Thursday, January 22, 2015

Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to disease etiology?

 Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to disease etiology?

 Atsushi Kobayashia,
 Piero Parchib,c,
 Masahito Yamadad,
 Paul Browne,
 Daniela Saverionic,
 Yuichi Matsuuraf,
 Atsuko Takeuchia,
 Shirou Mohria and
 Tetsuyuki Kitamotoa#
 + Author Affiliations

 aDepartment of Neurological Science, Tohoku University Graduate School of Medicine, Sendai, Japan
 bIRCCS, Istituto delle Scienze Neurologiche, Bologna, Italy
 cDepartment of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy
 dDepartment of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan
 eLaboratoire Français des Biotechnologies (LFB), Les Ulis, France
 fInfluenza and Prion Disease Research Center, National Institute of Animal Health, Tsukuba, Japan.

 ABSTRACT

 The genotype at polymorphic codon 129 of the PRNP gene has a profound influence on both phenotypic expression and prion strain susceptibility in humans. For example, while the most common sporadic Creutzfeldt-Jakob disease (CJD) subtype, sporadic CJD-MM1 (M1 strain), induces a single phenotype after experimental transmission regardless of the codon 129 genotype of the recipient animal, the phenotype elicited by sporadic CJD-VV2 (V2 strain), the second most common subtype, varies according to the host codon 129 genotype. In particular, the propagation of the V2 strain in codon 129 methionine homozygotes has only been linked to acquired forms of CJD such as plaque-type dura mater graft-associated CJD (dCJD), a subgroup of iatrogenic CJD with distinctive phenotypic features, but never observed in sporadic CJD. In the present study, we describe atypical CJD cases carrying the codon 129 methionine homozygosity, in a neurosurgeon and in a patient with a medical history of neurosurgery without dural grafting, showing the distinctive phenotypic features and transmission properties of plaque-type dCJD. These findings raise the possibility that the two cases, previously considered as sporadic CJD, might actually be acquired CJD caused by infection with the V2 strain. Thus, careful analyses of phenotypic features and transmission properties in atypical cases may be useful to distinguish acquired from sporadic cases of CJD.

 IMPORTANCE Susceptibility and phenotypic expression of Creutzfeldt-Jakob disease (CJD) depend on both the prion strain and genotype at polymorphic codon 129 of the PRNP gene. For example, propagation of the second most common sporadic CJD strain (V2 strain) into the codon 129 methionine homozygotes has been linked to plaque-type dura mater graft-associated CJD (dCJD), a subgroup of iatrogenic CJD with distinctive phenotypic features, but never observed in sporadic CJD. In the present study, we describe atypical CJD cases in a neurosurgeon and in a patient with a medical history of neurosurgery without dural grafting, showing the distinctive phenotypic features and transmission properties of plaque-type dCJD. These findings raise the possibility that the two cases, previously considered as sporadic CJD, might actually be acquired CJD caused by infection with the V2 strain.

 FOOTNOTES

 ↵#Coresponding author:
 Tetsuyuki Kitamoto; Department of Neurological Science, Tohoku University Graduate School of Medicine, Sendai, Japan; E-mail, kitamoto@med.tohoku.ac.jp
 Copyright © 2015, American Society for Microbiology. All Rights Reserved.


 http://jvi.asm.org/content/early/2015/01/19/JVI.03183-14.abstract?papetoc


 Sunday, October 27, 2013

 A Kiss of a Prion: New Implications for Oral Transmissibility

 http://transmissiblespongiformencephalopathy.blogspot.com/2013/10/a-kiss-of-prion-new-implications-for.html


 UPDATE* NOVEMBER 16, 2014

 vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???

 Friday, January 10, 2014

 Greetings again Friends, Neighbors, and Colleagues,

 I would kindly like to follow up on ‘vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???’ ran across an old paper from 1984, that some might find interest in, and I will update the link with this old science paper from 1984, a 2010 paper from Japan, and some information on scrapie transmission. The paper from Japan first, then the 1984 paper, and then the scrapie transmission studies.

 ***The occurrence of contact cases raises the possibility that transmission in families may be effected by an unusually virulent strain of the agent.

 http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/vpspr-sgss-sffi-tse-iatrogenic-by.html



 From: Terry S. Singeltary Sr.
 Sent: Saturday, November 15, 2014 9:29 PM
 To: Terry S. Singeltary Sr.
 Subject: THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE R. G. WILL 1984

 THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE

 R. G. WILL

 1984

 snip...

 http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/vpspr-sgss-sffi-tse-iatrogenic-by.html



 Friday, January 10, 2014

 vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???

 http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/vpspr-sgss-sffi-tse-iatrogenic-by.html


 Tuesday, December 30, 2014

 TSEAC USA Reason For Recalls Blood products, collected from a donors considered to be at increased risk for Creutzfeldt-Jakob Disease (CJD), were distributed END OF YEAR REPORT 2014

 http://tseac.blogspot.com/2014/12/tseac-usa-reason-for-recalls-blood.html


 Sunday, December 14, 2014

 ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD strains, TSE prion aka Mad Cow Disease United States of America Update December 14, 2014 Report

 http://transmissiblespongiformencephalopathy.blogspot.com/2014/12/alert-new-variant-creutzfeldt-jakob.html


 Thursday, January 15, 2015

 41-year-old Navy Commander with sporadic Creutzfeldt–Jakob disease CJD TSE Prion: Case Report

 http://creutzfeldt-jakob-disease.blogspot.com/2015/01/41-year-old-navy-commander-with.html


 Saturday, January 17, 2015

 *** Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed with the extremely rare Creutzfeldt-Jakob disease

 http://creutzfeldt-jakob-disease.blogspot.com/2015/01/becky-lockhart-46-utahs-first-female.html

 http://www.regulations.gov/#!documentDetail;D=APHIS-2014-0107-0003


 who’s kidding whom $$$ i.e. USDA INC AND THE OIE


 2014

 ***Moreover, L-BSE has been transmitted more easily to transgenic mice overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.

 ***It has been suggested that some sporadic CJD subtypes in humans may result from an exposure to the L-BSE agent.

 *** Lending support to this hypothesis, pathological and biochemical similarities have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129 of PRNP) [17], and between L-BSE infected non-human primate and another sCJD subtype (MM genotype) [15].

 snip...

 http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4213560/pdf/viruses-06-03766.pdf


 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


 Thursday, August 12, 2010

 Seven main threats for the future linked to prions

 First threat

 The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

 *** Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans.

 *** These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

 Second threat

 snip...

 http://www.neuroprion.org/en/np-neuroprion.html


 *** HUMAN MAD COW DISEASE nvCJD TEXAS CASE NOT LINKED TO EUROPEAN TRAVEL CDC ***

 Sunday, November 23, 2014

 *** Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in June 2014 confirmed as USA case NOT European ***

 the patient had resided in Kuwait, Russia and Lebanon. The completed investigation did not support the patient's having had extended travel to European countries, including the United Kingdom, or travel to Saudi Arabia. The specific overseas country where this patient’s infection occurred is less clear largely because the investigation did not definitely link him to a country where other known vCJD cases likely had been infected.

 http://vcjd.blogspot.com/2014/11/confirmed-variant-creutzfeldt-jakob.html


 *** U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001

 http://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html


 Wednesday, October 09, 2013

 WHY THE UKBSEnvCJD ONLY THEORY IS SO POPULAR IN IT'S FALLACY, £41,078,281 in compensation REVISED

 http://creutzfeldt-jakob-disease.blogspot.com/2013/10/why-ukbsenvcjd-only-theory-is-so.html


 Tuesday, March 11, 2014

 Science and Technology Committee Oral evidence: Blood, tissue and organ screening, HC 990 Wednesday 5 March 2014 SPORADIC CJD

 Actually, it is nearer 2 per million per year of the population will develop sporadic CJD, but your lifetime risk of developing sporadic CJD is about 1 in 30,000. So that has not really changed. When people talk about 1 per million, often they interpret that as thinking it is incredibly rare. They think they have a 1-in-a-million chance of developing this disease. You haven’t. You’ve got about a 1-in-30,000 chance of developing it.

 http://creutzfeldt-jakob-disease.blogspot.com/2014/03/science-and-technology-committee-oral.html


 Sunday, March 09, 2014

 A Creutzfeldt-Jakob Disease (CJD) Lookback Study: Assessing the Risk of Blood Borne Transmission of Classic Forms of Creutzfeldt-Jakob Disease

 *** FDA TSEAC CIRCUS AND TRAVELING ROAD SHOW FOR THE TSE PRION DISEASES ***

 http://creutzfeldt-jakob-disease.blogspot.com/2014/03/a-creutzfeldt-jakob-disease-cjd.html

 Friday, February 14, 2014

 Creutzfeldt-Jakob disease (CJD) biannual update (February 2014), with briefing on novel human prion disease National CJD Research and Surveillance Unit NCJDRSU

 http://creutzfeldt-jakob-disease.blogspot.com/2014/02/creutzfeldt-jakob-disease-cjd-biannual.html

 Tuesday, February 11, 2014

 Novant Health Forsyth Medical Center Information on potential CJD exposure

 http://creutzfeldt-jakob-disease.blogspot.com/2014/02/novant-health-forsyth-medical-center.html

 Thursday, April 17, 2014

 Novant: Three more may have been exposed to disease CJD

 http://creutzfeldt-jakob-disease.blogspot.com/2014/04/novant-three-more-may-have-been-exposed.html

 Wednesday, January 15, 2014

 *** INFECTION PREVENTION AND CONTROL OF CJD, VCJD AND OTHER HUMAN PRION DISEASES IN HEALTHCARE AND COMMUNITY SETTINGS Variably Protease-Sensitive Prionopathy (VPSPr) January 15, 2014

 http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/infection-prevention-and-control-of-cjd.html

 Saturday, November 16, 2013

 Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

 Infect Control Hosp Epidemiol.

 http://creutzfeldt-jakob-disease.blogspot.com/2013/11/management-of-neurosurgical-instruments.html

 Tuesday, May 28, 2013

 Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance

 http://creutzfeldt-jakob-disease.blogspot.com/2013/05/late-in-life-surgery-associated-with.html

 Wednesday, November 27, 2013

 NHS failed to sterilise surgical instruments contaminated with 'mad cow' disease

 http://transmissiblespongiformencephalopathy.blogspot.com/2013/11/nhs-failed-to-sterilise-surgical.html

 Thursday, January 23, 2014

 Medical Devices Containing Materials Derived from Animal Sources (Except for In Vitro Diagnostic Devices) [Docket No. FDA–2013–D–1574]

 http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/medical-devices-containing-materials.html

 Thursday, April 12, 2012

 Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010

 Eurosurveillance, Volume 17, Issue 15, 12 April 2012

 Research articles

 http://creutzfeldt-jakob-disease.blogspot.com/2012/04/health-professions-and-risk-of-sporadic.html

 Tuesday, May 28, 2013

 Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance

 http://creutzfeldt-jakob-disease.blogspot.com/2013/05/late-in-life-surgery-associated-with.html

 Tuesday, May 21, 2013

 CJD, TSE, PRION, BLOOD Abstracts of the 23rd Regional Congress of the International Society of Blood Transfusion, Amsterdam, The Netherlands, June 2-5, 2013

 http://transmissiblespongiformencephalopathy.blogspot.com/2013/05/cjd-tse-prion-blood-abstracts-of-23rd.html

 Tuesday, March 5, 2013

 Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION)

 FDA believes current regulation protects the public from BSE but reopens comment period due to new studies

 http://transmissiblespongiformencephalopathy.blogspot.com/2013/03/use-of-materials-derived-from-cattle-in_6452.html

 Thursday, October 25, 2012

 Current limitations about the cleaning of luminal endoscopes and TSE prion risk factors there from

 Article in Press

 http://creutzfeldt-jakob-disease.blogspot.com/2012/10/current-limitations-about-cleaning-of.html

 Saturday, January 16, 2010

 Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al

 Evidence For CJD/TSE Transmission Via Endoscopes

 From Terry S. Singletary, Sr flounder@wt.net 1-24-3

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

 http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html

 Professor Michael Farthing wrote:

 Louise Send this to Bramble (author) for a comment before we post. Michael

 http://creutzfeldt-jakob-disease.blogspot.com/2010/01/evidence-for-cjd-tse-transmission-via.html

 Tuesday, July 31, 2012

 11 patients may have been exposed to fatal disease Creutzfeldt-Jakob Disease CJD Greenville Memorial Hospital

 http://creutzfeldt-jakob-disease.blogspot.com/2012/07/11-patients-may-have-been-exposed-to.html

 Thursday, August 02, 2012

 CJD case in Saint John prompts letter to patients Canada CJD case in Saint John prompts letter to patients

 http://creutzfeldt-jakob-disease.blogspot.com/2012/08/cjd-case-in-saint-john-prompts-letter.html

 Saturday, February 12, 2011

 Another Pathologists dies from CJD, another potential occupational death ?

 another happenstance of bad luck, a spontaneous event from nothing, or friendly fire ???

 http://creutzfeldt-jakob-disease.blogspot.com/2011/02/another-pathologists-dies-from-cjd.html

 Thursday, July 08, 2010

 GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010

 http://creutzfeldt-jakob-disease.blogspot.com/2010/07/global-clusters-of-creutzfeldt-jakob.html

 Sunday, May 10, 2009

 Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

 http://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html

 Thursday, January 29, 2009

 Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008

 (WARNING TO Neurosurgeons and Ophthalmologists)

 Volume 15, Number 2-February 2009 Research

 http://creutzfeldt-jakob-disease.blogspot.com/2009/01/medical-procedures-and-risk-for.html

 Wednesday, August 20, 2008

 Tonometer disinfection practice in the United Kingdom: A national survey

 http://creutzfeldt-jakob-disease.blogspot.com/2008/08/tonometer-disinfection-practice-in.html

 Tuesday, August 12, 2008

 Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)

 http://creutzfeldt-jakob-disease.blogspot.com/2008/08/biosafety-in-microbiological-and.html

 Monday, December 31, 2007

 Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation

 http://creutzfeldt-jakob-disease.blogspot.com/2007_12_01_archive.html

 Subject: CJD: update for dental staff

 Date: November 12, 2006 at 3:25 pm PST

 1: Dent Update. 2006 Oct;33(8):454-6, 458-60.

 CJD: update for dental staff.

 http://seac992007.blogspot.com/2008/06/seac-2008-one-hundredth-meeting-of.html

 Friday, July 19, 2013

 Beaumont Hospital in Dublin assessing patients for CJD

 http://creutzfeldt-jakob-disease.blogspot.com/2013/07/beaumont-hospital-in-dublin-assessing.html

 Saturday, September 21, 2013

 CJD CONFIRMED in patient at New Hampshire Department of Health and Human Services (DHHS), Catholic Medical Center (CMC), and the Manchester Health Department (MHD)

 http://creutzfeldt-jakob-disease.blogspot.com/2013/09/cjd-confirmed-in-patient-at-new.html

 Thursday, September 26, 2013

 Minimise transmission risk of CJD and vCJD in healthcare settings Guidance

 http://creutzfeldt-jakob-disease.blogspot.com/2013/09/minimise-transmission-risk-of-cjd-and.html

 Tuesday, April 01, 2014

 Questions linger in U.S. CJD cases 2005, and still do in 2014

 http://creutzfeldt-jakob-disease.blogspot.com/2014/04/questions-linger-in-us-cjd-cases-2005.html

 CJD QUESTIONNAIRE USA

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

 http://cjdquestionnaire.blogspot.com/

 CJD VOICE

 http://creativegumbo.net/cjdvoice/

 *** 1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8

 Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.

 Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

 Laboratory of Central Nervous System Studies, National Institute of

 Neurological Disorders and Stroke, National Institutes of Health,

 Bethesda, MD 20892.

 Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

 PMID: 8006664 [PubMed - indexed for MEDLINE]

 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract

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

 http://www.pnas.org/content/97/7/3418.full

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

 http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/

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

 http://www.landesbioscience.com/journals/prion/NicholsPRION3-3.pdf

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

 http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract

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

 http://transmissiblespongiformencephalopathy.blogspot.com/2013/07/rapid-assessment-of-bovine-spongiform.html

 PPo4-4:

 Survival and Limited Spread of TSE Infectivity after Burial

 http://www.prion2010.org/bilder/prion_2010_program_latest_w_posters_4_.pdf?139&PHPSESSID=a30a38202cfec579000b77af81be3099

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

 http://cdmrp.army.mil/prevfunded/nprp/NPRP_Summit_Final_Report.pdf

 Diagnosis and Reporting of Creutzfeldt-Jakob Disease

 Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

 Diagnosis and Reporting of Creutzfeldt-Jakob Disease

 To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

 Terry S. Singeltary, Sr Bacliff, Tex

 1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.

 http://jama.jamanetwork.com/article.aspx?articleid=1031186


 26 March 2003

 Terry S. Singeltary, retired (medically) CJD WATCH

 I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

 http://www.neurology.org/content/60/2/176/reply#neurology_el_535


 2 January 2000

 British Medical Journal

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

 http://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well


 15 November 1999

 British Medical Journal

 vCJD in the USA * BSE in U.S.

 http://www.bmj.com/rapid-response/2011/10/28/re-vcjd-usa-bse-us


 The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003 doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI

 Tracking spongiform encephalopathies in North America

 Original

 Xavier Bosch

 “My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.” 49-year—old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt—Jakob ...

 http://www.thelancet.com/journals/laninf/article/PIIS1473-3099(03)00715-1/abstract


 Suspect symptoms

 What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

 28 Mar 01

 Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

 Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

 "This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb...

 http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html


 Evidence for zoonotic potential of ovine scrapie prions

 Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 Article tools Citation Reprints Rights & permissions Article metrics

 Abstract

 Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human ​prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

 Subject terms: Biological sciences• Medical research At a glance

 http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html


 The Pathological Protein:

 Mad Cow, Chronic Wasting, and Other Deadly Prion Diseases

 Philip Yam

 ''Answering critics like Terry Singeltary, who feels that the US undercounts CJD, Schonberger _conceded_ that the current surveillance system has errors but stated that most of the errors will be confined to the older population''....end

 snip...

 His combative, blunt, opinion- ated style sometimes borders on obsessive ranting that earns praise from some officials and researchers but infuriates others especially when he repeats his conviction that "the government has lied to us, the feed industry has lied to us all over a buck." As evidence, Singeltary cites the USDA's testing approach, which targets downer cows and examined 19,900 of them in 2002. To him, the USDA should test 1 mil- lion cattle, because the incidence of BSE may be as low as one in a mil- lion, as it was in some European countries. That the U.S. does not, he thinks, is a sign that the government is really not interested in finding mad cows because of fears of an economic disaster.

 Singeltary got into the field of transmissible spongiform encepha- lopathy in 1997, just after his mother died of sporadic CJD. She had an especially aggressive version the Heidenhain variant that first causes the patient to go blind and then to deteriorate rapidly She died just ten weeks after her symptoms began. Singeltary, who said he had watched his grandparents die of cancer, considered her death by CJD to be much, much worse: "It's something you never forget." Her uncon- trollable muscle twitching became so bad "that it took three of us to hold her one time," Singeltary recalled. "She did everything but levitate in bed and spin her head."

 http://www.amazon.com/The-Pathological-Protein-Chronic-Diseases/dp/0387955089

 14th ICID International Scientific Exchange Brochure -

 Final Abstract Number: ISE.114

 Session: International Scientific Exchange

 Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

 T. Singeltary

 Bacliff, TX, USA

 Background:

 An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

 Methods:

 12 years independent research of available data

 Results:

 I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

 Conclusion:

 I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

 http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf


 re-Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease

 Received July 24, 2014; Accepted September 16, 2014; Published November 3, 2014

 Singeltary comment ;

 http://www.plosone.org/annotation/listThread.action?root=82860


 RE: re-Human Prion Diseases in the United States

 part 2 flounder replied to flounder on 02 Jan 2010 at 21:26 GMT

 No competing interests declared.

 see full text ;

 http://www.plosone.org/annotation/listThread.action?root=363


 *** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;


 http://www.plosone.org/annotation/listThread.action;jsessionid=635CE9094E0EA15D5362B7D7B809448C?root=7143


 "Löcher wie in einem Schweizer Käse" hat auch Terry Singeltary im Regelwerk der FDA ausgemacht. Der Texaner kam auf einem tragischen Umweg zu dem Thema: Nachdem seine Mutter 1997 binnen weniger Wochen an der Creutzfeldt-Jakob-Krankheit gestorben war, versuchte er, die Ursachen der Infektion aufzuspüren. Er klagte auf die Herausgabe von Regierungsdokumenten und arbeitete sich durch Fachliteratur; heute ist er überzeugt, dass seine Mutter durch die stetige Einnahme von angeblich kräftigenden Mitteln erkrankte, in denen - völlig legal - Anteile aus Rinderprodukten enthalten sind.


 Von der Fachwelt wurde Singeltary lange als versponnener Außenseiter belächelt. Doch mittlerweile sorgen sich auch Experten, dass ausgerechnet diese verschreibungsfreien Wundercocktails zur Stärkung von Intelligenz, Immunsystem oder Libido von den Importbeschränkungen ausgenommen sind. Dabei enthalten die Pillen und Ampullen, die in Supermärkten verkauft werden, exotische Mixturen aus Rinderaugen; dazu Extrakte von Hypophyse oder Kälberföten, Prostata, Lymphknoten und gefriergetrocknetem Schweinemagen. In die USA hereingelassen werden auch Blut, Fett, Gelatine und Samen. Diese Stoffe tauchen noch immer in US-Produkten auf, inklusive Medizin und Kosmetika. Selbst in Impfstoffen waren möglicherweise gefährliche Rinderprodukte enthalten. Zwar fordert die FDA schon seit acht Jahren die US-Pharmaindustrie auf, keine Stoffe aus Ländern zu benutzen, in denen die Gefahr einer BSE-Infizierung besteht. Aber erst kürzlich verpflichteten sich fünf Unternehmen, darunter Branchenführer wie GlaxoSmithKline, Aventis und American Home Products, ihre Seren nur noch aus unverdächtigem Material herzustellen.


 "Its as full of holes as Swiss Cheese" says Terry Singeltary of the FDA regulations. ...

 http://www.spiegel.de/spiegel/print/d-18578755.html


 New York Times Magazine The Case of the Cherry Hill Cluster By D.T. MAX

 Published: March 28, 2004

 snip...

 Skarbek did not know how to surmount this objection. But she was a go-getter. She wasn't about to give up on her cluster so easily. Fortunately, she was in contact with Terry Singeltary. She had seen his name quoted often on the Web in articles on C.J.D. and mad cow. Singeltary lost his mother to an extremely rare strain of sporadic C.J.D. in 1997. Soon after, he learned that a year earlier to the day, the mother of his next-door neighbor died of the disease. Since that time, he has become convinced that these sporadic cases are not sporadic at all, that mad cow is now a disease of humans in America. He said he believes that his mother was accidentally infected during surgery and the mother of his neighbor from taking nutritional supplements made from high-risk bovine tissue, which he calls ''mad cow in a pill.''

 Singeltary has a sloping face and slicked-back hair. He is nearsighted, with small blue eyes. He looks like Lewis Carroll's White Rabbit. From his living room in Bacliff, Tex., he dominates the listservs and message boards of an online debate over sporadic C.J.D. -- the scientists who say it exists; the heartbroken family members who doubt it. Early, deep in his grief, he would sign his e-mail messages to scientists, ''I am the madson of a deadmom who died of madcow.'' Singeltary turned out to be helpful for Skarbek. He pointed her to a paper that was published in 2002 in the journal of the European Molecular Biology Organization by John Collinge, the premier prion researcher in England. Collinge argued that experiments conducted in mice suggest that infections with mad cow can sometimes look like sporadic C.J.D. Collinge accepted the implications: he recommended that ''serious consideration should be given'' to the idea that some of the more recent sporadic C.J.D. cases in Europe were in fact related to mad cow disease.

 http://www.artsci.wustl.edu/~anthro/articles/The%20Case%20of%20the%20Cherry%20Hill%20Cluster.htm


 Sunday, January 11, 2015

 Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission

 http://www.regulations.gov/#!documentDetail;D=APHIS-2014-0107-0003

 http://bovineprp.blogspot.com/2015/01/docket-no-aphis-2014-0107-bovine.html


 Evidence for zoonotic potential of ovine scrapie prions

 Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 Article tools Citation Reprints Rights & permissions Article metrics

 Abstract

 Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human ​prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

 Subject terms: Biological sciences• Medical research At a glance

 http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6821.html


 Suspect symptoms

 What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

 28 Mar 01 Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

 Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

 "This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb...

 2001

 Suspect symptoms

 What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?

 28 Mar 01

 Like lambs to the slaughter

 31 March 2001

 by Debora MacKenzie Magazine issue 2284.

 FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

 Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.

 Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.

 "This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.

 Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.

 Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.

 As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.

 "The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.

 But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.

 People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.

 But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."

 There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.

 Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.

 http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html

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

 5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

 snip...

 R. BRADLEY

 http://collections.europarchive.org/tna/20080102222950/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf


 1: J Infect Dis 1980 Aug;142(2):205-8

 Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

 Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

 Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

 snip...

 The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.

 PMID: 6997404

 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract


 Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"

 Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.

 snip...

 76/10.12/4.6

 http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf


 Nature. 1972 Mar 10;236(5341):73-4.

 Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).

 Gibbs CJ Jr, Gajdusek DC.

 Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

 Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

 C. J. GIBBS jun. & D. C. GAJDUSEK

 National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

 SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).

 http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html


 http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html


 Saturday, December 13, 2014

 *** Terry S. Singeltary Sr. Publications TSE prion disease Peer Review ***

 Diagnosis and Reporting of Creutzfeldt-Jakob Disease

 Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

 snip...

 http://transmissiblespongiformencephalopathy.blogspot.com/2014/12/terry-s-singeltary-sr-publications-tse.html


 layperson

 Thank you,

 Sincerely,

 Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net

posted by Terry S. Singeltary Sr. at 10:46 AM

Saturday, January 17, 2015

Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed with the extremely rare Creutzfeldt-Jakob disease

 


Greetings Honorable Governor Herbert Sir and Utah Government officials et al, 
 
in reply to ;
 
 
“I have known and been a friend of the Lockhart family for 25 years and I am terribly saddened by the news of Becky’s sudden and unexpected passing.
 
 
Becky Lockhart 46, Utah’s first female House speaker, dies diagnosed with the extremely rare Creutzfeldt-Jakob disease CJD TSE Prion
 
Becky Lockhart, Utah’s first female House speaker, dies
 
Posted 2:14 pm, January 17, 2015, by Ben Winslow, Tiffany DeMasters, Andrew Klay and Mark Green, Updated at 05:42pm, January 17, 2015
 
See the two videos above for a report on Lockhart’s passing as well as a segment detailing her life and influence in Utah politics.
 
SALT LAKE CITY — Becky Lockhart, the first woman Speaker of the House in Utah, died Saturday at age 46, her family confirmed.
 
In a statement, the Lockhart family said she passed away at her Provo home around noon surrounded by friends and family. Lockhart died “from an unrecoverable and extremely rare neurodegenerative brain disease,” the family said.
 
“She was at peace and surrounded by her family,” said family spokesman Sen. Curt Bramble in the statement. “It’s a credit to world-class doctors and Becky’s indomitable spirit that they were able to have these past days together with her. The outpouring of prayers and positive thoughts continue to help sustain the family, and they thank everyone for their support.”
 
At a news conference Saturday afternoon, doctors at University Medical Center and Lockhart’s family disclosed that she had been diagnosed with the extremely rare Creutzfeldt-Jakob disease. They said it is a “one in a million” diagnosis and the Speaker initially sought treatment for vertigo.
 
The degenerative disease progressed quickly.
 
 
 
Becky Lockhart, history-making Utah lawmaker, dies at 46
 
By ROBERT GEHRKE | The Salt Lake Tribune
 
 
 
My sincere condolences to the family and friends of the House Speaker Becky Lockhart. I am deeply saddened hear this.
 
with that said, with great respect, I must ask each and every one of you Politicians that are so deeply saddened to hear of this needless death of the Honorable House Speaker Becky Lockhart, really, cry me a friggen river. I am seriously going to ask you all this...I have been diplomatic for about 17 years and it has got no where. people are still dying. so, are you all stupid or what??? how many more need to die ??? how much is global trade of beef and other meat products that are not tested for the TSE prion disease, how much and how many bodies is this market worth? (I’m still a meat eater, don’t ask me why). NOT counting friendly fire i.e iatrogenic CJD TSE prion (who cares about second hand transmission$), nobody cares about any traceback anyone$$$). someone please tell me a number, how many more need to die, before this CJD TSE Prion mutates from one of the many TSEs in many different species right here in the USA, and really becomes nasty, say like the way CWD transmits in cervids (you all do know there is more than one strain of CWD in cervid)??? how many lobbyist does it take for you all to keep buying into this BSe Bullshit Encephalopathy BSe, science that is 30+ years old the USDA OIE Inc keep going by, ......really what the f... does it take to wake you folks up$$$ how many bodies??? how young do they have to go??? like the UK, down in the low teens? do you people not know what iatrogenic means? really...somebody please tell me, are we really going to keep going down this road??? I apologize for this rant, the cuss words, but these needless deaths are getting younger and younger from the infamous sporadic CJDs, the count is rising from year to year, with the none detected, misdiagnosed, keep going on as such, and I am getting old and gray. I watched my mother die from this damn disease on December 14, 1997. over the previous few months, at times, she jerked so bad it took 3 adults to hold her down to keep her from hurting herself, all the while screaming what’s wrong with me, why can’t I stop this. she did everything but spin her head 360 degrees on her shoulders. you never forget. YOU NEVER FORGET. then you listen to the same store bought junk science for the next 17 years via the USDA and OIE inc. I’m tired of listening to it. probable does not matter to anyone, but I said back in 2003, after that infamous day on December 23, 2003 when the USDA et al lost their BSE Gold care i.e. BSE FREE status. I said back then and was laughed at that the TSE Prion was linked to Alzheimer’s and other neurological disease, and I said that sporadic CJD was linked to mad cow disease some how. I have said that CWD in cervids is a great risk factor to humans, along with the atypical TSE prion disease. today, all has come to pass, all has come true, but nobody bothers to read the science. ...again, sorry about the cussing, but this really made me angry and I never been very diplomatic, but with this, and the recent case of the Navy Commander that was 41 years old, and all the past young victims of the TSE prion aka mad cow type disease in the USA, these cases of CJD TSE prion mad cow type disease cases in the USA are getting younger and younger, the names of these types disease are now varying from the sCJD subtypes, to the sporadic FFI, sporadic GSS, to the infamous VPSPr TSE prion disease. on my Mothers grave, you all better take heed...those fool hearted politicians that kept eating a hamburger for the camera’s to prove beef was safe, were just fools. it goes so much further than that. you people wanted that damn BSE MRR policy of the BSE GBR risk assessments after that fateful day, well, you got it. now, how long are you willing to keep passing the TSE prion mad cow type agent around.
 
I’m suppose to be retiring from this ongoing nightmare anyway....please stay with me, I urge you all to read the science, peer review, the confidential data from back in the BSE Inquiry days, the FOIA on feed and and TSE prion here in the USA, the truth is here, file it away, or not, all over my failed endeavor to try and warn and rid the word of the CJD TSE prion aka mad cow type disease. I have wasted 17 years daily. nobody would listen to me, I have no PhDs. I am only a layperson.
 
with great respect, and sincere urgency, I send you the following science, please read and try to understand this. that’s all I can say, and I have probably said too much...sorry I cussed...somebody please listen. I am not a Brad Pitt type star that can speak to Congress, there to busy trying to shut the government down (either side), both side have ignored this debacle, but for whatever it’s worth, I am going to give it a shot here...hey, the Australian Cattlemen Ass. Brad Bellinger read some of my statement’s in the Hansard’s Parliament, some maybe someone is listening.........
 
I kindly and respectfully submit the following ;
 
 
Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission ;

I believe that there is more risk to the world from Transmissible Spongiform Encephalopathy TSE prion aka mad cow type disease now, coming from the United States and all of North America, than there is risk coming to the USA and North America, from other Countries. I am NOT saying I dont think there is any risk for the BSE type TSE prion coming from other Countries, I am just saying that in 2015, why is the APHIS/USDA/FSIS/FDA still ignoring these present mad cow risk factors in North America like they are not here?

North America has more strains of TSE prion disease, in more species (excluding zoo animals in the early BSE days, and excluding the Feline TSE and or Canine TSE, because they dont look, and yes, there has been documented evidence and scientific studies, and DEFRA Hound study, that shows the canine spongiform encephalopathy is very possible, if it has not already happened, just not documented), then any other Country in the world. Mink TME, Deer Elk cervid CWD (multiple strains), cBSE cattle, atypical L-type BSE cattle, atypical H-type BSE cattle, atyical HG type BSE cow (the only cow documented in the world to date with this strain), typical sheep goat Scrapie (multiple strains), and the atypical Nor-98 Scrapie, which has been linked to sporadic CJD, Nor-98 atypical Scrapie has spread from coast to coast. sporadic CJD on the rise, with different strains mounting, victims becoming younger, with the latest nvCJD human mad cow case being documented in Texas again, this case, NOT LINKED TO EUROPEAN TRAVEL CDC.

typical BSE can propagate as nvCJD and or sporadic CJD (Collinge et al), and sporadic CJD has now been linked to atypical BSE, Scrapie and atypical Scrapie, and scientist are very concerned with CWD TSE prion in the Cervid populations. in my opinion, the BSE MRR policy, which overtook the BSE GBR risk assessments for each country, and then made BSE confirmed countries legal to trade mad cow disease, which was all brought forth AFTER that fateful day December 23, 2003, when the USA lost its gold card i.e. BSE FREE status, thats the day it all started. once the BSE MRR policy was shoved down every countries throat by USDA inc and the OIE, then the legal trading of Scrapie was validated to be a legal trading commodity, also shoved through by the USDA inc and the OIE, the world then lost 30 years of attempted eradication of the BSE TSE prion disease typical and atypical strains, and the BSE TSE Prion aka mad cow type disease was thus made a legal trading commodity, like it or not. its all about money now folks, trade, to hell with human health with a slow incubating disease, that is 100% fatal once clinical, and forget the fact of exposure, sub-clinical infection, and friendly fire there from i.e. iatrogenic TSE prion disease, the pass it forward mode of the TSE PRION aka mad cow type disease. its all going to be sporadic CJD or sporadic ffi, or sporadic gss, or now the infamous VPSPr. ...problem solved $$$

the USDA/APHIS/FSIS/FDA triple mad cow BSE firewall, well, that was nothing but ink on paper.

for this very reason I believe the BSE MRR policy is a total failure, and that this policy should be immediately withdrawn, and set back in place the BSE GBR Risk Assessments, with the BSE GBR risk assessments set up to monitor all TSE PRION disease in all species of animals, and that the BSE GBR risk assessments be made stronger than before.

lets start with the recent notice that beef from Ireland will be coming to America.

Ireland confirmed around 1655 cases of mad cow disease. with the highest year confirming about 333 cases in 2002, with numbers of BSE confirmed cases dropping from that point on, to a documentation of 1 confirmed case in 2013, to date. a drastic decrease in the feeding of cows to cows i.e. the ruminant mad cow feed ban, and the enforcement of that ban, has drastically reduced the number of BSE cases in Europe, minus a few BABs or BARBs. a far cry from the USDA FDA triple BSE firewall, which was nothing more than ink on paper, where in 2007, in one week recall alone, some 10 MILLION POUNDS OF BANNED POTENTIAL MAD COW FEED WENT OUT INTO COMMERCE IN THE USA. this is 10 years post feed ban. in my honest opinion, due to the blatant cover up of BSE TSE prion aka mad cow disease in the USA, we still have no clue as to the true number of cases of BSE mad cow disease in the USA or North America as a whole. ...just saying.

Number of reported cases of bovine spongiform encephalopathy (BSE) in farmed cattle worldwide* (excluding the United Kingdom)

Country/Year

snip...please see attached pdf file, with references of breaches in the USA triple BSE mad cow firewalls, and recent science on the TSE prion disease. ...TSS

Attachments

 (1)

Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission

View Attachment:
 
http://www.regulations.gov/#!documentDetail;D=APHIS-2014-0107-0003
 
Sunday, January 11, 2015
 
Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission
 
 
 
 
 
 
Sporadic Creutzfeldt–Jakob Disease in a Navy Commander: Case Report and Review of the Literature
 
LT Xin Wei , MC USN
 
Carlson Lawall , MD
 
CAPT Patricia Pepper , MC USN
 
Naval Medical Center San Diego, 34800 Bob Wilson Drive, San Diego, CA 92134.
 
 
Abstract ABSTRACT
 
Sporadic Creutzfeldt–Jakob disease is a rare disorder that typically presents as rapidly progressive dementia. We present a case of a highly functioning 41-year-old Navy Commander with sporadic Creutzfeldt–Jakob disease, to include the diagnostic challenges, rapid clinical deterioration, and limited treatment options.
 
REFERENCES
 
1. Geschwind MD, Shu H, Haman A, Sejvar JJ, Miller BL: Rapidly progressive dementia. Ann Neurol 2008; 64(1): 97–108. 2. Rosenbloom MH, Atri A: The evaluation of rapidly progressive dementia. Neurologist 2011; 17(2): 67–74. 3. Coulthart MB, Jansen GH, Olsen E, et al: Diagnostic accuracy of cerebrospinal fluid protein markers for sporadic Creutzfeldt-Jakob disease in Canada: a 6-year prospective study. BMC Neurol 2011; 11: 133. 4. Skillback T, Rosén C, Asztely F, Mattsson N, Blennow K, Zetterberg H: Diagnostic performance of cerebrospinal fluid total tau and phosphorylated tau in Creutzfeldt-Jakob disease: results from the Swedish Mortality Registry. JAMA Neurol 2014; 71(4): 476–83. 5. Orru CD, Bongianni M, Tonoli G, et al: A test for Creutzfeldt–Jakob disease using nasal brushings. N Engl J Med 2014; 371: 519–29. 6. Moda F, Gambetti P, Notari S, et al: Prions in the urine of patients with variant Creutzfeldt–Jakob disease. N Engl J Med 2014; 371: 530–9. 7. Rabinovici GD, Wang PN, Levin J, et al: First symptom in sporadic Creutzfeldt-Jakob disease. Neurology 2006; 66: 286–7. 8. Holman RC, Khan AS, Belay ED, Schonberger LB: Creutzfeldt-Jakob disease in the United States, 1979–1994: using national mortality data to assess the possible occurrence of variant cases. Emerg Infect Dis 1996; 2: 333–7. 9. Brown P, Cathala F, Castaigne P, Gajdusek DC: Creutzfeldt-Jakob disease: clinical analysis of a consecutive series of 230 neuropathologically verified cases. Ann Neurol 1986; 20: 597–602. 10. WHO: Variant Creutzfeldt-Jakob Disease. Available at http://www.who.int/mediacentre/factsheets/fs180/en/; accessed August 11, 2014. 11. Beghi E, Gandolfo C, Ferrarese C, et al: Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease: facts and uncertainties underlying the causal link between animal and human diseases. Neurol Sci 2004; 25(3): 122–9. 12. Hammersmith KM, Cohen EJ, Rapuano CJ, Laibson PR: Creutzfeldt-Jakob disease following corneal transplantation. Cornea 2004; 23(4): 406–8. 13. Thomas JG, Chenoweth CE, Sullivan SE: Iatrogenic Creutzfeldt-Jakob disease via surgical instruments. J Clin Neurosci 2013; 20(9): 1207–12. 14. Zaman SM, Hill FG, Palmer B, et al: The risk of variant Creutzfeldt-Jakob disease among UK patients with bleeding disorders, known to have received potentially contaminated plasma products. Haemophilia 2011; 17(6): 931–7. 15. Shiga Y, Miyazawa K, Sato S, et al: Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology 2004; I63: 443–9. 16. CDC: CDC's Diagnostic Criteria for Creutzfeldt-Jakob Disease (CJD), 2010. Available at http://www.cdc.gov/ncidod/dvrd/cjd/diagnostic_criteria.html; accessed September 1, 2013. 17. Vitali P, Maccagnano E, Caverzasi E, et al: Diffusion-weighted MRI hyperdensity patterns differentiate CJD from other rapid dementias. Neurology 2011; 76(20): 1711–9. 18. USCF: CJD Treatments and Trials. Available at http://memory.ucsf.edu/cjd/medical/treatments; accessed November 5, 2013. 19. Geschwind MD, Kuo AL, Wong KS, et al: Quinacrine treatment trial for sporadic Creutzfeldt-Jakob disease. Neurology 2013; 81(23): 2015–23. 20. Haik S, Marcon G, Mallet A, et al: Doxycycline in Creutzfeldt-Jakob disease: a phase 2, randomised, double-blind, placebo-controlled trial. Lancet Neurol 2014; 13(2): 150–8.
 
 
we now know that some sporadic CJD has been linked to atypical BSE, atypical scrapie, and recent to typical scrapie again, and the CWD in cervids is a very real risk factor.
 
this is another unusually young victim for the sporadic CJD. I have argued for 17 years that the diagnostic criteria to differentiate between the nvCJD and the sporadic CJD is far from perfect. I kindly submit the following ;
 
*** HUMAN MAD COW DISEASE nvCJD TEXAS CASE NOT LINKED TO EUROPEAN TRAVEL CDC ***
 
Sunday, November 23, 2014
 
*** Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in June 2014 confirmed as USA case NOT European ***
 
the patient had resided in Kuwait, Russia and Lebanon. The completed investigation did not support the patient's having had extended travel to European countries, including the United Kingdom, or travel to Saudi Arabia. The specific overseas country where this patient’s infection occurred is less clear largely because the investigation did not definitely link him to a country where other known vCJD cases likely had been infected.
 
 
Sunday, December 14, 2014
 
*** ALERT new variant Creutzfeldt Jakob Disease nvCJD or vCJD, sporadic CJD strains, TSE prion aka Mad Cow Disease United States of America Update December 14, 2014 Report ***
 
 
UPDATE* NOVEMBER 16, 2014 vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???
 
Friday, January 10, 2014
 
Greetings again Friends, Neighbors, and Colleagues,
 
I would kindly like to follow up on ‘vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???’ ran across an old paper from 1984, that some might find interest in, and I will update the link with this old science paper from 1984, a 2010 paper from Japan, and some information on scrapie transmission. The paper from Japan first, then the 1984 paper, and then the scrapie transmission studies.
 
***The occurrence of contact cases raises the possibility that transmission in families may be effected by an unusually virulent strain of the agent.
 
 
From: Terry S. Singeltary Sr.
 
Sent: Saturday, November 15, 2014 9:29 PM
 
To: Terry S. Singeltary Sr.
 
Subject: THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE R. G. WILL 1984
 
THE EPIDEMIOLOGY OF CREUTZFELDT-JAKOB DISEASE
 
R. G. WILL
 
1984
 
snip...old link take a while to load...
 
 
my link...
 
vpspr-sgss-sffi-tse-iatrogenic TSE Prion, what if ???
 
 
Friday, January 10, 2014
 
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???
 
 
Sunday, April 06, 2014
 
*** SPORADIC CJD and the potential for zoonotic transmission there from, either directly or indirectly via friendly fire iatrogenic mode, evidence to date ***
 
 
Sunday, June 29, 2014
 
*** Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014
 
 
Thursday, January 15, 2015
 
*** 41-year-old Navy Commander with sporadic Creutzfeldt–Jakob disease CJD TSE Prion: Case Report
 
 
Tuesday, December 30, 2014
 
TSEAC USA Reason For Recalls Blood products, collected from a donors considered to be at increased risk for Creutzfeldt-Jakob Disease (CJD), were distributed END OF YEAR REPORT 2014
 
 
Tuesday, February 8, 201 2002ish DeepThroat
 
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people.........Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why???? than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....
 
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!
 
And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...
 
Thanks as always for your help.
 
(Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"
 
again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
 
You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
 
==============end...TSS=============
 
*** U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001 ***
 
 
Thursday, February 24, 2011
 
The risk of variant Creutzfeldt-Jakob disease among UK patients with bleeding disorders, known to have received potentially contaminated plasma products
 
 
BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein
 
*** Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPSc type 2.
 
These data suggest that more than one BSEderived prion strain might infect humans;
 
***it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.
 
snip...
 
These studies further strengthen the evidence that vCJD is caused by a BSE-like prion strain.
 
Also, remarkably, the key neuropathological hallmark of vCJD, the presence of abundant florid PrP plaques, can be recapitulated on BSE or vCJD transmission to these mice.
 
***However, the most surprising aspect of the studies was the finding that an alternate pattern of disease can be induced in 129MM Tg35 mice from primary transmission of BSE, with a molecular phenotype indistinguishable from that of a subtype of sporadic CJD. This finding has important potential implications as it raises the possibility that some humans infected with BSE prions may develop a clinical disease indistinguishable from classical CJD associated with type 2 PrPSc. This is, in our experience, the commonest molecular sub-type of sporadic CJD. In this regard, it is of interest that the reported incidence of sporadic CJD has risen in the UK since the 1970s (Cousens et al., 1997)...
 
 
-------- Original Message --------
 
Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD
 
Date: Thu, 28 Nov 2002 10:23:43 -0000
 
From: "Asante, Emmanuel A" e.asante@ic.ac.uk
 
To: "'flounder@wt.net'" flounder@wt.net
 
Dear Terry,
 
I have been asked by Professor Collinge to respond to your request. I am a Senior Scientist in the MRC Prion Unit and the lead author on the paper. I have attached a pdf copy of the paper for your attention.
 
Thank you for your interest in the paper.
 
In respect of your first question, the simple answer is, ***yes. As you will find in the paper, we have managed to associate the alternate phenotype to type 2 PrPSc, the commonest sporadic CJD. It is too early to be able to claim any further sub-classification in respect of Heidenhain variant CJD or Vicky Rimmer's version. It will take further studies, which are on-going, to establish if there are sub-types to our initial finding which we are now reporting. The main point of the paper is that, as well as leading to the expected new variant CJD phenotype, BSE transmission to the 129-methionine genotype can lead to an alternate phenotype which is indistinguishable from type 2 PrPSc.
 
I hope reading the paper will enlighten you more on the subject. If I can be of any further assistance please to not hesitate to ask. Best wishes.
 
Emmanuel Asante
 
<>
 
____________________________________
 
Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email: e.asante@ic.ac.uk (until 9/12/02) New e-mail: e.asante@prion.ucl.ac.uk (active from now)
 
____________________________________
 
Thursday, August 12, 2010
 
Seven main threats for the future linked to prions
 
First threat
 
The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.
 
***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.
 
Second threat
 
snip...
 
 
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...
 
 
 
Sunday, January 11, 2015
 
Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission
 
 
 
Tuesday, December 23, 2014
 
*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION
 
 
 
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease
 
***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.
 
 
*** PPo3-7: Prion Transmission from Cervids to Humans is Strain-dependent
 
*** Here we report that a human prion strain that had adopted the cervid prion protein (PrP) sequence through passage in cervidized transgenic mice efficiently infected transgenic mice expressing human PrP,
 
*** indicating that the species barrier from cervid to humans is prion strain-dependent and humans can be vulnerable to novel cervid prion strains.
 
PPo2-27:
 
Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions
 
*** Our findings suggest that CWD prions have the capability to infect humans, and that this ability depends on CWD strain adaptation, implying that the risk for human health progressively increases with the spread of CWD among cervids.
 
PPo2-7:
 
Biochemical and Biophysical Characterization of Different CWD Isolates
 
*** The data presented here substantiate and expand previous reports on the existence of different CWD strains.
 
 
Envt.07:
 
Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease
 
***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further clarification of whether CWD may be transmissible to humans.
 
 
>>>CHRONIC WASTING DISEASE , THERE WAS NO ABSOLUTE BARRIER TO CONVERSION OF THE HUMAN PRION PROTEIN<<<
 
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
 
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
 
Wednesday, January 01, 2014
 
Molecular Barriers to Zoonotic Transmission of Prions
 
*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.
 
*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.
 
 
 
PRION2013 CONGRESSIONAL ABSTRACTS CWD
 
Sunday, August 25, 2013
 
HD.13: CWD infection in the spleen of humanized transgenic mice
 
***These results indicate that the CWD prion may have the potential to infect human peripheral lymphoid tissues.
 
Oral.15: Molecular barriers to zoonotic prion transmission: Comparison of the ability of sheep, cattle and deer prion disease isolates to convert normal human prion protein to its pathological isoform in a cell-free system ***However, they also show that there is no absolute barrier ro conversion of human prion protein in the case of chronic wasting disease.
 
PRION2013 CONGRESSIONAL ABSTRACTS CWD
 
Sunday, August 25, 2013
 
***Chronic Wasting Disease CWD risk factors, humans, domestic cats, blood, and mother to offspring transmission
 
 
Friday, November 09, 2012
 
*** Chronic Wasting Disease CWD in cervidae and transmission to other species
 
 
there is in fact evidence that the potential for cwd transmission to humans can NOT be ruled out.
 
I thought your readers and hunters and those that consume the venison, should have all the scientific facts, personally, I don’t care what you eat, but if it effects me and my family down the road, it should then concern everyone, and the potential of iatrogenic transmission of the TSE prion is real i.e. ‘friendly fire’, medical, surgical, dental, blood, tissue, and or products there from...like deer antler velvet and TSE prions and nutritional supplements there from, all a potential risk factor that should not be ignored or silenced. ...
 
the prion gods at the cdc state that there is ;
 
''no strong evidence''
 
but let's see exactly what the authors of this cwd to human at the cdc state ;
 
now, let’s see what the authors said about this casual link, personal communications years ago. see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ????
 
“Our conclusion stating that we found no strong evidence of CWD transmission to humans”
 
From: TSS (216-119-163-189.ipset45.wt.net)
 
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
 
Date: September 30, 2002 at 7:06 am PST
 
From: "Belay, Ermias"
 
To:
 
Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
 
Sent: Monday, September 30, 2002 9:22 AM
 
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
 
Dear Sir/Madam,
 
In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.
 
That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
 
Ermias Belay, M.D. Centers for Disease Control and Prevention
 
-----Original Message-----
 
From:
 
Sent: Sunday, September 29, 2002 10:15 AM
 
To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV
 
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
 
Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS
 
Thursday, April 03, 2008
 
A prion disease of cervids: Chronic wasting disease
 
2008 1: Vet Res. 2008 Apr 3;39(4):41
 
A prion disease of cervids: Chronic wasting disease
 
Sigurdson CJ.
 
snip...
 
*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
 
snip...
 
full text ;
 
 
 
 
***********CJD REPORT 1994 increased risk for consumption of veal and venison and lamb***********
 
CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994
 
Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss)
 
These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...
 
Table 9 presents the results of an analysis of these data.
 
There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).
 
Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.
 
There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).
 
The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).
 
There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).
 
The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).
 
snip...
 
It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).
 
snip...
 
In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...
 
snip...
 
In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)
 
snip...see full report ;
 
 
Thursday, October 10, 2013
 
*************CJD REPORT 1994 increased risk for consumption of veal and venison and lamb**************
 
 
CJD9/10022
 
October 1994
 
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.
 
 
Thursday, October 10, 2013
 
*************CJD REPORT 1994 increased risk for consumption of veal and venison and lamb**************
 
 
Sunday, December 28, 2014
 
CHRONIC WASTING DISEASE CWD TSE PRION DISEASE AKA MAD DEER DISIEASE USDA USAHA INC DECEMBER 28, 2014
 
 
*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.
 
 
cwd exposure, and iatrogenic CJD, what if ???
 
*** our results raise the possibility that CJD cases classified as VV1 may include cases caused by iatrogenic transmission of sCJD-MM1 prions or food-borne infection by type 1 prions from animals, e.g., chronic wasting disease prions in cervid. In fact, two CJD-VV1 patients who hunted deer or consumed venison have been reported (40, 41). The results of the present study emphasize the need for traceback studies and careful re-examination of the biochemical properties of sCJD-VV1 prions. ***
 
 
snip...see full text ;
 
 
Thursday, January 2, 2014
 
*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***
 
 
*** We hypothesize that both BSE prions and CWD prions passaged through felines will seed human recPrP more efficiently than BSE or CWD from the original hosts, evidence that the new host will dampen the species barrier between humans and BSE or CWD. The new host effect is particularly relevant as we investigate potential means of trans-species transmission of prion disease.
 
 
PLEASE REMEMBER, all iatrogenic CJD is, is sporadic CJD, until the route, source, time of the iatrogenic event is documented, and then put into the public domain, which very seldom happens do to it’s long incubation period, and the lack of trace back efforts. ...terry
 
Sunday, July 06, 2014
 
Dietary Risk Factors for Sporadic Creutzfeldt-Jakob Disease: A Confirmatory Case-Control Study
 
Conclusions—The a priori hypotheses were supported.
 
*Consumption of various meat products may be one method of transmission of the infectious agent for sCJD.
 
 
Tuesday, November 04, 2014
 
*** Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011
 
 
Thursday, March 20, 2014
 
CHRONIC WASTING DISEASE CWD TSE PRION OF CERVID AND THE POTENTIAL FOR HUMAN TRANSMISSION THEREFROM 2014
 
 
Tuesday, July 01, 2014
 
*** CHRONIC WASTING DISEASE CWD TSE PRION DISEASE, GAME FARMS, AND POTENTIAL RISK FACTORS THERE FROM ***
 
 
Thursday, July 03, 2014
 
*** How Chronic Wasting Disease is affecting deer population and what’s the risk to humans and pets? ***
 
 
Friday, January 16, 2015
 
*** Indiana SENATE BILL No. 442 Miller Pete Hunting wildlife Removes exotic mammals from the animals that may be propagated or offered for hunting at a shooting preserve Makes it a Class C misdemeanor
 
 
 
UTAH CWD MAP
 
 
 
 
 
Scrapie Sheep and Goats is a risk factor for sporadic CJD
 
 
Tuesday, December 16, 2014
 
Evidence for zoonotic potential of ovine scrapie prions
 
Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 Article tools Citation Reprints Rights & permissions Article metrics
 
Abstract
 
Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human ​prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.
 
Subject terms: Biological sciences• Medical research At a glance
 
 
was this a case of Bioterrorism ???
 
Because he was so well traveled as a Special Forces soldier, his family said he may have eaten contaminated beef in England, where more than 125 persons have contracted the disease after eating beef from cows that were fed products rendered from scrapie-infected sheep. Scrapie is a form of a brain disorder found in sheep.
 
The soldier also told his family that in 2001 he ate a sheep's brain while stationed in Oman. However, while the disease is linked to cattle that have eaten sheep-byproducts, there has been no evidence of direct transfer from sheep to humans, according to the CJD Foundation.
 
Friday, August 22, 2008
 
Creutzfeldt Jakob Disease and Veterans and how they are treated at death UPDATED AUGUST 28, 2008
 
 
why do we not want to do TSE transmission studies on chimpanzees $
 
5. A positive result from a chimpanzee challenged severly would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.
 
snip...
 
R. BRADLEY
 
 
1: J Infect Dis 1980 Aug;142(2):205-8
 
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.
 
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
 
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.
 
snip...
 
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.
 
PMID: 6997404
 
 
Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"
 
Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.
 
snip...
 
76/10.12/4.6
 
 
Nature. 1972 Mar 10;236(5341):73-4.
 
Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).
 
Gibbs CJ Jr, Gajdusek DC.
 
Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
 
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)
 
C. J. GIBBS jun. & D. C. GAJDUSEK
 
National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland
 
SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).
 
 
 
 
 
2001
 
Suspect symptoms
 
What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?
 
28 Mar 01
 
Like lambs to the slaughter
 
31 March 2001
 
by Debora MacKenzie Magazine issue 2284.
 
FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.
 
Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.
 
Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.
 
"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.
 
Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.
 
Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.
 
As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.
 
"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.
 
But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.
 
People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.
 
But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."
 
There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.
 
Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.
 
 
*** Sunday, January 11, 2015 ***
 
*** Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission ***
 
 
 
Tuesday, December 23, 2014
 
*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION ***
 
 
Sunday, December 28, 2014
 
*** Reverse Freedom of Information Act request rFOIA FSIS USDA APHIS TSE PRION aka BSE MAD COW TYPE DISEASE December 2014 ***
 
 
Sunday, December 28, 2014 CHRONIC WASTING DISEASE CWD TSE PRION DISEASE AKA MAD DEER DISIEASE USDA USAHA INC DECEMBER 28, 2014
 
 
*** Alzheimer’s disease and the potential for the Iatrogenic spread, friendly fire, pass it forward mode of transmission, medical, surgical, dental, blood, tissue ***
 
Self-Propagative Replication of Ab Oligomers Suggests Potential Transmissibility in Alzheimer Disease
 
Received July 24, 2014; Accepted September 16, 2014; Published November 3, 2014
 
Singeltary comment ;
 
 
Parkinson's Disease and Alpha Synuclein: Is Parkinson's Disease a Prion-Like Disorder?
 
Recent evidence now suggests the possibility that α-synuclein is a prion-like protein and that PD is a prion-like disease.
 
 
Researchers' Discovery May Revolutionize Treatment of ALS Sep 20, 2011
 
Dr. Neil CashmanPrioNet Canada researchers in Vancouver confirm prion-like properties in Amyotrophic Lateral Sclerosis (ALS)
 
September 20, 2011 - Vancouver, BC: A team of researchers from the University of British Columbia and the Vancouver Coastal Health Research Institute have found a key link between prions and the neurodegenerative disease ALS (Amyotrophic Lateral Sclerosis), also known as Lou Gehrig's disease.
 
 
Wednesday, January 5, 2011
 
ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011
 
Prions
 
David W. Colby1,* and Stanley B. Prusiner1,2
 
Patients suffering from Parkinson's disease who received fetal grafts of substantia nigral cells later showed aberrantly folded a-synuclein in Lewy bodies within the transplanted grafts, arguing that a-synuclein acted like a prion (Kordower et al. 2008; Li et al. 2008; Olanow and Prusiner 2009). Taken together, these findings argue that prion-like, self-propagating states feature in many different, if not all, neurodegenerative diseases.
 
 
Transmission of multiple system atrophy prions to transgenic mice
 
Joel C. Wattsa,b, Kurt Gilesa,b, Abby Oehlerc, Lefkos Middletond, David T. Dextere, Steve M. Gentlemane, Stephen J. DeArmonda,c, and Stanley B. Prusinera,b,1 Author Affiliations
 
Prions are proteins that adopt alternative conformations, which become self-propagating. Increasing evidence argues that prions feature in the synucleinopathies that include Parkinson’s disease, Lewy body dementia, and multiple system atrophy (MSA).
 
Our results provide compelling evidence that α-synuclein aggregates formed in the brains of MSA patients are transmissible and, as such, are prions.
 
The MSA prion represents a unique human pathogen that is lethal upon transmission to Tg mice and as such, is reminiscent of the prion causing kuru, which was transmitted to chimpanzees nearly 5 decades ago.
 
 
 
Tuesday, September 16, 2014
 
*** mad cow scaremongers consumerfreedom.com December 20, 2003 article and a 2014 review
 
 
 
Among Singeltary's worries now, he said, are widespread violations of an August 1997 ban on feeding animal products to U.S. cattle. The FDA reported in January that hundreds of feed manufacturers were not complying with regulations designed to keep BSE out of this country.
 
(That same month, a Purina Mills feedlot near San Antonio told the FDA that a "very low level" of cow parts had been found in cattle feed. The company voluntarily removed 1,222 animals who had been fed the prohibited materials.)
 
He obtained copies of FDA letters to various feed mills that had been found in violation of the regulations and immediately sent them by e-mail to hundreds of people around the world.
 
Singeltary might not be so zealous in getting the word out if he weren't convinced that someone is covering up the truth.
 
"They used to say BSE would never transmit to humans," he said, "and it has. They lied about the feed ban being in place.
 
"I've lost faith in the whole process. I've discovered too many things."
 
 
Tuesday, March 16, 2010
 
COMMONWEALTH OF AUSTRALIA Hansard Import restrictions on beef FRIDAY, 5 FEBRUARY 2010 AUSTRALIA
 
COMMONWEALTH OF AUSTRALIA
 
Proof Committee Hansard
 
RRA&T 2 Senate Friday, 5 February 2010
 
RURAL AND REGIONAL AFFAIRS AND TRANSPORT
 
[9.03 am]
 
BELLINGER, Mr Brad, Chairman, Australian Beef Association CARTER, Mr John Edward, Director, Australian Beef Association CHAIR—Welcome. Would you like to make an opening statement? Mr Bellinger—Thank you. The ABA stands by its submission, which we made on 14 December last year, that the decision made by the government to allow the importation of beef from BSE affected countries is politically based, not science based. During this hearing we will bring forward compelling new evidence to back up this statement. When I returned to my property after the December hearing I received a note from an American citizen. I will read a small excerpt from the mail he sent me in order to reinforce the dangers of allowing the importation of beef from BSE affected countries. I have done a number of press releases on this topic, and this fellow has obviously picked my details up from the internet. His name is Terry Singeltary and he is from Bacliff, Texas. He states, and rightfully so: You should be worried. Please let me explain. I’ve kept up with the mad cow saga for 12 years today, on December 14th 1997, some four months post voluntary and partial mad cow feed ban in the USA, I lost my mother to the Heinemann variant Creutzfeldt-Jakob disease (CJD). I know this is just another phenotype of the infamous sporadic CJDs. Here in the USA, when USA sheep scrapie was transmitted to USA bovine, the agent was not UK BSE—it was a different strain. So why then would human TSE from USA cattle look like UK CJD from UK BSE? It would not. So this accentuates that the science is inconclusive still on this devastating disease. He goes on to state:
 
snip...see full text 110 pages ;
 
 
for those interested, please see much more here ;
 
 
 
 
This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:
 
 
Owens, Julie From: Terry S. Singeltary Sr. [flounder9@verizon.net]
 
Sent: Monday, July 24, 2006 1:09 PM To: FSIS RegulationsComments
 
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE) Page 1 of 98 8/3/2006
 
Greetings FSIS, I would kindly like to comment on the following ;
 
 
Nature | Editorial
 
Needless conflict Nature 485, 279–280 (17 May 2012) doi:10.1038/485279b Published online 16 May 2012
 
 
 
Tuesday, December 2, 2014
 
UK EXPORTS OF MBM TO WORLD Bovine Spongiform Encephalopathy BSE TSE Prion aka Mad Cow Disease
 
USA, NORTH AMERICA, MBM (or any potential TSE prion disease) EXPORTS TO THE WORLD (?) [protected by the BSE MRR policy] $$$
 
 
 
*** Qualitative Analysis of BSE Risk Factors in the United States
 
February 13, 2000 at 3:37 pm PST (BSE red book)
 
 
 
Tuesday, July 14, 2009 U.S.
 
*** Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book
 
Date: February 14, 2000 at 8:56 am PST
 
WHERE did we go wrong $$$
 
 
 
Friday, November 22, 2013
 
Wasting disease is threat to the entire UK deer population CWD TSE PRION disease in cervids
 
***SINGELTARY SUBMISSION
 
The Scottish Parliament’s Rural Affairs, Climate Change and Environment Committee has been looking into deer management, as you can see from the following press release,
 
***and your email has been forwarded to the committee for information:
 
 
 
 
 
 
 
Sunday, July 21, 2013
 
Welsh Government and Food Standards Agency Wales Joint Public Consultation on the Proposed Transmissible Spongiform Encephalopathies (Wales) Regulations 2013
 
*** Singeltary Submission WG18417
 
 
 
*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.
 
 
 
 
 
Friday, December 5, 2014
 
***SPECIAL ALERT***
 
The OIE recommends strengthening animal disease surveillance worldwide
 
OIE BSE TSE PRION AKA MAD COW DISEASE ?
 
 
‘’the silence was deafening’’ ...tss
 
 
 
 
Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis *video*
 
 
 
 
 
Saturday, December 13, 2014
 
*** Terry S. Singeltary Sr. Publications TSE prion disease Peer Review ***
 
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
 
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
 
snip...
 
 
 
layperson
 
Thank you,
 
Sincerely,
 
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net
 
 
 
 
 

posted by Terry S. Singeltary Sr. at 10:36 PM