Saturday, January 19, 2019

Tennessee CWD Update Given TFWC 2019 Meeting CWD Positives jump 62 cases total to date 86 confirmed

Tennessee 62 additional samples from Hardeman and Fayette counties have been confirmed CWD positive, apparently, from the additional 24 cases reported, bringing a total for Tennessee CWD confirmed to date 86 if my ciphering is correct...terry


Tennessee CWD Update Given at First TFWC 2019 Meeting 

Friday, January 18, 2019 | 09:26pm 

MEMPHIS --- An update on the status of chronic wasting disease (CWD) was presented during the first meeting of 2019 which concluded Friday at the Duck Unlimited national headquarters.

Chuck Yoest, CWD coordinator for the Tennessee Wildlife Resources Agency, made a presentation on the agency’s chronic wasting disease response. Yoest informed the commission the agency has sampled more than 4,800 deer this season in Tennessee. Also, he shared that 62 samples from Hardeman and Fayette counties have been confirmed CWD positive.

The agency anticipates more positive CWD results from these areas since it is placing a heavy emphasis on sampling there according to its response plan. Yoest also said that public meeting held in Bolivar earlier in January in regard to CWD, had an attendance of about 400.

Jamie Feddersen, TWRA migratory gamebird program leader, gave a preview of the 2019-20 season. Changes in the federal framework require the TWRA to update its proclamation each year. The changes presented at the meeting were based mainly on hunter input.

Anticipated federal rules will now allow states to have Jan. 31 as the last day of duck season. Previously federal rules prevented states from having duck season any later than the last Sunday in January. Hunter input indicates the desire to end the duck season Jan. 31. The agency recommends the Reelfoot Foot Zone phase 1 season be Nov. 16-19 and the statewide phase 1 season Nov. 29-Dec. 2. The Reelfoot and statewide zone phase 2 season would be Dec. 7-Jan. 31.

For woodcock season, there was support to provide more hunting days in January so the agency is proposing a split season. For crow, there was support for more hunting days in cooler weather, The agency is also proposing a split season for crow.

There was a presentation from Ducks Unlimited representative DU’s Dave Kostersky. On his annual visit from DU Canada, he reported that there was another dry fall and again officials were hoping for a wet spring in the habitat corridor. He annually makes the visit to provide an update on the partnership and conditions in Canada. 

Frank Fiss, TWRA Fisheries Division chief, presented an overview of the fish dealer license to address TFWC questions about license requirements. A fish dealer license is required for bait dealers, fish farmers and operators of pay lakes. In Tennessee, anglers who fish at a licensed pay lake are not required to have a fishing license. This exemption is common among most surrounding states. 

Dale Hall, Ducks Unlimited chief executive officer, was honored by the TFWC with a resolution. He has been DU’s top official since 2010 and previously served the U.S. Fish and Wildlife for 31 years, the four as its director.

 The commission will consider four rulemaking changes. The TWRA is establishing rules regarding public record requests and will consider changes to the fees associated with motorized boat registrations.

 The commission’s established a permanent Tennessee’s Native Son license. When initially created, the Native Son license had an expiration date of Feb. 28, 2019. The change allows the TWRA to continue selling the Native Son license into the future.

The commission passed an amended rule in regard to permit and access fees for non-motorized vessels the rule creates a permit for outfitters that establishes minimum operating standards and associated fees.

---TWRA--- 

MONDAY, JANUARY 07, 2019 

Tennessee TWRA 11 Additional Deer Preliminarily CWD Positive in Fayette and Hardeman Counties


SATURDAY, DECEMBER 15, 2018 

Tennessee Preliminarily Detects Ten Chronic Wasting Disease Cases; Enacts CWD Response Plan Friday, December 14, 2018


SATURDAY, DECEMBER 15, 2018 

Tennessee Preliminarily Detects Ten Chronic Wasting Disease Cases; Enacts CWD Response Plan Friday, December 14, 2018


WEDNESDAY, NOVEMBER 22, 2017

Tennessee Four Charged with Illegal Importation of Deer Carcasses from a CWD Positive State


Saturday, January 07, 2017 

Tennessee Republican representative Bud Hulsey wants to weaken CWD Carcass Ban rule and put other states at risk 


SATURDAY, APRIL 13, 2013

Tennessee Launches CWD Herd Certification Program in the wake of legislation for game farms


2013

Greetings Tennessean Hunters et al, and politicians,

well, the writing is on the wall Tennessee hunters.

it’s only a matter a time for Tennessee and CWD, and the big ag and officials can’t wait for it $$$

it’s only a matter of time now Tennesseans, and your state too will be full of CWD.

sad...


Monday, November 12, 2012

Tennessee The White-tailed Deer Breeding and Farming Act pushes to legalize deer farming 2012


Tennessee dad faces fatal, untreatable illness as family hopes for cure

Alexandria Hein By Alexandria Hein | Fox News

Tony Gibson, a 32-year-old welder and ironworker, initially showed symptoms of confusion and forgetfulness earlier this year. (iStock)

A Tennessee father who was given a year to live after being diagnosed with an extremely rare degenerative illness now requires 24-hour care at a nursing facility as his family hopes for a cure for the currently untreatable condition. Tony Gibson, a 32-year-old welder and ironworker, initially showed symptoms of confusion and forgetfulness earlier this year, his wife, Danielle, told News Channel 5.

Gibson was diagnosed with Creutzfeldt-Jakob Disease (CJD), which according to the National Institute of Neurological Disorders and Stroke, is a degenerative, fatal brain disorder that strikes in about one in a million per year, worldwide. It’s estimated that 350 Americans are diagnosed each year, although it’s typically diagnosed in older patients with symptoms beginning at around 60, and death occurring within one year.

Patients may first exhibit memory issues, behavioral changes, visual disturbances and lack of coordination before it advances to mental deterioration, blindness, weakness of extremities and coma. Gibson told the news outlet doctors believe her husband’s case is sporadic, which occurs in patients with no known risk factors and accounts for about 85 percent of all CJD cases. There are two other types of CJD, with one often compared to mad cow disease.

According to NINDS, symptoms of sporadic CJD are comparable to those of Alzheimer’s and Huntington’s disease, but deterioration occurs more quickly in CJD patients. While there are studies underway, no successful treatment has been developed.

In a Facebook post honoring CJD Awareness Day on Nov. 12, Gibson wrote that her husband went from being a strong man to a 90-year-old within months.

“This is the most devastating thing I’ve ever seen,” Danielle Gibson, who is caring for the couple’s four children at-home, told New Channel 5. “I’ve seen a lot of terrible things. I’ve seen ALS, but this has to be the worst.” 


SATURDAY, APRIL 13, 2013 

Tennessee Launches CWD Herd Certification Program in the wake of legislation for game farms


MONDAY, JANUARY 07, 2019 

Tennessee TWRA 11 Additional Deer Preliminarily CWD Positive in Fayette and Hardeman Counties


Tennessee TWRA CHRONIC WASTING DISEASE RESPONSE PLAN 

TWRA Chronic Wasting Disease Response Plan

Executive Summary

Chronic Wasting Disease (CWD) is a transmissible, fatal, neurological disease affecting members of the Cervidae (deer) Family. Common members of this family include white-tailed deer (Odocoileus virginianus), elk (Cervus elaphus canadensis), mule deer (Odocoileus hemionus), moose (Alces alces), caribou (Rangifer tarandus), red deer (Cervus elaphus elaphus), and fallow deer (Dama dama). Wild free-ranging members of the deer family found in Tennessee include white-tailed deer and elk. Currently, there are no known cases of CWD transmission to humans (Belay et al. 2004). However, the Centers for Disease Control (CDC) recently published guidance on human handling of venison harvested from areas with CWD, recommending that hunters submit a sample of their deer or elk harvested from a CWD endemic area for CWD testing prior to consuming the venison and avoiding consumption of known positive animals.

In the late 1960’s, CWD was first recognized in captive mule deer in Colorado. The disease has since been detected in Arkansas, Illinois, Iowa, Kansas, Maryland, Michigan, Minnesota, Mississippi, Missouri, Montana, Nebraska, New Mexico, New York, North Dakota, Ohio, Oklahoma, Pennsylvania, South Dakota, Texas, Utah, Virginia, West Virginia, Wisconsin and Wyoming. Additionally, CWD is present in the Canadian provinces of Alberta and Saskatchewan.

Monitoring for CWD in Tennessee began in 2002. To date, 12,282 free-ranging deer and 109 free-ranging elk have been tested for the disease. CWD has not been detected in Tennessee, but it remains crucial for the Tennessee Wildlife Resources Agency (TWRA) to remain vigilant in its efforts to keep the disease out of Tennessee, and continue sampling efforts to ensure early detection if it were to occur in the state. Additionally, appropriate and immediate actions will be warranted to limit the negative impacts of CWD if ever found in Tennessee.

The TWRA recognizes that CWD in Tennessee would have significant biological, ecological, economic and sociological implications. CWD represents a serious long-term threat to cervid populations in the state. The purpose of this response plan is to provide direction, guidelines and a specific course of action for monitoring and managing CWD in Tennessee, if it were to occur. This plan outlines four over-arching goals aimed at preventing and minimizing the impacts of CWD on native deer and elk populations in Tennessee:

● Prevention

o Employ appropriate preventive measures to minimize the risk of CWD entering the state.

● Early Detection

o Implement appropriate sampling strategies (methodologies, sample size and location) throughout the state to ensure early detection.

● Containment

o Implement appropriate monitoring strategies to determine prevalence and spatial distribution of CWD, if detected.

o Employ appropriate management actions that will limit the spread of CWD and eliminate or maintain the disease at a low prevalence, if detected.

o Determine the origin of any CWD positive cervid.

● Communications

o Distribution of accurate and effective information on CWD to the public, Agency staff, the Tennessee Fish and Wildlife Commission (TFWC) and other stakeholders.

Accomplishing these goals will minimize the impact of CWD on native white-tailed deer and elk in the state. The management of CWD will require a long-term commitment and adaptive approach that will be continually refined as the science of CWD detection and management advances.

Once established, eradication of CWD is unlikely due to the persistence of prions (the infectious agent) in the environment (Williams and Miller 2002). The TWRA CWD Response Plan focuses on prevention, early detection and control of the disease. In the event that CWD is detected in Tennessee, our plan provides details on TWRA actions that will be undertaken to contain and monitor the disease within a defined area. The Plan identifies response and support teams, with specific roles and responsibilities outlined. It also outlines internal and external notification procedures, as well as a Communications Strategy that will be employed throughout the different stages of CWD management. In operationalizing the plan, TWRA will seek the development of partnerships with private citizens and other governmental agencies to manage CWD if it were to occur in Tennessee.

I.INTRODUCTION 

snip...

Importation and possession of live white-tailed deer is illegal in Tennessee. 

However, whitetailed deer may be incidentally contained within a property with high enough fencing to prevent escape and there is no restriction on high-fencing of properties. Although white-tailed deer are likely contained within these properties, the ownership of these deer remains with the state.

TWRA is also responsible for permitting private big game wildlife preserves, but the regulatory authority for cervids other than white-tailed deer in these enclosures rests with the Tennessee TWRA Chronic Wasting Disease Response Plan TWRA CWD Response Plan update ‐ 2018 Page 8

Department of Agriculture (TDA)6

. Currently, there is a moratorium on the establishment of new private big game wildlife preserves.

Tennessee Department of Agriculture

Importation and possession of live cervids, other than white-tailed deer and wild elk, is legal in Tennessee. The TDA, including the State Veterinarian, has regulatory authority over these activities. More specifically, the State Veterinarian has authority over sanitary disposal of any dead animal, and disease related issues with all live animals. Furthermore, the State Veterinarian can order vaccination, quarantine and destruction of any animal.

It is not entirely known how many captive cervid facilities exist in the state since they are not required to be registered or permitted unless they are involved in interstate movement of CWD susceptible cervids. Operators of cervid facilities involved in interstate movement of CWD susceptible species are required to participate in TDA’s CWD Herd Certification Program (HCP) (Appendix B). Participating in the CWD HCP is voluntary for those facilities not involved in interstate movement of Cervidae. Therefore, some captive cervid facilities are unknown by TDA and TWRA. As a result, TWRA is working to identify the locations of all captive cervid facilities in Tennessee and map them to assist with CWD prevention and/or control efforts.

United States Department of Agriculture Veterinary Services

If CWD is found in a captive cervid herd, the United States Department of Agriculture (USDA) Veterinary Services will work in concert with the State Veterinarian to develop a herd plan outlining protocol for animal movement into and out of the facility and possible euthanasia, disposal, indemnity, etc. In a case where white-tailed deer have been incidentally contained within a CWD-positive captive cervid facility, TWRA will work with USDA and the State Veterinarian to properly manage these whitetails following a USDA herd plan. 

II. PRE-DETECTION PREPARATION

Regulatory Action

There is no vaccination or treatment for CWD, thus prevention is the first and most effective strategy for minimizing the chances of CWD becoming established in Tennessee. TWRA has implemented several regulatory mechanisms to minimize the chances that a CWD-positive animal (live or dead) will be brought into the state. First, as noted previously, the importation and possession of live white-tailed deer and wild elk into Tennessee is prohibited by state statute 6 Tennessee Code Annotated, Title 70, Chapter 4 ‐ 70‐4‐403

TWRA Chronic Wasting Disease Response Plan

TWRA CWD Response Plan update ‐ 2018 Page 9

Second, TWRA regulates the shooting of captive cervids on Private Wildlife Preserves. Since 2009, a moratorium has been placed on the establishment of new big game wildlife preserves. Third, TWRA restricts the transportation of cervid carcasses from areas outside Tennessee to include only a) meat that has bones removed; b) antlers, antlers attached to cleaned skull plates, or cleaned skulls; c) cleaned teeth; d) finished taxidermy and antler products; e) hides and tanned products. Finally, TWRA established regulations to prohibit the use or possession of cervid urine, except synthetic, for hunting, to become effective March 1, 2019.

If CWD is detected in Tennessee, TWRA will shift its focus to active monitoring and containment of the disease in the area where the detection is made (Note: CWD surveillance activities will continue in the remainder of the state, in an effort to maintain an early detection system for CWD). The following regulatory options will be considered as strategies to minimize its prevalence in the population and to contain its spread:

● A ban on feeding and rehabilitating wild cervids in defined TWRA CWD Management Zone (CMZ).

● Ban on removal of cervid carcasses and parts from defined TWRA CWD Management Zone.

● Mandatory sampling of hunter-harvested deer and elk from within TWRA CWD Management Zone at physical checking stations.

● Increase deer bag limits, extended deer seasons and/or allow additional weapon types in TWRA CWD Management Zone(s).

● TWRA-sanctioned culling actions in TWRA CWD Management Zone.

● Mandate disposal requirements for hunter-killed cervids taken in TWRA CWD Management Zone.

Surveillance Sampling

The TWRA Disease Coordinator will be responsible for keeping an inventory of equipment and supplies for CWD sampling (Appendix C) and a CWD response (Appendix D). These items will be distributed to field staff as needed or upon request.

Pre-CWD

To facilitate TWRA’s goal of early detection of CWD, sampling efforts is incorporating a risk-based weighted surveillance strategy that takes into account 1) TWRA biologists’ perceptions of risk factors, 2) surrounding states’ surveillance, regulations, and CWD status, and

3) a framework for future CWD sampling (Appendix A). Obex and retropharyngeal lymph node samples will be taken from elk, while only retropharyngeal tissues will be collected from whitetailed deer. If a sufficient number of samples cannot be obtained by TWRA alone in some areas, 

TWRA Chronic Wasting Disease Response Plan TWRA CWD Response Plan update ‐ 2018 Page 10

taxidermists and meat processors receiving cervids and/or cervid parts from these areas may be enlisted to provide additional animals for sampling.

Early detection of CWD can also be enhanced by focusing more intensive sampling on reportedly sick deer and elk. To facilitate this and increase awareness of sick deer and elk, the Agency will encourage the public to report cervids appearing unhealthy and whenever possible, these animals will be sampled and tested. In cases where sampling cannot occur, the location of the reportedly sick cervid will be documented.

Post-CWD

In the event of a confirmed positive detection of CWD, enhanced sampling will commence in a defined CWD Management Zone, with sampling procedures similar to those outlined in Appendix A. The TWRA Wildlife Disease Coordinator, TWRA Wildlife Veterinarian, and CWD Response Team leader (i.e., Asst. Chief of Wildlife & Forestry) will consult on the appropriate sample size and methodologies for obtaining samples, depending on the situation, within the CWD Management Zone. The purpose of this monitoring will be to establish a baseline prevalence rate and distribution of CWD within the CWD Management Zone.

III. RESPONSE TO A CWD POSITIVE

Notification Process

The TWRA Disease Coordinator and/or UT/TWRA Wildlife Veterinarian, upon receiving notification of a preliminary CWD positive sample from a USDA-approved laboratory, will ensure key personnel within the Agency and the Tennessee Fish and Wildlife Commission (TFWC) are notified. This will include but not be limited to the Executive Director, the Assistant Director of Field Operations, the Chief of the Wildlife and Forestry Division (WFD), the Chief of the Boating and Law Enforcement Division, the Deer and Elk Program Leaders, and Captive Wildlife Program Coordinator, the Regional Wildlife Program Managers, the Chairman of the TFWC, and the Chairman of the Wildlife Management Committee of the TFWC (Appendix E).

The above mentioned personnel will be made aware that a preliminary positive case of CWD has been found in Tennessee via ELISA test and a confirming IHC test is being conducted at the USDA Veterinary Services Laboratory in Ames, Iowa. If the confirmatory test results in a nondetect finding, no further action besides proper notifications will be taken. If the original sample is insufficient for a confirmatory test, the initial positive test will be accepted as a positive for an index case.

Upon notification of a confirmed positive CWD test result by a USDA-approved laboratory, the TWRA Disease Coordinator will immediately notify the Executive Director, the Chief of the 

TWRA Chronic Wasting Disease Response Plan TWRA CWD Response Plan update ‐ 2018 Page 11

Wildlife & Forestry Division and the Assistant Director of Field Operations; intradepartmental notification will proceed as outlined in Appendix F. The TWRA Executive Director will notify the Governor’s Office, all TFWC members, the Commissioner of Agriculture, the Commissioner of the Department of Health, the Commissioner of the Department of Environment and Conservation, the Tennessee State Veterinarian, and the U.S. Fish and Wildlife Service. Concurrently, the Assistant Director of Field Operations’ designees will notify the relevant Agency partners and the wildlife chiefs in bordering states. Also, TWRA’s Legislative Liaison will contact legislators in district(s) where the CWD-positive case occurred, prior to a statewide news release being distributed by TWRA’s Chief of Communications and Outreach. The news release will include a media packet providing background information on CWD, a synopsis of TWRA’s CWD sampling efforts, and any other CWD-related materials deemed appropriate.

The Chief of the Wildlife & Forestry Division and Chief of Communications and Outreach and/or their designees will be assigned as TWRA CWD media contacts through which all CWDrelated questions from the public and the media will be routed, including public appearances and interviews. These persons will comprise the TWRA’s CWD Media Team (Appendix G). The Media Team will develop and use specific talking points when interacting with the media and members of the public about CWD. Along with TWRA staff, TFWC members should also direct media related requests and public inquiries to the TWRA Media Team. The Media Team is also responsible for TWRA’s social media communication regarding CWD. Other TWRA staff and TFWC members should refrain from posting/commenting on CWD matters and leave all CWD-related communications to the Media Team. Lastly, the Media Team is responsible for coordinating well-advertised CWD public meetings to be held in the area of the CWD detection.

Response Teams

The Assistant Director of Field Operations or their designee will serve as the CWD Administrative Team Leader (Appendix H). Additional Agency leadership will serve on the CWD Administrative Team to support the Field Response Team’s (Appendix I) activities. The CWD Administrative Team leader will immediately activate the CWD Field Response Team upon positive confirmation of CWD in the state. The CWD Field Response Team may include cervid collection teams, carcass transport teams, and biological data collection teams. The CWD Field Response Team will be led by the Assistant Chief of the Wildlife & Forestry Division and will include appropriate field staff, as deemed necessary for the specific situation and response (Appendix I).

Management Actions

Once established, eradication of CWD from a locality is difficult due to the persistence of prions in the environment. Therefore, once a CWD-positive is detected in Tennessee or in an 

TWRA Chronic Wasting Disease Response Plan TWRA CWD Response Plan update ‐ 2018 Page 12

area within 25 miles of the border, TWRA will initiate its response efforts focused on determining the prevalence and distribution of CWD within a defined CWD Management Zone. The goals of these efforts will be containment and management of the disease, including eradication if feasible. There are at least 3 likely scenarios of CWD detection in Tennessee that would precipitate management actions by TWRA: 1) Detection in a free-ranging white-tailed deer or elk within the borders of Tennessee; 2) Detection in a captive cervid facility within Tennessee, and; 3) Detection in a free-ranging or captive cervid in an adjacent state, within 25 miles of the Tennessee border.

Detection in a free-ranging wild cervid within the borders of Tennessee:

● Once CWD has been confirmed in a wild cervid, a CWD Management Zone (CMZ) will be developed. The Management Zone will be comprised of a Core Zone (approximately 5-mile radius), a 10-mile High Risk Zone, and a 25-mile Buffer Zone (Figure 2).

● The Field Response Team will obtain all supplies and materials needed to conduct field activities from the respective TWRA regional office, or supplies will be drop shipped to the specific location where needed.

● A CWD Monitoring Strategy will be employed, with the objective of determining the prevalence and distribution of the disease within the CWD Management Zone.

● Special regulations for the CMZ may be adopted by the TFWC, as indicated in Regulatory Actions (pages 8-9). For example, if the first positive occurs during an archery-only or muzzleloader/archery deer hunting season, a special gun hunting season may be utilized to supplement harvest and CWD sampling in the CMZ. To help facilitate sample collection during deer hunting seasons, mandatory check stations may be established within the CMZ.

● Hunter-harvested deer within the CMZ will be sampled, and individually identified.

CWD test results will be available to hunters once they are finalized and accessible by the Agency.

● If needed, processors and taxidermists within the CMZ will be recruited to obtain additional biological samples of harvested cervids for CWD testing.

● If needed, additional testing within the CMZ will commence as soon as possible. The Field Response Team will contact private landowners obtaining permission to access private lands within the CMZ to secure additional samples through TWRA collection operations. Agency staff will also coordinate with managers of any public lands within the CMZ to obtain access and samples as needed. Samples will also be collected from road-killed specimens, and sampling of sick or cervids found dead.

● All sampling locations will be recorded using GPS coordinates and entered into a GIS database maintained by TWRA’s GIS staff.

● If an additional positive animal(s) is identified within the CMZ, the CMZ will be expanded as appropriate to ensure that all areas of potential CWD presence are included 

TWRA Chronic Wasting Disease Response Plan TWRA CWD Response Plan update ‐ 2018 Page 13

in the CMZ. Consultation with the TWRA Wildlife Veterinarian, TWRA Wildlife Disease Coordinator, Tennessee State Veterinarian, and others will help to determine the appropriate size and extent of the CWD Management Zone.

● The CWD Response Team will coordinate with the TDA and USDA to conduct inspections and monitoring of all captive cervid facilities within a CWD Management Zone.

● Response efforts will be scaled back if additional positives are not detected after five full sampling seasons following the last positive detected. Appropriate hunting regulations during and after the 5-year response period will be determined by the TWRA based on the estimated cervid population size at that time.

Figure 2. Example CWD Management Zone (with 5-mile Core Zone, 10-mile High Risk Zone, and 25-mile Buffer Zone), and counties.

Detection in a captive cervid facility within Tennessee:

Captive cervids (excluding incidentally fenced white-tailed deer) are regulated by the TDA and USDA. However, a CWD-positive cervid from a captive facility will activate TWRA’s CWD Response Plan, with the CWD-positive captive facility serving as the center point of the Core Zone and CMZ. We recommend the following actions be considered/taken by the appropriate partners if CWD is detected in a captive cervid facility within Tennessee, or if a captive cervid facility has transferred or received cervids within the past 5 years from an out-ofstate captive facility in which CWD is detected: 

TWRA Chronic Wasting Disease Response Plan TWRA CWD Response Plan update ‐ 2018 Page 14

● TDA and/or USDA ensure fences are maintained to avoid possible escapes. Additionally, recommend double fencing to exclude wild cervids from direct contact with cervids inside the enclosure.

● Immediate quarantine of the facility to ensure no cervid movement in or out of the enclosure. ● Depopulation and sanitary disposal of all cervids in the facility and decontamination of the facility to the maximum extent possible. TWRA may depopulate and test any incidentally contained white-tailed deer.

● Epidemiological investigation of cervids in contact with CWD infected animals to determine the origin and prevent further infection.

● If records indicate a captive cervid from such a facility leaves Tennessee, then the final destination state wildlife agency will be notified along with other relevant agencies.

● If the facility is a shooting preserve, shooters who may have killed any cervids within the facility will be notified that the facility tested positive for CWD.

● Wild cervid sampling outside the facility will be conducted in accordance with the guidelines and procedures described in the Detection in a free-ranging wild cervid within the borders of Tennessee section of this plan.

Detection in a free-ranging or captive cervid in an adjacent state, within 25 miles of the Tennessee border

● The TWRA will coordinate with the state wildlife agency in the adjoining state where CWD has been detected. TWRA staff will obtain information from that state’s CWD response program.

● If the index location is within 25 miles of the Tennessee border, a CWD Management Zone will be established within that portion of a 25-mile radius falling within Tennessee. Response procedures will be implemented using the protocol(s) outlined in the Detection in a free-ranging wild cervid within the borders of Tennessee section of this plan. 

snip...

see full text;


New Requirements for Captive Deer Herds Following CWD Detection 

Monday, December 24, 2018 | 09:43am  

NASHVILLE —After the confirmed detection of chronic wasting disease (CWD) in ten wild deer, Tennessee Department of Agriculture (TDA) Commissioner Jai Templeton is implementing emergency rules to prevent further spread of the disease.

Hunters harvested the deer in Fayette and Hardeman counties. Targeted sampling by the Tennessee Wildlife Resource Agency (TWRA) indicated the presence of CWD.

CWD has no known risk to the health of humans or livestock. However, testing is recommended prior to consuming deer or elk meat harvested within the CWD Management Zone, which includes Fayette, Hardeman, and McNairy Counties. CWD is a contagious and deadly neurological disorder that affects cervids, which are animals in the deer family including deer, elk, moose, caribou, and reindeer.

“We have been working hard to prepare for this potential threat,” Commissioner Templeton said. “In collaboration with TWRA, the United States Department of Agriculture, hunters, and captive herd owners, we have developed a response plan. That plan is critical in protecting the wild and captive deer and elk in our state.”

With the new emergency rules in place, owners of captive deer and elk will be required to report their herd inventory, location, and any sick animals to the State Veterinarian. They will also be required to report deaths among their fenced captive cervids within 24 hours and make the carcass available to TDA for further testing.

Additionally, the importation of captive cervids into the state and the movement of captive deer or elk within the state require prior approval and a permit from the State Veterinarian, as well as USDA-approved identification. The requirements from the new emergency rule do not apply to white-tailed deer and wild elk, which are prohibited from being retained in captive facilities.

“Just a few weeks before this detection, we joined TWRA, USDA, and other partners for a tabletop exercise to discuss and finalize a response plan,” State Veterinarian Dr. Charlie Hatcher said. “Now, we’re following through. We encourage captive herd owners to keep a close eye on the animals in their care and report any signs of illness immediately.”

A voluntary program administered by TDA, the Tennessee CWD Herd Certification Program was put in place in 2013 to provide uniform herd certification standards and to support the domestic and international marketability of cervid herds. Facilities can be certified as disease-free after five years of program enrollment with no evidence of disease, and the program is required for the interstate movement of CWD-susceptible cervids. For more information about CWD, visit CWDinTennessee.com.



8-2007 An Experimental Release of Elk into Great Smoky Mountains National Park 

Jennifer Lynn Murrow University of Tennessee - Knoxville


Four Charged with Illegal Importation of Deer Carcasses from a CWD Positive State 

Wednesday, November 22, 2017 | 10:19am

Four Charged with Illegal Importation of Deer Carcasses from a CWD Positive State

MORRISTOWN --- Tennessee Wildlife Resources Agency wildlife officers have filed charges on four hunters who illegally imported white-tailed deer carcasses from a state with the confirmed presence of Chronic Wasting Disease (CWD). The hunters allegedly brought the entire deer carcasses into Tennessee which had not been properly prepared as required by law.

On the opening day of muzzleloader season, Nov. 11, and the opening day of rifle season, Nov. 18, Carter County TWRA wildlife officers Dennis Ward and John Ripley charged four hunters with illegally importing deer carcasses from Virginia, a state that confirmed the presence of CWD in 2009. Last year, only portions of Virginia where CWD had been detected were banned. However, this year, importation restrictions apply to the entire state.

In the effort to help prevent CWD from entering Tennessee, TWRA has placed importation restrictions for cervids, including deer, moose, and elk carcasses from any state that has found a positive case of CWD. Carcasses and other cervid parts from the CWD-positive states that may be brought into or possessed in Tennessee include:

Meat that has bones removed Antlers, antlers attached to clean skull plates, or cleaned skulls (no meat or tissues) Cleaned teeth Finished taxidermy, hides and tanned products

A list of states and Canadian provinces that are included in the restriction can be found at http://www.tn.gov/twra/article/cwd-carcass-importation-restrictions

---TWRA---

From TWRA Region IV, Morristown


News Release 

Tennessee Attorney Pleads Guilty to Illegally Killing Trophy White-Tailed Deer in Illinois 

February 4, 2008 Contact: Division of Public Affairs External Affairs Telephone: 703-358-2220 Website: https://www.fws.gov/external-affairs/public-affairs/ 

Allen W. Blevins, 41, of Knoxville, Tenn., pleaded guilty today in federal court to illegally killing and transporting white-tailed deer, a misdemeanor violation of the Lacey Act, a federal wildlife protection law. Blevins admitted to illegally killing three deer while he was employed as a guide at Hadley Creek Outfitters, a business located in Pike County, Illinois, and agreed to forfeiture of three illegally-taken deer head mounts.

The plea was accepted by U.S. Magistrate Judge Byron Cudmore who then ordered Blevins to pay a $7,500 fine and forfeit the mounts of the illegally killed deer. The fine will be used to fund continuing state and federal investigations of wildlife law violations.

In October 2004, Blevins used a bow and arrow to kill a trophy 10-point white-tailed deer in Pike County, Ill. Blevins then illegally transported the deer to Tennessee where he lied to officials and falsified documents to make it appear he had killed the deer in Tennessee. Blevins then returned to Illinois and illegally killed and transported two more deer, including another trophy 10-point buck during the month of November 2004

Blevins, an attorney and founding partner of Blevins, Kizer and Gammeltoft, P.C., of Knoxville, Tenn., was employed as a guide for Hadley Creek Outfitters at the time he illegally killed and transported the deer. Photos of the trophy deer illegally killed by Blevins were posted on Hadley Creek Outfitters website for promotional uses. The mounts of these illegally killed deer were also displayed in public to promote other Blevins’ business ventures. In addition to being an attorney and hunting guide, Blevins is also listed as the President of Whitetail Investment Properties, an investment group that uses filmed deer hunts to sell real estate on numerous outdoor and hunting television shows.

Blevins’ guilty plea is the result of an investigation into illegal hunting conducted by special agents and investigators of the U.S. Fish and Wildlife Service, the Illinois Department of Natural Resources and the Tennessee Wildlife Resources Agency.

“The willingness of the U.S. Attorney’s Office to prosecute cases such as this one helps us protect our nation’s natural resources,” said U.S. Fish and Wildlife Service Special Agent Tim Santel. “If all self-professed hunters acted with blatant disregard for wildlife laws as Blevins did, there would be no trophy animals left to hunt.”

The Central District of Illinois’ U.S. Attorney’s Office, represented by Assistant U.S. Attorney Gregory M. Gilmore, negotiated the plea agreement.

The interstate transportation of wildlife-- including hides or parts--obtained in violation of state law violates the Lacey Act. The Lacey Act is a federal wildlife protection law and each violation carried a possible maximum fine of $100,000 and/or one year in prison.

The mission of the U.S. Fish and Wildlife Service is working with others to conserve, protect and enhance fish, wildlife, plants and their habitats for the continuing benefit of the American people. We are both a leader and trusted partner in fish and wildlife conservation, known for our scientific excellence, stewardship of lands and natural resources, dedicated professionals and commitment to public service. For more information on our work and the people who make it happen, visit http://www.fws.gov

3-FWS-


FOR IMMEDIATE RELEASE 

Thursday, February 28, 2013 

Professional Hunter To Pay $10,000 Fine For Lacey Act Violation In Kansas 

KANSAS CITY, KAN. – A professional hunter from Tennessee will has been sentenced to three years on federal supervised release for a Lacey Act violation in Kansas, U.S. Attorney Barry Grissom said today. The hunter will lose his hunting privileges throughout the United States for six months, as well as paying a $10,000 fine and $10,000 in restitution.

William “Spook” Spann, 50, Dickson, Tenn., pleaded guilty to a misdemeanor count of transporting across state lines wildlife that was taken unlawfully in Kansas. In his plea, Spann admitted that in mid-November 2007 he unlawfully took a white-tailed deer in Stafford County, Kan. Spann took the deer on land owned by another person, in violation of Spann’s hunting permit, which entitled him to hunt only on land that he owned.

On a scouting trip, Spann and a cameraman spotted a deer at a distance of several hundred yards with the wind blowing in their faces so that the deer would not be able to hear or smell their approach. With a video camera rolling, Spann stalked to within 10 yards of the deer. Spann drew his bow and killed the deer with an arrow.

Federal investigators served a search warrant at Spann’s home in Tennessee, where they seized the antlers of the Kansas deer. 

Grissom commended the U.S. Fish and Wildlife Service, the Kansas Department of Wildlife, Parks and Tourism, the Tennessee Wildlife Resources Agency and Assistant U.S. Attorney Chris Oakley for their work on the case.


2 0 1 6 Annual Law Enforcement and Violation Report 47

ILLEGAL HUNTING IN GMU 61

Two men from Colorado and two men from Tennessee have been convicted of illegal hunting activities. As a result, two of them paid substantial fines; one man will spend time in jail and another is performing community service. All of the men could also lose their hunting and fishing privileges in Colorado and in 43 other states.

The convictions followed long-term investigations by Colorado Parks and Wildlife and the U.S. Fish and Wildlife Service. The violations were committed over the course of a decade in prized Game Management Unit 61 where few elk licenses are available each season and many people wait 20 years or more to draw a tag. GMU 61 is located on the Uncompahgre Plateau west of Montrose.

“We take it seriously when poachers steal wildlife from all of us, especially when they are profiting from that poaching; and we will do everything we can to see that those individuals are brought to justice,” said Renzo DelPiccolo, Area Wildlife Manager for Colorado Parks and Wildlife in Montrose. “Sometimes it takes years to investigate and settle wildlife cases, but that does not deter state and federal investigators from pursuing these crimes.”

Beginning in about 1999 and continuing through 2011, Gerald Lee Sickels (“Sickels”), 42, of Nucla, operated as an illegal, unlicensed outfitter and took clients on multi-day hunts for which he charged $1,000 to $3,000. During that time, at least 17 bull elk were killed illegally in GMU 61 by Sickels and his out-of-state clients. At least one mountain lion was also killed illegally. Sickels instructed his clients to purchase other hunting licenses to help cover up the illegal activity.

Sickels and his assistant, Jay Remy Grierson (“Grierson”), 46, also of Nucla, were indicted by a federal grand jury in November 2014 for violations of the Lacey Act, a federal law that bans illegal trafficking of wildlife. They faced six counts of conspiracy and interstate sale of unlawfully taken big-game.

Sickels eventually pleaded guilty to one felony count of conspiring to violate the Lacey Act. On Nov. 7, 2016, he was sentenced in federal court in Denver to one year of “intermittent incarceration” and one year of probation for conspiring to violate the Lacey Act. Sickels must report to a local detention facility on all nonwork days, on all vacation days, and on all holidays during the one-year period. During the probation, he is prohibited from hunting or acting as a hunting guide. He also had to give up his 1997 Toyota pick-up truck and a Fleetwood camping trailer, both of which were used in the commission of federal crimes.

2 0 1 6 Annual Law Enforcement and Violation Report 48

Grierson pleaded guilty to three misdemeanor violations of the Lacey Act and was sentenced in March 2016 to two years of probation and 40 hours of community service.

Ben Williamson (“Williamson”), 61, of Morristown, Tennessee, during a trip in 2004 to GMU 61, unlawfully killed two elk, one a 6x6 and the other a 7x8. In 2009, his son, Brett Williamson, 26, who did not have a hunting license, killed a 6x6 bull elk. He returned in 2010 and, again without a license, killed two 6x6 bull elk. The two men were charged with misdemeanor violations of the Lacey Act and each paid a fine of $6,500. They also were required to forfeit their trophy mounts.

Officers from the Tennessee Department of Wildlife Resources assisted in the investigation by conducting interviews and seizing evidence.

The Colorado Parks and Wildlife Commission Hearing Examiner will review each case and make a determination regarding suspension of the men’s hunting and fishing license privileges. Through a nationwide cooperative agreement known as the Interstate Wildlife Violator Compact, the men could lose their license privileges in the other 43 participating states.

The case was prosecuted by the Environmental Crimes Section of the U.S. Department of Justice, Environment and Natural Resources Division.


FOR IMMEDIATE RELEASE 

Thursday, September 13, 2018 

Two Louisiana Men Sentenced for Smuggling Live White-Tailed Deer into Mississippi Hattiesburg, Miss. – 

Edward L. Donaldson Jr., 75, and John Jared Oertling, 42, both residents of Pearl River, St. Tammany Parish, Louisiana, were sentenced in Hattiesburg Tuesday on charges of conspiring to smuggle live white-tailed deer into Mississippi, announced U.S. Attorney Mike Hurst and Special Agent in Charge Luis Santiago of the U.S. Fish and Wildlife Service, Office of Law Enforcement.

United States District Judge Keith Starrett sentenced Donaldson and Oertling each to three years of probation, a fine of $10,000, and a worldwide prohibition from hunting of any type for one year. Oertling was also sentenced to serve 6 months of house arrest under location monitoring. Judge Starrett further ordered that the 1,031 acre high-fenced enclosure, known as Turkey Trot, and owned by Donaldson’s daughter and Oertling’s wife, is to be quarantined by the Mississippi Department of Wildlife, Fisheries, and Parks for 5 years and the white-tailed deer inside are to be subjected to testing for chronic wasting disease paid for by Donaldson and Oertling in the amount of $120,000.

U.S. Fish and Wildlife Service Special Agent in Charge Luis Santiago stated: "We take our mission working with the Mississippi Department of Wildlife, Fisheries, and Parks and the citizens of Mississippi in conserving, protecting, and enhancing fish, wildlife, plants and their habitats very seriously. The U.S. Fish and Wildlife Service, Office of Law Enforcement, considers the potential spread of disease caused by the illegal commercialization of wildlife resources a high priority, and we will continue to work closely with our State partners to assist them in these important investigations."

This case was investigated by the U.S. Fish and Wildlife Service, Office of Law Enforcement, U.S. Department of Agriculture – Office of the Inspector General, and the Mississippi Department of Wildlife, Fisheries, and Parks. It is being prosecuted by Assistant United States Attorneys Darren J. LaMarca and Dave Fulcher.


FOR IMMEDIATE RELEASE 

Thursday, September 14, 2017 

Two Louisiana Residents Charged with Smuggling Live White-Tailed Deer into Mississippi 

Hattiesburg, Miss. – Edward L. Donaldson Jr., 75, and John Jared Oertling, 42, both residents of Pearl River, St. Tammany Parish, Louisiana, were charged in a Criminal Information with conspiracy to violate the Lacey Act for importing live white-tailed deer into Mississippi, announced Acting U.S. Attorney Harold Brittain and Special Agent in Charge Luis Santiago of the U.S. Fish and Wildlife Service, Office of Law Enforcement.

Mississippi law makes it unlawful to import live white-tailed deer into the State of Mississippi and authorizes the Mississippi Department of Wildlife, Fisheries and Parks Commission with the responsibility of establishing regulations governing the importation of white-tailed deer with the emphasis on preventing the introduction of disease. The Commission established a regulation that mirrors the state statute, prohibiting the importation of live white- tailed deer into the State of Mississippi. The Lacey Act makes it unlawful for any person to import, export, transport, sell, receive, acquire or purchase wildlife that were taken, possessed, transported or sold in violation of any law or regulation of any state.

According to the Criminal Information, from February of 2010 through November of 2012, Edward L. Donaldson Jr., and John Jared Oertling did knowing and willfully conspire to transport live white-tailed deer in interstate commerce in violation of state and federal laws. Donaldson Jr. and Oertling manage a 1,031-acre high fenced enclosure in Forrest County, Mississippi known as Turkey Trott Ranch. Jill Marie Donaldson, wife of Oertling and daughter of Donaldson is the owner of Turkey Trott Ranch.

The Information also alleges that it was part of the conspiracy that the defendants would unlawfully smuggle the live white-tailed deer into Mississippi for the purpose of breeding and killing trophy white-tailed buck deer. Donaldson Jr. and Oertling learned in 2012 that the live white-tailed deer purchased and imported from Pennsylvania to Turkey Trott Ranch in Forrest County, Mississippi came from a herd of captive white-tailed deer in PA that tested positive for Chronic Wasting Disease (CWD).

Chronic Wasting Disease (CWD) is the chief threat to deer and elk populations in North America. The disease, which ultimately ends in death of infected animals, is a transmissible neurological disease that produces small lesions in the brain of deer and elk and is characterized by loss of body condition and behavioral abnormalities.

"This case demonstrates our continuing commitment, together with our federal and state law enforcement partners, to investigate and prosecute those who choose to violate the federal Lacey Act by illegally importing white-tailed deer into our state," said Acting U.S. Attorney Brittain.

U.S. Fish and Wildlife Service Special Agent in Charge Luis Santiago stated "We take our mission working with the Mississippi Department of Wildlife, Fisheries, and Parks and the citizens of Mississippi in conserving, protecting, and enhancing fish, wildlife, plants and their habitats very seriously. The U.S. Fish and Wildlife Service, Office of Law Enforcement considers the potential spread of disease caused by the illegal commercialization of wildlife resources a high priority, and we will continue to work closely with our State partners to assist them in these important investigations."

This case was investigated by the U.S. Fish and Wildlife Service, Office of Law Enforcement, U.S. Department of Agriculture – Office of the Inspector General, and the Mississippi Department of Wildlife, Fisheries, and Parks. It is being prosecuted by Criminal Division Chief Darren J. LaMarca.


FOR IMMEDIATE RELEASE 

Tuesday, October 17, 2017 Two 

Louisiana Residents Plead Guilty to Smuggling Live White-Tailed Deer into Mississippi 

Hattiesburg, Miss. – Edward L. Donaldson Jr., 75, and John Jared Oertling, 42, both residents of Pearl River, St. Tammany Parish, Louisiana, pled guilty today to conspiracy to violate the Lacey Act for importing live white-tailed deer into Mississippi, announced U.S. Attorney Mike Hurst and Special Agent in Charge Luis Santiago of the U.S. Fish and Wildlife Service, Office of Law Enforcement.

Mississippi law makes it unlawful to import live white-tailed deer into the State of Mississippi and authorizes the Mississippi Department of Wildlife, Fisheries and Parks Commission with the responsibility of establishing regulations governing the importation of white-tailed deer with the emphasis on preventing the introduction of disease. The Commission established a regulation that mirrors the state statute, prohibiting the importation of live white- tailed deer into the State of Mississippi. The Lacey Act makes it unlawful for any person to import, export, transport, sell, receive, acquire or purchase wildlife that were taken, possessed, transported or sold in violation of any law or regulation of any state.

Donaldson and Oertling admitted to United States District Judge Keith Starrett to purchasing and transporting live white-tailed deer into Mississippi in violation of state and federal law from February 2010 through November 2012. Donaldson and Oertling manage a 1,031 acre high fenced enclosure in Forrest County, Mississippi, known as Turkey Trott Ranch .

Donaldson and Oertling admitted that the live white-tailed deer purchased and imported from Pennsylvania to Turkey Trott Ranch in Forrest County, Mississippi, came from a herd of captive white-tailed deer in Pennsylvania that tested positive for Chronic Wasting Disease (CWD).

Chronic Wasting Disease (CWD) is the chief threat to deer and elk populations in North America. The disease, which ultimately ends in death of infected animals, is a transmissible neurological disease that produces small lesions in the brain of deer and elk and is characterized by loss of body condition and behavioral abnormalities.

This is the third such case brought by federal authorities against South Mississippi landowners caught importing white-tailed deer since February of 2014. "The illegal transportation and importation of live animals across state lines can have a potentially devastating impact on the health and safety of our citizens. This case demonstrates our continuing commitment, together with our federal and state law enforcement partners, to hunt down and prosecute those who choose to violate federal law," said U.S. Attorney Hurst.

U.S. Fish and Wildlife Service Special Agent in Charge Luis Santiago stated: "We take our mission working with the Mississippi Department of Wildlife, Fisheries, and Parks and the citizens

of Mississippi in conserving, protecting, and enhancing fish, wildlife, plants and their habitats very seriously. The U.S. Fish and Wildlife Service, Office of Law Enforcement considers the potential spread of disease caused by the illegal commercialization of wildlife resources a high priority, and we will continue to work closely with our State partners to assist them in these important investigations."

This case was investigated by the U.S. Fish and Wildlife Service, Office of Law Enforcement, U.S. Department of Agriculture – Office of the Inspector General, and the Mississippi Department of Wildlife, Fisheries, and Parks. It is being prosecuted by Criminal Division Chief Darren J. LaMarca.


FOR IMMEDIATE RELEASE

Monday, August 3, 2015

Father and Son Sentenced for Illegal Deer Trafficking

COLUMBUS, Ohio – Donald W. Wainwright Sr., 49, of Live Oak, Fla., was sentenced in U.S. District Court to 21 months in prison and a $125,000 fine for 12 charges related to violating the Lacey Act, one count of conspiracy and one count of wire fraud. His son, Donald W. Wainwright, Jr., 29, of Live Oak, Fla., was sentenced to four months of house arrest and three years of probation for eight charges related to violating the Lacey Act.

Carter Stewart, U.S. Attorney for the Southern District of Ohio, Gregory Jackson, Special Agent in Charge, United States Fish and Wildlife Service Office of Law Enforcement, Chief Scott Zody, Ohio Department of Natural Resources Division of Wildlife, Franklin County Prosecutor Ron O’Brien, the Florida Fish and Wildlife Conservation Commission and Georgia Department of Natural Resources announced the sentences handed down by U.S. District Chief Judge Edmund A. Sargus, Jr.

According to court documents, the co-conspirators trafficked in live white-tailed deer. Wainwright Sr. owned hunting preserves in Logan County, Ohio, and Live Oak, Florida; both preserves were named Valley View Whitetails. Wainwright Jr. was part-time resident and part-time operator of the site in Ohio.

Wainwright Sr. illegally shipped deer to Florida from Ohio and attempted to ship deer to Georgia from Ohio. The deer herds involved with these shipments were not certified to be free from chronic wasting disease, tuberculosis and brucellosis. Federal Law requires interstate shipment of deer to be certified to be disease free. As a result, deer herds in Florida were potentially exposed to these diseases. His attempted shipment to Georgia was intercepted on I-71 South, about 50 miles from the Ohio River, when Ohio Wildlife officers noticed deer noses and antlers inside a cargo trailer and pulled over a truck driven by Wainwright Sr.’s employees.

“Trophy-sized white-tailed deer can sell for hundreds of thousands of dollars apiece if the animals come from herds that have been certified by government agricultural officials to be free from disease,” U.S. Attorney Stewart said. “Farmers are intensely interested in the disease status of white-tailed deer herds because their diseases can be transmitted to cattle and humans with potentially fatal results.”

Wainwright Sr. placed federal identification tags from a certified deer that had previously died into the ear of uncertified deer they were selling. He then sold breeding services and semen from the deer to breeders around the United States.

The defendants also sold illegal white-tailed deer hunts at Valley View Whitetails of Ohio. They induced clients from around the country to hunt at Valley View Whitetails of Ohio – charging customers from $1,000 to $50,000 to kill deer inside his high fence preserve when Wainwright did not have a hunting preserve license. The customers then took the bucks back to their home states, including: Florida, Michigan, Alabama and Virginia.

"Chronic wasting disease can decimate wild deer and elk populations and we take egregious violations like this very seriously," said U.S. Fish and Wildlife Service Special Agent in Charge Gregory Jackson. "We would like to thank our law enforcement counterparts in Ohio, Florida and Georgia for sharing their expertise and resources to fully investigate this case.”

Wainwright Sr. pleaded guilty on February 27, 2015, to 12 charges related to violating the Lacey Act, one count of conspiracy and one count of wire fraud. He was also sentenced to 200 hours of community service to be served in a parks system and ordered to publish an article in The Deer Breeders Gazette.

Wainwright Jr. pleaded guilty on February 17, 2015, to eight charges related to violating the Lacey Act.

Under the Lacey Act, it is unlawful to import, export, transport, sell or purchase wildlife, fish or plants that were taken, possessed, transported or sold in violation of a state, federal or foreign law. When it was passed in 1900, the Lacey Act became the first federal law protecting wildlife.

U.S. Attorney Stewart commended the cooperative investigation by law enforcement, as well as Special Assistant United States Attorney Heather Robinson with the Franklin County Prosecutor’s Office and Assistant United States Attorneys Peter Glenn-Applegate and J. Michael Marous, who are representing the United States in this case.


FOR IMMEDIATE RELEASE Tuesday, September 15, 2015 Third Defendant Sentenced in Illegal Deer Trafficking Case Georgia man ordered to pay $1.6 million COLUMBUS, Ohio – Benjamin N. Chason, 61, of Climax, Ga. pleaded guilty and was sentenced in U.S. District Court for three charges related to violating the Lacey Act. Chason was ordered to pay $1.6 million in fines and restitution, the largest sum of money ordered of an individual to pay for a wildlife crime in the United States.

Of the $1.6 million, $600,000 is to be paid into the Ohio Department of Natural Resources Wildlife Habitat Fund $200,000 to the Federal Endangered Species and Wildlife Diversity Fund, $400,000 to Columbus and Franklin County Metro Parks and $100,000 to the Ohio DNR Division of Wildlife Turn in a Poacher (TIP) program.

Carter Stewart, U.S. Attorney for the Southern District of Ohio, Gregory Jackson, Special Agent in Charge, United States Fish and Wildlife Service Office of Law Enforcement, Chief Scott Zody, Ohio Department of Natural Resources Division of Wildlife, Franklin County Prosecutor Ron O’Brien, the Florida Fish and Wildlife Conservation Commission and Georgia Department of Natural Resources announced the sentence, which was unsealed yesterday.

According to court documents, Chason and co-conspirator Donald W. Wainwright, Sr., trafficked in live white-tailed deer. Wainwright Sr. owned hunting preserves in Logan County, Ohio, and Live Oak, Florida; both preserves were named Valley View Whitetails. Donald Wainwright, Jr. was part-time resident and part-time operator of the site in Ohio. Chason was part-owner of Valley View Whitetails in Ohio and also owned an extensive high-fenced property containing white-tailed deer in Climax, Ga.

Wainwright Sr. illegally shipped deer to Florida from Ohio and attempted to ship deer to Georgia from Ohio. The deer herds involved with these shipments were not certified to be free from chronic wasting disease, tuberculosis and brucellosis. Federal law requires interstate shipments of deer to be certified to be disease-free; because the deer in the present case were not certified as disease-free, deer herds (both captive and wild) in Florida were potentially exposed to these diseases. Tuberculosis and brucellosis can also be transmitted from deer to cows and humans.

The attempted shipment to Georgia was intercepted on I-71 South, about 50 miles from the Ohio River, when Ohio Division of Wildlife officers noticed deer noses and antlers inside a cargo trailer and pulled over a truck driven by Wainwright Sr.’s employees.

Wainwright Sr. and Chason placed federal identification tags from a certified deer that had previously died into the ear of an uncertified deer they were selling. They then sold breeding services and semen from the deer to breeders around the United States.

The defendants also sold illegal white-tailed deer hunts at Valley View Whitetails of Ohio. They induced clients from around the country to hunt at Valley View Whitetails of Ohio – charging customers from $1,000 to $50,000 to kill deer inside his high fence preserve when Wainwright did not have a hunting preserve license. The customers then took the bucks back to their home states, including: Florida, Michigan, Alabama and Virginia.

“Illegal sale and transport of white-tailed deer are serious crimes and I appreciate the teamwork and cooperation between all of the agencies involved to help obtain these convictions,” ODNR Division of Wildlife Chief Scott Zody said.

"We are pleased to see the positive results in this investigation. The U.S. Fish and Wildlife Service, Office of Law Enforcement considers the potential spread of disease caused by the illegal commercialization of fish and wildlife resources a high priority, and we will continue to work closely with our State partners to assist them in these important investigations," said Edward Grace, U.S. Fish and Wildlife Service Deputy Assistant Director for Law Enforcement.

Chason pleaded guilty on May 1, 2014. Besides being ordered to pay restitution, Chason was sentenced to three years of probation and four months of home confinement. Chason also agreed to publish a statement in North American Whitetail Magazine and perform 150 hours community service in an Ohio or Georgia State Park.

Wainwright Sr. pleaded guilty on February 27, 2015, to 12 charges related to violating the Lacey Act, one count of conspiracy and one count of wire fraud. He was sentenced to 21 months in prison, a $125,000 fine 200 hours of community service to be served in a parks system and ordered to publish an article in The Deer Breeders Gazette.

Wainwright Jr. pleaded guilty on February 17, 2015, to eight charges related to offering illegal hunts in violation the Lacey Act and was sentenced to four months of house arrest and three years of probation.

Under the Lacey Act, it is unlawful to import, export, transport, sell or purchase wildlife, fish or plants that were taken, possessed, transported or sold in violation of a state, federal or foreign law. When it was passed in 1900, the Lacey Act became the first federal law protecting wildlife.

U.S. Attorney Stewart commended the cooperative investigation by law enforcement, as well as Special Assistant United States Attorney Heather Robinson with the Franklin County Prosecutor’s Office and Assistant United States Attorneys J. Michael Marous and Peter Glenn-Applegate, who represented the United States in this case.


FOR IMMEDIATE RELEASE Friday, February 27, 2015 Florida Man Pleads Guilty To Illegal Deer Trafficking

COLUMBUS – Donald W. Wainwright Sr., 49, of Live Oak, Florida, pleaded guilty in U.S. District Court to 12 charges related to violating the Lacey Act, one count of conspiracy and one count of wire fraud.

Carter Stewart, U.S. Attorney for the Southern District of Ohio, Greg Jackson, Special Agent in Charge, United States Fish and Wildlife Service Office of Law Enforcement, Chief Scott Zody, Ohio Department of Natural Resources Division of Wildlife, Franklin County Prosecutor Ron O’Brien, the Florida Fish and Wildlife Conservation Commission and Georgia Department of Natural Resources announced the plea entered into today before U.S. District Chief Judge Edmund A. Sargus, Jr.

According to court documents, Wainwright illegally trafficked in live white-tailed deer. Wainwright owned hunting preserves in Logan County, Ohio, and Live Oak, Florida; both preserves were named Valley View Whitetails. Wainwright illegally shipped deer to Florida from Ohio and attempted to ship deer to Georgia from Ohio. The deer herds involved with these shipments were not certified to be free from chronic wasting disease, tuberculosis and brucellosis. Federal Law requires interstate shipment of deer to be certified to be disease free. As a result, deer herds in Florida were potentially exposed to these diseases. Wainwright’s attempted shipment to Georgia was intercepted on I-71 South, about 50 miles from the Ohio River, when Ohio Wildlife officers noticed deer noses and antlers inside a cargo trailer and pulled over a truck driven by Wainwright’s employees.

Wainwright also sold illegal white-tailed deer hunts at Valley View Whitetails of Ohio. The defendant induced clients from around the country to hunt at Valley View Whitetails of Ohio – charging customers from $1,000 to $50,000 to kill deer inside his high fence preserve when Wainwright did not have a hunting preserve license. The customers then took the bucks back to their home states, including: Florida, Michigan, Alabama and Virginia.

Wainwright pleaded guilty to 12 charges related to violating the Lacey Act, one count of conspiracy and one count of wire fraud. The parties have proposed a sentence of 21 months in prison and a fine of $125,000.00.

“One of the many dangers of illegal wildlife trafficking is its potential to spread disease,” U.S. Attorney Stewart said. “My office is committed to combating this serious threat.”

Under the Lacey Act it is unlawful to import, export, sell or purchase wildlife or transported, or sold: 1) in violation of U.S. law or 2) in interstate or foreign commerce involving any fish, wildlife, or plants taken possessed or sold in violation of State law. When it was passed in 1900, the Lacey Act became the first federal law protecting wildlife.

U.S. Attorney Stewart commended the cooperative investigation by law enforcement, as well as Special Assistant United States Attorney Heather Robinson and Assistant United States Attorneys Peter Glenn-Applegate and J. Michael Marous, who are representing the United States in this case. 


Tennessee STRATEGIC ELK MANAGEMENT PLAN 2018-2027

Tennessee STRATEGIC ELK MANAGEMENT PLAN 2018-2027

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Based on elk populations in other eastern states, TWRA biologists believed the ERZ could sustain a herd of up to1,400-2,000 elk. As a result, the restoration proposal called for 400 elk to be released on the NCWMA, beginning in 2000 (Tennessee Wildlife Resources Agency 2000). Elk Island National Park (EINP) in Alberta, Canada, was chosen as the source of Tennessee’s elk due to its long-term disease testing protocols and their significant distance from wild cervids infected with chronic wasting disease. The Canadian Food Inspection Agency, EINP, and TWRA coordinated with the United States Department of Agriculture (USDA) to conduct disease testing and vaccinations of the elk before they were imported into the U.S. and ultimately released on NCWMA. All elk were tested for brucellosis, bovine tuberculosis, bluetongue, Epizootic Hemorrhagic Disease, infectious bovine rhinotracheitis, bovine virus diarrhea, and leptospirosis.

On December 19, 2000, several hundred people witnessed the reintroduction of 50 elk into Tennessee at Horsebone Ridge on NCWMA (Lupardus 2005). In 2001 and 2002, an additional 36 and 50 elk, respectively, were released at the same site (Table 1).

From 2002-2005 no elk were available from EINP. However, in 2003 LBL had 31 surplus elk available for TWRA that originated from EINP (Lupardus 2005). TWRA imported and released these elk, but was unable to obtain additional elk from LBL until 2008; when it obtained and released 34 more (Table 1). The 2008 importation was contested by a Tennessee captive elk farmer filing a lawsuit, but the eventual ruling favored TWRA, authorizing importation and release. No additional elk releases have occurred since the 2008 release, and new elk importations will not be recommended during the span of this strategic plan due to concerns of disease introductions. A total of 201 elk were released on North Cumberland WMA from 2000-2008 (Table 1). 

Table 1: Release dates, composition, and source of elk released in Tennessee’s Elk Restoration Zone
Release Date # of Elk Adult Males Adult Females Calves Source 

12/19/2000 50 10 27 13 Elk Island National Park Alberta, Canada 

2/28/2001 36 10 4 22 Elk Island National Park Alberta, Canada 

2/14/2002 50 6 19 25 Elk Island National Park Alberta, Canada 

2/23/2003 31 8 16 7 Land Between the Lakes, Kentucky 

3/6/2008 34 17 9 8 Land Between the Lakes, Kentucky 

Total 201 51 75 75

Additional reintroductions of elk occurred by the National Park Service in the Cataloochee Valley in North Carolina in 2001 and 2002 when 25 and 27 elk were released into the Great Smoky Mountains National Park, respectively. The current estimated elk population in the Park is 150+ animals. Some animals have been reported outside the national park boundaries in Cocke County, Tennessee. However, this population of elk occurs well outside the established ERZ in the Cumberland Mountains and for all intents and purposes, are outside the scope of this plan. Tennessee Code Annotated 70-4-116 (f) (2) (b) presented in Appendix B addresses elk occurring outside the ERZ. 


The Zoo Tennessee

Zoo Hunting Club

1595 Prince Lane Grand Junction TN 38039

The Zoo Hunting Club is comprised of 1,842 acres of the finest whitetail deer and turkey habitat in the mid South. In addition, the property holds a spring fed lake, loaded with largemouth bass and bream.

Offered exclusively by Crews Investment Properties

Mike Slattery (901) 756-2822 x 224 Cell (901) 487-5775 mslattery@crewsproperties.com 




Texas Chronic Wasting Disease CWD TSE Prion Symposium 2018 posted January 2019 VIDEO SET 18 CLIPS

18 videos   85 views   Updated 4 days ago
1 Opening



13:26 Texas CWD Symposium: Welcome and Introductions Texas Parks and Wildlife 



1:00:49 Texas CWD Symposium: CWD Overview Texas Parks and Wildlife 



28:04 Texas CWD Symposium: Myths, Facts, and Legends Texas Parks and Wildlife 



26:42 Texas CWD Symposium: CWD Drives population decline Texas Parks and Wildlife



31:08 Texas CWD Symposium: Pre-symptomatic prion detection Texas Parks and Wildlife



26:58 Texas CWD Symposium: Transmission by Saliva, Feces, Urine & Blood Texas Parks and Wildlife



31:06 Texas CWD Symposium: Sampling CWD Texas Parks and Wildlife



24:10 Texas CWD Symposium: Advantages and Limitations Texas Parks and Wildlife



27:06 Texas CWD Symposium: Diagnostics and Detection Texas Parks and Wildlife



28:56 Texas CWD Symposium: CWD in Wyoming 30+ Yrs Later Texas Parks and Wildlife



31:40 Texas CWD Symposium: CWD Management & Response in Wisconsin Texas Parks and Wildlife



31:46 Texas CWD Symposium: Missouri's Approach to CWD Surveillance Texas Parks and Wildlife



33:35 Texas CWD Symposium: CWD Management in Texas Texas Parks and Wildlife



27:24 Texas CWD Symposium: Report on Ante-Mortem Testing in Texas Texas Parks and Wildlife



47:11 Texas CWD Symposium: Overview of CWD Positive Breeder Facilities Texas Parks and Wildlife



30:43 Texas CWD Symposium: What we have learned after 21 years Texas Parks and Wildlife



25:09 Texas CWD Symposium: Hunter/Landowner Perspective Texas Parks and Wildlife



1:38:33 Texas CWD Symposium: Panel Discussion: Challenges in Texas Texas Parks and Wildlife



SATURDAY, JANUARY 19, 2019 

Texas Chronic Wasting Disease CWD TSE Prion Symposium 2018 posted January 2019 VIDEO SET 18 CLIPS


MONDAY, JANUARY 14, 2019 

Evaluation of iatrogenic risk of CJD transmission associated with Chronic Wasting Disease TSE Prion in Texas TAHC TPWD

It is a dimension as vast as space and as timeless as infinity. It is the middle ground between light and shadow, between science and superstition, and it lies between the pit of man's fears and the summit of his knowledge. This is the dimension of imagination. It is NOT, an area which we call the Twilight Zone, but an area that believes junk science, and the very industries and lobbyist some Texas Hunters, the cervid industry, that insist on shoving the fake news down their throats, we call this ted nugent junk science, and in TEXAS, sometimes you just can't fix stupid, this is where the rubber meets the road, here's your sign!

chronic wasting disease cwd tse prion aka mad deer elk disease, if you consume a cwd tse prion positive cervid, then months, years, decades later, go on to have surgery, dental, ophthalmology, endoscopy, donate tissue, blood, organs, you then expose those medical theaters and tissue, blood, organs, that are incubating the infectious cwd tse prion disease, to everyone that comes in contact.

these are not memes, these are actual statements from hunters/industry in Texas about CWD tse prion.

God help them, and us...terry

''Got a call today from TPWD, I’ve got a mule deer that tested early positive for CWD. I’m soon to turn into a zombie because I have already been eating it. They advised not to consume any of the meat...too late! They want to come confiscate what meat is left once they get more results back from another lab.''

snip...


FRIDAY, JANUARY 04, 2019 

TEXAS TPWD CONFIRMED CWD TSE PRION 3 WTD in Medina, Dallam, and Hartley Counties, and in 3 MD in Hudspeth, Hartley, and El Paso Counties


*** Hartley County Sheep with Scrapie, and CWD in Hartley county ??? 

*** Friday, April 22, 2016 

*** Texas Scrapie Confirmed in a Hartley County Sheep where CWD was detected in a Mule Deer 


SUNDAY, MAY 14, 2017 

85th Legislative Session 2017 AND THE TEXAS TWO STEP Chronic Wasting Disease CWD TSE Prion, and paying to play


Wednesday, May 04, 2016 

TPWD proposes the repeal of §§65.90 -65.94 and new §§65.90 -65.99 Concerning Chronic Wasting Disease - Movement of Deer Singeltary Comment Submission 


TUESDAY, DECEMBER 16, 2014

Texas 84th Legislature 2015 H.R. No. 2597 Kuempel Deer Breeding Industry TAHC TPWD CWD TSE PRION 


SUNDAY, DECEMBER 14, 2014

TEXAS 84th Legislature commencing this January, deer breeders are expected to advocate for bills that will seek to further deregulate their industry


***> TEXAS HISTORY OF CHRONIC WASTING DISEASE CWD TSE PRION

***> Singeltary on Texas Chronic Wasting Disease CWD TSE Prion History


FRIDAY, DECEMBER 28, 2018 

***> Chronic Wasting Disease CWD TSE Prion 2019 Where The Rubber Meets The Road 


Greetings Texas Hunters et al, 

it's to the poing of ad nauseam, the sheer amount of cervid industry reps in the regulatory policy making decisions of chronic wasting disease cwd tse prion, and it reminds me of the USDA OIE et al and mad cow disease. they are the very reason we are in this nightmare...imo...terry


2019 CWD TSE PRION RESEARCH UPDATES

see; ''This is very likely to have parallels with control efforts for CWD in cervids.''

Rapid recontamination of a farm building occurs after attempted prion removal


Abstract

The transmissible spongiform encephalopathy scrapie of sheep/goats and chronic wasting disease of cervids are associated with environmental reservoirs of infectivity. 

Preventing environmental prions acting as a source of infectivity to healthy animals is of major concern to farms that have had outbreaks of scrapie and also to the health management of wild and farmed cervids. 

Here, an efficient scrapie decontamination protocol was applied to a farm with high levels of environmental contamination with the scrapie agent. 

Post-decontamination, no prion material was detected within samples taken from the farm buildings as determined using a sensitive in vitro replication assay (sPMCA). 

A bioassay consisting of 25 newborn lambs of highly susceptible prion protein genotype VRQ/VRQ introduced into this decontaminated barn was carried out in addition to sampling and analysis of dust samples that were collected during the bioassay. 

Twenty-four of the animals examined by immunohistochemical analysis of lymphatic tissues were scrapie-positive during the bioassay, samples of dust collected within the barn were positive by month 3. 

The data illustrates the difficulty in decontaminating farm buildings from scrapie, and demonstrates the likely contribution of farm dust to the recontamination of these environments to levels that are capable of causing disease.

snip...

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.


Saturday, January 5, 2019 

***> Rapid recontamination of a farm building occurs after attempted prion removal 


Wednesday, January 16, 2019 

***> Early preclinical detection of prions in the skin of prion-infected animals


snip...see much more here:


***> 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. 


***> 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. 

TITLE: PATHOLOGICAL FEATURES OF CHRONIC WASTING DISEASE IN REINDEER AND DEMONSTRATION OF HORIZONTAL TRANSMISSION 

*** DECEMBER 2016 CDC EMERGING INFECTIOUS DISEASE JOURNAL CWD HORIZONTAL TRANSMISSION 


Scrapie Confirmed in a Hartley County Sheep 2016


Passage of scrapie to deer results in a new phenotype upon return passage to sheep


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

Location: Virus and Prion Research

Title: Passage of scrapie to deer results in a new phenotype upon return passage to sheep)
Author 

item Greenlee, Justin
item Kokemuller, Robyn
item Moore, Sarah
item West Greenlee, N

Submitted to: Prion 

Publication Type: Abstract Only 

Publication Acceptance Date: 3/15/2017 

Publication Date: N/A 

Citation: N/A

Interpretive Summary:

Technical Abstract: Aims: We previously demonstrated that scrapie has a 100% attack rate in white-tailed deer after either intracranial or oral inoculation. Samples from deer that developed scrapie had two different western blot patterns: samples derived from cerebrum had a banding pattern similar to the scrapie inoculum, but samples from brainstem had a banding pattern similar to CWD. In contrast, transmission of CWD from white-tailed deer to sheep by the intracranial route has a low attack rate and to-date oronasal exposure has been unsuccessful. The purpose of this study was to determine if sheep are susceptible to oronasal exposure of the scrapie agent derived from white-tailed deer. 

Methods: At approximately 5 months of age, Suffolk sheep of various PRNP genotypes were challenged by the oronasal route with 10% brain homogenate derived from either the cerebrum or the brainstem of scrapie-affected deer. Genotypes represented in each inoculation group were VV136RR154QQ171 (n=2), AA136RR154QQ171 (n=2), and AV136RR154QR171 (n=1). After inoculation, sheep were observed daily for clinical signs. Upon development of clinical signs, sheep were killed with an overdose of pentobarbital sodium and necropsied. Tissue samples were tested for the presence of PrPSc by EIA, western blot, and immunohistochemistry (IHC). The No. 13-7 scrapie inoculum used for the deer has a mean incubation period of 20.1 months in sheep with the AA136RR154QQ171 genotype and 26.7 months in sheep with the VV136RR154QQ171 genotype. 

Results: Sheep inoculated oronasally with WTD derived scrapie developed disease, but only after inoculation with the inoculum from the cerebrum that had a scrapie-like profile. The first sheep to develop clinical signs at approximately 29 months post inoculation had the VV136RR154QQ171 genotype. Eventually sheep of the AA136RR154QQ171 genotype developed clinical signs, but at a mean incubation of 52 months. At 62 months post-inoculation, none of the sheep inoculated with material from the deer brainstem have developed clinical disease. 

Conclusions: The No. 13-7 inoculum used in the original deer experiment readily infects white-tailed deer and sheep of various genotypes by the oronasal route. When inoculum is made from different brain regions of No 13-7 scrapie-infected deer from either cerebrum with a scrapie-like western blot pattern or brainstem with a CWD-like western blot pattern, sheep with the VV136RR154QQ171 genotype are the first to develop clinical signs. This is in contrast to the original No. 13-7 inoculum that has a faster incubation period in sheep with the AA136RR154QQ171 genotype. Similar to experiments conducted with CWD, sheep oronasally inoculated with brainstem material from deer with a CWD-like molecular profile have no evidence of disease after 62 months of incubation. While scrapie is not known to occur in free-ranging populations of white-tailed deer, experimental cases are difficult to differentiate from CWD. This work raises the potential concern that scrapie infected deer could serve as a confounding factor to scrapie eradication programs as scrapie from deer seems to be transmissible to sheep by the oronasal route.


Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: Transmission of the agent of sheep scrapie to deer results in PrPSc with two distinct molecular profiles Authors
item Greenlee, Justin item Moore, Sarah - item Smith, Jodi item West Greenlee, Mary - item Kunkle, Robert
Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: March 31, 2015 Publication Date: May 25, 2015 Citation: Greenlee, J., Moore, S.J., Smith, J.., West Greenlee, M.H., Kunkle, R. 2015.
Scrapie transmits to white-tailed deer by the oral route and has a molecular profile similar to chronic wasting disease and distinct from the scrapie inoculum. 

Prion 2015. p. S62. 

Technical Abstract: The purpose of this work was to determine susceptibility of white-tailed deer (WTD) to the agent of sheep scrapie and to compare the resultant PrPSc to that of the original inoculum and chronic wasting disease (CWD). We inoculated WTD by a natural route of exposure (concurrent oral and intranasal (IN); n=5) with a US scrapie isolate. All scrapie-inoculated deer had evidence of PrPSc accumulation. PrPSc was detected in lymphoid tissues at preclinical time points, and deer necropsied after 28 months post-inoculation had clinical signs, spongiform encephalopathy, and widespread distribution of PrPSc in neural and lymphoid tissues. Western blotting (WB) revealed PrPSc with 2 distinct molecular profiles. WB on cerebral cortex had a profile similar to the original scrapie inoculum, whereas WB of brainstem, cerebellum, or lymph nodes reveal PrPSc with a higher profile resembling CWD. Homogenates with the 2 distinct profiles from WTD with clinical scrapie were further passaged to mice expressing cervid prion protein and intranasally to sheep and WTD. In cervidized mice, the two inocula have distinct incubation times. Sheep inoculated intranasally with WTD derived scrapie developed disease, but only after inoculation with the inoculum that had a scrapie-like profile. The WTD study is ongoing, but deer in both inoculation groups are positive for PrPSc by rectal mucosal biopsy. 

In summary, this work demonstrates that WTD are susceptible to the agent of scrapie, two distinct molecular profiles of PrPSc are present in the tissues of affected deer, and inoculum of either profile type readily passes to deer.
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: Scrapie transmits to white-tailed deer by the oral route and has a molecular profile similar to chronic wasting disease Authors
item Greenlee, Justin item Moore, S - item Smith, Jodi - item Kunkle, Robert item West Greenlee, M -
Submitted to: American College of Veterinary Pathologists Meeting Publication Type: Abstract Only Publication Acceptance Date: August 12, 2015 Publication Date: N/A
Technical Abstract: The purpose of this work was to determine susceptibility of white-tailed deer (WTD) to the agent of sheep scrapie and to compare the resultant PrPSc to that of the original inoculum and chronic wasting disease (CWD). We inoculated WTD by a natural route of exposure (concurrent oral and intranasal (IN); n=5) with a US scrapie isolate. All scrapie-inoculated deer had evidence of PrPSc accumulation. PrPSc was detected in lymphoid tissues at preclinical time points, and deer necropsied after 28 months post-inoculation had clinical signs, spongiform encephalopathy, and widespread distribution of PrPSc in neural and lymphoid tissues. Western blotting (WB) revealed PrPSc with 2 distinct molecular profiles. WB on cerebral cortex had a profile similar to the original scrapie inoculum, whereas WB of brainstem, cerebellum, or lymph nodes revealed PrPSc with a higher profile resembling CWD. Homogenates with the 2 distinct profiles from WTD with clinical scrapie were further passaged to mice expressing cervid prion protein and intranasally to sheep and WTD. In cervidized mice, the two inocula have distinct incubation times. Sheep inoculated intranasally with WTD derived scrapie developed disease, but only after inoculation with the inoculum that had a scrapie-like profile. The WTD study is ongoing, but deer in both inoculation groups are positive for PrPSc by rectal mucosal biopsy. 

In summary, this work demonstrates that WTD are susceptible to the agent of scrapie, two distinct molecular profiles of PrPSc are present in the tissues of affected deer, and inoculum of either profile readily passes to deer.
*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep.
White-tailed Deer are Susceptible to Scrapie by Natural Route of Infection
Jodi D. Smith, Justin J. Greenlee, and Robert A. Kunkle; Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS
Interspecies transmission studies afford the opportunity to better understand the potential host range and origins of prion diseases. Previous experiments demonstrated that white-tailed deer are susceptible to sheep-derived scrapie by intracranial inoculation. The purpose of this study was to determine susceptibility of white-tailed deer to scrapie after a natural route of exposure. Deer (n=5) were inoculated by concurrent oral (30 ml) and intranasal (1 ml) instillation of a 10% (wt/vol) brain homogenate derived from a sheep clinically affected with scrapie. Non-inoculated deer were maintained as negative controls. All deer were observed daily for clinical signs. Deer were euthanized and necropsied when neurologic disease was evident, and tissues were examined for abnormal prion protein (PrPSc) by immunohistochemistry (IHC) and western blot (WB). One animal was euthanized 15 months post-inoculation (MPI) due to an injury. At that time, examination of obex and lymphoid tissues by IHC was positive, but WB of obex and colliculus were negative. Remaining deer developed clinical signs of wasting and mental depression and were necropsied from 28 to 33 MPI. Tissues from these deer were positive for scrapie by IHC and WB. Tissues with PrPSc immunoreactivity included brain, tonsil, retropharyngeal and mesenteric lymph nodes, hemal node, Peyer’s patches, and spleen. This work demonstrates for the first time that white-tailed deer are susceptible to sheep scrapie by potential natural routes of inoculation. In-depth analysis of tissues will be done to determine similarities between scrapie in deer after intracranial and oral/intranasal inoculation and chronic wasting disease resulting from similar routes of inoculation.
see full text ;
PO-039: A comparison of scrapie and chronic wasting disease in white-tailed deer
Justin Greenlee, Jodi Smith, Eric Nicholson US Dept. Agriculture; Agricultural Research Service, National Animal Disease Center; Ames, IA USA
White-tailed deer are susceptible to the agent of sheep scrapie by intracerebral inoculation
snip...
It is unlikely that CWD will be eradicated from free-ranging cervids, and the disease is likely to continue to spread geographically [10]. However, the potential that white-tailed deer may be susceptible to sheep scrapie by a natural route presents an additional confounding factor to halting the spread of CWD. This leads to the additional speculations that
1) infected deer could serve as a reservoir to infect sheep with scrapie offering challenges to scrapie eradication efforts and
2) CWD spread need not remain geographically confined to current endemic areas, but could occur anywhere that sheep with scrapie and susceptible cervids cohabitate.
This work demonstrates for the first time that white-tailed deer are susceptible to sheep scrapie by intracerebral inoculation with a high attack rate and that the disease that results has similarities to CWD. These experiments will be repeated with a more natural route of inoculation to determine the likelihood of the potential transmission of sheep scrapie to white-tailed deer. If scrapie were to occur in white-tailed deer, results of this study indicate that it would be detected as a TSE, but may be difficult to differentiate from CWD without in-depth biochemical analysis.
2012
PO-039: A comparison of scrapie and chronic wasting disease in white-tailed deer
Justin Greenlee, Jodi Smith, Eric Nicholson US Dept. Agriculture; Agricultural Research Service, National Animal Disease Center; Ames, IA USA
snip...
The results of this study suggest that there are many similarities in the manifestation of CWD and scrapie in WTD after IC inoculation including early and widespread presence of PrPSc in lymphoid tissues, clinical signs of depression and weight loss progressing to wasting, and an incubation time of 21-23 months. Moreover, western blots (WB) done on brain material from the obex region have a molecular profile similar to CWD and distinct from tissues of the cerebrum or the scrapie inoculum. However, results of microscopic and IHC examination indicate that there are differences between the lesions expected in CWD and those that occur in deer with scrapie: amyloid plaques were not noted in any sections of brain examined from these deer and the pattern of immunoreactivity by IHC was diffuse rather than plaque-like.
*** After a natural route of exposure, 100% of WTD were susceptible to scrapie.
Deer developed clinical signs of wasting and mental depression and were necropsied from 28 to 33 months PI. Tissues from these deer were positive for PrPSc by IHC and WB. Similar to IC inoculated deer, samples from these deer exhibited two different molecular profiles: samples from obex resembled CWD whereas those from cerebrum were similar to the original scrapie inoculum. On further examination by WB using a panel of antibodies, the tissues from deer with scrapie exhibit properties differing from tissues either from sheep with scrapie or WTD with CWD. Samples from WTD with CWD or sheep with scrapie are strongly immunoreactive when probed with mAb P4, however, samples from WTD with scrapie are only weakly immunoreactive. In contrast, when probed with mAb’s 6H4 or SAF 84, samples from sheep with scrapie and WTD with CWD are weakly immunoreactive and samples from WTD with scrapie are strongly positive. This work demonstrates that WTD are highly susceptible to sheep scrapie, but on first passage, scrapie in WTD is differentiable from CWD.
2011
*** After a natural route of exposure, 100% of white-tailed deer were susceptible to scrapie.

FRIDAY, DECEMBER 28, 2018 

***> Chronic Wasting Disease CWD TSE Prion 2019 Where The Rubber Meets The Road 


see a few updated state by state cwd tissue sampling confirmations...

FRIDAY, JANUARY 18, 2019 

Alabama WFF Ramps Up Chronic Wasting Disease CWD TSE Prion Sampling Effort


MONDAY, AUGUST 27, 2018 

Arkansas AGFC Many CWD testing options available for 2018-19 deer season with 369 positive as of January 8, 2018


MONDAY, JANUARY 14, 2019 

Evaluation of iatrogenic risk of CJD transmission associated with Chronic Wasting Disease TSE Prion in Texas TAHC TPWD

It is a dimension as vast as space and as timeless as infinity. It is the middle ground between light and shadow, between science and superstition, and it lies between the pit of man's fears and the summit of his knowledge. This is the dimension of imagination. It is NOT, an area which we call the Twilight Zone, but an area that believes junk science, and the very industries and lobbyist some Texas Hunters, the cervid industry, that insist on shoving the fake news down their throats, we call this ted nugent junk science, and in TEXAS, sometimes you just can't fix stupid, this is where the rubber meets the road, here's your sign!

chronic wasting disease cwd tse prion aka mad deer elk disease, if you consume a cwd tse prion positive cervid, then months, years, decades later, go on to have surgery, dental, ophthalmology, endoscopy, donate tissue, blood, organs, you then expose those medical theaters and tissue, blood, organs, that are incubating the infectious cwd tse prion disease, to everyone that comes in contact.

these are not memes, these are actual statements from hunters/industry in Texas about CWD tse prion.

God help them, and us...terry

''Got a call today from TPWD, I’ve got a mule deer that tested early positive for CWD. I’m soon to turn into a zombie because I have already been eating it. They advised not to consume any of the meat...too late! They want to come confiscate what meat is left once they get more results back from another lab.''

snip...


FRIDAY, JANUARY 04, 2019 

TEXAS TPWD CONFIRMED CWD TSE PRION 3 WTD in Medina, Dallam, and Hartley Counties, and in 3 MD in Hudspeth, Hartley, and El Paso Counties


*** Hartley County Sheep with Scrapie, and CWD in Hartley county ??? 

*** Friday, April 22, 2016 

*** Texas Scrapie Confirmed in a Hartley County Sheep where CWD was detected in a Mule Deer 


SUNDAY, MAY 14, 2017 

85th Legislative Session 2017 AND THE TEXAS TWO STEP Chronic Wasting Disease CWD TSE Prion, and paying to play


Wednesday, May 04, 2016 

TPWD proposes the repeal of §§65.90 -65.94 and new §§65.90 -65.99 Concerning Chronic Wasting Disease - Movement of Deer Singeltary Comment Submission 


TUESDAY, DECEMBER 16, 2014

Texas 84th Legislature 2015 H.R. No. 2597 Kuempel Deer Breeding Industry TAHC TPWD CWD TSE PRION 


SUNDAY, DECEMBER 14, 2014

TEXAS 84th Legislature commencing this January, deer breeders are expected to advocate for bills that will seek to further deregulate their industry


***> TEXAS HISTORY OF CHRONIC WASTING DISEASE CWD TSE PRION

***> Singeltary on Texas Chronic Wasting Disease CWD TSE Prion History


SATURDAY, JANUARY 19, 2019

Wyoming WGFD confirmed a cow elk has tested positive for CWD TSE Prion in Elk Hunt Area 37


THURSDAY, JANUARY 17, 2019 

Missouri MDC reports 28 more deer with CWD TSE Prion total now stands at 103 confirmed cases


THURSDAY, JANUARY 17, 2019 

Michigan reports more CWD TSE Prion aka mad deer disease 115 cases to date


SATURDAY, JANUARY 12, 2019 

Mississippi Confirmed 2 more CWD Positives 6 to date


SATURDAY, JANUARY 12, 2019 

Iowa CWD TSE Prion confirmed in 14 deer including first positive in Dubuque County


MONDAY, JANUARY 07, 2019 

Tennessee TWRA 11 Additional Deer Preliminarily CWD Positive in Fayette and Hardeman Counties


THURSDAY, JANUARY 03, 2019 

Minnesota CWD discovery in Houston County prompts additional late-season deer hunts


WEDNESDAY, DECEMBER 26, 2018 
South Dakota Chronic Wasting Disease CWD TSE Prion Update 397 positive to date


MONDAY, DECEMBER 17, 2018 

Nebraska Confirms 131 Cases of CWD detected for first time in Valley, Keya Paha counties


FRIDAY, DECEMBER 14, 2018 

Montana Four more deer test positive for CWD TSE Prion bringing total for 2018 to 26


TUESDAY, DECEMBER 04, 2018 

Ohio Changes in CWD Sample Submission for IHC Testing, Ohio is considered free of CWD?


SATURDAY, NOVEMBER 10, 2018 

Pennsylvania Thirty-Eight Deer Test Positive for Chronic Wasting Disease on Fulton and Bedford County Deer Farms


FRIDAY, JUNE 08, 2018 

Pennsylvania Two Deer on Blair County Hobby Farm, One on Lancaster County Breeding Farm Test Positive for Chronic Wasting Disease cwd tse prion


unday, January 06, 2013

USDA TO PGC ONCE CAPTIVES ESCAPE

*** "it‘s no longer its business.”


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


COLORADO THE ORIGIN OF CHRONIC WASTING DISEASE CWD TSE PRION?

*** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep. 

IN CONFIDENCE, REPORT OF AN UNCONVENTIONAL SLOW VIRUS DISEASE IN ANIMALS IN THE USA 1989


ALSO, one of the most, if not the most top TSE Prion God in Science today is Professor Adriano Aguzzi, and he recently commented on just this, on a cwd post on my facebook page August 20 at 1:44pm, quote;

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

MONDAY, SEPTEMBER 25, 2017

Colorado Chronic Wasting Disease CWD TSE Prion Mandatory Submission of test samples in some areas and zoonosis


FRIDAY, DECEMBER 14, 2018 

Wisconsin Hundreds Of Escapes From State Deer Farms Reported Since 2013 December 14, 2018


MONDAY, DECEMBER 10, 2018 

Wisconsin DATCP Confirms 11 additional animals from a deer farm in Washington County tested positive for CWD TSE Prion


TUESDAY, NOVEMBER 20, 2018 

WISCONSIN Captive CWD TSE Prion Lotto Entitlement Program Pays Out Again Indemnity From Taxpayers $330,000 To Farmers So Far This Year


WEDNESDAY, OCTOBER 03, 2018 

WISCONSIN CAVES TO GAME FARM INDUSTRY AGAIN WHILE STATE FALLS FURTHER INTO THE ABYSS OF MAD DEER DISEASE CWD TSE PRION


TUESDAY, SEPTEMBER 25, 2018 

Wisconsin CWD TSE PRION PLAN preferred option disposal in a landfill OR public land is acceptable to leave the carcass in the spot of the kill

stupid is, as stupid does, sometimes you can't fix stupid...tss


WEDNESDAY, JUNE 13, 2018 

Wisconsin DATCP NVSL confirmed 21 WTD from a deer farm Iowa County tested positive for chronic wasting disease (CWD)


FRIDAY, FEBRUARY 16, 2018 

Wisconsin Deer from Now-Quarantined PA Lancaster County Farm Tests Positive for Chronic Wasting Disease CWD TSE Prion


FRIDAY, JANUARY 18, 2019 

Norwegian Veterinary Institute analyzed over 33,000 samples from Cronic Wasting Disease (CWD) 2018


FRIDAY, DECEMBER 28, 2018 

***> Chronic Wasting Disease CWD TSE Prion 2019 Where The Rubber Meets The Road 



2019


Rapid recontamination of a farm building occurs after attempted prion removal


Kevin Christopher Gough, BSc (Hons), PhD1, Claire Alison Baker, BSc (Hons)2, Steve Hawkins, MIBiol3, Hugh Simmons, BVSc, MRCVS, MBA, MA3, Timm Konold, DrMedVet, PhD, MRCVS3 and Ben Charles Maddison, BSc (Hons), PhD2

Author affiliations

School of Veterinary Medicine and Science, The University of Nottingham, Loughborough, UK ADAS, School of Veterinary Medicine and Science, The University of Nottingham, Loughborough, UK Animal Sciences Unit, Pathology Department, Animal & Plant Health Agency Weybridge, New Haw, Addlestone, Surrey, UK E-mail for correspondence; ben.maddison@adas.co.uk

Abstract

The transmissible spongiform encephalopathy scrapie of sheep/goats and chronic wasting disease of cervids are associated with environmental reservoirs of infectivity. 

Preventing environmental prions acting as a source of infectivity to healthy animals is of major concern to farms that have had outbreaks of scrapie and also to the health management of wild and farmed cervids. 

Here, an efficient scrapie decontamination protocol was applied to a farm with high levels of environmental contamination with the scrapie agent. 

Post-decontamination, no prion material was detected within samples taken from the farm buildings as determined using a sensitive in vitro replication assay (sPMCA). 

A bioassay consisting of 25 newborn lambs of highly susceptible prion protein genotype VRQ/VRQ introduced into this decontaminated barn was carried out in addition to sampling and analysis of dust samples that were collected during the bioassay. 

Twenty-four of the animals examined by immunohistochemical analysis of lymphatic tissues were scrapie-positive during the bioassay, samples of dust collected within the barn were positive by month 3. 

The data illustrates the difficulty in decontaminating farm buildings from scrapie, and demonstrates the likely contribution of farm dust to the recontamination of these environments to levels that are capable of causing disease.

snip...

PrPC is ubiquitous in its distribution in vivo2 and with both scrapie and CWD the in vivo dissemination of infectivity is also widespread with PrPSc usually accumulating within peripheral lymphatic tissues before the CNS.3 4 With scrapie, PrPSc can be secreted/ excreted via a multiplicity of routes including saliva,5 6 milk,7 faeces,8 skin9 and urine.10 The accumulation of this material within the environment (particularly the built farm environment),11 12 creates levels of infectivity that can be transmitted to naïve animals. These reservoirs of infectivity can remain infectious for prolonged periods of time, in one such recorded incident at least 16 years.13 The advent of high sensitivity prion replication assays such as protein misfolding cyclic amplification (PMCA) with application to sheep/goat scrapie14 15 has allowed the monitoring of prions within environments.11

Attempts to decontaminate pens on a scrapie-affected farm and measuring efficacy using a sheep bioassay were previously reported.12 It was concluded that the failure of effective decontamination within that study was likely to have been due to the incomplete farm decontamination and the presence of dust containing infectious prions that recontaminated the pen surfaces. The serial protein misfolding cyclic amplification (sPMCA) technique was recently used to confirm the presence of prions within extracts prepared from dust samples that had settled on sterile surfaces.16 Given the presence of mobile infectious prions within dust, it was proposed that for effective scrapie decontamination emphasis should be given to the removal of all sources of dust within the decontamination strategy for a farm. More recently, the sPMCA technique has been used by the authors' laboratory to look at effective methods of decontaminating prions bound to concrete surfaces within the laboratory setting.17 This study demonstrated that current methodology based on a one-hour exposure to 20000 ppm free chlorine was likely to be ineffective at removing surface-bound scrapie prion. However, there was an enhanced effectiveness of this chemical decontamination when using multiple applications over four hours. Here, a study is described where a scrapie-affected farm was decontaminated using four applications of 20000 ppm free chlorine to livestock barns and concreted areas. The decontamination also included a high-level clean of the buildings that had housed sheep to remove all traces of dust as far as practicable before the chemical decontamination procedure. Following these treatments the surfaces within the barn were demonstrably free from prion using a sensitive sPMCA assay. The presence of any residual infectivity was then evaluated by sheep bioassay and dust samples collected during the bioassay were assayed for prion seeding activity by sPMCA.

snip...

Discussion

The authors' previous work on this farm indicated that dust harbours low levels of mobile scrapie prions that can accumulate on surfaces16 and this is likely to perpetuate transmission of scrapie within such a farm environment.12 In addition, previous in vitro modelling of scrapie prions bound to a concrete ‘fomite’ demonstrated that prion seeding activity could be inactivated by four applications of 20,000 ppm free chlorine as measured by a sPMCA assay. This previous modelling demonstrated that residual contamination of the swab extract with hypochlorite at levels which would inhibit the sPMCA are unlikely, and the authors consider these results as reduction in seeding titre.17 Here, this same decontamination methodology was tested within a farm-scale study which also included steps to remove dust within the barns. This study demonstrated that this thorough decontamination method applied to a farm with a high incidence of naturally acquired scrapie was sufficient to remove scrapie prions on surfaces to levels that were undetectable by sPMCA, one of the most sensitive biochemical assays for prions. The authors' sPMCA assay has an limit of detection of around 1–10pg scrapie-infected sheep brain per sPMCA reaction. The authors assume that the samples negative by sPMCA had less than this amount (of brain equivalent) within the extracts that were prepared. This treatment together with measures designed to minimise the amount of dust retained within the buildings (vacuuming all surfaces, pressure washing and then hypochlorite treatment) was expected to have removed all infectivity from the buildings and the concrete areas surrounding them, and it was anticipated that the sheep bioassay would confirm absence of infective prion.

However, the introduction into this decontaminated barn of 25 VRQ/VRQ sheep (a genotype highly susceptible to classical scrapie) demonstrated that all animals, with the exception of 1 lamb that died at 122 dpe, had detectable PrPSc in lymphoid tissue, indicating infection with the scrapie agent. This included 14 animals (54 per cent) that were PrPSc-positive on the first RAMALT analysis at 372 dpe or 419 dpe. Although infected sheep were removed based on a positive RAMALT result, it is possible that lateral transmission or subsequent contamination of the environment from infected sheep had contributed to the rapid spread of scrapie in nearly all sheep. It has been shown previously that objects in contact with scrapie-infected sheep, such as water troughs and fence posts, can act as a reservoir for infection.23 As in the authors' previous study,12 the decontamination of this sheep barn was not effective at removing scrapie infectivity, and despite the extra measures brought into this study (more effective chemical treatment and removal of sources of dust) the overall rates of disease transmission mirror previous results on this farm. With such apparently effective decontamination (assuming that at least some sPMCA seeding ability is coincident with infectivity), how was infectivity able to persist within the environment and where does infectivity reside? Dust samples were collected in both the bioassay barn and also a barn subject to the same decontamination regime within the same farm (but remaining unoccupied). Within both of these barns dust had accumulated for three months that was able to seed sPMCA, indicating the accumulation of scrapie-containing material that was independent of the presence of sheep that may have been incubating and possibly shedding low amounts of infectivity.

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.

Acknowledgements The authors thank the APHA farm staff, Tony Duarte, Olly Roberts and Margaret Newlands for preparation of the sheep pens and animal husbandry during the study. The authors also thank the APHA pathology team for RAMALT and postmortem examination.

Funding This study was funded by DEFRA within project SE1865. 

Competing interests None declared. 


Saturday, January 5, 2019 

***> Rapid recontamination of a farm building occurs after attempted prion removal 



Wednesday, January 16, 2019 

***> Early preclinical detection of prions in the skin of prion-infected animals


snip...see much more here:



***> 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. 




***> 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. 


Gudmundur Georgsson,1 Sigurdur Sigurdarson2 and Paul Brown3

Correspondence

Gudmundur Georgsson ggeorgs@hi.is

1 Institute for Experimental Pathology, University of Iceland, Keldur v/vesturlandsveg, IS-112 Reykjavı´k, Iceland

2 Laboratory of the Chief Veterinary Officer, Keldur, Iceland

3 Bethesda, Maryland, USA

Received 7 March 2006 Accepted 6 August 2006

In 1978, a rigorous programme was implemented to stop the spread of, and subsequently eradicate, sheep scrapie in Iceland. Affected flocks were culled, premises were disinfected and, after 2–3 years, restocked with lambs from scrapie-free areas. Between 1978 and 2004, scrapie recurred on 33 farms. 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. Of special interest was one farm with a small, completely self-contained flock where scrapie recurred 18 years after culling, 2 years after some lambs had been housed in an old sheephouse that had never been disinfected. Epidemiological investigation established with near certitude that the disease had not been introduced from the outside and it is concluded that the agent may have persisted in the old sheep-house for at least 16 years.

TITLE: PATHOLOGICAL FEATURES OF CHRONIC WASTING DISEASE IN REINDEER AND DEMONSTRATION OF HORIZONTAL TRANSMISSION 


 *** DECEMBER 2016 CDC EMERGING INFECTIOUS DISEASE JOURNAL CWD HORIZONTAL TRANSMISSION 


SEE;

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

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

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



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).



Dr. Paul Brown Scrapie Soil Test BSE Inquiry Document



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. 



New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication 



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



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



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



Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals 



PPo4-4: 

Survival and Limited Spread of TSE Infectivity after Burial 




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 



Wednesday, December 16, 2015 

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



***> 2018 URGENT DATA <***


***2018***

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 #

5R01NS088604-04

Application #

9517118

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 2018-08-01 Budget End 2019-07-31 Support Year 4 Fiscal Year 2018 Total Cost Indirect Cost Institution Name Case Western Reserve University Department Pathology Type Schools of Medicine DUNS # 077758407 City Cleveland State OH Country United States Zip Code 44106

 Related projects

NIH 2018 R01 NS Cervid to human prion transmission Kong, Qingzhong / Case Western Reserve University 

NIH 2017 R01 NS Cervid to human prion transmission Kong, Qingzhong / Case Western Reserve University 

NIH 2016 R01 NS Cervid to human prion transmission Kong, Qingzhong / Case Western Reserve University 

NIH 2015 R01 NS Cervid to human prion transmission Kong, Qingzhong / Case Western Reserve University $337,507


ZOONOTIC CHRONIC WASTING DISEASE CWD TSE PRION UPDATE

here is the latest;

PRION 2018 CONFERENCE 

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

Hermann M. Schatzl, Samia Hannaoui, Yo-Ching Cheng, Sabine Gilch (Calgary Prion Research Unit, University of Calgary, Calgary, Canada) Michael Beekes (RKI Berlin), Walter Schulz-Schaeffer (University of Homburg/Saar, Germany), Christiane Stahl-Hennig (German Primate Center) & Stefanie Czub (CFIA Lethbridge). 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 detected in spinal cord and brain of some 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 pre-clinical 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.. 

***> 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. <*** 


READING OVER THE PRION 2018 ABSTRACT BOOK, LOOKS LIKE THEY FOUND THAT from this study ; 

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.. 

SEEMS THAT THEY FOUND Highly endemic states had a higher rate of prion disease mortality compared to non-CWD states. 

AND ANOTHER STUDY; 

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.. 

IN THIS STUDY, THERE WERE autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017, 

AND 

included 104 patients. SEEMS THEY FOUND THAT The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%), 

AND 

THAT The Majority of cases were male (60%), AND half of them had exposure to wild game. 

snip...see more on Prion 2017 Macaque study from Prion 2017 Conference and other updated science on cwd tse prion zoonosis below...terry 



just out CDC...see;

Research Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions 

Marcelo A. Barria

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) M. A. Barria et al. 

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. 


Molecular Barriers to Zoonotic Transmission of Prions 

Marcelo A. Barria, Aru Balachandran, Masanori Morita, Tetsuyuki Kitamoto, Rona Barron, Jean Manson, Richard Knight, James W. Ironside, and Mark W. Headcorresponding author 

snip... 

The conversion of human PrPC by CWD brain homogenate in PMCA reactions was less efficient when the amino acid at position 129 was valine rather than methionine. 

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

snip... 

However, we can say with confidence that under the conditions used here, none of the animal isolates tested were as efficient as C-type BSE in converting human PrPC, which is reassuring. 

***Less reassuring is the finding that there is no absolute barrier to the conversion of human PrPC by CWD prions in a protocol using a single round of PMCA and an entirely human substrate prepared from the target organ of prion diseases, the brain. 


Prion 2017 Conference Abstracts 

CWD 2017 PRION CONFERENCE 

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 

Subject: PRION 2017 CONFERENCE 

DECIPHERING NEURODEGENERATIVE DISORDERS 

VIDEO PRION 2017 CONFERENCE DECIPHERING NEURODEGENERATIVE DISORDERS 

*** PRION 2017 CONFERENCE VIDEO 



ZOONOTIC, ZOONOSIS, CHRONIC WASTING DISEASE CWD TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION 

10. ZOONOTIC, ZOONOSIS, CHRONIC WASTING DISEASE CWD TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION AKA MAD DEER ELK DISEASE IN HUMANS, has it already happened, that should be the question... 

''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)

EFSA Panel on Biological Hazards (BIOHAZ) Antonia Ricci Ana Allende Declan Bolton Marianne Chemaly Robert Davies Pablo Salvador Fernández Escámez ... See all authors 

First published: 17 January 2018 https://doi.org/10.2903/j.efsa.2018.5132 ; 

also, see; 

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. 

snip... 

The tissue distribution of infectivity in CWD‐infected cervids is now known to extend beyond CNS and lymphoid tissues. While the removal of these specific tissues from the food chain would reduce human dietary exposure to infectivity, exclusion from the food chain of the whole carcass of any infected animal would be required to eliminate human dietary exposure. 


zoonosis zoonotic cervid tse prion cwd to humans, preparing for the storm 

***An alternative to modeling the species barrier is the cell-free conversion assay which points to CWD as the animal prion disease with the greatest zoonotic potential, after (and very much less than) BSE..116*** 


 To date there is no direct evidence that CWD has been or can be transmitted from animals to humans. 

However, initial findings from a laboratory research project funded by the Alberta Prion Research Institute (APRI) and Alberta Livestock Meat Agency (ALMA), and led by a Canadian Food Inspection Agency (CFIA) scientist indicate that CWD has been transmitted to cynomolgus macaques (the non-human primate species most closely related to humans that may be used in research), through both the intracranial and oral routes of exposure. 

Both infected brain and muscle tissues were found to transmit disease. 

Health Canada’s Health Products and Food Branch (HPFB) was asked to consider the impact of these findings on the Branch’s current position on CWD in health products and foods. 

Summary and Recommendation: 

snip...

Health Portfolio partners were recently made aware of initial findings from a research project led by a CFIA scientist that have demonstrated that cynomolgus macaques can be infected via intracranial exposure and oral gavage with CWD infected muscle. 

These findings suggest that CWD, under specific experimental conditions, has the potential to cross the human species barrier, including by enteral feeding of CWD infected muscle. 


*** WDA 2016 NEW YORK *** 

We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. 

In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species. 

***We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions. 

Student Presentations Session 2 

The species barriers and public health threat of CWD and BSE prions 

Ms. Kristen Davenport1, Dr. Davin Henderson1, Dr. Candace Mathiason1, Dr. Edward Hoover1 1Colorado State University 

Chronic wasting disease (CWD) is spreading rapidly through cervid populations in the USA. Bovine spongiform encephalopathy (BSE, mad cow disease) arose in the 1980s because cattle were fed recycled animal protein. 

These and other prion diseases are caused by abnormal folding of the normal prion protein (PrP) into a disease causing form (PrPd), which is pathogenic to nervous system cells and can cause subsequent PrP to misfold. CWD spreads among cervids very efficiently, but it has not yet infected humans. On the other hand, BSE was spread only when cattle consumed infected bovine or ovine tissue, but did infect humans and other species. 

The objective of this research is to understand the role of PrP structure in cross-species infection by CWD and BSE. To study the propensity of each species’ PrP to be induced to misfold by the presence of PrPd from verious species, we have used an in vitro system that permits detection of PrPd in real-time. 

We measured the conversion efficiency of various combinations of PrPd seeds and PrP substrate combinations. 

We observed the cross-species behavior of CWD and BSE, in addition to feline-adapted CWD and BSE. We found that CWD adapts to a new host more readily than BSE and that human PrP was unexpectedly prone to misfolding by CWD prions. In addition, we investigated the role of specific regions of the bovine, deer and human PrP protein in resistance to conversion by prions from another species. 

***We have concluded that the human protein has a region that confers unusual susceptibility to conversion by CWD prions. CWD is unique among prion diseases in its rapid spread in natural populations. BSE prions are essentially unaltered upon passage to a new species, while CWD adapts to the new species. This adaptation has consequences for surveillance of humans exposed to CWD. Wildlife Disease Risk Communication Research Contributes to Wildlife Trust Administration Exploring perceptions about chronic wasting disease risks among wildlife and agriculture professionals and stakeholders 



TUESDAY, SEPTEMBER 12, 2017 

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


SATURDAY, JANUARY 27, 2018 

CDC CHRONIC WASTING DISEASE CWD TSE PRION UPDATE REPORT USA JANUARY 2018


Subject: CDC CHRONIC WASTING DISEASE CWD TSE PRION UPDATE REPORT USA JANUARY 2018

CHRONIC WASTING DISEASE CWD TSE PRION IS THE USA AND NORTH AMERICA'S MAD COW DISEASE. 

THE USDA INC ET AL WORKED VERY HARD CONCEALING BSE TSE PRION IN CATTLE. they almost succeeded $$$

BUT CWD TSE PRION IN CERVIDS IS A DIFFERENT BEAST, THE COVER UP THERE, USDA INC COULD NOT CONTAIN.

SPORADIC CJD IS 85%+ OF ALL HUMAN TSE PRION DISEASE.

SPORADIC CJD HAS NOW BEEN LINKED TO TYPICAL AND ATYPICAL BSE, SCRAPIE, AND CWD.

SPORADIC/SPONTANEOUS TSE HAS NEVER BEEN PROVEN.

***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.*** 


CDC CWD TSE PRION UPDATE USA JANUARY 2018

As of January 2018, CWD in free-ranging deer, elk and/or moose has been reported in at least 22 states in the continental United States, as well as two provinces in Canada. In addition, CWD has been reported in reindeer and moose in Norway, and a small number of imported cases have been reported in South Korea. The disease has also been found in farmed deer and elk. CWD was first identified in captive deer in the late 1960s in Colorado and in wild deer in 1981. By the 1990s, it had been reported in surrounding areas in northern Colorado and southern Wyoming. Since 2000, the area known to be affected by CWD in free-ranging animals has increased to at least 22 states, including states in the Midwest, Southwest, and limited areas on the East Coast.. It is possible that CWD may also occur in other states without strong animal surveillance systems, but that cases haven’t been detected yet. Once CWD is established in an area, the risk can remain for a long time in the environment. The affected areas are likely to continue to expand. Nationwide, the overall occurrence of CWD in free-ranging deer and elk is relatively low. However, in several locations where the disease is established, infection rates may exceed 10 percent (1 in 10), and localized infection rates of more than 25 percent (1 in 4) have been reported. The infection rates among some captive deer can be much higher, with a rate of 79% (nearly 4 in 5) reported from at least one captive herd. As of January 2018, there were 186 counties in 22 states with reported CWD in free-ranging cervids... 

Chronic Wasting Disease Among Free-Ranging Cervids by County, United States, January 2018 

snip.... 


*** 2017-2018 CWD TSE Prion UPDATE


*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies. 


you can see more evidence here ;


Wednesday, May 24, 2017 

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



WEDNESDAY, SEPTEMBER 08, 2010

CWD PRION CONGRESS SEPTEMBER 8-11 2010

PRION 2010

International Prion Congress: From agent to disease September 8–11, 2010 Salzburg, Austria


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 (216-119-163-189.ipset45.wt.net)

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

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

From: "Belay, Ermias"

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

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

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

Dear Sir/Madam,

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

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

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

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


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. 

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).*** 




SEE; Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007 FoodNet Population Survey

Monday, May 23, 2011

CDC Assesses Potential Human Exposure to Prion Diseases Travel Warning

Public release date: 23-May-2011

Contact: Francesca Costanzo adajmedia@elsevier.com 215-239-3249 Elsevier Health Sciences

CDC assesses potential human exposure to prion diseases Study results reported in the Journal of the American Dietetic Association

Philadelphia, PA, May 23, 2011 – Researchers from the Centers for Disease Control and Prevention (CDC) have examined the potential for human exposure to prion diseases, looking at hunting, venison consumption, and travel to areas in which prion diseases have been reported in animals. Three prion diseases in particular – bovine spongiform encephalopathy (BSE or “Mad Cow Disease”), variant Creutzfeldt-Jakob disease (vCJD), and chronic wasting disease (CWD) – were specified in the investigation. The results of this investigation are published in the June issue of the Journal of the American Dietetic Association.

“While prion diseases are rare, they are generally fatal for anyone who becomes infected. More than anything else, the results of this study support the need for continued surveillance of prion diseases,” commented lead investigator Joseph Y. Abrams, MPH, National Center for Emerging and Zoonotic Infectious Diseases, CDC, Atlanta.”But it’s also important that people know the facts about these diseases, especially since this study shows that a good number of people have participated in activities that may expose them to infection-causing agents.”

Although rare, human prion diseases such as CJD may be related to BSE. Prion (proteinaceous infectious particles) diseases are a group of rare brain diseases that affect humans and animals. When a person gets a prion disease, brain function is impaired. This causes memory and personality changes, dementia, and problems with movement. All of these worsen over time. These diseases are invariably fatal. Since these diseases may take years to manifest, knowing the extent of human exposure to possible prion diseases could become important in the event of an outbreak.

CDC investigators evaluated the results of the 2006-2007 population survey conducted by the Foodborne Diseases Active Surveillance Network (FoodNet). This survey collects information on food consumption practices, health outcomes, and demographic characteristics of residents of the participating Emerging Infections Program sites. The survey was conducted in Connecticut, Georgia, Maryland, Minnesota, New Mexico, Oregon, and Tennessee, as well as five counties in the San Francisco Bay area, seven counties in the Greater Denver area, and 34 counties in western and northeastern New York.

Survey participants were asked about behaviors that could be associated with exposure to the agents causing BSE and CWD, including travel to the nine countries considered to be BSE-endemic (United Kingdom, Republic of Ireland, France, Portugal, Switzerland, Italy, the Netherlands, Germany, Spain) and the cumulative length of stay in each of those countries. Respondents were asked if they ever had hunted for deer or elk, and if that hunting had taken place in areas considered to be CWD-endemic (northeastern Colorado, southeastern Wyoming or southwestern Nebraska). They were also asked if they had ever consumed venison, the frequency of consumption, and whether the meat came from the wild.

The proportion of survey respondents who reported travel to at least one of the nine BSE endemic countries since 1980 was 29.5%. Travel to the United Kingdom was reported by 19.4% of respondents, higher than to any other BSE-endemic country. Among those who traveled, the median duration of travel to the United Kingdom (14 days) was longer than that of any other BSE-endemic country.. Travelers to the UK were more likely to have spent at least 30 days in the country (24.9%) compared to travelers to any other BSE endemic country. The prevalence and extent of travel to the UK indicate that health concerns in the UK may also become issues for US residents.

The proportion of survey respondents reporting having hunted for deer or elk was 18.5% and 1.2% reported having hunted for deer or elk in CWD-endemic areas. Venison consumption was reported by 67.4% of FoodNet respondents, and 88.6% of those reporting venison consumption had obtained all of their meat from the wild. These findings reinforce the importance of CWD surveillance and control programs for wild deer and elk to reduce human exposure to the CWD agent. Hunters in CWD-endemic areas are advised to take simple precautions such as: avoiding consuming meat from sickly deer or elk, avoiding consuming brain or spinal cord tissues, minimizing the handling of brain and spinal cord tissues, and wearing gloves when field-dressing carcasses.

According to Abrams, “The 2006-2007 FoodNet population survey provides useful information should foodborne prion infection become an increasing public health concern in the future. The data presented describe the prevalence of important behaviors and their associations with demographic characteristics. Surveillance of BSE, CWD, and human prion diseases are critical aspects of addressing the burden of these diseases in animal populations and how that may relate to human health.”

###

The article is “Travel history, hunting, and venison consumption related to prion disease exposure, 2006-2007 FoodNet population survey” by Joseph Y. Abrams, MPH; Ryan A. Maddox, MPH; Alexis R Harvey, MPH; Lawrence B. Schonberger, MD; and Ermias D. Belay, MD. It appears in the Journal of the American Dietetic Association, Volume 111, Issue 6 (June 2011) published by Elsevier.

In an accompanying podcast CDC’s Joseph Y. Abrams discusses travel, hunting, and eating venison in relation to prion diseases. It is available at http://adajournal.org/content/podcast. ;


Thursday, May 26, 2011

Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007 FoodNet Population Survey

Journal of the American Dietetic Association Volume 111, Issue 6 , Pages 858-863, June 2011.

Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007 FoodNet Population Survey

Joseph Y. Abrams, MPH, Ryan A. Maddox, MPH , Alexis R. Harvey, MPH , Lawrence B. Schonberger, MD , Ermias D. Belay, MD

Accepted 15 November 2010. Abstract Full Text PDF References .

Abstract

The transmission of bovine spongiform encephalopathy (BSE) to human beings and the spread of chronic wasting disease (CWD) among cervids have prompted concerns about zoonotic transmission of prion diseases. Travel to the United Kingdom and other European countries, hunting for deer or elk, and venison consumption could result in the exposure of US residents to the agents that cause BSE and CWD. The Foodborne Diseases Active Surveillance Network 2006-2007 population survey was used to assess the prevalence of these behaviors among residents of 10 catchment areas across the United States. Of 17,372 survey respondents, 19.4% reported travel to the United Kingdom since 1980, and 29.5% reported travel to any of the nine European countries considered to be BSE-endemic since 1980. The proportion of respondents who had ever hunted deer or elk was 18.5%, and 1.2% had hunted deer or elk in a CWD–endemic area. More than two thirds (67.4%) reported having ever eaten deer or elk meat. Respondents who traveled spent more time in the United Kingdom (median 14 days) than in any other BSE-endemic country. Of the 11,635 respondents who had consumed venison, 59.8% ate venison at most one to two times during their year of highest consumption, and 88.6% had obtained all of their meat from the wild. The survey results were useful in determining the prevalence and frequency of behaviors that could be important factors for foodborne prion transmission. 


 PLUS, THE CDC DID NOT PUT THIS WARNING OUT FOR THE WELL BEING OF THE DEER AND ELK ; 

Thursday, May 26, 2011

Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007 FoodNet Population Survey

Journal of the American Dietetic Association Volume 111, Issue 6 , Pages 858-863, June 2011. 


NOR IS THE FDA recalling this CWD positive elk meat for the well being of the dead elk ;

Wednesday, March 18, 2009

Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS AND FIELD CORRECTIONS: FOODS CLASS II 


Transmissible Spongiform Encephalopathies

Spongiform Encephalopathy in Captive Wild ZOO BSE INQUIRY 


 BSE INQUIRY

CJD9/10022

October 1994

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane 

BerksWell Coventry CV7 7BZ

Dear Mr Elmhirst,

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

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

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

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

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

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


*** 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). ***

*** 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). ***

*** 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 ; 


PRION 2018 CONFERENCE
O3 Experimental studies on prion transmission barrier and TSE pathogenesis in large animals 

Rosa Bolea(1), Acín C(1)Marín B(1), Hedman C(1), Raksa H(1), Barrio T(1), Otero A(1), LópezPérez O(1), Monleón E(1),Martín-Burriel(1), Monzón M(1), Garza MC(1), Filali H(1),Pitarch JL(1), Garcés M(1), Betancor M(1), GuijarroIM(1), GarcíaM(1), Moreno B(1),Vargas A(1), Vidal E(2), Pumarola M(2), Castilla J(3), Andréoletti O(4), Espinosa JC(5), Torres JM(5), Badiola JJ(1). 

1Centro de Investigación en Encefalopatías y Enfermedades Transmisibles Emergentes, VeterinaryFaculty, Universidad de Zaragoza; Zaragoza,Spain.2 RTA, Centre de Recerca en Sanitat Animal (CReSA, IRTA-UAB) 3 4 INRA, ÉcoleVétérinaire, Toulouse, France.5CIC bioGUNE, Prion researchlab, Derio, Spain CISA- INIA, Valdeolmos, Madrid 28130, Spain. 

Experimental transmission of Transmissible Spongiform Encephalopathies (TSE) has been understood and related with several factors that could modify the natural development of these diseases. In fact, the behaviour of the natural disease does not match exactly in each animal, being modified by parameters such as the age at infection, the genotype, the breed or the causative strain. Moreover, different TSE strains can target different animal species or tissues, what complicate the prediction of its transmissibility when is tested in a different species of the origin source. The aim of the experimental studies in large animals is to homogenize all those factors, trying to minimize as much as possible variations between individuals. These effects can be flattened by experimental transmission in mice, in which a specific strain can be selected after several passages. With this objective, several experimental studies in large animals have been developed by the presenter research team. 

Classical scrapie agent has been inoculated in cow, with the aim of demonstrate the resistance or susceptibility of this species to the first well known TSE; Atypical scrapie has been inoculated in sheep (using several routes of infection), cow and pig, with the objective of evaluating the potential pathogenicity of this strain; Classical Bovine Spongiform Encephalopathy (BSE) has been inoculated in goats aiming to demonstrate if the genetic background of this species could protect against this strain; goat BSE and sheep BSE have been inoculated in goats and pigs respectively to evaluate the effect of species barrier; and finally atypical BSE has been inoculated in cattle to assess the transmissibility properties of this newly introduced strain. 

Once the experiments have been carried out on large animal species, a collection of samples from animals studied were inoculated in different types of tg mice overexpressing PrPcin order to study the infectivity of the tissues, and also were studied using PMCA. 

In summary, the parameters that have been controlled are the species, the strain, the route of inoculation, the time at infection, the genotype, the age, and the environmental conditions. 

To date, 

***> eleven of the atypical scrapie intracerebrally inoculated sheep have succumbed to atypical scrapie disease; 

***> six pigs to sheep BSE; 

***> one cow to classical scrapie; 

***> nine goats to goat BSE and 

***> five goats to classical BSE. 

***> PrPSC has been demonstrated in all cases by immunohistochemistry and western blot. 

=====> PRION CONFERENCE 2018 


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 5="" 6="" at="" by="" detected="" eia.="" examined="" group="" in="" intracranial="" least="" lymphoid="" month="" months="" of="" one="" pigs="" positive="" prpsc="" quic="" the="" tissues="" was="">6 months group, 5/6 pigs in the oral <6 4="" and="" group="" months="" 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. 




Early preclinical detection of prions in the skin of prion-infected animals

Zerui Wang1,2, Matteo Manca3, Aaron Foutz4, Manuel V. Camacho1 , Gregory J. Raymond3, Brent Race3, Christina D. Orru3, Jue Yuan1 , Pingping Shen1,2, Baiya Li1,5, Yue Lang1,2, Johnny Dang1 , Alise Adornato1 , Katie Williams3, Nicholas R. Maurer1 , Pierluigi Gambetti1 , Bin Xu6, Witold Surewicz7, Robert B. Petersen1,8, Xiaoping Dong9, Brian S. Appleby1,4,10, Byron Caughey 3, Li Cui2, Qingzhong Kong1,4,10,11 & Wen-Quan Zou1,2,4,9,10,11

snip...

Although the reasons for early and widespread presence of PrPSc in the skin remain unclear, possibilities include the spread of the prion inoculum itself, or endogenously replicating prions, from the brain through the peripheral nerves to the skin within the 2–3 weeks required for the first detection by our ultrasensitive
sPMCA and RT-QuIC assays. PrP seeding activity has been detected in the blood in the prion-infected hamsters and deer immediately after peripheral inoculation including oral, nasal, or blood route30. However, no reports have shown that PrPSc is consistently detectable in the blood of prion-infected hamsters within 2 weeks post intracerebral inoculation. Thus, the early spread of PrPSc from the brain-to-the skin in the intracerebrally 263K-inoculated hamsters is likely either not through the blood or, if initially from the blood, requires time-dependent concentration or replication in the skin to become detectable.

snip...

Skin PrPSc may derive from urine or fecal prion contamination in addition to possible skin shedding due to scratching or biting each other. Indeed, scrapie infectivity was reported in the urine of prion-infected mice coincident with lymphocytic nephritis during their preclinical and clinical stages of prion infection35,36. It was also observed in their urine in intracerebrally inoculated hamsters even without any apparent inflammation21. In addition, deer with clinical CWD and mild to moderate nephritis were found to have sPMCA-detectable PrPSc and CWD-infectivity in urine22. Using sPMCA, PrPSc was detected in urine of ~80% of the hamsters intraperitoneally inoculated with 263K prions at the symptomatic stage23. Notably, PrPSc was detected in urine, but only at the terminal stage of disease in intracerebrally inoculated hamsters, except for a few days immediately after oral administration24. Similar to the observations by Gonzalez-Romero et al.23, Murayama et al. also found that not all infected hamsters had detectable urine PrPSc even at the terminal stage24. The skin PrPSc detected early in the intracerebrally infected hamsters, but not in the co-habitated-negative controls, at 2 wpi suggests that skin prions may not result from urine at the early stage of infection.



why do we not want to do TSE transmission studies on chimpanzees $

5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. 

***> I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. 

***> Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

snip...


TUESDAY, MAY 31, 2011 

Chronic Wasting Disease DOI: 10.1007/128_2011_159 # Springer-Verlag Berlin Heidelberg 2011 


Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) A TOTAL FAILURE $$$


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. 


Friday, December 14, 2012

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

snip.....

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

Animals considered at high risk for CWD include:

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

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

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

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

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

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

snip.....

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

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

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

snip.....

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

snip.....

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

snip.....

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

snip.....

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

snip.....


TUESDAY, APRIL 18, 2017 

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


SUNDAY, DECEMBER 02, 2018 

CWD TSE PRION, REGULATORY LEGISLATION, PAY TO PLAY, and The SPREAD of Chronic Wasting Disease


Prion Conference 2018

O5 Prion Disease in Dromedary Camels 

Babelhadj B (1), Di Bari MA (2), Pirisinu L (2), Chiappini B (2), Gaouar SB (3), Riccardi G (2), Marcon S (2), Agrimi U (2), Nonno R (2), Vaccari G (2) (1) École Normale Supérieure Ouargla. Laboratoire de protection des écosystèmes en zones arides et semi arides University Kasdi Merbah Ouargla, Ouargla, Algeria; (2) Istituto Superiore di Sanità, Department of Food Safety, Nutrition and Veterinary Public Health, Rome, Italy (3) University Abou Bekr Bélkaid, Tlemcen, Algeria. 

Prions are responsible for fatal and transmissible neurodegenerative diseases including CreutzfeldtJakob disease in humans, scrapie in small ruminants and bovine spongiform encephalopathy (BSE). Following the BSE epidemic and the demonstration of its zoonotic potential, general concerns have been raised on animal prions. 

Here we report the identification of a prion disease in dromedary camels (Camelus dromedarius) in Algeria and designate it as Camel Prion Disease (CPD). In the last years, neurological symptoms have been observed in adult male and female dromedaries presented for slaughter at the Ouargla abattoir. The symptoms include weight loss, behavioral abnormalities and neurological symptoms such as tremors, aggressiveness, hyper-reactivity, typical down and upwards movements of the head, hesitant and uncertain gait, ataxia of the hind limbs, occasional falls and difficult getting up. During 2015 and 2016, symptoms suggestive of prion disease were observed in 3.1% of 2259 dromedaries presented at ante-mortem examination. Laboratory diagnosis was obtained in three symptomatic dromedaries, sampled in 2016 and 2017, by the detection of typical neurodegeneration and disease-specific prion protein (PrPSc) in brain tissues. 

Histopathological examination revealed spongiform change, gliosis and neuronal loss preferentially in grey matter of subcortical brain areas. Abundant PrPSc deposition was detected in the same brain areas by immunohistochemistry and PET-blot. Western blot analysis confirmed the presence of PK-resistant PrPSc, whose N-terminal cleaved PK-resistant core was characterized by a mono-glycosylated dominant form and by a distinctive N-terminal cleavage, different from that observed in BSE and scrapie. 

PrPSc was also detected, by immunohistochemistry, in all sampled lymph nodes (cervical, prescapular and lumbar aortic) of the only animal from which they were collected. 

The PRNP sequence of the two animals for which frozen material was available, showed 100% nucleotide identity with the PRNP sequence already reported for dromedary camel. 

Overall, these data demonstrate the presence of a prion disease in dromedary camelswhose nature, origin and spread need further investigations. However, our preliminary observations on the rather high prevalence of symptomatic dromedaries and the involvement of lymphoid tissues, are consistent with CPD being an infectious disease. In conclusion, the emergence of a new prion disease in a livestock species of crucial importance for millions of people around the world, makes urgent to assess the risk for humans and to develop policies able to control the spread of the disease in animals and to minimize human exposure. 


CDC

New Outbreak of TSE Prion in NEW LIVESTOCK SPECIES

Mad Camel Disease

Volume 24, Number 6—June 2018 Research 

Prion Disease in Dromedary Camels, Algeria

Abstract

Prions cause fatal and transmissible neurodegenerative diseases, including Creutzfeldt-Jakob disease in humans, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE). After the BSE epidemic, and the associated human infections, began in 1996 in the United Kingdom, general concerns have been raised about animal prions. We detected a prion disease in dromedary camels (Camelus dromedarius) in Algeria. Symptoms suggesting prion disease occurred in 3.1% of dromedaries brought for slaughter to the Ouargla abattoir in 2015–2016. We confirmed diagnosis by detecting pathognomonic neurodegeneration and disease-specific prion protein (PrPSc) in brain tissues from 3 symptomatic animals. Prion detection in lymphoid tissues is suggestive of the infectious nature of the disease. PrPSc biochemical characterization showed differences with BSE and scrapie. Our identification of this prion disease in a geographically widespread livestock species requires urgent enforcement of surveillance and assessment of the potential risks to human and animal health.

SNIP...

The possibility that dromedaries acquired the disease from eating prion-contaminated waste needs to be considered.

Tracing the origin of prion diseases is challenging. In the case of CPD, the traditional extensive and nomadic herding practices of dromedaries represent a formidable factor for accelerating the spread of the disease at long distances, making the path of its diffusion difficult to determine. Finally, the major import flows of live animals to Algeria from Niger, Mali, and Mauritania (27) should be investigated to trace the possible origin of CPD from other countries.
Camels are a vital animal species for millions of persons globally. The world camel population has a yearly growth rate of 2.1% (28). In 2014, the population was estimated at ≈28 million animals, but this number is probably underestimated.. Approximately 88% of camels are found in Africa, especially eastern Africa, and 12% are found in Asia. Official data reported 350,000 dromedaries in Algeria in 2014 (28).
On the basis of phenotypic traits and sociogeographic criteria, several dromedary populations have been suggested to exist in Algeria (29). However, recent genetic studies in Algeria and Egypt point to a weak differentiation of the dromedary population as a consequence of historical use as a cross-continental beast of burden along trans-Saharan caravan routes, coupled with traditional extensive/nomadic herding practices (30).
Such genetic homogeneity also might be reflected in PRNP. Studies on PRNP variability in camels are therefore warranted to explore the existence of genotypes resistant to CPD, which could represent an important tool for CPD management as it was for breeding programs for scrapie eradication in sheep.
In the past 10 years, the camel farming system has changed rapidly, with increasing setup of periurban dairy farms and dairy plants and diversification of camel products and market penetration (13). This evolution requires improved health standards for infectious diseases and, in light of CPD, for prion diseases.
The emergence of another prion disease in an animal species of crucial importance for millions of persons worldwide makes it necessary to assess the risk for humans and develop evidence-based policies to control and limit the spread of the disease in animals and minimize human exposure. The implementation of a surveillance system for prion diseases would be a first step to enable disease control and minimize human and animal exposure. Finally, the diagnostic capacity of prion diseases needs to be improved in all countries in Africa where dromedaries are part of the domestic livestock.

***> IMPORTS AND EXPORTS <***

***SEE MASSIVE AMOUNTS OF BANNED ANIMAL PROTEIN AKA MAD COW FEED IN COMMERCE USA DECADES AFTER POST BAN ***


USA MAD COW CASE 2018 FLORIDA

WEDNESDAY, SEPTEMBER 26, 2018 

JAVMA In Short Update USDA announces detection of atypical BSE


ZOONOSIS OF SCRAPIE TSE PRION

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. 

============== 


***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. 

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

Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a,

Natalia Fernandez-Borges a. and Alba Marin-Moreno a

"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. France

Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion... Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier.

To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents.

These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant.

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. 

***> why do we not want to do TSE transmission studies on chimpanzees $

5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. 

***> I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. 

***> Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

snip...

R. BRADLEY



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. 


***> 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. <***

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

Emmanuel E. Comoy, Jacqueline Mikol, Sophie Luccantoni-Freire, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Valérie Durand, Capucine Dehen, Olivier Andreoletti, Cristina Casalone, Juergen A. Richt, Justin J. Greenlee, Thierry Baron, Sylvie L. Benestad, Paul Brown & Jean-Philippe Deslys Scientific Reports volume 5, Article number: 11573 (2015) | Download Citation

Abstract 

Classical bovine spongiform encephalopathy (c-BSE) is the only animal prion disease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD) in humans and having guided protective measures for animal and human health against animal prion diseases. Recently, partial transmissions to humanized mice showed that the zoonotic potential of scrapie might be similar to c-BSE. We here report the direct transmission of a natural classical scrapie isolate to cynomolgus macaque, a highly relevant model for human prion diseases, after a 10-year silent incubation period, with features similar to those reported for human cases of sporadic CJD. Scrapie is thus actually transmissible to primates with incubation periods compatible with their life expectancy, although fourfold longer than BSE. Long-term experimental transmission studies are necessary to better assess the zoonotic potential of other prion diseases with high prevalence, notably Chronic Wasting Disease of deer and elk and atypical/Nor98 scrapie.

SNIP...

Discussion We describe the transmission of spongiform encephalopathy in a non-human primate inoculated 10 years earlier with a strain of sheep c-scrapie. Because of this extended incubation period in a facility in which other prion diseases are under study, we are obliged to consider two alternative possibilities that might explain its occurrence. We first considered the possibility of a sporadic origin (like CJD in humans). Such an event is extremely improbable because the inoculated animal was 14 years old when the clinical signs appeared, i.e. about 40% through the expected natural lifetime of this species, compared to a peak age incidence of 60–65 years in human sporadic CJD, or about 80% through their expected lifetimes. 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.

The second possibility is a laboratory cross-contamination. Three facts make this possibility equally unlikely. First, handling of specimens in our laboratory is performed with fastidious attention to the avoidance of any such cross-contamination. Second, no laboratory cross-contamination has ever been documented in other primate laboratories, including the NIH, even between infected and uninfected animals housed in the same or adjacent cages with daily intimate contact (P. Brown, personal communication). Third, the cerebral lesion profile is different from all the other prion diseases we have studied in this model19, with a correlation between cerebellar lesions (massive spongiform change of Purkinje cells, intense PrPres staining and reactive gliosis26) and ataxia. The iron deposits present in the globus pallidus are a non specific finding that have been reported previously in neurodegenerative diseases and aging27. Conversely, the thalamic lesion was reminiscent of a metabolic disease due to thiamine deficiency28 but blood thiamine levels were within normal limits (data not shown). The preferential distribution of spongiform change in cortex associated with a limited distribution in the brainstem is reminiscent of the lesion profile in MM2c and VV1 sCJD patients29, but interspecies comparison of lesion profiles should be interpreted with caution. It is of note that the same classical scrapie isolate induced TSE in C57Bl/6 mice with similar incubation periods and lesional profiles as a sample derived from a MM1 sCJD patient30.

We are therefore confident that the illness in this cynomolgus macaque represents a true transmission of a sheep c-scrapie isolate directly to an old-world monkey, which taxonomically resides in the primate subdivision (parvorder of catarrhini) that includes humans. With an homology of its PrP protein with humans of 96.4%31, cynomolgus macaque constitutes a highly relevant model for assessing zoonotic risk of prion diseases. Since our initial aim was to show the absence of transmission of scrapie to macaques in the worst-case scenario, we obtained materials from a flock of naturally-infected sheep, affecting animals with different genotypes32. This c-scrapie isolate exhibited complete transmission in ARQ/ARQ sheep (332 ± 56 days) and Tg338 transgenic mice expressing ovine VRQ/VRQ prion protein (220 ± 5 days) (O. Andreoletti, personal communication). From the standpoint of zoonotic risk, it is important to note that sheep with c-scrapie (including the isolate used in our study) have demonstrable infectivity throughout their lymphoreticular system early in the incubation period of the disease (3 months-old for all the lymphoid organs, and as early as 2 months-old in gut-associated lymph nodes)33. In addition, scrapie infectivity has been identified in blood34, milk35 and skeletal muscle36 from asymptomatic but scrapie infected small ruminants which implies a potential dietary exposure for consumers.

Two earlier studies have reported the occurrence of clinical TSE in cynomolgus macaques after exposures to scrapie isolates. In the first study, the “Compton” scrapie isolate (derived from an English sheep) and serially propagated for 9 passages in goats did not transmit TSE in cynomolgus macaque, rhesus macaque or chimpanzee within 7 years following intracerebral challenge1; conversely, after 8 supplementary passages in conventional mice, this “Compton” isolate induced TSE in a cynomolgus macaque 5 years after intracerebral challenge, but rhesus macaques and chimpanzee remained asymptomatic 8.5 years post-exposure8. However, multiple successive passages that are classically used to select laboratory-adapted prion strains can significantly modify the initial properties of a scrapie isolate, thus questioning the relevance of zoonotic potential for the initial sheep-derived isolate. The same isolate had also induced disease into squirrel monkeys (new-world monkey)9. A second historical observation reported that a cynomolgus macaque developed TSE 6 years post-inoculation with brain homogenate from a scrapie-infected Suffolk ewe (derived from USA), whereas a rhesus macaque and a chimpanzee exposed to the same inoculum remained healthy 9 years post-exposure1. This inoculum also induced TSE in squirrel monkeys after 4 passages in mice. Other scrapie transmission attempts in macaque failed but had more shorter periods of observation in comparison to the current study. Further, it is possible that there are differences in the zoonotic potential of different scrapie strains.

The most striking observation in our study is the extended incubation period of scrapie in the macaque model, which has several implications. Firstly, our observations constitute experimental evidence in favor of the zoonotic potential of c-scrapie, at least for this isolate that has been extensively studied32,33,34,35,36. The cross-species zoonotic ability of this isolate should be confirmed by performing duplicate intracerebral exposures and assessing the transmissibility by the oral route (a successful transmission of prion strains through the intracerebral route may not necessarily indicate the potential for oral transmission37). However, such confirmatory experiments may require more than one decade, which is hardly compatible with current general management and support of scientific projects; thus this study should be rather considered as a case report.

Secondly, transmission of c-BSE to primates occurred within 8 years post exposure for the lowest doses able to transmit the disease (the survival period after inoculation is inversely proportional to the initial amount of infectious inoculum). The occurrence of scrapie 10 years after exposure to a high dose (25 mg) of scrapie-infected sheep brain suggests that the macaque has a higher species barrier for sheep c-scrapie than c-BSE, although it is notable that previous studies based on in vitro conversion of PrP suggested that BSE and scrapie prions would have a similar conversion potential for human PrP38.

Thirdly, prion diseases typically have longer incubation periods after oral exposure than after intracerebral inoculations: since humans can develop Kuru 47 years after oral exposure39, an incubation time of several decades after oral exposure to scrapie would therefore be expected, leading the disease to occur in older adults, i.e. the peak age for cases considered to be sporadic disease, and making a distinction between scrapie-associated and truly sporadic disease extremely difficult to appreciate.

Fourthly, epidemiologic evidence is necessary to confirm the zoonotic potential of an animal disease suggested by experimental studies. A relatively short incubation period and a peculiar epidemiological situation (e.g., all the first vCJD cases occurring in the country with the most important ongoing c-BSE epizootic) led to a high degree of suspicion that c-BSE was the cause of vCJD. Sporadic CJD are considered spontaneous diseases with an almost stable and constant worldwide prevalence (0.5–2 cases per million inhabitants per year), and previous epidemiological studies were unable to draw a link between sCJD and classical scrapie6,7,40,41, even though external causes were hypothesized to explain the occurrence of some sCJD clusters42,43,44. However, extended incubation periods exceeding several decades would impair the predictive values of epidemiological surveillance for prion diseases, already weakened by a limited prevalence of prion diseases and the multiplicity of isolates gathered under the phenotypes of “scrapie” and “sporadic CJD”.

Fifthly, considering this 10 year-long incubation period, together with both laboratory and epidemiological evidence of decade or longer intervals between infection and clinical onset of disease, no premature conclusions should be drawn from negative transmission studies in cynomolgus macaques with less than a decade of observation, as in the aforementioned historical transmission studies of scrapie to primates1,8,9. Our observations and those of others45,46 to date are unable to provide definitive evidence regarding the zoonotic potential of CWD, atypical/Nor98 scrapie or H-type BSE. The extended incubation period of the scrapie-affected macaque in the current study also underscores the limitations of rodent models expressing human PrP for assessing the zoonotic potential of some prion diseases since their lifespan remains limited to approximately two years21,47,48. This point is illustrated by the fact that the recently reported transmission of scrapie to humanized mice was not associated with clinical signs for up to 750 days and occurred in an extreme minority of mice with only a marginal increase in attack rate upon second passage13. The low attack rate in these studies is certainly linked to the limited lifespan of mice compared to the very long periods of observation necessary to demonstrate the development of scrapie. Alternatively, one could estimate that a successful second passage is the result of strain adaptation to the species barrier, thus poorly relevant of the real zoonotic potential of the original scrapie isolate of sheep origin49. The development of scrapie in this primate after an incubation period compatible with its lifespan complements the study conducted in transgenic (humanized) mice; taken together these studies suggest that some isolates of sheep scrapie can promote misfolding of the human prion protein and that scrapie can develop within the lifespan of some primate species.

In addition to previous studies on scrapie transmission to primate1,8,9 and the recently published study on transgenic humanized mice13, our results constitute new evidence for recommending that the potential risk of scrapie for human health should not be dismissed. Indeed, human PrP transgenic mice and primates are the most relevant models for investigating the human transmission barrier. To what extent such models are informative for measuring the zoonotic potential of an animal TSE under field exposure conditions is unknown. During the past decades, many protective measures have been successfully implemented to protect cattle from the spread of c-BSE, and some of these measures have been extended to sheep and goats to protect from scrapie according to the principle of precaution. Since cases of c-BSE have greatly reduced in number, those protective measures are currently being challenged and relaxed in the absence of other known zoonotic animal prion disease. We recommend that risk managers should be aware of the long term potential risk to human health of at least certain scrapie isolates, notably for lymphotropic strains like the classical scrapie strain used in the current study. Relatively high amounts of infectivity in peripheral lymphoid organs in animals infected with these strains could lead to contamination of food products produced for human consumption. Efforts should also be maintained to further assess the zoonotic potential of other animal prion strains in long-term studies, notably lymphotropic strains with high prevalence like CWD, which is spreading across North America, and atypical/Nor98 scrapie (Nor98)50 that was first detected in the past two decades and now represents approximately half of all reported cases of prion diseases in small ruminants worldwide, including territories previously considered as scrapie free... 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.



Saturday, December 15, 2018 

***> ADRD Summit RFI Singeltary COMMENT SUBMISSION BSE, SCRAPIE, CWD, AND HUMAN TSE PRION DISEASE December 14, 2018


SATURDAY, JANUARY 5, 2019

Low levels of classical BSE infectivity in rendered fat tissue 


***> FRIDAY, DECEMBER 14, 2018 MAD COW USA FLASHBACK Texas Style

FRIDAY DECEMBER 14, 2018 


THURSDAY, JANUARY 3, 2019 

MAD COW USDA DISEASE BSE TSE Prion 


THURSDAY, OCTOBER 22, 2015 

Former Ag Secretary Ann Veneman talks women in agriculture and we talk mad cow disease USDA and what really happened

HOW TO COVER UP MAD COW DISEASE IN TEXAS




TUESDAY, JANUARY 1, 2019 

CHILDHOOD EXPOSURE TO CADAVERIC DURA


JUST OUT CDC;

Tuesday, November 20, 2018

Eyes of CJD patients show evidence of prions Finding could help early diagnosis, raise concern for eye exams and transplants.


Singeltary 1999

***> THE EYES HAVE IT, CJD, AND THEY COULD BE STEALING THEM FROM YOUR LOVED ONE!...year 1999

i said that 20 years ago about this very thing. but did anyone listen...no!

prepare for the storm...terry

year 1999 to 2000


Subject: RE-The Eyes Have It (cjd) and they could be stealing them from your loved one... "pay back time" 

Date: Sat, 16 Sep 2000 10:04:26 -0700 

From: "Terry S. Singeltary Sr." 

Reply-To: Bovine Spongiform Encephalopathy 

To: BSE-L@uni-karlsruhe.de

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

Greetings List Members,

I hate to keep kicking a madcow, but this still is very disturbing to me. Not only for the recipient of the cornea's, but as well, for the people whom would be operated on, using the same tools that were used to put those stolen cornea's in the recipient with. No history of this donor or his family (re-ffi), or anything would be known, using stolen organs and or tissue's. I just think this is not only wrong, but very dangerous to a great many other people, as this is one of the most infectious tissues of TSE's. It seems that this practice of stealing organ/tissue happens more than we think. Anyway, the family of the victim which had their cornea's stolen, are now suing. In the example I used with my Mother, if 3 months before, she would have been in a catastrophic accident (car wreck, whatever), no autopsy (for whatever reason), no family (for whatever reason), she lay in the morgue, and after 4 hours, they come steal the cornea's, lot of people could have been infected, just because of lack of medical history of donor/family. It may be hypothetical, but very real. We need to stop the spread of this disease.

kind regards, Terry S. Singeltary Sr., Bacliff, Texas USA 

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

Previous story--

Cadaver corneal transplants -- without family permission...

Cadaver corneal transplants -- without family permission Houston, Texas channel 11 news 28 Nov 99

Reported by Terry S. Singeltary Sr.son of CJD victim

"It was a story about how the Lions eye bank were harvesting corneas from victims in the Morgue, without their consent. Under Texas law, this appears to be legal (remember Texas has the Veggie liable law). Even if Family says no, this appears to happen, from what the news story said.

They said the only way to prevent this, is to fill out a form, stating not to have this done. So if you don't fill out the form, they can do this. How many people don't know about the form? 

 This is not only disgusting and appalling, it could be highly infectious. Without proper background checking of the donors, on their physical history, checking on past dementia, and/or family history, some of these unfortunate victims, could be passing a human TSE. 

 Response Jill Spitler Clevelland Eye Bank: 

 "No, we are not stealing.........Yes, you do have such a law in the state of Texas, but not all your state Eye Banks utilize the law. The Eye Bank that you're speaking of is only one of 43 certified Eye Bank throughout the USA. 

 And there are measure taken per the Medical Standards of the Eye Bank Association of America, the certifying body for eye banks and per FDA regulations to address those concerns that you speak of. 

 I would suggest that those interested/concern with transplant contact their local agencies. The Eye Bank Association of America has a web. site . Further if anyone has problems contacting or finding out about their local organization(s), call me or e-mail me I would be glad to help. My e-mail address is jill@clevelandeyebank.org

 Terry Singeltary responds: 

 "Explain this to the family in Houston who went to their loved ones funeral, only to find out that the loved one that was in the casket, had their corneas removed without their permission, without the consent of the victim or it's family. They would not have known it, only for the funny look the victim had. So, they questioned, only to find out, the corneas, had in fact, been removed without consent. 

 I call that stealing, regardless what the law states. This type of legal grave robbing is not a logical thing to do without knowing any type of background of the victims medical past, which really will not prove anything due to the incubation period. Eye tissue being potentially a highly infective source, there are risks here. 

 Should they not at least know of the potential ramifications of TSE's (the person receiving the corneas)? 

 Should there not be some sort of screening? 

 Should there be some sort of moral issue here? 

 If this is the case, and in fact, they can come take your corneas, without your consent, then what will they start taking next, without your consent? 

 Lets look at a hypothetical situation: 

 What would happen if my Mom (DOD 12-14-97 hvCJD) would have gotten into a car wreck and died, before the symptoms of CJD appeared. Not much money, so there was no autopsy. What would have happened to that recipient of those infecting corneas?" 

 Comment (webmaster): Actual transmission of CJD by means of corneal transplant may or may not be rare. The incidence of infectivity in older people could be fairly high; this is not to be confused with the lower incidence of symptomatic (clinical) CJD. It is very unlikely that familial CJD would have been diagnosed in earlier generations; however, without interviewing the family even known kindreds would not be excluded. 

 In blood donation, a much stricter policy is followed, even though corneal transplant may be far more dangerous (being a direct link to the brain and not going through purification steps). 

 Since highly sensitive tests for pre-clinical CJD are now available, it would make sense to screen corneas for CJD, just as they are screened for AIDS, hepatitus, and a host of other conditions. 



Eye procedure raises CJD concerns

BySTEVE MITCHELL, Medical Correspondent

WASHINGTON, Nov. 18 (UPI) -- A New York man who died from a rare brain disorder similar to mad cow disease in May underwent an eye procedure prior to his death that raises concerns about the possibility of transmitting the fatal disease to others, United Press International has learned.

The development comes on the heels of the announcement Thursday by U.S. Department of Agriculture officials of a possible second case of mad cow disease in U.S. herds.

Richard Da Silva, 58, of Orange County, N.Y., died from Creutzfeldt Jakob disease, an incurable brain-wasting illness that strikes about one person per million.

Richard's wife Ann Marie Da Silva told UPI he underwent a check for the eye disease glaucoma in 2003, approximately a year before his death. The procedure involves the use of a tonometer, which contacts the cornea -- an eye tissue that can contain prions, the infectious agent thought to cause CJD.

Ann Marie's concern is that others who had the tonometer used on them could have gotten infected.

A 2003 study by British researchers suggests her concerns may be justified. A team led by J.W. Ironside from the National Creutzfeldt-Jakob Disease Surveillance Unit at the University of Edinburgh examined tonometer heads and found they can retain cornea tissue that could infect other people -- even after cleaning and decontaminating the instrument.

"Retained corneal epithelial cells, following the standard decontamination routine of tonometer prisms, may represent potential prion infectivity," the researchers wrote in the British Journal of Ophthalmology last year. "Once the infectious agent is on the cornea, it could theoretically infect the brain."

Prions, misfolded proteins thought to be the cause of mad cow, CJD and similar diseases, are notoriously difficult to destroy and are capable of withstanding most sterilization procedures.

Laura Manuelidis, an expert on these diseases and section chief of surgery in the neuropathology department at Yale University, agreed with the British researchers that tonometers represent a potential risk of passing CJD to other people.

Manuelidis told UPI she has been voicing her concern about the risks of corneas since 1977 when her own study, published in the New England Journal of Medicine, showed the eye tissue, if infected, could transmit CJD.

At the time the procedure was done on Richard Da Silva, about a year before he died, she said it was "absolutely" possible he was infectious.

The CJD Incidents Panel, a body of experts set up by the U.K. Department of Health, noted in a 2001 report that procedures involving the cornea are considered medium risk for transmitting CJD. The first two patients who have a contaminated eye instrument used on them have the highest risk of contracting the disease, the panel said.

In 1999, the U.K. Department of Health banned opticians from reusing equipment that came in contact with patients' eyes out of concern it could result in the transmission of variant CJD, the form of the disease humans can contract from consuming infected beef products.

Richard Da Silva was associated with a cluster of five other cases of CJD in southern New York that raised concerns about vCJD.

None of the cases have been determined to stem from mad cow disease, but concerns about the cattle illness in the United States could increase in light of the USDA announcement Thursday that a cow tested positive on initial tests for the disease. If confirmed, this would be the second U.S. case of the illness; the first was detected in a Washington cow last December. The USDA said the suspect animal disclosed Thursday did not enter the food chain. The USDA did not release further details about the cow, but said results from further lab tests to confirm the initial tests were expected within seven days.

Ann Marie Da Silva said she informed the New York Health Department and later the eye doctor who performed the procedure about her husband's illness and her concerns about the risk of transmitting CJD via the tonometer.

The optometrist -- whom she declined to name because she did not want to jeopardize his career -- "didn't even know what this disease was," she said.

"He said the health department never called him and I called them (the health department) back and they didn't seem concerned about it," she added. "I just kept getting angrier and angrier when I felt I was being dismissed."

She said the state health department "seems to have an attitude of don't ask, don't tell" about CJD.

"There's a stigma attached to it," she said. "Is it because they're so afraid the public will panic? I don't know, but I don't think that the answer is to push things under the rug."

New York State Department of Health spokeswoman Claire Pospisil told UPI she would look into whether the agency was concerned about the possibility of transmitting CJD via tonometers, but she had not called back prior to story publication.

Disposable tonometers are readily available and could avoid the risk of transmitting the disease, Ironside and colleagues noted in their study. Ann Marie Da Silva said she asked the optometrist whether he used disposable tonometers and "he said 'No, it's a reusable one.'"

Ironside's team also noted other ophthalmic instruments come into contact with the cornea and could represent a source of infection as they are either difficult to decontaminate or cannot withstand the harsh procedures necessary to inactivate prions. These include corneal burrs, diagnostic and therapeutic contact lenses and other coated lenses.

Terry Singletary, whose mother died from a type of CJD called Heidenhain Variant, told UPI health officials were not doing enough to prevent people from being infected by contaminated medical equipment.

"They've got to start taking this disease seriously and they simply aren't doing it," said Singletary, who is a member of CJD Watch and CJD Voice -- advocacy groups for CJD patients and their families.

U.S. Centers for Disease Control and Prevention spokeswoman Christine Pearson did not return a phone call from UPI seeking comment. The agency's Web site states the eye is one of three tissues, along with the brain and spinal cord, that are considered to have "high infectivity."

The Web site said more than 250 people worldwide have contracted CJD through contaminated surgical instruments and tissue transplants. This includes as many as four who were infected by corneal grafts. The agency noted no such cases have been reported since 1976, when sterilization procedures were instituted in healthcare facilities.

Ironside and colleagues noted in their study, however, many disinfection procedures used on optical instruments, such as tonometers, fail. They wrote their finding of cornea tissue on tonometers indicates that "no current cleaning and disinfection strategy is fully effective."

Singletary said CDC's assertion that no CJD cases from infected equipment or tissues have been detected since 1976 is misleading.

"They have absolutely no idea" whether any cases have occurred in this manner, he said, because CJD cases often aren't investigated and the agency has not required physicians nationwide report all cases of CJD.

"There's no national surveillance unit for CJD in the United States; people are dying who aren't autopsied, the CDC has no way of knowing" whether people have been infected via infected equipment or tissues, he said.

Ann Marie Da Silva said she has contacted several members of her state's congressional delegation about her concerns, including Rep. Sue Kelly, R-N.Y., and Sen. Charles Schumer, D-N.Y.

"Basically, what I want is to be a positive force in this, but I also want more of a dialogue going on with the public and the health department," she said.


Friday, December 04, 2009

New guidance on decontamination of trial contact lenses and other contact devices has been revealed for CJD AND vCJD


SUNDAY, JANUARY 17, 2016 

Of Grave Concern Heidenhain Variant Creutzfeldt Jakob Disease



TUESDAY, NOVEMBER 20, 2018 

CDC Eyes of CJD patients show evidence of prions concerns for iatrogenic transmission


MONDAY, NOVEMBER 19, 2018 

Benefit cuts hit mad cow disease sufferer A girl born severely disabled from vCJD may lose her home under universal credit


2006-2007

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory

TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. 

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007. 

With all the science to date refuting it, to continue to validate this myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, surgical, blood, medical, cosmetics etc. 

I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. 

This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. 

Accumulation and Transmission are key to the threshold from subclinical to clinical disease, and of that, I even believe that physical and or blunt trauma may play a role of onset of clinical symptoms in some cases, but key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. 

BUT, to continue with this myth that the U.K. strain of BSE one strain in cows, and the nv/v CJD, one strain in humans, and that all the rest of human TSE is one single strain i.e. sporadic CJD (when to date there are 6 different phenotypes of sCJD), and that no other animal TSE transmits to humans, to continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. 

ONE was enough for me, My Mom, hvCJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. 

WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? 

Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. 

There must be a proper classification that will differentiate between all these human TSE in order to do this. 

With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously.

My name is Terry S. Singeltary Sr. and I am no scientist, no doctor and have no PhDs, but have been independently researching human and animal TSEs since the death of my Mother to the Heidenhain Variant of Creutzfeldt Jakob Disease on December 14, 1997 'confirmed'.

...END

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. 


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.


BRITISH MEDICAL JOURNAL

BMJ

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well....

02 January 2000

Terry S Singeltary

retired


US scientists develop a possible test for BSE

BMJ 1999; 319 doi: https://doi.org/10.1136/bmj.319.7220.1312b (Published 13 November 1999) Cite this as: BMJ 1999;319:1312

Rapid responses Response

Re: vCJD in the USA * BSE in U.S.

15 November 1999

Terry S Singeltary

NA

medically retired


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

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

Ermias D. Belay, Ryan A. Maddox, Pierluigi Gambetti, Lawrence B. Schonberger

First published January 28, 2003, DOI: https://doi.org/10.1212/01.WNL.0000036913.87823.D6

Abstract

Transmissible spongiform encephalopathies (TSEs) attracted increased attention in the mid-1980s because of the emergence among UK cattle of bovine spongiform encephalopathy (BSE), which has been shown to be transmitted to humans, causing a variant form of Creutzfeldt-Jakob disease (vCJD). The BSE outbreak has been reported in 19 European countries, Israel, and Japan, and human cases have so far been identified in four European countries, and more recently in a Canadian resident and a US resident who each lived in Britain during the BSE outbreak. To monitor the occurrence of emerging forms of CJD, such as vCJD, in the United States, the Centers for Disease Control and Prevention has been conducting surveillance for human TSEs through several mechanisms, including the establishment of the National Prion Disease Pathology Surveillance Center. Physicians are encouraged to maintain a high index of suspicion for vCJD and use the free services of the pathology center to assess the neuropathology of clinically diagnosed and suspected cases of CJD or other TSEs.

Received May 7, 2002. Accepted August 28, 2002.


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?


Reply to Singletary Ryan A. Maddox, MPH Other Contributors: Published March 26, 2003 

Mr. Singletary raises several issues related to current Creutzfeldt- Jakob disease (CJD) surveillance activities. Although CJD is not a notifiable disease in most states, its unique characteristics, particularly its invariably fatal outcome within usually a year of onset, make routine mortality surveillance a useful surrogate for ongoing CJD surveillance.[1] In addition, because CJD is least accurately diagnosed early in the course of illness, notifiable-disease surveillance could be less accurate than, if not duplicative of, current mortality surveillance.[1] However, in states where making CJD officially notifiable would meaningfully facilitate the collection of data to monitor for variant CJD (vCJD) or other emerging prion diseases, CDC encourages the designation of CJD as a notifiable disease.[1] Moreover, CDC encourages physicians to report any diagnosed or suspected CJD cases that may be of special public health importance (e.g., vCJD, iatrogenic CJD, unusual CJD clusters).

As noted in our article, strong evidence is lacking for a causal link between chronic wasting disease (CWD) of deer and elk and human disease,[2] but only limited data seeking such evidence exist. Overall, the previously published case-control studies that have evaluated environmental sources of infection for sporadic CJD have not consistently identified strong evidence for a common risk factor.[3] However, the power of a case-control study to detect a rare cause of CJD is limited, particularly given the relatively small number of subjects generally involved and its long incubation period, which may last for decades. Because only a very small proportion of the US population has been exposed to CWD, a targeted surveillance and investigation of unusual cases or case clusters of prion diseases among persons at increased risk of exposure to CWD is a more efficient approach to detecting the possible transmission of CWD to humans. In collaboration with appropriate local and state health departments and the National Prion Disease Pathology Surveillance Center, CDC is facilitating or conducting such surveillance and case- investigations, including related laboratory studies to characterize CJD and CWD prions.

Mr. Singletary also expresses concern over a recent publication by Asante and colleagues indicating the possibility that some sporadic CJD cases may be attributable to bovine spongiform encephalopathy (BSE).[4] The authors reported that transgenic mice expressing human prion protein homozygous for methionine at codon 129, when inoculated with BSE prions, developed a molecular phenotype consistent with a subtype of sporadic CJD. Although the authors implied that BSE might cause a sporadic CJD-like illness among persons homozygous for methionine, the results of their research with mice do not necessarily directly apply to the transmission of BSE to humans. If BSE causes a sporadic CJD-like illness in humans, an increase in sporadic CJD cases would be expected to first occur in the United Kingdom, where the vast majority of vCJD cases have been reported. In the United Kingdom during 1997 through 2002, however, the overall average annual mortality rate for sporadic CJD was not elevated; it was about 1 case per million population per year. In addition, during this most recent 6-year period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of UK sporadic CJD deaths.[3, 5] Furthermore, surveillance in the UK has shown no increase in the proportion of sporadic CJD cases that are homozygous for methionine (Will RG, National CJD Surveillance Unit, United Kingdom, 2003; personal communication).

References

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Diagnosis and reporting of Creutzfeldt-Jakob disease. JAMA 2001;285:733-734.

2. Belay ED, Maddox RA, Gambetti P, Schonberger LB. Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States. Neurology 2003;60:176-181.

3. Belay ED. Transmissible spongiform encephalopathies in humans. Annu Rev Microbiol 1999;53:283-314.

4. Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J 2002;21:6358-6366.

5. The UK Creutzfeldt-Jakob Disease Surveillance Unit. CJD statistics. Available at: http://www.cjd.ed.ac.uk/figures.htm. Accessed February 18, 2003.

Competing Interests: None declared.


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..” ............................ 




Terry S. Singeltary Sr.