2026 USDA EXPLANATORY NOTES ANIMAL AND PLANT HEALTH INSPECTION SERVICE CWD, Scrapie, BSE, and CJD, Singeltary Review
“In 2024, APHIS tested over eight percent of farmed cervids within the HCP States for CWD and confirmed 26 CWD-positive herds. The infected herds are currently under State quarantines or have been depopulated. APHIS determines the use of Federal indemnity payments within the CWD program on a case-by-case basis.”
Indemnity or Entitlement CWD TSE Prion Captive Program Update Nov 2026?
2026 USDA EXPLANATORY NOTES - ANIMAL AND PLANT HEALTH INSPECTION SERVICE
APHIS works with State agencies to encourage cervid owners to certify their herds by meeting the requirements in the CWD Herd Certification Program (HCP) Standards. APHIS’ voluntary national CWD HCP helps States, Tribes, and the cervid industry control CWD in farmed cervids by allowing the interstate movement only from certified herds. Currently, 28 States participate in the national CWD HCP. APHIS determines the use of Federal indemnity payments within the CWD program on a case-by-case basis. APHIS also coordinates a voluntary cervid TB herd accreditation program.
In 2024, APHIS made approximately $12 million available for cooperative agreements with States and Tribal governments to further develop and implement CWD surveillance, testing, management, and response activities, including the further development and evaluation of techniques and strategies to prevent or control CWD in farmed and wild cervid populations. APHIS funded cooperative agreements with 30 States, 12 universities, and 10 Tribes and Tribal Organizations for CWD projects in 2024.
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A) A decrease of $6,992,000 and 0 FTE for chronic wasting disease projects. At the direction of Congress, APHIS has facilitated the distribution of funds to further develop and implement CWD surveillance, testing, management, and response activities, including development and evaluation of techniques and strategies to prevent or control CWD in wild and farmed cervids. In recent years, the agency has been unable to fully utilize the funding. Therefore, the Agency proposed to reduce funding in 2026 by $6.992 million. APHIS will continue to use information gathered from previously funded projects to improve the prevention or control of the disease spread in wild and farmed cervid populations. At the proposed funding level, APHIS will continue to fund the highest priority proposals that address CWD research, management, and response activities in farmed and wild cervids.
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Cervids
APHIS works with State agencies to encourage cervid owners to prevent CWD in their herds by enrolling in and meeting the requirements of the CWD Herd Certification Program (HCP) Standards. The goal of the HCP is to provide a consistent, national approach to control the incidence of CWD in farmed cervids and prevent the interstate spread of CWD. In 2024, there were 1,524 herds across 28 States participating in the CWD HCP in the U.S. In 2024, APHIS tested over eight percent of farmed cervids within the HCP States for CWD and confirmed 26 CWD-positive herds. The infected herds are currently under State quarantines or have been depopulated. APHIS determines the use of Federal indemnity payments within the CWD program on a case-by-case basis.
Additionally, APHIS made approximately $12 million available for cooperative agreements with States and Tribal governments to further develop and implement CWD surveillance, testing, management, and response activities. This includes improving management of CWD-affected farmed and wild cervid populations; improving management of CWD-affected areas or premises; researching amplification assays and other new diagnostic methods; and developing and delivering educational materials or programs. APHIS funded cooperative agreements with 30 States, 12 universities, and 10 Tribes and Tribal Organizations for CWD projects.
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Chronic wasting disease (CWD) has been detected in 35 States and impacts numerous wild and farmed populations of deer and elk. Concerns about the impacts of CWD on wild and farmed cervid populations continue to prompt research studies to reduce the spread of the disease and minimize the impact on cervid populations and stakeholders. APHIS researchers use an investigative laboratory procedure called protein misfolding cyclic amplification to amplify minute amounts of CWD infectious material to a level that can be detected. APHIS initiated several multi-
APHIS initiated several multi-2026 USDA EXPLANATORY NOTES - ANIMAL AND PLANT HEALTH INSPECTION SERVICE 22-89
year studies including both field and lab work that involves modern amplification methods which supports research on molecular interactions of CWD to landscape and ecological processes. Additionally, NWRC is housing white-tailed deer at the newly built prion laboratory in Fort Collins, Colorado, to investigate CWD transmission and environmental prion persistence. In 2024, NWRC is continuing to develop a sample archive to retain cervid samples collected by Federal and State agencies for use in diagnostic development and future research.
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Sheep and Goat
Scrapie is a fatal, degenerative disease affecting the central nervous system of sheep and goats. Infected flocks can experience significant production losses. The National Scrapie Eradication Program (NSEP) focuses on improving the health of domestic sheep and goats, reducing scrapie- associated economic losses, and increasing international marketing opportunities. APHIS and State animal health personnel implement NSEP standards to prevent, monitor, and eradicate classical scrapie throughout the United States. All 50 States maintain a Consistent State status under NSEP, where States must conduct an active scrapie control program which is verified through Consistent State reviews. In 2024, APHIS conducted Consistent State reviews in Arizona, Wyoming, Maine, Vermont, New Hampshire, Massachusetts, Connecticut, and Rhode Island. Regulatory scrapie slaughter surveillance efforts began in 2003 and were designed to identify scrapie infected flocks and herds by sampling animals at slaughter. Since then, the program has collected samples from approximately 773,000 animals at slaughter, and only 471 sheep have tested positive for classical scrapie. There hasn’t been a classical scrapie detection since 2021. In 2024, APHIS collected samples from more than 25,000 sheep and goats for scrapie testing, with no animals testing positive for classical or non-classical. Unlike classical scrapie, non-classical scrapie is either not laterally transmissible or is transmissible at a very low rate. The WOAH and APHIS determined that it is not a disease of trade concern.
The NSEP’s voluntary flock certification, the Scrapie Free Flock Certification Program (SFCP), enables producers to enhance the marketability of their animals by monitoring them for scrapie and reducing the risk of introducing scrapie which provides participants an avenue to export sheep
2026 USDA EXPLANATORY NOTES - ANIMAL AND PLANT HEALTH INSPECTION SERVICE 22-52
and goats. In 2024, 161 flocks were enrolled in SFCP. Of these, 31 were export certified (scrapie- free), 22 were export monitored (working towards documenting scrapie freedom), and 108 were select monitored (reduced scrapie risk).
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Bovine spongiform encephalopathy BSE, widely referred to as “mad cow disease,” is a progressive and fatal neurologic disease of cattle. The disease is caused by a transmissible abnormal prion protein. BSE is not a contagious disease and therefore is not spread through casual contact between cattle or with other species. BSE detections are separated into two distinct categories, classical and atypical. Classical BSE occurs through the consumption of contaminated feed. While classical BSE was identified as a significant threat in the 1990s, most years there are no detections made worldwide. This is a result of the successful implementation of effective control measures on an international scale. Atypical BSE refers to naturally and sporadically occurring forms, which are believed to occur in all bovine populations at a very low rate, and which have only been identified in older bovines when conducting surveillance. APHIS works with the USDA FSIS and the Food and Drug Administration to conduct ongoing BSE surveillance, allowing the United States to maintain BSE Negligible Risk status per the World Organisation for Animal Health’s (WOAH) standards to facilitate trade.
The WOAH evaluates countries that submit a request for disease freedom and assigns a points- based risk status for BSE. The BSE surveillance program uses WOAH's weighted surveillance points system, which reflects that the best BSE surveillance program focus on obtaining quality samples from targeted populations rather than looking at the entire adult cattle population. The WOAH’s surveillance points system also incorporates a country’s history with the disease, the implementation and enforcement of cattle feed regulations, and their overall BSE surveillance. As
2026 USDA EXPLANATORY NOTES - ANIMAL AND PLANT HEALTH INSPECTION SERVICE 22-50
of September 1, 2024, the Agency tested for BSE in 21,854 cattle, exceeding the WOAH’s international surveillance standards by nine times the minimum requirement. As a result, APHIS’ BSE surveillance program reassures consumers and international trading partners regarding our ability to detect a problem if one should arise. No cases of classical BSE were detected in 2024.
“Unlike classical scrapie, non-classical scrapie is either not laterally transmissible or is transmissible at a very low rate. The WOAH and APHIS determined that it is not a disease of trade concern.”
remember that…terry
“No cases of classical BSE were detected in 2024.”
what about atypical BSE?
atypical BSE is not reportable, even though it transmits to cattle, sheep, primate by oral routes…terry
USDA EXPLANATORY NOTES ANIMAL AND PLANT HEALTH INSPECTION SERVICE 2025-2014 CHRONIC WASTING DISEASE CWD TSE CERVID
2025 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
In 2023, eight percent of the farmed cervids in the HCP were tested for CWD at APHIS and State laboratories.
Of the 303,242 farmed cervids tested in 2023, APHIS confirmed 22 new CWD positive farmed cervid herds.
APHIS provided Federal indemnity to depopulate one of the newly identified positive herds and approved an indemnity payment for a second positive herd which will be provided in 2024 once depopulation occurs. The remaining infected herds are under State quarantines.
2024 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
Cervids
In 2022, 7 percent of the 285,589 farmed cervids in the HCP participating states were tested for CWD at State and APHIS laboratories.
APHIS confirmed 23 new CWD positive farmed cervid herds.
APHIS provided Federal indemnity to depopulate nine of the newly identified positive herds in 2022. The remaining infected herds are under State quarantines. APHIS determines the use of Federal indemnity payments within the CWD program on a case-by-case basis.
2023 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
Cervids
Currently, 28 States participate in the national CWD HCP. In FY 2021, more than 20,502 farmed cervids were tested for CWD at State and APHIS laboratories.
As a result, APHIS identified 35 new CWD positive farmed cervid herds.
APHIS provided Federal indemnity to depopulate nine of the newly identified deer herds in FY 2021. The remaining infected herds are under State quarantines. APHIS determines the use of Federal indemnity payments within the CWD program on a case-by-case basis.
2022 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
Cervids
Currently, 28 States participate in the national CWD HCP. In FY 2020, more than 11,182 farmed cervids were tested for CWD at State and APHIS laboratories.
As a result, APHIS identified 22 new CWD positive farmed cervid herds.
APHIS provided Federal indemnity to depopulate 15 of the 22 newly identified deer herds in FY 2020.
Four additional farmed cervid herds that were identified as CWD positive herds in FY 2019, were indemnified in FY 2020.
The remaining infected herds are under State quarantines.
2021 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
In FY 2019 APHIS tested more than 11,000 farmed cervids for CWD.
As a result, APHIS identified 17 new CWD positive farmed cervid herds.
APHIS provided Federal indemnity to depopulate 7 of the 17 newly identified deer herds in FY 2019. The remaining infected herds found in FY 2019 are under State quarantines.
2020 USDA EXPLANATORY NOTES – ANIMAL AND PLANT HEALTH INSPECTION SERVICE
Cervids
In FY 2018, APHIS identified 15 new CWD positive farmed cervid herds (14 deer herds and 1 reindeer herd).
The reindeer herd in Illinois was the first confirmed case of CWD in a reindeer in North America.
APHIS provided Federal indemnity to depopulate seven of the 15 newly identified deer herds in FY 2018.
The Agency also provided funding for the test and removal of 161 high risk animals that were in close proximity to reactors.
The remaining herds in FY 2018 are under State quarantines.
The Agency determines the use of Federal indemnities within the CWD program on a case-by-case basis. 20-59
2019 President’s Budget Animal and Plant Health Inspection Service
Cervids
APHIS’ voluntary national CWD Herd Certification Plan (HCP) helps States, Tribes, and the cervid industry control CWD in farmed cervids by allowing the interstate movement only from certified herds.
Currently, 28 States participate in the national CWD HCP and the program tested 23,053 farmed cervids for CWD.
In FY 2017, eight new CWD positive farmed corvid herds were identified– one white-tail deer in Iowa, one white-tail deer herd in Minnesota, one white-tail and mule deer herd in Minnesota, one white-tail and sika deer herd in Michigan, three white-tail deer herds in Pennsylvania, and one white-tail deer herd in Texas.
APHIS provided Federal indemnity to depopulate the Iowa herd, the white-tail deer herd in Minnesota, one herd in Pennsylvania and the Texas herd. The State depopulated the Michigan herd. The remaining herds are under State quarantines. One Texas herd used Federal indemnity to remove and test select, high-risk animals to inform the epidemiological investigation and to evaluate the performance of ante-mortem tests.
The Agency determines the use of Federal indemnities within the CWD program on a case-by-case basis.
2018 President’s Budget Animal and Plant Health Inspection Service
Cervids
APHIS’ voluntary national CWD Herd Certification Plan (HCP) helps States, Tribes, and the cervid industry control CWD in farmed cervids by allowing the interstate movement only from certified herds considered to be low risk.
Currently, 29 States participate in the national CWD HCP.
In FY 2016, the program tested 14,503 farmed cervids for CWD and identified seven new CWD positive farmed cervid herds – two white-tail deer herds in Texas, three white-tail deer herds in Wisconsin, one elk herd in Colorado and one elk herd in Iowa. The elk herd in Colorado was depopulated without Federal indemnity and the rest of the herds are under State quarantines. One Texas herd used Federal indemnity to remove and test select animals to inform the epidemiological investigation and to evaluate 20-72 the performance of ante-mortem tests.
The use of Federal indemnities within the CWD program is determined on a case-by-case basis. APHIS is also conducting several pilot projects related to new technologies. In FY 2016, the Agency
2017 Explanatory Notes Animal and Plant Health Inspection Service
Cervids
APHIS’ voluntary national CWD Herd Certification Plan (HCP) helps States, Tribes, and the cervid industry control CWD in farmed cervids by allowing the interstate movement only from certified herds considered to be low risk.
Currently, 30 States participate in the national CWD HCP: 29 have Approved Status and 1 has Provisional Approved Status. States that meet the CWD HCP requirements have Approved Status and States that do not meet CWD HCP program requirements but have developed a work plan and time frame with APHIS to complete those requirements have Provisional Approved Status.
In FY 2015, the program tested approximately 20,000 farmed cervids for CWD and identified eight new CWD positive farmed white-tailed deer herds – one in Utah, one in Pennsylvania, two in Ohio, two in Wisconsin, and two in Texas.
APHIS depopulated five of these herds (Pennsylvania, Utah, and one each in Wisconsin, Texas, and Ohio). Six elk herds in Colorado, four elk herds in Nebraska, one white-tailed deer herd in Wisconsin and one white-tailed deer herd in Texas remained in quarantine at the end of FY 2015.
APHIS also provided indemnity for and was the lead agency for the depopulation and disposal of four large CWD infected farmed cervid herds in Pennsylvania, Ohio, Utah, and Texas. In cooperation with the National Agricultural Statistics Service, APHIS conducted the first national study of the U.S. farmed-cervid industry in FY 2015. The study provides baseline industry statistics, a description of production practices and challenges, producer-reported disease occurrences, and an overview of health management and biosecurity practices.
2016 Explanatory Notes Animal and Plant Health Inspection Service
In FY 2014, the program tested approximately 20,000 farmed cervids for CWD.
Two new CWD positive farmed white-tailed deer herds were identified – one in Pennsylvania and one in Wisconsin.
The program depopulated the PA herd and two additional CWD positive herds in quarantine since FY 2012 in Iowa (white-tailed deer herd) and Minnesota (red deer herd).
Six elk herds in Colorado, four elk herds in Nebraska, and one white-tailed deer in Wisconsin remained in quarantine at the end of FY 2014.
There also are numerous CWD exposed herds that are epidemiologically linked to CWD positive herds that remain in State quarantine pending completion of the epidemiology investigations.
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APHIS provided indemnity for and was the lead agency for the depopulation and disposal of two large CWD infected farmed cervid herds in Iowa and Minnesota.
APHIS also provided indemnity for and assisted with the 20-80 appraisal and depopulation of a CWD infected farmed cervid herd in Pennsylvania.
APHIS also provided assistance to States with outbreak investigation, assessment of risk posed by infected or exposed animals, development of herd plans and continues to develop strategies for the purpose of controlling and managing CWD in farmed cervids.
2015 Explanatory Notes Animal and Plant Health Inspection Service
In FY 2013, the program tested approximately 18,100 farmed cervids for CWD, a fatal, degenerative disease that affects the central nervous system and lymphoid system of cervids. Through this routine surveillance, no new CWD cases were reported in farmed cervids in FY 2013. The last CWD positive herd was reported in FY 2012 in an Iowa white tail deer herd. Twelve positive herds remain (seven elk herds in Colorado, three elk herds in Nebraska, one white tail deer herd in Iowa, and one red deer herd in Minnesota).
2014 Explanatory Notes Animal and Plant Health Inspection Service
In 2007, the cervid industry in the United States included 5,600 deer farms and 1,900 elk farms with an economic value of $894 million that supported nearly 30,000 jobs.
APHIS’ main cervid activities are testing approximately 15,000 captive cervids for tuberculosis each year and supporting the chronic wasting disease (CWD) herd certification program (HCP). The joint tuberculosis (TB) and brucellosis proposed rule will represent significant changes to TB activities in captive cervids when implemented; it proposes to bring cervids into the regulatory program for brucellosis, as requested by stakeholders. As a result, surveillance will be enhanced and the number of captive cervids that are tested for TB annually is expected to increase. In 2014, APHIS will implement a survey to evaluate the effectiveness and impact of new APHIS regulations on the industry. Additionally, approval for a new diagnostic test for TB in captive cervids is expected to occur by FY 2013. The CWD HCP allows participating States to enroll herd owners
18-26
to meet minimum Federal standards to achieve and maintain a herd certification status. APHIS approves State applications for the national voluntary CWD herd certification program, conducts periodic reviews to ensure compliance, and supports confirmatory testing of presumptive CWD cases.
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Without continued program funding, there would be a reduced preparedness, surveillance, and response to equine/cervid health issues that could increase the likelihood of disease spread resulting in larger and more serious disease outbreaks, lack of national standards leading to a patchwork of State level requirements which diminish interstate commerce, and loss in international credibility regarding U.S. animal health status.
Approximately 59 percent of the Equine and Cervid Health funding will be used for salaries and benefits, less than 1 percent for cooperative agreements and programmatic contracts, and the remaining supports normal operating costs such as travel, supplies, rent and utilities.
Reduce lower priority program activities (-$1.295 million) APHIS will reduce lower priority equine and cervid program activities in 2014, including eliminating Federal contributions for addressing CWD.
CWD is a degenerative neurological illness affecting elk and deer (cervids) in North America.
***> APHIS has determined that continued efforts to manage CWD are not practical and therefore considers this to be a low priority for the Agency.
***> On August 13, 2012, the rule that established uniform standards for a voluntary Federal-State cooperative CWD HCP and interstate movement requirements became effective. Implementation of the interstate movement of cervids was implemented in December 2012.
***> Many States have herd certification programs in place, and the incidence of CWD detections in farmed cervids is decreasing.
***> With the regulatory framework in place, continued APHIS activity, while useful, is no longer essential.
Stakeholders can continue to carry on program activities. The success of the voluntary HCP is based upon cooperation and shared responsibility among the Federal government and State and local interests. However, since these are local or regional disease spread issues, State and local governments are better positioned to take a more active role and to better anticipate and plan for local or regional needs. APHIS will continue to conduct higher priority equine and cervid health activities and address concerns when identified. APHIS will reassign staff years to other Equine and Cervid Health activities as practical and reduce the overall staff years by eliminating the positions when vacancies arise. Reduction in Agency-level operating expenses (-$66,000) A reduction of $66,000 is requested for this line item related to Agency-level cost savings measures and operating efficiencies. Please refer to second paragraph on page 18-18.
18-27
Pay Increase (+$15,000)
An increase of $15,000 for pay costs which includes $3,000 for annualization of the FY 2013 pay raise and $12,000 for the anticipated FY 2014 pay raise.
***> APHIS has determined that continued efforts to manage CWD are not practical and therefore considers this to be a low priority for the Agency.
***> On August 13, 2012, the rule that established uniform standards for a voluntary Federal-State cooperative CWD HCP and interstate movement requirements became effective. Implementation of the interstate movement of cervids was implemented in December 2012.
***> Many States have herd certification programs in place, and the incidence of CWD detections in farmed cervids is decreasing.
***> With the regulatory framework in place, continued APHIS activity, while useful, is no longer essential.
***> The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite its subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA viewed it as a wildlife problem and consequently not their province!” page 26.
July 31, 2026
Classical Scrapie Confirmed in Oregon Sheep Through Routine Surveillance
Featured Image Post
Routine slaughter testing detected the case. There is no known risk to people, and most Oregon producers are unaffected.
The Oregon Department of Agriculture (ODA), in collaboration with the US Department of Agriculture's (USDA) Animal and Plant Health Inspection Service (APHIS), is announcing the confirmation of classical scrapie in a sheep slaughtered in a facility in Oregon. The animal was tested as part of USDA's ongoing national scrapie surveillance program, which is designed to identify classical scrapie in sheep and goats at slaughter facilities.
Classical scrapie is a fatal, degenerative prion disease of sheep and goats. The disease can be present in apparently healthy animals, making slaughter surveillance critical for early detection. Classical scrapie typically requires several years to produce clinical signs, and affected animals generally live only months after symptom onset.
Upon confirmation, ODA began an epidemiological investigation to identify the flock of origin and determine whether any potentially exposed animals are located within Oregon. This joint response supports the national effort to eradicate classical scrapie from the United States. Before this confirmation, the United States had gone more than five years without a case. The United States was positioned to meet WOAH's 7-year benchmark for self-declared scrapie freedom in January 2028. USDA says the confirmed detection could reset that timeline and may push the earliest possible date for national scrapie-free status to approximately 2033.
APHIS expects minimal impact on trade. Most countries restrict live animal exports only from affected flocks or premises, and products such as wool remain unaffected.
ODA asks Oregon sheep and goat producers to take four steps:
Keep official identification current. Sheep and goats need official ID before they leave the premises where they were born, so animals can be traced if a case is found. Watch for signs. Rubbing or scratching against fences and posts, tremors, an unsteady or high-stepping walk, changes in behavior, and weight loss in an animal that is still eating well. Call your veterinarian right away if you see those signs. Oregon law requires veterinarians to notify ODA by phone as soon as they suspect scrapie, before any lab testing. You can also call ODA's disease reporting line directly at 503-986-4711.
Ask your veterinarian about breeding for scrapie resistance. Some sheep carry genetics that make them highly resistant to classical scrapie, and breeding for those traits protects a flock over time.
For more information about Oregon's scrapie surveillance program, producers may visit ODA's scrapie website at https://oda.direct/scrapie. More information about scrapie and APHIS' eradication program is available at:www.aphis.usda.gov .
Producers or owners who suspect an animal disease should contact their veterinarian to evaluate the animal or herd. Scrapie is a reportable disease in Oregon. Veterinarians and diagnostic laboratories must immediately report suspected cases to ODA's State Veterinarian.
USDA Confirms Detection of Classical Scrapie in a Slaughtered Sheep
The U.S. Department of Agriculture’s (USDA) Animal and Plant Health Inspection Service (APHIS) has confirmed classical scrapie in a sheep slaughtered at a facility in Oregon. Final diagnostic results and genotype analysis were completed by the National Veterinary Services Laboratories (NVSL), with confirmatory testing indicating classical scrapie. As required, APHIS has notified the World Organisation for Animal Health (WOAH) of the confirmed detection.
Classical scrapie is a fatal, degenerative prion disease of sheep and goats. Prior to this confirmation, the United States had gone more than 5 years without a case. The United States was positioned to meet WOAH’s 7‑year benchmark for self‑declared scrapie freedom in January 2028. This confirmed detection could reset that timeline and may push the earliest possible date for national scrapie‑free status to approximately 2033.
APHIS and State animal health officials have initiated an epidemiological investigation to determine the flock of origin. The affected animal did not have an ear tag, which may limit traceability and highlights the critical importance of strong animal identification practices nationwide. To support the investigation, NVSL will conduct tissue‑matching and parentage testing on the affected sheep and two additional animals from the same accession.
APHIS expects minimal impact on trade. Most countries restrict live animal exports only from affected flocks or premises, and products such as wool remain unaffected.
USDA encourages producers to reinforce key practices that have advanced scrapie eradication efforts nationwide:
Strict compliance with ear tagging and traceability requirements to ensure exposed animals can be accurately tracked.
Robust surveillance participation, including submission of clinical suspects and routine monitoring.
Use of genetically resistant breeding stock, which significantly reduces disease susceptibility and depopulation needs.
Continued partnership with APHIS and State officials to support investigation and long‑term freedom efforts.
More information about scrapie and APHIS’ eradication program is available at www.aphis.usda.gov.
April 22, 2016
Scrapie Confirmed in a Hartley County Sheep
AUSTIN - Texas Animal Health Commission (TAHC) officials have confirmed scrapie in a Hartley County ewe. The ewe was tested by TAHC after the owner reported signs of weight loss and lack of coordination to their local veterinarian. The premises was quarantined and a flock plan for monitoring is being developed by the TAHC and USDA.
"The TAHC is working closely with the flock owner, sharing all of the options for disease eradication," said Dr. David Finch, TAHC Region 1 Director. "We are thankful the producer was proactive in identifying a problem and seeking veterinary help immediately."
Texas leads the nation in sheep and goat production. Since 2008, there have been no confirmed cases of scrapie in Texas. The last big spike in Texas scrapie cases was in 2006 when nine infected herds were identified and the last herd was released from restrictions in 2013.
According to USDA regulations, Texas must conduct adequate scrapie surveillance by collecting a minimum of 598 sheep samples annually. Since USDA slaughter surveillance started in FY 2003, the percent of cull sheep found positive for scrapieat slaughter (once adjusted for face color) has decreased 90 percent.
Scrapie is the oldest known transmissible spongiform encephalopathies, and under natural conditions only sheep and goats are known to be affected by scrapie. It is a fatal disease that affects the central nervous system of sheep and goats. It is not completely understood how scrapie is passed from one animal to the next and apparently healthy sheep infected with scrapie can spread the disease. Sheep and goats are typically infected as young lambs or kids, though adult sheep and goats can become infected.
The most effective method of scrapie prevention is to maintain a closed flock. Raising replacement ewes, purchasing genetically resistant rams and ewes,or buying from a certified-free scrapie flock are other options to reduce the risk of scrapie. At this time the resistant genetic markers in goats have not been identified, therefore it is important to maintain your sheep and goat herds separately.
The incubation period for Scrapie is typically two to five years. Producers should record individual identification numbers and the seller's premise identification number on purchase and sales records. These records must be maintained for a minimum of five years.
Producers should notify the Texas Animal Health Commission (800-550-8242) or the USDA-Austin Office (512-383-2400) if they have an adult sheep or goat with neurologic signs such as incoordination, behavioral changes, or intense itching with wool loss. Producers may order scrapie identification tags by calling 866-873-2824. For more information, please visit our website at:
http://www.tahc.texas.gov/animal_health/scrapie/scrapie.htm
web.archive.org/web/20160607024701/http://www.tahc.texas.gov/news/pr/2016/2016-04-22_TAHCScrapie.pdf
Scrapie: The flock identified in April 2016 remains under quarantine in Hartley County.
Atypical Nor-98 Scrapie USA To Date
USA atypical Nor-98, CH1641, Scrapie, TSE Prion, Update 2026
USA atypical Nor-98 Scrapie Update 2025 USA Scrapie typical and atypical USA
FY 2023, 16,646 from sheep and 8,726 from goats.
* There have been 491 NVSL confirmed positive animals (474 classical cases – 471 sheep and 3 goats) and
***> 19 Nor98- like cases since the beginning of RSSS. Figure 3 depicts RSSS collection sites in FY 2023.
In 2023, APHIS collected samples from more than 26,000 sheep and goats for scrapie testing. Out of the total number of animals tested in 2023, no animals tested positive for classical scrapie and
***> one sheep tested positive for non-classical scrapie (Nor98-like)...
In 2022, APHIS collected samples from more than 23,000 sheep and goats for scrapie testing. Out of the total number of animals tested in 2022, no animals tested positive for classical scrapie and
***> one sheep tested positive for non-classical scrapie (Nor98-like).
In FY 2021, APHIS collected samples from more than 30,000 sheep and goats for scrapie testing. Out of the total number of samples processed and reported in FY 2021,
***> one sheep tested positive for classical scrapie and ***> one sheep tested positive for non-classical scrapie (Nor98-like).
In FY 2020, APHIS collected samples from 33,839 sheep and goats for scrapie testing. This number represents sample results reported by October 15, 2020 and is expected to slightly increase as the remaining results are processed and reported. No animals tested positive for classical scrapie.
***> Two sheep tested positive at slaughter for non-classical scrapie (Nor98-like).
In FY 2019, APHIS collected samples from 34,730 sheep and goats for scrapie testing, ***> detecting 7 classical scrapie positive animals. Of these animals, five sheep and one goat were from a source flock in Pennsylvania that was found in August 2018, depopulated in October 2018, and tested for scrapie in November 2018. A second goat, which was from an Indiana herd, was sampled at slaughter in June 2019. The source flock completed a cleanup plan and was placed on a 5-year monitoring plan. The source herd of the positive Indiana goat no longer contained any exposed animals and was also placed on a 5-year monitoring plan. A trace-back investigation narrowed the goat’s birth herd to two possible herds. Animals in both herds tested negative for scrapie and were placed on 5-year monitoring plans. There were no classical scrapie cases detected in slaughter sheep in FY 2019.
***> Also in FY 2019, two sheep tested positive at slaughter for non-classical scrapie (Nor98-like) and were traced back to Colorado flocks.
***> In FY 2018, APHIS collected samples from 43,625 sheep and goats for scrapie testing, detecting three positive (0.0068%) cases. These figures are based on sample submissions and testing completed by September 30, 2018. FY 2018 values are expected to change when testing is completed for all animals sampled in FY 2018.
***> In October 2017, a non-classical scrapie case was detected in a sheep from Virginia sampled at slaughter. As a result, the non- classical scrapie affected flock was placed on a 5-year monitoring plan.
***> In FY 2016, the program identified one flock infected with classical scrapie and one infected with Nor98-like scrapie through slaughter surveillance, and two flocks infected with classical scrapie through on-farm surveillance.
***> An additional 10 sheep were confirmed with classical scrapie through testing of sheep depopulated from these infected flocks as part of flock clean-up activities conducted in FY 2016.
***> The Nor98-like scrapie affected flock will be placed on a 5-year monitoring plan.
Atypical Nor-98 Scrapie TSE Prion USA State by State Update January 2021
Atypical Nor-98 Scrapie TSE Prion USA State by State Update January 2021
Nor98 cases Diagnosed in the US. To Date
Nor98 cases Diagnosed in the US.
Flock of Origin State FY
Wyoming 2007
Indiana 2007
Pennsylvania 2008
Oregon 2010
Ohio 2010
Pennsylvania 2010
Untraceable 2010
California 2011
Montana 2016
Utah 2017
Montana 2017
Virginia 2018
Colorado 2019
Colorado 2019
Wyoming 2020
Montana 2020
Pennsylvania 2021
Personal Communication from USDA et al Mon, Jan 4, 2021 11:37 am...terry
TUESDAY, SEPTEMBER 22, 2020
APHIS USDA MORE SCRAPIE ATYPICAL Nor-98 Confirmed USA September 15 2020
17 cases of the Nor98 in the USA to date, location, unknown...tss
17 Nor98-like cases since the beginning of RSSS.
17 Nor98-like cases since the beginning of RSSS. No animals have tested positive for classical scrapie in FY 2021.
TUESDAY, SEPTEMBER 22, 2020
APHIS USDA MORE SCRAPIE ATYPICAL Nor-98 Confirmed USA September 15 2020
MONDAY, JULY 27, 2020
APHIS USDA Nor98-like scrapie was confirmed in a sheep sampled at slaughter in May 2020
MONDAY, JULY 13, 2020
Efficient transmission of classical scrapie agent x124 by intralingual route to genetically susceptible sheep with a low dose inoculum
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;
WEDNESDAY, MAY 29, 2019
***> Incomplete inactivation of atypical scrapie following recommended autoclave decontamination procedures
THURSDAY, DECEMBER 31, 2020
Autoclave treatment of the classical scrapie agent US No. 13-7 and experimental inoculation to susceptible VRQ/ARQ sheep via the oral route results in decreased transmission efficiency
Snip…see full text;
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.***
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.
O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations
*** 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.
==============
PRION 2015 CONFERENCE
***> 14) Concluding that atypical scrapie can transmit to Humans and that its strain properties change as it transmits between species <***
*** Grant Agreement number: 222887 ***
*** Project acronym: PRIORITY ***
*** Project title: Protecting the food chain from prions: shaping European priorities through basic and applied research Funding ***
Scheme: Large-scale integrating project Period covered: from Oct. 1, 2009 to Sept. 30, 2014
Name of the scientific representative of the project's co-ordinator1, Title and Organisation: Jesús R. Requena, Ph.D., Associate Professor, Department of medicine, University of Santiago de Compostela, Spàin. Tel: 34-881815464 Fax: 34-881815403 E-mail: jesus.requena@usc.es
Project website¡ Marcador no definido. address: www.prionpriority.eu
PRIORITY, PROJECT FINAL REPORT
***> 14) Concluding that atypical scrapie can transmit to Humans and that its strain properties change as it transmits between species <***
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see full text;
Block D: Prion epidemiology
Studies on atypical scrapie were identified as a key element of this block, given the potential risk associated to this agent. We studied the permeability of Human, bovine and porcine species barriers to atypical scrapie agent transmission. Experiments in transgenic mice expressing bovine, porcine or human PrPC suggest that this TSE agent has the intrinsic ability to propagate across these species barriers including the Human one. Upon species barrier passage the biological properties and phenotype of atypical scrapie seem to be altered. Further experiments are currently ongoing (in the framework of this project but also in other projects) in order to: (i) characterize the properties of the prion that emerged from the propagation of atypical scrapie in tg Hu; (ii) to confirm that the phenomena we observed are also true for atypical scrapie isolates other than the ones we have studied.
In parallel, studies in sheep have concluded that:
*** Atypical scrapie can be transmitted by both oral and intracerebral route in sheep with various PRP genotypes
*** Low but consistent amount of infectivity accumulates in peripheral tissue (mammary gland, lymph nodes, placenta, skeletal muscles, nerves) of sheep incubating atypical scrapie.
*** The combination of data from all our studies leads us to conclude that:
*** Atypical scrapie passage through species barriers can lead to the emergence of various prions including classical BSE (following propagation in porcine PRP transgenic mice).
*** Atypical scrapie can propagate, with a low efficacy, in human PrP expressing mice. This suggests the existence of a zoonotic potential for this TSE agent.
snip...
We advance our main conclusions and recommendations, in particular as they might affect public policy, including a detailed elaboration of the evidence that led to them. Our main recommendations are:
a. The issue of re-introducing ruminant protein into the food-chain The opinion of the members of Priority is that sustaining an absolute feed ban for ruminant protein to ruminants is the essential requirement, especially since the impact of non-classical forms of scrapie in sheep and goats is not fully understood and cannot be fully estimated. Therefore, the consortium strongly recommends prohibiting re-introduction of processed ruminant protein into the food-chain. Arguments in support of this opinion are:
• the large (and still uncharacterized) diversity of prion agents that circulate in animal populations;
• the uncertainties related to prion epidemiology in animal populations;
• the unknown efficacy of industrial processes applied to reduce microbiological risk during processed animal protein (PAP) production on most prion agents; • the intrinsic capacity of prions to cross interspecies transmission barriers; • the lack of sensitive methodology for identifying cross contamination in food.
• the evolution of natural food chains in nature (i.e. who eats whom or what) has generated an efficient barrier preventing, to some extent, novel prion epidemies and that this naturally evolved ecology should be respected.
The consortium is also hesitant to introduce processed ruminant proteins into fish food considering the paucity of data on prion infections in fishes and sea animals including those of mammalian origin, and the risk of establishing an environmental contamination of the oceans that cannot be controlled.
b. Atypical prion agents and surveillance
Atypical prion agents (see below) will probably continue to represent the dominant form of prion diseases in the near future, particularly in Europe.
*** Atypical L-type BSE has clear zoonotic potential, as demonstrated in experimental models.
*** Similarly, there are now some data that seem to indicate that the atypical scrapie agent can cross various species barriers.
*** Moreover, the current EU policy for eradicating scrapie (genetic selection in affected flocks) is ineffective for preventing atypical scrapie.
*** The recent identification of cell-to-cell propagation and the protein-encoded strain properties of human neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, suggest that they bear the potential to be transmissible even if not with the same efficiency as CJD. More epidemiological data from large cohorts are necessary to reach any conclusion on the impact of their transmissibility on public health. Re-evaluations of safety precautions may become necessary depending on the outcome of these studies. In that context it would appear valuable
• to develop knowledge related to the pathogenesis and inter-individual transmission of atypical prion agents in ruminants (both intra- and inter-species)
• to improve the sensitivity of detection assays that are applied in the field towards this type of agent
• to maintain a robust surveillance of both animal and human populations
c. The need for extended research on prions
Intensified searching for a molecular determinants of the species barrier is recommended, since this barrier is a key for many important policy areas - risk assessment, proportional policies, the need for screening of human products and food. In this respect, prion strain structural language also remains an important issue for public health for the foreseeable future. Understanding the structural basis for strains and the basis for adaptation of a strain to a new host will require continued fundamental research. Prions maintain a complex two-way relationship with the host cell and fundamental research is needed on mechanisms for their transmission, replication and cause of nervous system dysfunction and death.
Early detection of prion infection, ideally at preclinical stage, also remains crucial for development of effective treatment strategies in humans affected by the disease.
Position of the Priority consortium
Nearly 30 years ago, the appearance in the UK of Bovine Spongiform Encephalopathy (BSE) quickly brought the previously obscure “prion diseases” to the spotlight. The ensuing health and food crises that spread throughout Europe had devastating consequences. In the UK alone, there were more than 36,000 farms directly affected by BSE and the transmission of BSE prions to humans via the food chain has caused over 200 people in Europe to die from variant Creutzfeldt-Jakob disease (vCJD) (http://www.cjd.ed.ac.uk
Origins of prion epidemies
Classical BSE now appears to be under control, with 18 EU Member States having achieved the World Organisation for Animal Health (Office International Epizooties) „negligible risk‟ status (May 2014; http://www.oie.int/en/animal-health-in-the-world/official-disease-status/bse/list-of-bse-risk-status/), and the remaining MS assessed as „controlled‟ risk. Of note, research, including EU-funded research, has played a key role in this success: while the origin of the infection was never defined, the principle driver of the epidemic was identified as prions in Meat and Bone Meal (MBM). Tests based on prion protein-specific antibodies were developed, allowing detection of infected animals, and a better understanding of disease pathogenesis and the distribution of infectivity in edible tissues; experimental investigation of transmission barriers between different species allowed a rational estimation of risks, etc. All of this led to the implementation of rational and effective policies, such as the MBM ban to protect the animal feed chain, and the Specified Risk Material (SRM) regulations to protect the human food chain.
In spite of this progress, prions are still a threat. Epidemiological re-assessment indicates that the ∼10 year incubation period separating the peaks of the BSE and the vCJD epidemics is probably too short. In addition, results from a large number of human tonsil and appendix analyses in the UK suggest that there may be a high number of asymptomatic individuals who are positive for the disease-associated conformer prion protein PrPSc. While vCJD is the only form of human prion disease that has been consistently demonstrated to have lymphoreticular involvement, there has been no systematic investigation of lymphoid tissue in cases with other prion diseases.
The human prion problem
The clinical cases of vCJD identified to date have all shared a common PrP genotype (M129M), although one pre-clinical case was confirmed as an M129V heterozygote, and it has been mooted that perhaps only the M129M proportion of the population is susceptible. However, in the UK appendix study, PrP accumulation was described in samples representing every codon 129 genotype, raising the possibility that genotype does not confer resistance but instead modulates incubation period. Apart from the two UK studies, the lymphoid tissues of non-CJD patients have not been examined for the presence of PrPSc, so, these cases may not solely represent pre-clinical vCJD, but also other forms of prion disease.
Recent experiments in highly susceptible mouse models indicate the presence of infectivity in blood or blood components at late disease stages in sporadic CJD. The significance of this experimental finding for humans has to be explored in more detail and, at the present time, there is no evidence for the transmission of prions via blood in sporadic CJD. However a likely scenario is that all those with signs of infection or abnormal PrP accumulation in peripheral tissue could have infective blood, posing the risk for transmission via blood products, which has been clearly demonstrated in experimental models, and confirmed in several cases of vCJD in man. Altogether, these data clearly demonstrate the potential risk of a second wave of vCJD, particularly when the number people identified with lymphoid accumulation of PrPSc (16/32,411) gives a prevalence estimate in the UK of 493 per million, much higher than the number of clinical cases seen to date.
The animal prion problem
An increasing number of reports on cases of “atypical” BSE in cattle throughout the EU and beyond may lead to a new epidemic, particularly since we still do not understand all factors determining the species barrier. Ovine scrapie is another concern, because it could mask ovine BSE, presumably transmissible to humans. Scrapie is endemic and not likely to be eradicated soon, although current control measures are effective at greatly reducing disease incidence. Atypical forms, which may be spontaneous, are not affected by these control measures and these forms of disease will persist in the global animal population. The low prevalence of these disease forms makes effective surveillance very challenging. However, there is a clear risk attendant on ignoring these cases without an understanding of their possible zoonotic potential, particularly when most forms of human disease have no established aetiology. In summary, atypical cases of BSE and scrapie presently clearly outnumber classical cases in cattle and sheep in all member states.
We will highlight the state-of-the-art knowledge and point out scientific challenges and the major questions for research. Strategic objectives and priorities in Europe in the future for research that aims to control, eliminate or eradicate the threat posed by prions to our food and health are also indicated.
The Priority project has focused on 4 themes, namely the structure, function, conversion and toxicity of prions; detection of prions; mechanisms of prion transmission and spreading and epidemiology of prion diseases. This paper summarizes the opinions/positions reached within these themes at the end of the project.
see;
“Unlike classical scrapie, non-classical scrapie is either not laterally transmissible or is transmissible at a very low rate. The WOAH and APHIS determined that it is not a disease of trade concern.”
Classical BSE emergence from Nor98/atypical scrapie: Unraveling the shift vs. selection dichotomy in the prion field
Sara Canoyra, Alba Marín-Moreno, Juan Carlos Espinosa, +5 , and Juan María Torres
jmtorres@inia.csic.esAuthors Info & Affiliations Edited by Byron Caughey, National Institute of Allergy and Infectious Diseases (National Institutes of Health), Hamilton, MT; received January 17, 2025; accepted June 7, 2025 by Editorial Board Member Lila M. Gierasch July 15, 2025 122 (29) e2501104122 https://doi.org/10.1073/pnas.2501104122 1,865 2 Metrics
Significance
Classical bovine spongiform encephalopathy (c-BSE) is a fatal cattle prion disease transmissible to humans as variant Creutzfeldt–Jakob Disease (vCJD). Understanding how c-BSE emerges is crucial for preventing future outbreaks and protecting public health. Two main hypotheses explain prion adaptation during cross-species transmission: “conformational shift or deformed templating,” where the species barrier forces a change to a different pathological prion protein, and “conformational selection,” where the species barrier filters preexisting conformers. Our results demonstrate that the conformational shift mechanism explains the emergence of c-BSE when Nor98/atypical scrapie (AS) is transmitted to cattle. This is significant because AS, found in sheep and goats worldwide, can convert to c-BSE. Preventing AS from entering the food chain is crucial to reduce c-BSE/vCJD risks.
Abstract
Prion diseases can manifest with distinct phenotypes in a single species, a phenomenon known as prion strains. Upon cross-species transmission, alterations in the disease phenotype can occur, interpreted as the emergence of a new strain. Two main and non–mutually exclusive evolutionary hypotheses have been proposed to explain this phenomenon: the “conformational shift” or “deformed templating” and the “conformational selection.” The conformational shift hypothesis proposes that the introduction of a new host prion protein (PrPC) forces a change in the conformation of the pathological prion protein (PrPSc), causing the new prion strain emergence. On the contrary, the conformational selection model postulates that prion isolates are a conglomerate of PrPSc conformations with relative distribution frequencies, wherein the species barrier acts as a filter selecting the one fittest for the new species environment. Previous studies reported the emergence of the classical bovine spongiform encephalopathy agent (c-BSE) upon transmission of Nor98/atypical scrapie (AS) onto a bovine PrP. This study investigates the evolutionary dichotomy of this c-BSE emergence by using prion strain thermostability combined with protein misfolding cyclic amplification to distinguish between both strains. Our results suggest that the conformational shift could be the principal mechanism responsible for the c-BSE emergence. Furthermore, the selection model was dismissed as the key mechanism based on the analysis of an artificial c-BSE and AS mixture. The ability of the AS conformers to shift conformation to a c-BSE one supports the hypothesis that the epidemic c-BSE prion may have originated from the transmission of AS in cattle.
“The ability of the AS conformers to shift conformation to a c-BSE one supports the hypothesis that the epidemic c-BSE prion may have originated from the transmission of AS in cattle.”
“Unlike classical scrapie, non-classical scrapie is either not laterally transmissible or is transmissible at a very low rate. The WOAH and APHIS determined that it is not a disease of trade concern.”
UH OH!…terry
Volume 26, Number 6—June 2020
Research
Radical Change in Zoonotic Abilities of Atypical BSE Prion Strains as Evidenced by Crossing of Sheep Species Barrier in Transgenic Mice
Atypical BSE prions showed a modification in their zoonotic ability after adaptation to sheep-PrP producing agents able to infect TgMet129 and TgVal129, bearing features that make them indistinguishable of sporadic Creutzfeldt-Jakob disease prions.
Conversion of the BASE Prion Strain into the BSE Strain: The Origin of BSE?
Raffaella Capobianco, Cristina Casalone, Silvia Suardi, Michela Mangieri, Claudia Miccolo, Lucia Limido, Marcella Catania, Giacomina Rossi, Giuseppe Di Fede, Giorgio Giaccone, Maria Grazia Bruzzone, Ludovico Minati, Cristiano Corona, [ ... ], Fabrizio Tagliavini [ view all ]
Published: March 9, 2007
In this study, we show that an atypical form of spongiform encephalopathy of cattle, termed BASE, is caused by a prion strain distinct from that of classical BSE.
***> Noteworthy, this newly characterized prion strain has the ability to convert into the classical BSE strain upon serial transmission to inbred mouse lines.
Assessment of the Zoonotic Potential of Atypical Scrapie Prions in Humanized Mice Reveals Rare Phenotypic Convergence but Not Identity With Sporadic Creutzfeldt-Jakob Disease Prions
Alba Marín-Moreno , Fabienne Reine , Laetitia Herzog , Naima Aron , Florence Jaffrézic , Jean-Luc Vilotte , Human Rezaei , Olivier Andréoletti , Davy Martin , Vincent Béringue Author Notes
The Journal of Infectious Diseases, Volume 230, Issue 1, 15 July 2024, Pages 161–171, https://doi.org/10.1093/infdis/jiae093
Published: 24 February 2024
Conclusions The results obtained suggest a low zoonotic potential for AS. Rare adaptation may allow the emergence of prions phenotypically resembling those spontaneously forming in humans.
Scrapie Field Trial Experiments Mission, Texas, The Moore Air Force Base Scrapie TSE Prion Experiment 1964 How Did CWD Get Way Down In Medina County, Texas?
DISCUSSION
Observations of natural outbreaks of scrapie indicated that the disease spread from flock to flock by the movement of infected, but apparently normal, sheep which were incubating the disease.
There was no evidence that the disease spread to adjacent flocks in the absent of such movements or that vectors or other host species were involved in the spread of scrapie to sheep or goats; however, these possibilities should be kept open...
snip...PLEASE READ THE FULL REPORT ON THE SCRAPIE FIELD TRIAL EXPERIMENTS IN MISSION, TEXAS!!!
SCRAPIE TSE Prion USA RAPID RESPONSE URGENT UPDATES DECEMBER 25, 2025 SCRAPIE TSE Prion USA RAPID RESPONSE URGENT UPDATES DECEMBER 25, 2025
Accomplishments
***> 1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure.
ARS Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies 2025
snip…
Accomplishments
***> 1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure. It has long been suggested that prion disease in deer (chronic wasting disease (CWD)) was caused by the prion agent from sheep. The prion disease that affects sheep, scrapie, has been recognized for hundreds of years. However, chronic wasting disease, a similar disease found in WTD, has only been recognized since the 1960s. ARS researchers in Ames, Iowa, showed that white-tailed deer sick with scrapie from sheep can infect other deer under conditions mimicking natural exposure. Furthermore, this work shows that CWD is difficult to differentiate from WTD infected with scrapie. WTD scrapie prions accumulate in the lymphoreticular system in a manner similar to CWD, meaning that environmental contamination may occur through feces, saliva, and other body fluids of scrapie affected WTD as has been shown for CWD. The presence of WTD infected with scrapie could confound mitigation efforts for chronic wasting disease. This information informs regulatory officials, the farmed cervid industry, and officials tasked with protecting animal health such as state Departments of Agriculture, Natural Resources, or Parks and Wildlife with regard to a disease similar to CWD but arising from sheep scrapie that could be present in WTD that have contact with scrapie affected sheep and/or goats.
snip…
Accomplishments
***> 1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure.
Title: Characterization of classical sheep scrapie in white-tailed deer after experimental oronasal exposure
Author item Greenlee, Justin item MOORE, SARAH - Orise Fellow item Cassmann, Eric item LAMBERT, ZOE - Orise Fellow item Kokemuller, Robyn item Smith, Jodi item Kunkle, Robert item KONG, QINGZHONG - Case Western Reserve University (CWRU) item WEST GREENLEE, HEATHER - Iowa State University
Submitted to: Journal of Infectious Diseases Publication Type: Peer Reviewed Journal Publication Acceptance Date: 11/4/2022 Publication Date: 11/8/2022
snip…
Technical Abstract: Scrapie is a prion disease of sheep and goats that is associated with widespread accumulation of abnormal prion protein (PrPSc) in the central nervous and lymphoid tissues. Chronic wasting disease (CWD) is the natural prion disease of cervid species and is similar to scrapie in sheep. The purpose of this study was to determine susceptibility of white-tailed deer (WTD) to the scrapie agent. We inoculated WTD (n=5) by a concurrent oral and intranasal exposure with the scrapie agent from sheep and (n=6) with the scrapie agent from goats. All deer exposed to the agent of scrapie from sheep 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 lesions, and widespread distribution of PrPSc in neural and lymphoid tissues. Western blots done on samples from the brainstem, cerebellum, and lymph nodes of scrapie-infected WTD have a molecular profile similar to CWD and distinct from western blots of samples from the cerebral cortex, retina, or the original sheep scrapie inoculum. WTD are susceptible to the agent of scrapie from sheep and differentiation from CWD may be difficult.
Our findings establish that white-tailed deer are susceptible to WTD scrapie and that the presence of WTD scrapie agent in the lymphoreticular system suggests the handling of suspected cases should be consistent with current CWD guidelines because environmental shedding may occur.
snip…
The potential for zoonoses of cervid-derived PrPSc is still not well understood (6,18,45–47); however, interspecies transmission can increase host range and zoonotic potential (48–50). Therefore, to protect herds and the food supply, suspected cases of WTD scrapie should be handled the same as cases of CWD.
see;
Bovine Spongiform Encephalopathy BSE
USA BSE Testing and Surveillance?
Bottom line, USA is testing so few cows for BSE (<25k tested annually), you don’t find much mad cow disease testing only less than 25k a year, the mad cows have to find you, and the did in 2023, South Carolina BSE
BUT, even at those low testing figures, the USA did just confirm another case of BSE just here recently. Feed ban has failed terribly, and CWD is spreading in the USA, at an alarming rate. Recent transmission studies show oral transmission of CWD of Cervid to cattle. Studies also show links of sporadic CJD to BSE, Scrapie, and CWD. It’s a Whole new game of Prion poker now$$$
The U.S. has reported an atypical case of Bovine Spongiform Encephalopathy (BSE) in a beef cow approximately five years old or older at a slaughter plant in South Carolina. The animal never entered slaughter channels and at no time presented a risk to the food supply or to human health in the U.S. The radio frequency identification tag present on the animal is associated with a herd in Tennessee. APHIS and veterinary officials in South Carolina and Tennessee are gathering more information during this ongoing investigation.
This is the nation’s 7th detection of BSE, of the six previous U.S. cases, the first, in 2003 was the only case of classical BSE, which was from a cow imported from Canada. The rest of the cases have been atypical BSE. The animal was tested as part of the USDA’s Animal and Plant Health Inspection Service’s routine surveillance of cattle that are deemed unsuitable for slaughter.
National Cattlemen’s Beef Association chief veterinarian Dr. Kathy Simmons says USDA’s ongoing BSE surveillance program has tested more than one million cattle since the program began, ensuring that the agency’s interlocking supply chain safety products are working. She says the incidence of BSE in the U.S. is extremely low and will remain so.
U.S. Cattlemen’s Association president Justin Tupper says the swift detection of this case proves that the systems and protocols put in place are working. He says the organization is grateful to the nationwide team of veterinarians, animal health officials, meat inspectors, and others who ensure the well-being of the U.S. cattle herd.
Atypical BSE generally occurs in older cattle and seems to arise rarely and spontaneously in all cattle populations.
The World Organization for Animal Health (WOAH) recognizes the U.S. as negligible risk for BSE, the lowest possible risk in the world. Per WOAH guidelines in determining this status, atypical BSE cases do not impact official BSE risk status and this finding of an atypical case will not change the negligible risk status of the U.S., and should not lead to any trade issues.
Tags: Animal and Plant Health Inspection Service, animal health, bovine spongiform encephalopathy, BSE, cattle beef, US Cattlemen's Association, USCA, USDA
Wednesday, May 24, 2023
***> WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification
SATURDAY, MAY 20, 2023
***> Tennessee State Veterinarian Alerts Cattle Owners to Disease Detection Mad Cow atypical L-Type BSE
MAY 19, 2023
2 weeks before the announcement of this recent mad cow case in the USA, i submitted this to the APHIS et al;
***> APPRX. 2 weeks before the recent mad cow case was confirmed in the USA, in Tennessee, atypical L-Type BSE, I submitted this to the APHIS et al;
Document APHIS-2023-0027-0001 BSE Singeltary Comment Submission May 2, 2023
''said 'burden' cost, will be a heavy burden to bear, if we fail with Bovine Spongiform Encephalopathy BSE TSE Prion disease, that is why this information collection is so critical''...
Wednesday, May 24, 2023
WAHIS, WOAH, OIE, United States of America Bovine spongiform encephalopathy Immediate notification
Given that cattle have been successfully infected by the oral route, at least for L-BSE, it is reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle are exposed to contaminated feed. In addition, based on reports of atypical BSE from several countries that have not had C-BSE, it appears likely that atypical BSE would arise as a spontaneous disease in any country, albeit at a very low incidence in old cattle. In the presence of livestock industry practices that would allow it to be recycled in the cattle feed chain, it is likely that some level of exposure and transmission may occur. As a result, since atypical BSE can be reasonably considered to pose a potential background level of risk for any country with cattle, the recycling of both classical and atypical strains in the cattle and broader ruminant populations should be avoided.
Annex 7 (contd) AHG on BSE risk assessment and surveillance/March 2019
34 Scientific Commission/September 2019
3. Atypical BSE
The Group discussed and endorsed with minor revisions an overview of relevant literature on the risk of atypical BSE being recycled in a cattle population and its zoonotic potential that had been prepared ahead of the meeting by one expert from the Group. This overview is provided as Appendix IV and its main conclusions are outlined below. With regard to the risk of recycling of atypical BSE, recently published research confirmed that the L-type BSE prion (a type of atypical BSE prion) may be orally transmitted to calves1 . In light of this evidence, and the likelihood that atypical BSE could arise as a spontaneous disease in any country, albeit at a very low incidence, the Group was of the opinion that it would be reasonable to conclude that atypical BSE is potentially capable of being recycled in a cattle population if cattle were to be exposed to contaminated feed. Therefore, the recycling of atypical strains in cattle and broader ruminant populations should be avoided.
4. Definitions of meat-and-bone meal (MBM) and greaves
Consumption of L-BSE–contaminated feed may pose a risk for oral transmission of the disease agent to cattle.
Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.
''H-TYPE BSE AGENT IS TRANSMISSIBLE BY THE ORONASAL ROUTE''
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
***> atypical BSE and atypical Scrapie <***
Atypical BSE in cattle
THE recent diagnosis of two atypical bovine spongiform encephalopathy (BSE) cases in Great Britain (March 2023 in Cornwall and December 2024 in Dumfries and Galloway) and one in the Republic of Ireland (in November 2023) warrants a reminder about this notifiable disease.
Since 2005, a total of 17 cases have been detected in Great Britain.1 Unlike classical BSE, which resulted in over 180,000 cases in Great Britain and was predominantly associated with the consumption of feed contaminated with the BSE agent, and where the last case was confirmed in Ayrshire in May 2024, atypical BSE is believed to be a spontaneous disease in cattle found in approximately one in 1,000,000 tested cattle based on French data,2 similar to the sporadic Creutzfeldt- Jakob disease in people. There is currently no evidence that atypical BSE causes a disease in people, although it can be transmitted experimentally to other species by intracerebral inoculation, including primates.3–5 The World Organisation for Animal Health does not include atypical BSE in its geographical BSE risk status assessment.
Despite differences in terms of epidemiological, molecular and biological phenotype compared with classical BSE, atypical BSE is currently treated as if it were classical BSE in accordance with EU and UK legislation: once a case is identified, all cohort animals born and reared with the affected animal during the first 12 months of its life, and all offspring born within 24 months of its clinical onset, are culled and tested for BSE, which does seem to be at odds with the hypothesis that it is a spontaneous disease. This is more a precautionary measure to maintain confidence in the beef trade and protect consumers while more knowledge about this disease is obtained.
Almost all current knowledge on atypical BSE is based on experimental infection because this spontaneous
VET RECORD | 29 March–12 April 2025
disease has generally only been found in aged downer cows, which is difficult to replicate experimentally in the host species. Intracerebral inoculation of brain tissue from an affected cow causes disease in cattle in less than two years, unlike the natural disease that usually occurs in animals over eight years of age.
The vast majority of cases have been identified by active monitoring of fallen stock or emergency slaughter of cattle, where only the brain sample of various stages of autolysis is generally available. Little is known of where the atypical BSE agent can be found in natural disease, other than in the brain, because all the cases confirmed have been identified after death through active surveillance, by which time most peripheral tissue has been disposed of. Limited material from a single case of a naturally affected cow was tested in Italy by mouse bioassay, which found infectivity in muscle.6 In experimental disease generated by intracerebral inoculation of cattle, infectivity can be detected in the brain and spinal cord, ganglia, peripheral nerves and skeletal muscles, similar to classical BSE, but not in peripheral lymphoid tissue.6–8
Early reporting of clinical suspects is needed so that the live animal or the whole carcase can be delivered to an APHA regional laboratory for tissue sampling. This is made more difficult due to the subtlety of clinical signs based on experimental disease. Clinical cases may not be as over- reactive or nervous as classical BSE cases; some may, in fact, be dull, but what most cases have in common is that they have difficulty getting up and eventually end up as downer cows, and only the clinical history may reveal some prior behavioural or locomotor changes. High creatinine kinase serum levels and nibbling in response to scratching the tail head or back were some features in experimental disease,8, 9 but it is not known whether this is also seen in natural disease.
In general, BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment, where the blood results do not support the presence of a metabolic disease and where the cause cannot be determined with confidence.
Since BSE is a notifiable disease, suspected cases of BSE in Great Britain must be reported to the local APHA office.
Changes are imminent in the reporting of fallen stock cattle, which will require the owner to state whether the animal displayed signs of changes in behaviour, sensation or locomotion before death, in addition to the likely cause of death or disease. This is to obtain a better profile of the clinical history, if cattle are retrospectively diagnosed as BSE cases, which has happened in all BSE cases confirmed since 2010: none has been reported as a clinical suspect.
“BSE should be considered as a differential diagnosis in all downer cows that do not respond to treatment”
Timm Konold, TSE lead scientist
Brenda Rajanayagam, workgroup leader for the data systems group
APHA Weybridge, New Haw, Addlestone, Surrey KT15 3NB email: timm.konold@apha.gov.uk
Keith Meldrum, former chief veterinary officer The Orchard, Swaynes Lane, Guildford, Surrey GU1 2XX
References
1 APHA. Cattle: TSE surveillance statistics. Overview of Great Britain statistics. 2025. https://bit.ly/4ho5Nds (accessed 19 March 2025)
Atypical BSE In Cattle
see latest on BSE TSE Prion EU;
Title: Transmission of atypical BSE: a possible origin of Classical BSE in cattle
Authors: Sandor Dudas1, Samuel James Sharpe1, Kristina Santiago-Mateo1, Stefanie Czub1, Waqas Tahir1,2, *
Affiliation: 1National and WOAH reference Laboratory for Bovine Spongiform Encephalopathy, Canadian Food inspection Agency, Lethbridge Laboratory, Lethbridge, Canada. 2Department of Biological Sciences, University of Lethbridge, Lethbridge, Alberta, Canada.
*Corresponding and Presenting Author: waqas.tahir@inspection.gc.ca
Background: Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease of cattle and is categorized into classical and atypical forms. Classical BSE (CBSE) is linked to the consumption of BSE contaminated feed whereas atypical BSE is considered to be spontaneous in origin. The potential for oral transmission of atypical BSE is yet to be clearly defined.
Aims: To assess the oral transmissibility of atypical BSE (H and L type) in cattle. Should transmission be successful, determine the biochemical characteristics and distribution of PrPSc in the challenge cattle.
Material and Methods: For oral transmission, calves were fed with 100 g of either H (n=3) or L BSE (n=3) positive brain material. Two years post challenge, 1 calf from each of the H and L BSE challenge groups exhibited behavioural signs and were euthanized. Various brain regions of both animals were tested by traditional and novel prion detection methods with inconclusive results. To detect infectivity, brain homogenates from these oral challenge animals (P1) were injected intra-cranially (IC) into steer calves. Upon clinical signs of BSE, 3/4 of IC challenged steer calves were euthanized and tested for PrPSc with ELISA, immunohistochemistry and immunoblot.
Results: After 6 years of incubation, 3/4 animals (2/2 steers IC challenged with brain from P1 L-BSE oral challenge and 1/2 steer IC challenged with brain from P1 H-BSE oral challenge) developed clinical disease. Analysis of these animals revealed high levels of PrPSc in their brains, having biochemical properties similar to that of PrPSc in C-BSE.
Conclusion: These results demonstrate the oral transmission potential of atypical BSE in cattle. Surprisingly, regardless of which atypical type of BSE was used for P1 oral challenge, PrPSc in the P2 animals acquired biochemical characteristics similar to that of PrPSc in C-BSE, suggesting atypical BSE as a possible origin of C-BSE in UK.
Presentation Type: Oral Presentation
Funded by: CFIA, Health Canada, Alberta Livestock and Meat Agency, Alberta Prion Research Institute
Grant Number: ALMA/APRI: 201400006, HC 414250
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE)
Component 6: Transmissible Spongiform Encephalopathies (TSEs)
Problem Statement 6A: Determine pathobiology of prion strains.
The naturally occurring lysine to glutamic acid substitution (E211K in the bovine prion protein) results in short incubation periods for H-type bovine spongioform encephalopathy (BSE).
Virus and Prion Research Unit, National Animal Disease Center, Ames, Iowa
Classical BSE (C-BSE) is a prion disease of cattle that was responsible for the "mad cow disease" epizootic in Europe in the 1980s. C-BSE was determined to cause the human prion disease vCJD. Since then, atypical spontaneous strains of BSE were identified. H-BSE is one of those strains. Much research has explored the origins of C-BSE, and strain emergence from atypical H-BSE is one hypothesis. An H-BSE case was determined to have a germline mutation, an E211K substitution in the prion protein gene, which is analogous to a hereditary human prion disease. ARS scientists in Ames, Iowa reported the transmission of H-BSE from cattle, with and without the germline prion protein amino acid substitution, to cattle with various prion genotypes: EE211 (wild-type), EK211, and KK211. Results indicated a significantly shorter incubation period in K containing cattle compared to prion wild-type cattle. The scientists also explored the possibility that the C-BSE strain might have occurred after serial passages of EK211 and KK211 containing H-BSE in cattle, but results did not support this concept. This information is important to prion researchers, veterinary diagnostic laboratories, and those involved with establishing regulatory guidelines.
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research
Title: Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation
Author item Greenlee, Justin item Cassmann, Eric item MOORE, SARA JO - Oak Ridge Institute For Science And Education (ORISE) item WEST GREENLEE, HEATHER - Iowa State University
Submitted to: Meeting Abstract Publication Type: Abstract Only Publication Acceptance Date: 6/24/2022 Publication Date: 9/16/2022 Citation: Greenlee, J.J., Cassmann, E.D., Moore, S., West Greenlee, H.M. 2022.
Cattle with the EK211 PRNP polymorphism are susceptible to the H-type bovine spongiform encephalopathy agent from either E211K or wild type donors after oronasal inoculation.
Prion 2022 Conference abstracts: pushing the boundaries. 16(1):150. https://doi.org/10.1080/19336896.2022.2091286.
DOI: https://doi.org/10.1080/19336896.2022.2091286
Interpretive Summary:
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (H-BSE) was reported in a cow with a previously unreported prion protein polymorphism (E211K). The E211K polymorphism is heritable and homologous to the E200K mutation in humans that is the most frequent PRNP mutation associated with familial Creutzfeldt-Jakob disease. Although the prevalence of the E211K polymorphism is low, cattle carrying the K211 allele develop H-type BSE with a rapid onset after experimental inoculation by the intracranial route. The purpose of this study was to investigate whether the agents of H-type BSE or H-type BSE associated with the E211K polymorphism transmit to wild type cattle or cattle with the K211 allele after oronasal exposure. Wild type (EE211) or heterozygous (EK211) cattle were oronasally inoculated with the H-BSE agent from either the US 2004 case (wild type donor; n=3) or from the US 2006 case with the E211K polymorphism (n=4). Cattle were observed daily throughout the course of the experiment for the development of clinical signs. When signs were noted, animals were euthanized and necropsied. Cattle were confirmed positive for abnormal BSE prions by enzyme immunoassay (EIA; Idexx HerdChek BSE Ag Test), anti-PrP immunohistochemistry (IHC) on brainstem, and microscopic examination for vacuolation. Three-out-of-four (75%) calves with the EK211 genotype developed clinical signs of H-BSE including inattentiveness, loss of body condition, weakness, ataxia, and muscle fasciculations and were euthanized. Two of the positive EK211 steers received H-BSE US 2004 inoculum (Incubation Period (IP): 59.3 and 72.3 months) while the other positive steer received the E211K H-BSE inoculum (IP: 49.7 months). EIA confirmed that abundant misfolded protein (O.D. 2.57-4.0) in the brainstem, and IHC demonstrated PrPSc throughout the brain. All cattle in the EE211 recipient group remain asymptomatic for the duration of the experiment (approximately 7 years post-inoculation).
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
Highlights
This study demonstrates that the H-type BSE agent is transmissible by the oronasal route. Cattle with the EK211 genotype are oronasally susceptible to small doses of the H-BSE agent from either EK211 or EE211 (wild type) donors. Wild-type EE211 cattle remained asymptomatic for the duration of the experiment with this small dose (0.1g) of inoculum. These results reinforce the need for ongoing surveillance for classical and atypical BSE to minimize the risk of potentially infectious tissues entering the animal or human food chains.
Title: A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation
Author item MOORE, S - Orise Fellow item WEST GREENLEE, M - Iowa State University item Smith, Jodi item Vrentas, Catherine item Nicholson, Eric item Greenlee, Justin
Submitted to: Frontiers in Veterinary Science Publication Type: Peer Reviewed Journal Publication Acceptance Date: 8/30/2016 Publication Date: 9/15/2016 Citation: Moore, S.J., West Greenlee, M.H., Smith, J.D., Vrentas, C.E., Nicholson, E.M., Greenlee, J.J. 2016.
A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211cattle following intracranial inoculation.
Frontiers in Veterinary Science. 3:78. Interpretive
Summary: Cases of bovine spongiform encephalopathy (BSE) or mad cow disease can be subclassified into at least 3 distinct disease forms with the predominate form known as classical BSE and the others collectively referred to as atypical BSE. Atypical BSE can be further subdivided into H-type and L-type cases that are distinct from classical BSE and from each other. Both of the atypical BSE subtypes are believed to occur spontaneously, whereas classical BSE is spread through feeding contaminated meat and bone meal to cattle. Work by other research groups suggests that the stability of the distinguishing features of atypical BSE cases (phenotypical stability) can change to closely resemble classical BSE after experimental passage implicating atypical BSE as a possible origin of classical BSE. Interestingly, one case of H-type BSE in the US was associated with an inherited mutation in the prion protein gene referred to as E211K. The purpose of this work was to compare wild type and cattle with the E211K mutation after experimental inoculation with either classical BSE or H-BSE from the original E211K case. This study demonstrates that the disease features of E211K BSE-H remain stable when transmitted to cattle without the K211 polymorphism. In addition, passage of classical BSE to cattle with the K211 polymorphism results in disease with features consistent with classical BSE and not a switch to atypical BSE-H as a result of the K211 polymorphism. As the origin of classical, feedborne BSE remains unknown and low numbers of atypical BSE are diagnosed each year, parties with interest in the cattle and beef industries and regulatory officials responsible for safe feeding practices of cattle will be interested in this work.
Technical Abstract: In 2006, a case of H-type bovine spongiform encephalopathy (BSE-H) was diagnosed in a cow that was associated with a heritable polymorphism in the bovine prion protein gene (PRNP) resulting in a lysine for glutamine amino acid substitution at codon 211 (called E211K) of the prion protein. Although the prevalence of this polymorphism is low, cattle carrying the K211 allele may be predisposed to rapid onset of BSE-H when exposed or to the potential development of a genetic BSE. This study was conducted to better understand the relationship between the K211 polymorphism and its effect on BSE phenotype. BSE-H from the US 2006 case was inoculated intracranially (IC) in one PRNP wild type (EE211) calf and one EK211 calf. In addition, one wild type calf and one EK211 calf were inoculated IC with brain homogenate from a US 2003 classical BSE case. All cattle developed clinical disease. The survival times of the E211K BSE-H inoculated EK211 calf (10 months) was shorter than the wild type calf (18 months). This genotype effect was not observed in classical BSE inoculated cattle (both 26 months). Significant changes in retinal function were observed in H-type BSE challenged cattle only. Cattle challenged with the same inoculum showed similar severity and neuroanatomical distribution of vacuolation and disease-associated prion protein deposition in the brain, though differences in neuropathology were observed between E211K BSE-H and classical BSE inoculated animals. Western blot results for brain tissue from challenged animals were consistent with the inoculum strains. This study demonstrates that the phenotype of E211K BSE-H remains stable when transmitted to cattle without the K211 polymorphism, and exhibits a number of features that differ from classical BSE in both wild type and heterozygous EK211 animals.
THURSDAY, JUNE 5, 2025
World Organisation for Animal Health (WOAH) downgrades UK’s BSE risk rating to negligible, what could go wrong?
Why did USDA decrease the number of samples per year in 2006?
After the first confirmation of BSE in an animal in Washington State in December 2003, USDA evaluated its BSE surveillance efforts in light of that finding. We determined that we needed to immediately conduct a major surveillance effort to help determine the prevalence of BSE in the United States. Our goal over a 12-18 month period was to obtain as many samples as possible from the segments of the cattle population where we were most likely to find BSE if it was present. This population of cattle was exhibiting some signs of disease. We conducted this enhanced surveillance effort from June 2004 - August 2006. In that time, we collected a total of 787,711 samples and estimated the prevalence of BSE in the United States to be between 4-7 infected animals in a population of 42 million adult cattle. We consequently modified our surveillance efforts based on this prevalence estimate to a level we can monitor for any potential changes, should they occur. Our statistical analysis indicated that collecting approximately 40,000 samples per year from the targeted cattle population would enable us to conduct this monitoring.
THE reason to reduce BSE testing to <25k annually was painfully obvious, more atypical BSE cases started showing up, so testing was reduced to testing numbers where it is less likely a case is detected here in the US, imho…terry
BSE surveillance began in 1990. USDA has an ongoing, comprehensive, interagency surveillance program to detect signs of BSE in the United States. USDA's BSE surveillance program samples approximately 40,000 animals each year and targets cattle populations where the disease is most likely to be found.
The targeted population for ongoing surveillance focuses on cattle exhibiting signs of central nervous disorders or any other signs that may be associated with BSE, including emaciation or injury, and dead cattle, as well as non-ambulatory animals. Samples from the targeted population are taken at farms, veterinary diagnostic laboratories, public health laboratories, slaughter facilities, veterinary clinics, and livestock markets. In addition, approximately 5,000 samples each year are collected from renderers and similar salvage facilities.
USDA will help defray costs incurred for transportation, disposal, storage and testing. For more information on the USDA-APHIS BSE Ongoing Surveillance Plan, visit their website.
Bovine Spongiform Encephalopathy (BSE): Ongoing Surveillance Program
Current Monthly Test Results
APHIS reports ongoing surveillance test totals monthly.
Beginning in Fiscal Year 2016, the BSE ongoing surveillance program will sample approximately 25,000 animals each year. Under the program, USDA will continue to collect samples from a variety of sites and from the cattle populations where the disease is most likely to be detected
| Month | Number of Tests |
|---|---|
| October 2016 | 985 |
| November 2016 | 2,314 |
| December 2016 | 1,975 |
| January 2017 | 1,387 |
Fiscal Year 2007-2016 Test Results
For Fiscal Years 2007-2015, the BSE ongoing surveillance program had a target of testing approximately 40,000 animals per year.
OIE Surveillance Points: design prevalence of 1 case per 100,000 U.S. adult cattle and 95% confidence; 300,000 OIE points over 7 years, or 42,857 OIE points on average per year are required.
BSurvE Surveillance Points: design prevalence of 1 case per 1,000,000 U.S. adult cattle and 95% confidence; 3,000,000 points over 7 years, or 428,571 BSurvE points on average per year are required.
Samples Tested, OIE Points, and BSurvE Points by Fiscal Year
Fiscal Year | Number of Valid Tests | OIE Points | BSurvE Points |
|---|---|---|---|
| FY 2016 | 26,564 | 547,959 | 1,314,579 |
FY 2015 | 40,902 | 592,353 | 1,414,630 |
FY 2014 | 41,291 | 678,622 | 1,642,452 |
FY 2013 | 43,173 | 1,048,554 | 2,505,042 |
FY 2012 | 42,202 | 1,066,637 | 2,523,447 |
FY 2011 | 40,482 | 997,360 | 2,344,436 |
FY 2010 | 44,301 | 948,593 | 2,233,643 |
FY 2009 | 44,217 | 1,036,849 | 2,442,400 |
FY 2008 | 43,145 | 1,121,624 | 2,677,665 |
FY 2007* | 43,336 | 1,487,215 | 3,621,117 |
* FY2007 - first complete year of BSE Ongoing Surveillance | |||
BSE Ongoing Surveillance Program Cumulative Totals
- Valid Tests From September 1, 2006 to February 2, 2017: 416,274
- OIE Points From September 1, 2006 to February 2, 2017: 9,726,651
- BSurvE Points From September 1, 2006 to February 2, 2017: 23,204,789
(see "BSE Ongoing Surveillance Plan" for information on OIE and BSurvE points)
2006 Ongoing BSE Surveillance Plan
Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program
An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007.
snip...
4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half
http://www.usda.gov/oig/webdocs/sarc070619.pdf
NEW URL LINK;
Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program – Phase II and Food Safety and Inspection Service Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III
UNITED STATES DEPARTMENT OF AGRICULTURE OFFICE OF INSPECTOR GENERAL Washington, D.C. 20250 January 25, 2006 REPLY TO ATTN OF: 50601-10-KC
TO: W. Ron DeHaven Administrator Animal and Plant Health Inspection Service Barbara Masters Administrator Food Safety and Inspection Service ATTN: William J. Hudnall Deputy Administrator Marketing Regulatory Program Business Services William C. Smith Assistant Administrator Office of Program Evaluation, Enforcement, and Review
FROM: Robert W. Young /s/ Assistant Inspector General for Audit
SUBJECT: Animal and Plant Health Inspection Service - Bovine Spongiform Encephalopathy (BSE) Surveillance Program - Phase II and Food Safety and Inspection Service - Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III
This report presents the results of our audit of the enhanced BSE surveillance program and controls over specified risk materials and advanced meat recovery products. Your written response to the official draft report, dated January 20, 2006, is included as exhibit G with excerpts of the response and the Office of Inspector General’s (OIG) position incorporated into the Findings and Recommendations section of the report, where applicable. We accept the management decisions for all recommendations. Please follow your agency’s internal procedures in forwarding documentation for final action to the Office of the Chief Financial Officer (OCFO). We are providing a separate memorandum to the agencies and OCFO that provides specific information on the actions to be completed to achieve final action. We appreciate your timely response and the cooperation and assistance provided to our staff during the audit USDA/OIG-A/50601-10-KC/ Page i
Executive Summary
Animal and Plant Health Inspection Service - Bovine Spongiform Encephalopathy (BSE) Surveillance Program - Phase II and Food Safety and Inspection Service - Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III
Results in Brief This report evaluates elements of the interlocking safeguards in place to protect United States (U.S.) beef from Bovine Spongiform Encephalopathy, widely known as BSE or "mad cow disease." Since 1990, the U.S. Department of Agriculture (USDA), Animal and Plant Health Inspection Service (APHIS), has led a multi-agency effort to monitor and prevent BSE from entering the food supply. After discovering a BSE-positive cow in December 2003, APHIS expanded its BSE surveillance program. To further protect the food supply, USDA banned materials identified as being at risk of carrying BSE (specified risk materials (SRM)), such as central nervous system tissue. As part of this effort, USDA’s Food Safety and Inspection Service (FSIS) required beef slaughter and processing facilities to incorporate controls for handling such materials into their operational plans. Onsite FSIS inspectors also inspect cattle for clinical signs in order to prevent diseased animals from being slaughtered for human consumption. To evaluate the effectiveness of the safeguards, we assessed APHIS’ implementation of the expanded surveillance program, as well as FSIS’ controls to prevent banned SRMs from entering the food supply.
In June 2004, APHIS implemented its expanded surveillance program; participation by industry in this surveillance program is voluntary. As of May 2005, over 350,000 animals were sampled and tested for BSE. To date, two animals tested positive for BSE; one tested positive after implementation of the expanded surveillance program.
USDA made significant efforts to implement the expanded BSE surveillance program. Much needed to be done in a short period of time to establish the necessary processes, controls, infrastructure, and networks to assist in this effort. In addition, extensive outreach and coordination was undertaken with other Federal, State, and local entities, private industry, and laboratory and veterinary networks. This report provides an assessment as to the progress USDA made in expanding its surveillance effort and the effectiveness of its controls and processes. This report also discusses the limitations of its program and data in assessing the prevalence of BSE in the U.S. herd.
snip...
40 ELISA test procedures require two additional (duplicate) tests if the initial test is reactive, before final interpretation. If either of the duplicate tests is reactive, the test is deemed inconclusive.
41 Protocol for BSE Contract Laboratories to Receive and Test Bovine Brain Samples and Report Results for BSE Surveillance Standard Operating Procedure (SOP), dated October 26, 2004.
42 The NVSL conducted an ELISA test on the original material tested at the contract laboratory and on two new cuts from the sample tissue.
43 A visual examination of brain tissue by a microscope.
44 A localized pathological change in a bodily organ or tissue.
SNIP...
PLEASE SEE FLAMING EVIDENCE THAT THE USDA ET AL COVERED UP MAD COW DISEASE IN TEXAS ;
PAGE 43;
Section 2. Testing Protocols and Quality Assurance Controls
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FULL TEXT 130 PAGES
http://www.usda.gov/oig/webdocs/50601-10-KC.pdf
NEW URL LINK;
Merry Christmas, i remember that cow well, December 2003. MOM DOD 12/14/97
WHAT about those 9,200 suspect BSE secret test???
"These 9,200 cases were different because brain tissue samples were preserved with formalin, which makes them suitable for only one type of test--immunohistochemistry, or IHC."
THIS WAS DONE FOR A REASON!
THE IHC test has been proven to be the LEAST LIKELY to detect BSE/TSE in the bovine, and these were probably from the most high risk cattle pool, the ones the USDA et al, SHOULD have been testing. ...TSS
SCIENCE NEWS JULY 13, 2004 / 5:02 PM
USDA advised against mad cow test in 2002
By STEVE MITCHELL, United Press International
"Why are we using Bio-Rad instead of Prionics if they are as bad as the (USDA) would have us believe with all these 'inconclusives?' asked Terry Singletary, coordinator of CJD Watch, an advocacy group for patients and family members. His mother died of a rare form of CJD called Heidenhain Variant, which has not been linked with mad cow disease.
SCIENCE NEWS MAY 11, 2004 / 10:15 PM
USDA orders silence on mad cow in Texas
By STEVE MITCHELL, United Press International
Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program – Phase II
and
Food Safety and Inspection Service Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III
Consumer Health
Inspector to file charges against USDA
By Steve Mitchell Sep 6, 2005, 22:46 GMT
The Mad Cow That Stole Christmas, 20 Years Later, What Has Changed, Nothing
THE USA has systematically covered up mad cow disease, in my honest opinion, the USA mad cow disease today, is Chronic Wasting Disease CWD TSE Prion disease in Cervid, they can't cover that up.
Chronic Wasting Disease CWD TSE Prion Transmission To Livestock By Oral Route
***> CWD to Cattle by oral route Transmission of the chronic wasting disease agent from elk to cattle after oronasal exposure
Justin Greenlee, Jifeng Bian, Zoe Lambert, Alexis Frese, and Eric Cassmann Virus and Prion Research Unit, National Animal Disease Center, USDA-ARS, Ames, IA, USA
Aims: The purpose of this study was to determine the susceptibility of cattle to chronic wasting disease agent from elk.
Materials and Methods: Initial studies were conducted in bovinized mice using inoculum derived from elk with various genotypes at codon 132 (MM, LM, LL). Based upon attack rates, inoculum (10% w/v brain homogenate) from an LM132 elk was selected for transmission studies in cattle. At approximately 2 weeks of age, one wild type steer (EE211) and one steer with the E211K polymorphism (EK211) were fed 1 mL of brain homogenate in a quart of milk replacer while another 1 mL was instilled intranasally. The cattle were examined daily for clinical signs for the duration of the experiment. One steer is still under observation at 71 months post-inoculation (mpi).
Results: Inoculum derived from MM132 elk resulted in similar attack rates and incubation periods in mice expressing wild type or K211 bovine PRNP, 35% at 531 days post inoculation (dpi) and 27% at 448 dpi, respectively. Inoculum from LM132 elk had a slightly higher attack rates in mice: 45% (693 dpi) in wild type cattle PRNP and 33% (468) in K211 mice. Inoculum from LL132 elk resulted in the highest attack rate in wild type bovinized mice (53% at 625 dpi), but no K211 mice were affected at >700 days. At approximately 70 mpi, the EK211 genotype steer developed clinical signs suggestive of prion disease, depression, low head carriage, hypersalivation, and ataxia, and was necropsied. Enzyme immunoassay (IDEXX) was positive in brainstem (OD=4.00, but non-detect in retropharyngeal lymph nodes and palatine tonsil. Immunoreactivity was largely limited to the brainstem, midbrain, and cervical spinal cord with a pattern that was primarily glia-associated.
“Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.”
Strain characterization of chronic wasting disease in bovine-PrP transgenic mice
Conclusions: Altogether, these results exhibit the diversity of CWD strains present in the panel of CWD isolates and the ability of at least some CWD isolates to infect bovine species. Cattle being one of the most important farming species, this ability represents a potential threat to both animal and human health, and consequently deserves further study.
Prion 2023 Congress Organizing Committee and the NeuroPrion Association, we invite you to join us for the International Conference Prion2023 from 16-20 October 2023 in Faro, Portugal.
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
“Conclusions: Cattle with the E211K polymorphism are susceptible to the CWD agent after oronasal exposure of 0.2 g of infectious material.”
***> CWD Transmits to Pigs By Oral Routes
Outbreak of cwd in pigs in Texas and Arkansas;
Chronic wasting disease prions in cervids and wild pigs in North America Preliminary Outbreak Assessment DEFRA 26 January 2026 Department for Environment, Food and Rural Affairs
Preliminary Outbreak Assessment
Chronic wasting disease prions in cervids and wild pigs in North America
26 January 2026
Disease report
“They analysed over 300 brain and lymph node samples from 178 wild pigs living across Arkansas and Texas, USA…the researchers identified CWD prions in up to 37% of the lymph node samples and 15% of brain samples. The lowest detection rates were in the Texas samples (below 16%), matching the lower CWD prevalence in the state’s cervid population.”
***> white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure
Research Project: Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies
Location: Virus and Prion Research
Title: Differentiation of scrapie from chronic wasting disease in white-tailed deer
Accomplishments
1. 01 Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure. It has long been suggested that prion disease in deer (chronic wasting disease (CWD)) was caused by the prion agent from sheep. The prion disease that affects sheep, scrapie, has been recognized for hundreds of years. However, chronic wasting disease, a similar disease found in WTD, has only been recognized since the 1960s. ARS researchers in Ames, Iowa, showed that white-tailed deer sick with scrapie from sheep can infect other deer under conditions mimicking natural exposure. Furthermore, this work shows that CWD is difficult to differentiate from WTD infected with scrapie. WTD scrapie prions accumulate in the lymphoreticular system in a manner similar to CWD, meaning that environmental contamination may occur through feces, saliva, and other body fluids of scrapie affected WTD as has been shown for CWD. The presence of WTD infected with scrapie could confound mitigation efforts for chronic wasting disease. This information informs regulatory officials, the farmed cervid industry, and officials tasked with protecting animal health such as state Departments of Agriculture, Natural Resources, or Parks and Wildlife with regard to a disease similar to CWD but arising from sheep scrapie that could be present in WTD that have contact with scrapie affected sheep and/or goats.
***> “Determined that white-tailed deer (WTD) infected with scrapie from sheep can transmit the disease to other deer under conditions mimicking natural exposure. It has long been suggested that prion disease in deer (chronic wasting disease (CWD)) was caused by the prion agent from sheep.”
Volume 27, Number 12—December 2021 Dispatch Experimental Oronasal Transmission of Chronic Wasting Disease Agent from White-Tailed Deer to Suffolk Sheep
2017 Annual Report
Scrapie to Pigs
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…
***> CWD Transmits To Pigs
Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, 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.
CWD to Pigs By Oral Route
Detection of infectivity in orally inoculated pigs using mouse bioassay raises the possibility that naturally exposed pigs could act as a reservoir of CWD infectivity. Currently, swine rations in the U.S. could contain animal derived components including materials from deer or elk. In addition, feral swine could be exposed to infected carcasses in areas where CWD is present in wildlife populations. The current feed ban in the U.S. is based exclusively on keeping tissues from TSE infected cattle from entering animal feeds. These results indicating the susceptibility of pigs to CWD, 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.
https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017
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.
In the US, feeding of ruminant by-products to ruminants is prohibited, but feeding of ruminant materials to swine, mink, and poultry still occurs. In addition, scavenging of CWD-affected cervid carcasses by feral pigs presents a potential risk for CWD exposure.
snip…
This study demonstrates that pigs can serve as potential hosts for CWD, although with low attack rates and scant PrPcwd accumulation. Detection of infectivity in orally challenged pigs using mouse bioassay raises the possibility that naturally exposed pigs act as a reservoir of CWD infectivity, even though affected pigs do not develop overt clinical signs or readily detectable PrPcwd.
***> CWD to deer by oral route, minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent)
Very low oral exposure to prions of brain or saliva origin can transmit chronic wasting disease
Nathaniel D. Denkers , Clare E. Hoover , Kristen A. Davenport, Davin M. Henderson, Erin E. McNulty, Amy V. Nalls, Candace K. Mathiason, Edward A. Hoover
Published: August 20, 2020
Abstract
The minimum infectious dose required to induce CWD infection in cervids remains unknown, as does whether peripherally shed prions and/or multiple low dose exposures are important factors in CWD transmission. With the goal of better understand CWD infection in nature, we studied oral exposures of deer to very low doses of CWD prions and also examined whether the frequency of exposure or prion source may influence infection and pathogenesis. We orally inoculated white-tailed deer with either single or multiple divided doses of prions of brain or saliva origin and monitored infection by serial longitudinal tissue biopsies spanning over two years. We report that oral exposure to as little as 300 nanograms (ng) of CWD-positive brain or to saliva containing seeding activity equivalent to 300 ng of CWD-positive brain, were sufficient to transmit CWD disease. This was true whether the inoculum was administered as a single bolus or divided as three weekly 100 ng exposures. However, when the 300 ng total dose was apportioned as 10, 30 ng doses delivered over 12 weeks, no infection occurred. While low-dose exposures to prions of brain or saliva origin prolonged the time from inoculation to first detection of infection, once infection was established, we observed no differences in disease pathogenesis. These studies suggest that the CWD minimum infectious dose approximates 100 to 300 ng CWD-positive brain (or saliva equivalent), and that CWD infection appears to conform more with a threshold than a cumulative dose dynamic.
“We report that oral exposure to as little as 300 nanograms (ng) of CWD-positive brain or to saliva containing seeding activity equivalent to 300 ng of CWD-positive brain, were sufficient to transmit CWD disease.”
***> USA Report, Scrapie, CWD, BSE, TSE, Cattle, Sheep, Pigs, Cervid, Humans, Zoonotic, 2026
April 2026
salt licks, soil, and CWD
https://www.vetinst.no/rapporter-og-publikasjoner/rapporter/2026/oppdatert-kunnskapsgrunnlag-om-skrantesjuke-cwd-i-norge/_/attachment/inline/9f324a71-ee7e-466f-bccd-7df1e51c73d8:eb1d2f7c783a814eb7cea26e695dec6a335c9907/2026%2058%20Oppdatert%20kunnskapsgrunnlag%20om%20skrantesjuke%20Innspill.pdf
REPORT 58/2026 Updated knowledge base on chronic wasting disease (CWD) in Norway – veterinary assessment
“Exposure to prions in the environment is considered the second most important source of CWD transmission. In as-yet unpublished studies, "seeding activity" was detected in soil samples from salt licks in Nordfjella. Most of these detections occurred in Zone 1, though one was also found at a salt lick in Zone 2. The number of detections decreased between 2017 and 2020, and no seeding activity was detected in samples from 2021 or 2024. Given that infected animals have been identified on Hardangervidda, it is reasonable to assume that similar localized environmental contamination may exist there as well, just as it did in Nordfjella.”
snip…
Permanent salt licks with soil runoff represent clear sources of infection and are likely significant for the spread of disease both within and between populations of cervids. The use of such salt licks should cease in the peripheral zones surrounding infected areas. They should be replaced by measures that do not encourage animals to congregate or consume soil.
snip…
6.3.1 Surveillance of the Filefjell, Fram, Vågå, and Lom reindeer management units
For the Filefjell, Vågå, Fram, and Lom reindeer management units, extensive sampling should be conducted to ensure the earliest possible detection of transmissible CWD, should the disease still be present in Nordfjella. This surveillance should be maintained even if these units have achieved a high level of assurance regarding the absence of CWD, as the primary purpose of the surveillance is early warning.
The surveillance should encompass both "risk animals" (animals at higher risk of carrying the disease) and slaughtered animals (older than one year). The approach may be evaluated annually, depending on the progress of reindeer re-establishment in Nordfjella Zone 1. Animals exhibiting abnormal behavior or clinical signs should be recorded, ensuring that data on the "risk animals" examined are available. This will strengthen the credibility of the documentation regarding CWD status, both nationally and internationally.
Consideration may be given to including surveillance for environmental contamination within these reindeer management units. This would be most relevant in Filefjell, specifically at salt licks or feeding sites that are not fenced off. Ideally, the establishment of such sites—where animals congregate—should be avoided. Reindeer enclosures—that is, gathering areas used for activities such as ear-tagging and slaughter—may also serve as appropriate locations for collecting environmental samples.
snip…
***> “ Surveillance for CWD should be maintained in Selbu. This is important for establishing a long-term data series in a geographical area where multiple cases of sporadic CWD have been detected. A long-term data series demonstrating that the prevalence of sporadic CWD is not increasing would help reinforce the understanding that sporadic CWD has a spontaneous etiology.”
***> remember that…terry
https://www.vetinst.no/rapporter-og-publikasjoner/rapporter/2026/oppdatert-kunnskapsgrunnlag-om-skrantesjuke-cwd-i-norge/
So, this is what we leave our children and grandchildren , and what about land values?
"Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation."
Detection of prions in soils contaminated by multiple routes
snip…
Detection of prions in soils contaminated by multiple routes
snip…
Results: We are able to detect prion seeding activity at multiple types of environmental hotspots, including carcass sites, contaminated captive facilities, and scrapes (i.e. urine and saliva). Differences in relative prion concentration vary depending on the nature and source of the contamination. Additionally, we have determined that prion seeding activity is retained for at least fifteen years at a contaminated site following attempted remediation.
Conclusions: Detection of prions in the environment is of the utmost importance for controlling chronic wasting disease spread. Here, we have demonstrated a viable method for detection of prions in complex environmental matrices. However, it is quite likely that this method underestimates the total infectious prion load in a contaminated sample, due to incomplete recovery of infectious prions. Further refinements are necessary for accurate quantification of prions in such samples, and to account for the intrinsic heterogeneities found in the broader environment.
=====end
Prion 2023 Abstracts
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
Conclusions: Detection of prions in the environment is of the utmost importance for controlling chronic wasting disease spread. Here, we have demonstrated a viable method for detection of prions in complex environmental matrices. However, it is quite likely that this method underestimates the total infectious prion load in a contaminated sample, due to incomplete recovery of infectious prions. Further refinements are necessary for accurate quantification of prions in such samples, and to account for the intrinsic heterogeneities found in the broader environment.
=====end
Prion 2023 Abstracts
https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://web.archive.org/web/20250828201533/https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf
https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
JOURNAL OF GENERAL VIROLOGY Volume 87, Issue 12
Infectious agent of sheep scrapie may persist in the environment for at least 16 years
***> 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.
https://www.microbiologyresearch.org/content/journal/jgv/10.1099/vir.0.82011-0
Rapid recontamination of a farm building occurs after attempted prion removal
First published: 19 January 2019 https://doi.org/10.1136/vr.105054
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 scrapie positive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.
https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054
***>This is very likely to have parallels with control efforts for CWD in cervids.
https://pubmed.ncbi.nlm.nih.gov/30602491/
https://www.researchgate.net/profile/Syed-Zahid-Shah/publication/378314391_Meeting-book-final-version_prion_2023/links/65d44dad28b7720cecdca95f/Meeting-book-final-version-prion-2023.pdf
JOURNAL OF GENERAL VIROLOGY Volume 87, Issue 12
Infectious agent of sheep scrapie may persist in the environment for at least 16 years
***> 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.
https://www.microbiologyresearch.org/content/journal/jgv/10.1099/vir.0.82011-0
Rapid recontamination of a farm building occurs after attempted prion removal
First published: 19 January 2019 https://doi.org/10.1136/vr.105054
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 scrapie positive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.
https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054
***>This is very likely to have parallels with control efforts for CWD in cervids.
https://pubmed.ncbi.nlm.nih.gov/30602491/
Tuesday, June 9, 2026
Hazard identification for the risks to terrestrial animal health from the import of hay and straw, BSE, CWD, Scrapie, TSE, Prion
OFFICIAL MINUTES OF THE 429th COMMISSION MEETING Texas Animal Health Commission August 25, 2026
There have been four more CWD positive herds that have been depopulated for a total of $827,000 in indemnity.
SATURDAY, SEPTEMBER 19, 2026
Texas 5th Captive Herd Depopulated This Year Due To Positive CWD TSE Prion
Texas CWD TSE Prion Cases Climb To 1,420 Detected To Date and TPWC Meeting November 2026 Singeltary Submission
TPW Commission Meeting Transcript CWD TSE PrP January 22, 2026 TEXAS PARKS AND WILDLIFE COMMISSION Singeltary Submission
Chronic Wasting Disease Herd Certification Program Standards Singeltary Comment
Posted by the Animal and Plant Health Inspection Service on Oct 26, 2026
Comment from Singeltary Sr., Terry
Attachments 1
APHIS USDA CWD Captive
TUESDAY, SEPTEMBER 30, 2025
USDA National Scrapie Program History and Bovine Spongiform Encephalopathy BSE TSE Update 2025
Cattle with the E211K polymorphism, and gCJD linked to a glutamic acid to lysine substitution at codon 200 (E200K) of PRNP, what if?
Cattle with the E211K vs Humans E200K of PRNP, what if?
ARS Research Elucidating the Pathobiology and Transmission of Transmissible Spongiform Encephalopathies 2025 Annual Report
Price of TSE Prion Poker Goes Up Again…terry
TUESDAY, JANUARY 20, 2026
Pathogenesis, Transmission and Detection of Zoonotic Prion Diseases Project Number 5P01AI077774-14 2025
SATURDAY, JANUARY 10, 2026
***> Neuropsychiatric symptoms in sporadic Creutzfeldt-Jakob disease, a review
WEDNESDAY, OCTOBER 15, 2025
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT 2025
FRIDAY, NOVEMBER 21, 2025
While no one was watching: Tenuous status of CDC prion unit, risk of CWD to people worry scientists
National Prion Disease Surveillance Data Data are updated quarterly.
Last updated on: June 30th, 2026
Annual Referred and Confirmed Cases Annual surveillance referrals received by the NPDPSC and the number of cases confirmed as prion disease. Data are organized by calendar year and include disease subtype classifications.
Annual Referred and Confirmed Cases - Table Data
Year1 Total Referrals2 Prion Disease Sporadic Familial iCJD vCJD
1999 & earlier 386 234 203 28 3 0
2000 145 102 90 12 0 0
2001 209 118 110 8 0 0
2002 241 144 124 18 2 0
2003 259 160 137 21 2 0
2004 314 180 163 16 0 1(3)
2005 331 179 157 21 1 0
2006 364 179 159 17 1 2(4)
2007 374 211 192 19 0 0
2008 384 220 204 16 0 0
2009 396 231 210 20 1 0
2010 402 247 219 28 0 0
2011 390 238 214 24 0 0
2012 413 244 221 23 0 0
2013 418 258 223 34 1 0
2014 355 208 185 21 1 1(5)
2015 400 261 242 19 0 0
2016 394 278 249 29 0 0
2017 377 265 246 19 0 0
2018 311 222 203 18 1 0
2019 434 279 258 21 0 0
2020 369 254 228 25 1 0
2021 343 246 224 22 0 0
2022 343 229 209 20 0 0
2023 326 239 217 21 1 0
2024 343 265 248 16 0 0
2025 349 263 225 31 0 0
2026 193 82 37 4 0 0
TOTAL 9,563(6) 6,036(7) 5,397(8) 571(9) 15 4
1 0
1 Listed based on the year of death or, if not available, on the year of referral;
2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted. Includes 40 autopsy-coordinated cases pending tissue receipt;
3 Disease acquired in the United Kingdom;
4 Disease acquired in the United Kingdom in one case and Saudi Arabia in the other.
5 Disease possibly acquired in a Middle Eastern or Eastern European country;
6 Includes 60 cases in which the diagnosis is pending (3 from 2025, and 57 from 2026), and 25 inconclusive cases;
7 Includes 49 (1 from 2024, 23 from 2025, and 41 from 2026) cases with type determination pending, in which the diagnosis of vCJD has been excluded.
8 The sporadic cases include 5,298 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 96 cases of Variably Protease-Sensitive Prionopathy (VPSPr), and 43 cases of sporadic Fatal Insomnia (sFI).
9 Total does not include 353 Familial cases diagnosed by blood only.
NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD SURVEILLANCE TABLES OF CASES EXAMINED NPDPSC Last updated March 31st, 2026
NPDPSC Table of Cases Examined Updated quarterly. Last updated on: March 31st, 2026
Year Total Neuropath Referrals2 Prion Disease Sporadic Familial iCJD vCJD
1999 & earlier 386 234 203 28 3 0
2000 145 102 90 12 0 0
2001 209 118 110 8 0 0
2002 241 144 124 18 2 0
2003 259 160 137 21 2 0
2004 314 180 163 16 0 13
2005 331 179 157 21 1 0
2006 364 179 159 17 1 24
2007 374 211 192 19 0 0
2008 384 220 204 16 0 0
2009 396 231 210 20 1 0
2010 402 247 219 28 0 0
2011 390 238 214 24 0 0
2012 413 244 221 23 0 0
2013 418 258 223 34 1 0
2014 355 208 185 21 1 15
2015 400 261 242 19 0 0
2016 394 278 249 29 0 0
2017 377 265 246 19 0 0
2018 311 222 203 18 1 0
2019 434 279 258 21 0 0
2020 369 254 228 24 1 0
2021 343 246 224 22 0 0
2022 343 229 209 20 0 0
2023 325 239 217 21 1 0
2024 344 266 249 16 0 0
2025 351 236 214 29 0 0
2026 98 11 2 1 0 0
TOTAL 9,4706 5,9597 5,3528 5659 15 4
Year CSF Only & RT-QuIC Positive10
2015 139
2016 186
2017 225
2018 261
2019 306
2020 308
2021 325
2022 339
2023 314
2024 411
2025 401
2026 104
TOTAL 331911
1 Listed based on the year of death or, if not available, on the year of referral;
2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted. Includes 44 autopsy-coordinated cases pending tissue receipt;
3 Disease acquired in the United Kingdom;
4 Disease acquired in the United Kingdom in one case and Saudi Arabia in the other.
5 Disease possibly acquired in a Middle Eastern or Eastern European country;
6 Includes 61 cases in which the diagnosis is pending (12 from 2025, and 49 from 2026), and 25 inconclusive cases;
7 Includes 23 (23 from 2025) cases with type determination pending, in which the diagnosis of vCJD has been excluded.
8 The sporadic cases include 5,214 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 96 cases of Variably Protease-Sensitive Prionopathy (VPSPr), and 42 cases of sporadic Fatal Insomnia (sFI).
9 Total does not include 349 Familial cases diagnosed by blood only.
10 Lists the number of positive RT-QuIC cases for which we have not received tissue.
11 Includes the number of positive RT-QuIC results reported from Mayo Clinic (12 from 2024, 90 from 2025, and 26 from 2026).
2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted. Includes 44 autopsy-coordinated cases pending tissue receipt;
3 Disease acquired in the United Kingdom;
4 Disease acquired in the United Kingdom in one case and Saudi Arabia in the other.
5 Disease possibly acquired in a Middle Eastern or Eastern European country;
6 Includes 61 cases in which the diagnosis is pending (12 from 2025, and 49 from 2026), and 25 inconclusive cases;
7 Includes 23 (23 from 2025) cases with type determination pending, in which the diagnosis of vCJD has been excluded.
8 The sporadic cases include 5,214 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 96 cases of Variably Protease-Sensitive Prionopathy (VPSPr), and 42 cases of sporadic Fatal Insomnia (sFI).
9 Total does not include 349 Familial cases diagnosed by blood only.
10 Lists the number of positive RT-QuIC cases for which we have not received tissue.
11 Includes the number of positive RT-QuIC results reported from Mayo Clinic (12 from 2024, 90 from 2025, and 26 from 2026).
ai answer to my question on funding for prion disease 2025 2026;
Federal funding for prion disease surveillance through the Centers for Disease Control and Prevention (CDC) was heavily contested for the 2026 fiscal year. While the President’s proposed budget requested the complete elimination of specific funding lines for human prion disease surveillance, Congress restored the budget. The final budget legislation provided level or slightly increased funding for these critical initiatives.
please note; FY26 President's Budget Prion = 0 $
Budget Area Total Current FY25 Funding FY26 President's Budget % Change from Current
snip…
Emerging and Zoonotic Infectious Diseases
snip…
Prion Disease $8,000,000 $0 $9,000,000 $1,000,000
snip…
Total* $9,217,495,000 $5,470,349,000 -40.65% $9,152,090,000 -0.71% $7,480,880,000 -18.84% -18.26% $9,202,991,000 -0.16%
***> 2023 Professor John Collinge on tackling prion diseases <***
“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”
There is accumulating evidence also for iatrogenic AD.
Understanding prion biology, and in particular how propagation of prions leads to neurodegeneration, is therefore of central research importance in medicine.
Front. Public Health, 12 June 2024
Sec. Infectious Diseases: Epidemiology and Prevention Volume 12 - 2024 |
https://doi.org/10.3389/fpubh.2024.1411489
Updated global epidemiology atlas of human prion diseases
The top seven countries in PrD cases were the USA (n = 5,156), France (n = 3,276), Germany (n = 3,212), Italy (n = 2,995), China (n = 2,662), the UK (n = 2,521), Spain (n = 1,657), and Canada (n = 1,311).
Research Letter
December 11, 2023
Change in Epidemiology of Creutzfeldt-Jakob Disease in the US, 2007-2020
Matthew A. Crane, BS1; Sameer Nair-Desai, BS2; Alison Gemmill, PhD3; et al John A. Romley, PhD4; John C. Probasco, MD5
Author Affiliations Article Information JAMA Neurol. 2024;81(2):195-197. doi:10.1001/jamaneurol.2023.4678
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive and universally fatal prion disease.1 Research on CJD in the US showed stable incidence from 1979 to 2006, though recent trends are not as well described.2 The incidence of sporadic CJD, the most common type, is higher among older patients.1,2 Due to aging populations worldwide, the epidemiology of CJD is evolving.3 We examined death certificate data from 2007 to 2020 to better understand recent US trends of CJD.
Snip…
Discussion Our findings indicate the reported incidence of CJD has risen considerably, disproportionately affecting older and female individuals. These trends align with data from Japan3 and could be influenced by changing demographics. However, our findings may also reflect improved detection of CJD with new diagnostic tools, such as magnetic resonance imaging and real-time quaking-induced conversion testing. This study is limited by a reliance on death certificate data for estimating CJD incidence. While research supports this approach,5 such data may be subject to miscoding or misdiagnosis. Results from both neuropathologic and genetic testing may complement death certificate data and enhance surveillance.6 The findings underscore the changing landscape of CJD and suggest a need for monitoring among the aging US population.
Eur J Epidemiol. 2023; 38(7): 757–764. Published online 2023 May 16. doi: 10.1007/s10654-023-01004-5 PMCID: PMC10276107PMID: 37191829 Received: 31 January 2023 / Accepted: 6 April 2023 / Published online: 16 May 2023 © The Author(s) 2023
The role of environmental factors on sporadic Creutzfeldt-Jakob disease mortality: evidence from an age-period-cohort analysis
Abstract
Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common form of prion diseases. The causes of sCJD are still unknown and exogenous factors may play a role. Worldwide, the number of patients with sCJD has progressively increased over time. This increase can be partly explained by increasing life expectancy and better case ascertainment, but a true increase in the number of sCJD cases cannot be excluded. We estimated mortality rates from sCJD in France (1992–2016) and studied variation in mortality rates by age, period, and time.
snip...
The overall sCJD mortality rate was 4.58 per 1,000,000 person-years (95% CI=4.39–4.78) (Table S1).
snip...
Besides risk factors explored in case-control studies, the possibility of zoonotic risk factors remains a possibility that could account for an exogenous origin in some sCJD cases. Research on atypical forms of BSE (L-BSE, H-BSE) has revealed molecular similarities between the L-BSE strain and molecular subtypes of human sCJD, in particular the MV2 subtype [39]. Furthermore, L-BSE has been experimentally transmitted to non-human primates as efficiently as classical BSE responsible for vCJD in humans, and could be even more virulent [40–42]. The zoonotic risk associated with natural sheep scrapie has also been recently updated with the demonstration of an intracerebral transmission of scrapie to mice expressing the human prion protein during serial passages, as well as transmission of scrapie to primates. These observations highlight the possibility of a causal link between exposure to sheep scrapie and sCJD in some cases [43, 44]. A large increase in animal product consumption and the generalization of mechanically separated meat in developed countries over the last century may have contribute to increase the zoonotic prion pressure [45]. It would be of interest to observe the effect of safety measures implemented since the “mad cow crisis” to avoid population prion exposure on sCJD mortality in the next decades.
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025
From the first full year of reporting CJD TSE in the US in 2000, where 90 cases of CJD was reported that year, to today, where in September 2025, the number of CJD cases reported in the last full year reporting, which would have been 2024, the number of CJD cases for 2024 was 249 cases. So, from the first full year 2000 CJD cases were 90 cases confirmed in that year, to 2024, where 2024 CJD statistics rose to 249 confirmed CJD cases in a single year. A dramatic increase in deaths, from figures that don’t seem to be dramatic. But thes figures today, they are not from “better surveillance”, that dog don’t hunt no more. They have been saying this for over 25 years, year after year, well it’s time to call it for what it is, Human Transmissible Spongiform Encephalopathy TSE Prion cases are rising, and it’s NOT because of better surveillance, or just a “happenstance of bad luck, that 85%+ of all human cases, sporadic CJD, including VPSPr, just happen spontaneously, no, it’s because of unknown environmental factors, and or iatrogenic factors, imho…terry
US NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD TSE REPORT SEPTEMBER 2025
TUESDAY, DECEMBER 12, 2023
CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE UPDATE USA DECEMBER 2023
May 28, 2026
New study explores potential cross-species spread of chronic wasting disease
A serious wildlife disease is prompting growing concern among scientists, conservationists and public health experts
i remember R Bradley comments early BSE days…
“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”
http://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf
chronic wasting disease cwd tse prion update May 2026
cwd, tse, prion, cervid, cattle, sheep, pigs, primates, oh my!
think ‘friendly fire’, iatrogenic, terrible thought, but, uwhat if?
WEDNESDAY, MAY 27, 2026
Limited transmission of cervid prions to nonhuman primates provides insights into the zoonotic potential of chronic wasting disease
***> Cervid CWD TSE PrP Transmission to Humans, has it already happened and being masked as sporadic CJD, What if? July 2026 Review
https://chronic-wasting-disease.blogspot.com/2026/07/cervid-cwd-tse-prp-transmission-to.html
https://prpsc.proboards.com/thread/224/cervid-transmission-humans-july-review
https://chronic-wasting-disease.blogspot.com/2026/07/cervid-cwd-tse-prp-transmission-to.html
https://prpsc.proboards.com/thread/224/cervid-transmission-humans-july-review
2001 Singeltary on CJD, Journal of American Medical Association
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
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.
February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
Author Affiliations
JAMA. 2001;285(6):733-734. doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 Singeltary Journal of Neurology
26 MARCH 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Terry S. Singeltary, retired (medically)
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?
MONDAY, JUNE 1, 2026
NATIONAL PRION DISEASE PATHOLOGY SURVEILLANCE CENTER CJD SURVEILLANCE TABLES OF CASES EXAMINED NPDPSC Last updated March 31st, 2026
Terry S. Singeltary Sr.
