Chronic Wasting Disease Found In A White-Tailed Deer In Maryland

Chronic Wasting Disease Found In A White-Tailed Deer In Maryland


Annapolis, Md. (February 10, 2011) — The Maryland Department of Natural Resources (DNR) received laboratory confirmation on February 10, 2011 that a white-tailed deer harvested in Maryland tested positive for chronic wasting disease (CWD). This is the first confirmed case of CWD in Maryland. A hunter in Allegany County reported taking the deer on November 27, 2010 in Green Ridge State Forest. Maryland joins 20 other states and Canadian provinces with CWD documented in deer, elk or moose.





"Our team of wildlife professionals has been preparing for this result for some time so we are well-informed and ready to limit the impact of this event,” said Paul Peditto, Director of DNR’s Wildlife and Heritage Service. “We have sampled intensively for this disease since 2002 and see this as an unfortunate but somewhat inevitable outcome. The good news is that our preparation and planning ensure a sound scientific foundation for our response to this single positive test result. With the continued cooperation of hunters, farmers, deer processors and landowners who have supported our monitoring effort, we will manage this deer disease consistent with the best available science and with minimal impact on our deer population and the people who enjoy these great animals.”





“Concerns over CWD should not stop anyone from enjoying venison,” added Peditto, who explained that only four species of the deer family are known to be susceptible to CWD: elk, mule deer, moose and white-tailed deer. Of these, only the white-tailed deer occurs in the wild in Maryland and there are no reported cases of transmission to humans or other animals.





As always, hunters are advised to exercise caution and never consume the meat of sick animals. Hunters are also advised to avoid contact with the brain, spinal column or lymph nodes of deer — all of which are normally removed during the butchering process.





This is the first positive sample out of nearly 6,800 deer tested in Maryland since 1999. From 2002 until 2009 that sampling occurred statewide. In 2010, sampling efforts were focused on Allegany and western Washington counties due to the presence of positive cases in nearby West Virginia and Virginia. West Virginia first detected CWD in Hampshire County in 2005 and it was found in Frederick County, Virginia in early 2010.





“Maryland will continue to work closely with the wildlife professionals in our adjacent states to share information and coordinate response efforts. However, our primary goal is to ensure the public is fully-informed and knows what we know when we know it. We want to be certain that every interested Marylander understands this disease and recognizes that there is no risk to people, pets or domestic livestock. As in every other state with CWD, we will respond appropriately while ultimately learning to live with this disease with little impact to our wildlife or citizens,” Peditto concluded.

For more information on CWD in Maryland and the DNR Response Plan, please visit the DNR Website at







http://www.dnr.maryland.gov/wildlife/Hunt_Trap/deer/disease/cwdinformation.asp






http://www.dnr.state.md.us/dnrnews/pressrelease2011/021011a.asp







Wednesday, November 16, 2011

Chronic wasting disease found in Big Horn basin deer Wyoming's deer hunt area 165



http://chronic-wasting-disease.blogspot.com/2011/11/chronic-wasting-disease-found-in-big.html






Wednesday, November 16, 2011

Wisconsin Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011



http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/wisconsin-creutzfeldt-jakob-disease-cwd.html






COLORADO




Sunday, November 13, 2011




COLORADO CWD CJD TSE PRION REPORTING 2011




http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/colorado-cwd-cjd-tse-prion-reporting.html








WYOMING




Monday, November 14, 2011




WYOMING Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011




http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/wyoming-creutzfeldt-jakob-disease-cwd.html








Monday, January 05, 2009

CWD, GAME FARMS, BAITING, AND POLITICS



Saturday, September 06, 2008


Chronic wasting disease in a Wisconsin white-tailed deer farm 79% INFECTION RATE Contents: September 1 2008, Volume 20, Issue 5

Journal of Veterinary Diagnostic Investigation Vol. 20 Issue 5, 698-703 Copyright © 2008 by the American Association of Veterinary Laboratory Diagnosticians


--------------------------------------------------------------------------------



Case Reports


Chronic wasting disease in a Wisconsin white-tailed deer farm


Delwyn P. Keane1, Daniel J. Barr, Philip N. Bochsler, S. Mark Hall, Thomas Gidlewski, Katherine I. O'Rourke, Terry R. Spraker and Michael D. Samuel Correspondence: 1Corresponding Author: Delwyn Keane, University of Wisconsin, Wisconsin Veterinary Diagnostic Laboratory, 445 Easterday Lane, Madison, WI 53706. mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!x-usc:mailto:Delwyn.Keane@wvdl.wisc.edu


In September 2002, chronic wasting disease (CWD), a prion disorder of captive and wild cervids, was diagnosed in a white-tailed deer (Odocoileus virginianus) from a captive farm in Wisconsin. The facility was subsequently quarantined, and in January 2006 the remaining 76 deer were depopulated. Sixty animals (79%) were found to be positive by immunohistochemical staining for the abnormal prion protein (PrPCWD) in at least one tissue; the prevalence of positive staining was high even in young deer. Although none of the deer displayed clinical signs suggestive of CWD at depopulation, 49 deer had considerable accumulation of the abnormal prion in the medulla at the level of the obex. Extraneural accumulation of the abnormal protein was observed in 59 deer, with accumulation in the retropharyngeal lymph node in 58 of 59 (98%), in the tonsil in 56 of 59 (95%), and in the rectal mucosal lymphoid tissue in 48 of 58 (83%). The retina was positive in 4 deer, all with marked accumulation of prion in the obex. One deer was considered positive for PrPCWD in the brain but not in the extraneural tissue, a novel observation in white-tailed deer. The infection rate in captive deer was 20-fold higher than in wild deer. Although weakly related to infection rates in extraneural tissues, prion genotype was strongly linked to progression of prion accumulation in the obex. Antemortem testing by biopsy of recto–anal mucosal-associated lymphoid tissue (or other peripheral lymphoid tissue) may be a useful adjunct to tonsil biopsy for surveillance in captive herds at risk for CWD infection.



http://jvdi.org/cgi/content/abstract/20/5/698?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=prion&searchid=1&FIRSTINDEX=0&volume=20&issue=5&resourcetype=HWCIT






PLEASE NOTE 76 DEER WERE DEPOPULATED. SIXTY ANIMALS (79%) WERE FOUND TO BE POSITIVE BY IMMUNOHISTOCHEMICAL STAINING FOR THE ABNORMAL PRION PROTEIN (PrPCWD) IN AT LEAST ONE TISSUE; THE PREVALENCE OF POSITIVE STAINING WAS HIGH IN YOUNG DEER. ...TSS



Title: Chronic wasting disease in a Wisconsin captive white-tailed deer farm


Authors


Keane, Delwyn - U OF WIS, WIS VET DIAG LA Barr, Daniel - U OF WIS, WIS VET DIAG LA Bochsler, Philip - U OF WIS, WIS VET DIAG LA Hall, S - USDA, APHIS, VS, NVSL Gidlewski, Thomas - USDA, APHIS, VS O`rourke, Katherine Spraker, Terry - CO STATE UNIVERSITY Samuel, Michael - US GEOLOGIC SERVICE


Submitted to: Journal of Veterinary Diagnostic Investigation Publication Type: Peer Reviewed Journal Publication Acceptance Date: May 5, 2008 Publication Date: N/A



Interpretive Summary:


Chronic wasting disease is a fatal disease of deer and elk. Clinical signs, including weight loss, frequent urination, excessive thirst, and changes in behavior and gait, have been reported in mule deer and elk with this disorder. Clinical signs in captive white tailed deer are less well understood. In a previous study, a captive facility housed 200 deer, of which half were positive for the disease with no clinical signs reported. In this study, we examined 78 white tailed deer from a captive facility with a history of chronic wasting disease and no animals with clinical signs. Examination of the brain and lymph nodes demonstrated that the abnormal prion protein, a marker for disease, was observed in 60 of the deer. Biopsy of the rectal mucosa, a test that can be performed on live deer, detected 83% of the infected animals. The prion genetics of the deer was strongly linked to the rate of infection and to disease progression. The results demonstrate that clinical signs are a poor indicator of the disease in captive white tailed deer and that routine testing of live deer and comprehensive necropsy surveillance may be needed to identify infected herds. Technical Abstract: Chronic wasting disease CWD is a transmissible spongiform encephalopathy or prion disease of deer and elk in North America. All diseases in this family are characterized by long preclinical incubation periods following by a relatively short clinical course. Endpoint disease is characterized by extensive deposits of aggregates of the abnormal prion protein in the central nervous system,. In deer, the abnormal prion proteins accumulate in some peripheral lymphoid tissues early in disease and are therefore suitable for antemortem and preclinical postmortem diagnostics and for determining disease progression in infected deer. In this study, a herd of deer with previous CWD diagnoses was depopulated. No clinical suspects were identified at that time. Examination of the brain and nodes demonstrated that 79% of the deer were infected. Of the deer with abnormal prion in the peripheral lymphoid system, the retropharyngeal lymph node was the most reliable diagnostic tissue. Biopsy of the rectal mucosal tissue, a site readily sampled in the restrained or chemically immobilized deer, provided an accurate diagnosis in 83% of the infected deer. The retina in the eye of the deer was positive only in late stage cases. This study demonstrated that clinical signs are a poor indicator of disease, supports the use of the retropharyngeal lymph node as the most appropriate postmortem sample, and supports a further evaluation of the rectal mucosal tissue biopsy as an antemortem test on a herd basis.



http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=218153





http://chronic-wasting-disease.blogspot.com/2008/11/commentary-crimes-hurt-essence-of.html





http://chronic-wasting-disease.blogspot.com/2009/01/cwd-game-farms-baiting-and-politics.html





Thursday, August 28, 2008

cwd, feeding, and baiting piles



A. ORAL TRANSMISSION to deer and elk is a very efficient mode for transmission. HIGH PROTEIN FEED will transmit CWD. high protein feed have been fed to deer and elk, especially in game farms.



B. COMMINGLING OF DISEASED ANIMALS AROUND BAIT PILE. chance of the environment in the surrounding areas becoming infected, from feces, urine, shedding, becoming a hot bed for animals to feed, congregate, and become exposed, and there is still very much the possibility of lateral transmission, especially with the CWD TSE, in deer and elk.



WHY ignore sound science ?



IT's the same with pouring a bottle of 100% deer urine scent formulas all over you and the environment, this is a proven mode of transmission. SO WHY DO IT?



leave your egos, and testosterone at the door, and look at the transmission studies, this is NOT rocket science. ...TSS






http://chronic-wasting-disease.blogspot.com/2008/08/cwd-feeding-and-baiting-piles.html









Saturday, November 12, 2011


Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease

Fri, 22 Sep 2006 09:05:59 -0500


http://chronic-wasting-disease.blogspot.com/2011/11/human-prion-disease-and-relative-risk.html







Monday, June 27, 2011


Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates


http://chronic-wasting-disease.blogspot.com/2011/06/zoonotic-potential-of-cwd-experimental.html









Wednesday, January 5, 2011



ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011 Prions


David W. Colby1,* and Stanley B. Prusiner1,2


+ Author Affiliations


1Institute for Neurodegenerative Diseases, University of California, San Francisco, San Francisco, California 94143 2Department of Neurology, University of California, San Francisco, San Francisco, California 94143 Correspondence: stanley@ind.ucsf.edu



SNIP...



Greetings,



I believe the statement and quote below is incorrect ;



"CWD has been transmitted to cattle after intracerebral inoculation, although the infection rate was low (4 of 13 animals [Hamir et al. 2001]). This finding raised concerns that CWD prions might be transmitted to cattle grazing in contaminated pastures."



Please see ;



Within 26 months post inoculation, 12 inoculated animals had lost weight, revealed abnormal clinical signs, and were euthanatized. Laboratory tests revealed the presence of a unique pattern of the disease agent in tissues of these animals. These findings demonstrate that when CWD is directly inoculated into the brain of cattle, 86% of inoculated cattle develop clinical signs of the disease.



http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=194089






"although the infection rate was low (4 of 13 animals [Hamir et al. 2001])."




shouldn't this be corrected, 86% is NOT a low rate. ...




kindest regards,



Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518




MARCH 1, 2011


UPDATED CORRESPONDENCE FROM AUTHORS OF THIS STUDY I.E. COLBY, PRUSINER ET AL, ABOUT MY CONCERNS OF THE DISCREPANCY BETWEEN THEIR FIGURES AND MY FIGURES OF THE STUDIES ON CWD TRANSMISSION TO CATTLE ;



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


From: David Colby


To: flounder9@verizon.net


Cc: stanley@XXXXXXXX


Sent: Tuesday, March 01, 2011 8:25 AM


Subject: Re: FW: re-Prions David W. Colby1,* and Stanley B. Prusiner1,2 + Author Affiliations


Dear Terry Singeltary,


Thank you for your correspondence regarding the review article Stanley Prusiner and I recently wrote for Cold Spring Harbor Perspectives. Dr. Prusiner asked that I reply to your message due to his busy schedule. We agree that the transmission of CWD prions to beef livestock would be a troubling development and assessing that risk is important. In our article, we cite a peer-reviewed publication reporting confirmed cases of laboratory transmission based on stringent criteria. The less stringent criteria for transmission described in the abstract you refer to lead to the discrepancy between your numbers and ours and thus the interpretation of the transmission rate. We stand by our assessment of the literature--namely that the transmission rate of CWD to bovines appears relatively low, but we recognize that even a low transmission rate could have important implications for public health and we thank you for bringing attention to this matter.


Warm Regards, David Colby


--


David Colby, PhDAssistant ProfessorDepartment of Chemical EngineeringUniversity of Delaware




====================END...TSS==============




SNIP...SEE FULL TEXT ;



http://betaamyloidcjd.blogspot.com/2011/01/enlarging-spectrum-of-prion-like.html






UPDATED DATA ON 2ND CWD STRAIN


Wednesday, September 08, 2010


CWD PRION CONGRESS SEPTEMBER 8-11 2010


http://chronic-wasting-disease.blogspot.com/2010/09/cwd-prion-2010.html







http://chronic-wasting-disease.blogspot.com/






http://transmissiblespongiformencephalopathy.blogspot.com/








LAYPERSON



Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA


TSS

North Dakota Another 3F2 Mule Deer Tests Positive for CWD

News Release Archives - November 2011



November 22, 2011


Another 3F2 Mule Deer Tests Positive for CWD

A mule deer taken from unit 3F2 during opening weekend of the deer gun season has tested positive for chronic wasting disease.



Dr. Dan Grove, North Dakota Game and Fish Department wildlife veterinarian, said a hunter shot a doe in western Grant County and submitted the head for testing as part of the hunter-harvested surveillance program. Testing was performed at Michigan State University. As of Tuesday, Nov. 22, Game and Fish was awaiting verification of initial tests results from a lab at Iowa State University.



“According to the hunter, the animal looked healthy,” Grove said. “It showed no visible signs of having any health issues.”



This is the third deer to test positive for CWD, and all three were from taken from unit 3F2 in southwestern North Dakota. The first two were during the 2009 and 2010 deer gun seasons. All three were within 15 miles of each other.



“The latest positive emphasizes the importance of continued monitoring along with current and expanding CWD restrictions in and around this unit,” Grove said.



The hunter-harvested surveillance program annually collects samples taken from hunter-harvested deer in specific regions of the state. In addition to unit 3F2, samples during the 2011 deer gun season were collected from units in the central third of the state.



CWD affects the nervous system of members of the deer family and is always fatal. Scientists have found no evidence that CWD can be transmitted naturally to humans or livestock.





http://gf.nd.gov/multimedia/news/2011/11/111108.html







Monday, December 13, 2010


North Dakota Another Deer From 3F2 Tests Positive for CWD


http://chronic-wasting-disease.blogspot.com/2010/12/north-dakota-another-deer-from-3f2.html





Wednesday, November 16, 2011

Chronic wasting disease found in Big Horn basin deer Wyoming's deer hunt area 165



http://chronic-wasting-disease.blogspot.com/2011/11/chronic-wasting-disease-found-in-big.html






Wednesday, November 16, 2011


Wisconsin Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011



http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/wisconsin-creutzfeldt-jakob-disease-cwd.html






COLORADO




Sunday, November 13, 2011




COLORADO CWD CJD TSE PRION REPORTING 2011




http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/colorado-cwd-cjd-tse-prion-reporting.html







WYOMING




Monday, November 14, 2011




WYOMING Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011




http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/wyoming-creutzfeldt-jakob-disease-cwd.html





Monday, January 05, 2009


CWD, GAME FARMS, BAITING, AND POLITICS



Saturday, September 06, 2008


Chronic wasting disease in a Wisconsin white-tailed deer farm 79% INFECTION RATE Contents: September 1 2008, Volume 20, Issue 5

Journal of Veterinary Diagnostic Investigation Vol. 20 Issue 5, 698-703 Copyright © 2008 by the American Association of Veterinary Laboratory Diagnosticians


--------------------------------------------------------------------------------



Case Reports


Chronic wasting disease in a Wisconsin white-tailed deer farm


Delwyn P. Keane1, Daniel J. Barr, Philip N. Bochsler, S. Mark Hall, Thomas Gidlewski, Katherine I. O'Rourke, Terry R. Spraker and Michael D. Samuel Correspondence: 1Corresponding Author: Delwyn Keane, University of Wisconsin, Wisconsin Veterinary Diagnostic Laboratory, 445 Easterday Lane, Madison, WI 53706. mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000040/!x-usc:mailto:Delwyn.Keane@wvdl.wisc.edu


In September 2002, chronic wasting disease (CWD), a prion disorder of captive and wild cervids, was diagnosed in a white-tailed deer (Odocoileus virginianus) from a captive farm in Wisconsin. The facility was subsequently quarantined, and in January 2006 the remaining 76 deer were depopulated. Sixty animals (79%) were found to be positive by immunohistochemical staining for the abnormal prion protein (PrPCWD) in at least one tissue; the prevalence of positive staining was high even in young deer. Although none of the deer displayed clinical signs suggestive of CWD at depopulation, 49 deer had considerable accumulation of the abnormal prion in the medulla at the level of the obex. Extraneural accumulation of the abnormal protein was observed in 59 deer, with accumulation in the retropharyngeal lymph node in 58 of 59 (98%), in the tonsil in 56 of 59 (95%), and in the rectal mucosal lymphoid tissue in 48 of 58 (83%). The retina was positive in 4 deer, all with marked accumulation of prion in the obex. One deer was considered positive for PrPCWD in the brain but not in the extraneural tissue, a novel observation in white-tailed deer. The infection rate in captive deer was 20-fold higher than in wild deer. Although weakly related to infection rates in extraneural tissues, prion genotype was strongly linked to progression of prion accumulation in the obex. Antemortem testing by biopsy of recto–anal mucosal-associated lymphoid tissue (or other peripheral lymphoid tissue) may be a useful adjunct to tonsil biopsy for surveillance in captive herds at risk for CWD infection.



http://jvdi.org/cgi/content/abstract/20/5/698?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=prion&searchid=1&FIRSTINDEX=0&volume=20&issue=5&resourcetype=HWCIT






PLEASE NOTE 76 DEER WERE DEPOPULATED. SIXTY ANIMALS (79%) WERE FOUND TO BE POSITIVE BY IMMUNOHISTOCHEMICAL STAINING FOR THE ABNORMAL PRION PROTEIN (PrPCWD) IN AT LEAST ONE TISSUE; THE PREVALENCE OF POSITIVE STAINING WAS HIGH IN YOUNG DEER. ...TSS



Title: Chronic wasting disease in a Wisconsin captive white-tailed deer farm


Authors


Keane, Delwyn - U OF WIS, WIS VET DIAG LA Barr, Daniel - U OF WIS, WIS VET DIAG LA Bochsler, Philip - U OF WIS, WIS VET DIAG LA Hall, S - USDA, APHIS, VS, NVSL Gidlewski, Thomas - USDA, APHIS, VS O`rourke, Katherine Spraker, Terry - CO STATE UNIVERSITY Samuel, Michael - US GEOLOGIC SERVICE


Submitted to: Journal of Veterinary Diagnostic Investigation Publication Type: Peer Reviewed Journal Publication Acceptance Date: May 5, 2008 Publication Date: N/A



Interpretive Summary:


Chronic wasting disease is a fatal disease of deer and elk. Clinical signs, including weight loss, frequent urination, excessive thirst, and changes in behavior and gait, have been reported in mule deer and elk with this disorder. Clinical signs in captive white tailed deer are less well understood. In a previous study, a captive facility housed 200 deer, of which half were positive for the disease with no clinical signs reported. In this study, we examined 78 white tailed deer from a captive facility with a history of chronic wasting disease and no animals with clinical signs. Examination of the brain and lymph nodes demonstrated that the abnormal prion protein, a marker for disease, was observed in 60 of the deer. Biopsy of the rectal mucosa, a test that can be performed on live deer, detected 83% of the infected animals. The prion genetics of the deer was strongly linked to the rate of infection and to disease progression. The results demonstrate that clinical signs are a poor indicator of the disease in captive white tailed deer and that routine testing of live deer and comprehensive necropsy surveillance may be needed to identify infected herds. Technical Abstract: Chronic wasting disease CWD is a transmissible spongiform encephalopathy or prion disease of deer and elk in North America. All diseases in this family are characterized by long preclinical incubation periods following by a relatively short clinical course. Endpoint disease is characterized by extensive deposits of aggregates of the abnormal prion protein in the central nervous system,. In deer, the abnormal prion proteins accumulate in some peripheral lymphoid tissues early in disease and are therefore suitable for antemortem and preclinical postmortem diagnostics and for determining disease progression in infected deer. In this study, a herd of deer with previous CWD diagnoses was depopulated. No clinical suspects were identified at that time. Examination of the brain and nodes demonstrated that 79% of the deer were infected. Of the deer with abnormal prion in the peripheral lymphoid system, the retropharyngeal lymph node was the most reliable diagnostic tissue. Biopsy of the rectal mucosal tissue, a site readily sampled in the restrained or chemically immobilized deer, provided an accurate diagnosis in 83% of the infected deer. The retina in the eye of the deer was positive only in late stage cases. This study demonstrated that clinical signs are a poor indicator of disease, supports the use of the retropharyngeal lymph node as the most appropriate postmortem sample, and supports a further evaluation of the rectal mucosal tissue biopsy as an antemortem test on a herd basis.



http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=218153





http://chronic-wasting-disease.blogspot.com/2008/11/commentary-crimes-hurt-essence-of.html





http://chronic-wasting-disease.blogspot.com/2009/01/cwd-game-farms-baiting-and-politics.html





Thursday, August 28, 2008

cwd, feeding, and baiting piles



A. ORAL TRANSMISSION to deer and elk is a very efficient mode for transmission. HIGH PROTEIN FEED will transmit CWD. high protein feed have been fed to deer and elk, especially in game farms.



B. COMMINGLING OF DISEASED ANIMALS AROUND BAIT PILE. chance of the environment in the surrounding areas becoming infected, from feces, urine, shedding, becoming a hot bed for animals to feed, congregate, and become exposed, and there is still very much the possibility of lateral transmission, especially with the CWD TSE, in deer and elk.



WHY ignore sound science ?



IT's the same with pouring a bottle of 100% deer urine scent formulas all over you and the environment, this is a proven mode of transmission. SO WHY DO IT?



leave your egos, and testosterone at the door, and look at the transmission studies, this is NOT rocket science. ...TSS






http://chronic-wasting-disease.blogspot.com/2008/08/cwd-feeding-and-baiting-piles.html




Saturday, November 12, 2011


Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease

Fri, 22 Sep 2006 09:05:59 -0500


http://chronic-wasting-disease.blogspot.com/2011/11/human-prion-disease-and-relative-risk.html




Monday, June 27, 2011


Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates


http://chronic-wasting-disease.blogspot.com/2011/06/zoonotic-potential-of-cwd-experimental.html





Wednesday, January 5, 2011



ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011 Prions


David W. Colby1,* and Stanley B. Prusiner1,2


+ Author Affiliations


1Institute for Neurodegenerative Diseases, University of California, San Francisco, San Francisco, California 94143 2Department of Neurology, University of California, San Francisco, San Francisco, California 94143 Correspondence: stanley@ind.ucsf.edu



SNIP...



Greetings,



I believe the statement and quote below is incorrect ;



"CWD has been transmitted to cattle after intracerebral inoculation, although the infection rate was low (4 of 13 animals [Hamir et al. 2001]). This finding raised concerns that CWD prions might be transmitted to cattle grazing in contaminated pastures."



Please see ;



Within 26 months post inoculation, 12 inoculated animals had lost weight, revealed abnormal clinical signs, and were euthanatized. Laboratory tests revealed the presence of a unique pattern of the disease agent in tissues of these animals. These findings demonstrate that when CWD is directly inoculated into the brain of cattle, 86% of inoculated cattle develop clinical signs of the disease.



http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=194089






"although the infection rate was low (4 of 13 animals [Hamir et al. 2001])."




shouldn't this be corrected, 86% is NOT a low rate. ...




kindest regards,



Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518




MARCH 1, 2011


UPDATED CORRESPONDENCE FROM AUTHORS OF THIS STUDY I.E. COLBY, PRUSINER ET AL, ABOUT MY CONCERNS OF THE DISCREPANCY BETWEEN THEIR FIGURES AND MY FIGURES OF THE STUDIES ON CWD TRANSMISSION TO CATTLE ;



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


From: David Colby


To: flounder9@verizon.net


Cc: stanley@XXXXXXXX


Sent: Tuesday, March 01, 2011 8:25 AM


Subject: Re: FW: re-Prions David W. Colby1,* and Stanley B. Prusiner1,2 + Author Affiliations


Dear Terry Singeltary,


Thank you for your correspondence regarding the review article Stanley Prusiner and I recently wrote for Cold Spring Harbor Perspectives. Dr. Prusiner asked that I reply to your message due to his busy schedule. We agree that the transmission of CWD prions to beef livestock would be a troubling development and assessing that risk is important. In our article, we cite a peer-reviewed publication reporting confirmed cases of laboratory transmission based on stringent criteria. The less stringent criteria for transmission described in the abstract you refer to lead to the discrepancy between your numbers and ours and thus the interpretation of the transmission rate. We stand by our assessment of the literature--namely that the transmission rate of CWD to bovines appears relatively low, but we recognize that even a low transmission rate could have important implications for public health and we thank you for bringing attention to this matter.


Warm Regards, David Colby


--


David Colby, PhDAssistant ProfessorDepartment of Chemical EngineeringUniversity of Delaware




====================END...TSS==============




SNIP...SEE FULL TEXT ;



http://betaamyloidcjd.blogspot.com/2011/01/enlarging-spectrum-of-prion-like.html




UPDATED DATA ON 2ND CWD STRAIN


Wednesday, September 08, 2010


CWD PRION CONGRESS SEPTEMBER 8-11 2010


http://chronic-wasting-disease.blogspot.com/2010/09/cwd-prion-2010.html



http://chronic-wasting-disease.blogspot.com/




http://transmissiblespongiformencephalopathy.blogspot.com/






LAYPERSON



Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA


TSS

Chronic wasting disease found in Big Horn basin deer Wyoming's deer hunt area 165

News Release




Contact: Dennie Hammer Dennie.Hammer@wgf.state.wy.us



11/15/2011



Cody Regional Office 2820 State Highway 120 Cody, WY 82414 Phone: (307) 527-7322 ext. *817 ? Fax: (307) 587-5430 http://gf.state.wy.us



CHRONIC WASTING DISEASE DISCOVERED IN DEER HUNT AREA 165



CODY- Chronic wasting disease (CWD), a fatal neurological disease of deer, elk, and moose has been discovered in deer hunt area 165, bringing the known total CWD areas in the Big Horn Basin to fifteen out of thirty-nine.



A white-tailed deer taken on October 15, 2011 near the Greybull River has tested positive for the disease. Hunt area 165 borders deer CWD endemic hunt areas 122 to the north, 124 to the east, and 125 to the south. The disease is now known to occur in Big Horn Basin deer hunt areas 37, 39, 41, 42, 46, 47, 51, 119, 120, 122, 124, 125, 127, 164, and 165. After a review of available scientific data, the World Health Organization in December 1999 stated, "There is currently no evidence that CWD in cervidae (deer and elk) is transmitted to humans." In 2004, Dr. Ermias Belay of the Center for Disease Control said, "The lack of evidence of a link between CWD transmission and unusual cases of CJD, [Creutzfeldt-Jakob disease, a human prion disease] despite several epidemiological investigations, suggest that the risk, if any, of transmission of CWD to humans is low." Nonetheless to avoid risk, both organizations say parts or products from any animal that looks sick and/or tests positive for CWD should not be eaten. Cody region personnel continue to collect samples through hunter field checks, and at CWD sampling stations.



For more information on chronic wasting disease visit the Chronic Wasting Disease Alliance website at www.cwd-info.org. -WGFD-



http://gf.state.wy.us/downloads/pdf/RegionalNews/dh-CWD-165.pdf




Monday, November 14, 2011



WYOMING Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011



http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/wyoming-creutzfeldt-jakob-disease-cwd.html





TSS

Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease Fri, 22 Sep 2006 09:05:59 -0500

IN REPLY TO ;

Subject: Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease

From: "Terry S. Singeltary Sr."

Reply-To: Bovine Spongiform Encephalopathy

Date: Fri, 22 Sep 2006 09:05:59 -0500

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

Volume 12, Number 10—October 2006 Research

Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease

W. John Pape†, Jeri Forster*, C. Alan Anderson‡§, Patrick Bosque‡¶, Michael Miller#, and Samantha MaWhinney

Abstract The transmission of the prion disease bovine spongiform encephalopathy (BSE) to humans raises concern about chronic wasting disease (CWD), a prion disease of deer and elk. In 7 Colorado counties with high CWD prevalence, 75% of state hunting licenses are issued locally, which suggests that residents consume most regionally harvested game. We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD). The relative risk (RR) of CJD for CWD-endemic county residents was not significantly increased (RR 0.81, 95% confidence interval [CI] 0.40–1.63), and the rate of CJD did not increase over time (5-year RR 0.92, 95% CI 0.73–1.16). In Colorado, human prion disease resulting from CWD exposure is rare or nonexistent. However, given uncertainties about the incubation period, exposure, and clinical presentation, the possibility that the CWD agent might cause human disease cannot be eliminated.

snip...

CWD has existed in wild deer and elk of northeastern Colorado for well over 2 decades. However, neither the number of CJD deaths in CWD-endemic counties nor the rate of CJD in CWD-endemic counties or in Colorado as a whole have increased. Although our findings are consistent with those of other studies that suggest no connection between CWD and human TSEs (5,12), we cannot exclude the possibility that an isolated case of human disease associated with the CWD agent has occurred or may yet occur. However, our findings do suggest that death from CJD remains rare in Colorado.

http://wwwnc.cdc.gov/eid/article/12/10/06-0019_article.htm



Oral.40: Monitoring the Potential Transmission of Chronic Wasting Disease to Humans




Ermias D. Belay,1,† Joseph Abrams,1 Janell Kenfield,2 Kelly Weidenbach,3 Ryan A. Maddox,1 Elisabeth Lawaczeck2 and Lawrence B. Schonberger1



1 Centers for Disease Control and Prevention; Atlanta, GA USA; 2 Colorado Department of Public Health and Environment; Denver, CO USA; 3 Wyoming Department of Health; Cheyenne, WY USA†Presenting author; Email: EBelay@cdc.gov



Introduction: Chronic wasting disease (CWD) has been occurring for several decades among wild cervids in Colorado and Wyoming. The increasing detection of CWD in an additional 12 US states and two Canadian provinces may have resulted in increased human exposure to CWD. Although studies have evaluated the possible transmission of CWD to humans in laboratory models, a reliable assessment requires conducting epidemiologic and laboratory studies designed to identify prion disease among humans exposed to CWD and generating scientific evidence causally linking the two illnesses.



Methods: In collaboration with the Centers for Disease Control and Prevention, the Wyoming Department of Health and the Colorado Department of Public Health and Environment established a long-term follow-up study of hunter data to monitor the potential CWD transmission to humans. Personal identifiers from deer or elk hunter database are cross-checked with mortality data to determine their mortality status and causes of death.



Results: In Colorado, the hunter data include about 4.9 million records of licenses purchased during 1995–2008, representing about 1.1 million hunters. Overall, 48% of hunters purchased a license to hunt in areas that included CWD positive game units and 47% to hunt anywhere in Colorado. In Wyoming, the data include about 1.2 million records of licenses purchased during 1996-2009, representing about 0.5 million hunters; 34% of hunters purchased a license to hunt in areas that included CWD positive game units and 28% to hunt anywhere in Wyoming. During the study period three Colorado hunters (expected number: 3-15 cases) and three Wyoming hunters (expected number: 0-6 cases) were identified to have died of Creutzfeldt-Jakob disease (CJD).



Conclusions: No evidence suggests that the CJD incidence is higher than expected among persons who hunted in Colorado or Wyoming. The hunter data are valuable for monitoring the potential transmission of CWD to humans. Ongoing assessment and long-term follow-up of the hunter population is necessary because human prion diseases are associated with long latency periods and the pathogenicity of CWD might change over time.







http://www.prion2011.ca/files/PRION_2011_-_Posters_(May_5-11).pdf

 

 

i wish to update this old post to the BSE-L, and some may like it for their files. ...kind regards, terry


"We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD)."



Tuesday, November 08, 2011

Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008 Vol. 37, No. 3-4, 2011

Original Paper

Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.



http://creutzfeldt-jakob-disease.blogspot.com/2011/11/can-mortality-data-provide-reliable.html




Friday, November 04, 2011

Diagnostic accuracy of cerebrospinal fluid protein markers for sporadic Creutzfeldt-Jakob disease in Canada: a 6-year prospective study Research article


http://creutzfeldt-jakob-disease.blogspot.com/2011/11/diagnostic-accuracy-of-cerebrospinal.html



Oral.42:

Prion Seeding Activity in Cerebrospinal Fluid from Sporadic Creutzfeldt-Jakob Disease Patients Using Real-Time QuIC Analysis: A Potential New Diagnostic Test?

Lynne I. McGuire,1,† Alexander H. Peden,1 Nigel Appleford,2 Gary Mallinson,2 Christina Orru,3 Jason Wilham,3 Greg Raymond,3 Mary Andrews,1 Mark W. Head,1 Byron Caughey,3 Robert Will,1 Richard Knight1 and Alison Green,1

1 NCJDSU, University of Edinburgh; Edinburgh, UK; 2 Bristol Institute for Transfusion Sciences, NHS Blood and Transplant; Bristol, UK; 3 Laboratory of Persistent Viral Disease, NIAID Rocky Mountain Laboratories, National Institutes of Health; Hamilton, MT USA†Presenting author; Email: lmcguir1@staffmail.ed.ac.uk

Since its introduction into the diagnostic criteria for sporadic CJD in 1998, the analysis of cerebrospinal fluid (CSF) for 14-3-3 has become a widely accepted investigation in patients with suspected sporadic CJD. However, a number of reports have raised concerns about its lack of specificity. This has prompted the search for a more specific and disease-related pre-mortem diagnostic test for sporadic CJD. The ability of PrPSc to convert PrPC into protease-resistance isoforms has been exploited using a variety of techniques such as protein misfolding cyclic amplification (PMCA) and quaking induced conversion (QuIC). A recent adaptation of QuIC (real-time QuIC) has been described which incorporates thioflavin T (ThT) in the reaction mixture. The ThT binds to the aggregated PrP causing a change in the ThT emission spectrum that can be monitored in real-time. Recent studies have shown that CSF samples from hamsters inoculated with experimental scrapie, sheep with scrapie and patients with sporadic CJD can be correctly identified using real-time QuIC.1,2 We now describe the findings of an investigation into the value of real-time QuIC in the diagnosis of sCJD. A blinded panel of CSF samples from 56 neuropathologically confirmed cases of sCJD and from 53 patients who were initially suspected of having sCJD but who were found to have an alternative diagnosis were analyzed. Of the 56 patients with sCJD 51 were found to give a positive response with real-time QuIC. In contrast only one patient from the control group was found to be positive. The sensitivity and specificity was 91% and 98%, respectively. The corresponding sensitivity and specificity of CSF 14-3-3 was 91% and 55%, respectively. These results suggest that real-time QuIC has the potential to be a more specific pre-mortem CSF test for sCJD than CSF 14-3-3.

References

1. Atarashi R, Satoh K, Sano K, Fuse T, Yamanaka H, Yamaguchi N, et al. Ultrasensitive human prion detection in cerebrospinal fluids by real-time quaking induced conversion. Prion 2010; 4:214

2. Wilham JM, Orru CD, Benssen RA, Atarashi R, Sano K, Race B, et al. Rapid end-point quantitation of prion seeding activity with sensitivity comparable to bioassays. PLoS 2010; 6:1-15

http://www.prion2011.ca/files/PRION_2011_-_Posters_(May_5-11).pdf




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

Thursday, May 26, 2011

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

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

http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/travel-history-hunting-and-venison.html



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

Wednesday, March 18, 2009

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

http://chronic-wasting-disease.blogspot.com/2009/03/noahs-ark-holding-llc-dawson-mn-recall.html



Monday, June 27, 2011

Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates

http://chronic-wasting-disease.blogspot.com/2011/06/zoonotic-potential-of-cwd-experimental.html


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 ;

http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html


CJD9/10022

October 1994

Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ

Dear Mr Elmhirst,

CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT

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

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

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

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

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

http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf


This is an interesting editorial about the Mad Cow Disease debacle, and it's ramifications that will continue to play out for decades to come ;

Monday, October 10, 2011

EFSA Journal 2011 The European Response to BSE: A Success Story

snip...

EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.

snip...

http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1


http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf



see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;

http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html


Wednesday, November 09, 2011

Case report Sporadic fatal insomnia in a young woman: A diagnostic challenge: Case Report TEXAS

HOW TO TURN A POTENTIAL MAD COW VICTIM IN THE USA, INTO A HAPPENSTANCE OF BAD LUCK, A SPONTANEOUS MUTATION FROM NOTHING.

OR WAS IT $$$

http://creutzfeldt-jakob-disease.blogspot.com/2011/11/case-report-sporadic-fatal-insomnia-in.html


Thursday, August 4, 2011

Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis, Date aired: 27 Jun 2011 (SEE VIDEO)

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/terry-singeltary-sr-on-creutzfeldt.html



Sunday, August 21, 2011

The British disease, or a disease gone global, The TSE Prion Disease (SEE VIDEO)

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/british-disease-or-disease-gone-global.html


Saturday, March 5, 2011

MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA

http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html


TSS

layperson



http://chronic-wasting-disease.blogspot.com/


http://bse-atypical.blogspot.com/



http://scrapie-usa.blogspot.com/


http://nor-98.blogspot.com/



http://bseusa.blogspot.com/


http://madporcinedisease.blogspot.com/


http://felinespongiformencephalopathyfse.blogspot.com/


http://caninespongiformencephalopathy.blogspot.com/


http://equinespongiformencephalopathy.blogspot.com/


http://transmissible-mink-encephalopathy.blogspot.com/


http://transmissiblespongiformencephalopathy.blogspot.com/


http://creutzfeldt-jakob-disease.blogspot.com/


http://sporadicffi.blogspot.com/


http://prionpathy.blogspot.com/


http://prionopathy.blogspot.com/


http://vcjd.blogspot.com/


http://vcjdblood.blogspot.com/


http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html


http://cjdquestionnaire.blogspot.com/


TSS

=================END...2011===============


========Date: Fri, 22 Sep 2006 09:05:59 -0500=====


Subject: Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease

From: "Terry S. Singeltary Sr."

Reply-To: Bovine Spongiform Encephalopathy

Date: Fri, 22 Sep 2006 09:05:59 -0500

Content-Type: text/plain

Parts/Attachments: text/plain (146 lines)

Reply

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

Subject: Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease Date: September 22, 2006 at 7:00 am PST

Volume 12, Number 10–October 2006 Research Human Prion Disease and Relative Risk Associated with Chronic Wasting Disease Samantha MaWhinney,* W. John Pape,† Jeri E. Forster,* C. Alan Anderson,‡§ Patrick Bosque,‡¶ and Michael W. Miller# *University of Colorado at Denver and Health Sciences Center, Denver, Colorado, USA; †Colorado Department of Public Health and Environment, Denver, Colorado, USA; ‡University of Colorado School of Medicine, Denver, Colorado, USA; §Denver Veteran's Affairs Medical Center, Denver, Colorado, USA; ¶Denver Health Medical Center, Denver, Colorado, USA; and #Colorado Division of Wildlife, Fort Collins, Colorado, USA

Suggested citation for this article

The transmission of the prion disease bovine spongiform encephalopathy (BSE) to humans raises concern about chronic wasting disease (CWD), a prion disease of deer and elk. In 7 Colorado counties with high CWD prevalence, 75% of state hunting licenses are issued locally, which suggests that residents consume most regionally harvested game. We used Colorado death certificate data from 1979 through 2001 to evaluate rates of death from the human prion disease Creutzfeldt-Jakob disease (CJD). The relative risk (RR) of CJD for CWD-endemic county residents was not significantly increased (RR 0.81, 95% confidence interval [CI] 0.40–1.63), and the rate of CJD did not increase over time (5-year RR 0.92, 95% CI 0.73–1.16). In Colorado, human prion disease resulting from CWD exposure is rare or nonexistent. However, given uncertainties about the incubation period, exposure, and clinical presentation, the possibility that the CWD agent might cause human disease cannot be eliminated.

An emerging wildlife epizootic of chronic wasting disease (CWD) (1), a contagious prion disease among mule deer, white-tailed deer, and Rocky Mountain elk, has potential public health implications (2–5). CWD is related to other mammalian transmissible spongiform encephalopathies (TSEs), such as Creutzfeldt-Jakob disease (CJD) in humans, bovine spongiform encephalopathy (BSE) in cattle, and scrapie in sheep. In prion diseases, a normally produced cellular protein accumulates in an abnormal, misfolded, and aggregated form (6), which results in neuron destruction and a universally fatal outcome after a prolonged incubation period.

CWD infects wild and captive deer and elk in several US states and Canadian provinces. The highest reported disease prevalence is in a contiguous region, spanning parts of Colorado, Wyoming, and Nebraska (Figure 1), where the estimated disease prevalence is 5% in mule deer, 2% in white-tailed deer, and 0.5% in elk (7). CWD was first noted in captive deer at a research station in north-central Colorado near Fort Collins in the 1960s (1) and later in a wild elk near Estes Park in 1981 (8). No clear epidemiologic connections have been found between original cases and more recent cases, which suggests that unidentified risk factors may be contributing to the relatively wide and unpredictable geographic distribution of CWD (2–4).

Humans and animals can acquire TSEs by consuming prion-contaminated food. Outbreaks of prion disease include an epidemic of kuru among the cannibalistic Fore tribe of the New Guinea highlands (9) and an epizootic of BSE in the United Kingdom, caused by feeding to cattle protein supplements derived from prion-infected cattle offal (10). Food-based prion transmission between species also occurs, although a phenomenon known as the "species barrier" decreases transmission efficiency. In vitro studies (11,12) indicate that this natural barrier reduces human susceptibility to animal prion diseases, including CWD. As yet, no cases of human prion disease have been linked with CWD (5,13–15), and natural transmission of CWD to humans or traditional domestic livestock seems unlikely (2,3,5,12,14,16,17).

The otherwise reassuring molecular evidence of species barriers is clouded by the disparate experiences with scrapie and BSE as foodborne human pathogens. Scrapie exposure has not been demonstrated to increase CJD risk, despite extensive human exposure (18). Conversely, in Britain the consumption of BSE-infected cattle led to an epidemic of variant CJD (vCJD), beginning in the mid-1990s (19–23). As of June 2006, however, only 161 cases of vCJD have been identified in the United Kingdom (24), despite the dietary exposure of millions of Britons to the BSE agent. In addition, recent studies indicate that large numbers of cases of vCJD are unlikely to occur in Britain in the future (25). Because the CWD agent is distinct from the BSE agent (12,26–29) and the type and degree of human exposure to these 2 agents differ, the risk for CWD transmission to humans cannot be directly extrapolated from the BSE and vCJD epidemics (30).

Because no completely reliable experimental animal model exists for testing the potential for CWD to cause CJD (30), human case investigations and epidemiologic studies remain valuable tools for assessing the potential risk associated with CWD exposure (5). Data that define human CWD exposure from consumption of infected deer or elk do not exist. However, in 7 northeastern Colorado counties (Boulder, Larimer, Logan, Morgan, Phillips, Sedgwick, and Weld) that are considered CWD-endemic areas (7) (Figure 1), the Colorado Division of Wildlife (CDOW) hunter license records indicate ˜75% (38,458 of 51,048) of deer and elk hunting licenses purchased from 1995 through 2001 were issued locally (CDOW, unpub. data), which suggests that county residents consume most regionally harvested game. Using Colorado death certificate data from 1979 through 2001, we modeled whether residence in a CWD-endemic county affected the risk-adjusted probability that a death is from CJD. We also examined whether the probability that a death is from CJD increased over time. To account for the possibility that CJD may have been misclassified, we also conducted sensitivity analyses using an expanded definition of event, similar to criteria used by Majeed et al. (31).

Materials and Methods Study Population Colorado death certificate data from 1979 through 2001 were used. Deaths during 1979-1998 and 1999-2001 were classified by the ICD-9 and ICD-10 codes, respectively. Sporadic CJD is extremely rare in persons <30 years of age (32), and vCJD cases have not been reported in patients <12 years (33). Therefore, we restricted all analyses to deaths occurring at >12 years, which provided 506,335 eligible deaths. We classified deaths as due to CJD if the codes 046.1 (ICD-9) or A81.0 (ICD-10) were listed as either the direct or contributory cause (events = 65).

Additional Colorado death certificate data used included age at death, sex, and marital status. We considered marital status as a predictor, because it may influence whether symptoms are recognized, which subsequently increases the likelihood of diagnosing CJD. Years of education data were not collected before 1989; therefore, this variable was not considered as a predictor. Figure 2 contains individual characteristics for persons who died in Colorado with CJD listed on the death certificate and smoothed population CJD rates (34).

In 1998, the Colorado Department of Public Health and Environment (CDPHE) initiated human prion disease surveillance. From 1998 through 2001, CDPHE identified 20 Colorado resident deaths consistent with prion disease (Table 1). For 10 of these 20 deaths, CJD was confirmed by examination of brain tissue from biopsy or autopsy specimens. Three deaths were classified as probable CJD; rapidly progressive dementia clinically consistent with prion disease was supported by nonspecific tests. Seven of the 20 CJD deaths were classified as suspected CJD because the diagnosis was made without autopsy, biopsy, or supportive testing. In 14 of these 20 deaths, the ICD code indicated CJD. Inexplicably, the remaining 6 patients who died (4 with confirmed CJD, 0 with probable CJD, and 2 with suspected CJD) had a medical record consistent with CJD, but the deaths were not coded as such. Three of these deaths (2 with confirmed CJD, 0 with probable CJD, and 1 with suspected CJD) were identified under our expanded definition. CDPHE review of the death certificates for the 6 misclassified deaths found that CJD was not reported as a cause of death and that the ICD-10 codes were consistent with the stated cause of death.

Statistical Considerations A review of Colorado death certificates identified 65 deaths with CJD listed on the death certificate from 1979 through 2001; from all causes, 81,916 and 424,419 persons >12 years died in the CWD-endemic and non–CWD-endemic counties, respectively. We were interested in testing whether the relative risk (RR) was greater than 1.0, where RR is the probability of a CJD death, given residence in a CWD-endemic county, divided by the corresponding probability in a non–CWD-endemic county. The RR is approximated by the odds ratio for a rare event such as death from CJD. Assuming a 2-sided ?2 test with a significance level of 0.05, we had >85% power to detect an unadjusted RR of 2.47. Assuming 65 CJD deaths, this corresponds to 21 (2.56 cases/10,000 deaths) and 44 (1.04 cases/10,000 deaths) deaths in the CWD-endemic and non–CWD-endemic counties, respectively.

We conducted separate analyses for the primary predictors of interest: residence in a CWD-endemic county and death year. The RRs and 95% confidence intervals (CIs) were estimated by using logistic regression in SAS (SAS Institute, Cary, NC, USA). Covariates in the multivariable analysis were death age, sex, ICD classification, and marital status. The CWD county analysis also was adjusted for death year. For death year, ICD classification was considered as an effect modifier.

Results Characteristics of Persons Who Died Descriptive characteristics by CWD endemicity of county are presented in Table 2. Due to the large sample size, statistical significance was observed for all covariates, although most differences were relatively small. Those who died in CWD-endemic counties were more likely to be white, >70 years of age, and married or widowed rather than divorced.

Univariate Analyses Univariate analyses allowed us to describe event characteristics. Table 3 contains the univariate RRs and corresponding 95% CIs for available predictors. CWD-endemic counties contributed 16.18% of total deaths but only 13.85% of deaths with CJD listed on the death certificate (p = 0.61) (Figure 2). This finding corresponds to an unadjusted CJD rate in CWD-endemic counties of 1.10/10,000 deaths; in non–CWD-endemic counties, this rate was 1.32/10,000 deaths. We saw a slight decrease in CJD risk over time (p = 0.54); 43.08% of CJD deaths occurred before 1989. CJD risk decreased with age of death; 46.15% of CJD deaths occurred in persons 56–70 years of age and 40.00% in those >70 years. Given this younger population, predictable changes occurred in the distribution of marital status.

Multivariable Models Table 4 contains the adjusted RRs for CWD endemicity of county and year of death. An RR >1.0 is consistent with the hypothesis of an increased risk for death from CJD, given residence in a CWD-endemic county. In the multivariable model, residing in a CWD-endemic county did not achieve statistical significance (RR 0.80, 95% CI 0.40–1.62). Death year remained not significant after adjusting for the additional covariates (for every 5-year increase, RR 0.92, 95% CI 0.73–1.16).

Death Rates In addition to analyzing death certificate data, we computed annual age-standardized CJD death rates per million population (Figure 3) for the CWD-endemic and non–CWD-endemic counties (34). These population rates were age-standardized by using the 2001 age distribution for Colorado. Smoothed age–standardized rates were similar to the crude population rates for Colorado shown in Figure 2 (smoothed median 0.88, range 0.65–0.94). As expected, given the smaller population size, more variability was observed for these rates in the CWD-endemic counties (smoothed median 0.67 per million, range 0.11–1.37), than the non–CWD-endemic counties (smoothed median 0.96 per million, range 0.73–1.01). Overall, annual crude population rates were slightly lower than age-standardized rates in both the disease-endemic counties (smoothed median 0.52 per million, range 0.09–1.29) and non–disease-endemic counties (smoothed median 0.85 per million, range 0.76–1.01) (data not shown).

Expanded Definition Analyses We considered that if CWD were transmissible to humans, then it might be manifested with different signs and symptoms than typical sporadic CJD, resulting in misdiagnosis or classification under a different ICD code. Therefore, in addition to assessing data for CJD, we conducted sensitivity analyses using an expanded definition (Appendix Tables 1 and 2). This definition increased the number of event codes to 29 ICD-9 and 30 ICD-10 (events 1,911). These codes corresponded to neurodegenerative syndromes in which signs are exhibited that are prominent in some forms of prion disease. To minimize false-positive results, we did not consider death from Alzheimer disease after 55 years of age as an event. In the United Kingdom, most vCJD cases have occurred in persons <55 years, with a median age at death of 28 years (range 14–74 years) (35). In addition, among patients >55 years, the incidence of age-related neurodegenerative diseases tends to obscure all but dramatic increases in conditions that may be attributable to CWD exposure. Therefore, to increase specificity, we also considered the expanded definition restricted to deaths in persons 12–55 years, which provided 89,033 eligible deaths (events 339). The adjusted expanded definition RRs for CWD endemicity of county and year of death are contained in Table 4. Under the expanded definition, we see a decrease in risk over time (p<0.0001), although significance is lost when the analysis is restricted to deaths of those who died before the age of 55 years. Discussion CWD has occurred in free-ranging deer and elk in northeastern Colorado for >25 years (7,8), so some persons likely have been exposed to the CWD agent. The human risk from exposure to CWD cannot be quantified because identifying exposed persons is not possible. The CDOW records indicate that ˜75% of deer and elk hunting licenses in 7 northeastern Colorado counties with high CWD prevalence are issued locally, which indicates that residents consume most game harvested in this region. Using Colorado death certification data from 1979 through 2001, we modeled the risk for a CJD death with CWD-endemic county residence as the exposure of interest. Similarly, we examined whether CJD deaths have increased overall. Given the possibility of misclassification of CJD and human TSEs, sensitivity analyses were conducted for expanded event definitions.

Human prion disease is rare, and increased risk due to CWD exposure appears to be subtle or nonexistent. No significant difference was found in the proportion of deaths from CJD in CWD-endemic versus non–CWD-endemic counties (adjusted RR 0.81, 95% CI 0.40–1.63). The upper CI value does not exclude an increased risk for CWD-endemic county residents, but it is inconsistent with a dramatic increase in that risk. Clearly, using residence in a CWD-endemic county as a surrogate for exposure has several limitations. The most obvious is that many persons with no history of hunting or deer and elk consumption are included in the exposed cohort. Conversely, exposed persons may live outside these counties. Given the potentially long incubation periods associated with prion diseases, ample opportunity would exist for infected persons to move from disease-endemic counties before the onset of illness. Moreover, other unrecognized risk factors (i.e., familial CJD or iatrogenic sources of infection) could confound epidemiologic investigations.

When Colorado CJD rates were examined over time, no significant change in CJD deaths was demonstrated (5-year RR 0.92, 95% CI 0.73–1.16). Although finding that risk for deaths from neurologic disease decreased over time under our expanded event definition is reassuring (5-year RR 0.81, 95% CI 0.77–0.84), this analysis should be interpreted with caution. The findings could be influenced by the lack of specificity in the definition and the switch from ICD-9 to ICD-10 codes in 1999. After excluding deaths in persons >55 years of age in the expanded definition, the results became inconclusive.

Although an increase in CJD deaths has not been observed in Colorado, due to the long incubation periods of prion diseases, infected persons may not have had sufficient time for disease to develop or may have left the state before disease onset. Although the prevalence and known range of CWD has increased over time (2–4), CWD exposure may be decreasing due to ongoing efforts by the public health and wildlife management agencies (2–4). Active education about CWD has been ongoing in northeastern Colorado since 1995. This information campaign includes several specific recommendations to minimize exposure for hunters, meat processors, and taxidermists (4). In addition, since 1994, testing has been available for game harvested in CWD-endemic counties, thereby removing a proportion of harvested, CWD-infected deer and elk from the human food chain.

Identifying cases of human prion disease remains a challenge. How human prion disease linked to CWD would be manifested clinically or pathologically is not clear. The probability of CJD being accurately diagnosed is influenced by changes in diagnostic practices; access to medical care, particularly specialized neurologic consultations; and the availability of diagnostic testing, including autopsy and postmortem pathologic examinations. Improved case ascertainment should result from the establishment of the National Prion Disease Pathology Surveillance Center, which offers free diagnostic testing, complemented by increased Colorado surveillance efforts, including classifying human prion diseases as a physician-reportable condition, funding to pay for autopsies, and outreach to neurologists, pathologists, and coroners (36). Increased publicity about BSE, CWD, and human TSEs may have led to changes in diagnostic practices or case recognition, particularly in CWD-endemic areas due to a perceived association of CWD with human disease.

Death certificate data undoubtedly underestimate the prevalence of CJD. A limitation of this study is that diagnosed human TSE cases may not be recorded as CJD on the death certificate. Between 1998 and 2001, CDPHE surveillance identified 6 persons who died with a medical history of CJD for whom CJD was not reported on the death certificate; therefore, those deaths were not captured as events in our survey, although 3 of these deaths were identified under our expanded definition. Given that CDPHE surveillance overlapped only the past 4 years of our study, we could not reclassify these additional TSE deaths as CJD without introducing an obvious bias in the analysis of year of death. As a post hoc sensitivity analysis to our primary CJD endpoint in the CWD county analysis, we reclassified these 6 missed cases as events and computed the unadjusted RR. Although including these cases changed the CWD county point estimate from 0.83 (95% CI 0.41–1.68) to 1.16 (95% CI 0.64–2.12), the results remained highly nonsignificant (p = 0.63). The results of this sensitivity analysis should be interpreted with caution as increasing awareness of CJD is unlikely to be uniform across a state or country. In our analysis, this heterogeneous distribution may have resulted in an increase in misclassification bias over time, such that reclassifying cases that were not identified on the death certificate led to identifying an excess of CJD that was unrelated to exposure in the CWD-endemic counties.

Despite increased scrutiny, evidence of increased CJD in Colorado has not yet been demonstrated. Smoothed Colorado CJD annual rates based on death certificate data are consistently <1 case per million population (median 0.88, range 0.65–0.94). In the United Kingdom, which arguably has the most comprehensive human prion disease surveillance, the annual crude mortality rates from sporadic CJD per million population were 0.86, 1.08, 0.84, and 0.57 in England, Wales, Scotland, and Northern Ireland, respectively, over the period from 1990 to 2003 (35). The overall mortality rate from sporadic CJD from 1999 through 2002 in Australia, Canada, United Kingdom, and 8 additional European countries was estimated to be 1.39 per million population >10 years, although rates were highly variable across countries (0.48–2.23) (37). Approximately 84% of Colorado's population is >10 years of age (38) such that the comparable median is 1.05 (range 0.77–1.12). Thus Colorado's CJD rates appear comparable to or below other reported rates.

Continued case surveillance remains crucial for identifying and characterizing human prion disease (5). Recognition of CWD transmission to humans will likely require the identification of a human TSE patient with a history of exposure to deer or elk, evaluation of the clinical course and pathologic features at autopsy, and characterization of the prion strain in laboratory studies. Additional epidemiologic studies, such as a case-control study or cohort study that compares hunter license data with death certificate data, also should be conducted. Until the health risks from CWD can be fully ascertained, preventative steps to reduce exposure to the CWD agent and other animal prion disease agents (e.g., BSE, scrapie) should continue (5,30).

CWD has existed in wild deer and elk of northeastern Colorado for well over 2 decades. However, neither the number of CJD deaths in CWD-endemic counties nor the rate of CJD in CWD-endemic counties or in Colorado as a whole have increased. Although our findings are consistent with those of other studies that suggest no connection between CWD and human TSEs (5,12), we cannot exclude the possibility that an isolated case of human disease associated with the CWD agent has occurred or may yet occur. However, our findings do suggest that death from CJD remains rare in Colorado.

Acknowledgments We thank Ken Gershman for his support, input on study design, and manuscript review; Mary Chase for compiling the death certificate data; and Mary Lloyd for compiling hunting license data. We also thank T. Sanders for manuscript review.

This work was funded by an Emerging Infections Program grant from CDC and the Colorado Division of Wildlife (M.W.M.).

Dr MaWhinney is an associate professor of preventive medicine and biometrics at the University of Colorado at Denver and the Health Sciences Center. Her primary research interests are the application of biostatistical methods to infectious disease data.

References Williams ES, Young S. Chronic wasting disease of captive mule deer: A spongiform encephalopathy. J Wildl Dis. 1980;16:89–98. Williams ES, Miller MW. Chronic wasting disease in North American deer and elk. Rev Sci Tech. 2002;21:305–16. Williams ES, Miller MW. Transmissible spongiform encephalopathies in non-domestic animals: origin, transmission, and risk factors. Rev Sci Tech. 2003;22:145–56. Williams ES, Miller MW, Kreeger TJ, Kahn RH, Thorne ET. Chronic wasting disease of deer and elk: a review with recommendations for management. J Wildl Manage. 2002;66:551–63. Belay ED, Maddox RA, Williams ES, Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and potential transmission to humans. Emerg Infect Dis. 2004;10:977–84. Prusiner SB. Shattuck lecture–neurodegenerative diseases and prions. N Engl J Med. 2001;344:1516–26. Miller MW, Williams ES, McCarty CW, Spraker TR, Kreeger TJ, Larsen CT, et al. Epizootiology of chronic wasting disease in free-ranging cervids in Colorado and Wyoming. J Wildl Dis. 2000;36:676–90. Spraker TR, Miller MW, Williams ES, Getzy DM, Adrian WJ, Schoonveld GG, et al. Spongiform encephalopathy in free-ranging mule deer (Odocoileus hemionus), white-tailed deer (Odocoileus virginianus), and Rocky Mountain elk (Cervus elaphus nelsoni) in northcentral Colorado. J Wildl Dis. 1997;33:1–6. Alpers MP. Epidemiology and clinical aspects of kuru. In: Prusiner SB, McKinley MP, editors. Prions: novel infectious pathogens causing scrapie and Creutzfeldt-Jakob disease. San Diego: Academic Press; 1987. p. 451?65. Anderson RM, Donnelly CA, Ferguson NM, Woolhouse ME, Watt CJ, Udy HJ, et al. Transmission dynamics and epidemiology of BSE in British cattle. [Erratum, Nature 1997;386:302]. Nature. 1996;382:779–88. Raymond GJ, Hope J, Kocisko DA, Priola SA, Raymond LD, Bossers A, et al. Molecular assessment of the potential transmissibilities of BSE and scrapie to humans. Nature. 1997;388:285–8. Raymond GJ, Bossers A, Raymond LD, O'Rourke KI, McHolland LE, Bryant PK III, et al. Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease. EMBO J. 2000;19:4425–30. World Health Organization. WHO consultation on public health and animal transmissible spongiform encephalopathies: epidemiology, risk and research requirements. Geneva: The Organization; 2000. p. 52. Belay ED, Gambetti P, Schonberger LB, Parchi P, Lyon DR, Capellari S, et al. Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Arch Neurol. 2001;58:1673–8. Food and Drug Administration Transmissible Spongiform Encephalopathy Advisory Committee. Transcripts of open meeting, January 18, 2001, Bethesda, Maryland, USA [cited 20006 Aug 8]. Available from http://www.fda.gov/ohrms/dockets/ac/01/transcripts/3681t2_02.pdf Hamir AN, Cutlip RC, Miller JM, Williams ES, Stack MJ, Miller MW, et al. Preliminary findings on the experimental transmission of chronic wasting disease agent of mule deer to cattle. J Vet Diagn Invest. 2001;13:91–6. Gould DH, Voss JL, Miller MW, Bachand AM, Cummings BA, Frank AA. Survey of cattle in northeast Colorado for evidence of chronic wasting disease: geographical and high-risk targeted sample. J Vet Diagn Invest. 2003;15:274–7. Will RG. Epidemiology of Creutzfeldt-Jakob disease. Br Med Bull. 1993;49:960–70. Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet. 1996;347:921–5. Ghani AC, Ferguson NM, Donnelly CA, Hagenaars TJ, Anderson RM. Epidemiological determinants of the pattern and magnitude of the vCJD epidemic in Great Britain. Proc Biol Sci. 1998;265:2443–52. Ghani AC, Ferguson NM, Donnelly CA, Anderson RM. Predicted vCJD mortality in Great Britain. Nature. 2000;406:583–4. Ghani AC, Ferguson NM, Donnelly CA, Anderson RM. Updated projections of future vCJD deaths in the UK. BMC Infect Dis. 2003;3:4. Ghani AC, Ferguson NM, Donnelly CA, Anderson RM. Factors determining the pattern of the variant Creutzfeldt-Jakob disease (vCJD) epidemic in Great Britain. Proc Biol ci. 2003;270:689–98. National Creutzfeldt-Jakob Disease Surveillance Unit. CJD statistics (June 2, 2006) [cited 2006 Aug 8]. Available from http://www.cjd.ed.ac.uk/figures.htm Clarke P, Ghani AC. Projections of the future course of the primary vCJD epidemic in the UK: inclusion of subclinical infection and the possibility of wider genetic susceptibility. Journal of the Royal Society Interface. 2005;2:19–31. Bruce ME, Will RG, Ironside JW, McConnell I, Drummond D, Suttie A, et al. Transmission to mice indicate that 'new variant' CJD is caused by the BSE agent. Nature. 1997;389:498–501. Bruce M, Chree A, Williams ES, Fraser H. Perivascular PrP amyloid in the brains of mice infected with chronic wasting disease. Brain Pathol. 2000;10:662–3. Race RE, Raines A, Baron TGM, Miller MW, Jenny A, Williams ES. Comparison of abnormal prion protein glycoform patterns from transmissible spongiform encephalopathy agent-infected deer, elk, sheep, and cattle. J Virol. 2002;76:12365–8. Hamir AN, Kunkle RA, Cutlip RC, Miller JM, O'Rourke KI, Williams ES, et al. Experimental transmission of chronic wasting disease agent to cattle by the intracerebral route. J Vet Diagn Invest. 2005;17:276–81. Bosque PJ. Bovine spongiform encephalopathy, chronic wasting disease, scrapie, and the threat to humans from prion disease epizootics. Curr Neurol Neurosci Rep. 2002;2:488–95. Majeed A, Lehmann P, Kirby L, Coleman MP. Mortality from dementias and neurodegenerative disorders in people aged 15–64 in England and Wales in 1979–96. BMJ. 1998;317:320–1. Collinge J, Rossor M. A new variant of prion disease. Lancet. 1996;347:916–7. National Creutzfeldt-Jakob Disease Surveillance Unit. Incidence of variant Creutzfeldt-Jakob disease onsets and deaths in the UK, January 1994–December 2004 [cited 2006 Aug 8]. Available from http://www.cjd.ed.ac.uk/vcjdq.htm Colorado Demography Office. Population totals [cited 2006 Aug 8]. Available from http://dola.colorado.gov/demog National Creutzfeldt-Jakob Disease Surveillance Unit. Creutzfeldt-Jakob disease surveillance in the UK, twelfth annual report, 2003 [cited 2006 Aug 8]. Available from http://www.cjd.ed.ac.uk/twelfth/rep2003.htm Belay ED, Maddox RA, Gambetti P, Schonberger LB. Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States. Neurology. 2003;60:176–81. Ladogana A, Puopolo M, Croes EA, Budka H, Jarius C, Collins S, et al. Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada. Neurology. 2005;64:1586–91. Colorado Demography Office. Population by age and gender [cited 2006 Aug 8]. Available from http://dola.colorado.gov/demog/CreateTable1.cfm Figures Figure 1. Location of chronic wasting disease (CWD)–endemic area in northeastern Colorado, USA... Figure 2. Colorado deaths 1979–2001 (left axis) with Creutzfeldt-Jakob disease (CJD) listed as the direct or contributory cause on the death certificate... Figure 3. Annual age-standardized Creutzfeldt-Jakob (CJD) death rates per million population were calculated for chronic wasting disease (CWD)–endemic...

Tables Table 1. ICD codes and corresponding event classifications for human prion disease deaths of Colorado residents, 1998–2001 Table 2. Characteristics of persons who died at ages >12 years, Colorado, 1979–2001 Table 3. Univariate relative risk estimates of available risk factors for Creutzfeldt-Jakob disease, data from Colorado death certificates, 1979–2001 Table 4. Results for primary predictors from multivariable analyses for CJD and expanded event definitions, data from Colorado death certificates, 1979–2001 Appendix Table 1. ICD-9 code classifications for expanded event definition, 1979–1998 Appendix Table 2. ICD-10 code classifications for expanded event definition, 1999–2001

Suggested Citation for this Article MaWhinney S, Pape WJ, Forster JE, Anderson CA, Bosque P, Miller MW. Human prion disease and relative risk associated with chronic wasting disease. Emerg Infect Dis [serial on the Internet]. 2006 Oct [date cited]. Available from http://www.cdc.gov/ncidod/EID/vol12no10/06-0019.htm

http://www.cdc.gov/ncidod/EID/vol12no10/06-0019.htm?s_cid=eid06_0019_e



TSS

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Elk escape from captive cervid facility in Pennsylvania near West Virginia border

West Virginia Division of Natural Resources

www.wvdnr.gov

Earl Ray Tomblin, Governor

Frank Jezioro, Director

News Release: November 4, 2011

Facebook: WV Commerce - State Parks

Hoy Murphy, Public Information Officer 304-957-9365 hoy.r.murphy@wv.gov

Contact: Curtis Taylor, Wildlife Resources Section Chief

304-558-2771 DNR.Wildlife@wv.gov

Elk escape from captive cervid facility in Pennsylvania near West Virginia border

SOUTH CHARLESTON, W.Va. – The West Virginia Division of Natural Resources (WVDNR) has confirmed with officials from the Pennsylvania Department of Agriculture (PDA) that at least two elk, including one adult bull and one cow, have escaped from a captive cervid facility (deer and elk farms) in Greene County, Pa. Greene County shares a common border with Marshall, Wetzel and Monongalia counties in West Virginia. The elk escaped from a captive cervid facility located approximately three miles from the West Virginia-Pennsylvania border.

The PDA regulates captive cervid facilities in Pennsylvania. A representative of the agency was unaware if the recent escaped elk were tagged. The WVDNR regulates captive cervid facilities in West Virginia. In West Virginia, all captive cervids in breeding facilities must be ear-tagged, and there are currently no reported elk escapes from any facility in West Virginia.

A bull elk has been seen recently in Wetzel County, W.Va., according to WVDNR officials. There have been no reports of cow elk sightings in either Wetzel County, W.Va., or Greene County, Pa. No free-ranging wild elk live within 150 miles of Wetzel County. The elk sighted in Wetzel County is likely the escaped animal from the captive facility in Pennsylvania.

Contact between escaped captive deer or elk and free-ranging white-tailed deer increases the risk of disease transmission from the captive animals to the native herd, according WVDNR biologists. The movement and/or escape of captive deer and elk increases this risk of contact and are one of the many possible modes of transmission for Chronic Wasting Disease (CWD) from captive cervids to free-ranging white-tailed deer.

The State of Missouri recently documented CWD in a captive cervid facility. Texas Parks and Wildlife had to euthanize a large captive deer herd after illegal importation of white-tailed deer from a captive facility in Arkansas.

“Monitoring and protecting West Virginia’s deer herd from CWD and other diseases is crucial to West Virginia’s economy and its natural resources,” said WVDNR Director Frank Jezioro. “Deer hunting provides tremendous recreational opportunities for hunters and wildlife viewers, has a large economic impact on its rural communities, and brings in many out-of-state hunters each season to West Virginia.”

WVDNR advises residents in Marshall, Wetzel and Monongalia counties to contact the Farmington District Office at 304-825-6787 if they see an elk in these counties. Hunters are reminded that it is illegal to harvest any free-ranging elk in West Virginia.

**DNR**

http://www.wvdnr.gov/2011news/11news217.shtm




CWD, GAME FARMS, BAITING, AND POLITICS


http://chronic-wasting-disease.blogspot.com/2009/01/cwd-game-farms-baiting-and-politics.html



http://chronic-wasting-disease.blogspot.com/2008/08/cwd-feeding-and-baiting-piles.html



Thursday, June 09, 2011

Detection of CWD prions in salivary, urinary, and intestinal tissues of deer: potential mechanisms of prion shedding and transmission

Nicholas J. Haley1, Candace K. Mathiason1, Scott Carver1, Mark Zabel1, Glenn C. Telling2, and Edward A. Hoover1,*

1 Department of Microbiology, Immunology, and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, Colorado, USA

2 Department of Molecular Biology and Genetics, University of Kentucky, Lexington, Kentucky, USA

* Corresponding author. Mailing address: Department of Microbiology, Immunology, and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO, 80523. Phone: (970)491-7587, Fax: (970)491-0523. Email: Edward.Hoover@colostate.edu

ABSTRACT

Efficient horizontal transmission is a signature trait of chronic wasting disease (CWD) in cervids. Infectious prions shed into excreta appear to play a key role in this facile transmission, as has been demonstrated by bioassay in cervid and transgenic species and serial protein misfolding cyclic amplification (sPMCA). However, the source(s) of infectious prions in these body fluids have yet to be identified. In the present study, we analyzed tissues proximate to saliva, urine, and feces production by sPMCA in an attempt to elucidate this unique aspect of CWD pathogenesis. Oropharyngeal, urogenital, and gastrointestinal tissues, along with blood and obex from CWD-exposed cervids (comprising 27 animals and >350 individual samples) were analyzed and scored based on apparent relative CWD burden. PrPCWD-generating activity was detected in a range of tissues, and was highest in salivary gland, urinary bladder, and the distal intestinal tract. In the same assays, blood from the same animals and unseeded normal brain homogenate controls (n= 116 of 117) remained negative. PrP-converting activity in peripheral tissues varied from 10-11 to 100 - fold that found in brain of the same animal. Deer with highest levels of PrPCWD amplification in the brain had higher and more widely disseminated prion amplification in excretory tissues. Interestingly, PrPCWD was not demonstrable by conventional western blotting in these excretory tissues, suggesting low prion burden or the presence of protease-sensitive infectious prions destroyed by harsh proteolytic treatments. These findings offer unique insights into the transmission of CWD in particular, and prion infection and trafficking overall.



http://jvi.asm.org/cgi/content/abstract/JVI.00425-11v1


http://wolftracksproductions.yuku.com/topic/4987/Detection--CWD-prions--salivary-urinary--intestinal-tissues


http://www.buckmasters.com/social/forums/cwd-and-deer-diseases/detection-of-cwd-prions-in-salivary-urinary-and-intestinal-tissues-of-deer-potential-mechanisms.aspx



Saturday, May 14, 2011

Modeling Routes of Chronic Wasting Disease Transmission: Environmental Prion Persistence Promotes Deer Population Decline and Extinction

http://chronic-wasting-disease.blogspot.com/2011/05/modeling-routes-of-chronic-wasting.html




Thursday, February 17, 2011

Environmental Sources of Scrapie Prions

http://scrapie-usa.blogspot.com/2011/02/environmental-sources-of-scrapie-prions.html




Tuesday, October 25, 2011

CWD Update 102 October 20, 2011

http://chronic-wasting-disease.blogspot.com/2011/10/cwd-update-102-october-20-2011.html




EFSA Journal 2011 The European Response to BSE: A Success Story

This is an interesting editorial about the Mad Cow Disease debacle, and it's ramifications that will continue to play out for decades to come ;

Monday, October 10, 2011

EFSA Journal 2011 The European Response to BSE: A Success Story

snip...

EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and ***chronic wasting disease (CWD) agents) might have zoonotic potential.

snip...



http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1


http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf



see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;



http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html



Monday, June 27, 2011

Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates


http://chronic-wasting-disease.blogspot.com/2011/06/zoonotic-potential-of-cwd-experimental.html




Saturday, October 29, 2011


Discovery of CWD in Missouri Reinforces Need for Vigilance in Texas


http://chronic-wasting-disease.blogspot.com/2011/10/discovery-of-cwd-in-missouri-reinforces.html




Sunday, July 03, 2011

Chronic Wasting Disease fears prompt new authority for Pennsylvania Game Commission director


http://chronic-wasting-disease.blogspot.com/2011/07/chronic-wasting-disease-fears-prompt.html


http://chronic-wasting-disease.blogspot.com/



TSS