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Health officials still waiting for CJD results "As of now, we haven't heard anything," said Tom Shanahan, spokesman for the Idaho Department of Health and Welfare on Friday afternoon. "We are hoping to get the results soon." Health officials received the preliminary results on the woman, which revealed that she did indeed die of a prion disease. Shanahan said the department hopes to get the final results from the National Prion Disease Pathology Surveillance Center at Cleveland's Case Western Reserve University early next week. A sixth person, a man from Elmore County diagnosed with CJD, is still alive, Shanahan said. Of the five women, four were from Twin Falls County and one woman was from Minidoka County. All six were over the age of 60. The only way to confirm a case of CJD and its type is by examining brain tissue. Autopsies were performed on three of the women and their brain tissue was sent to the National Prion Disease Pathology Surveillance Center to be studied. Two of the women died before their CJD was reported by their physicians and autopsies were not performed, Shanahan said. All six people were between the ages of 60 and 83 and given their ages, Idaho health officials suspect the women had sporadic CJD and not the variant form of CJD that people get when they eat meat from a cow with bovine spongiform encephalopathy - commonly known as mad cow disease. The average age of people who've died from sporadic CJD is 68. The average age of people who've died from the variant form of CJD is 28, according to the Centers for Disease Control and Prevention. CJD is a fatal disease carried by prions, an abnormal form of protein in the bloodstream. Prions cause folding of normal protein in the brain, leading to brain damage. Symptoms include dementia and other neurological signs. Its victims usually die within four or five months after onset of the disease, according to the Centers for Disease Control and Prevention. The unusually high number of cases has drawn attention from state health officials as well as the CDC. Normally, the disease infects just one person per 1 million people worldwide a year. In the United States, fewer than 300 cases of CJD are reported each year, according to the CDC. In Idaho, there were an average of 1.1 cases a year between 1984 and 2004, Shanahan said. "The most cases we've had in a single year was three until now," he said. State epidemiologist Dr. Christine Hahn, along with Chris Carter of the federal public health office in Idaho and Cheryle Becker, an epidemiologist with South Central District Health, examined medical records and Becker conducted surveys with all the victims' families. The Department of Health and Welfare is now entering the information into a computer program to determine if the six people had anything in common. "The state is compiling the data and finalizing the information," Becker said. "We're going back and evaluating the data we have. We're conversing with the CDC about all the information." Two clusters of sporadic CJD deaths in recent years occurred in New Jersey, involving 12 deaths; and in Oregon, involving 14 deaths. Those clusters, however, unfolded over a period of 36 months and 24 months, and in population areas of 1.7 million and 3.4 million, respectively. Times-News writer Sandy Miller can be reached at 735-3264 or by e-mail at smiller@magicvalley.com. Story published at magicvalley.com on Saturday, August 27, 2005 http://www.magicvalley.com/articles/2005/08/27/news_localstate/news_local_state.2.txt >>>Two clusters of sporadic CJD deaths in recent years occurred in New Jersey, involving 12 deaths; and in Oregon, involving 14 deaths. <<< http://www.jifsan.umd.edu/tse/Rawlings.htm what i considered a cluster of CJD victims in southeast Texas, 1: Ann Clin Lab Sci 2001 Apr;31(2):211-2 A cluster of Creutzfeldt-Jacob disease patients from Nassau County, Adikari D, Farmer P. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11337912&dopt=Abstract A Cluster of Creutzfeldt-Jacob Disease Patients from Nassau County, New February, 2001 Annals of Clinical and Laboratory Science Vol. 31 Creutzfeldt-Jacob Disease (CJD) is a rare and transmissible neurological We report 7 cases of CJD from North Shore University Hospital, a The authors are concerned that the number of CJD cases in our catchment http://www.organicconsumers.org/madcow/cluster501.cfm ====================================================== Farmer PM, Kane WC, Hollenberg-Sher J. Incidence of Creutzfeldt-Jakob disease in Brooklyn and Staten Island. N Engl J Med. 1978 Feb 2;298(5):283-4. No abstract available. 2: Nosal R, Kapoor A, Shanin R. Cluster of cases of Creutzfeldt-Jakob disease--Ontario. There is one report of a possible cluster of CJD cases in Canada; http://www.cdc.gov/ncidod/eid/vol3no1/stratton.htm An Evaluation of a Suspected Cluster of Creutzfeldt-Jakob Disease (CJD) in New Jersey May 2004 Division of Epidemiology, Environmental and Occupational Health snip... 17 5. Evaluation of suspected cluster of CJD The evidence does not support the existence of an outbreak of CJD among attendees at the GSRT, nor does it suggest that case-patients with CJD were exposed to BSEcontaminated beef in the period 1988 to 1992 or at any other time at the Garden State Racetrack in New Jersey. a. The largest and earliest cattle outbreak of BSE in the world occurred in the UK and to date the number of infected cattle has been many orders of magnitudes greater than the number of reported probable and definite vCJD cases in the UK. Thus, an outbreak of either variant or sporadic CJD in the US without more evidence of BSE in this country is extremely unlikely. b. All persons throughout the world with BSE-related vCJD tested as of January 2004 have had a specific genetic make-up characteristic of about 40% of the general population (Will et al, 2004). None of the three pathologically confirmed CJD casepatients who were tested for this marker were documented to have it. c. The UK’s National CJD Surveillance Unit reports no evidence that sporadic CJD has been caused by BSE. There was an increase in sporadic CJD in the UK from 1990-1996 due to improved case ascertainment (SEAC, 2004). This is to be expected for any disease 18 following the institution of a new and active surveillance system. However, there has been no unusually high incidence of sporadic forms of CJD from 1997-2003 in the UK, despite the much heavier exposure of the UK population to the BSE agent between 1980 and 1996 compared to the US population (UK Department of Health). d. Ongoing comparisons of exposures of sporadic CJD case-patients with controls in Europe, as conducted and interpreted by experts in the UK, have been reported to provide no convincing evidence of an increased risk of sporadic forms of CJD through dietary habits (Will et al., 2004). e. As illustrated in Figure 1, all but two of the presumed food-related deaths due to the BSE agent in the UK occurred in person younger than 55 years of age. In contrast, based on the information available at NJDHSS through April 2004, 10 of the 11 decedents with definite or probable CJD between 1995-2003 about whom JS has raised concerns were >55 at the time of death. f. None of the six patients in this report who had brain tissue submitted for examination at the NPDPSC were documented to have vCJD, the form associated with BSE exposure. Rather, they each had either a non-prion disease or form of sporadic CJD that differed from each other. These laboratory data provide scientific evidence against a theory that any of these illnesses were related to each other or to BSE. h. There is no evidence of an increased number of CJD case-patients in the region closest to the GSRT, in adjacent Philadelphia County, or in New York City. F. Future CJD surveillance plans in New Jersey 1. General The key to investigating potential causes of CJD in the US is to encourage brain autopsies of all patients who die of suspected CJD. New Jersey is among the states that require reporting by medical providers of suspected case-patients with CJD. Because physicians nationally and in NJ generally do an inadequate job of reporting all reportable diseases, states use additional methods to ensure as complete ascertainment of cases of interest as possible. The NJDHSS currently also reviews NJ hospital billing data and death certificate data to detect case-patients with CJD that were not reported by physicians, making it more likely to have complete ascertainment of case-patients than other states. The CDC also reviews national mortality data from the National Center for Health Statistics. When a CJD case-patient under 55 years of age is reported, the CDC contacts the state health department to review the details of this case-patient. The NJDHSS periodically sends out to all physicians in the state, and to every newly licensed physician, information on reporting requirements for all diseases and conditions listed in the Administrative Code regulations (N.J.A.C. 8:57), including CJD. The NJDHSS plans to notify physicians in the next few months about the recently adopted revised regulations pertaining to all reportable diseases, including materials such as pocket cards and magnets to remind physicians and other health care providers about their reporting responsibilities. In addition, the NJDHSS will be sending a specific letter prepared by the NPDPSC to neurologists and pathologists, informing them of the need to consider CJD in the differential diagnosis of a patient with a rapidly progressive neurological disorder with dementia, and the free services provided by the NPDPSC. Furthermore, the NPDPSC is prepared to make arrangements and provide for the costs of the autopsy. The NJDHSS will continue to review all suspected cases-patients with CJD detected through passive reporting by health providers, and by reviewing hospital billing data and death certificate data. It will encourage physicians to obtain permission from family members for autopsies of suspected case-patients at the time of death and submit brain tissue and other laboratory data to the NPDPSC for expert analysis. The CJD program at the CDC also has notified the NJDHSS that it intends to provide limited funds, as it does to several other states, to enhance CJD surveillance activities in the state. The NJDHSS plans to enhance education on prion disease, targeted to neurologists and pathologists, the physicians most likely to be in a position to diagnose a potential case of CJD. Unfortunately, autopsies in general are now only rarely done and this is especially true for suspected CJD case-patients where pathologists are reluctant to perform autopsies (Louie at al, 2004). However, as a result of the effort made by the autopsy program of the NPDPSC, the number of autopsies performed in CJD casepatients is currently close to 60% of the number of expected cases. 2. Continued evaluation of the suspected CJD cluster at the GSRT The NJDHSS will update its report on the suspected cluster of CJD case-patients at the GSRT once it receives the information from the pending case-patients. If there are additional reported case-patients that need to be assessed, the NJDHSS will evaluate those that were NJ residents, and will refer the non-NJ resident case-patients to other states for evaluation, where appropriate. Given the epidemiologic and pathologic evidence described above, the NJDHSS believes that conducting a case-control study by interviewing next-of-kin or friends of the definite and probable cases is unwarranted at this point for assessing the proposed hypothesis that BSE-contaminated beef in the US caused an increased incidence or cluster of CJD cases in New Jersey. The proposed hypothesis is not supported by the weight of the evidence presented in this report and the CDC report (CDC, 2004a) to justify conducting such a research project that is very unlikely to yield meaningful information. http://www.state.nj.us/health/eoh/cjd2004.pdf Beginning in June 2003, the New Jersey Department of Health and Senior Services (NJDHSS) and CDC were notified of a suspected cluster of deaths caused by Creutzfeldt-Jakob disease (CJD) in persons reportedly linked to Garden State Racetrack in Cherry Hill, New Jersey. Concerns were raised that these deaths might have resulted from consumption of meat contaminated with the agent causing bovine spongiform encephalopathy (BSE, commonly called "mad cow disease") served at racetrack restaurants during 1988--1992. Consumption of BSE-contaminated cattle products has been linked to a new variant form of CJD (vCJD) in humans. This report summarizes the results of an investigation that determined the deaths were not linked causally to a common source of infection. The findings underscore the need for physicians to arrange for brain autopsies of all patients with clinically suspected or diagnosed CJD. Available clinical and neuropathologic findings were reviewed for 17 suspected CJD deaths referred to NJDHSS and CDC. To investigate the deaths of these 17 persons, all of whom were reportedly linked to Garden State Racetrack, health-care providers were contacted and relevant medical records obtained by NJDHSS, other state health departments, and CDC. Providers were asked to submit available brain autopsy tissue to the National Prion Disease Pathology Surveillance Center (NPDPSC), a national prion disease diagnostic referral laboratory established by CDC and the American Association of Neuropathologists. Sufficient demographic and clinical information was available to classify 11 of the 17 deaths as resulting from a definite or probable case of a classic form of CJD*, on the basis of World Health Organization criteria (1). Of the remaining six decedents, neuropathologic analyses documented that three deaths resulted from causes unrelated to either vCJD or classic CJD (Table 1). Three deaths reported as resulting from CJD remain under investigation. Excluding the three deaths for which CJD was ruled out, the 14 remaining deaths occurred over a period of approximately 9.25 years (1995--2004); the average number of cases per complete year (i.e., excluding 2004) was 1.44 (range: zero to three cases). Eleven of the 14 decedents were male; median age was 69.5 years (range: 50--83 years). Six of the decedents resided in New Jersey, four in Pennsylvania, and one each in Connecticut, Delaware, Maryland, and Virginia. Neuropathologic analysis in the five definite cases with available brain tissue specimens was diagnostic of classic CJD; none had the characteristic pathologic findings of vCJD. A genotype at codon 129 of the prion protein gene (a genetic marker associated with specific subtypes of CJD) was determined for three of the five CJD deaths confirmed pathologically (Table 1). None of the decedents had the methionine homozyogosity or the characteristic Western blot pattern present for persons with vCJD. In addition, the reported CJD subtypes differed from one another. For the six deaths without tissue diagnosis, available clinical and diagnostic evidence, including illness duration, electroencephalographic patterns, and presence of protein 14-3-3 (a marker for classic CJD) in cerebrospinal fluid was consistent with a probable diagnosis of classic CJD (Tables 1 and 2). None of the decedents had a diagnosis of vCJD. For 1995--2002, using CDC's national multiple cause-of-death file (2002 data are preliminary) compiled annually by the National Center for Health Statistics, the annual death rate from CJD in the United States has been stable at approximately one case per 1 million persons per year (Figure 1). The CJD death rate for New Jersey during the same period was similar. In 2001, Garden State Racetrack was closed permanently. The number and ages of all persons visiting or dining at the racetrack is unknown. However, according to New Jersey Racing Commission records, attendance at the racetrack during 1988--1992 was approximately 4.1 million. Based on an annual CJD rate of 3.4 cases per 1 million persons (CDC, unpublished data, 2004) and an overall death rate from all causes of 2.9% for persons aged >50 years, the occurrence over approximately 9.25 years (1995--2004) of at least 14 CJD-related deaths among as few as 300,000 persons aged >50 years would not be unusual. This number is within the estimated range of the number of persons attending and dining at the racetrack, given the known attendance. Reported by: P Gambetti, MD, National Prion Disease Pathology Surveillance Center, Case Western Reserve Univ, Cleveland, Ohio. J Hadler, MD, Connecticut Dept of Public Health. A Hathcock, PhD, M Drees, MD, Delaware Health and Social Svcs. D Blythe, MD, Maryland Dept of Health and Mental Hygiene. E Bresnitz, MD, M Gerwel, MD, New Jersey Dept of Health and Senior Svcs. M Hawkins, MD, Philadelphia Dept of Public Health; A Weltman, MD, Pennsylvania Dept of Health. J Marr, MD, A Buckler, MD, C Novak, MD, Virginia Health Dept. C Rothwell, MS, K Kochanek, MA, R Anderson, PhD, Div of Vital Statistics, National Center for Health Statistics; J Sejvar, MD, E Belay, MD, R Maddox, MPH, A Curns, MPH, R Holman, MS, L Schonberger, MD, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases, CDC. Editorial Note: CJD is a neurodegenerative disease characterized by rapidly progressive dementia associated with brain pathology marked by diffuse spongiform degeneration; the disease is invariably fatal (2). According to the leading hypothesis, CJD is caused by an unconventional transmissible agent, an abnormal protein (i.e., prion) that is able to induce abnormal folding of normal cellular proteins, leading to neuronal death. Prions are believed to cause transmissible spongiform encephalopathies (TSEs) that include scrapie in sheep, BSE in cattle, chronic wasting disease (CWD) in deer and elk, and CJD in humans. Two major forms of CJD have been recognized, classic and variant (3). Classic CJD has been recognized since the early 1920s and is characterized by certain distinct clinical and diagnostic features (Table 2). The most common form of classic CJD is believed to occur sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions. This sporadic disease occurs worldwide at a rate of approximately one case per 1 million population per year, although rates of up to two cases per million are not unusual (4). Risk increases with age, and in persons aged >50 years, the annual rate is approximately 3.4 cases per million. Variant CJD was first described in 1996 in the United Kingdom and has different clinical characteristics than classic CJD (Table 2) (2,3). The median age at death for vCJD patients is 28 years, compared with 68 years for patients with classic CJD (Figure 2). In addition, all vCJD cases have neuropathologic findings distinctly different from those of classic CJD (5), and all have had a particular genetic profile (i.e., homozygosity for methionine) at codon 129 of the prion protein gene (4). Thus, cases of vCJD can be distinguished from classic CJD on the basis of clinical and pathologic data. Epidemiologic and laboratory evidence indicate that the agent causing BSE in cattle can be transmitted to humans via consumption of BSE-contaminated cattle products, causing vCJD (2,3). However, this evidence also suggests that the risk is low for having vCJD, even after consumption of contaminated product. In 1996, because of the emergence of vCJD in the United Kingdom, CDC enhanced its surveillance for CJD in the United States (6). No evidence has indicated that any of the 17 reported deaths resulted from vCJD. The CJD subtypes were determined in four decedents, and the subtype in each differed from the others; this heterogeneity provides scientific evidence against a common etiology for these cases. Although one study reported that BSE-infected mice expressing methionine homozygosity at codon 129 produced prions with a molecular phenotype consistent with a subtype of classic CJD (7), these animal data cannot be reliably extrapolated to humans in the absence of other supporting evidence. In 2003, the Spongiform Encephalopathy Advisory Committee of the United Kingdom concluded that these data did "not provide strong evidence to support" the hypothesis that exposure to BSE can produce a sporadic CJD-like phenotype in humans (8). In the United Kingdom, where the largest epidemic of BSE has occurred and an unusually large proportion of the population has been exposed to the BSE agent, the absence of an unusually high incidence of classic CJD patients or an elevated proportion of CJD patients with methionine homozygosity at codon 129 (9) supports the lack of association between BSE and sporadic CJD. In the United Kingdom, prion disease experts have looked specifically for evidence of BSE-related disease other than vCJD among classic CJD cases. No evidence of a new phenotype has been uncovered (R.G. Will, M.D., National CJD Surveillance Unit, Western General Hospital, Edinburgh, Scotland, personal communication, 2004). Neuropathologic evaluation, particularly by immunohistochemistry or Western blot, is the most definitive method to 1) diagnose human prion diseases, 2) monitor for vCJD and various subtypes of CJD, and 3) detect the possible emergence of new prion diseases in the United States. Although not all decedents in this investigation had pathologic specimens available for review, demonstration of the absence of a classic CJD or vCJD diagnosis in certain patients and diagnosis of classic CJD in others indicated these patients did not die from BSE-related disease. This investigation underscores the need for physicians to pursue autopsies of all decedents with clinically suspected and diagnosed CJD and to use the TSE diagnostic services provided free of charge by NPDPSC. Information regarding this surveillance center is available at http://www.cjdsurveillance.com or by telephone, 404-639-3091. CDC will continue to work with and support state health officials in New Jersey and nationally to conduct surveillance for CJD. Better defining the normal occurrence of subtypes of sporadic CJD and other TSEs will facilitate earlier recognition of vCJD or any other human prion disease that might emerge in the United States. * Those types of CJD that differ from vCJD and usually indicate sporadic CJD. References World Health Organization. Global surveillance, diagnosis, and therapy of human transmissible spongiform encephalopathies: report of a WHO consultation, 1998. WHO/EMC/ZDI/98.9. Available at http://www. who.int/emcdocuments/tse/docs/whoemczdi989.pdf. Table 1 **Questions or messages regarding errors in formatting should be addressed to mmwrq@cdc.gov. Page converted: 5/7/2004 Morbidity and Mortality Weekly Report This page last reviewed 5/7/2004 7/14/00 snip... http://www.mad-cow.org/UKCJD/CJD_news9.html and how far does the Queniborough CJD cluster really reach ? I was just looking at this email you sent me. Thurcaston snip... I've just been talking to professor bob will who runs the CJD Could you contact them asap and ask if they will speak to us? snip... Sadly, everything points to Mrs Soukup being a victim of sporadic CJD. snip... personal email tss since then, we have a documented case of nv/v CJD in 74 year old. 1: Cent Eur J Public Health 2003 Mar;11(1):19-22 Analysis of unusual accumulation of Creutzfeldt-Jakob disease cases Mad'ar R, Maslenova D, Ranostajova K, Straka S, Baska T. Institute of Epidemiology, Jessenius Faculty of Medicine, Comenius While familial cases of Creutzfeldt-Jakob disease are extremely rare PMID: 12690798 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12690798&dopt=Abstract ------------------------------------------------------------------------ 1: Eur J Epidemiol 1991 Sep;7(5):520-3 Mitrova E, Huncaga S, Hocman G, Nyitrayova O, Tatara M. Institute of Preventive and Clinical Medicine, Bratislava. Epidemic-like occurrence of Creutzfeldt-Jakob disease was observed in http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1761109&dopt=Abstract Morbidity and Mortality Weekly Report Weekly February 21, 2003 / Vol. 52 / No. 7 Fatal Degenerative Neurologic Illnesses in Men Who Participated in Wild Game Feasts — Wisconsin, 2002 depar department tment of health and human ser services vices Centers for Disease Control and Prevention INSIDE 128 Atrial Fibrillation as a Contributing Cause of Death and Medicare Hospitalization — United States, 1999 131 Potential Exposures to Airborne and Settled Surface Dust in Residential Areas of Lower Manhattan Following the Collapse of the World Trade Center — New York City, November 4–December 11, 2001 136 Smallpox Vaccine Adverse Events Among Civilians — United States, January 24–February 18, 2003 136 Notice to Readers Creutzfeldt-Jakob disease (CJD) is a fatal neurologic disorder in humans. CJD is one of a group of conditions known as transmissible spongiform encephalopathies (TSEs), or prion diseases, that are believed to be caused by abnormally configured, host-encoded prion proteins that accumulate in the central nervous tissue (1). CJD has an annual incidence of approximately 1 case per million population in the United States (1) and occurs in three forms: sporadic, genetically determined, and acquired by infection. In the latter form, the incubation period is measured typically in years. Recent evidence that prion infection can cross the species barrier between humans and cattle has raised increasing public health concerns about the possible transmission to humans of a TSE among deer and elk known as chronic wasting disease (CWD) (2). During 1993–1999, three men who participated in wild game feasts in northern Wisconsin died of degenerative neurologic illnesses. This report documents the investigation of these deaths, which was initiated in August 2002 and which confirmed the death of only one person from CJD. Although no association between CWD and CJD was found, continued surveillance of both diseases remains important to assess the possible risk for CWD transmission to humans. Case Reports Case 1. In December 1992, a Wisconsin man aged 66 years with a history of seizures since 1969 sought treatment for recurring seizures, increasing forgetfulness, and worsening hand tremors. Electroencephalographic (EEG) examination demonstrated focal epileptiform activity and nonspecific diffuse abnormalities, but no specific diagnosis was made. In February 1993, he was hospitalized for increasing confusion, ataxia, and movement tremors of his extremities. A magnetic resonance image (MRI) demonstrated mild, nonspecific enhancement along the inferior parasagittal occipital lobe. A repeat EEG showed bifrontal intermittent, short-interval, periodic sharp waves, suggesting a progressive encephalopathy; a diagnosis of CJD was suspected. The man died later that month; neuropathologic examination of brain tissue during autopsy indicated subacute spongiform encephalopathy, compatible with CJD. The man was a lifelong hunter who ate venison frequently. He hunted primarily in northern Wisconsin but also at least once in Montana. He hosted wild game feasts at his cabin in northern Wisconsin from 1976 until shortly before his death. Fixed brain tissue obtained during the autopsy was sent for analysis to the National Prion Disease Pathology Surveillance Center (NPDPSC) and reexamined at the institution where the autopsy was conducted. Histopathologic examination did not substantiate the diagnosis of prion disease. In addition, 27 brain tissue sections were negative for prions by immunostaining despite positive antibody reactions against other proteins (controls), which indicated that other epitopes in the tissue samples were preserved. Case 2. In May 1999, a Minnesota man aged 55 years with no previous history of a neurologic disease sought evaluation and treatment following a 3-month history of progressive difficulty in writing and unsteadiness of gait. A computerized 126 MMWR February 21, 2003 SUGGESTED CITATION Centers for Disease Control and Prevention. [Article Title]. MMWR 2003;52:[inclusive page numbers]. Centers for Disease Control and Prevention Julie L. Gerberding, M.D., M.P.H. Director David W. Fleming, M.D. Deputy Director for Public Health Science Dixie E. Snider, Jr., M.D., M.P.H. Associate Director for Science Epidemiology Program Office Stephen B. Thacker, M.D., M.Sc. Director Office of Scientific and Health Communications John W. Ward, M.D. Director Editor, MMWR Series Suzanne M. Hewitt, M.P.A. Managing Editor, MMWR Series David C. Johnson (Acting) Lead Technical Writer/Editor Jude C. Rutledge Teresa F. Rutledge Jeffrey D. Sokolow, M.A. Writers/Editors Lynda G. Cupell Malbea A. Heilman Visual Information Specialists Quang M. Doan Erica R. Shaver Information Technology Specialists Division of Public Health Surveillance and Informatics Notifiable Disease Morbidity and 122 Cities Mortality Data Robert F. Fagan Deborah A. Adams Felicia J. Connor Lateka Dammond Patsy A. Hall Pearl C. Sharp The MMWR series of publications is published by the Epidemiology Program Office, Centers for Disease Control and Prevention (CDC), U.S. Department of Health and Human Services, Atlanta, GA 30333. tomography (CT) scan and MRI examination of his head did not indicate any abnormality. In June 1999, he was hospitalized following onset of dementia, speech abnormalities, and myoclonic jerking. An EEG indicated left-hemispheric periodic sharp waves and moderate generalized background slowing; CJD was diagnosed clinically. In July 1999, following worsening symptoms and development of right upper extremity dystonia, the patient died. Neuropathologic evaluation of brain tissue during autopsy demonstrated widespread subcortical spongiform lesions, consistent with CJD. The man was not a hunter but had a history of eating venison. He made an estimated 12 visits to the cabin where the wild game feasts were held, but he participated in only one feast during the mid-1980s. Sections of fixed and frozen brain tissue obtained during autopsy were analyzed at NPDPSC, and prion disease was confirmed by immunohistochemical and Western blot testing. The Western blot characteristics and prion disease phenotype in this patient were consistent with the most common form of sporadic CJD, classified as M/M (M/V) 1 (3). Subsequent genetic typing confirmed the presence of methionine homozygosity (M/M) at codon 129 of the patient’s prion protein gene. Case 3. In June 1992, a Wisconsin man aged 65 years sought treatment for progressive slowing of speech, worsening memory, and personality changes. By January 1993, his speech was reduced to one-word utterances. Neurologic examination showed a flat affect, decreased reflexes, and apraxia. A CT head scan showed mild atrophy, and an EEG was normal. Pick’s disease was diagnosed. By May, he was unable to perform any daily living activities; he died in August 1993. Neuropathologic evaluation of brain tissue during autopsy showed symmetrical frontal lobe cerebral cortical atrophy and mild temporal lobe atrophy. No Pick’s bodies or spongiform lesions were observed. The man had a history of eating venison and participated regularly in wild game feasts held at the cabin owned by patient 1. He was a lifelong hunter and hunted mostly in Wisconsin but also in Wyoming and British Columbia. No game was brought to the wild game feasts from his hunting trips outside of Wisconsin. Examination of fixed brain tissue sent to NPDPSC demonstrated no lesions indicative of CJD, and immunohistochemical testing with antibody to the prion protein did not demonstrate the granular deposits seen in prion diseases. Epidemiologic Investigation Wild game feasts consisting of elk, deer, antelope, and other game that occurred at a cabin in northern Wisconsin owned by patient 1 began in 1976 and continued through 2002. Vol. 52 / No. 7 MMWR 127 These feasts typically involved 10–15 participants and usually occurred on weekends before or during hunting seasons in the fall and occasionally in the spring. Wild game brought to these feasts usually were harvested in Wisconsin, but three men who attended these feasts reported hunting in the western United States and bringing game back to Wisconsin. These activities took place in Colorado (near the towns of Cortez, Trinidad, Collbran, Durango, and Meeker), Wyoming (near the towns of Gilette and Cody), and Montana (near the town of Malta). CWD was not known to be endemic in these areas at the time that these hunting activities took place. Information was obtained for 45 (85%) of 53 persons who were identified as possibly participating in the wild game feasts; all were male. Information was obtained by direct interview or from family members of decedents. Of the 45 persons, for whom information was obtained, 34 were reported to have attended wild game feasts. Seven of the 34 feast attendees were deceased, including the three patients. None of the four other decedents had a cause of death attributed to or associated with a degenerative neurologic disorder. None of the living participants had any signs or symptoms consistent with a degenerative neurologic disorder. Reported by: JP Davis, MD, J Kazmierczak, DVM, M Wegner, MD, R Wierzba, Div of Public Health, State of Wisconsin Dept of Health and Family Svcs. P Gambetti, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, Ohio. L Schonberger, MD, R Maddox, MPH, E Belay, MD, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases; V Hsu, MD, EIS Officer, CDC. Editorial Note: CWD was first described in the United States in the 1960s and classified as a TSE in 1978. Previously localized to a contiguous endemic area in northeastern Colorado and southeast Wyoming, since 2000, CWD has been found in free-ranging deer or elk in Illinois, Nebraska, New Mexico, South Dakota, Wisconsin, and outside the previously known endemic areas of Colorado and Wyoming. CWD has been identified also in captive deer or elk in Colorado, Kansas, Minnesota, Montana, Nebraska, Oklahoma, South Dakota, and Wisconsin (4). Because a variant form of CJD, with specific neuropathologic and molecular characteristics that distinguish it from sporadic CJD, has been associated with eating cattle products infected with a prion that causes bovine spongiform encephalopathy (5), concern has been raised about the possibility that the prion associated with CWD might be transmitted to humans in a similar way. In this investigation, because only one of the three cases in Wisconsin had neuropathologic confirmation of a prion disease, no association could be made between case participation in the wild game feasts and the development of CJD. Although patient 2 had confirmed CJD, he was unlikely to have eaten CWD-infected venison at these feasts because venison and other game from outside Wisconsin that was served at these feasts did not originate from known CWD-endemic areas, and the man participated in the feasts only once. In addition, the prion disease in this case was consistent with the most common form of sporadic CJD, without apparent unusual neuropathologic or molecular characteristics that might occur if the prion related to CWD had been responsible for the disease. The findings in this report are subject to at least two limitations. First, not all members participating in wild game feasts could be identified, and not all persons listed as participating could be contacted for interviews. Second, interviews that were conducted required recall of events that occurred up to 25 years ago, limiting the detail or accuracy of events. However, the similar responses obtained from different sources support the accuracy of the investigation findings. A previous investigation of unusually young CJD patients in whom the transmission of CWD was suspected also did not provide convincing evidence for a causal relationship between CWD and CJD (2). However, limited epidemiologic investigations cannot rule out the possibility that CWD might play a role in causing human illness. Ongoing surveillance of CJD, particularly in states with CWD, is important to assess the risk, if any, for CWD transmission to humans. Because the confirmation of CJD and the detection of a new prion disease require neuropathologic study of brain tissue, physicians are encouraged to contact NPDPSC (http:// www.cjdsurveillance.com; telephone, 216-368-0587) to confirm diagnoses of CJD and to distinguish its various subtypes. Because of the known severity of TSEs in humans and the possibility that the CWD prion can affect humans, animals with evidence of CWD should be excluded from the human food or animal feed chains. Hunters and wild venison consumers should follow precautionary guidelines available from the Wisconsin Department of Agriculture, Trade, and Consumer Protection (http://datcp.state.wi.us/core/consumerinfo) to prevent potential exposures to the CWD agent. References 1. Belay E. Transmissible spongiform encephalopathies in humans. Annu Rev Microbiol 1999;53:283–314. 2. Belay E, Gambetti P, Schonberger L, et al. Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Arch Neurol 2001;58:1673–8. 3. Parchi P, Giese A, Capellari S, et al. Classification of sporadic Creutzfeldt- Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999;46:224–33. 4. U.S. Department of Agriculture. Positive CWD cases: cumulative through Dec 2002 (including farm herds already depopulated). Available at http://aphisweb.aphis.usda.gov/vs/nahps//cwd/USAMapOf InfectedHerds.jpg. 5.Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt- Jakob disease in the UK. Lancet 1996;347:921–5. http://www.cdc.gov/mmwr/PDF/wk/mm5207.pdf Perspective Chronic Wasting Disease and Potential Transmission to Humans Suggested citation for this article: Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004 Jun [date cited]. Available from: http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been detected in other parts of the United States. Although detection in some areas may be related to increased surveillance, introduction of CWD due to translocation or natural migration of animals may account for some new foci of infection. Increasing spread of CWD has raised concerns about the potential for increasing human exposure to the CWD agent. The foodborne transmission of bovine spongiform encephalopathy to humans indicates that the species barrier may not completely protect humans from animal prion diseases. Conversion of human prion protein by CWD-associated prions has been demonstrated in an in vitro cell-free experiment, but limited investigations have not identified strong evidence for CWD transmission to humans. More epidemiologic and laboratory studies are needed to monitor the possibility of such transmissions. snip... Transmission to Other Animals Although CWD does not appear to occur naturally outside the cervid family, it has been transmitted experimentally by intracerebral injection to a number of animals, including laboratory mice, ferrets, mink, squirrel monkeys, and goats (1,26). In an experimental study, the CWD agent was transmitted to 3 of 13 intracerebrally injected cattle after an incubation period of 22 to 27 months (27). The susceptibility of cattle intracerebrally challenged with the agent of this disease was substantially less than that observed after intracerebral scrapie challenge: nine of nine cattle succumbed to scrapie challenge after intracerebral injection (28). In ongoing experimental studies, after >6 years of observation, no prion disease has developed in 11 cattle orally challenged with the CWD agent or 24 cattle living with infected deer herds (E.S. Williams and M.W. Miller, unpub. data) (1). In addition, domestic cattle, sheep, and goat residing in research facilities in close contact with infected cervids did not develop a prion disease. Analysis by immunohistochemical studies of the tissue distribution of prions in CWD-infected cervids identified the agent in the brain, spinal cord, eyes, peripheral nerves, and lymphoreticular tissues (Table 1) (29,30). Distribution of the CWD agent outside of the brain seems to be less widespread in elk than in deer (2). Involvement of the tonsils and peripheral nerves early in the course of experimental and natural prion infection suggests the possible involvement of the lymphoreticular and peripheral nervous systems in the pathogenesis and transmission of the disease (2,12,30,31). Risk for Transmission to Humans In 2001, the case of a 25-year-old man who reportedly died of a prion disease after an illness lasting ≈22 months was investigated (Table 2). Although this man had hunted deer only rarely, his grandfather hunted deer and elk throughout much of the 1980s and 1990s and regularly shared the venison with the case-patient's family. The grandfather primarily hunted in southeastern Wyoming, around the known CWD-endemic area. The case-patient's illness began with a seizure and progressed to fatigue, poor concentration, and depression. Memory loss, ataxia, speech abnormalities, combative behavior, and recurrent seizures also developed. Histopathologic, immunohistochemical, and Western blot testing of brain autopsy samples confirmed a prion disease diagnosis. Analysis of the prion protein gene indicated a P102L mutation coupled with valine at the polymorphic codon 129 in the mutant allele, confirming a diagnosis of Gerstmann-Sträussler-Scheinker syndrome (GSS). This case-patient was unusually young even for a person with a GSS P102L mutation. It remains unknown whether the possible exposure of the case-patient to CWD-infected venison potentially contributed to the early onset of his prion disease. In 2001, two additional CJD patients 26 and 28 years of age were reported from a single state (Table 2) (34). The patients grew up in adjacent counties and had illness onset within several months of each other. As a result of this fact and their unusually young age, a possible environmental source of infection, including exposure to CWD-infected venison, was considered. One of the patients died after an illness lasting 5–6 months that was characterized by progressive aphasia, memory loss, social withdrawal, vision disturbances, and seizure activity leading to status epilepticus and induced coma. Histopathologic, immunohistochemical, and Western blot testing of brain biopsy and autopsy samples confirmed a CJD diagnosis. The patient's disease phenotype corresponded to the MM2 sporadic CJD subtype reported by Parchi et al. (35). This patient did not hunt, and family members provided no history of regularly eating venison. The patient may have occasionally eaten venison originating from the Upper Peninsula of Michigan while away from home during his college years. However, ongoing surveillance has not detected CWD in Michigan deer (36). The second patient died from an illness lasting <16 months. The patient's illness began with behavioral changes, including unusual outbursts of anger and depression. Confusion, memory loss, gait disturbances, incontinence, headaches, and photophobia also developed. Western blot analysis of frozen brain biopsy tissue confirmed a prion disease diagnosis. Immunohistochemical analysis of brain tissue obtained after the patient's death showed prion deposition consistent with GSS. A prion protein gene analysis could not be performed because appropriate samples were lacking. However, prion protein gene analysis of a blood sample from one of the patient's parents indicated a GSS P102L mutation. The patient did not hunt but may have eaten venison from Michigan once when he was 1–2 years old. The GSS diagnosis greatly reduced the likelihood that the two patients reported from adjacent counties had disease with a common origin. Recently, rare neurologic disorders resulting in the deaths of three men who participated in "wild game feasts" in a cabin owned by one of the decedents created concern about the possible relationship of their illnesses with CWD (Table 2) (37). Two of the patients reportedly died of CJD, and the third died from Pick's disease. More than 50 persons were identified as possibly participating in these feasts; the three patients were the only participants reported to have died of a degenerative neurologic disorder. Reanalysis of autopsy brain tissues from the three patients at the National Prion Disease Pathology Surveillance Center indicated that two of them had no evidence of a prion disease by immunohistochemical analysis. CJD was confirmed in the third patient, who had clinicopathologic, codon 129, and prion characteristics similar to the most common sporadic CJD subtype (MM1/MV1) (35). This patient participated in the feasts only once, perhaps in the mid-1980s. In addition, the investigation found no evidence that the deer and elk meat served during the feasts originated from the known CWD-endemic areas of Colorado and Wyoming. In 2003, CJD in two deer and elk hunters (54 and 66 years of age) was reported (38). The report implied that the patients had striking neuropathologic similarities and that their illness may represent a new entity in the spectrum of prion diseases. A third patient (63 years of age), who was also purported to have been a big game hunter, was subsequently reported from the same area. However, none of the three patients were reported to have eaten venison from the CWD-endemic areas of the western United States. The 66-year-old patient hunted most of his life in Washington State. Although information about the 54-year-old patient was limited, there was no evidence that he hunted in CWD-endemic areas. The third patient was not a hunter but ate venison harvested from Pennsylvania and Washington. The neuropathologic changes, Western blot profile, and genotype at codon 129 of the three patients each fit the MM1, VV1, or VV2 sporadic CJD subtype, indicating absence of phenotypic similarity among the cases or atypical neuropathologic features (35). To date, only two nonfamilial CJD cases with a positive history of exposure to venison obtained from the known CWD-endemic areas have been reported. One of the patients was a 61-year-old woman who grew up in an area where this disease is known to be endemic, and she ate venison harvested locally. She died in 2000, and analysis of autopsy brain specimens confirmed that the patient's CJD phenotype fit the MM1 subtype, with no atypical neuropathologic features. The second patient was a 66-year-old man who was reported to have eaten venison from two deer harvested in a CWD-endemic area. Both deer tested negative for CWD, and the patient's illness was consistent with the MM1 CJD phenotype. Despite the decades-long endemicity of CWD in Colorado and Wyoming, the incidence of CJD and the age distribution of CJD case-patients in these two states are similar to those seen in other parts of the United States. From 1979 to 2000, 67 CJD cases from Colorado and 7 from Wyoming were reported to the national multiple cause-of-death database. The average annual age-adjusted CJD death rate was 1.2 per million persons in Colorado and 0.8 in Wyoming. The proportion of CJD patients who died before age 55 in Colorado (13.4%) was similar to that of the national (10.2%). The only CJD case-patient <30 years of age in Colorado had iatrogenic CJD linked to receipt of human growth hormone injections. CJD was not reported in persons <55 years of age in Wyoming during the 22-year surveillance period. ...snip...end http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm Research Authors Hamir, Amirali Interpretive Summary: This communication reports final observations on experimental transmission of chronic wasting disease (CWD) from mule deer to cattle. Thirteen calves were inoculated into the brain with brain suspension from mule deer naturally affected with CWD. Three other calves were kept as uninoculated controls. The experiment was terminated 6 years post inoculation (PI). During that time, abnormal prion protein was demonstrated in the brain and spinal cord of 5 cattle by laboratory tests. However, consistent clinical signs and microscopic changes were not seen in any of these cattle. Age related changes were seen in both inoculated and control cattle. Findings of this study show that only 38% of the inoculated cattle were positive for CWD agent. Although inoculation directly into the brain is an unnatural route of exposure, and is the most severe challenge possible, this experiment shows that CWD transmission in cattle could have long incubation periods (up to 5 years). This finding suggests that oral exposure of cattle to CWD agent, a more natural potential route of exposure, would require not only a much larger dose of inoculum, but also, may not result in amplification of CWD agent within brain and spinal cord tissues during the normal lifespan of cattle. It is possible that a second bovine passage of material (cattle brain infected with CWD) from this study may result in a larger incidence of affected cattle with a shortened incubation time, and may produce different clinical and pathological findings. Such a study is now in progress. Also, experimental inoculations of cattle with CWD isolates from white-tailed deer and elk are needed to compare findings with the present study and these studies will be initiated in the near future. Impact: Results of this study show that although cattle inoculated directly into the brain with CWD succumb to the disease, the attack rate was rather small (38%) with this unnatural route of transmission. It is speculated that the oral route of infection may not result in replication of the agent during normal lifespan of cattle. North American Equity ResearchNew York13 January 2004 BSE (Mad Cow) Update: · There have been seven cases of human sCJD clusters identified in the The second table, below, shows what portion of the state's total The CDC Is Currently Investigating the New Jersey Cluster JPMorgan Equity Research Ratings Distribution, as of December 31, 2003 THE most disturbing factor of this topic are the new atypical TSEs BSE prions propagate as either variant CJD-like or sporadic CJD-like http://embojournal.npgjournals.com/cgi/content/full/21/23/6358 THE new findings of BASE in cattle in Italy of Identification of a http://www.pnas.org/cgi/content/abstract/0305777101v1 Characterization of two distinct prion strains http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471 Adaptation of the bovine spongiform encephalopathy agent to primates http://www.pnas.org/cgi/content/full/041490898v1 In an experimental study of the transmissibility of BSE to the pig, http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?holding=npg&cmd=Retrieve&db=PubMed&list_uids=10684682&dopt=Abstract THE recent discoveries of previously unidentified strains of FULL TEXT APPRX. 91 PAGES UK Strategy for Research and 2004-2007 http://www.mrc.ac.uk/pdf-uk_strategy_v5.2.pdf WHEN in fact, the findings from Marsh and the findings at In Confidence - Perceptions of unconventional slow virus diseases of http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf Evidence That Transmissible Mink Encephalopathy http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf In Confidence - Perceptions of unconventional slow virus diseases http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf Gerald Wells: Report of the Visit to USA, April-May 1989 snip... The general opinion of those present was that BSE, as an snip... It is clear that USDA have little information and _no_ regulatory snip... 3. Prof. A. Robertson gave a brief account of BSE. The US approach snip... http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf To be published in the Proceedings of the Evidence That Transmissible Mink Encephalopathy R.F. Marsh* and G.R. Hartsough •Department of Veterinary Science, University of Wisconsin-Madison, Madison, ABSTRACT INTRODUCTION Transmissible mink encephalopathy (TME) was first reported in 1965 by Hartsough OBSERVATIONS AND RESULTS A New Incidence of TME. In April of 1985, a mink rancher in Stetsonville, Wisconsin Experimental Transmission. The clinical diagnosis of TME was confirmed by DISCUSSION ACKNOWLEDGEMENTS REFERENCES MARSH http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf snip... A The Present Position with respect to Scrapie Scrapie is a natural disease of sheep and goats. It is a slow The field problem has been reviewed by a MAFF working group It is clear that scrapie in sheep is important commercially and Recently the question has again been brought up as to whether Whether true or not. the hypothesis that these agents might be snip... 76/10.12/4.6 http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf http://www.bseinquiry.gov.uk/files/yb/1976/10/12002001.pdf The BSE Inquiry / Statement No 61D Professor Robert Will Issued 16/12/1999 (Scheduled to give oral evidence 16 December 1999) snip... 23 54. In February 1996, Dr M Zeidler drew up a list of cases of CJD aged less than 30 years identified from the world literature (provided with my first statement [WS061]). I contacted colleagues in other countries to ask for specific information on four of these cases. In early March I received telephone calls from colleagues confirming that: · Plaques had not been identified on neuropathological examination in three cases of CJD aged 14,16 and 20 years from the USA and Canada. · That the single case of sporadic CJD that we had identified in the world literature aged less than 30 years with plaque deposition was probably a hereditary form of human prion disease. snip... http://www.bseinquiry.gov.uk/files/ws/s061d.pdf HOUND STUDY AS implied in the Inset 25 we must not _ASSUME_ that snip... http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf 76 pages on hound study; http://www.bseinquiry.gov.uk/files/sc/seac16/tab04.pdf DEFRA GTN: Mr T S Singeltary 21 November 2001 Dear Mr Singeltary TSE IN HOUNDS Thank you for e-mail regarding the hounds survey. I am sorry for the long delay in responding. As you note, the hound survey remains unpublished. However the Spongiform Encephalopathy Advisory Committee (SEAC), the UK Government's independent Advisory Committee on all aspects related to BSE-like disease, gave the hound study detailed consideration at their meeting in January 1994. As a summary of this meeting published in the BSE inquiry noted, the Committee were clearly concerned about the work that had been carried out, concluding that there had clearly been problems with it, particularly the control on the histology, and that it was more or less inconclusive. However was agreed that there should be a re-evaluation of the pathological material in the study. Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be gained from it. The Chairman concluded that there were varying opinions within the Committee on further work. It did not suggest any further transmission studies and thought that the lack of clinical data was a major weakness. Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the authors felt that it would not stand up to peer review and hence it was never published. As noted above, and in the detailed minutes of the SEAC meeting in June 95, SEAC considered whether additional work should be performed to examine dogs for evidence of TSE infection. Although the Committee had mixed views about the merits of conducting further work, the Chairman noted that when the Southwood Committee made their recommendation to complete an assessment of possible spongiform disease in dogs, no TSEs had been identified in other species and hence dogs were perceived as a high risk population and worthy of study. However subsequent to the original recommendation, made in 1990, a number of other species had been identified with TSE ( e.g. cats) so a study in hounds was less critical. For more details see- As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. Thus unfortunately, I am unable to help with your request to supply information on the hound survey directly. My only suggestion is that you contact one of the researchers originally involved in the project, such as Gerald Wells. He can be contacted at the following address. Dr Gerald Wells, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey, KT 15 3NB, UK You may also wish to be aware that since November 1994 all suspected cases of spongiform encephalopathy in animals and poultry were made notifiable. Hence since that date there has been a requirement for vets to report any suspect SE in dogs for further investigation. To date there has never been positive identification of a TSE in a dog. I hope this is helpful Yours sincerely 4 HUGH MCDONAGH Mr. Singeltary. I hope this finds you well. As you are aware I left the USDA last year. I can only update you on the sheep before that time. Contact was established with the UK on doing the bioassay studies. They agreed. However, we were prioritized after their own needs, hence the delay. I am aware that there are now additional labs in Europe running the mouse bioassay strain typing. You will have to contact USDA for further word. ========== Dear Mr SingeltaryThank you for you enquiry to the SEAC secretariat about mouse bioassayscommissioned by the USDA to investigate TSE cases in imported sheep. After making a number of enquiries, it appears that Defra were not involvedwith this work. However, it is possible that a UK research laboratory wascontacted by the USDA about such tests but I have been unable to find outany further information. You may wish to make further enquiries with theUSDA. Yours sincerely Tom Barlow -----Original Message----- From: Terry S. Singeltary Sr. [mailto:flounder@wt.net] Hello Tabitha, A kind greetings from Texas. ...snip...end We have to be careful that we don't get so set in the way we do things that Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't Dr. Keller: Tissues are routinely tested, based on which tissue provides an Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't ============================================== On August 17, 2005, no inconclusive test results were reported. National Veterinary Services Laboratory (NVSL) Immunohistochemistry (IHC) The BSE enhanced surveillance program involves the use of a rapid screening It should be noted that since This was done to ensure that samples inappropriate for the http://www.aphis.usda.gov/lpa/issues/bse/surveillance/figure2f.html Aug. 15, 2005, 11:39PM 1,000 citations issued on mad cow regulations U.S. government inspectors cited meatpackers more than 1,000 times over a "No specified risk materials got into the food supply" as a result of any of The release of the noncompliance reports comes as Japan, normally the Many of the violations cited by the USDA were related to paperwork mistakes, Consumer group Public Citizen said it was still reviewing all the documents The meat industry disagreed. "Some groups will no doubt attempt to use this information as evidence of The USDA ordered that the risk materials be removed from slaughtered cattle The second case was confirmed in June, at an undisclosed location in Texas. The noncompliance citations generally broke down into five categories: not Reuters News contributed to this report. TSS
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