absence of epidemiological evidence suggesting transmission into humans. Studies in non-human primates were not conclusive so far, with oral transmission into new-world monkeys and no transmission into old-world monkeys. Our consortium has challenged 18 Cynomolgus macaques with characterized CWD material, focusing on oral transmission with muscle tissue. Some macaques have orally received a total of 5 kg of muscle material over a period of 2 years. After 5-7 years of incubation time some animals showed clinical symptoms indicative of prion disease, and prion neuropathology and PrPSc deposition were found in spinal cord and brain of euthanized animals. PrPSc in immunoblot was weakly detected in some spinal cord materials and various tissues tested positive in RT-QuIC, including lymph node and spleen homogenates. To prove prion infectivity in the macaque tissues, we have intracerebrally inoculated 2 lines of transgenic mice, expressing either elk or human PrP. At least 3 TgElk mice, receiving tissues from 2 different macaques, showed clinical signs of a progressive prion disease and brains were positive in immunoblot and RT-QuIC. Tissues (brain, spinal cord and spleen) from these and preclinical mice are currently tested using various read-outs and by second passage in mice. Transgenic mice expressing human PrP were so far negative for clear clinical prion disease (some mice >300 days p.i.). In parallel, the same macaque materials are inoculated into bank voles. Taken together, there is strong evidence of transmissibility of CWD orally into macaques and from macaque tissues into transgenic mouse models, although with an incomplete attack rate. The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology. Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP. The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.

See also poster P103

***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.

=====

WA16 Monitoring Potential CWD Transmission to Humans

Belay ED

Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA.

The spread of chronic wasting disease (CWD) in animals has raised concerns about increasing human exposure to the CWD agent via hunting and venison consumption, potentially facilitating CWD transmission to humans. Several studies have explored this possibility, including limited epidemiologic studies, in vitro experiments, and laboratory studies using various types of animal models. Most human exposures to the CWD agent in the United States would be expected to occur in association with deer and elk hunting in CWD-endemic areas. The Centers for Disease Control and Prevention (CDC) collaborated with state health departments in Colorado, Wisconsin, and Wyoming to identify persons at risk of CWD exposure and to monitor their vital status over time. Databases were established of persons who hunted in Colorado and Wyoming and those who reported consumption of venison from deer that later tested positive in Wisconsin. Information from the databases is periodically cross-checked with mortality data to determine the vital status and causes of death for deceased persons. Long-term follow-up of these hunters is needed to assess their risk of development of a prion disease linked to CWD exposure.

=====

P166 Characterization of CJD strain profiles in venison consumers and non-consumers from Alberta and Saskatchewan

Stephanie Booth (1,2), Lise Lamoureux (1), Debra Sorensen (1), Jennifer L. Myskiw (1,2), Megan Klassen (1,2), Michael Coulthart (3), Valerie Sim (4)

(1) Zoonotic Diseases and Special Pathogens, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg (2) Department of Medical Microbiology and Infectious Diseases, University of Manitoba, Winnipeg (3) Canadian CJD Surveillance System, Public Health Agency of Canada, Ottawa (4) Division of Neurology, Department of Medicine Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton.

Chronic wasting disease (CWD) is spreading rapidly through wild cervid populations in the Canadian provinces of Alberta and Saskatchewan. While this has implications for tourism and hunting, there is also concern over possible zoonotic transmission to humans who eat venison from infected deer. Whilst there is no evidence of any human cases of CWD to date, the Canadian CJD Surveillance System (CJDSS) in Canada is staying vigilant. When variant CJD occurred following exposure to BSE, the unique biochemical fingerprint of the pathologic PrP enabled a causal link to be confirmed. However, we cannot be sure what phenotype human CWD prions would present with, or indeed, whether this would be distinct from that see in sporadic CJD. Therefore we are undertaking a systematic analysis of the molecular diversity of CJD cases of individuals who resided in Alberta and Saskatchewan at their time of death comparing venison consumers and non-consumers, using a variety of clinical, imaging, pathological and biochemical markers. Our initial objective is to develop novel biochemical methodologies that will extend the baseline glycoform and genetic polymorphism typing that is already completed by the CJDSS. Firstly, we are reviewing MRI, EEG and pathology information from over 40 cases of CJD to select clinically affected areas for further investigation. Biochemical analysis will include assessment of the levels of protease sensitive and resistant prion protein, glycoform typing using 2D gel electrophoresis, testing seeding capabilities and kinetics of aggregation by quaking-induced conversion, and determining prion oligomer size distributions with asymmetric flow field fractionation with in-line light scattering. Progress and preliminary data will be presented. Ultimately, we intend to further define the relationship between PrP structure and disease phenotype and establish a baseline for the identification of future atypical CJD cases that may arise as a result of exposure to CWD.

=====

Source Prion Conference 2018 Abstracts




Volume 24, Number 8—August 2018 Research Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions

Marcelo A. BarriaComments to Author , Adriana Libori, Gordon Mitchell, and Mark W. Head Author affiliations: National CJD Research and Surveillance Unit, University of Edinburgh, Edinburgh, Scotland, UK (M.A. Barria, A. Libori, M.W. Head); National and OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario, Canada (G. Mitchell)

Abstract Chronic wasting disease (CWD) is a contagious and fatal neurodegenerative disease and a serious animal health issue for deer and elk in North America. The identification of the first cases of CWD among free-ranging reindeer and moose in Europe brings back into focus the unresolved issue of whether CWD can be zoonotic like bovine spongiform encephalopathy. We used a cell-free seeded protein misfolding assay to determine whether CWD prions from elk, white-tailed deer, and reindeer in North America can convert the human prion protein to the disease-associated form. We found that prions can convert, but the efficiency of conversion is affected by polymorphic variation in the cervid and human prion protein genes. In view of the similarity of reindeer, elk, and white-tailed deer in North America to reindeer, red deer, and roe deer, respectively, in Europe, a more comprehensive and thorough assessment of the zoonotic potential of CWD might be warranted.

snip...

Discussion Characterization of the transmission properties of CWD and evaluation of their zoonotic potential are important for public health purposes. Given that CWD affects several members of the family Cervidae, it seems reasonable to consider whether the zoonotic potential of CWD prions could be affected by factors such as CWD strain, cervid species, geographic location, and Prnp–PRNP polymorphic variation. We have previously used an in vitro conversion assay (PMCA) to investigate the susceptibility of the human PrP to conversion to its disease-associated form by several animal prion diseases, including CWD (15,16,22). The sensitivity of our molecular model for the detection of zoonotic conversion depends on the combination of 1) the action of proteinase K to degrade the abundant human PrPC that constitutes the substrate while only N terminally truncating any human PrPres produced and 2) the presence of the 3F4 epitope on human but not cervid PrP. In effect, this degree of sensitivity means that any human PrPres formed during the PMCA reaction can be detected down to the limit of Western blot sensitivity. In contrast, if other antibodies that detect both cervid and human PrP are used, such as 6H4, then newly formed human PrPres must be detected as a measurable increase in PrPres over the amount remaining in the reaction product from the cervid seed. Although best known for the efficient amplification of prions in research and diagnostic contexts, the variation of the PMCA method employed in our study is optimized for the definitive detection of zoonotic reaction products of inherently inefficient conversion reactions conducted across species barriers. By using this system, we previously made and reported the novel observation that elk CWD prions could convert human PrPC from human brain and could also convert recombinant human PrPC expressed in transgenic mice and eukaryotic cell cultures (15).

A previous publication suggested that mule deer PrPSc was unable to convert humanized transgenic substrate in PMCA assays (23) and required a further step of in vitro conditioning in deer substrate PMCA before it was able to cross the deer–human molecular barrier (24). However, prions from other species, such as elk (15) and reindeer affected by CWD, appear to be compatible with the human protein in a single round of amplification (as shown in our study). These observations suggest that different deer species affected by CWD could present differing degrees of the olecular compatibility with the normal form of human PrP.

The contribution of the polymorphism at codon 129 of the human PrP gene has been extensively studied and is recognized as a risk factor for Creutzfeldt-Jakob disease (4). In cervids, the equivalent codon corresponds to the position 132 encoding methionine or leucine. This polymorphism in the elk gene has been shown to play an important role in CWD susceptibility (25,26). We have investigated the effect of this cervid Prnp polymorphism on the conversion of the humanized transgenic substrate according to the variation in the equivalent PRNP codon 129 polymorphism. Interestingly, only the homologs methionine homozygous seed–substrate reactions could readily convert the human PrP, whereas the heterozygous elk PrPSc was unable to do so, even though comparable amounts of PrPres were used to seed the reaction. In addition, we observed only low levels of human PrPres formation in the reactions seeded with the homozygous methionine (132 MM) and the heterozygous (132 ML) seeds incubated with the other 2 human polymorphic substrates (129 MV and 129 VV). The presence of the amino acid leucine at position 132 of the elk Prnp gene has been attributed to a lower degree of prion conversion compared with methionine on the basis of experiments in mice made transgenic for these polymorphic variants (26). Considering the differences observed for the amplification of the homozygous human methionine substrate by the 2 polymorphic elk seeds (MM and ML), reappraisal of the susceptibility of human PrPC by the full range of cervid polymorphic variants affected by CWD would be warranted.

In light of the recent identification of the first cases of CWD in Europe in a free-ranging reindeer (R. tarandus) in Norway (2), we also decided to evaluate the in vitro conversion potential of CWD in 2 experimentally infected reindeer (18). Formation of human PrPres was readily detectable after a single round of PMCA, and in all 3 humanized polymorphic substrates (MM, MV, and VV). This finding suggests that CWD prions from reindeer could be more compatible with human PrPC generally and might therefore present a greater risk for zoonosis than, for example, CWD prions from white-tailed deer. A more comprehensive comparison of CWD in the affected species, coupled with the polymorphic variations in the human and deer PRNP–Prnp genes, in vivo and in vitro, will be required before firm conclusions can be drawn. Analysis of the Prnp sequence of the CWD reindeer in Norway was reported to be identical to the specimens used in our study (2). This finding raises the possibility of a direct comparison of zoonotic potential between CWD acquired in the wild and that produced in a controlled laboratory setting. (Table).

The prion hypothesis proposes that direct molecular interaction between PrPSc and PrPC is necessary for conversion and prion replication. Accordingly, polymorphic variants of the PrP of host and agent might play a role in determining compatibility and potential zoonotic risk. In this study, we have examined the capacity of the human PrPC to support in vitro conversion by elk, white-tailed deer, and reindeer CWD PrPSc. Our data confirm that elk CWD prions can convert the human PrPC, at least in vitro, and show that the homologous PRNP polymorphisms at codon 129 and 132 in humans and cervids affect conversion efficiency. Other species affected by CWD, particularly caribou or reindeer, also seem able to convert the human PrP. It will be important to determine whether other polymorphic variants found in other CWD-affected Cervidae or perhaps other factors (17) exert similar effects on the ability to convert human PrP and thus affect their zoonotic potential.

Dr. Barria is a research scientist working at the National CJD Research and Surveillance Unit, University of Edinburgh. His research has focused on understanding the molecular basis of a group of fatal neurologic disorders called prion diseases.

Acknowledgments We thank Aru Balachandran for originally providing cervid brain tissues, Abigail Diack and Jean Manson for providing mouse brain tissue, and James Ironside for his critical reading of the manuscript at an early stage.

This report is independent research commissioned and funded by the United Kingdom’s Department of Health Policy Research Programme and the Government of Scotland. The views expressed in this publication are those of the authors and not necessarily those of the Department of Health or the Government of Scotland.

Author contributions: The study was conceived and designed by M.A.B. and M.W.H. The experiments were conducted by M.A.B. and A.L. Chronic wasting disease brain specimens were provided by G.M. The manuscript was written by M.A.B. and M.W.H. All authors contributed to the editing and revision of the manuscript.



Prion 2017 Conference Abstracts
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. 
PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS ABSTRACTS REFERENCE
8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.


SATURDAY, FEBRUARY 23, 2019 

Chronic Wasting Disease CWD TSE Prion and THE FEAST 2003 CDC an updated review of the science 2019


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

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. "


Transmission Studies

Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS

resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.

snip.... 


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. 


*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.

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 

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


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 ; 


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

sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven.

if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;



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 

*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***

> 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
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
*** 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).***
CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL

Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY 

Date: Fri, 18 Oct 2002 23:12:22 +0100 

From: Steve Dealler 

Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member 

To: BSE-L@ References: <3daf5023 .4080804="" wt.net="">

Dear Terry,

An excellent piece of review as this literature is desparately difficult to get back from Government sites.

What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!

Steve Dealler =============== 


''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).''

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 ;




Stephen Dealler is a consultant medical microbiologist  deal@airtime.co.uk 

BSE Inquiry Steve Dealler

Management In Confidence

BSE: Private Submission of Bovine Brain Dealler

snip...see full text;

MONDAY, FEBRUARY 25, 2019

***> MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019


Jeff Schwan was 26 years old when he died from CJD.

***> I urge everyone to watch this video closely...terry

*** You can see video here and interview with Jeff's Mom, and scientist telling you to test everything and potential risk factors for humans ***

***> ''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).''

***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***

***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<*** 

***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***

***> 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.<*** 

***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***


 ***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.***

Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.

https://www.nature.com/articles/srep11573 

O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations 
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France 

Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). 

Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. 

*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, 

***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), 

***is the third potentially zoonotic PD (with BSE and L-type BSE), 

***thus questioning the origin of human sporadic cases. 

We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. 

=============== 

***thus questioning the origin of human sporadic cases*** 

=============== 

***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. 

============== 

https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf 

***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. 

***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. 

***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. 

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20 

PRION 2016 TOKYO

Saturday, April 23, 2016

SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016

Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online

Taylor & Francis

Prion 2016 Animal Prion Disease Workshop Abstracts

WS-01: Prion diseases in animals and zoonotic potential

Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. 

These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. 

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

Title: Transmission of scrapie prions to primate after an extended silent incubation period) 

*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. 

*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. 

*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. 

http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160

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

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

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

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

snip...

The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.

PMID: 6997404


Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"

Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the scrapie problem urgent if the sheep industry is not to suffer grievously.

snip...

76/10.12/4.6


Nature. 1972 Mar 10;236(5341):73-4.

Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).

Gibbs CJ Jr, Gajdusek DC.

Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

C. J. GIBBS jun. & D. C. GAJDUSEK

National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).



Wednesday, February 16, 2011

IN CONFIDENCE

SCRAPIE TRANSMISSION TO CHIMPANZEES

IN CONFIDENCE

reference...

RB3.20

TRANSMISSION TO CHIMPANZEES

1. Kuru and CJD have been successfully transmitted to chimpanzees but scrapie and TME have not.

2. We cannot say that scrapie will not transmit to chimpanzees. There are several scrapie strains and I am not aware that all have been tried (that would have to be from mouse passaged material). Nor has a wide enough range of field isolates subsequently strain typed in mice been inoculated by the appropriate routes (i/c, ilp and i/v) :

3. I believe the proposed experiment to determine transmissibility, if conducted, would only show the susceptibility or resistance of the chimpanzee to infection/disease by the routes used and the result could not be interpreted for the predictability of the susceptibility for man. Proposals for prolonged oral exposure of chimpanzees to milk from cattle were suggested a long while ago and rejected.

4. In view of Dr Gibbs' probable use of chimpazees Mr Wells' comments (enclosed) are pertinent. I have yet to receive a direct communication from Dr Schellekers but before any collaboration or provision of material we should identify the Gibbs' proposals and objectives.

5. A positive result from a chimpanzee challenged severely would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

6. A negative result would take a lifetime to determine but that would be a shorter period than might be available for human exposure and it would still not answer the question regarding mans' susceptibility. In the meantime no doubt the negativity would be used defensively. It would however be counterproductive if the experiment finally became positive. We may learn more about public reactions following next Monday' s meeting.

R. Bradley

23 September 1990

CVO (+Mr Wells' comments)

Dr T W A Little

Dr B J Shreeve

90/9.23/1.1.


IN CONFIDENCE CHIMPANZEES

CODE 18-77 Reference RB3.46

Some further information that may assist in decision making has been gained by discussion with Dr Rosalind Ridley.

She says that careful study of Gajdusek's work shows no increased susceptibility of chimpanzees over New World Monkeys such as Squirrel Monkeys. She does not think it would tell you anything about the susceptibility to man. Also Gajdusek did not, she believes, challenge chimpanzees with scrapie as severely as we did pigs and we know little of that source of scrapie. Comparisons would be difficult. She also would not expect the Home Office to sanction such experiments here unless there was a very clear and important objective that would be important for human health protection. She doubted such a case could be made. If this is the case she thought it would be unethical to do an experiment abroad because we could not do it in our own country.

Retrospectively she feels they should have put up more marmosets than they did. They all remain healthy. They would normally regard the transmission as negative if no disease resulted in five years.

We are not being asked for a decision but I think that before we made one we should gain as much knowledge as we can. If we decided to proceed we would have to bear any criticisms for many years if there was an adverse view by scientists or­media. This should not be undertaken lightly. There is already some adverse comment here, I gather, on the pig experiment though that will subside.

The Gibbs' (as' distinct from Schellekers') study is somewhat different. We are merely supplying material for comparative studies in a laboratory with the greatest experience of human SEs in the world and it has been sanctioned by USDA (though we do not know for certain yet if chimpanzees specifically will be used). This would keep it at a lower profile than if we conducted such an experiment in the UK or Europe.

I consider we must have very powerful and defendable objectives to go beyond Gibbs' proposed experiments and should not initiate others just because an offer has been made.

Scientists have a responsibility to seek other methods of investigative research other than animal experimentation. At present no objective has convinced me we need to do research using Chimpanzees - a species in need of protection. Resisting such proposals would enable us to communicate that information to the scientist and the public should the need arise. A line would have been drawn.

CVO cc Dr T Dr B W A Little Dr B J Shreeve

R Bradley

26 September 1990

90/9.26/3.2


this is tse prion political theater here, i.e. what i call TSE PRION POKER...tss



3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs.

snip...

PAGE 26

Transmission Studies

Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS

resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.

The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite its subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA veiwed it as a wildlife problem and consequently not their province! ...page 26. 

snip...see;

IN CONFIDENCE

PERCEPTIONS OF UNCONVENTIONAL SLOW VIRUS DISEASE OF ANIMALS IN THE USA

GAH WELLS

REPORT OF A VISIT TO THE USA

APRIL-MAY 1989


TUESDAY, MAY 11, 2021

A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet

https://creutzfeldt-jakob-disease.blogspot.com/2021/05/a-unique-presentation-of-creutzfeldt.html

Saturday, May 1, 2021 

Clinical Use of Improved Diagnostic Testing for Detection of Prion Disease


JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 April 26,2005

Mr. Terry Singeltary

P.O. Box 

Bacliff, Texas 77518

Dear Mr. Singeltary:

In response to your recent request for my assistance, I have contacted the National Institutes of Health. I will write you again as soon as I receive a reply. I appreciate having the opportunity to represent you in the United States Senate and to be of service in this matter.

Sincerely,

JOHN CORNYN United States Senator JC:djl 

=============== 

JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305

May 18,2005

Mr. Terry Singeltary

P.O. Box 

Bacliff, Texas 77518

Dear Mr. Singeltary:

Enclosed is the reply I received from the Department of Health and Human Services in response to my earlier inquiry on your behalf. I hope this will be useful to you. I appreciate having the opportunity to represent you in the United States Senate. Thank you for taking time to contact me. Sincerely,

JOHN CORNYN United States Senate JC:djl Enclosure

DEPARTMENT OF HEALTH & HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke NINDS Building 31, Room 8A52 31 Center Dr., MSC 2540 Bethesda, Maryland 20892-2540 Phone: 301-496-9746 Fax: 301-496-0296 Email: [log in to unmask]

May 10, 2005

The Honorable John Cornyn United States Senator Occidental Tower5005 LBJ Freeway, Suite 1150Dallas, Texas 75244-6199

Dear Senator Cornyn:

Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about the preservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Research program for many years. I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand his desire that any tissues that could help investigators unravel the puzzle of this deadly disease are preserved. I hope he will be pleased to learn that all the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved. (The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification.) The purpose of gathering these brains and tissues is to help scientists learn about CJD. To that end, some of the NINDS-held samples are distributed to investigators who can demonstrate that they have a compelling research or public health need for such materials. For example, samples have been transferred to NIH grantee Dr. Pierluigi Gambetti, who heads the National Prion Diseases Pathology Surveillance Center at Case Western Reserve University in Ohio and works with the Centers for Disease Control and Prevention to monitor all cases of CJD in the United States. Dr. Gambetti studies the tissues to learn about the formation, physical and chemical properties, and pathogenic mechanisms of prion proteins, which are believed to be involved inthe cause of CJD. Samples have also been transferred to Dr. David Asher, at the U.S. Food and Drug Administration, for use in assessing a potential diagnostic test for CJD.

Page 2 - The Honorable John Cornyn

in closing, we know that donating organs and tissue from loved ones is a very difficult and personal choice that must often be made at the most stressful of times. We at the NINDS are grateful to those stalwart family members who make this choice in the selfless hope that it will help others afflicted with CJD. We also know the invaluable contribution such donations make to the advancement of medical science, and we are dedicated to the preservation of all of the tissue samples that can help in our efforts to overcome CJD.

I hope this information is helpful to you in responding to Mr. Singeltary. Sincerely,

Story C. Landis, Ph.D. Director, National Institute of Neurological Disorders and Stroke

snip...see full text;



Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. 

JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease 

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.. 

Terry S. Singeltary, Sr Bacliff, Tex 1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. 


doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003. 

Volume 3, Issue 8, August 2003, Page 463 

“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem..” 



January 28, 2003; 60 (2) VIEWS & REVIEWS

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States Terry S. Singeltary, retired (medically) 

Published March 26, 2003

26 March 2003

Terry S. Singeltary, retired (medically) CJD WATCH

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?


SPORADIC CJD LAYING ODDS


In brief

BMJ 2000; 320 doi: https://doi.org/10.1136/bmj.320.7226.8/b (Published 01 January 2000)

Cite this as: BMJ 2000;320:8

Rapid Response:

02 January 2000

Terry S Singeltary

retired

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.

The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;

"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."

Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.

Something else I find odd, page 16;

"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."

A few more factors to consider, page 15;

"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."

"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."

"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."

Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow.

Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.

To be continued...

Terry S. Singeltary Sr.

Bacliff, Texas USA

Competing interests: No competing interests


Rapid response to:

US scientists develop a possible test for BSE

15 November 1999

Terry S Singeltary

NA

BMJ 1999; 319 doi: https://doi.org/10.1136/bmj.319.7220.1312b (Published 13 November 1999)

Cite this as: BMJ 1999;319:1312

Article Related content Article metrics 

Rapid responses 

Response Rapid Response: Re: vCJD in the USA * BSE in U.S. In reading the recent article in the BMJ about the potential BSE tests being developed in the U.S. and Bart Van Everbroeck reply. It does not surprize me, that the U.S. has been concealing vCJD. There have been people dying from CJD, with all the symptoms and pathological findings that resemble U.K. vCJD for some time. It just seems that when there is one found, they seem to change the clarical classification of the disease, to fit their agenda. I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age. Also, my Mom died a most hideous death, Heidenhain Variant Creutzfeldt Jakob disease. Her symptoms resemble that of all the U.K. vCJD victims. She would jerk so bad at times, it would take 3 of us to hold her down, while she screamed "God, what's wrong with me, why can't I stop this." 1st of symptoms to death, 10 weeks, she went blind in the first few weeks. But, then they told me that this was just another strain of sporadic CJD. They can call it what ever they want, but I know what I saw, and what she went through. Sporadic, simply means, they do not know. My neighbors Mom also died from CJD. She had been taking a nutritional supplement which contained the following; vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. As I said, this woman taking these nutritional supplements, died from CJD. The particular batch of pills that was located, in which she was taking, was tested. From what I have heard, they came up negative, for the prion protein. But, in the same breath, they said their testing, may not have been strong enough to pick up the infectivity. Plus, she had been taking these type pills for years, so, could it have come from another batch?

CWD is just a small piece of a very big puzzle. I have seen while deer hunting, deer, squirrels and birds, eating from cattle feed troughs where they feed cattle, the high protein cattle by products, at least up until Aug. 4, 1997.

So why would it be so hard to believe that this is how they might become infected with a TSE. Or, even by potentially infected land. It's been well documented that it could be possible, from scrapie. Cats becoming infected with a TSE. Have you ever read the ingredients on the labels of cat and dog food? But, they do not put these tissues from these animals in pharmaceuticals, cosmetics, nutritional supplements, hGH, hPG, blood products, heart valves, and the many more products that come from bovine, ovine, or porcine tissues and organs. So, as I said, this CWD would be a small piece of a very big puzzle. But, it is here, and it most likely has killed. You see, greed is what caused this catastrophe, rendering and feeding practices. But, once Pandora's box was opened, the potential routes of infection became endless.

No BSE in the U.S.A.? I would not be so sure of that considering that since 1990;

Since 1990 the U.S. has raised 1,250,880,700 cattle;

Since 1990 the U.S. has ONLY checked 8,881 cattle brains for BSE, as of Oct. 4, 1999;

There are apprx. 100,000 DOWNER cattle annually in the U.S., that up until Aug. 4, 1997 went to the renders for feed;

Scrapie running rampant for years in the U.S., 950 infected FLOCKS, as of Aug. 1999;

Our feeding and rendering practices have mirrored that of the U.K. for years, some say it was worse. Everything from the downer cattle, to those scrapie infected sheep, to any roadkill, including the city police horse and the circus elephant went to the renders for feed and other products for consumption. Then they only implemented a partial feed ban on Aug. 4, 1997, but pigs, chickens, dogs, and cats, and humans were exempt from that ban. So they can still feed pigs and chickens those potentially TSE tainted by-products, and then they can still feed those by-products back to the cows. I believe it was Dr. Joe Gibbs, that said, the prion protein, can survive the digestinal track. So you have stopped nothing. It was proven in Oprah Winfrey's trial, that Cactus Cattle feeders, sent neurologically ill cattle, some with encephalopathy stamped on the dead slips, were picked up and sent to the renders, along with sheep carcasses. Speaking of autopsies, I have a stack of them, from CJD victims. You would be surprised of the number of them, who ate cow brains, elk brains, deer brains, or hog brains.

I believe all these TSE's are going to be related, and originally caused by the same greedy Industries, and they will be many. Not just the Renders, but you now see, that they are re-using medical devices that were meant for disposal. Some medical institutions do not follow proper auto- claving procedures (even Olympus has put out a medical warning on their endescopes about CJD, and the fact you cannot properly clean these instruments from TSE's), and this is just one product. Another route of infection.

Regardless what the Federal Government in the U.S. says. It's here, I have seen it, and the longer they keep sweeping it under the rug and denying the fact that we have a serious problem, one that could surpass aids (not now, but in the years to come, due to the incubation period), they will be responsible for the continued spreading of this deadly disease.

It's their move, it's CHECK, but once CHECKMATE has been called, how many thousands or millions, will be at risk or infected or even dead. You can't play around with these TSE's. I cannot stress that enough. They are only looking at body bags, and the fact the count is so low. But, then you have to look at the fact it is not a reportable disease in most states, mis-diagnosis, no autopsies performed. The fact that their one-in-a- million theory is a crude survey done about 5 years ago, that's a joke, under the above circumstances. A bad joke indeed........

The truth will come, but how many more have to die such a hideous death. It's the Government's call, and they need to make a serious move, soon. This problem, potential epidemic, is not going away, by itself.

Terry S. Singeltary Sr.

Bacliff, Texas 77518 USA


Competing interests: No competing interests


Terry S. Singeltary Sr., Bacliff, Texas USA, 7718 <flounder9@verizon.net>

posted by Terry S. Singeltary Sr. at 2:45 PM

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