Friday, December 7, 2012

S. Korea confirms first death caused by Creutzfeldt-Jakob disease ?

S. Korea confirms first death caused by Creutzfeldt-Jakob disease


Publish By David K. Barger Updated 06/12/2012 8:41 pm in Health&Lifestyle / no comments



SEOUL, Nov. 29 – The first death in South Korea caused by neurodegenerative Creutzfeldt-Jakob disease was confirmed by health authorities on Tuesday.


The Center for Disorder Control (KCDC) announced that a 54-year- old woman was diagnosed with iatrogenic Creutzfeldt-Jakob disease (iCJD) after she died in July this year.


She is believed to have been infected during her brain surgery 23 years ago when she had dead brain tissue replaced with a graft of a dura mater product named Lyodura. The victim did not show symptoms until the occurrence of paralysis in her face and toe in June last year.


The KCDC said the transplanted dura mater originated from the body of a CJD patient. The center said it will track down potential patients who received similar transplants with Lyoduras around 1987.


Some 400 cases of iCDJ have been reported in 20 countries, 200 of which were caused after dura mater transplant operations. KCDC, meanwhile, stressed that there is no correlation between this case and the Variant Creutzfeldt-Jakob disease, which is often referred to as human mad cow disease.









> S. Korea confirms first death caused by Creutzfeldt-Jakob disease



???




CONFUCIUS CONFUSED AGAIN ?




Thursday, December 8, 2011


S. Korea confirms second case of iatrogenic Creutzfeldt-Jakob disease 48-year-old man 2011/12/08 11:08 KST







Wednesday, November 30, 2011


First iCJD Death Confirmed in Korea






Development of Policy and Strategy for the Control of Creutzfeldt-Jakob Disease in Korea


Choi SI, Jeong BH, Kim YS.


Korean J Epidemiol


2005 Jun 27(1):81-89. Korean.




Total References:16 Cited Korean References:2 Times Cited:0





PURPOSE: Through the understanding of the current status of transmissible spongiform encephalopathy(TSE), this study was conducted to contribute to the development of policy and strategy for the control of TSE in Korea in order to keep Korea as a bovine spongiform encephalopathy(BSE)- and variant Creutzfeldt-Jakob disease(vCJD)-free country. BSE and vCJD cases have not been found in Korea.




During 2001-2004, the number of patients who have been diagnosed as a definite or probable CJD was 121, which are consisted of 62 male and 59 female(average age: 63 years old). The occurrence of the patients was 5-59 people per year until 2003 and has been gradually increasing due to the recent increase in the diagnostic rate rather than the increase of the incidence.




In 2004, the annual occurrence of sporadic CJD(sCJD) in Korea was 1 people per million, which is similar to the average occurrence rate of the world. Two cases of chronic wasting disease(CWD) in deer were found in Chungcheongbuk-do, one in August 2001 and one in October 2001. After that, 4 more CWD-affected deer have been reported in Kyungsangnam-do area in November 2004.




We have also examined the possibility that Korean CJD occurred as a result of dietary exposure to BSE. Fortunately, all of Korean CJD patients were not vCJD cases. However, if BSE occurs in Korea, there is a great potential for most of the Korean population to be easily infected with BSE due to their highly susceptible genotype to BSE infection as well as their traditional food habit. In 2003, the total number of people who left Korea was almost identical with the total number of people who entered Korea. However, we could not analyze the number of people who visited or stayed in the UK and Europe during 1980s~1990s, in which BSE was prevalent in Europe, because there was no statistical data available.


Affiliation:


Ilsong Institute of Life Science, Hallym University, Anyang. yskim@hallym.ac.kr Department of Microbiology, Hallym University College of Medicine, Chunchon.









J Korean Med Sci. 2010 July; 25(7): 1097–1100.


Published online 2010 June 16. doi: 10.3346/jkms.2010.25.7.1097


PMCID: PMC2890893 Familial Creutzfeldt-Jakob Disease with V180I Mutation


fCJD due to the V180I, as our patient's, is extremely rare, with all reported cases have come from Japan with one from France (11, 12), and two in the United states in a non-Asian (Geschwind et al. unpublished results) (13). To our knowledge this is the first reported case in South Korea.







Korean Med Sci. 1998 Jun;13(3):234-240. Published online 2009 June 22. Copyright © 1998 The Korean Academy of Medical Sciences



Molecular analysis of prion protein gene (PRNP) in Korean patients with Creutzfeldt-Jakob disease




B H Jeong, W K Ju, K Huh, E A Lee, I S Choi, J H Im, E K Choi and Y S Kim Institute of Environment & Life Science and Department of Microbiology, College of Medicine, Hallym University, Chunchon, Korea. This




Abstract



Creutzfeldt-Jakob disease (CJD), a relatively uncommon human dementia, is caused by an unconventional slow infectious agent. Several cases of CJD, clinically or histopathologically diagnosed, have been reported in Korea. In order to confirm the diagnosis of CJD and also differential diagnosis of sporadic and familial types of CJD in Korea, we studied two patients who had symptoms of CJD. The histopathological and immunohistochemical studies showed spongiform neurodegeneration and expression of abnormal isoform of prion protein (PrPSc) in astrocytes. Thus, these two patients were diagnosed CJD. To investigate whether these patients were sporadic or familial type of CJD, the molecular analyses of the prion protein gene (PRNP) were done by restriction fragment length polymorphism (RFLP) and DNA sequencing. In the cases of a healthy Korean and two CJD patients, no point mutation was detected in the known hot spots (178, 180, 200, 210, and 232) and they exhibited wild type PRNP sequences. We concluded that both patients have a sporadic type of CJD, but not familial type.








J Korean Med Sci. 1991 Sep;6(3):273-278. Published online 2009 May 26. Copyright © 1991 The Korean Academy of Medical Sciences



A case of Creutzfeldt-Jakob disease



Jae Kwan Cha, Myung Ho Kim, Suck Jun Oh and Eun Kyung Hong Department of Neurology, College of Medicine, Hanyang University, Seoul, Korea. Abstract


Creutzfeldt-Jakob disease (CJD) is presumably caused by a slow infectious pathogen or prion. The principal clinical features of Creutzfeldt-Jakob disease are dementia, pyramidal and extrapyramidal symptoms and signs, cerebellar dysfunction, and myoclonus. The patient rapidly deteriorates, declines to a vegetative state, becomes comatous, and is ultimately dead within several months. The authors present a case of Creutzfeldt-Jakob disease, proved by clinical findings, typical serial EEG, and pathologic features.




Keywords: Creutzfeldt-Jakob disease.










SEE OTHER iCJD cases recently ;




6 December 2012


HONG KONG CHP closely monitors a probable case of sporadic Creutzfeldt-Jakob Disease


The Centre for Health Protection (CHP) of the Department of Health today (December 6) received a report from the health authority of Macao concerning a probable case of sporadic Creutzfeldt-Jakob Disease (CJD) affecting a 51-year-old female Macao resident who had undergone a brain operation in a private hospital in Hong Kong.


According to the health authority of Macao, the woman who developed progressive dementia with limbs spasm, unstable gait, muscle twitching and visual disturbance suggestive of CJD, has been admitted to a hospital in Macao on October 30. Her clinical diagnosis was sporadic CJD and the patient is now in severe condition.


Investigation by the Macao health authority revealed that the woman had received a brain surgery in a private hospital in Hong Kong on September 19.


Upon receipt of the report, the CHP acted in accordance with the international practice and advised the hospital to contact the patients who had undergone procedures using the same set of instruments as the patient since September 19.


According to the current understanding, the transmission risk is extremely low. The contact tracing is a precautionary measure.


A CHP spokesman said, "CJD is a rare disease that affects the brain and is thought to be caused by the build-up of an abnormal, transmissible protein called 'prion' in the brain."


The spokesman said surgical instruments used on the patient should have been sterilised but may not be enough to eradicate the prions which are the causing agents of the disease. Contact tracing conducted by the hospital is under way.


The clinical feature of CJD is characterised by dementia and difficulties in walking. Early symptoms include memory loss, unsteady gait and loss of co-ordination of limbs. These dementia-like symptoms will worsen and twitching of limbs and trunk will occur. Besides, visual disturbance, abnormal behaviour and seizures can occur. Most patients die within one to two years after onset of symptoms.


Since CJD is not transmitted through casual contact, isolation of patients is not necessary. To prevent the disease from spreading, tissue or organ transplant from any CJD patients or re-use of potentially contaminated surgical instruments should be avoided.


Ends/Thursday, December 6, 2012








Scientific Committee on Emerging and Zoonotic Diseases


Epidemiology of Creutzfeldt-Jakob Disease in Hong Kong






Tuesday, July 31, 2012


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






Thursday, August 02, 2012


CJD case in Saint John prompts letter to patients Canada


CJD case in Saint John prompts letter to patients






Friday, August 24, 2012


Iatrogenic prion diseases in humans: an update






Friday, November 23, 2012


sporadic Creutzfeldt-Jakob Disease update As at 5th November 2012 UK, USA, AND CANADA






Sunday, December 2, 2012


CANADA 19 cases of mad cow disease SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’






Thursday, December 06, 2012


Hong Kong CHP closely monitors a probable case of sporadic Creutzfeldt-Jakob Disease






Sunday, June 17, 2012


MBC’s PD Notebook infamous mad cow program victorious in final lawsuits South Korea







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








Tuesday, May 13, 2008


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







Wednesday, February 10, 2010


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







Saturday, April 10, 2010


TOYOTA VS MAD COW DISEASE USA OIE BSE MRR IMPORT AND EXPORT TRADE WARS










Tuesday, November 08, 2011


Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008


Vol. 37, No. 3-4, 2011 Original Paper Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.






Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis












full text with source references ;







Friday, November 23, 2012


sporadic Creutzfeldt-Jakob Disease update As at 5th November 2012 UK, USA, AND CANADA

 








Saturday, October 6, 2012



TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 2011 Annual Report







Tuesday, November 6, 2012



Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and Sporadic CJD, November-December 2012 update








Sunday, December 2, 2012


CANADA 19 cases of mad cow disease SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’











TSS

Sunday, June 17, 2012

MBC’s PD Notebook infamous mad cow program victorious in final lawsuits South Korea

MBC’s infamous mad cow program victorious in final lawsuits

Posted on : Jun.16,2012 12:32 KST

프린트

Though containing inaccuracies, Supreme Court rules program was a timely warning on mad cow disease

By Yeo Hyeon-ho, senior staff writer The production team of MBC’s “PD Notebook” program has emerged victorious from all of the seven civil and criminal lawsuits filed against. The suits were filed due to a report the program made about mad cow disease and alleged dangers associated with American beef.

On June 15, the Supreme Court stated that it had upheld an earlier ruling in which New Frontier Party (NFP) lawmaker Shim Jae-chul was unsuccessful in his claim seeking 500 million won (around US$430,000) in damages from PD Diary on the grounds that it distorted comments he made on the safety of US beef. Delivering its verdict, the court said the program’s report “emphasized or expressed rhetorically experts’ belief that cows with BSE are not safe even after specified risk materials have been removed, and that [such cows] were not used for food in other countries, either.”

The ruling brought an end to all of the six civil and one criminal lawsuits triggered by the report. On September 2 last year, the Supreme Court upheld a ruling that had declared producer Cho Neung-hee and four other members of PD Diary‘s production team not guilty after they were indicted on charges of libeling former Food, Agriculture, Forestry and Fisheries minister Chung Woon-chun. In its ruling, the court indirectly criticized prosecutors for making unreasonable indictments, saying, “It is necessary to exercise caution when punishing media figures on charges of defaming the character of a public official for his or her policy decisions.”

A lawsuit filed by the Ministry of Food, Agriculture, Forestry and Fisheries (MIFAFF) against MBC to demand a correction and the broadcast of material making contrary arguments was sent back to Seoul High Court when the Supreme Court overturned it. On December 1 2011, the case ended in defeat for the MIFAFF. A claim for 2.4 billion won (US$20.6 million) in damages filed by the group “Lawyers for Citizens” on behalf of a coalition of members of the public, had its original ruling of defeat for the plaintiffs upheld on May 10. Three other lawsuits, including one filed by US beef importer A-Meat against PD Notebook and actor Kim Min-seon, were withdrawn in the mean time.

The PD Notebook incident was triggered when the Korean government decided to restart imports of American beef in 2008. Public anger over the risk of mad cow disease brought thousands of citizens out to protest the decision by the then-new Lee Myung-bak administration. The government and pro-government media diverted the focus of the matter to make PD Diary look to be the culprit. An absurd scene developed whereby the government tried to shift responsibility for its decision onto the media.

The Supreme Court acknowledged that although the PD Notebook report did contain some untrue material, such as the case of Aretha Vinson, whose death was found much later on not to have been due to BSE, it was a timely warning of the problems left by the government’s hasty negotiations and the dangers of BSE-infected beef. Its victory in all five lawsuits brought against it to claim a total of 4.3 billion won (US$36.9 million) in damages, not only by Shim but also by beef importers, at once confirms the validity of the report and can be regarded as proof that the government and prosecutors’ claims were unreasonable.

After hearing the ruling on June 15, Cho sent out an emotional tweet, saying, “We’ve won all our cases after four years and two months. Now it’s our turn to file lawsuits. We will hold to account [those guilty of] media manipulation, reports [in some media] that have ignored media codes of conduct, and acts that have demolished constitutional order.”

Please direct questions or comments to [english@hani.co.kr]

 http://english.hani.co.kr/arti/english_edition/e_national/538052.html




***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




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



Wednesday, January 13, 2010

High Court Rules In Favor of PD Notebook 01-13-2010 21:21

http://usdavskorea.blogspot.com/2010/01/high-court-rules-in-favor-of-pd.html



Saturday, August 22, 2009

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

Sent: Saturday, August 22, 2009 3:18 PM

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

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

WHOM is to say, whether or not, one would rather die fast from cyanide, or slow from mad cow disease. some would rather die in a fast mode, than say slowly, an agonizing death, from Creutzfeldt Jakob Disease ??? IN a free society, would one not have the right of free speech to voice that opinion ???

WHOM is to say, that some sub-types of the CJD in the USA, is not from the atypical mad cow disease in the USA ???

WHAT DID ONE OF THE TOP USA SCIENTIST ON PRIONS AND TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY SAY ABOUT USA 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





***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





snip...please see full text ;

Saturday, August 22, 2009

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

Sent: Saturday, August 22, 2009 3:18 PM

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



However, i can tell you that it was the media and the womans doctor here in the USA that first reported she had human form of mad cow disease. and that's what PD NOTEBOOK and many other media outlets published, until the USDA (?) which first announced that it was not nvCJD. ...

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

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

Preliminary test results indicate that a 22-year-old Portsmouth woman who died in April did not have an illness that has been associated with eating contaminated beef, a U.S. Department of Agriculture official said this week.

"The epidemiologic characteristics of the illness and preliminary results of the neuropathologic testing of brain tissue obtained at autopsy indicate that the patient did not die of" a variant of Creutzfeldt-Jakob Disease (vCJD), which has been associated with mad cow disease, said Richard Raymond, USDA undersecretary for food safety. He was speaking Sunday to a group of Korean and American reporters in Washington.

"The U.S. Centers for Disease Control and Prevention has just provided us with that information, and I felt it was important to share with you today," he told the group. The comment appears on a statement on the safety of the U.S. beef supply that is available on the USDA's Food Safety and Inspection Service Web site.

Aretha Vinson died April 9 at Bon Secours Maryview Medical Center in Portsmouth. She had a degenerative brain condition that can be caused by a wide variety of things, and concerns were raised that she might have had vCJD. The state health department, CDC and the University of Virginia were involved in testing to determine the cause of death and whether it indicated a public health concern.

"There's no threat to the general public," said Michelle Peregoy, a Virginia Department of Health spokeswoman. She would not say more, citing patient confidentiality issues.

Nancy Young, (757) 446-2947, nancy.young@pilotonline.com

http://hamptonroads.com/2008/05/portsmouth-woman-did-not-die-mad-cowrelated-condition-usda-says



Virginia State University student Aretha Vinson has contracted a rare brain disorder called Creutzfeldt-Jakob Disease, a variant of Mad Cow Disease. Virginia state health officials are investigating how Vinson contracted the disease. Doctors suspect Vinson contracted the disease after having gastric bypass surgery, possibly from tainted medical instruments. Vinson's family says her health worsened after having gastric bypass surgery three months prior to the diagnosis.

UPDATE: Vinson passed away at 5:30pm on Wednesday, April 9, 2008.

http://www.mahalo.com/stub/aretha-vinson



The program, which now faces charges for playing up the possibility that the woman died of vCJD, said, “The CDC last Thursday announced the cause of death of Aretha Vinson, who died of symptoms similar to vCJD.” It quoted the CDC as saying although the suspected case received international media attention, the National Prion Disease Pathology Surveillance Center determined that the cause of death was not due to vCJD, a finding, it pointed out, that was similar to an announcement by the Department of Agriculture.

http://english.chosun.com/w21data/html/news/200806/200806170006.html



22 year old sporadic CJD ???

we will never know ;

In a telephone interview with the Chosun Ilbo, CDC spokesman Dave Daigle on Monday said the centers posted the announcement after performing their own checkup once the NPDPSC finished its investigation. He added that because the CDC only provide information on diseases, they have no plans to make a separate press release on the issue including the result of the investigation. ...

http://english.chosun.com/w21data/html/news/200806/200806170006.html



Mother remembers daughter's final moments before her death

Portsmouth woman who may have contracted rare dise

Updated: Wednesday, 08 Oct 2008, 12:25 AM EDT Published : Monday, 07 Apr 2008, 11:33 PM EDT

PORTSMOUTH, VA. (WAVY.com) -- Workers took apart the hospital bed that stood in the living room. It was the very bed Aretha Vinson laid in before her final trip to the hospital last week.

Her mother, Robin Vinson said, "As they were taking the bed down I felt like I was losing my daughter again."

Robin lost her 22-year-old daughter Aretha Thursday night after a short battle with what doctors suspect to be a rare brain disease called Creutzfeldt-Jakob disease.

Doctors told Vinson "there was no treatment for this condition, neither a cure" and she says it just ripped her heart.

After finding out her daughter may have a rare disease, Vinson says her daughter deteriorated quickly. One day Aretha was talking, her last words being "thank you Jesus," and the next she was communicating only through gestures.

"I said Aretha if you could hear me, squeeze my hand, and she did it," her mother said. Aretha squeezed her mom's hand twice, but the third time she got nothing. That's when the news came that Aretha was brain dead and the Vinson family had to make the decision to remove her from life support.

"A decision that will be with me for the rest of my life," Vinson says. Vinson says her daughter's death wasn't in vain. She says she knows Aretha's death will provide answers to a number of questions.

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

Previous Coverage:

Portsmouth woman who may have contracted rare disease, has died

PORTSMOUTH, VA. (WAVY.com) -- WAVY.com has learned the Portsmouth woman who may have had a rare disease, has passed away.

Doctors believe Vinson may have contracted variant Creutzfeld-Jakob Disease, or CJD. They won't know for sure until an autopsy is performed. The health department is working with the Centers for Disease Control. Officials say it could take months to figure out if Vison had CJD.

We're told Vinson went into a coma on Friday and never regained consciousness.

Stay with WAVY.com and WAVY News 10 for any further developments.

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

Previous Coverage:

Portsmouth woman may have contracted rare brain disease Filed by Jason Marks

PORTSMOUTH, VA. (WAVY.com) -- Doctors say the chances of having Cruetzfeldt-Jakob Disease or CJD are one in a million, but believe a Portsmouth woman may have contracted the disease.

Health Department officials say this is an isolated case and they don't believe it's spreading from person to person.

"Why this disease," asked Robin Vinson. "Why my daughter?"

Those are just two of the many questions running through Robin Vinson's mind. Her daughter Aretha could possibly have one of the rarest diseases found in the world.

"To see her in the state in which she is in, it's only through the grace of God to give me the strength to stand and watch her with such limitations," added Vinson.

Aretha,22, is confined to a hospital bed, unconscious, inside Maryview Medical Center. Her family says her health rapidly got worse after having gastrobypass surgery less than three months ago. They say her motor skills began to worsen and her memory began to fade.

"This is a very rare condition," said Dr. Demetria Lindsay, Director of the Portsmouth Health Department. "It is not easily passed from person to person. You can not get it from casual contact or even close contact."

Doctors say there are only about 200 cases of CJD in the United States every year. They say it's passed through surgeries and in rare situations by eating meat. The Health Department says there is no reason to worry, but they are trying to figure out how Aretha got it.

"They don't know a whole lot about this disease, however we have educated ourselves by going through the Internet," added Vinson. "We found out there is so much involved and that there is no treatment available and there is no cure."

The last time Robin was able to speak to Aretha was Friday. Robin tells us Aretha's condition worsens everyday.

Aretha's family tells us she was a bright woman who just graduated with honors from Virginia State last year - she wanted to be a teacher. They say they're trying to enjoy every moment they have left with her.

"You know when she was born is was such a special moment," added Vinson. "It was the best a mother can ask for and now my daughter is near death and every moment I have with her is a precious moment."

 http://www.wavy.com/dpp/search/Mother_remembers_daughter_s_final_moments_before_her_death_852224



Now, the cdc et al usually hide behind patient confidentiality to hide cjd cases. but in this 22 years old, confidentiality was not the case, she was well known around the world, and the cdc et al chose to hide her final diagnosis. why, because it was another young victim in the USA with sporadic CJD ???

SEE FULL TEXT ;

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



Monday, April 5, 2010 UPDATE - CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER ----- Original Message -----

From: Katie Glisic To: Terry S. Singeltary Sr. Sent: Monday, April 05, 2010 7:39 AM Subject: Re: [cjdsurv] CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

Dear Mr. Singelteary,

Thank you for contacting the Center. We are aware of Ms. Andablo's case and are in the process of testing, however thank you very much for the enclosed information. As you are aware, there have been no confirmed cases of vCJD (Mad Cow) contracting in the United States. The Center has been testing and diagnosing cases of CJD for over 10 years and reporting our findings to the Centers for Disease Control and Prevention. Of the two cases we have documented, neither was contracting in the US. One was contracted in the UK where cases of Mad Cow are known and one was contracted in Saudi Arabia.

Please note, we also performed the testing on Ms. Vinson and her family is well informed of the diagnosis provided by the Center. The United States government as well as the USDA go to great lengths to ensure that the US beef consumed by our country is both safe and continually tested to prevent outbreaks of food born illnesses such as Mad Cow. Please rest assured that Dr. Gambetti and the rest of the Center's researchers work tirelessly to provide accurate diagnosis to families with loved ones suffering from CJD.

On Fri, Apr 2, 2010 at 12:38 PM, Terry S. Singeltary Sr. wrote:

Greetings Professor Gambetti Sir,

A kind greetings from sunny Bacliff, Texas !

Sadly, i thought i should pass this data to you. You are probably much aware of this, but it seems odd that no media has commented? also, the last suspect nvCJD case i remember here in the USA, it was the USDA that the so called 'confidentiality agreements between family', and came out officially first and over ruled the nvCJD and called in sporadic CJD, well, i don't think they ever even officially called it that, but Aretha N. Vinson family knows what she had, and PD Notebook won in the end ;

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

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

(towards the end of this blog...tss)

 http://prionunitusaupdate2008.blogspot.com/2010/04/update-cjd-texas-38-year-old-female.html



see post below for more details ;

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




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





Friday, May 9, 2008

USDA VS KOREA typical or atypical BSe

Honorable people of Korea,

a kind and warm greetings from Texas.

i cannot sit here and allow my government to lie to the korean people. i have been following the issue of Transmissible Spongiform Encephalopathy for over a decade, since the death of my mother to the Heidenhain Variant of Creutzfeldt Jakob Disease, confirmed. i have followed this on a daily basis since 12-14-97. there is so much i want to tell you, that the USDA and the FDA are not telling you.

i almost got to see your beautiful country, but in the last minute, my trip was cancelled. i was asked to come speak a year or so ago ;

Name: Terry S. Singeltary Sr. Date: Jan 26, 2007 Dear Terry S. Singeltary Sr. My name in Seoungwon Lee and I work for National Assemblywoman (MP) Sang-Jeong Sim, in South Korea. Below is a message from Mr. Kwon. Please get back to us regarding the letter. Thank you. Seoungwon Lee Legislative Assistant National Assembly Republic of Korea

Dear Terry S. Singeltary Sr.

Greetings from Korea.

I have learned about what you and your family have been through a internet. Being the father of a small family, I can only imagine the sense of loss that you and your family still must feel regarding your mother.

Through the internet, many people here in Korea have learnt about the story of your family and it has created a great deal of concern. Your experience had particular relevance for the citizens here, for the Korean government, in connection to the free trade agreement it is negotiating with the United States, is about to restart the sale of the unsafe American beef to the general public.

The Korean government has already imported the first load American beef and is currently going through the inspection period. There has been a great deal of controversy regarding the safety of these products, and a precipitous increase in public interest regarding the causes and symptoms of vCJD.

It is in this context that we are preparing a session together with National Assembly members and relevant NGOs to hear from those who have had direct experience with the human variant of BSE. We are very interested in hearing about the symptoms that your mother showed, your views on the response from the US government to the many deaths that resulted from this disease, the reaction from the general public, and any other area that you would wish to speak about. There would also be experts and academics in the area to speak at the session as well. We believe that it would greatly contribute to raising awareness about the issue, as well as help politicians and civic activists to consider the repercussions of the beef import issue.

We would like to request your help in this regard. We are planning for the session to be held in Seoul on the 23rd of this November. We would very much like to have you present in order to help prevent such tragic incidents from happening in Korea. We would, of course, pay for the trip and accommodations for the duration of your stay. If you were to participate, we could also meet with members from the agricultural committee of the National Assembly and from other related organizations to urge more interest to the issue.

Please do not hesitate to offer suggestions or ask us any questions that you might have. We look forward to a positive response and to meeting you in Seoul.

Thank you. Sincerely, Sim Sang-Jeong Head of the Democratic Labor Party Parliamentary Committee on the Korea-US FTA Member of the National Assembly Republic of Korea

http://www.fpif.org/fpiftxt/3940



"South Korea may demand revision of US beef import pact"

"The agreement, struck last month, has been widely criticized as making too"

"many concessions to the United States"

THE PEOPLE of Korea _should_ be mad about the importing of USA beef into their Country. can you believe these regulations? even IF a BSE case(s) are documented in the USA, the people of Korea still cannot suspend the importing of U.S. beef, NO matter how many more mad cows the USA finds, until a thorough epidemiological investigation is finished. please remember, it took over a year and literally an act of congress to confirm the atypical mad cow in Texas before they finally finish that epidemiological investigation, and even after all that, the Koreans still cannot ban USA beef, until the OIE recognizes an adverse change in the classification of the U.S. BSE status. Considering the USDA and the OIE collaborated to seal the deal of the BSE MRR policy (the legal trading of all strains of TSE globally, just for commodities and futures sake, human health was not even considered), I doubt the OIE would ever change the BSE status for the USA, no matter how many more mad cows are found. It's all about money folks. WE are talking years now, before the Koreans could ever suspend USA beef due to a BSE case(s) ever being documented in the USA, due to these stupid regulations. This is nothing more than FORCE FEEDING KOREA USDA MAD COW BEEF, i.e. all for a dollar, to hell with human health on a disease with an incubation period of years if not a decade or more.

Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;

***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***

Progress Report from the National Prion Disease Pathology Surveillance Center

An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD

April 3, 2008

http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45



NEXT QUESTION, _if_ BSE is not in the USA (just not documented for many different reasons), and only atypical BSE is in the USA (plus CWD, plus, many strains of Scrapie, and Now the Nor-98 documented in 5 different states, plus TME, then why would human mad cow in the USA look like the UK nvCJD from UK BSE cows ? it was shown long ago in studies at Mission Texas that experimental transmission of USA Scrapie to USA Bovine, DID NOT LOOK LIKE UK BSE. so again, in short, why would human mad cow in the USA look like human mad cow in the UK i.e. the (nvCJD). however, i believe that BSE has been in the USA untested and undocumented for years. why on earth then does the USDA refuse to allow creekstone or anyone else test their product? simple, if you don't look/test, you don't find (see my USA VS CANADA mad cow testing blog in my reference links below) ;

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.



http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm



http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf



Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html



MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html



CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/



Honorable people of Korea, young and old, rich and poor, I wish to send this data to you, I hope you dont mind, and please spread the truth. the fda ruminant-to-ruminant feed ban has failed terribly since the inception on 8-4-97. it was nothing but ink on paper. however, the usda fda et al does not tell the honorable people of Korea this. The feed ban is one of those firewalls the FDA et al like to boast about all the time. In 2007, in one weekly enforcement report, the fda recalled 10,000,000+ pounds of BANNED MAD COW FEED, 'in commerce', and i can tell you that most of it was fed out ;

10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. MBM IN COMMERCE USA 2007

Date: March 21, 2007 at 2:27 pm PST REASON Blood meal used to make cattle feed was recalled because it was cross-contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement. VOLUME OF PRODUCT IN COMMERCE 42,090 lbs. DISTRIBUTION WI

REASON Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement. VOLUME OF PRODUCT IN COMMERCE 9,997,976 lbs. DISTRIBUTION ID and NV

END OF ENFORCEMENT REPORT FOR MARCH 21, 2007

skroll down to bottom ;

http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.htm



Wednesday, May 2, 2012

ARS FLIP FLOPS ON SRM REMOVAL FOR ATYPICAL L-TYPE BASE BSE RISK HUMAN AND ANIMAL HEALTH

http://transmissiblespongiformencephalopathy.blogspot.com/2012/05/ars-flip-flops-on-srm-removal-for.html



CDC 2012

Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model

Nadine Mestre-Francés, Simon Nicot, Sylvie Rouland, Anne-Gaëlle Biacabe, Isabelle Quadrio, Armand Perret-Liaudet, Thierry Baron, and Jean-Michel Verdier

We report transmission of atypical L-type bovine spongiform encephalopathy to mouse lemurs after oral or intracerebral inoculation with infected bovine brain tissue. After neurologic symptoms appeared, transmissibility of the disease by both inoculation routes was confirmed by detection of disease-associated prion protein in samples of brain tissue.

snip...

Conclusions

We demonstrated that the agent of L-BSE can be transmitted by the oral route from cattle to mouse lemurs. As expected, orally inoculated animals survived longer than IC-inoculated animals. Orally inoculated lemurs had less severe clinical signs and symptoms, with no evidence of motor dysfunction. It was previously suggested that the agent of L-BSE might be involved in the foodborne transmission of a prion disease in mink (11,12), a species in which several outbreaks of transmissible mink encephalopathy had been identified, notably in the United States (13).

Our study clearly confirms, experimentally, the potential risk for interspecies oral transmission of the agent of L-BSE. In our model, this risk appears higher than that for the agent of classical BSE, which could only be transmitted to mouse lemurs after a first passage in macaques (14). We report oral transmission of the L-BSE agent in young and adult primates. Transmission by the IC route has also been reported in young macaques (6,7). A previous study of L-BSE in transgenic mice expressing human PrP suggested an absence of any transmission barrier between cattle and humans for this particular strain of the agent of BSE, in contrast to findings for the agent of classical BSE (9). Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.


 http://wwwnc.cdc.gov/eid/article/18/1/pdfs/11-1092.pdf


 http://transmissiblespongiformencephalopathy.blogspot.com/2011/12/oral-transmission-of-l-type-bovine.html





P.9.21

Molecular characterization of BSE in Canada

Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada

Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.

Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis.

Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.

Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *** It also suggests a similar cause or source for atypical BSE in these countries.

 http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf





Subject: Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

Emmanuel E. Comoy1*, Cristina Casalone2, Nathalie Lescoutra-Etchegaray1, Gianluigi Zanusso3, Sophie Freire1, Dominique Marcé1, Frédéric Auvré1, Marie-Magdeleine Ruchoux1, Sergio Ferrari3, Salvatore Monaco3, Nicole Salès4, Maria Caramelli2, Philippe Leboulch1,5, Paul Brown1, Corinne I. Lasmézas4, Jean-Philippe Deslys1

1 Institute of Emerging Diseases and Innovative Therapies, CEA, Fontenay-aux-Roses, France, 2 Istituto Zooprofilattico Sperimentale del Piemonte, Turin, Italy, 3 Policlinico G.B. Rossi, Verona, Italy, 4 Scripps Florida, Jupiter, Florida, United States of America, 5 Genetics Division, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts, United States of America

Abstract Top Background

Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform Encephalopathy (cBSE). Atypical forms of BSE, which remain mostly asymptomatic in aging cattle, were recently identified at slaughterhouses throughout Europe and North America, raising a question about human susceptibility to these new prion strains.

Methodology/Principal Findings

Brain homogenates from cattle with classical BSE and atypical (BASE) infections were inoculated intracerebrally into cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously demonstrated to be susceptible to the original strain of cBSE. The resulting diseases were compared in terms of clinical signs, histology and biochemistry of the abnormal prion protein (PrPres). The single monkey infected with BASE had a shorter survival, and a different clinical evolution, histopathology, and prion protein (PrPres) pattern than was observed for either classical BSE or vCJD-inoculated animals. Also, the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine.

Conclusion/Significance

Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products.

Citation: Comoy EE, Casalone C, Lescoutra-Etchegaray N, Zanusso G, Freire S, et al. (2008) Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate. PLoS ONE 3(8): e3017. doi:10.1371/journal.pone.0003017

Editor: Neil Mabbott, University of Edinburgh, United Kingdom

Received: April 24, 2008; Accepted: August 1, 2008; Published: August 20, 2008

Copyright: © 2008 Comoy et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Funding: This work has been supported by the Network of Excellence NeuroPrion.

Competing interests: CEA owns a patent covering the BSE diagnostic tests commercialized by the company Bio-Rad.

* E-mail: emmanuel.comoy@cea.fr

snip...

In summary, we have transmitted one atypical form of BSE (BASE) to a cynomolgus macaque monkey that had a shorter incubation period than monkeys infected with classical BSE, with distinctive clinical, neuropathological, and biochemical features; and have shown that the molecular biological signature resembled that seen in a comparatively uncommon subtype of sporadic CJD. We cannot yet say whether BASE is more pathogenic for primates (including humans) than cBSE, nor can we predict whether its molecular biological features represent a clue to one cause of apparently sporadic human CJD. However, the evidence presented here and by others justifies concern about a potential human health hazard from undetected atypical forms of BSE, and despite the waning epizoonosis of classical BSE, it would be premature to abandon the precautionary measures that have been so successful in reversing the impact of cBSE. We would instead urge a gradual, staged reduction that takes into account the evolving knowledge about atypical ruminant diseases, and both a permanent ban on the use of bovine central nervous system tissue for either animal or human use, and its destruction so as to eliminate any risk of environmental contamination.

 http://www.plosone.org/article/info:doi%2F10.1371%2Fjournal.pone.0003017




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


 http://usdavskorea.blogspot.com/



2012 atypical l-type BASE BSE documented in California...

R-CALF United Stockgrowers of America

“Fighting for the U.S. Cattle Producer”

For Immediate Release Contact: R-CALF USA CEO Bill Bullard

June 14, 2012 Phone: 406-252-2516; r-calfusa@r-calfusa.com

R-CALF USA Calls USDA Dishonest and Corrupt; Submits Fourth Request for Extension

Billings, MT – Yesterday, June 13, R-CALF USA sent a letter to U.S. Secretary of Agriculture Tom Vilsack requesting an extension of the public comment period in the proposed rule titled “Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products” (Proposed Rule). This is the fourth request for an extension made by the group.

While the comment period for the rule was reopened for a short period after the initial deadline, it did not provide enough time for the public to review and comment on such a comprehensive Proposed Rule. R-CALF USA is concerned that the U.S. Department of Agriculture (USDA) is ignoring the multiple requests for extension because upon thorough review the public will see the Proposed Rule as corrupt and dishonest. These deceitful tactics might not be noticed if organizations and individuals do not have adequate time to carefully read and respond.

R-CALF USA requests that USDA extend the comment period for the Proposed Rule for at least 60 days following the completion of USDA’s epidemiological investigation of the recently detected BSE case in California. This would allow adequate time for thorough and meaningful comments.

In the letter, R-CALF USA CEO Bill Bullard states the “Proposed Rule is riddled with inconsistencies, contradictions and outright lies.” Bullard further states that USDA “has perjured itself in its pursuit of [Vilsack’s] political agenda to expose U.S. cattle and the public to an increased risk of BSE.”

In support of its assertion that the Proposed Rule contains outright lies, R-CALF USA cites a specific example where USDA told a U.S. district court that its proposal would eliminate the possibility of BSE-infectivity, but now the agency claims in the Proposed Rule that it has not stated that the risk is “eliminated.”

“Your agency is wholly lacking in accountability, creditability, and integrity. It is impossible for the public interest to even be considered, let alone protected, by an agency that resorts to outright lies in order to further its own political agenda,” the letter states.

# # #

R-CALF USA (Ranchers-Cattlemen Action Legal Fund, United Stockgrowers of America) is a national, nonprofit organization dedicated to ensuring the continued profitability and viability of the U.S. cattle industry. For more information, visit www.r-calfusa.com or, call 406-252-2516.

http://r-calfusa.com/news_releases/2012/120614Fourth.pdf



Wednesday, June 13, 2012

MEXICO IS UNDER or MIS DIAGNOSING CREUTZFELDT JAKOB DISEASE AND OTHER PRION DISEASE SOME WITH POSSIBLE nvCJD

http://creutzfeldt-jakob-disease.blogspot.com/2012/06/mexico-is-under-or-mis-diagnosing.html



IN SHORT, AND IN A NUT SHELL ;

(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,

http://www.oie.int/eng/Session2007/RF2006.pdf



Friday, May 25, 2012

R-CALF USDA’s New BSE Rule Eliminates Important Protections Needed to Prevent BSE Spread

http://bseusa.blogspot.com/2012/05/r-calf-usdas-new-bse-rule-eliminates.html



Saturday, May 26, 2012

Are USDA assurances on mad cow case 'gross oversimplification'?

SNIP...

What irks many scientists is the USDA’s April 25 statement that the rare disease is “not generally associated with an animal consuming infected feed.”

The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown, one of the world’s experts on this type of disease who retired recently from the National Institutes of Health. "(The agency) has no foundation on which to base that statement.”

“We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an official with the USDA during the Clinton Administration now at Mississippi State.

In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows the origins of atypical cases of BSE,” she said

The argument about feed is critical because if feed is the cause, not a spontaneous mutation, the California cow could be part of a larger outbreak.

SNIP...

http://bseusa.blogspot.com/2012/05/are-usda-assurances-on-mad-cow-case.html



Sunday, May 27, 2012

GAIN REPORT BSE Case in United States Will Not Affect Trade, States Canadian Food Inspection Agency

http://madcowusda.blogspot.com/2012/05/gain-bse-case-in-united-states-will-not.html



PO-028: Oral transmission of L-type bovine spongiform encephalopathy (L-BSE) in primate model Microcebus murinus

Nadine Mestre-Frances,1 Simon Nicot,2 Sylvie Rouland,1 Anne-Gaëlle Biacabe,2 Isabelle Quadrio,3 Armand Perret-Liaudet,3 Thierry Baron,2 Jean-Michel Verdier1 1IN SER M UM2; Montpellier, France; 2Anses; Lyon, France; 3Hopitaux Civils de Lyon; Lyon, France

An atypical form of bovine spongiform encephalopathy has been identified in cattle in Europe, North America and Japan and was designed as L-type BSE (L-BSE) due to the lower apparent molecular mass of the unglycosylated, protease-resistant prion protein (PrPres) detected by western blot compared with classical BSE. Experimental evidences from studies in transgenic mice expressing human PrP and in primate models suggest a higher risk of transmission to humans of the L-BSE form than for classical BSE agent. However, a major unresolved issue concerns the potential transmissibility of the L-BSE agent by oral route. To address this question, we infected mouse lemurs (Microcebus murinus), a non-human primate model, with L-BSE by intracerebral or oral route.

Four adult lemurs were intracerebrally (IC) inoculated with 5mg of L-BSE infected brain homogenate of an atypical French BSE case (02-2528). Four young and four adult animals were fed with 5 mg or 50 mg of infected brain. After sacrifice, the brain tissues were biochemically and immunocytochemically investigated for PrPres.

The 4 animals IC inoculated died at 19 and 22 months postinoculation (mpi). They developed blindness, tremor, abnormal posture, incoordinated movements, balance loss. Symptoms get worse according to the disease progression, until severe ataxia. Severe spongiosis was evidenced into the thalamus, the striatum, the mesencephalon, and the brainstem, whereas into the cortex the vacuolisation was weaker. Strong deposits of PrPres were detected into the thalamus, the striatum, and the hippocampus whereas in the cerebral cortex, PrPres was prominently accumulated in plaques.

The orally inoculated animals showed similar clinical symptoms occurring between 27 and 34 mpi. Disease was characterized by progressive prostration, loss of appetite and poor appearance of the fur. Only one adult animal showed disequilibrium. PrPres was strongly accumulated only in the striatum and thalamus and weakly into the cortex. No plaques were evidenced. Two animals that were orally challenged at the age of two years are still alive and healthy 34 months after inoculation. The western blot analysis showed uniform molecular profiles, irrespective of the route or dose of infection, and included notably a PrPres form with low apparent molecular mass (~19 kDa) similar to the PrPres in the original cattle brain. However, the PrPres profile in lemurs was characterized by a higher proportion of di- and mono-glycosylated species (up to 95% of the total signal) than in the bovine L-BSE inoculum (~80%). In addition, small amounts of PrPres were detected by western blotting in the spleen of three animals (one intra-cerebrally inoculated and two fed with 5 mg of cattle brain).

Here, we demonstrate that the L-BSE agent can be transmitted by oral route from cattle to young and adult mouse lemurs. In comparison to IC inoculated animals, orally challenged lemurs were characterized by longer survival periods as expected with this route of infection.

http://www.landesbioscience.com/journals/prion/03-Prion6-2-Transmission-and-strains.pdf



Subject: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68

Comment from Terry Singeltary Document ID: APHIS-2008-0010-0008 Document Type: Public Submission This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products Docket ID: APHIS-2008-0010 RIN:0579-AC68

Topics: No Topics associated with this document View Document: More Document Subtype: Public Comment Status: Posted Received Date: March 22 2012, at 12:00 AM Eastern Daylight Time Date Posted: March 22 2012, at 12:00 AM Eastern Daylight Time Comment Start Date: March 16 2012, at 12:00 AM Eastern Daylight Time Comment Due Date: May 15 2012, at 11:59 PM Eastern Daylight Time Tracking Number: 80fdd617 First Name: Terry Middle Name: S. Last Name: Singeltary City: Bacliff Country: United States State or Province: TX Organization Name: CJD TSE PRION Submitter's Representative: CONSUMERS

Comment: comment submission Document ID APHIS-2008-0010-0001

Greetings USDA,

OIE et al, what a difference it makes with science, from one day to the next. i.e. that mad cow gold card the USA once held. up until that fateful day in December of 2003, the science of BSE was NO IMPORTS TO USA FROM BSE COUNTRY. what a difference a day makes$ now that the shoe is on the other foot, the USDA via the OIE, wants to change science again, just for trade $ I implore the OIE decision and policy makers, for the sake of the world, to refuse any status quo of the USA BSE risk assessment. if at al, the USA BSE GBR should be raise to BSE GBR IV, for the following reasons. North America is awash with many different TSE Prion strains, in many different species, and they are mutating and spreading. IF the OIE, and whatever policy makers, do anything but raise the risk factor for BSE in North America, they I would regard that to be highly suspicious. IN fact, it would be criminal in my opinion, because the OIE knows this, and to knowingly expose the rest of the world to this dangerous pathogen, would be ‘knowingly’ and ‘willfully’, just for the almighty dollar, once again. I warned the OIE about all this, including the risk factors for CWD, and the fact that the zoonosis potential was great, way back in 2002. THE OIE in collaboration with the USDA, made the legal trading of the atypical Nor-98 Scrapie a legal global commodity. yes, thanks to the OIE and the USDA et al, it’s now legal to trade the atypical Nor-98 Scrapie strain all around the globe. IF you let them, they will do the same thing with atypical BSE and CWD (both strains to date). This with science showing that indeed these TSE prion strains are transmissible. I strenuously urge the OIE et al to refuse any weakening to the USA trade protocols for the BSE TSE prion disease (all strains), and urge them to reclassify the USA with BSE GBR IV risk factor.

SEE REFERENCE SOURCES IN ATTACHMENTS

PLEASE SEE Terry S. Singeltary Sr. _Attachment_ WORD FILE ;

http://www.regulations.gov/#!documentDetail;D=APHIS-2008-0010-0008


http://www.regulations.gov/#!docketDetail;D=APHIS-2008-0010



***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



MAD COW USDA ATYPICAL L-TYPE BASE BSE, the rest of the story...

***Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model

http://wwwnc.cdc.gov/eid/article/18/1/pdfs/11-1092.pdf



***Infectivity in skeletal muscle of BASE-infected cattle

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2009/prion2009_bookofabstracts.pdf


***feedstuffs- It also suggests a similar cause or source for atypical BSE in these countries.

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2009/prion2009_bookofabstracts.pdf



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

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



The present study demonstrated successful intraspecies transmission of H-type BSE to cattle and the distribution and immunolabeling patterns of PrPSc in the brain of the H-type BSE-challenged cattle. TSE agent virulence can be minimally defined by oral transmission of different TSE agents (C-type, L-type, and H-type BSE agents) [59]. Oral transmission studies with H-type BSEinfected cattle have been initiated and are underway to provide information regarding the extent of similarity in the immunohistochemical and molecular features before and after transmission.

In addition, the present data will support risk assessments in some peripheral tissues derived from cattle affected with H-type BSE.

http://www.veterinaryresearch.org/content/pdf/1297-9716-42-79.pdf



in the url that follows, I have posted

SRM breaches first, as late as 2011.

then

MAD COW FEED BAN BREACHES AND TONNAGES OF MAD COW FEED IN COMMERCE up until 2007, when they ceased posting them.

then,

MAD COW SURVEILLANCE BREACHES.

Friday, May 18, 2012

Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States Friday May 18, 2012

http://transmissiblespongiformencephalopathy.blogspot.com/2012/05/update-from-aphis-regarding-detection.html



Wednesday, May 30, 2012

PO-028: Oral transmission of L-type bovine spongiform encephalopathy (L-BSE) in primate model Microcebus murinus

http://bse-atypical.blogspot.com/2012/05/po-028-oral-transmission-of-l-type.html



Identification of a second bovine amyloidotic spongiform encephalopathy: Molecular similarities with sporadic Creutzfeldt–Jakob disease

Cristina Casalone*†, Gianluigi Zanusso†‡, Pierluigi Acutis*, Sergio Ferrari‡, Lorenzo Capucci§, Fabrizio Tagliavini¶, Salvatore Monaco‡ , and Maria Caramelli* *Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d’Aosta, Via Bologna, 148, 10195 Turin, Italy; ‡Department of Neurological and Visual Science, Section of Clinical Neurology, Policlinico G.B. Rossi, Piazzale L.A. Scuro, 10, 37134 Verona, Italy; §Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Via Bianchi, 9, 25124 Brescia, Italy; and ¶Istituto Nazionale Neurologico ‘‘Carlo Besta,’’ Via Celoria 11, 20133 Milan, Italy

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved December 23, 2003 (received for review September 9, 2003)

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are mammalian neurodegenerative disorders characterized by a posttranslational conversion and brain accumulation of an insoluble, protease-resistant isoform (PrPSc) of the host-encoded cellular prion protein (PrPC). Human and animal TSE agents exist as different phenotypes that can be biochemically differentiated on the basis of the molecular mass of the protease-resistant PrPSc fragments and the degree of glycosylation. Epidemiological, molecular, and transmission studies strongly suggest that the single strain of agent responsible for bovine spongiform encephalopathy (BSE) has infected humans, causing variant Creutzfeldt–Jakob disease. The unprecedented biological properties of the BSE agent, which circumvents the so-called ‘‘species barrier’’ between cattle and humans and adapts to different mammalian species, has raised considerable concern for human health. To date, it is unknown whether more than one strain might be responsible for cattle TSE or whether the BSE agent undergoes phenotypic variation after natural transmission. Here we provide evidence of a second cattle TSE. The disorder was pathologically characterized by the presence of PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid deposition in typical BSE cases, and by a different pattern of regional distribution and topology of brain PrPSc accumulation. In addition, Western blot analysis showed a PrPSc type with predominance of the low molecular mass glycoform and a protease- resistant fragment of lower molecular mass than BSE-PrPSc. Strikingly, the molecular signature of this previously undescribed bovine PrPSc was similar to that encountered in a distinct subtype of sporadic Creutzfeldt–Jakob disease.

Phenotypic Similarities Between BASE and sCJD. The transmissibility of CJD brains was initially demonstrated in primates (27), and classification of atypical cases as CJD was based on this property (28). To date, no systematic studies of strain typing in sCJD have been provided, and classification of different subtypes is based on clinical, neuropathological, and molecular features (the polymorphic PRNP codon 129 and the PrPSc glycotype) (8, 9, 15, 19). The importance of molecular PrPSc characterization in assessing the identity of TSE strains is underscored by several studies, showing that the stability of given disease-specific PrPSc types is maintained upon experimental propagation of sCJD, familial CJD, and vCJD isolates in transgenic PrP-humanized mice (8, 29). Similarly, biochemical properties of BSE- and vCJDassociated PrPSc molecules remain stable after passage to mice expressing bovine PrP (30). Recently, however, it has been reported that PrP-humanized mice inoculated with BSE tissues may also propagate a distinctive PrPSc type, with a ‘‘monoglycosylated- dominant’’ pattern and electrophoretic mobility of the unglycosylated fragment slower than that of vCJD and BSE (31). Strikingly, this PrPSc type shares its molecular properties with the a PrPSc molecule found in classical sCJD. This observation is at variance with the PrPSc type found in M V2 sCJD cases and in cattle BASE, showing a monoglycosylated-dominant pattern but faster electrophoretic mobility of the protease-resistant fragment as compared with BSE. In addition to molecular properties of PrPSc, BASE and M V2 sCJD share a distinctive pattern of intracerebral PrP deposition, which occurs as plaque-like and amyloid-kuru plaques. Differences were, however, observed in the regional distribution of PrPSc. While inM V2 sCJD cases the largest amounts of PrPSc were detected in the cerebellum, brainstem, and striatum, in cattle BASE these areas were less involved and the highest levels of PrPSc were recovered from the thalamus and olfactory regions.

In conclusion, decoding the biochemical PrPSc signature of individual human and animal TSE strains may allow the identification of potential risk factors for human disorders with unknown etiology, such as sCJD. However, although BASE and sCJD share several characteristics, caution is dictated in assessing a link between conditions affecting two different mammalian species, based on convergent biochemical properties of diseaseassociated PrPSc types. Strains of TSE agents may be better characterized upon passage to transgenic mice. In the interim until this is accomplished, our present findings suggest a strict epidemiological surveillance of cattle TSE and sCJD based on molecular criteria.

 http://www.pnas.org/content/101/9/3065.full.pdf+html



Employment Listings position: Post Doctoral Fellow | Atypical BSE in Cattle

Closing date: December 24, 2009

Anticipated start date: January/February 2010

Employer: Canadian and OIE Reference Laboratories for BSE CFIA Lethbridge Laboratory, Lethbridge/Alberta

The Canadian and OIE reference laboratories for BSE are extensively involved in prion diseases diagnosis and research. With a recent increase in research activities and funding, the laboratory is looking to fill two post doctoral fellow positions. Both positions will be located at the Canadian Food Inspection Agency (CFIA) Lethbridge Laboratory which offers biosaftey level 3 (BSL3) and BSL2 laboratory space and is well equipped for molecular and morphologic prion research. The facility also has a BSL3 large animal housing wing and a state of the art post mortem room certified for prion work. Successful candidates will have the opportunity to visit other laboratories to cooperate in various aspects of the projects and to be trained in new techniques and acquire new skills. With a recent increase in prion disease expertise and research in Alberta and Canada, these positions will offer significant exposure to cutting edge prion science via videoconferencing, meetings, workshops and conferences. These interactions will also provide a valuable opportunity to present research findings and discuss potential future work opportunities and collaborations with other Canadian and international research groups.

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.

This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.

Responsibilities include:

Driving research at the National and OIE BSE reference lab to ensure project milestones are met successfully. Contributing to the preparation of project progress reports. Directing technical staff working on the project. Communicating and discussing results, progress and future direction with project principle investigator(s). Communicating with collaborative project partners.

Qualifications:

Successful completion of a PhD degree in an area focusing on or related to prion diseases. Extensive experience with molecular and/or morphologic techniques used in studying prion diseases and/or other protein misfolding disorders. Ability to think independently and contribute new ideas. Excellent written and oral communication skills. Ability to multitask, prioritize, and meet challenges in a timely manner. Proficiency with Microsoft Office, especially Word, PowerPoint and Excel.

How to apply:

Please send your application and/or inquiry to: Dr. Stefanie Czub, DVM, Ph.D. Head, National and OIE BSE Reference Laboratory Canadian Food Inspection Agency Lethbridge Laboratory P.O. Box 640, Township Road 9-1 Lethbridge, AB, T1J 3Z4 Canada

phone: +1-403-382-5500 +1-403-382-5500 ext. 5549 email: stefanie.czub@inspection.gc.ca

Contact Info:

Last Updated: 12/10/2009 1:35:18 PM

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



price of prion poker goes up again $$$

Sunday, June 3, 2012

A new neurological disease in primates inoculated with prion-infected blood or blood components

http://transmissiblespongiformencephalopathy.blogspot.com/2012/06/new-neurological-disease-in-primates.html



Monday, June 11, 2012

Guidance for Industry Draft Guidance for Industry: Amendment to “Guidance for Industry: Revised Preventive Measures to Reduce the Possible Risk of Transmission of Creutzfeldt-Jakob Disease and Variant Creutzfeldt-Jakob Disease by Blood and Blood Products”

http://creutzfeldt-jakob-disease.blogspot.com/2012/06/guidance-for-industry-draft-guidance.html



Sunday, February 12, 2012

National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas

http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/national-prion-disease-pathology.html



Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis


 http://www.youtube.com/watch?v=zf3lfz9NrT4


 http://www.youtube.com/watch?v=c0tWkNvhO4g


 http://www.youtube.com/watch?v=zf3lfz9NrT4&feature=results_main&playnext=1&list=PL780BE2AF0B62A944




full text with source references ;

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





Thursday, April 26, 2012

FDA Statement on USDA Announcement of Positive BSE Test Result For Immediate Release: April 26, 2012

http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/fda-statement-on-usda-announcement-of.html





TSS