Human Prion Disease Surveillance in Washington State, 2006-2017
Original Investigation | Neurology
Human Prion Disease Surveillance in Washington State, 2006-2017
Liliana Sánchez-González, MD, MPH; Ryan A. Maddox, PhD; Larissa C. Lewis, RN; Janis E. Blevins, MSM-HC; Elizabeth J. Harker, MPH; Brian S. Appleby, MD; Marissa K. Person, MSPH; Lawrence B. Schonberger, MD; Ermias D. Belay, MD; Chas DeBolt, RN, MPH; Kathryn H. Lofy, MD
Key Points
Question What are the results of human prion disease surveillance in Washington state?
Findings In this cross-sectional study using state surveillance data from 2006 to 2017, 143 human prion disease cases were detected, with an average annual age-adjusted incidence consistent with national reports. The majority of cases (94%) were sporadic, and no cases met criteria for a variant Creutzfeldt-Jakob disease diagnosis.
Meaning These findings indicate that prion disease surveillance in Washington state is beneficial for monitoring epidemiological trends, facilitating accurate diagnoses, and detecting variant Creutzfeldt-Jakob disease or other emerging human prion diseases should they occur.
Abstract
IMPORTANCE Human prion disease surveillance is critical to detect possible cases of variant Creutzfeldt-Jakob disease and other acquired forms of prion disease in the United States. Results are presented here that describe 12 years of surveillance in Washington, the only US state that has reported the presence of classic bovine spongiform encephalopathy, an animal prion disease that has been shown to transmit to humans.
OBJECTIVE To describe the current prion disease surveillance system in Washington and the epidemiological and clinical results of surveillance from 2006 through 2017.
DESIGN,SETTING,ANDPARTICIPANTS This cross-sectional study reports findings from the human prion disease surveillance system in place in Washington state from January 1, 2006, through December 31, 2017. Participants included Washington residents with a clinical suspicion of human prion disease or suggestive test results from the National Prion Disease Pathology Surveillance Center or with prion disease listed as a cause of death on the death certificate. Data for this report were analyzed from June 1, 2016, to July 1, 2020.
EXPOSURE Human prion disease diagnosis.
MAIN OUTCOMES AND MEASURES The main outcome was incidence of human prion disease
cases, including identification of variant Creutzfeldt-Jakob disease.
RESULTS A total of 143 human prion disease cases were detected during the study period, none of which met criteria for a variant Creutzfeldt-Jakob disease diagnosis. Among 137 definite or probable cases, 123 (89.8%) occurred in persons aged 55 years or older, with a median age at death of 66 years (range, 38-84 years). Most patients were White (124 [92.5%] among 134 with reported race), and slightly over half were male (70 [51.1%]). The average annual age-adjusted prion disease incidence was 1.5 per million population per year, slightly higher than the national rate of 1.2 per million. A total of 99 cases (69.2%) were confirmed by neuropathology. Sporadic prion disease was the most common diagnosis, in 134 cases (93.7%), followed by familial prion disease in 8 cases (5.6%). One iatrogenic prion disease case (0.7%) was also reported.
CONCLUSIONS AND RELEVANCE The findings of this cross-sectional study suggest that demographic characteristics of patients with prion disease in Washington are consistent with national findings. The slightly higher incidence rate may be due to the state’s enhanced surveillance activities, including close collaboration with key partners and educational efforts targeted toward health care providers. Results indicate that surveillance will continue to be beneficial for monitoring epidemiological trends, facilitating accurate diagnoses, and detecting variant Creutzfeldt-Jakob disease or other emerging human prion disease cases.
JAMA Network Open. 2020;3(10):e2020690. doi:10.1001/jamanetworkopen.2020.20690
Author affiliations and article information are listed at the end of this article.
Open Access. This is an open access article distributed under the terms of the CC-BY License.
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JAMANetworkOpen | Neurology HumanPrionDiseaseSurveillanceinWashingtonState,2006-2017 Introduction
Prion diseases, also called transmissible spongiform encephalopathies, are a group of rare, fatal, neurodegenerative diseases that occur in animals and humans. These diseases are characterized by the conversion of normal prion proteins into abnormal, pathogenic agents known as prions.1-3 This conversion process can be sporadic, related to a genetic mutation, or induced by the uptake of the pathogenic prions.3,4 The accumulation of prions is associated with neuronal injury leading to spongiform changes in the central nervous system.5
The most common human prion disease (HPD) is Creutzfeldt-Jakob disease (CJD), with an age-adjusted incidence of 1.2 cases per million population per year in the United States, similar to the incidence reported in other countries.6,7 Sporadic CJD accounts for approximately 85% of CJD cases and familial CJD for 10% to 15%.3 Other less common prion diseases include fatal familial insomnia, sporadic fatal insomnia, Gerstmann-Straüssler-Scheinker disease, variably protease-sensitive prionopathy, and acquired prion disease.
In 1996, a new, acquired HPD, termed variant CJD (vCJD), was described in the United Kingdom.8 It was linked to the consumption of prion-contaminated beef or beef products from cattle afflicted with bovine spongiform encephalopathy (BSE).9 Later, several patients with vCJD were determined to have contracted their illness through receipt of blood from donors who subsequently developed vCJD.10,11 As of January 2020, 232 cases of vCJD have been described around the world.12 This number includes 4 patients who were diagnosed in the United States but were likely exposed to the infectious agent outside the country.13 Variant and iatrogenic (ie, health care–acquired) CJD accounts for less than 1% of the total number of HPD cases.
In December 2003, a dairy cow imported from Canada into the state of Washington was diagnosed with BSE. Beef from the slaughtered cow had been processed for human consumption, and a recall of all beef from cattle slaughtered the same day at the involved slaughter plant was requested.14 In response to this incident, the Washington State Department of Health (WA DOH) established an enhanced HPD surveillance system the following year and has maintained a centralized database of all suspected cases in the state since that time. Human prion disease has been a distinct notifiable condition in Washington (Washington Administrative Code 246-101-101) since February 2011. Once clinically diagnosed, cases are to be reported within 3 business days to public health authorities (ie, local health jurisdictions).15 Before 2011, HPD was notifiable under the category of “other rare diseases of public health significance.”
The objectives of surveillance activities in Washington are (1) to establish background incidence rates and monitor trends in the epidemiology of HPD in the state, (2) to detect the possible emergence of vCJD or a possible new HPD, (3) to detect and help prevent potential iatrogenic CJD, and (4) to facilitate accurate HPD diagnoses. To aid in accomplishing these objectives, and in collaboration with the US Centers for Disease Control and Prevention (CDC), WA DOH personnel work closely with the National Prion Disease Pathology Surveillance Center (NPDPSC) at Case Western Reserve University, which is supported by the CDC and sponsored by the American Association of Neuropathologists. A major purpose of the NPDPSC is to provide state-of-the-art diagnostic testing for clinically suspected HPD cases.16
This article describes the current HPD surveillance system in place in Washington and the epidemiological and clinical results of surveillance activities during the years 2006 through 2017.
Methods
This cross-sectional study was conducted using data obtained through the HPD surveillance system in Washington state for HPD cases with date of death from January 1, 2006, through December 31, 2017. Prion disease surveillance is a component of regular WA DOH duties; associated activities are not considered research and are not reviewed by an ethics committee. The presentation and
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JAMANetworkOpen | Neurology HumanPrionDiseaseSurveillanceinWashingtonState,2006-2017 discussion of study findings followed the Strengthening the Reporting of Observational Studies in
Epidemiology (STROBE) reporting guideline.
Case Finding
The WA DOH receives prion disease reports from 3 main sources: the NPDPSC, death certificate data, and health care professionals, including physicians and infection control professionals. On rare occasions, notifications from the general public have also been received.
The NPDPSC provides a WA DOH prion epidemiologist (including L.S.-G. or L.L.) with a copy of the results of brain autopsies and tests performed at the center for Washington residents or those ordered by Washington health care professionals. Among the possible tests are cerebrospinal fluid (CSF) Tau protein, CSF 14-3-3 protein, second-generation real-time quaking-induced conversion (RT-QuIC), brain biopsy, and blood PRNP genotyping. For some cases, the center may arrange for expert reviews of available magnetic resonance imaging results. The prion epidemiologist combines these NPDPSC data with available WA DOH information from case reports and investigations.
All patients in Washington with at least 1 positive test result (ie, positive 14-3-3, positive RT-QuIC, or tau protein level >1150 mg/mL) are investigated. Patients for whom clinical or epidemiological suspicion persists despite negative results are discussed with the CDC to determine the need for additional follow-up. The WA DOH conducts most of the investigations, although some of the 35 local health jurisdictions in the state conduct their own.
The WA DOH prion epidemiologist maintains routine and frequent communication with the State Vital Statistics epidemiologist. All death certificates with literal text and/or codes corresponding with HPDs (eg, Creutzfeldt-Jakob, fatal familial insomnia, prion disease, Gerstmann- Sträussler-Scheinker) are reviewed to determine whether the case is in the prion epidemiologist’s database and to establish whether further follow-up is necessary.
Case Investigation
Data on demographics, clinical presentation and course, and infection prevention and control are collected. Race and ethnicity are obtained from the demographics section of available medical records, if noted, with options being defined by the hospitals and medical institutions; otherwise, these data are obtained from the death certificate. These characteristics were assessed because previous studies have shown differences in prion disease incidence by race.6,17
After notification of a suspected case, the prion epidemiologist obtains and reviews medical records and tests results. Facilities where the patient had been hospitalized are contacted, and interviews with clinicians, infection control professionals, and/or families are pursued to collect information on clinical presentation and risk factors for acquired prion disease, such as previous neurosurgery or receipt of a cadaver-derived pituitary hormone. Information regarding prion diseases and infection control measures are shared with infection control professionals.
The prion epidemiologist encourages health care professionals to discuss the possibility of autopsy with the patient’s caregivers when appropriate to obtain neuropathological confirmation of the diagnosis. Information regarding the NPDPSC’s autopsy service is given to the health care professional; information regarding other services available at the NPDPSC (eg, genetic testing for familial prion disease) and support services (eg, CJD Foundation) is given to families.
Cases of CJD are classified as sporadic (definite, probable, or possible), familial, or iatrogenic according to the current CDC Diagnostic Criteria.18 Cases lacking histopathological confirmation, genetic testing, and information indicating an iatrogenic source of infection or familial disease are classified as sporadic by default. Cases with insufficient information available for designation as definite, probable, or possible CJD are classified as physician-diagnosed CJD, provided CJD is listed as a cause of death on the death certificate and a prion disease diagnosis cannot be ruled out. Cases are followed until a prion disease diagnosis is excluded or confirmed or the patient dies. If biopsy or autopsy is performed, the follow-up continues until final results of brain tissue tests are received, a type of prion disease is established, and the case is closed.
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Cases in which the patient is younger than 55 years or those with unusual clinical presentation or diagnostic results, concerning exposures, or any other special characteristics are discussed with the CDC and the NPDPSC in order to determine the need for further testing or public health measures.
Data Collection and Statistical Analysis
All investigated cases (confirmed or not) are included in a confidential line list maintained by the WA DOH prion epidemiologist. Cases are also included in the Public Health Information Management System database. This database contains demographic, clinical, and infection control information regarding patients with notifiable conditions reported to WA DOH. When available, medical records for the HPD cases reported and investigated in Washington with date of death between January 1, 2006, and December 31, 2017, were reviewed and the abstracted information analyzed. Data analysis was conducted from June 1, 2016, to July 1, 2020.
Tau protein results were available from 2007 through 2017, second-generation RT-QuIC results were available starting in April 2015, and 14-3-3 results were available for the full 12 years included in this study. Incidence rates and demographic statistics were calculated based on cases classified as sporadic prion disease (definite or probable), familial prion disease, or iatrogenic CJD. Information regarding onset, clinical presentation, CSF test results, electroencephalography results, brain imaging studies, and pathology results were analyzed using SAS statistical software, version 9.4 (SAS Institute, Inc). Average annual age-adjusted incidence rates were calculated using the year 2000 as the standard population. Statistical significance was set at P < .05, and a 2-sided P value was calculated from the 95% CIs of the rate difference assuming a normal distribution.
Results
A total of 143 HPD cases in Washington with date of death during 2006 through 2017 were detected (Table 1) and classified according to CDC criteria. Most of the cases (n = 134 [93.7%]) were classified as sporadic prion disease, 8 cases (5.6%) as familial prion disease, and 1 case (0.7%) as iatrogenic CJD. No cases of vCJD were identified. The average annual age-adjusted prion disease incidence was 1.5 per million population per year. For the periods 2006-2009, 2010-2012, 2013-2015, and 2015- 2017, the incidence was 1.6, 1.3, 1.5, and 1.5 cases per million population, respectively, demonstrating stability. Demographic characteristics are described in Table 2. Among 137 definite or probable cases, 123 (89.8%) occurred in persons aged 55 years or older, with a median age at death of 66 years (range, 38 to 84 years). Most patients were White (n = 124 [92.5%] among 134 with reported race), and slightly over half were male (n = 70 [51.1%]). Average annual age-adjusted incidence between men and women (1.6 vs 1.4 per million, respectively) was not different (P = .35). The highest average annual age-specific incidence rate, 9.1 per million, was observed in those aged 75 to 84 years.
Of the total 134 sporadic prion disease cases, 92 (68.7%) were neuropathologically confirmed (ie, definite cases); these cases included sporadic CJD (n = 90 [97.8%]), sporadic fatal insomnia (n = 1 [1.1%]), and variably protease-sensitive prionopathy (n = 1 [1.1%]) (Figure). Among the 90 definite sporadic CJD cases, 38 (42.2%) were the MM1 subtype, 10 (11.1%) were MV1, 10 (11.1%) were VV2, 8 (8.9%) were MM1-2, 8 (8.9%) were MV1-2, 7 (7.8%) were MV2, 5 (5.6%) were VV1-2, and 3 (3.3%) were MM2; 1 sporadic CJD case for whom frozen autopsy tissue was unavailable for analysis was
Table 1. Human Prion Disease Cases Reported in Washington State, 2006-2017
Disease category
Sporadic Familial Iatrogenic Total
Definite Probable
92 36 8 NA 1 NA 101 36
Possible Physician-diagnosed Total
4 2 NA NA NA NA 4 2
134 8
1 143
Abbreviation: NA, not applicable.
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considered by NPDPSC to be consistent with the MM(MV)1 subtype. Of the remaining sporadic prion disease cases (n = 42), most were classified as probable CJD (n = 36 [85.7%]). Test results for second-generation RT-QuIC in CSF were available for 10 of the probable CJD cases, 9 of which were positive, indicating likely true prion disease given the test’s very high specificity.19 Four of the 42 cases (9.5%) met criteria for possible CJD, and the remaining 2 cases lacked sufficient information for classification and were designated as physician-diagnosed cases; both of these patients were older than 60 years, making a vCJD diagnosis unlikely.
Of the 8 familial prion disease cases, 7 were familial CJD, and 1 was Gerstmann-Straüssler- Scheinker disease. All familial cases were classified as definite, including 2 that lacked neuropathologic testing but met clinical criteria and had a genetic mutation and/or positive family history. Three of the familial CJD cases had the E200K mutation; the remaining 4 cases had T183A, T188R, or D178N mutations, or a 5 octapeptide insert. The Gerstmann-Straüssler-Scheinker case had a 9 octapeptide insert.
The iatrogenic CJD case was associated with cadaver-derived human growth hormone administration during childhood and is part of an ongoing US outbreak of human growth hormone– associated CJD.20 It also had the unusual, distinct pathological phenotype reported as likely specific for iatrogenic CJD.20-22
Cases were reported from 26 of 35 local health jurisdictions (74.3%). There was no clear geographic clustering. One-third of the cases (n = 55 [38.4%]) occurred among residents of King and Pierce counties, which include the cities of Seattle and Tacoma and represent 41% of the state’s population.23
A majority of all prion disease cases (n = 99 [69.2%]), including 9 of the 15 cases (60.0%) in patients younger than 55 years at death, were neuropathologically confirmed by brain biopsy (n = 3), autopsy (n = 89), or both (n = 7). The annual percentage of cases confirmed by neuropathology ranged from 56.3% to 90.0%. Of the 99 confirmed cases, 85 (85.9%) had a CSF 14-3-3 protein result available; of those, 67 (78.8%) had a positive test result, whereas 10 (11.8%) were ambiguous and 8 (9.4%) were negative. Seven of the 8 confirmed cases with a negative 14-3-3 protein result also had the Tau protein test performed; 2 had a Tau protein level greater than 1150 mg/mL, and 5 did not.
Case-defining symptoms and signs reported for sporadic prion disease patients are summarized in Table 3. All patients presented with rapidly progressive dementia. Cerebellar abnormalities were reported in 103 cases (75.2%), and approximately half of patients reported extrapyramidal abnormalities (n = 68 [49.6%]) and visual abnormalities (n = 61 [44.5%]). Akinetic mutism and pyramidal signs were the least commonly reported clinical features. The WA DOH surveillance activities described in the Methods continue uninterrupted, and no vCJD diagnoses have been made in Washington after the time frame of this study.
Discussion
Since the identification of a BSE-positive dairy cow in the state in 2003, WA DOH, in collaboration with the CDC and the NPDPSC, has been conducting HPD surveillance. During the 12-year period evaluated in this cross-sectional study, the vast majority of HPD cases identified were classified as sporadic CJD, and no vCJD cases were detected. The average annual age-adjusted incidence, 1.5 cases per million population, was stable during this period with no apparent geographical clustering. Higher incidence rates were reported among decedents who were male, White, and aged 55 years or older, congruous with national findings.6 The finding of an average annual age-adjusted incidence slightly higher than the national rate, 1.2 per million, may be at least partially explained by the enhanced prion disease surveillance conducted in Washington, which has led to improved case detection. Efforts have been made in the state to increase awareness among neurologists and other medical providers regarding prion disease and the services available at the NPDPSC for diagnosis and confirmation purposes.
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Table 2. Demographic Characteristics
of 137 Decedents With Definite and Probable Human Prion Disease in Washington State, 2006-2017a
Characteristic
Total
Age at death, y
<55 55-64 65-74 75-84
Sex Male
Female Race/ethnicityb
White
Asian
Black
Native American
Initial reporter
National Prion Disease Pathology Surveillance Center
Physician or health care facility
Death certificate Funeral home Other
No. (%)
137 (100.0)
14 (10.2) 46 (33.6) 48 (35.0) 29 (21.2)
70 (51.1) 67 (48.9)
124 (92.5) 5 (3.7)
4 (3.0)
1 (0.7)
104 (75.9)
21 (15.3)
6 (4.4) 1 (0.7) 5 (3.6)
a Fourpossiblecasesand2physician-diagnosed cases excluded.
b Race/ethnicityunknownin3cases.
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The median age at death (66 years) is comparable to national findings and findings from other industrialized countries.24-26 As expected, the vast majority of cases occurred among persons aged 55 years or older. The proportion of people aged 65 years or older in Washington increased from 12.3% in 2010 to 16.2% in 201923; as the state population continues to age, a corresponding increase in the number of HPD cases is expected. According to official estimates for 2010,24 82.3% of Washington state residents are White, 7.3% are Asian, 3.7% are Black, and 1.8% are American Indian/ Alaska Native. Consistent with previous studies,2,6 White decedents were overrepresented in our findings, comprising 92.5% of the definite and probable HPD cases.
Since 2003, 5 additional cases of BSE, all atypical, have been identified in other states; no further BSE cases have been reported in Washington, which remains the only state where classic BSE has been found.14 This fact, combined with the recall of beef products in Washington after the BSE-infected cow’s slaughter, increases the importance of the state’s human prion disease surveillance.
Recently, concerns about the potential for chronic wasting disease (CWD), a prion disease of deer, elk, and moose, to transmit to humans have also been raised.27 Chronic wasting disease has not yet been found in Washington despite extensive surveillance conducted between 2001 and 2012, when more than 5000 animals were tested without finding any positive results. Currently, the Washington Department of Fish and Wildlife conducts surveillance targeting only animals with clinical signs of CWD. Should CWD be found in Washington in the future, ongoing HPD surveillance will be beneficial in assessing whether there is any connection between animal and human disease.
Figure. Definite and Probable Human Prion Disease Cases by Type and Year of Death, Washington State, 2006 to 2017
14 12 10
8 6 4 2 0
Definite sporadic prion diseasea
Probable sporadic prion disease
Familial prion diseaseb
Iatrogenic CJD
2006 2007 2008
2009 2010
2011 2012 2013 2014 Year of death
2015 2016 2017
Results include 137 individuals. CJD indicates Creutzfeldt-Jakobdisease.
b Familial prion disease includes familial CJD and Gerstmann-Sträussler-Scheinkerdisease. and sporadic fatal insomnia.
a SporadicpriondiseaseincludessporadicCJD,variablyprotease-sensitiveprionopathy,
Table 3. Case-Defining Clinical Features for Definite and Probable Human Prion Disease Cases at Any Time of Disease, Washington State, 2006-2017
a Clinical finding
Rapidly progressive dementia Cerebellar abnormalities Extrapyramidal
Visual abnormalities Myoclonus
Pyramidal Akinetic mutism
No. (%)
Definite (n = 101) 101 (100.0)
72 (71.3)
41 (40.6)
51 (50.5)
35 (34.7)
14 (13.9)
11 (10.9)
Probable (n = 36)
36 (100.0) 31 (86.1) 27 (75.0) 10 (27.8) 21 (58.3) 9 (25.0) 12 (33.3)
Total (n = 137)
137 (100) 103 (75.2) 68 (49.6) 61 (44.5) 56 (40.9) 23 (16.8) 23 (16.8)
a Four possible cases and 2 physician-diagnosed cases excluded.
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Limitations
A limitation of this study is that only partial medical records were available for some patients, and the complete clinical presentation of the disease, including initial signs and symptoms, was sometimes difficult to ascertain. This limitation could potentially influence case classification, because some actual criteria and findings may not have been considered. In addition, the highly specific second- generation RT-QuIC CSF test was not regularly used by NPDPSC until 2015. Most cases, however, had neuropathologic confirmation. Collaboration with the NPDPSC, which performs the majority of premortem tests, enables a timely notification to WA DOH of suspected cases in the state and thus contributes to the number of subsequent autopsies performed.
Conclusions
In this cross-sectional study of Washington state’s HPD surveillance system, findings suggest that the demographic characteristics of patients with prion disease between 2006 and 2017 were consistent with national findings. Despite a statewide prion disease surveillance program being in place since 2004, neither vCJD nor another new prion disease has been detected in the state. Given the long incubation periods associated with prion diseases, ongoing vigilance and collaboration with surveillance partners continue to be necessary.
ARTICLE INFORMATION
Accepted for Publication: August 5, 2020.
Published: October 16, 2020. doi:10.1001/jamanetworkopen.2020.20690
Open Access: This is an open access article distributed under the terms of the CC-BY License. © 2020 Sánchez-González L et al. JAMA Network Open.
Corresponding Author: Liliana Sánchez-González, MD, MPH, Centers for Disease Control and Prevention, 1324 Calle Cañada, San Juan, Puerto Rico, 00920 (
naq5@cdc.gov).
Author Affiliations: Washington State Department of Health, Shoreline (Sánchez-González, Lewis, DeBolt, Lofy); Dengue Branch, Division of Vector Borne Diseases, Centers for Disease Control and Prevention, San Juan, Puerto Rico (Sánchez-González); Division of High-Consequence Pathogens and Pathology, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, Georgia (Maddox, Harker, Person, Schonberger, Belay); National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio (Blevins, Appleby); Hyland Software, Westlake, Ohio (Blevins); Texas Department of State Health Services, Austin (Harker).
Author Contributions: Dr Sánchez-González had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Concept and design: Sanchez-Gonzalez, Maddox, Schonberger, Lofy, DeBolt, Belay.
Acquisition, analysis, or interpretation of data: Sanchez-Gonzalez, Maddox, Appleby, Blevins, Person, Lewis, Lofy,
DeBolt, Belay, Harker.
Drafting of the manuscript: Sanchez-Gonzalez, Maddox, DeBolt.
Critical revision of the manuscript for important intellectual content: All authors. Statistical analysis: Maddox, Person, Harker.
Obtained funding: Schonberger, Appleby.
Administrative, technical, or material support: Appleby, Blevins, Lewis, Lofy, DeBolt. Supervision: Schonberger, Appleby, Lofy, DeBolt, Belay.
Conflict of Interest Disclosures: Dr Appleby reported receiving grants from the Centers for Disease Control and Prevention during the conduct of the study and financial support for test development from Ionis outside the submitted work. Dr Lewis reported receiving grants from the US Centers for Disease Control and Prevention during the conduct of the study. No other disclosures were reported.
Disclaimer: The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention.
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JAMA Network Open. 2020;3(10):e2020690. doi:10.1001/jamanetworkopen.2020.20690 (Reprinted) October 16, 2020 8/9
JAMANetworkOpen | Neurology HumanPrionDiseaseSurveillanceinWashingtonState,2006-2017 25. Heinemann U, Krasnianski A, Meissner B, et al. Creutzfeldt-Jakob disease in Germany: a prospective 12-year
surveillance. Brain. 2007;130(Pt 5):1350-1359. doi:10.1093/brain/awm063
26. GubbelsS,BacciS,LaursenH,etal.Descriptionandanalysisof12yearsofsurveillanceforCreutzfeldt-Jakob
disease in Denmark, 1997 to 2008. Euro Surveill. 2012;17(15):20142.
27. OsterholmMT,AndersonCJ,ZabelMD,ScheftelJM,MooreKA,ApplebyBS.Chronicwastingdiseasein cervids: implications for prion transmission to humans and other animal species. mBio. 2019;10(4):e01091-19. doi: 10.1128/mBio.01091-19
JAMA Network Open. 2020;3(10):e2020690. doi:10.1001/jamanetworkopen.2020.20690 (Reprinted) October 16, 2020 9/9
Subject: Washington State Human Prion diseasePrion Disease, Human
Familial Iatrogenic GSS Total Year sCJD CJD CJD VPSPr Syndrome (Definate or
Probable)
2008 17 0 0 0 0 17
2009 7 2 0 0 0 9
2010 7 1 0 0 0 8
2011 9 0 0 0 0 9
2012 14 1 0 1 0 16
2013 13 0 1 0 1 15
2014 11 1 0 0 0 12
2015 11 1 0 0 0 12
2016 17 1 0 0 0 18
2017 10 0 0 0 0 10
2018 15 0 0 0 0 15
sCJD: Spontaneous CJD GSS: Gerstmann-Straussler-Scheinker disease VPSPr: Variably protease-sensitive prionopath
Note: RT-QuIC testing became standard in 2015 and integrated in the case definition in 2018.
Source: Washington State Communicable Disease Report 2018 DOH 420-004 08/2019
There have been 6 cases of BSE identified in the United States. The following information provides descriptions of these 6 cases:
2018- Florida
2017 – Alabama
2012 - California
2006 - Alabama
2005 - Texas
2003 - Washington State
On December 23, 2003, the U.S. Department of Agriculture (USDA) announced a presumptive diagnosis of the first known case of BSE in the United States. It was in an adult Holstein cow from Washington State. This diagnosis was confirmed by an international reference laboratory in Weybridge, England, on December 25. Trace-back based on an ear-tag identification number and subsequent genetic testing confirmed that the BSE-infected cow was imported into the United States from Canada in August 2001.
Because the animal was non-ambulatory (a “downer cow”) at slaughter, brain tissue samples were taken by USDA’s Animal and Plant Health Inspection Service as part of its targeted surveillance for BSE. However the animal’s condition was attributed to complications from calving. After the animal was examined by a USDA Food Safety and Inspection Service (FSIS) veterinary medical officer both before and after slaughter, the carcass was released for use as food for human consumption. During slaughter, the tissues considered to be at high risk for the transmission of the BSE agent were removed.
On December 24, 2003, FSIS recalled beef from cattle slaughtered in the same plant on the same day as the BSE positive cow. (see Bovine Spongiform Encephalopathy in a Dairy Cow—Washington State, 2003.)
Preliminary Investigation Suggests BSE-Infected Cow in Washington State Was Likely Imported from Canada
On December 23, 2003, the U.S. Department of Agriculture (USDA) announced a presumptive diagnosis of bovine spongiform encephalopathy (BSE, or “mad cow” disease) in an adult Holstein cow from Washington State. Samples were taken from the cow on December 9 as part of USDA’s BSE surveillance program. The BSE diagnosis was made on December 22 and 23 by histopathology and immunohistochemical testing at the National Veterinary Services Laboratory, Ames, Iowa. The diagnosis was confirmed by an international reference laboratory in Weybridge, England, on December 25. Preliminary trace-back based on an ear-tag identification number suggests that the BSE-infected cow was imported into the United States from Canada in August 2001.
USDA, in close cooperation with Canadian agricultural authorities, has launched an epidemiologic investigation to determine the source of the disease. Beef from the slaughtered cow had been processed for human consumption. On December 23, 2003, the Food Safety and Inspection Service (FSIS), USDA announced the recall of all beef from cattle slaughtered on December 9 at the involved slaughter plant.
Strong evidence indicates that BSE has been transmitted to humans primarily in the United Kingdom, causing a variant form of Creutzfeldt-Jakob disease (vCJD). In the United Kingdom, where over 1 million cattle may have been infected with BSE, a substantial species barrier appears to protect humans from widespread illness. As of December 1, 2003, a total of 153 vCJD cases had been reported worldwide; of these, 143 cases had occurred in the United Kingdom. The risk to human health from BSE in the United States is extremely low.
CDC monitors the trends and current incidence of CJD in the United States by analyzing death certificate information from U.S. multiple cause-of-death data compiled by the National Center for Health Statistics. With the support of the Council of State and Territorial Epidemiologists, CDC conducts follow-up review of clinical and neuropathology records of CJD decedents younger than 55 years of age. In addition, during 1996-1997, in collaboration with the American Association of Neuropathologists (AANP), CDC established the National Prion Disease Pathology Surveillance Center External at Case Western Reserve University in Cleveland, Ohio. This pathology center provides free, state-of-the-art diagnostic services to U.S. physicians. It also helps to monitor the possible occurrence of emerging forms of prion diseases, such as vCJD, in the United States. For more information about the center visit its website at:
''A total of 143 human prion disease cases were detected during the study period, none of which met criteria for a variant Creutzfeldt-Jakob disease diagnosis.''
the ukbsenvcjd only theory has been proven wrong! more later in this document...terry
Subject: TECHNICAL BRIEFING BSE UPDATE USA DEC. 30, 2003 From: TSS <flounder@WT.NET> Reply To: Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> Date: Wed, 31 Dec 2003 02:05:07 +0100 Content-Type: text/plain Parts/Attachments: text/plain (685 lines) ######## Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> #########
Release No. 0451.03 Office of Communications 202-720-4623
Technical Briefing and Webcast with U.S. Government Officials On BSE Case Tuesday, Dec. 30 Welcome to today's technical briefing on the BSE situation. Today we are going to start off with an update by Dr. Ron DeHaven. He's the chief veterinary officer for USDA. We'll have a statement by Dr. Daniel Engeljohn, executive associate for policy from Food Safety Inspection Service. And then we'll have a statement by Dr. Lester M. Crawford. He's the deputy commissioner for the Food and Drug Administration.
I just want to point out a couple of things. Because of the number of people on this call, we would ask that you ask one question, no follow-ups. Also at the table here is Dr. Kenneth Petersen. He is the executive associate for regulatory operations at FSIS. He will be available as well to answer questions as needed. So the way it will work today is we'll let the statements go, and then we'll take three questions from the audio bridge; then we'll take three here, and then alternate. Dr. DeHaven will point you out. Wait for the mike to come to you. And then, with that, I think we're ready to begin. Dr. DeHaven?
DR. DEHAVEN: Thank you very much. Again, thank you all for being here. Ed mentioned that we will be making some statements. And, actually, I'm going to defer to my friend and colleague, Dr. Lester Crawford from Food and Drug Administration, and let him make the opening statement. Dr. Crawford?
DR. CRAWFORD: The Food and Drug Administration fully supports the safety policies announced today by the U.S. Department of Agriculture, which build on the principles and procedures that FDA and USDA have developed since 1997. These protective measures will add an additional layer of protection to the American public. In order to obtain these goals, FDA will evaluate the impacts of the new policies on the agency's resources, so that we can devise and implement the most effective and efficient additional layer of protection to the American public.
FDA will devote additional resources in order to do these increased responsibilities for protecting the safety of the food and feed supply. FDA will continue to rigorously enforce its measures to protect the public health against the BSE hazard. In the last six years, the agency has sponsored workshops, teleconferences and other outreach programs to stimulate cooperation of state, local and cross-border authorities in a vigilant surveillance for BSE. It has issued import alerts and bulletins to detain all products with processed animal protein from countries with BSE. It has requested blood centers to exclude blood donations by individuals who might be carriers of the BSE agent. And it has urged manufacturers of drugs, vaccines, medical devices and cosmetics to use only materials derived from cattle that are BSE free. The main focus of FDA's BSE prevention program has been regular inspections of all renderers and feed mills in the United States, more than 99 percent of whom have achieved full compliance with the 1997 FDA rule that prohibits the inclusion of most animal protein in feeds for cattle and other ruminants. The effectiveness of FDA's surveillance was most recently confirmed by the fact that all of the firms involved in the current BSE investigation were found to be in compliance with the FDA rule, and that the agency working with state and industry officials was able to halt the distribution of all of the meat and bone meal from the sick animal.
I cannot close without complimenting Secretary Veneman and the Department of Agriculture, who have been in complete communication with FDA. We have worked together and our agency applauds her leadership in this regard. We recognize that USDA is the lead agency. We want you to know that we feel like we are in complete communication, and we are working together on this, and in the end we will bring this to a satisfactory conclusion as fast as we possibly can.
Lastly, FDA has contained, as we have previously announced, all of the suspect rendered material. It is under our control, and it is being held at the present time.
DR. DEHAVEN: Dr. Crawford, thank you, and thank you for being here with us today.
Just a brief update on our investigation stemming from, again, a single positive cow slaughtered on December 9th, and she tested positive for BSE on December 23rd. Documentation is now available indicating that she was approximately six and a half years old at the time of slaughter, and our primary line of inquiry does lead to a farm in Alberta, Canada. The age of the animal is particularly important in that it does provide an explanation as to how she became infected, in that as a six-and-a-half-year-old with an approximate birth date of April 1997, she in fact would have been born before the feed ban went into place, either in Canada or the United States.
Multiple samples for DNA testing are in various stages of being submitted to two laboratories, one in Canada and one in the United States. We continue our trace-back and trace-forward investigations. We believe that the positive cow was one of 82 animals from the same Canadian herd that were permitted into the United States. We are conducting a painstaking records review to determine the transit, timing and current location of these 82 head of cattle. We do know that several are on the same premises where the positive cow was located immediately before she went to slaughter.
The positive cow we know had three calves while she was in the United States. The first animal, or first calf, was stillborn. The second is currently a yearling heifer, and is located on the index farm. And the third, a bull calf, is in a group of calves at another location, a calf feeding operation which is also under a state hold order.
I want to emphasize that these hold orders are not imposed because BSE is a contagious disease, or in any way to prevent the spread of this disease, since we know from the science that the disease is not spread by casual contact animal to animal. Rather, these hold orders are in place to ensure that we maintain the location of all animals that are of consequence and otherwise relevant to our ongoing investigation.
Just on a side note, if I could, I am hearing from my colleagues in the state of Washington that because of the incredible interest in this issue, there had been reports that reporters are visiting multiple dairy farms throughout the state. And while BSE cannot spread by this way, other diseases can be spread by people and equipment who move on and off the farm. So I would urge all farmers and visitors to take appropriate biosecurity precautions, and please let's respect the privacy and property of these individuals.
Finally, I just want to reiterate that the science supports our assertion that the meat is safe and nothing that we have announced today changes any of that. Over the last few days we have indicated that we are carefully considering appropriate changes to our system. Clearly that's a prudent thing to do given this new find in the United States, as well as the find in Canada in May of this year.
Today's announcement by the Secretary is clearly a result of that review. These actions do not in any way suggest that the meat produced in the current system is unsafe. For years we have had a feed ban in place. The high-risk materials from this positive cow were removed. And meat produced on the day that this positive cow was slaughtered is being recalled. Just like the meat recall, we are making these further enhancements to our system out of an abundance of caution.
I would again like to express my thanks to the owner of the index herd who has been extremely cooperative, as has the owner of the slaughter plant where this animal was slaughtered, the importers involved in the movement of these animals, and of course state officials in the state of Washington, and my friends and colleagues with the Canadian Food Inspection Agency. All of them have provided tremendous assistance as we proceed with this investigation.
And, again, my thanks to the news media. We have been working hard, or you have been working hard, to ensure accurate reporting of this very complex and rapidly evolving situation.
With that, let me provide an opportunity for my colleague from the Food Safety Inspection Service, Dr. Dan Engeljohn, to make a statement.
DR. ENGELJOHN: Thank you, Dr. DeHaven. The Secretary of Agriculture has very broad authority to be able to swiftly and effectively take the policy actions that she announced earlier today. Here at the Department of Agriculture we have the Federal Meat Inspection Act, which the Food Safety Inspection Service administers. Through the Food Safety Inspection Service - - we are the public health regulatory agency here at USDA -- that ensures that the food supply -- in particular the meat, poultry and eggs -- are in fact safe, properly labeled and, importantly, that they are fit for human consumption.
With the policies that the Secretary announced today, we will put in place, or intend to put in place, regulatory policies that will ensure that the federally-inspected facilities will have written procedures that will document how they intend to segregate these high-risk tissues from those which present lower risk or no risk at all. As the Secretary mentioned, we are going to focus on tissues that contain the potential for high infectivity. And because healthy-appearing animals may in fact have infectivity in those tissues, we'll concentrate on those tissues even in healthy-appearing animals. As the Secretary mentioned, we'll look at the small intestine and the tonsils of all cattle. We intend to prohibit the use of the brain and spinal cord and the vertebral column in cattle of older age that may have higher infectivity because of their age.
With those actions, I'd like to turn the microphone back over to you, Dr. DeHaven.
DR. DEHAVEN: All right. We'll open it up for questions. And, as was first mentioned, we'll go to our telephone bridge for the first three questions. So, operator, if we could please have the first question.
OPERATOR: Our first question is from Leah Beth Ward. Your line is now opened.
LEAH BETH WARD: Hi, Yakima Herald Republic. We have a large population here in the Yakima Valley that customarily consumes brains, spinal cord material. Are you saying that these delicacies to some of our people are now no longer going to be in the market?
DR. ENGELJOHN: Yes, I'll answer that question, from the Food Safety Inspection Service. The policies that we intend to put forward will focus on the highly-infective tissues, or the potentially highly-infective tissues from older animals. With that being said, those animals that are younger would still be able to be processed -- such as the brain and spinal cord -- could in fact be used for edible purposes.
DR. DEHAVEN: (?) So the ban in fact does -- would preclude those tissues, but only from animals over 30 months of age. And clearly that is a restriction that is based in science, and that we know from research studies that the prion, the infectious agent, typically does not appear in those tissues until well after that 30-month age mark.
Operator, next question?
OPERATOR: Our next question is from Scott Kilman. Please state your affiliation.
SCOTT KILMAN: Hi, this is Scott Kilman with Wall Street Journal. Dr. Crawford, the GAO report in 2002 made the point that the United States feed ban is far more permissive in countries that do have BSE in their cattle population -- for example, Europe. Now that the United States knows that BSE is here, and the surveillance done by the USDA suggests the possibility that it's here at the rate of one in a million, or 100 cattle in the United States, why hasn't the FDA immediately banned the use of cattle material in any feed for livestock, for any livestock?
DR. CRAWFORD: The question relates to the use of these materials in non- ruminants as I take it, and there is no scientific evidence that animals such as pigs and chickens can get the disease BSE, and therefore that action has not been taken.
DR. DEHAVEN: Next question, operator?
OPERATOR: Our next question is from Jeff St. John. Your line is now open.
JEFF ST. JOHN: Hi, this is Jeff St. John with the Tri-City Herald. I just wanted to clear up whether or not there was the determination that either the Sunnyside herd of calves or the Sunny Dene Ranch herd was definitely going to be destroyed, whether or not that was going to take place. I was wondering any details on when and where and who would be involved in that testing and destruction of those two herds.
DR. DEHAVEN: This is Dr. DeHaven. There has been no final determination at this point in terms of the disposition of those calves. We do know that on that calf feeding operation there are approximately 460 calves. We are today going through that group of animals, identifying them as according to age and gender, and then at that point we will make the appropriate determination as to which of the animals may need to be euthanized and destroyed. So no determination has been made yet in terms of which group, or all of those animals would need to be sacrificed, so we don't have at this point definitive plans exactly when and where that would take place.
Now we'll move to questions here in the room. Lady in the black sweater there, please. Please wait for the microphone.
SALLY SCHUFF: Thank you. I'm Sally Schuff with Feedstuffs. My question is for Dr. Crawford. Dr. Crawford, last year, in '02, FDA suggested or posted an advanced notice of rulemaking on possible expansions on the feed rule. Can you tell me if you will accelerate your rulemaking process on that based on the policy changes announced today?
DR. CRAWFORD: We are evaluating several possible changes in how FDA works to prevent BSE in conjunction with USDA, but we have not made a determination as to whether or not to go forward with some of those. Basically what we put forward was an advance notice of proposed rulemaking. We took comments from the public, both the scientific community, medical community, and the general public. And we have not developed a policy as to whether or not we will go forward with any of those at this point.
DR. DEHAVEN: Next question, please. Yes, sir, right here in the front row.
SHANKAR VEDANTAM: A question for you, Dr. DeHaven, Shankar Vedantam, the Washington Post. Given that you believe the animal from Washington state was more than six years old, and given that the animal that was found in Canada in May may have also been infected before the feed ban went into place, are you planning any increased surveillance for animals that are older than six to eight years old, than what you are doing right now? And in the same line of the surveillance question, Secretary Veneman talked about an increased identification program. Could you give us details of what that program would look like? Is it going to be from birth to slaughter, or is going to be from slaughter to the grocery stores, or from both to the grocery stores?
DR. DEHAVEN: Shankar, thank you for those questions. This is Dr. DeHaven. The fact that the animal that was found positive in Canada in May was older than six years old, and therefore not subject to the feed ban, and the fact that this animal now appears to have been older than six years old, and therefore not subject to the feed ban, is a significant point, as I mentioned in my opening remarks. It would suggest the fact that we are not finding other animals positive that the feed ban on both sides of the border has been very effective. And also emphasizes or confirms the emphasis in the report from the Harvard risk assessment that said that the most important thing we can do in terms of preventing the spread of the disease animal to animal is through an effective feed ban.
Our surveillance has always targeted older animals, particularly those that are showing central nervous system disorders, or those animals that are nonambulatory. And so that won't change. We do know that the disease with an incubation period typically of three to six years would suggest that that older population, especially those that have shown some clinical illness or are nonambulatory, would be our target population. I think the challenge for us now is with that population not going to slaughter we need to develop a system where we can still test those animals, whether they go to rendering, whether they be on the farm. And so we will be working very closely with those industries, and with the veterinary profession, as we come up with means where we can continue our surveillance on that high- target population.
As to the question on animal identification, we had been working actually for a year and a half with a cooperative effort involving state agencies, as well as all of the interested industries, in terms of a comprehensive animal identification system, one that would focus on an electronic ID that would be entered into an electronic system that would provide us very rapid access, and therefore the ability to very rapidly trace animals.
In terms of where that would happen or how that would happen, I think those are the kinds of details that are yet to be resolved, but we do have that plan, a basic implementation plan that we actually currently are in the process of working with the implementation teams -- a cooperative effort between state and private industry in terms of developing. And we will proceed with that from here.
Next question. Randy, please?
RANDY FABI: Randy Fabi with Reuters. I just -- well, first -- okay, I know I only get to get one shot at this, so on the product holding, is USDA testing all cattle? And, if so, after visiting the mad cow testing facility in Ames, it's a pretty dilapidated facility. So does the USDA actually have the capabilities to test more mad cow brains or just brains for mad cow disease.
DR. DEHAVEN: Randy, I'm sorry, I didn't understand the first part of your question.
RANDY FABI: In the product holding, the test and hold, is the USDA going to be testing all cattle for mad cow disease?
DR. DEHAVEN: No, let me clarify. This is Dr. DeHaven. Let me clarify what the process is intended to be. We are not doing a test-and-hold, if you will, where we would test every nonambulatory animal at slaughter. Rather, we would not allow nonambulatory animals to enter the food chain.
For those animals that do end up at a slaughter plant that then become nonambulatory, we would certainly target those for testing. And we would do a test and hold on those carcasses, so that we would in fact have a system where we could collect those samples, submit them to the laboratory, and have a rapid turnaround where we would then be able within a matter of 36 to 48 hours be able to report those results out, using the rapid test. So that is the plan at present. Again, as the Secretary mentioned, this is very preliminary information. The details and the exact mechanics of that are yet to be worked out.
We are currently doing that testing at our laboratory in Ames, Iowa, the National Veterinary Services Laboratories. We are currently in the middle of a modernization project with our colleagues in Agriculture Research Service to modernize those facilities. In fact, we have got a construction project that totals in excess of $400 million. We would anticipate with the early phases of that building, or of that facility, that are currently under construction, that we will soon be able to move the testing into those new facilities. In the meantime, I want to assure everyone that we are using state-of-the-art equipment. We have recognized experts internationally in the testing procedures. So while our facilities may not be pretty, they certainly have been effective, and they are doing world- class work in those facilities that we currently have.
Last question for inside the room here, the gentleman in the back there please?
BILL TOMSON: My name is Bill Tomson with Dow Jones. This may be a question for you, Dr. DeHaven, or Dr. Petersen -- I'm not quite sure. It's my understanding that because this cow was, as you said today and yesterday, older, predates the feed ban, and it was nonambulatory, but was not nonambulatory for central nervous system reasons, it was declared -- it was -- and so therefore the meat went on to a processing facility, and it was not condemned. So but it's not being proposed here that all animals that are older and predate the feed ban cannot be slaughtered?
DR. DEHAVEN: No, let me clarify. This is Dr. DeHaven. We are not suggesting that older animals would not be allowed to be slaughtered. Rather, we are saying nonambulatory animals will not be allowed to move to slaughter and enter into the food chain. We do want to continue to target that older population of animals for our surveillance testing, recognizing that with a disease with an incubation period of three to six years it's only natural, number one, that we target that older population; and, two, that we target animals that are showing some clinical illness, especially those that have central nervous system disorder, and especially those that would be nonambulatory.
We know that particularly in the early stages of an animal with clinical disease the clinical signs can be very subtle. It almost becomes a chicken- and-egg question: Did the animal become nonambulatory? Did she fall and break her leg, if you will, because she had CNS signs associated with BSE, or did she simply fall and might have been incubating the disease, and therefore we happen to discover the disease at the time of testing?
So I would emphasize this is a surveillance testing system intended not to definitively determine if every animal that goes to slaughter has the disease or not. The surveillance system has been focused on determining, one, if we have the disease, and at what prevalence do we have the disease? And by targeting that target population -- older animals, and particularly those older animals that are showing some clinical illness, especially central nervous system disorder, we get the most benefit, the greatest bang for your buck, if you will, in terms of the tests that we do run.
BILL TOMSON: I just want to --
MODERATOR: Bill, Bill, to be fair, I think we have to take some questions from the audio bridge now. There are well over 500 reporters there. Dr. DeHaven?
DR. DEHAVEN: Okay, operator if we could take the next question from the telephone bridge.
OPERATOR: Our next question is from Ron Haze (sp). Please state your affiliation.
RON HAZE (sp): Yes, this is Ron Haze with Clear Channel Ag Radio Networks. I wanted to know exactly what your plans are for those 81 animals as you continue your trace-back efforts. Do you intend to, as you identify them, to go ahead and slaughter them and test them for BSE?
DR. DEHAVEN: This is Dr. DeHaven. Thank you for the question. Our job, one, is to find those 81 animals. We want to do our best through the records that are available to us to find out, one, did they all come into the United States? Certainly we had 82 animals, the positive animal and 81 others that were permitted, that had all the necessary paperwork that would allow them to enter the United States. The first job is to find out which ones did come and where are they currently located.
We also know from the disease that the animals that would be of greatest concern would be what we call the birth cohorts: the animals that were in the herd at the same time as the positive animal that might have been exposed to the same feed source and therefore might also be harboring the disease. Clearly those animals we would want to identify for slaughter or for -- excuse me, for sacrificing and for testing. I think it would be premature to make a determination at this point as to what might happen with the other animals -- other animals that might have been in the herd, but clearly would not have been of the appropriate age to have been exposed to the same feed sources. Although I think immediately what we would do is the same thing we've done with the other animals that are of concern, and that is to put a hold or a quarantine order on them, find as much as we can out about their history, and then make a determination as to whether or not they need to be sacrificed and tested.
Next question, operator, please?
OPERATOR: Our next question is from Steve Mitchell. Please state your affiliation.
STEVE MITCHELL: Yes, this is Steve Mitchell with United Press International. You are talking about a turnaround for some of these animals that will be tested and then put on hold of 36 to 48 hours. So does that mean you are moving towards going to be implementing the so-called rapid tests that are in use in Europe?
DR. DEHAVEN: This is Dr. DeHaven. Indeed we are talking about using one of several rapid tests. There are several that have been used in Europe, as well as other parts of the world. And to have that kind of turnaround we would definitely be going to one or potentially more of those tests. Theoretically the plan as we have developed this thus far would suggest that we would obtain the samples, whether they be at rendering plants or on the farm, potentially those animals that go to slaughter and become nonambulatory after they arrive at slaughter. Those samples then would be sent by overnight express to our laboratory, to be received in the neighborhood of 10:00 the next morning. They would be set up to run in the laboratory, and have results out that afternoon. And if everything goes perfectly, and assuming negative results, we would report those results out the afternoon or early evening the day after they were collected.
So we are getting ourselves some flexibility there, recognizing that some samples might not be obtained in time to be ready for overnight express pick-up that day, so there might be a delay there. There's always the possibility that there could be complications in the laboratory. And clearly for those animals that would test positive on one of these screening tests, we would continue to hold product or carcasses in those situations pending testing on the other tests. So indeed for that kind of turnaround we would contemplate going to one of the rapid screening tests.
STEVE MITCHELL: Do you have any --
DR. DEHAVEN: I wonder if we could please go on to the next question.
OPERATOR: Our next question is from Andy Dworkin. Please state your affiliation.
ANDY DWORKIN: Yes, with the Oregonian. I was wondering, you guys have talked a lot in the last couple of days about the very open border in terms of both live animal trade and feed trade and other things between U.S. and Canada. Given that, why were some of these changes not made on the confirmation of Canada's first case, or at least for instance when Canada made its changes dealing with SRMs, why did the U.S. not do the same thing at that point in time, instead of waiting for a U.S. case to be found?
DR. DEHAVEN: This is Dr. DeHaven. I would answer that by saying that we have had, and continue to have, a very effective system in this country, and we know that our colleagues in Canada, even before they made the changes following their find of BSE in May of this year, had a very effective system. The current case I think is evidence of that, the fact that our surveillance system picked up this animal, the fact that we had a feed ban in place that has every appearance of having stopped any animal-to- animal spread through the feed, the fact that the risk materials from this particular animal didn't enter into the food chain. And just as a precautionary measure we're doing the recall. So, again, I think, one, we wanted to ensure that any steps that we take would be well thought out, well planned. We have had the benefit of the time since May to contemplate some of the changes that the Secretary announced today. But, in the meantime, we felt good about the system that we had in place. And, again, this situation would suggest that we had a very good reason to be confident in the system that we had in place. Having said that, we are taking these actions out of an abundance of caution, and not because we think that the system that has been in place has put our livestock or our public in jeopardy.
Last question from the telephone bridge, operator?
OPERATOR: The next question is from Mike McGuiness (sp). Please state your affiliation.
MIKE MCGUINESS (sp): Yes, I'm with Data Transmission Network. My question is we are getting reports that the U.S. consumer is willing to continue to buy beef. But has the export market that has been lost, does that come back in 2004 or not? And I'm not sure who wants to answer this.
DR. DEHAVEN: This is Dr. DeHaven, and I'll make an initial attempt at that, and we also have here with us Dr. Keith Collins, our chief economist within the department, and we would certainly provide Keith an opportunity to make any additional comments.
One of our concerns relative to the whole BSE situation internationally has been the fact that the response to a country reporting a case of BSE has been largely a response not based on the science, not based on international standards that are based on the science, but rather has been largely in response to public concern and overreaction. But, admittedly, the United States has been part of that problem, but we have been working very actively, especially since the find of the case in Canada in May of this year, to change that international overreaction based on public perception and not based on the science.
Indeed, as we have sent several delegations to our trading partners to discuss the trade implications of this disease, as they have sent delegations to the United States, one of our focal points, I would even suggest that our main focal point, has been to emphasize the science, to emphasize that we have very good science-based international standards that we collectively need to start implementing, and as has been pointed out time and again with this recent situation, not punish countries who do the right thing by having in place good systems and then get punished by reporting a case, recognizing that there are commodities that can safely be traded from countries even after they have the disease, and even from countries that have moderate or high prevalence of the disease. With that, Keith, anything to add to this?
Okay. So we'll take two more questions from here in the room, please. Yes, sir?
SETH BORENSTEIN: Seth Borenstein, Knight Ridder Newspapers. Not knowing my cattle as much as I should, in terms of the specified risk material that you are talking about in over 30-month cattle, one, is there any guesstimate on how many pounds or tons of cattle are we talking about a year this is affecting? And can you tell us sort of -- obviously people eat brains as a delicacy, but some of this may get in hot dogs -- you know, what cut around spinal cord that gets affected?
DR. DEHAVEN: For that we will defer to our colleagues in Food Safety Inspection Service. Dr. Engeljohn?
DR. ENGELJOHN: Yes, this is Dr. Engeljohn. The question that you had about the appearance of brain and other tissues in hot dogs and things such as that, we presently have requirements that many of the sausage items that you can purchase may in fact have variety meats in them or byproducts in them, that many items that are labeled as meat products have or would not have items such as brain in them. There are specialty items that may in fact have brain in them, but for the most part they are not produced here in this country. But the general sausage product would not have brain in it.
And your first question? I'm sorry?
SETH BORENSTEIN: The amount of tons of cattle --
DR. ENGELJOHN: Yes, I can't give you an exact figure for that. I can give you a general proportion of the number of animals that are in fact over 30 months of age. And in this country roughly 20 percent of the cattle that are slaughtered are over the age of 30 months.
MARK SHERMAN: Mark Sherman with the Associated Press. How many of the 81 cattle have you found, including how many of those are in the same index herd? And you said you would be testing those. Do you also plan to test the recalled meat just out of the same abundance of caution that you've spoken of so frequently?
DR. DEHAVEN: This is Dr. DeHaven. Let me address the question with regard to the 81 cattle, and then we can talk about testing, or let FSIS talk about the testing of the recalled meat. As far as the 81 cattle, the trace back of those animals is going to I think come to fruition, or our effort in that regard is going to come to fruition significantly in the next couple of days. We have a lot of source documents to tell us which animals were likely to have come and where they might have gone from there. Quite honestly, one of the limiting factors is the animal identification issue, and not all of the animals had the same kind of ID that the infected cow did have. So I can't tell you at this point exactly how many of those 81 animals we have traced out, or exactly where they are, other than to say that a number of those animals in fact have been found on the index herd, the premises where this positive cow left immediately before she went to slaughter.
On the testing of the meat, let me first explain that the testing that we have for this disease requires brain tissue. All of the tests require tissue from a very specific portion of the brain to do our testing to be relevant. So there is no test that we have available -- and I'll allow my FSIS colleagues to correct me -- but so far as I know no test that's available that we can apply to meat that would be readily available. And, here again, I think it's important that we emphasize scientifically that's not one of the risk tissues. That's not one of the parts of the animal where the infectious agent, the prion, goes. So even if we get that meat back, there wouldn't be anything there to look for in terms of infectious agent.
Anything to add to that?
DR. ENGELJOHN: No.
DR. DEHAVEN: Okay. And I understand that we are going to provide the opportunity for one absolutely final last question today.
DAN GOLDSTEIN: Thank you, Dr. DeHaven. Dan Goldstein, with Bloomberg News. I'm not a math whiz, but just looking at the numbers that Secretary Veneman provided, with between 50,000 and 100,000 downer cattle slaughtered every year, with the ban going into effect ostensibly soon after this announcement, that still would leave about 1,000 to 3,000 downer cattle essentially in the system after the December 23rd discovery of BSE. And obviously not every downer animal is diseased or has symptoms of BSE. But do you think it was wise to wait a week to do that? Why not just simply make that decision then a week ago, and get these potentially loaded guns out of the food chain?
DR. DEHAVEN: This is Dr. DeHaven. I'll take an attempt at that, and then provide FSIS colleagues an opportunity to comment as well.
We are again emphasizing operating out of an abundance of caution. So while there has been a week gone by since we confirmed the positive, or had presumptive positive results on this particular animal, I want to emphasize it's not as if we haven't had an effective system in place during that week's time. And I would also suggest that given the magnitude of the changes that the Secretary announced today, making those changes just one week after we received presumptive positive results is extremely fast action.
In the meantime, again I would emphasize that for those animals that might be in the system, they would have been subject to the same safeguards that have been in place and have protected us so well thus far. We have had a feed ban in place that certainly for those animals under six months of age should have kept them from becoming infected. Specified risk materials from this positive cow were kept out of the food system. And, again, the recall on this meat was done out of an abundance of caution and not because it represents a real risk to the public health.
So it's only prudent that we take these additional steps, given again the find a week ago in the United States, as well as taking into account that we have had an integrated market with Canada, and their find in May. But in the meantime, we have had an effective system in place and nothing that we are doing today -- none of the actions that we are doing today are being or should be taken as a suggestion that anything but safe beef has been made available in the United States up till now.
DR. PETERSEN: Dr. Petersen, FSIS. It's difficult to predict when these nonambulatory animals will come to a particular slaughter facility. And to suggest that by waiting we would somehow get caught up is really not consistent with how they come to market. If it's a recent injury, for example, they're going to come immediately, and that's a situation that I cannot predict. An animal that has perhaps been ill, it's a determination made on the particular clinical status of that animal to decide if they're going to get better, or should I go ahead and proceed to slaughter. And so those are kind of day-by-day decisions, so at some point we have to decide we're going to go forward, and we've decided to go forward, as the Secretary has indicated.
DAN GOLDSTEIN: Can't predict how many downer cattle went into the system prior to this?
DR. PETERSEN: Well, we know the trends over time, but it's not a daily trend or necessarily a weekly trend. They come and go, but we have seen relative consistency in the numbers over the years, and those are the numbers the Secretary indicated.
DR. DEHAVEN: Thank you for your questions. We appreciate the continued interest and responsible reporting. And, Ed, any update on the next one?
MODERATOR: Yes, the next briefing, we'll make an announcement. We'll keep you posted. Transcripts available on the web shortly thereafter this briefing. And for follow-up media call 202-720-4623.
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TSS
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Subject: Re: BSE USA TRANSCRIPT UPDATE DEC. 30, 2003 From: TSS <flounder@WT.NET> Reply To: Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> Date: Tue, 30 Dec 2003 18:18:51 -0600 Content-Type: text/plain Parts/Attachments: text/plain (118 lines) ######## Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> #########
Greetings List Members,
I tried to ask a question during the question period of this meeting today, but with 500 journalist on the line they did not seem to interested in getting a question from anyone connected to CJD. my question is;
In speaking of Amplifications of TSEs in the USA, we have not only this 1st _documented_ case of BSE (then you might add in what Marsh found in 1988 about an unrecognized or ignored TSE in cattle), but then you must add in all the CWD infected deer/elk, and you must add in all the Scrapie, all of which transmits to primates, all of which has been rendered and fed to animals for human consumption for years, and with the new findings from Asante/Collinge et al, that BSE transmission to the 129-methionine genotype can lead to an alternative phenotype which is indistinguishable from type 2 PrPsc, the commonest sporadic CJD, with all this now, will CDC/NIH make ALL PHENOTYPES OF CJD REPORTABLE NATIONALLY? Will you put into place an extensive CJD surveillance program as in the U.K.?
TSS
Quoting TSS <flounder@wt.net>:
> ######## Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> > ######### > > TRANSCRIPT > of > Agriculture Secretary Ann M. Veneman Announcing Additional Protection > Measures to Guard Against BSE > Washington DC > Dec. 30, 2003 > > SEC. ANN VENEMAN (USDA): Good afternoon, and thank you all for being here > with us once again today. I want to acknowledge some of the members of our > team who are here with us today. In the front row are Dr. Elsa Murano, our > under secretary for food safety here at USDA. Bill Hawks, who's our under > secretary for marketing and regulatory programs. Keith Collins, who's our > chief economist. And I want to give a special thanks to those who have been > providing you with the regular briefings at the technical briefings that > the media has been getting every day. And I think their information has > been very helpful and vital. > > Dr. Ron DeHaven has been a part of that. He's become someone you see often, > our chief veterinarian here at USDA; along with Dr. Ken Petersen of the > Food Safety and Inspection Service, and Dr. Sundlof from FDA who is not > here with us today, but the deputy director of FDA, Dr. Les Crawford, is > here. The deputy commissioner, Dr. Crawford, is here with us today. > > Also joining the technical briefing today will be Dr. Dan Engeljohn of the > Food Safety and Inspection Service. > > I'm going to make an opening statement this afternoon, and after that time > there's going to be a short break. They're going to reset the stage for the > regular technical briefing with our experts that will allow them to answer > specific technical questions and update you on the investigation itself. > > At the very beginning of that briefing, the technical briefing, Dr. > Crawford will provide a presentation on actions by the FDA that are related > to the announcements that we're making today. > > I was just one week ago today that I stood here before you hours after > learning of a single presumptive positive case of BSE, or what we call ‘Mad > Cow Disease,’ in Washington state, and I stood here to make that > announcement. In that short period of time, exhaustive efforts have been > made, countless hours have been spent, to investigate this finding. > > My thanks to everyone who has assisted in these efforts including our > partners at the federal, state and industry and international level > especially all of the hosts -- and many did this -- they altered their > holiday plans significantly to ensure our swift and effective response to > this incident. > > While this investigation is still in its early phases we've made a lot of > progress in both the trace-back and the trace-forward from the indexed cow. > Our investigative team on the ground, working with the state of Washington > and Canadian authorities, has done an outstanding job. I especially want to > thank Canadian officials for their excellent cooperation throughout this > process. > > As our technical experts have informed you, our main line of inquiry > indicates that the cow slaughtered in Washington state and found to have > BSE may have been born in Canada. We know that higher risk materials from > the animal did not--did not--enter the food chain. Recalls of meat and > product from this cow and others slaughtered at the same facility on the > same day have been implemented, and we continue to be guided by an > abundance of caution. > > But I will stress again that our food supply and the public health remain > safe. > > Judging by recent market actions, consumer confidence and demand for beef > are still relatively strong. We have initiated discussions with our trading > partners which are ongoing to assure them of the actions that we are > taking, and continue to take, to investigate this finding. Our USDA trade > team has been in Tokyo and South Korea. Our goal is to see trade resume as > quickly as possible. > > We are also making information available to the public as soon as it > becomes known to us. As you know, we have been updating on a daily basis > and our updates are virtually up to the minute. They are webcast across the > country and around the world and our materials are posted on our USDA > website at www.usda.gov. > > Sometimes we will tell you that this information is in a preliminary form, > but we make it available quickly in the public interest. > > For more than a decade the United States has had in place an aggressive > surveillance detection and response program for BSE. That program has > evolved over time as our understanding of BSE has changed. But
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Subject: WASHINGTON FIRM RECALLS BEEF PRODUCTS FOLLOWING PRESUMPTIVE BSE DETERMINATION From: "Terry S. Singeltary Sr." <flounder@WT.NET> Reply To: Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> Date: Wed, 24 Dec 2003 07:45:32 -0600 Content-Type: text/plain Parts/Attachments: text/plain (60 lines) ######## Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> #########
CLASS II RECALL Congressional and Public Affairs
HEALTH RISK: LOW Steven Cohen (202) 720-9113
FSIS-RC-067-2003
WASHINGTON FIRM RECALLS BEEF PRODUCTS FOLLOWING PRESUMPTIVE BSE DETERMINATION
WASHINGTON, Dec. 23, 2003 - Verns Moses Lake Meats, a Moses Lake, Wash., establishment, is voluntarily recalling approximately 10,410 pounds of raw beef that may have been exposed to tissues containing the infectious agent that causes bovine spongiform encephalopathy (BSE), the U.S. Department of Agriculture's Food Safety and Inspection Service (FSIS) announced today.
The beef subject to this Class II recall was produced on Dec. 9 and was shipped to several establishments where it was further processed. FSIS is continuing its investigation to ensure that all distribution of the beef products is correctly identified.
FSIS' designation of this recall as Class II is due to the extremely low likelihood that the beef being recalled contains the infectious agent that causes BSE. According to scientific evidence, the tissues of highest infectivity are the brain, spinal cord, and distal ileum, which were removed from the rest of the carcass at slaughter. Therefore, the meat produced were cuts that would not be expected to be infected or have an adverse public health impact, but are being recalled out of an abundance of caution.
Media and consumers with questions about the recall should contact Tom Ellestad, company secretary, at 509-765-4182.
Consumers with other food safety questions can phone the toll-free USDA Meat and Poultry Hotline at 1-888-MPHotline. The hotline is available in English and Spanish and can be reached from 10 a.m. to 4 p.m. (Eastern Time), Monday through Friday. Recorded food safety messages are available 24 hours a day.
BSE is a progressive neurological disease among cattle. It belongs to a family of diseases known as transmissible spongiform encephalopathies. Also included in that family of illnesses is the human disease, variant Creutzfeldt-Jakob Disease (vCJD), which is believed to be caused by eating neural tissue, such as brain and spinal cord, from BSE-affected cattle.
NOTE: Access news releases and other information at the FSIS Web site at <http://www.fsis.usda.gov/> http://www.fsis.usda.gov
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Subject: Re: Transcript of News Conference with Agriculture Secretary Ann M. Veneman on BSE From: Roland Heynkes <roland@HEYNKES.DE> Reply To: Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> Date: Wed, 24 Dec 2003 09:26:22 +0100 Content-Type: text/plain Parts/Attachments: text/plain (117 lines) ######## Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> #########
Dear Terry,
> SECRETARY VENEMAN: > ...
> As part of our response plan that farm has been quarantined. After the > animal was slaughtered meat was sent for processing to Midway Meats in > Washington State. USDA's Food Safety Inspection Service is working > quickly to accurately determine the final disposition of the products > from the animal. > > Even though the risks to human health is minimal based on current > evidence, we will take all appropriate actions out of an abundance > of caution. > > Since 1990 the U.S. Department of Agriculture has had an aggressive > surveillance program in place to ensure detection and a swift > response in the event of the introduction of BSE in this country. > thanks a lot for this jokes. I hope for you that this first case of BSE in the USA will be confirmed, because only a confirmed case of BSE in the USA will be able to stop your politicians spreading their stupid statements and lies. May be, that you now will get a useful testing program and real measures against BSE.
> The safety of our food supply and public health are high priorities > of this Administration and high priorities of USDA. In the last > year we have tested 20,526 head of cattle for BSE, which is triple > the level of the previous year of 2002. The presumptive positive > today is a result of our aggressive surveillance program. This is > a clear indication that our surveillance and detection program is > working. > After this statement there is only one sensible reaction possible. She should lose her job immediately.
> SECRETARY VENEMAN: Well, I would certainly hope that people will > remain confident in the food supply. As I said, we in some ways > had some experience with this because of the one find in Canada. > What we found because of the actions that were taken both in > Canada and in the United States with the case earlier this year > is that demand for beef did not diminish partly because we believe > the people in North America know that we have the strongest food > safety systems in the world. We have the protections in place, > and again, I, personally, do not hesitate to recommend to anyone > that beef is absolutely safe to eat. > Do you think that people in the USA really believe this nonsense?
> SECRETARY VENEMAN: That's--that, Randy, is what we're trying > to identify at this point. We do believe that the product from > the animal went to two further processing plants. This plant > was a very small plant. It just slaughters a few animals, and > our current understanding, and again it's very preliminary, > is that that product did go to further processing plants. > Yes of course it is much too early to know such things after only 3 weeks. ;) But in Germany we would know it after 3 days and meat from positive tested cattle has no chance to go into the food supply.
> But again, one thing that is important to remember is that > muscle cuts of meats have almost no risk. In fact, as far as > the science is concerned, I know of no science to show that > you can transmit BSE from muscle cuts of meat. So the fact > that it's gone to further processing is not significant in > terms of human health. > There are so many pretty well informed TSE scientists in the USA. Why is it so difficult for US politicians to become informed correctly?
> DR. MURANO: Let me first reiterate what the Secretary just said. You > should know that the tissues that are the infectious tissues from an > animal that has BSE, that is the central nervous system tissues, the > brains, spinal cord and so forth, of this animal did not enter the > food supply. Those tissues to rendering. So they did not enter the > food supply. That's very important to know. > More important to know would be the exact parameters of rendering in the USA and what happens with the MBM and animal fat after rendering.
> Again, the muscle cuts are where there is virtually no risk > of BSE. > Is Dr. Murano really so badly informed or does he prefer to lie? Is it possible that he never heard about the consequences of using a captive bolt? Is it really possible that he does not know the results of the Prusiner group? And do you really use slaughtering techniques in the USA, which avoid contamination of meat with spinal cord material?
> DR. MURANO: > The scientific community believes that there is no evidence to > demonstrate that muscle cuts or whole muscle meats that come > from animals that are infected with mad cow disease agent > themselves the meat itself is effective to human beings. > I am part of this scientific community and I see several lines of evidence for a TSE risk with meat from BSE cows. This guy should have a look into the scientific literature.
kind regards
Roland
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Subject: Transcript of News Conference with Agriculture Secretary Ann M. Veneman on BSE From: "Terry S. Singeltary Sr." <flounder@WT.NET> Reply To: Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> Date: Tue, 23 Dec 2003 22:54:19 -0600 Content-Type: text/plain Parts/Attachments: text/plain (381 lines) ######## Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> #########
Transcript of News Conference with Agriculture Secretary Ann M. Veneman on BSE
Tuesday, December 23, 2003 Washington D.C.
See attachment for full story
USDA News oc.news@usda.gov 202 720-9035
Release no. 0433.03 of News Conference with Agriculture Secretary Ann M. Veneman on BSE
Tuesday, December 23, 2003 Washington D.C.
SECRETARY VENEMAN: We are here this afternoon on such very short notice. Joining me today are Bill Hawks, USDA's Under Secretary for Marketing and Regulatory programs; Dr. Elsa Murano, our Under Secretary for Food Safety; and Dr. Ron DeHaven, our Deputy Administrator for Veterinary Services at the Animal Plant Health Inspection Service and our chief veterinarian here at USDA.
They will assist me in answering any questions that you have.
Today we received word from USDA's National Veterinary Services Laboratories in Iowa that a single Holstein cow from Washington State has tested as presumptive positive for BSE or what is widely known as mad cow disease.
Despite this finding we remain confident in the safety of our food supply. The risk to human health from BSE is extremely low. The animal tested was a downer cow or nonambulatory at the time of slaughter and was identified as part of USDA's targeted surveillance program.
The sample was taken on December 9th. It was tested and retested at our Ames facility using two tests including immuno-histo-chemistry, which is recognized as the gold standard for the detection of BSE by the World Health Organization and OIE, the Organization of International Epizootics.
A sample from this animal is being flown on a military aircraft to the central veterinary laboratory in Weybridge, England in order to confirm this finding. Our traceback indicates that the animal comes from a farm in Mabton, Washington, about 40 miles southeast of Yakima, Washington.
As part of our response plan that farm has been quarantined. After the animal was slaughtered meat was sent for processing to Midway Meats in Washington State. USDA's Food Safety Inspection Service is working quickly to accurately determine the final disposition of the products from the animal.
Even though the risks to human health is minimal based on current evidence, we will take all appropriate actions out of an abundance of caution.
-more- Since 1990 the U.S. Department of Agriculture has had an aggressive surveillance program in place to ensure detection and a swift response in the event of the introduction of BSE in this country. As part of that program we developed a response plan to be used if BSE is identified in the United States.
While this is a presumptive finding, we have activated that response plan today. We are making the appropriate notifications and confirmations under the plan and start-up activities are beginning.
I have been in contact with Secretary Ridge and I would emphasize that based on the information available this incident is not terrorist related nor is it related in any way to our nation's heightened alert status. I cannot stress this point strongly enough.
The safety of our food supply and public health are high priorities of this Administration and high priorities of USDA. In the last year we have tested 20,526 head of cattle for BSE, which is triple the level of the previous year of 2002. The presumptive positive today is a result of our aggressive surveillance program. This is a clear indication that our surveillance and detection program is working.
USDA has been training and planning for several years in case this situation presented itself. We continue to protect the U.S. food supply and the public health and safeguard American agriculture.
In October we announced findings from the Harvard Center for Risk Analysis that found that even if an infected animal were introduced into the U.S. animal agriculture system, the risk of spreading is low based on the safeguards and controls we have already put in place.
As part of our response to this situation we will provide daily briefings to update the public on the status. We will continue to provide you all of the information that we possibly can and do so as quickly as possible.
We have released this finding even before final confirmation in the U.K. because of our confidence in the testing that has already been carried out, and in the interest of protecting the food supply and public health. Information is available on our web site at www.usda.gov, and we will be updating that information frequently.
We will also have regularly recorded updates for you, and you may call a toll-free number, 1-866-4USDA-COM.
While this incident would represent the first finding of BSE in the United States, we have worked hard to ensure that our response is swift and effective. We will continue to work with partners such as the Food and Drug Administration and the Department of Homeland Security to protect our food supply and the public health.
At this time of year many Americans are making plans for the holidays and for food. We see no need for people to alter those plans or their eating habits or to do anything but have a happy and healthy holiday season. I plan to serve beef for my Christmas dinner. And we remain confident in the safety of our food supply.
I want to thank you all again for being here on this late hour and on such short notice. But we did feel it was important to update you on this important situation. Thank you. And we will be glad to take your questions. -more- PARTICIPANT: Danielson, Bloomberg News. Is there any connection with this finding to the incident in Alberta, Canada? That perhaps that cow came down from Canada in any way?
SECRETARY VENEMAN: It is way too early to tell, but I would think that the situation of trying to put those two incidences together would be doubtful, primarily because they're different kinds of animals. This was a Holstein cow.
MR. FAUVY: Randy Fabi with Reuters. Have you alerted any of your trading partners to this incidence, and, if so, have they--have any countries taken action against, close their borders to U.S. beef exports?
SECRETARY VENEMAN: Again, it's very early, Randy. We have--we are in the process of notifying a whole range of people at this point, including our trading partners, and I can't at this point anticipate what they may do in response to this announcement.
MR. SALANTE: Jonathan Salante with the Associated Press. What steps, if any, are you specifically taking to prevent the spread of this disease, and what assurances do we have that the beef is--that the other beef is not contaminated?
SECRETARY VENEMAN: Well, I think it's very important to recognize that this disease does not spread easily. One of the things that people are very confused about, and I found it as we went through situation when Canada had a single case of BSE is a lot of times people don't understand that this is not foot and mouth disease; it's not that highly contagious disease that you often see spread so quickly as you did in the U.K. at the beginning of 2001. So it's important to make that distinction.
We have been taking steps since 1990 to protect our beef supplies from this disease. We implemented a feed ban; we have required the removal of any kind of risk materials from an animal like this one, a downer animal, and we have a whole series of actions that have been taken to reduce, substantially, the risk to public health from this disease if it ever were found. And that's why we continue to believe that this finding, while unfortunate, does not pose any kind of significant risk to the human food chain.
MS. NAGEL: Sara Nagel, Fox News. Can you tell us who this will affect, what the chances that it could become more widespread here in the U.S. are?
SECRETARY VENEMAN: Well, I think at this point it's hard to tell, but again, the unfortunate find of a single case in Canada earlier this year gave us some experience of the type of investigation that we now have to do. We did not know when the Canada investigation started, whether or not there would be more cases or whether or not it would be an isolated case. Indeed, after several months of checking into the situation, it turned out to be an isolated case.
It is too early at this point to say whether or not this will be an isolated case. What I can tell you that we're doing is we're going back to the farm where this cow came from. We will be doing a complete investigation on farm and tracing the animal back to its origin.
MR. RIVALL: Sorry, Audy Rivall, ABC News. Tell us a little bit more about this particular farm. What do you plan to do about the other animals? Are you saying that it was quarantined, and you're--and it's going to test the other--the cows there?
-more-
And also, how concerned are you that the public outcry of people here on the street hear "mad cow" there could be some sort of hysteria associated with that? How concerned are you about that? And, certainly, you must agree that it's a possibility, are you not?
SECRETARY VENEMAN: Well, I would certainly hope that people will remain confident in the food supply. As I said, we in some ways had some experience with this because of the one find in Canada. What we found because of the actions that were taken both in Canada and in the United States with the case earlier this year is that demand for beef did not diminish partly because we believe the people in North America know that we have the strongest food safety systems in the world. We have the protections in place, and again, I, personally, do not hesitate to recommend to anyone that beef is absolutely safe to eat.
As to the farm, your other question, again we're in the very early stages of the investigation. We have a complete book of protocols that we're following with regard to how we would deal if we ever had an outbreak of BSE. We're following all those steps, including we've already located the farm, and that farm will be quarantined and an investigation will begin. Again, this was very recent breaking news so we're taking all steps that we can, and we will be continuing to update you, as we indicated.
MR. DUNN: I'm (inaudible) Dunn from the Washington Post. Could you give us some sense of narrative about the farm? Why was this particular farm being studied? Was this animal significant in some way that you did tests on this animal? How many other animals were there on this farm? How many other farms are approximate to this farm?
SECRETARY VENEMAN: Okay, first of all, the test was not done on the farm. The test was done when the animal was presented at the slaughter facility, and it is our standard operating procedure that what they call downer animals will be tested if they--if they come to the slaughter facility as a downer animal.
The farm has been identified since we got the test results back from the animal. We've then gone to the plant just this afternoon, found where the animal came from, and that's where the investigation will begin in terms of looking at whether or not there is any other impact on cows on that farm. But at this point the information with regard to the farm and the surrounding areas is still pretty preliminary. I don't have that information at this point.
MR. FABI: Randy Fabi with Reuters. I'm just--what is the likelihood that any of this cow made it into the food supply? I know that you have contacted the meat suppliers. Is there a recall underway?
SECRETARY VENEMAN: That's--that, Randy, is what we're trying to identify at this point. We do believe that the product from the animal went to two further processing plants. This plant was a very small plant. It just slaughters a few animals, and our current understanding, and again it's very preliminary, is that that product did go to further processing plants. But again, one thing that is important to remember is that muscle cuts of meats have almost no risk. In fact, as far as the science is concerned, I know of no science to show that you can transmit BSE from muscle cuts of meat. So the fact that it's gone to further processing is not significant in terms of human health. But we are doing the trace backs. We are looking at trace forwards, where did the product go. And we will take appropriate actions as we make the determinations as to where the product is and what has happened to it.
-more-
I think we -- I mentioned one of them, but there is actually two.
DR. MURANO: Let me first reiterate what the Secretary just said. You should know that the tissues that are the infectious tissues from an animal that has BSE, that is the central nervous system tissues, the brains, spinal cord and so forth, of this animal did not enter the food supply. Those tissues to rendering. So they did not enter the food supply. That's very important to know.
Now, the muscles cuts, as the Secretary said, went from the slaughter facility to another facility that did the deboning and that facility is Midway Meats, as the Secretary mentioned. Then from there we believe that it went to two other facilities. One is called Willamette and the second one is called Interstate Meat, both in Washington State.
Again, the muscle cuts are where there is virtually no risk of BSE. The material, the brain, spinal cord, distal ileum, which is where the BSE agent resides, those materials did not enter the food supply.
PARTICIPANT: (inaudible) with CNN. You said the health risks are minimal but what if someone did eat meat contaminated with this. What are the health risks?
SECRETARY VENEMAN: Well, again as Dr. Murano just indicated, there is virtually no chance that the meat has been contaminated and the agents, that would be the high risk agents in any animal have been removed from this particular animal so we really dont believe that there iswe believe that the risk of any kind of human health effect is extremely low.
PARTICIPANT: Yes, but what if you find another animal on the farm that is contaminated.
DR. MURANO: Well, you should know that the agent that causes mad cow disease as I said earlier resides mainly in those tissues that I mentioned, the brain, spinal cord, distal ileum, which were removed from this animal and sent to rendering so they were not in the food supply. The scientific community believes that there is no evidence to demonstrate that muscle cuts or whole muscle meats that come from animals that are infected with mad cow disease agent themselvesthe meat itself is effective to human beings. There is no evidence to show that and that is as far as we can state that. Its a good thing obviously that the infectious materials from this animal were removed and sent to rendering which is something that we do as standard practice on these downer animals that are tested by APHIS.
PARTICIPANT: Christopher (inaudible) with Reuters. I was wondering if you could tell us a little bit more about what is going to be happening in the U.K. specifically and what your timetable is for getting final results on those tests?
SECRETARY VENEMAN: Well, as I indicated, the sample is being flown to a laboratory in the U.K. which is one of the world's best laboratories for analyzing BSE. And that will take a number of days, probably -- probably three to five days to get final results on that sample.
So, again, we are getting it there as quickly as we possibly can. But the results that we have been able to confirm in our own laboratories have been something that we felt that we ought to take action on.
PARTICIPANT: You have said that you have quarantined the farm itself. Have you imposed any kind of quarantine on the slaughterhouse at the three facilities that are downstream?
-more- SECRETARY VENEMAN: Well, we have people that have gone into the slaughterhouse as well as are going into the downstream facilities, starting to review records. But we will be doing a complete review and investigation of the entire food chain where this animal might have been transferred during the process.
Well, I don't think you would normally impose quarantine on a plant. You impose quarantine on a farm. But we will be doing an investigation of the plants to determine exactly where the product might have gone.
[Pause.]
SECRETARY VENEMAN: Well, we are taking all appropriate action. We first need to identify where the product went before we can take action. I am not saying that we are not taking action. As I said in my opening remarks, we are going to take all appropriate actions based upon the investigation.
I know that the tendency is to want to know all the answers right away. And we decided that we couldn't wait to give the public the information about this situation, but we certainly don't have all answers today. And that is why we will be continuing to update both our web site and the call in number that I indicated earlier and we will be conducting daily press briefings to update you on what is going on.
Thank you.
# ==============================================
Docket Management Docket: 02N-0273 - Substances Prohibited From Use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed Comment Number: EC -10 Accepted - Volume 2
http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html
PART 2
http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be09.html
Asante/Collinge et al, 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;
http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm
PDF]Freas, William TSS SUBMISSION File Format: PDF/Adobe Acrobat - Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary Sr. [flounder@wt.net] Monday, January 08,200l 3:03 PM freas ...
http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf
my submission to FDA of 2001 was removed in 2019 on bse in the USA, here it is;
TSS
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THURSDAY, JANUARY 17, 2013
CANADA MBM LIVE CATTLE BSE TSE PRION TO USA
Date: Sat, 14 Jun 2003 02:23:12 +0200
Reply-To: Bovine Spongiform Encephalopathy
Sender: Bovine Spongiform Encephalopathy
From: Karin.Irgens@DYREHELSETILSYNET.NO
Subject: BSE Canada USA
######## Bovine Spongiform Encephalopathy #########
Hello all
Terry Singletary has provided the official US import and export statistics for the USA in 2002 and the first 3 months of 2003, for live cattle and MBM (meat and bone meal)
I have tried to figure out how many 'risk units' (external challenge) the USA has imported from Canada during 2002-2003.
The GBR (geographical BSE risk) assessment-method and criteria of the EU SSC are described in detail in the latest GBR opinion of the EU Scientific Sterring Committee.
snip...see full text;
Subject: risk from US exports of cattle and MBM From: Karin.Irgens@DYREHELSETILSYNET.NO Reply To: Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> Date: Sat, 14 Jun 2003 23:10:28 +0200 Content-Type: text/plain Parts/Attachments: text/plain (308 lines) ######## Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> #########
Hello all
Terry has now provided US export figures for 'breeding cattle' for 1999, in addition to export statistics for 2002 that he had already provided. On the basis of US export statistics for 1999 and 2002 for live cattle for breeding, and US export statistics for 2002 and 2003 for meat and bone meal (code 23.01.1000), we could try to calculate external challenge from the USA to importing countries. However, _we dont really know_ the situations in most of these importing countries (at least I dont know...). If live cattle from a BSE-infected country are imported to a country that does not have a rendering system for slaughter waste, there would be no resulting BSE-risk to the importing country. If an importing country decides to exclude waste from imported cattle from rendering, or to BSE-test all imported cattle slaughtered at > 30 months age, the risk will be considerably reduced. Canadian and US cattle exported for breeding in recent years may still be alive in the importing countries, and may now be excluded from rendering.
We dont know if all US exports of meat and bone meal "code 23.01.1000" were "mammalian" MBM or if some of it was poultry meal or MBM derived only from pigs. The same code 23.01.1000 can be used for registrations of exports/imports of mammalian MBM and poultry meal. The EU SSC does not consider poultry meal as a BSE-risk, but each country must prove how much of the imports really were poultry meal. It is possible that some countries, for religious reasons, would accept only MBM not derived from pig waste. If such countries imported only poultry meal, the risk would be very low.
We dont know if all registered US exports were correctly coded. It is possible and probable that some exports were in fact other products that shoud not have been coded as 23.01.1000.
And of course we dont know the final destination of "23.01.1000"-products exported by the US, whether or not the MBM reached cattle through feeding in the importing country.
It is therefore not possible to make any real risk estimate, not knowing what happened in each importing country. For countries already assessed by the SSC, such knowledge may be found in the already publisehd GBR assessments.
Here I can only add up exports from US to each country, and only for years 1999 and 2002 (+ january-march 2003), and roughly calculate the numbers of risk units _if_ these imports really represented a risk to cattle in the importing countries. (see list country list below).
It appears that the countries most at risk from US imports, especially MBM would be Bangladesh, Egypt, China, Indonesia, Malaysia, Mexico, Philippines, Taiwan, Thailand, Venezuela, Vietnam. Some other countries have imported much lower amounts of US cattle + MBM in 1999 and 2002/2003, but I dont know if these countries have imported similar (or higher) amounts from the USA in previous years.
According to Rev Sci Tech. 2003 Apr;22(1):237-49. Risk management of transmissible spongiform encephalopathies in Asia - Ozawa Y :
" ...significant quantities of feedstuffs of ruminant origin have been imported into Asia, which may mean that the BSE agent could have reached domestic cattle in most countries... Recycling of BSE through rendering plants is unlikely but cannot be totally excluded in some countries such as the People's Republic of China, India, Japan, Pakistan and Taipei China... "
"...The external challenge has been considerably reduced in recent years as most countries in Asia banned the importation of feedstuffs from _countries with BSE _ ..."
(my comments: but they did not ban MBM from the USA... I think China has the world's largest cattle population...)
quoted from a series of articles on CWD, in 2002 : http://cfapp.rockymountainnews.com/cwd/killer/
" ...the FDA has not imposed the same restrictions on exported MBM. In fact, since the American ban went into effect, annual U.S. exports of MBM have jumped from 291,000 tons to 467,000 tons, a 60 percent increase. American renderers aren't required to warn their foreign customers about feeding ruminant protein -- that rendered from sheep or cattle -- to cattle. However, three large renderers contacted by the News say they label their products that way regardless of the lack of regulations. Denver's National By-Products said it ships its MBM to China and Indonesia in large shipping containers, not in individually marked bags. But it stamps on its bills of lading a warning against feeding the product to ruminants. The stamp is in English. Once American meat and bone meal arrives in the purchasing country, the manufacturer has no further control over how it is labeled, said National By-Products district manager Ken Kage. A spokesman for the USDA and officials with the National Renderers Association say that foreign trade in U.S. MBM is not a problem because there have been no cases of mad cow disease in this country. Some countries importing MBM have had few if any rules concerning its use as cattle feed. Mexico, for example, implemented labeling rules only this year (2002), according to Alberto Celis, the National Renderers Association regional director for Latin America. That was news to many agricultural business people attending an animal feed trade show in Guadalajara in March. Representatives from three animal feed bag manufacturers said they had heard of no such regulations and that their bags remain warning free. Mexico exports over a million live cattle a year to the United States. Mexican cattlemen said these "feeder" cows are not typically fed animal protein, though there is little evidence that the government has an adequate inspection program to make certain. Mexican government officials responded that MBM rules were promulgated last summer, and that they will be vigorously enforced. They said Mexico stopped importing MBM from countries with a BSE problem in 1991 and that there are no known cases of BSE in the country. The World Health Organization says Mexico's experience with American MBM is reflected throughout the world. The United Nations agency was "concerned that some countries which received (MBM) materials do not have surveillance systems to detect the disease in animals or the human population," said WHO's Dr. Maura Ricketts at a news conference in December 2000. She said once the MBM leaves one country, it begins a "murky movement" that is almost impossible to track. Taking heed of such warnings, the European Union (EU) decided that the risk to public health was too great even if an importing country insisted that it would use MBM only as poultry feed -- which, along with pet food, is its major use in the U.S. The EU adopted the ban of all exports of MBM in 2000. Instead of adopting a similar policy, the USDA saw the ban as a golden opportunity. "Importing countries of EU MBM may be forced to seek alternative suppliers of animal protein meals, such as the United States," said a December 2000 report by the USDA. "The United States should be well positioned to take advantage of that situation to increase its own exports of MBM." And it has. Render, the magazine of the National Renderers Association, noted in its April issue that exports of many products were under competitive pressure from vegetable oils. But it noted "a bright spot is meat and bone meal exports that continue to increase." The chief foreign markets for American MBM, in order of sales amounts, were Indonesia, Mexico, Egypt, China, Canada, Thailand, Bangladesh, the Philippines and Venezuela. In 1998, Egypt imported 96,000 metric tons of MBM from the EU, and only 3,100 metric tons from the U.S. By 2001, the U.S dominated the Egyptian market, selling over 73,000 metric tons..."
(r.u = risk unit) (2003 = US exports of "23.01.1000"-products in the period January through March 2003)
Argentina 1999: 9 cattle for breeding (0,09 r.u.)
Australia 1999: 81 breeding cattle from USA (0,8 risk units) 2003: 5 tons MBM from USA (0,5 r.u.)
Bangladesh 2003: 2.217 tons MBM 2002: 12.630 tons MBM (1.484 r.u)
Belize: 2002: 27 cattle for breeding (0,27 r.u.)
Brazil 1999: 440 breeding cattle 2002: 134 breeding cattle (5,7 r.u.) 2002: 12 tons MBM 2003: 12 tons MBM (2,4 r.u.)
China 1999: 84 breeding cattle 2002: 40 + 190 + 26 breeding cattle (2,8 r.u.) 2002: 104.784 tons MBM 2003: 19.552 tons MBM (12.433 r.u)
Colombia 1999: 251 cattle for breeding 2002: 2.363 cattle for breeding (26 r.u.) 2002: 882 tons MBM 2003: 80 tons MBM (96 r.u.)
Colombia has > 100 r.u. from recent imports from the USA. Colombia was previously assessed in category II and might now be re-assessed in category III, unless there is proof that the additional external challenge from USA did not reach Colombian cattle.
Costa Rica 2002: 19 cattle for breeding (0,19 r.u.)
Dominican Republic: 1999: 45 cattle for breeding 2002: 220 cattle for breeding (2, 65 r.u.) 2003: 77 tons MBM (7,7 r.u.)
Ecuador 1999: 120 cattle for breeding (1,2 r.u.) 2002: 14 tons MBM (1,4 r.u.)
Egypt: 2002: 104.408 tons MBM 2003: 15.796 tons MBM (12.019 r.u.) Egypt would already have a high external challenge from previous imports from Europe.
Ghana: 2003: 41 tons MBM (4,1 r.u.)
Guatemala 1999: 23 cattle for breeding 2002: 26 cattle for breeding (0,49 r.u.)
Honduras 2002: 51 cattle for breeding (0,51 r.u.)
Hong Kong: 2002: 41 tons MBM 2003: 61 tons MBM (10 r.u.)
Indonesia 2002: 148.558 tons MBM 2003: 36.999 tons MBM (18.555 r.u.) (according to various figures from newspapers, Indonesia would prevously have been a major importer of British MBM)
Korea, South 1999: 248 cattle for breeding (2, 48 r.u.) 2002: 262 tons MBM (26 r.u.)
Lebanon 1999: 2.228 cattle for breeding (22, 3 r.u.)
Malaysia 2002: 7 cattle for breeding (0,07 r.u.) 2002: 12.646 tons MBM 2003: 2.209 tons MBM (1485 r.u.)
Mexico 1999: 8.780 cattle for breeding 2002: 10.888 cattle for breeding (196 r.u.) 2002: 93.685 other cattle ?? 2002: 62.204 tons MBM 2003: 14.756 tons MBM (7.696 r.u.)
New Zealand 2002: 21 tons MBM (2,1 r.u.)
Niger 2002: 57 tons MBM (5,7 r.u.)
Panama 1999: 17 cattle for breeding 2002: 59 cattle for breeding (0,76 r.u.) 2002: 172 tons MBM 2003: 57 tons MBM (23 r.u.) Panama, previously assessed in category I, might have 23 additonal risk units from recent US imports, and might be re-assessed in category II if this could have reached cattle.
Pakistan 1999: 11 cattle for breeding (0,11 r.u.)
Philippines 2002: 5.585 tons MBM 2003: 1.215 tons MBM (680 r.u.)
Russia: 2002: 390 tons MBM 2003: 1.520 tons MBM (191 r.u.) Russia would probably already be at risk from imports from EU and/or east-European countries.
Saudi Arabia 1999: 884 cattle for breeding (8, 84 r.u.)
Singapore 2003: 4 tons MBM (0, 4 r.u.)
South Africa: 2002: 40 tons MBM (4 r.u.)
Sri Lanka 2002: 351 tons MBM (35 r.u.)
Suriname 2002: 45 cattle for breeding (0,45 r.u.)
Taiwan 2002: 12.421 tons MBM 2003: 1.719 tons MBM (1.414 r.u.)
Thailand 2002: 36.476 tons MBM 2003: 7.314 tons MBM (4.379 r.u.)
United Arab Emirates 2003: 39 tons MBM (3,9 r.u.)
Uruguay: 1999: 7 cattle for breeding (0,07 r.u.)
Venezuela 1999: 473 cattle for breeding 2002: 169 cattle for breeding (6, 4 r.u.) 2002: 1.998 tons MBM (199 r.u.)
Vietnam 2002: 7.618 tons MBM 2003: 2.229 tons MBM (1.048 r.u.)
Best regards Karin Irgens
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Subject: SV: BSE-CWD-canada From: Karin.Irgens@DYREHELSETILSYNET.NO Reply To: Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> Date: Mon, 2 Jun 2003 22:20:13 +0200 Content-Type: text/plain Parts/Attachments: text/plain (278 lines) ######## Bovine Spongiform Encephalopathy <BSE-L@UNI-KARLSRUHE.DE> #########
Hello
In my posting yesterday I wrote: __ "...The results in different risk groups (all EU countries) in 2002 are: - 510 positive among 182.873 emergency slaughtered (ratio 27, 89 + per 10.000 tested) ..." __
I was probably half asleep... and I just discovered my error...
Here are the correct figures: Total 1.235.340 "emergency slaughtered" cattle tested in all EU countries in 2002 - 1.142 found positive - ratio 9,24 positive per 10.000 __
Robert wrote: Canada has a 3.5 M cattle population
According to Lyle Vanclief (http://www.inspection.gc.ca/english/anima/heasan/disemala/bseesb/debatee.sh tml Statement by The Honourable Lyle Vanclief to the House of Commons - May 26, 2003)
"...there are over 13 million cattle in Canada and 3.6 million that are slaughtered each year in Canada" I would assume that the Canadian 'adult' cattle population is somewhere around 40% of 13 million.
and ... " There are over half a million Canadian cattle in American feedlots and breeding herds"
According to the EU SSC report on Canada (July 2000), Canada tested 4.512 cattle for BSE in 8 years, (1992-1999). In 1999 they tested 895 brains from clinically suspect cattle and today, according to newspapers, they test around 1000/year. The % of samples from cattle older than 3-4 years is unknown (to me).
Robert: "... Only 2 "suspect" cases were reported for the year, which is astonishing. Obviously veterinary referrals are not a working system in Austria".
In Austria, it is true that there have been very few samples of clinical suspect cattle since the active surveillance program started in 2001. According to the EU SSC report on Austria (July 2000), between 1991 and 1997, approximately 60 to 80 brains of suspect cattle were examined forBSE each year. In 1998, 72 'clinical suspect' brain samples were examined in Austria, and in 1999 and 2000, respectively 137 and 280. Austria has only 1 million adult cattle.
It is understandable that the number of tests in passive surveillance decreased in the years with active surveillance (2001-2002) . Positive fallen stock or emergency slaughtered older than 24 months would have been detected by active surveillance, as well as positive "clinically healthy" older than 30 months. It was in this last group that Austria found its first BSE-positive.
In Germany, passive surveillance became satisfactory from 1997 (that is, "fulfilling the requirements of OIE and EC decision 98/272, which can be seen as a _minimal_ standard for BSE-surveillance....Today the ability to identify BSE-cases... is as good as it can be expected from a passive surveillance system..." (according to the EU SSC report on Germany (July 2000). During 1998-1999. around 900 brains/year from clinical suspect callte were examined for BSE in Germany. Today, much less (241 clinical suspect in 2002 - 214 in 2001).
The situation in Greece is similar to Austria. One sigle BSE-positive discovered in 2001 among 15.360 ""healthy cattle". Greece has 0,3 M adult cattle and most are slaughtered very young. The level of surveillance of 'risk cattle' is much better in Austria than in Greece.
Selection of "clinical suspects" seems to be a problem: Belgium has tested 242 and 279 clinical suspects respectively in 2001 and 2002, but found relatively few confirmed BSE-cases (9 + 5). Were most of the sampled cattle in Belgium maybe too young ?
Spain tested 464 clinical suspects in 2001 and found 9 positive. In 2002, Spain tested only 63 clinical suspects and found 17 positive. Maybe the selections by age were much better in Spain in 2002 ?
Does anyone know the age distribution of cattle tested for BSE in the USA and Canada ?
Robert : " ...In my opinion, the effectiveness or ineffectiveness of veterinary referrals as a means of detecting BSE hinges primarily on one factor: Political willingness to find the first case of BSE. Obviously, this willingness was present in the UK and France, but not Germany. Just as obviously, it is present in Canada and the USA (given the CWD experience)...."
my comment: I would agree that political willingness is very important - and may not have been present in Germany or Spain... But is it present in the USA _for BSE_ ? The willingness is now present for CWD, which was already a known disease in some US states. When they now test for CWD, as in Wisconsin and elsewhere, do they select "sick" animals only ? I dont think so. I think we should do much more TSE-testing on cervids in Europe. We won't know if we have CWD or not, unless we really look for it. Some European countries are starting active surveillance for CWD (ot other cervid-TSE), but this is still at low level.
"... Tuberculosis, polio, malaria, etc? Obviously such diseases are typically detected by clinical referral, followed by lab confirmation testing.
When I lived in France, until 1987, they still had compulsory tuberculosis screening by radiography, giving high radiation doses to the general low risk population, but they did _not_ screen risk groups such as the homeless or immigrant illegal workers sharing the same bed in 3 X 8 hours shifts...
Robert: "...By the way, all of your arguments could equally be applied to swine production: Are you advocating that EU countries start massive sample and test programs for swine? How do we know BSE is not present in these animals, which were not protected until a complete feed ban was put into place? ..."
comment: Most pigs are slaughtered around the age of 6 months. If BSE infectivity was present in pigs (from feeding), it would be in the intestine at that age, and presumably at concentrations far too low for detection by any of the validated rapid tests - although pig intestines used as casings might represent a risk. However, pigs slaughtered in the EU today are born long after the total feed ban in January 2001. I would be more worried about feeding of North American cattle with 'poultry offal meal' and MBM produced from pigs, with intestines and intestinal contents from pigs and poultry that have been fed ruminant-derived MBM. Not to mention 'poultry litter', still used in the USA in cattle feed (but not in Canada, they say) : "... Manure is not on the list of approved ingredients for animal feed in Canada, but U.S. regulations permit the use of poultry litter." http://cnews.canoe.ca/CNEWS/Canada/2003/06/01/101175-cp.html
Best regards
Karin Irgens
snip...end...tss
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NEW SCIENCE, YEARS LATER, ON ZOONOSIS OF THE BSE, SCRAPIE, CWD, TSE PRION!
***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***
Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.
https://www.nature.com/articles/srep11573
O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France
Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases).
Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.
*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases.
We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.
===============
***thus questioning the origin of human sporadic cases***
===============
***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.
==============
https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf
***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.
***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.
***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20
PRION 2016 TOKYO
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
Taylor & Francis
Prion 2016 Animal Prion Disease Workshop Abstracts
WS-01: Prion diseases in animals and zoonotic potential
Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.
These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20
Title: Transmission of scrapie prions to primate after an extended silent incubation period)
*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.
*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.
http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160
1: J Infect Dis 1980 Aug;142(2):205-8
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.
snip...
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.
PMID: 6997404
Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"
Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.
snip...
76/10.12/4.6
Nature. 1972 Mar 10;236(5341):73-4.
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
Gibbs CJ Jr, Gajdusek DC.
Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
C. J. GIBBS jun. & D. C. GAJDUSEK
National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).
Wednesday, February 16, 2011
IN CONFIDENCE
SCRAPIE TRANSMISSION TO CHIMPANZEES
IN CONFIDENCE
Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY
Date: Fri, 18 Oct 2002 23:12:22 +0100
From: Steve Dealler
Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member
To: BSE-L@ References: <3daf5023 .4080804="" wt.net="">
Dear Terry,
An excellent piece of review as this literature is desparately difficult to get back from Government sites.
What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!
Steve Dealler
===============
BSE Inquiry Steve Dealler
Management In Confidence
BSE: Private Submission of Bovine Brain Dealler
snip...see full text;
MONDAY, FEBRUARY 25, 2019
***> MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019
WEDNESDAY, AUGUST 5, 2020
1996-12-04: BBC - Horizon BSE1 - BSE2 The Invisible Enemy, The British Disease, CWD, sporadic CJD
MONDAY, DECEMBER 16, 2019
Chronic Wasting Disease CWD TSE Prion aka mad cow type disease in cervid Zoonosis Update
***> ''In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***
What if?
> However, to date, no CWD infections have been reported in people.
key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
Chronic Wasting Disease CWD TSE Prion aka mad deer disease zoonosis
We hypothesize that:
(1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues;
(2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence;
(3) Reliable essays can be established to detect CWD infection in humans; and
(4) CWD transmission to humans has already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches.
ZOONOTIC CHRONIC WASTING DISEASE CWD TSE PRION UPDATE
Prion 2017 Conference
First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1
University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen
This is a progress report of a project which started in 2009. 21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves.
Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice.
At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation.
PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS
PRION 2018 CONFERENCE
Oral transmission of CWD into Cynomolgus macaques: signs of atypical disease, prion conversion and infectivity in macaques and bio-assayed transgenic mice
Hermann M. Schatzl, Samia Hannaoui, Yo-Ching Cheng, Sabine Gilch (Calgary Prion Research Unit, University of Calgary, Calgary, Canada) Michael Beekes (RKI Berlin), Walter Schulz-Schaeffer (University of Homburg/Saar, Germany), Christiane Stahl-Hennig (German Primate Center) & Stefanie Czub (CFIA Lethbridge).
To date, BSE is the only example of interspecies transmission of an animal prion disease into humans. The potential zoonotic transmission of CWD is an alarming issue and was addressed by many groups using a variety of in vitro and in vivo experimental systems. Evidence from these studies indicated a substantial, if not absolute, species barrier, aligning with the absence of epidemiological evidence suggesting transmission into humans. Studies in non-human primates were not conclusive so far, with oral transmission into new-world monkeys and no transmission into old-world monkeys. Our consortium has challenged 18 Cynomolgus macaques with characterized CWD material, focusing on oral transmission with muscle tissue. Some macaques have orally received a total of 5 kg of muscle material over a period of 2 years.
After 5-7 years of incubation time some animals showed clinical symptoms indicative of prion disease, and prion neuropathology and PrPSc deposition were detected in spinal cord and brain of some euthanized animals. PrPSc in immunoblot was weakly detected in some spinal cord materials and various tissues tested positive in RT-QuIC, including lymph node and spleen homogenates. To prove prion infectivity in the macaque tissues, we have intracerebrally inoculated 2 lines of transgenic mice, expressing either elk or human PrP. At least 3 TgElk mice, receiving tissues from 2 different macaques, showed clinical signs of a progressive prion disease and brains were positive in immunoblot and RT-QuIC. Tissues (brain, spinal cord and spleen) from these and pre-clinical mice are currently tested using various read-outs and by second passage in mice. Transgenic mice expressing human PrP were so far negative for clear clinical prion disease (some mice >300 days p.i.). In parallel, the same macaque materials are inoculated into bank voles.
Taken together, there is strong evidence of transmissibility of CWD orally into macaques and from macaque tissues into transgenic mouse models, although with an incomplete attack rate.
The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.
Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP.
The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD..
***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***
READING OVER THE PRION 2018 ABSTRACT BOOK, LOOKS LIKE THEY FOUND THAT from this study ;
P190 Human prion disease mortality rates by occurrence of chronic wasting disease in freeranging cervids, United States
Abrams JY (1), Maddox RA (1), Schonberger LB (1), Person MK (1), Appleby BS (2), Belay ED (1) (1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA..
SEEMS THAT THEY FOUND Highly endemic states had a higher rate of prion disease mortality compared to non-CWD
states.
AND ANOTHER STUDY;
P172 Peripheral Neuropathy in Patients with Prion Disease
Wang H(1), Cohen M(1), Appleby BS(1,2) (1) University Hospitals Cleveland Medical Center, Cleveland, Ohio (2) National Prion Disease Pathology Surveillance Center, Cleveland, Ohio..
IN THIS STUDY, THERE WERE autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017,
AND
included 104 patients. SEEMS THEY FOUND THAT The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%),
AND
THAT The Majority of cases were male (60%), AND half of them had exposure to wild game.
snip…
see more on Prion 2017 Macaque study from Prion 2017 Conference and other updated science on cwd tse prion zoonosis below…terry
PRION 2019 ABSTRACTS
1. Interspecies transmission of the chronic wasting disease agent
Justin Greenlee
Virus and Prion Research Unit, National Animal Disease Center, USDA Agriculture Research Service
ABSTRACT
The presentation will summarize the results of various studies conducted at our research center that assess the transmissibility of the chronic wasting disease (CWD) agent to cattle, pigs, raccoons, goats, and sheep. This will include specifics of the relative attack rates, clinical signs, and microscopic lesions with emphasis on how to differentiate cross-species transmission of the CWD agent from the prion diseases that naturally occur in hosts such as cattle or sheep. Briefly, the relative difficulty of transmitting the CWD agent to sheep and goats will be contrasted with the relative ease of transmitting the scrapie agent to white-tailed deer.
53. Evaluation of the inter-species transmission potential of different CWD isolates
Rodrigo Moralesa, Carlos Kramma,b, Paulina Sotoa, Adam Lyona, Sandra Pritzkowa, Claudio Sotoa
aMitchell Center for Alzheimer’s disease and Related Brain Disorders, Dept. of Neurology, McGovern School of Medicine University of Texas Health Science Center at Houston, TX, USA; bFacultad de Medicina, Universidad de los Andes, Santiago, Chile
ABSTRACT
Chronic Wasting Disease (CWD) has reached epidemic proportions in North America and has been identified in South Korea and Northern Europe. CWD-susceptible cervid species are known to share habitats with humans and other animals entering the human food chain. At present, the potential of CWD to infect humans and other animal species is not completely clear. The exploration of this issue acquires further complexity considering the differences in the prion protein sequence due to species-specific variations and polymorphic changes within species. While several species of cervids are naturally affected by CWD, white-tailed deer (WTD) is perhaps the most relevant due to its extensive use in hunting and as a source of food. Evaluation of inter-species prion infections using animals or mouse models is costly and time consuming. We and others have shown that the Protein Misfolding Cyclic Amplification (PMCA) technology reproduces, in an accelerated and inexpensive manner, the inter-species transmission of prions while preserving the strain features of the input PrPSc. In this work, we tested the potential of different WTD-derived CWD isolates to transmit to humans and other animal species relevant for human consumption using PMCA. For these experiments, CWD isolates homozygous for the most common WTD-PrP polymorphic changes (G96S) were used (96SS variant obtained from a pre-symptomatic prion infected WTD). Briefly, 96GG and 96SS CWD prions were adapted in homologous or heterologous substrate by PMCA through several (15) rounds. End products, as well as intermediates across the process, were tested for their inter-species transmission potentials. A similar process was followed to assess seed-templated misfolding of ovine, porcine, and bovine PrPC. Our results show differences on the inter-species transmission potentials of the four adapted materials generated (PrPC/PrPSc polymorphic combinations), being the homologous combinations of seed/substrate the ones with the greater apparent zoonotic potential. Surprisingly, 96SS prions adapted in homologous substrate were the ones showing the easiest potential to template PrPC misfolding from other animal species. In summary, our results show that a plethora of different CWD isolates, each comprising different potentials for inter-species transmission, may exist in the environment. These experiments may help to clarify an uncertain and potentially worrisome public health issue. Additional research in this area may be useful to advise on the design of regulations intended to stop the spread of CWD and predict unwanted zoonotic events.
56. Understanding chronic wasting disease spread potential for at-risk species
Catherine I. Cullingham, Anh Dao, Debbie McKenzie and David W. Coltman
Department of Biological Sciences, University of Alberta, Edmonton AB, Canada
ABSTRACT
Genetic variation can be linked to susceptibility or resistance to a disease, and this information can help to better understand spread-risk in a population. Wildlife disease incidence is increasing, and this is resulting in negative impacts on the economy, biodiversity, and in some instances, human health. If we can find genetic variation that helps to inform which individuals are susceptible, then we can use this information on at-risk populations to better manage negative consequences. Chronic wasting disease, a fatal, transmissible spongiform encephalopathy of cervids (both wild and captive), continues to spread geographically, which has resulted in an increasing host-range. The disease agent (PrPCWD) is a misfolded conformer of native cellular protein (PrPC). In Canada, the disease is endemic in Alberta and Saskatchewan, infecting primarily mule deer and white-tail deer, with a smaller impact on elk and moose populations. As the extent of the endemic area continues to expand, additional species will be exposed to this disease, including bison, bighorn sheep, mountain goat, and pronghorn antelope. To better understand the potential spread-risk among these species, we reviewed the current literature on species that have been orally exposed to CWD to identify susceptible and resistant species. We then compared the amino acid polymorphisms of PrPC among these species to determine whether any sites were linked to susceptibility or resistance to CWD infection. We sequenced the entire PrP coding region in 578 individuals across at-risk populations to evaluate their potential susceptibility. Three amino acid sites (97, 170, and 174; human numbering) were significantly associated with susceptibility, but these were not fully discriminating. All but one species among the resistant group shared the same haplotype, and the same for the susceptible species. For the at-risk species, bison had the resistant haplotype, while bighorn sheep and mountain goats were closely associated with the resistant type. Pronghorn antelope and a newly identified haplotype in moose differed from the susceptible haplotype, but were still closely associated with it. These data suggest pronghorn antelope will be susceptible to CWD while bison are likely to be resistant. Based on this data, recommendations can be made regarding species to be monitored for possible CWD infection.
KEYWORDS: Chronic wasting disease; Prnp; wildlife disease; population genetics; ungulates
Thursday, May 23, 2019
Prion 2019 Emerging Concepts CWD, BSE, SCRAPIE, CJD, SCIENTIFIC PROGRAM Schedule and Abstracts
see full Prion 2019 Conference Abstracts
THURSDAY, OCTOBER 04, 2018
Cervid to human prion transmission 5R01NS088604-04 Update
snip…full text;
SATURDAY, FEBRUARY 09, 2019
Experts: Yes, chronic wasting disease in deer is a public health issue — for people
SATURDAY, FEBRUARY 23, 2019
Chronic Wasting Disease CWD TSE Prion and THE FEAST 2003 CDC an updated review of the science 2019
TUESDAY, NOVEMBER 04, 2014
Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011
Authors, though, acknowledged the study was limited in geography and sample size and so it couldn't draw a conclusion about the risk to humans. They recommended more study. Dr. Ermias Belay was the report's principal author but he said New York and Oneida County officials are following the proper course by not launching a study. "There's really nothing to monitor presently. No one's sick," Belay said, noting the disease's incubation period in deer and elk is measured in years. "
Transmission Studies
Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS
resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.
snip....
Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿
Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations
In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease
Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.
*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.
see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans”
From: TSS
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias"
To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam,
In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message-----
From: Sent: Sunday, September 29, 2002 10:15 AM
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.
snip...
*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
snip... full text ;
> However, to date, no CWD infections have been reported in people.
sporadic, spontaneous CJD, 85%+ of all human TSE, just not just happen. never in scientific literature has this been proven.
if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;
key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry
*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***
*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***
FRIDAY, JULY 26, 2019
Chronic Wasting Disease in Cervids: Implications for Prion Transmission to Humans and Other Animal Species
TUESDAY, JANUARY 21, 2020
***> 2004 European Commission Chronic wasting disease AND TISSUES THAT MIGHT CARRY A RISK FOR HUMAN FOOD AND ANIMAL FEED CHAINS REPORT UPDATED 2020
***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***
***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<***
***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***
***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<***
***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***
FRIDAY, OCTOBER 25, 2019
Experts testify United States is underprepared for bioterrorism threats Transmissible Spongiform Encephalopathy TSE Prion disease
Friday, December 14, 2012
DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012
snip.....
In the USA, under the Food and Drug Administration's BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. However, this recommendation is guidance and not a requirement by law. Animals considered at high risk for CWD include:
1) animals from areas declared to be endemic for CWD and/or to be CWD eradication zones and
2) deer and elk that at some time during the 60-month period prior to slaughter were in a captive herd that contained a CWD-positive animal.
Therefore, in the USA, materials from cervids other than CWD positive animals may be used in animal feed and feed ingredients for non-ruminants.
The amount of animal PAP that is of deer and/or elk origin imported from the USA to GB can not be determined, however, as it is not specified in TRACES.
It may constitute a small percentage of the 8412 kilos of non-fish origin processed animal proteins that were imported from US into GB in 2011.
Overall, therefore, it is considered there is a __greater than negligible risk___ that (nonruminant) animal feed and pet food containing deer and/or elk protein is imported into GB.
There is uncertainty associated with this estimate given the lack of data on the amount of deer and/or elk protein possibly being imported in these products.
snip.....
36% in 2007 (Almberg et al., 2011). In such areas, population declines of deer of up to 30 to 50% have been observed (Almberg et al., 2011). In areas of Colorado, the prevalence can be as high as 30% (EFSA, 2011). The clinical signs of CWD in affected adults are weight loss and behavioural changes that can span weeks or months (Williams, 2005). In addition, signs might include excessive salivation, behavioural alterations including a fixed stare and changes in interaction with other animals in the herd, and an altered stance (Williams, 2005). These signs are indistinguishable from cervids experimentally infected with bovine spongiform encephalopathy (BSE). Given this, if CWD was to be introduced into countries with BSE such as GB, for example, infected deer populations would need to be tested to differentiate if they were infected with CWD or BSE to minimise the risk of BSE entering the human food-chain via affected venison. snip..... The rate of transmission of CWD has been reported to be as high as 30% and can approach 100% among captive animals in endemic areas (Safar et al., 2008).
snip.....
In summary, in endemic areas, there is a medium probability that the soil and surrounding environment is contaminated with CWD prions and in a bioavailable form. In rural areas where CWD has not been reported and deer are present, there is a greater than negligible risk the soil is contaminated with CWD prion. snip..... In summary, given the volume of tourists, hunters and servicemen moving between GB and North America, the probability of at least one person travelling to/from a CWD affected area and, in doing so, contaminating their clothing, footwear and/or equipment prior to arriving in GB is greater than negligible... For deer hunters, specifically, the risk is likely to be greater given the increased contact with deer and their environment. However, there is significant uncertainty associated with these estimates.
snip.....
Therefore, it is considered that farmed and park deer may have a higher probability of exposure to CWD transferred to the environment than wild deer given the restricted habitat range and higher frequency of contact with tourists and returning GB residents.
snip.....
cwd scrapie pigs oral routes
***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <***
>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <***
***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%).
***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.
***> READ THIS VERY, VERY, CAREFULLY, AUGUST 1997 MAD COW FEED BAN WAS A SHAM, AS I HAVE STATED SINCE 1997! 3 FAILSAFES THE FDA ET AL PREACHED AS IF IT WERE THE GOSPEL, IN TERMS OF MAD COW BSE DISEASE IN USA, AND WHY IT IS/WAS/NOT A PROBLEM FOR THE USA, and those are;
BSE TESTING (failed terribly and proven to be a sham)
BSE SURVEILLANCE (failed terribly and proven to be a sham)
BSE 589.2001 FEED REGULATIONS (another colossal failure, and proven to be a sham)
these are facts folks. trump et al just admitted it with the feed ban.
see;
FDA Reports on VFD Compliance
John Maday
August 30, 2019 09:46 AM VFD-Form 007 (640x427)
Before and after the current Veterinary Feed Directive rules took full effect in January, 2017, the FDA focused primarily on education and outreach. ( John Maday ) Before and after the current Veterinary Feed Directive (VFD) rules took full effect in January, 2017, the FDA focused primarily on education and outreach to help feed mills, veterinarians and producers understand and comply with the requirements. Since then, FDA has gradually increased the number of VFD inspections and initiated enforcement actions when necessary. On August 29, FDA released its first report on inspection and compliance activities. The report, titled “Summary Assessment of Veterinary Feed Directive Compliance Activities Conducted in Fiscal Years 2016 – 2018,” is available online.
SUNDAY, SEPTEMBER 1, 2019
***> FDA Reports on VFD Compliance
[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
THURSDAY, SEPTEMBER 26, 2019
Veterinary Biologics Guideline 3.32E: Guideline for minimising the risk of introducing transmissible spongiform encephalopathy prions and other infectious agents through veterinary biologics
U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001
Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001
Date: Tue, 9 Jan 2001 16:49:00 -0800
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
snip...
[host Richard Barns] and now a question from Terry S. Singeltary of CJD Watch.
[TSS] yes, thank you, U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?
[no answer, you could hear in the back ground, mumbling and 'we can't. have him ask the question again.]
[host Richard] could you repeat the question?
[TSS] U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?
[not sure whom ask this] what group are you with?
[TSS] CJD Watch, my Mom died from hvCJD and we are tracking CJD world-wide.
[not sure who is speaking] could you please disconnect Mr. Singeltary
[TSS] you are not going to answer my question?
[not sure whom speaking] NO
snip...see full archive and more of this;
P.9.21 Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada
Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle. Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres.
Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis.
Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.
Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. * It also suggests a similar cause or source for atypical BSE in these countries.
*** It also suggests a similar cause or source for atypical BSE in these countries. ***
Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. *** This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *** It also suggests a similar cause or source for atypical BSE in these countries. ***
see page 176 of 201 pages...tss
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply;
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply;
2020
MONDAY, JULY 6, 2020
Guidance for reporting 2020 surveillance data on Transmissible Spongiform Encephalopathies (TSE)
SUNDAY, OCTOBER 4, 2020
Cattle Meat and Offal Imported from the United States of America, Canada and Ireland to Japan (Prions) Food Safety Commission of Japan
TUESDAY, SEPTEMBER 29, 2020
ISO's Updated 22442 Animal Tissue Standards — What Changed? TSE Prion!
TUESDAY, SEPTEMBER 22, 2020
APHIS USDA MORE SCRAPIE ATYPICAL Nor-98 Confirmed USA September 15 2020
''Why is USDA "only" testing 25,000 samples a year?
TUESDAY, AUGUST 18, 2020
Sheep Scrapie, Bovine BSE, Cervid CWD, ZOONOSIS, TSE Prion Roundup August 18, 2020
here's your sign...
SUNDAY, OCTOBER 11, 2020
Bovine adapted transmissible mink encephalopathy is similar to L-BSE after passage through sheep with the VRQ/VRQ genotype but not VRQ/ARQ
***> There have been 6 cases of BSE identified in the United States. The following information provides descriptions of these 6 cases:
2018- Florida
WEDNESDAY, APRIL 24, 2019
***> USDA Announces FLORIDA Atypical Bovine Spongiform Encephalopathy Detection Aug 29, 2018 A Review of Science 2019 <***
2017 – Alabama
TUESDAY, JULY 18, 2017
***> USDA announces Alabama case of Bovine Spongiform Encephalopathy Alabama
THURSDAY, JULY 20, 2017
***> USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589..200
2012 - California
2012 ATYPICAL L-TYPE BASE BSE TSE PRION CALIFORNIA ‘confirmed’ Saturday, August 4, 2012
*** Final Feed Investigation Summary - California BSE Case - July 2012
SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012
Summary Report BSE 2012
Executive Summary
Saturday, August 4, 2012
Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation
in the url that follows, I have posted
SRM breaches first, as late as 2011.
then
MAD COW FEED BAN BREACHES AND TONNAGES OF MAD COW FEED IN COMMERCE up until 2007, when they ceased posting them.
then,
MAD COW SURVEILLANCE BREACHES.
Friday, May 18, 2012
Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States Friday May 18, 2012
2012 ATYPICAL L-TYPE BASE BSE TSE PRION CALIFORNIA ‘confirmed’ Saturday, August 4, 2012
*** Final Feed Investigation Summary - California BSE Case - July 2012
SUNDAY, NOVEMBER 13, 2011
California BSE mad cow beef recall, QFC, CJD, and dead stock downer livestock
2006 - Alabama
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
THURSDAY, JULY 20, 2017
USDA OIE Alabama Atypical L-type BASE Bovine Spongiform Encephalopathy BSE animal feeds for ruminants rule, 21 CFR 589.200
TUESDAY, JULY 18, 2017
USDA announces Alabama case of Bovine Spongiform Encephalopathy Alabama
WEDNESDAY, JULY 19, 2017
OIE REPORT Bovine spongiform encephalopathy United States of America
Date: February 4, 2004 at 10:53 am PST
Alabama man dies of Creutzfeldt-Jakob disease, Alabama does not have to report CJD
The Associated Press
A DeKalb County resident who died last year was diagnosed with a form of a rare illness sometimes linked to mad cow disease, but it was unclear how he got the infection.
Doctors determined that a man who died in November while under hospice care suffered from Creutzfeldt-Jakob disease, Coroner Tom Wilson said Tuesday. The disease was listed as the cause of death on the death certificate, he said.
Health officials said the illness kills a few people each year in Alabama, but there has never been any sign of a link with mad cow disease, which has drawn wide attention since the Dec. 23 announcement that a cow in Washington state had tested positive for it.
Wilson declined further comment and referred questions to New Beacon Hospice, which cared for the victim in DeKalb County. Mary Colley of New Beacon refused comment, citing patient confidentiality laws.
Officials with the DeKalb County Public Health Department and the area health office covering northeast Alabama said they were unaware of the case. Doctors are not required to report cases of Creutzfeldt-Jakob to the state.
WHNT-TV of Huntsville reported that the man was diagnosed with sporadic Creutzfeldt-Jakob disease, a designation given cases where the source of the infection was unknown.
Researchers believe there is a connection between mad cow disease, or bovine spongiform encephalopathy, and a variation of Creutzfeldt-Jakob disease, a fatal disorder that causes rapid dementia and loss of muscle control.
Sharon Thompson, a nurse with the epidemiology office of the Alabama Department of Public Health in Montgomery, said four to five people die each year in Alabama of Creutzfeldt-Jakob disease.
None of those deaths have been from the variant of the disease sometimes linked to mad cow disease, she said. "There are cases of it that occur naturally," said Thompson.
About one person in 1 million died of Creutzfeldt-Jakob annually in the United States from 1979 through 1994, according to statistics from the Centers for Disease Control and Prevention in Atlanta.
ALABAMA CREUTZFELDT JAKOB DISEASE TSE PRION DISEASE REPORTING
***> cjd tse prion disease is not reportable apparently <***
2005 - Texas
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
THURSDAY, OCTOBER 22, 2015
Former Ag Secretary Ann Veneman talks women in agriculture and we talk mad cow disease USDA and what really happened
HOW TO COVER UP MAD COW DISEASE IN TEXAS
FRIDAY, DECEMBER 14, 2018
MAD COW USA FLASHBACK FRIDAY DECEMBER 14, 2018
2003 - Washington State
END...TSS
CHRONIC WASTING DISEASE CWD TSE PRION UPDATE
TUESDAY, OCTOBER 20, 2020
Public ownership of Texas’ white-tailed deer re-affirmed
SUNDAY, OCTOBER 21, 2018
Surveillance for variant CJD: should more children with neurodegenerative diseases have autopsies? Singeltary Review
THURSDAY, JULY 02, 2020
Variant Creutzfeldt–Jakob Disease Diagnosed 7.5 Years after Occupational Exposure
Volume 26, Number 8—August 2020
Sporadic Creutzfeldt-Jakob Disease among Physicians, Germany, 1993–2018 high proportion of physicians with sCJD were surgeons
MONDAY, OCTOBER 05, 2020
***> USA, UK, JAPAN, CJD TSE PRION STATISTICS UPDATE OCTOBER 2020
SATURDAY, SEPTEMBER 26, 2020
A nationwide trend analysis in the incidence and mortality of Creutzfeldt–Jakob disease in Japan between 2005 and 2014 with increasing trends of incidence and mortality
TUESDAY, MAY 19, 2020
China Sporadic Creutzfeldt-Jakob disease: A retrospective analysis of 104 cases
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor:
In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally..
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.
doi:10.1016/S1473-3099(03)00715-1 Copyright © 2003 Published by Elsevier Ltd. Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
Available online 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463
“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem..” ...
January 28, 2003; 60 (2) VIEWS & REVIEWS
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Ermias D. Belay, Ryan A. Maddox, Pierluigi Gambetti, Lawrence B. Schonberger
Abstract
Transmissible spongiform encephalopathies (TSEs) attracted increased attention in the mid-1980s because of the emergence among UK cattle of bovine spongiform encephalopathy (BSE), which has been shown to be transmitted to humans, causing a variant form of Creutzfeldt-Jakob disease (vCJD). The BSE outbreak has been reported in 19 European countries, Israel, and Japan, and human cases have so far been identified in four European countries, and more recently in a Canadian resident and a US resident who each lived in Britain during the BSE outbreak. To monitor the occurrence of emerging forms of CJD, such as vCJD, in the United States, the Centers for Disease Control and Prevention has been conducting surveillance for human TSEs through several mechanisms, including the establishment of the National Prion Disease Pathology Surveillance Center. Physicians are encouraged to maintain a high index of suspicion for vCJD and use the free services of the pathology center to assess the neuropathology of clinically diagnosed and suspected cases of CJD or other TSEs.
Received May 7, 2002. Accepted August 28, 2002.
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
Terry S. Singeltary, retired (medically)
Published March 26, 2003
26 March 2003
Terry S. Singeltary, retired (medically) CJD WATCH
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?
Reply to Singletary Ryan A. Maddox, MPH Other Contributors: Published March 26, 2003
Mr. Singletary raises several issues related to current Creutzfeldt- Jakob disease (CJD) surveillance activities. Although CJD is not a notifiable disease in most states, its unique characteristics, particularly its invariably fatal outcome within usually a year of onset, make routine mortality surveillance a useful surrogate for ongoing CJD surveillance.[1] In addition, because CJD is least accurately diagnosed early in the course of illness, notifiable-disease surveillance could be less accurate than, if not duplicative of, current mortality surveillance.[1] However, in states where making CJD officially notifiable would meaningfully facilitate the collection of data to monitor for variant CJD (vCJD) or other emerging prion diseases, CDC encourages the designation of CJD as a notifiable disease.[1] Moreover, CDC encourages physicians to report any diagnosed or suspected CJD cases that may be of special public health importance (e.g...., vCJD, iatrogenic CJD, unusual CJD clusters).
As noted in our article, strong evidence is lacking for a causal link between chronic wasting disease (CWD) of deer and elk and human disease,[2] but only limited data seeking such evidence exist. Overall, the previously published case-control studies that have evaluated environmental sources of infection for sporadic CJD have not consistently identified strong evidence for a common risk factor.[3] However, the power of a case-control study to detect a rare cause of CJD is limited, particularly given the relatively small number of subjects generally involved and its long incubation period, which may last for decades. Because only a very small proportion of the US population has been exposed to CWD, a targeted surveillance and investigation of unusual cases or case clusters of prion diseases among persons at increased risk of exposure to CWD is a more efficient approach to detecting the possible transmission of CWD to humans. In collaboration with appropriate local and state health departments and the National Prion Disease Pathology Surveillance Center, CDC is facilitating or conducting such surveillance and case- investigations, including related laboratory studies to characterize CJD and CWD prions.
Mr. Singletary also expresses concern over a recent publication by Asante and colleagues indicating the possibility that some sporadic CJD cases may be attributable to bovine spongiform encephalopathy (BSE).[4] The authors reported that transgenic mice expressing human prion protein homozygous for methionine at codon 129, when inoculated with BSE prions, developed a molecular phenotype consistent with a subtype of sporadic CJD. Although the authors implied that BSE might cause a sporadic CJD-like illness among persons homozygous for methionine, the results of their research with mice do not necessarily directly apply to the transmission of BSE to humans. If BSE causes a sporadic CJD-like illness in humans, an increase in sporadic CJD cases would be expected to first occur in the United Kingdom, where the vast majority of vCJD cases have been reported. In the United Kingdom during 1997 through 2002, however, the overall average annual mortality rate for sporadic CJD was not elevated; it was about 1 case per million population per year. In addition, during this most recent 6-year period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of UK sporadic CJD deaths.[3, 5] Furthermore, surveillance in the UK has shown no increase in the proportion of sporadic CJD cases that are homozygous for methionine (Will RG, National CJD Surveillance Unit, United Kingdom, 2003; personal communication)..
References
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Diagnosis and reporting of Creutzfeldt-Jakob disease. JAMA 2001;285:733-734.
2. Belay ED, Maddox RA, Gambetti P, Schonberger LB. Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States. Neurology 2003;60:176-181.
3. Belay ED. Transmissible spongiform encephalopathies in humans. Annu Rev Microbiol 1999;53:283-314.
4. Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J 2002;21:6358-6366.
Competing Interests: None declared.
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
2 January 2000 British Medical Journal U.S.
Scientist should be concerned with a CJD epidemic in the U.S., as well
15 November 1999 British Medical Journal hvCJD in the USA * BSE in U.S.
Volume 2: Science
4. The link between BSE and vCJD
Summary 4.29 The evidence discussed above that vCJD is caused by BSE seems overwhelming. Uncertainties exist about the cause of CJD in farmers, their wives and in several abattoir workers. It seems that farmers at least might be at higher risk than others in the general population. 1 Increased ascertainment (ie, increased identification of cases as a result of greater awareness of the condition) seems unlikely, as other groups exposed to risk, such as butchers and veterinarians, do not appear to have been affected. The CJD in farmers seems to be similar to other sporadic CJD in age of onset, in respect to glycosylation patterns, and in strain-typing in experimental mice. Some farmers are heterozygous for the methionine/valine variant at codon 129, and their lymphoreticular system (LRS) does not contain the high levels of PrPSc found in vCJD.
***>It remains a remote possibility that when older people contract CJD from BSE the resulting phenotype is like sporadic CJD and is distinct from the vCJD phenotype in younger people...end
BSE INQUIRY
SATURDAY, JUNE 23, 2018
CDC
***> Diagnosis of Methionine/Valine Variant Creutzfeldt-Jakob Disease by Protein Misfolding Cyclic Amplification
Volume 24, Number 7—July 2018 Dispatch
wasted days and wasted nights...Freddy Fender
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