Thursday, December 8, 2011

S. Korea confirms second case of iatrogenic Creutzfeldt-Jakob disease 48-year-old man

2011/12/08 11:08 KST



S. Korea confirms second case of Creutzfeldt-Jakob disease


SEOUL, Dec. 8 (Yonhap) -- South Korea's health authorities on Thursday confirmed the country's second case of Creutzfeldt-Jakob disease (CJD), a degenerative neurological disorder.



A 48-year-old man was diagnosed with iatrogenic CJD (iCJD) on Wednesday, according to the Center for Disease Control. The person's identity was withheld for privacy reasons.



The report of the country's second-ever iCJD case comes after a 54-year-old woman was found last month to have died from the same disease that is often transmitted by the use of defective prion proteins found in surgical tissue graft products.



The woman had received brain surgery using Lyodura, a tissue graft product, some 23 years ago. The KCDC said the man in the latest case had also received Lyodura during brain surgery in 1988.



This form of CJD has an incubation period of more than 20 years but once symptoms occur, death usually takes place within a year.



CJD is the most common of so-called human prion diseases with one person in every 1 million diagnosed each year worldwide. It is an invariably fatal illness with death occurring after the onset of dementia, hallucinations, coordination dysfunction and seizures.



The animal form of the disease is called bovine spongiform encephalopathy (BSE) which is commonly called mad cow disease. BSE also leaves holes in the brain that resemble a sponge.



bdk@yna.co.kr


(END)


http://english.yonhapnews.co.kr/business/2011/12/08/51/0502000000AEN20111208002700320F.HTML





Envt.15:



Prediction for Potential Risk Factors Through the Association Study Between Epidemiological Data and SNPs of Prion Protein Gene in the Korean Population and Suspected CJD Patients





Suyeon Kim,† Sol Moe Lee, Jae Wook Hyeon, Bo-Yeong Choi, Chi-Kyeong Kim, Jun Sun Park and Young Ran Ju





National Institute of Health, Korea CDC; Cheongwongun, Chungcheongbukdo, Korea;†Presenting author; Email: tenksy@nih.go.kr





Cases of the suspected CJD patients and reports of probable CJD have been increased due to a social uneasiness for import permission of beef from western countries reported BSE outbreak since 2008 in Korea CDC. It has been hard to definite diagnosis them due to Korean funeral culture though reports of probable sporadic CJD and genetic CJD have been increased in Korea. First of all, we need to clear the characteristics of PRNP gene in Korean population and to analyze the association between the endemic environmental factors and SNPs of the gene to predict the underlying cause.





We sequenced up to 5kb of the genomic region including the promoter region, exon I and exon II to analyze the correlation between SNPs of 185 suspected patients and diagnostic factors, and between SNPs of PRNP gene of 296 normal population and 60 epidemiologic factors like medical and familial history and diet. General statistical analyses were carried out by using SPSS statistic 18 (SPSS Inc., NY). Their significance levels were determined by the chi-square test (Fisher’s exact test).





We identified 19 SNPs in normal group and 15 SNPs gene in suspected patients’ group in their promoter and exon II regions. Our statistic analyses demonstrated that between rs1799990 (+385A>G; 129MV), rs28933385 (+598A>G; 200EK) and patient factors in suspected patients’ group showed significantly. In normal population group, between rs2756271 (-14605A>G), rs73612131 (-13537A>T), -14409 (C>T), rs1800014 (+655A>G; 219EK), +695(T>G; 232MR) and +591 (C>T; 197NN) SNPs and several demographic and dietary factors like an intake frequency of beef or ham were significantly associated.





In this study, we could predict the potential risk factors through the association study between SNPs of PRNP gene and several epidemiological factors. Especially, significance level of some dietary habits might show higher than other factors. However, we cannot entirely decide them risk factors of genetic markers in prion disease without identification of environmental or other causes of definite CJD patients though we found de novo SNP and significant result of PRNP. We expect to elucidate clearly their association through the combination of our results with other clinical information including additional clues.



PRION 2011



http://www.prion2011.ca/files/PRION_2011_Timetable-ENG_(May_5-11).pdf





landesbioscience.com





Wednesday, November 30, 2011


First iCJD Death Confirmed in Korea


http://creutzfeldt-jakob-disease.blogspot.com/2011/11/first-icjd-death-confirmed-in-korea.html






tss

Friday, September 2, 2011

Supreme Court clears ‘PD Notebook’ of distortion over USA MAD COW CONTROVERSY

Supreme Court clears ‘PD Notebook’ of distortion over USA MAD COW CONTROVERSY



Supreme Court clears ‘PD Notebook’ of distortion

2011-09-02 20:52

The Supreme Court on Friday cleared the directors of nationwide broadcaster MBC’s investigative program, “PD Notebook,” of charges that they had distorted some content regarding mad cow disease in the U.S.

The court upheld the Seoul High Court’s decision ruling them not guilty of defaming former Minister of Food, Agriculture, Forestry and Fisheries, Chung Woon-chun, through the program.

The producers on April 29, 2008 suggested in its program that people who consume U.S. beef could contract the human form of “mad cow disease” and showed a video clip of “downer” cows at U.S. farms. They also said that the Korean government was either ignorant of this or had intentionally turned a blind eye to it to proceed with the import resumption.

The news inspired nearly 2 million people to rally against the Lee Myung-bak administration’s decision to resume U.S. beef imports, and caused Lee’s approval rating to plummet.

A separate full panel of the Supreme Court on the same day remanded the Seoul High Court’s order that the program makers air apologetic and correctional content.

Through investigation, which involved the arrest of five directors, a raid into the office and seizure of the original films, the prosecutors claimed that several parts of the interview scenes were inaccurately translated, which could have misled the audience. MBC swiftly offered an apology.

A local court ordered the directors to air that “downer cows” do not necessary have bovine spongiform encephalopathy.

It also ruled that the contents alleging that Koreans are more susceptible to Creutzfeldt-Jakob Disease (human mad cow disease) and that the government has authorized the imports of five especially risky materials in regards to the CJD are false. A high court also ruled that MBC must air the government messages.

The court’s Friday rulings in favor of the PD Notebook could be referred as the judiciary’s guaranteeing the rights to express, observers said.

The prosecution’s rows of “excessive investigation” into the program ignited fierce disputes over the journalistic freedom and whether the government could interfere even if it contains errors.

In 2010, a local court rejected a collective lawsuit by nearly 2,500 people against the program for destabilizing the country through the “distorted reports.”

The Seoul Southern District Court said that the information may have been partially inaccurate, but its producers could not be responsible for the protests driven by the program.

By Bae Ji-sook (baejisook@heraldm.com)

http://www.koreaherald.com/national/Detail.jsp?newsMLId=20110902000758




seems the only distortion (lies), comes from the USDA et al. ...tss



Saturday, June 25, 2011


Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque

Emmanuel Comoy,1,† Valérie Durand,1 Evelyne Correia,1 Sophie Freire,1 Jürgen Richt,2 Justin Greenlee,3 Juan-Maria Torres,4 Paul Brown,1 Bob Hills5 and Jean-Philippe Deslys1

1Atomic Energy Commission; Fontenay-aux-Roses, France; 2Kansas State University; Manhattan, KS USA; 3USDA; Ames, IA USA; 4INIA; Madrid, Spain; 5Health Canada; Ottawa, ON Canada†Presenting author; Email: emmanuel.comoy@cea.fr

The epidemiology of Transmissible mink encephalopathy (TME) indicates an alimentary origin. Several inter-species transmission experiments have not succeeded in establishing with certainty any natural reservoir of this prion strain, although both ovine and bovine sources have been suspected. Cattle exposed to TME develop a spongiform encephalopathy that is distinct from classical Bovine Spongiform Encephalopathy (c-BSE).

Inoculation of c-BSE to cynomolgus macaque provided early evidence of a possible risk to humans, and remains an important model to define the risk of both primary (oral transmission from cattle to primate) and secondary (intravenous intra-species transmission) exposures. We have also evaluated the transmissibility of other cattle prion strains to macaques, including L- and H- atypical forms of BSE, namely BSE-L and BSE-H, and cattle-adapted TME.

BSE-L induced a neurological disease distinct from c-BSE. Peripheral exposures demonstrate the transmissibility of BSE-L by oral, intravenous, and intra-cerebral routes, with incubation periods similar to c-BSE. Cattle-adapted TME also induced a rapid disease in cynomolgus macaque. The clinical features, lesion profile, and biochemical signature of the induced disease was similar to the features observed in animals exposed to BSE-L, suggesting a link between the two prion strains. Secondary transmissions to a common host (transgenic mouse overexpressing bovine PrP) of cattle-TME and BSE-L before or after passage in primates induced diseases with similar incubation periods: like the c-BSE strain, these cattle strains maintained their distinctive features regardless of the donor species and passages.

If the link between TME and BSE-L is confirmed, our results would suggest that BSE-L in North America may have existed for decades, and highlight a possible preferential transmission of animal prion strains to primates after passage in cattle.


=====================end...tss====================



Risk.16: Clinical Disease in Cattle Experimentally Inoculated with All Types of BSE


Catherine Graham,1,† Michel Levy,2 Ed Pajor,2 Garth McGregor,1 Rheana Flitton1 and Stefanie Czub1

1Canadian Food Inspection Agency; Lethbridge, AB Canada; 2Faculty of Veterinary Medicine; University of Calgary; Calgary, AB Canada†Presenting author; Email: catherine.graham@inspection.gc.ca

Background. Classical, or C-type, bovine spongiform encephalopathy (BSE) has been extensively described in the literature. Recently, two novel forms of BSE, termed atypical BSE, have been reported in a number of countries. These new forms show differences in the biochemical characteristics of the prion protein and, where reported, tend to occur in aged animals but descriptions of clinical presentation are incomplete or absent.

Materials and Methods. Female Hereford/Angus cross calves were intracranially challenged at approximately five months of age with 1 ml of a 10% brain homogenate originating from Canadian field cases of BSE which had been previously classified as C-, L-, or H- type.

The animals were monitored during incubation period, and clinical disease is described using a standardized examination protocol. Incubation period, description and progression of clinical signs was recorded and videotaped for objective evaluation.

Results. All L- and H- type atypical BSE challenged animals began to display signs of clinical disease at approximately 11 months post inoculation, and disease progression was slow but constant until animals were euthanized. Clinical signs in all atypical BSE inoculated animals included hesitation at doors and gates, spontaneous muscle fasciculations and sensitivity to touch. Teeth grinding and excessive salivation are occasionally noted. Animals with L-type BSE are very anxious and show high levels of sensitivity to hand movement. One H-type animal shows periods of somnolence. Both H-type inoculated animals go down during handling and have difficulty rising and show sensitivity to movement around their head and neck area, but to a lesser degree than the L-type BSE inoculated animals. Interestingly, no locomotor abnormalities have been observed in either group.

C-type challenged animals remain normal at approximately 18 months post inoculation. Clinical disease in C-type inoculated animals from a previous transmission study was typically slow and intermittently displayed during the initial stages and after a period of two to four months was more consistent and progressive. Clinical signs in C-type BSE were as previously reported in the literature.

Discussion. The spectrum of clinical signs for all three types of BSE examined is similar. Incubation period is shorter for H- and L-type BSE as compared with C-type. Once clinical signs begin, progression is slow but relentless in atypical BSE, and more insidious in classical BSE. A summary of clinical signs presented in the three different types of BSE will be presented, and video of clinical disease will be displayed.


link url not available, please see PRION 2011 ;


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





=========================end...tss================


PLEASE NOTE *


Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.

snip...

The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...


http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf





snip...see full text ;


Saturday, June 25, 2011


Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque


"BSE-L in North America may have existed for decades"


http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/transmissibility-of-bse-l-and-cattle.html




Sunday, June 26, 2011

Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque

http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/risk-analysis-of-low-dose-prion.html




Thursday, June 23, 2011

Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits

http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/experimental-h-type-bovine-spongiform.html



Wednesday, June 15, 2011

Galveston, Texas - Isle port moves through thousands of heifers headed to Russia, none from Texas, Alabama, or Washington, due to BSE risk factor

http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/galveston-texas-isle-port-moves-through.html




Sunday, September 6, 2009


MAD COW USA 1997 SECRET VIDEO


http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html




Wednesday, February 10, 2010

The Honorable Ms. Kim Min-sun Anti-US Beef Actress Prevails in Court

http://usdavskorea.blogspot.com/2010/02/honorable-ms-kim-min-sun-anti-us-beef.html



Tuesday, June 17, 2008

Portsmouth woman did not die of mad cow-related condition, USDA says

UPDATE Updated Jun.17, 2008 08:34 KST

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/portsmouth-woman-did-not-die-of-mad-cow.html




Aretha N. Vinson

Portsmouth woman did not die of mad cow-related condition, ___USDA___ says

By Nancy Young The Virginian-Pilot © May 7, 2008

http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-atypical-bse.html



Korea data on PD Notebook

http://usdavskorea.blogspot.com/2008/06/south-korea-bans-rallies-against-us.html




Saturday, August 22, 2009

FREE Kim Min-sun, she is correct about mad cow fears from USDA BEEF

http://usdavskorea.blogspot.com/2009/08/free-kim-min-sun-she-is-correct-about.html




Friday, May 9, 2008

USDA VS KOREA typical or atypical BSe

http://usdavskorea.blogspot.com/2008/05/usda-vs-korea-typical-or-atypical-bse.html




Tuesday, April 14, 2009

Tons of USA Beef Suspected of Mad Cow Disease Sold KOREA

http://usdavskorea.blogspot.com/2009/04/tons-of-usa-beef-suspected-of-mad-cow.html




June 11, 2008, 10:14PM

U.S. slams door on revising S. Korea beef

import pact Pressed at home, Seoul had wanted 30-month limit on age of the cattle


http://www.chron.com/disp/story.mpl/nation/5832282.html




http://usdavskorea.blogspot.com/2008/06/us-slams-door-on-revising-s-korea-beef.html




Friday, June 20, 2008

USDA TO KOREA AND THE WORLD, EAT THAT AND LIKE IT

http://usdavskorea.blogspot.com/2008/06/usda-to-korea-and-world-eat-that-and.html




Why Americans, As Well as Koreans, Should Be Worried About Mad Cow Tainted USA Beef

By Terry S. Singeltary Sr. May 15, 2008

Straight to the Source

Web Note: This is an important commentary by Terry S. Singeltary Sr., on a recent Business Week story on the controversy in South Korea over their government's lifting on the ban on conventional (non-organic) beef, despite the fact that the USDA is still allowing slaughterhouse waste and blood and manure to be fed to cows, and refusing to test all cows at slaughter. See the Mad Cow section of the OCA website for in-depth information. Terry is a regular blogger on the OCA website on Mad Cow issues.

Ronnie Cummins

One Korean official says the probability of a human being catching a mad cow disease by eating U.S. beef is like the one of a golf player scoring a hole-in-one and then being killed by lightning.

this is typical BSe. you here industry groups comment 'your more likely to get hit by a car than die from CJD'. well, maybe so, but my mother and many more did not die from getting hit by a car, they died from CJD, my mothers being the hvCJD (confirmed), and my neighbors mother died from CJD (confirmed). the UKBSEnvCJD _only_ theory is incorrect. there are more strains of mad cow than the UK BSE in beef to nvCJD in humans in the UK. The deception by the USDA, FDA, and the Bush administration about mad cow disease, CJD, and all Transmissible Spongiform Encephalopathy over the past 8 years have been outrageous, to a point of being criminal. I am vested in nothing, but the truth.

snip...see full text ;

http://www.grassrootsnetroots.org/articles/article_12387.cfm




Tuesday, May 13, 2008

Concerned Americans against Mad Cow Disease STATEMENT OF SOLIDARITY with Koreans May 13, 2008

http://usdavskorea.blogspot.com/2008/05/concerned-americans-against-mad-cow.html




Tuesday, May 13, 2008

Concerned Americans against Mad Cow Disease STATEMENT OF SOLIDARITY with Koreans May 13, 2008

snip...

Re..Korea vs USDA beef (the truth)

Greetings KIWA et al and all Koreans,

A kind greetings from Bacliff, Texas.

I submit this data with great concern, sincerity, and in peace.

President Lee Myung-bak states ;

Greetings KIWA et al and all Koreans,

A kind greetings from Bacliff, Texas.

I submit this data with great concern, sincerity, and in peace.

President Lee Myung-bak states ;

"halt imports if another case of bovine spongiform encephalopathy turned up in the United States." now that is funny. a mad cow with BSE would have to drop from the sky onto the white house lawn and run around jerking, trembling, stumbling and staggering uncontrollably for months before anyone would every confirm a mad cow case with BSE, and then it would take an act of congress to confirm it, and President Lee Myung-bak knows this. it's all about commodities and futures $$$


Monday, May 5, 2008

STATEMENT OF DR. RICHARD RAYMOND USDA UNDERSECRETARY FOR FOOD SAFETY Regarding the Safety of the U.S. Food Supply please see full text with some additional information the good Dr. Raymond seems to have forgotten about ;

http://usdameatexport.blogspot.com/2008/05/statement-of-dr-richard-raymond-usda.html




snip... full text ;

http://usdavskorea.blogspot.com/2008_12_01_archive.html




Kim Min-sun is correct about mad cow fears from USDA BEEF

Kim Min-sun, who allegedly "misled the public to boycott U.S. beef consumption", did NOT mislead anyone. the fact of the matter is, the Korean Government, and it's people, are being mislead by the USA government i.e. USDA. the proof is here, you only need to watch this video and you will see whom has mislead whom... SEE THE DAMNING VIDEO NOW AT THE BOTTOM OF THE BLOG BELOW ;

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html




SOME FACTS AND UPDATED SCIENCE ON ALL STRAINS OF BSE TSE...TSS

Atypical BSE in Cattle

BSE has been linked to the human disease variant Creutzfeldt Jakob Disease (vCJD). The known exposure pathways for humans contracting vCJD are through the consumption of beef and beef products contaminated by the BSE agent and through blood transfusions. However, recent scientific evidence suggests that the BSE agent may play a role in the development of other forms of human prion diseases as well. These studies suggest that classical type of BSE may cause type 2 sporadic CJD and that H-type atypical BSE is connected with a familial form of CJD.

To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.

snip...see full text ;

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2




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




http://prionpathy.blogspot.com/2010/08/seven-main-threats-for-future-linked-to.html




http://prionpathy.blogspot.com/





Rural and Regional Affairs and Transport References Committee

The possible impacts and consequences for public health, trade and agriculture of the Government's decision to relax import restrictions on beef Final report June 2010

2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49

2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo-has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50

http://www.aph.gov.au/senate/committee/rrat_ctte/mad_cows/report/report.pdf




The conclusions state that, at present, the only TSE agent demonstrated to be zoonotic is the classical BSE agent. Active screening has allowed the identification of 3 new forms of animal TSEs (H-type atypical BSE, L-type atypical BSE, and atypical scrapie), but the information obtained has major limitations due to the unknown sensitivity of the current monitoring system for these TSEs. There is no epidemiological evidence to suggest that classical scrapie is zoonotic. The epidemiological data are too limited to conclude whether the atypical scrapie agent has a zoonotic potential. Transmission experiments to human PrP transgenic mice or primates suggest that some TSE agents other than the classical BSE agent in cattle (namely L-type atypical BSE, classical BSE in sheep, TME, CWD agents) might have zoonotic potential and indicate that that of the L-type atypical BSE agent appears similar or even higher than that of the classical BSE agent. A single study reported efficient transmission of a natural sheep classical scrapie isolate to primates.

Commentary ---------- Following to a request from the European Commission, the Panel on Biological Hazards (BIOHAZ) and the European Centre for Disease Prevention and Control (ECDC) were asked to deliver a scientific opinion on any possible epidemiological or molecular association between transmissible spongiform encephalopathies (TSEs) in animals and humans. The opinion reviews and discusses the existing scientific evidence that links animal and human TSEs currently known.

The opinion first considers the definition of zoonoses and the principles for the identification of zoonotic diseases, which can be based on evidence gathered from both epidemiological and laboratory studies. The opinion describes the challenges involved in identifying TSEs as zoonoses, due to the specific characteristics of TSE infections/diseases, such as the nature of TSE agents, the occurrence of animal and human TSEs, and the type of monitoring applied, the long incubation period of TSEs etc. The example of the process that led to establishing a link between bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) is reviewed. The epidemiological and laboratory criteria that can be used to investigate such a link are described in detail, since those criteria might be useful for the identification of links between other animal and human TSEs.

The opinion discusses the strain diversity of the TSE agents described in sheep, goats, cattle, cervids, and humans, based on the current knowledge, which highlights that multiple TSE agents exist in each species. The factors influencing the capacity of TSE agents to cross the species transmission barrier are then considered in detail, including the variability in host and donor PrP gene and protein, the TSE strain type involved and its interaction with the host PrP, and the route of infection.

The opinion critically assesses the tools and methodologies currently available to study and evaluate the possible association between animal and human TSEs. The use of epidemiology is discussed for TSEs in both animals and humans, and the possibility to compare the 2 sources of information is presented as a possible method to study the possible links.

Both in vivo and in vitro laboratory methods are considered and discussed, including neuropathology, transmission experiments involving different animal models (wild type and transgenic mice, primates and other species), biochemical methods, cell-free conversion assays, protein misfolding cyclic amplification (PMCA), and cell culture assays. Characteristics, advantages, and disadvantages of the different methods are reviewed, including the opportunity to collate data from different types of experiments for the study of potential associations between animal and human TSEs.

The opinion then reviews the scientific evidence currently available for the different animal and human TSEs, including classical BSE, atypical BSE (H-type and L-type), classical scrapie, atypical scrapie, chronic wasting disease (CWD), transmissible mink encephalopathy (TME), and human TSEs. In particular, the following aspects are systematically discussed for each TSE agent: epidemiology, pathogenesis, and in vivo and in vitro transmission experiments.

The opinion concludes that, at present, the only TSE agent demonstrated to be zoonotic is the classical BSE agent. With regard to human TSEs, detected cases of sporadic CJD are randomly distributed in time and geographical location. These observations have been interpreted as a supportive argument that sporadic CJD is not environmentally acquired. However, the epidemiological evidence in relation to sporadic CJD cannot be regarded as definitive, and the possibility that a small proportion of cases are zoonotic cannot be excluded.

It also concludes that a series of uncertainties in relation to the epidemiological patterns of animal and human TSEs indicate that even a rough comparison of the present epidemiological patterns of human and animal TSEs other than classical BSE is unlikely to be informative. Because of these uncertainties, it is an imperative to continue to carry out systematic surveillance of human TSE diseases, and to continue and improve the surveillance of animal TSE diseases.

The opinion highlights that the active screening has allowed the identification of 3 new forms of animal TSEs (L-type atypical BSE, H-type atypical BSE, and atypical scrapie), but that the information obtained has major limitations due to the unknown sensitivity of the current monitoring system for these TSEs.

There is no epidemiological evidence to suggest that classical scrapie is zoonotic. The epidemiological data are too limited to conclude whether the atypical scrapie agent has a zoonotic potential.

Transmission experiments to human PrP transgenic mice suggest that some TSE agents other than the classical BSE agent in cattle (namely L-type atypical BSE and classical BSE in sheep agents) might have zoonotic potential, whereas for other agents there is no evidence provided of a zoonotic potential (H-type atypical BSE and CWD), or no published studies are available (classical and atypical scrapie). In addition, transmission experiments to primates suggest that some TSE agents other than the classical BSE agent in cattle (namely L-type atypical BSE, classical BSE in sheep, TME, CWD agents) might have zoonotic potential. In particular, primates are highly permissive to L-type atypical BSE, even by the oral route.

The opinion emphasizes that laboratory transmission experiments indicate that the L-type atypical BSE agent has a significant zoonotic potential, which appears similar or even higher than that of the classical BSE agent. While transmission data for evaluating the zoonotic potential of classical scrapie in primates and human PrP transgenic mice are extremely limited or not yet available, a single study reported efficient transmission of a natural sheep classical scrapie isolate to primates.

The opinion concludes that human PrP transgenic mice and primates are currently the most relevant models for investigating the human transmission barrier, but the extent to which such models are informative for measuring the zoonotic potential of an animal TSE under field exposure conditions is unknown. It is unpredictable whether a TSE agent will transmit to a new host, and if the transmission principally occurs, what the transmission rate will be.

Based on the results obtained with in vitro conversion assays, the opinion concludes that there is probably no absolute molecular barrier to transmission of TSE agents between mammalian species. Results also suggest that these assays may be developed as a tool for quantifying the transmission barriers between species for different TSE agent strains; however, there is no means at the moment to transpose in vitro results into the likelihood of in vivo interspecies transmission.

-- Communicated by: Terry S Singeltary Sr

[ProMED-mail thanks Terry S Singeltary Sr for drawing attention to this comprehensive document which provides a current evaluation of experimental work designed to explore the zoonotic potential of the various recently recognised TSEs of domestic and other animals.

It is concluded that at present the only TSE agent demonstrated to be zoonotic is the classical BSE agent. Nor can it be entirely excluded at the present time that a small proportion of cases of sporadic CJD may be environmentally acquired. - Mod.CP]

******

http://www.promedmail.org/pls/apex/f?p=2400:1202:888892554804923::NO::F2400_P1202_CHECK_DISPLAY,F2400_P1202_PUB_MAIL_ID:X,88784




[Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed

http://whqlibdoc.who.int/publications/2003/9241545887.pdf



http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101




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.

page 114 ;

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



Saturday, July 23, 2011

CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE

http://transmissiblespongiformencephalopathy.blogspot.com/2011/07/cattle-heads-with-tonsils-beef-tongues.html




Saturday, November 6, 2010

TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU Berne, 2010 TAFS

INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation

http://madcowfeed.blogspot.com/2010/11/tafs1-position-paper-on-position-paper.html




Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR> Prion disease update 2010 (11)

PRION DISEASE UPDATE 2010 (11)

http://www.promedmail.org/pls/apex/f?p=2400:1001:5492868805159684::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,86129




Saturday, March 5, 2011

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

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




Sunday, August 21, 2011

The British disease, or a disease gone global, The TSE Prion Disease


snip...

CANADA CJD UPDATE 2011

CJD Deaths Reported by CJDSS1, 1994-20112 As of January 31, 2011

3. Final classification of 49 cases from 2009, 2010, 2011 is pending.

snip...

http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_0111-eng.pdf




USA 2011

USA

National Prion Disease Pathology Surveillance Center

Cases Examined1

(November 1, 2010)

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

1996 earlier 51 33 28 5 0 0

1997 114 68 59 9 0 0

1998 87 51 43 7 1 0

1999 121 73 65 8 0 0

2000 146 103 89 14 0 0

2001 209 119 109 10 0 0

2002 248 149 125 22 2 0

2003 274 176 137 39 0 0

2004 325 186 164 21 0 13

2005 344 194 157 36 1 0

2006 383 197 166 29 0 24

2007 377 214 187 27 0 0

2008 394 231 205 25 0 0

2009 425 258 215 43 0 0

2010 333 213 158 33 0 0

TOTAL 38315 22656 1907 328 4 3

1 Listed based on the year of death or, if not available, on year of referral;

2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;

3 Disease acquired in the United Kingdom;

4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;

5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

http://www.cjdsurveillance.com/pdf/case-table.pdf



Please notice where sporadic CJD cases in 1996 went from 28 cases, to 215 cases in 2009, the highest recorded year to date.

sporadic CJD is on a steady rise, and has been since 1996.

I also urge you to again notice these disturbing factors in lines 5 and 6 ;

5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

========end=====tss=====2011



snip...SEE FULL TEXT WITH VIDEOS ;


Sunday, August 21, 2011

The British disease, or a disease gone global, The TSE Prion Disease

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



Thursday, August 4, 2011

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



(see video here) ;


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




Sunday, August 21, 2011

The British disease, or a disease gone global, The TSE Prion Disease



(see video here)


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




Friday, June 17, 2011

Treatable neurological disorders misdiagnosed as Creutzfeldt-Jakob disease

http://creutzfeldt-jakob-disease.blogspot.com/2011/06/treatable-neurological-disorders.html




Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION



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




http://cjdquestionnaire.blogspot.com/




Wednesday, August 24, 2011

There Is No Safe Dose of Prions

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/there-is-no-safe-dose-of-prions.html




Wednesday, August 24, 2011

All Clinically-Relevant Blood Components Transmit Prion Disease following a Single Blood Transfusion: A Sheep Model of vCJD

http://transmissiblespongiformencephalopathy.blogspot.com/2011/08/all-clinically-relevant-blood.html




Wednesday, June 29, 2011

TSEAC Meeting August 1, 2011 donor deferral Saudi Arabia vCJD risk blood and blood products

http://tseac.blogspot.com/2011/06/tseac-meeting-august-1-2011-donor.html




Monday, May 23, 2011

Atypical Prion Diseases in Humans and Animals 2011

Top Curr Chem (2011)

DOI: 10.1007/128_2011_161

# Springer-Verlag Berlin Heidelberg 2011

Michael A. Tranulis, Sylvie L. Benestad, Thierry Baron, and Hans Kretzschmar

http://bse-atypical.blogspot.com/2011/05/atypical-prion-diseases-in-humans-and.html




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