Friday, December 5, 2014

Dr. Gambetti will be resigning as Director of the National Prion Disease Pathology

The National Prion Disease Pathology Surveillance Center

 

Supported by the Centers for Disease Control and Prevention (CDC)

 

Sponsored by the American Association of Neuropathologists (AANP)

 

____________________________________

 

Office: Advisory Committee:

 

Dr. Pierluigi Gambetti, Director Dr. Barbara Crain Dr. Bernardino Ghetti, Co-Director Dr. Stephen DeArmond Dr. Jiri Safar, Co-Director Dr. Daniel Perl Dr. Wenquan Zou, Associate Director Dr. Clive Hamlin, Director, Clinical Laboratory Dr. Shulin Zhang, Genetic Analyses Dr. Mark Cohen, Histopathology

 

Andrea Webb, Center Manager

 

To the families touched by prion diseases

 

Dear families,

 

I wish to inform you that I will be resigning as Director of the National Prion Disease Pathology Surveillance Center (NPDPSC) effective January 1st 2015. I will continue to work with the Center in an advisory position and as a consultant for special cases. I will also continue to serve as Medical Director of the CJD Foundation through 2015, a role I value immensely.

 

Dr. Jiri Safar, Associate Professor of Pathology and Neurology at Case Western Reserve University, will be the new Director. Dr. Safar graduated from Charles University Medical School, Prague, Czech Republic, where he trained in Neurology. Dr. Safar has made many contributions to prion research and is an internationally recognized expert in prion diseases. He worked with Dr. Carleton Gajdusek and with Dr. Stanley Prusiner, each of whom was awarded a Nobel Prize for their prion work.

 

The mission and basic activities of the NPDPSC will remain unchanged. The Center will continue to coordinate free-of-charge autopsies in all cases of suspected prion diseases, and will continue to perform and report diagnostic examinations of tissues, CSF and blood.

 

In 1997 I established the NPDPSC with the support of the Centers for Disease Control and Prevention (CDC). The collaboration of the Creutzfeldt-Jakob Disease (CJD) Foundation with our Center began in 1999 and intensified after 2002 when the Foundation moved to Akron under the leadership of Florence Kranitz, and collaboration has increased ever since. During 17 years of NPDPSC activity I had the opportunity to personally interact with many of you, the patient’s families, especially at the Annual CJD Foundation Family Conference. These interactions were of inestimable value to me, for they gave me and many others at the Center a firsthand glimpse into the personal anguish that prion diseases cause, as well as making me and the other researchers in attendance even more aware of our scientific limitations, and of the dramatic need for finding a treatment. I’m deeply grateful to you for these interactions that I will always treasure.

 

As Director I hope that the NPDSC has helped you somewhat, by giving you a sense of closure to help you deal with your grief.

 

Sincerely yours,

 


 

TSS

 

SPECIAL ALERT The OIE recommends strengthening animal disease surveillance worldwide

 

OIE BSE TSE PRION AKA MAD COW DISEASE ?

 

‘’the silence was deafening’’ ...tss

 


 

Sunday, November 23, 2014

 

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

 


 

Monday, November 3, 2014

 

USA CJD TSE PRION UNIT, TEXAS, SURVEILLANCE UPDATE NOVEMBER 2014

 

National Prion Disease Pathology Surveillance Center Cases Examined1 (October 7, 2014)

 

***6 Includes 11 cases in which the diagnosis is pending, and 19 inconclusive cases;

 

***7 Includes 12 (11 from 2014) cases with type determination pending in which the diagnosis of vCJD has been excluded.

 

***The sporadic cases include 2660 cases of sporadic Creutzfeldt-Jakob disease (sCJD),

 

***50 cases of Variably Protease-Sensitive Prionopathy (VPSPr)

 

***and 21 cases of sporadic Fatal Insomnia (sFI).

 


 

Monday, November 3, 2014

 

The prion protein protease sensitivity, stability and seeding activity in variably protease sensitive prionopathy brain tissue suggests molecular overlaps with sporadic Creutzfeldt-Jakob disease

 


 

Tuesday, November 04, 2014

 

The pathological and molecular but not clinical phenotypes are maintained after second passage of experimental atypical bovine spongiform encephalopathy in cattle

 


 

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

 


 

Tuesday, August 12, 2014

 

MAD COW USDA TSE PRION COVER UP or JUST IGNORANCE, for the record AUGUST 2014

 


 

Thursday, October 02, 2014

 

[Docket No. APHIS-2013-0064] Concurrence With OIE Risk Designations for Bovine Spongiform Encephalopathy

 


 

Saturday, August 14, 2010

 

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

 


 

2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006

 


 

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

 


 

Jeff Schwan, sporadic cjd, clustering, and BSE aka mad cow type disease, is there a link ? *video*

 


 

1997-11-10: Panorama - The british disease *video*

 


 

Sunday, September 6, 2009

 

MAD COW USA 1997 *video*

 


 


 


 


 


 


 


 


 


 


 


 


 

tss

Monday, November 3, 2014

USA CJD TSE PRION UNIT, TEXAS, SURVEILLANCE UPDATE NOVEMBER 2014

National Prion Disease Pathology Surveillance Center Cases Examined1 (October 7, 2014)

 

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

 

5 Disease possibly acquired in a Middle Eastern or Eastern European country;

 

6 Includes 11 cases in which the diagnosis is pending, and 19 inconclusive cases;

 

7 Includes 12 (11 from 2014) cases with type determination pending in which the diagnosis of vCJD has been excluded.

 

The sporadic cases include 2660 cases of sporadic Creutzfeldt-Jakob disease (sCJD),

 

50 cases of Variably Protease-Sensitive Prionopathy (VPSPr)

 

and 21 cases of sporadic Fatal Insomnia (sFI).

 

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

 

1996 & earlier 53 35 31 4 0 0

 

1997 114 68 59 9 0 0

 

1998 88 52 44 7 1 0

 

1999 123 74 65 8 1 0

 

2000 145 103 89 14 0 0

 

2001 212 120 110 10 0 0

 

2002 248 149 126 21 2 0

 

2003 265 167 137 30 0 0

 

2004 326 187 164 22 0 13

 

2005 343 194 157 36 1 0

 

2006 381 194 164 28 0 24

 

2007 377 213 184 29 0 0

 

2008 397 233 208 25 0 0

 

2009 421 255 213 41 1 0

 

2010 414 258 217 41 0 0

 

2011 410 256 215 41 0 0

 

2012 421 253 218 35 0 0

 

2013 439 280 221 57 1 0

 

2014 275 166 109 45 0 15

 

TOTAL 54526 32577 2731 503 7 4

 
http://www.cjdsurveillance.com/pdf/web_table.pdf


if you notice, if you add up the vpspr 50 cases and the sporadic FFI 21 cases, and then add to the total sporadic cjd cases of 2660, this is where the total of 2731 sporadic cases come from...seems they are lumping them all together as total sporadic cjd cases, and leaving the sporadic cjd text completly out of the picture now. smoooth. covering all bases I presume, and they moved the goal post in the middle of the game, again. niice...we all didn't fall off a shrimp boat yesterday$$$
 
 
 

 
Prion Disease Cases in Texas by Year, 2003-2012
Prion Disease Case Counts 2012
 
 
CJD_Graph_2012
 
CJD Age 2012
 
 
 
Last updated September 17, 2013
 
TEXAS CJD TSE PRION DISEASE SEE MAP ;
 
 
USA 4TH CASE VCJD (aka nvCJD) HUMAN MAD COW, THE SILENCE IS DEAFENING BSE, CWD, AND SCRAPIE TSE PRION DISEASE
 
Greetings DSHS, Dr. Fishcer, et al,
 
I know that most in the USA could care less about the CJD TSE prion disease aka mad cow type disease. but there are some of us here that will never forget.
 
you can cover up what ever you want. we all know. I have seen it happen too many times here in Texas with BSE TSE prion, either the typical or the atypical strains, or with the feed, or, with cwd, or scrapie as that goes, but we are still here, and we will never forget...
 
kind regards, terry
 
Creutzfeld-Jacob Disease (CJD) Emerging & Acute Infectious Disease Branch Michael Fischer
 
Marilyn Felkner
 
512-776-7676
 
512-776-7676
 
Chronic Wasting Disease Zoonosis Control Branch Eric Fonken
 
512-776-2155
 
Lab tests have confirmed a diagnosis of variant Creutzfeldt-Jakob Disease (CJD) in a patient who recently died in Texas. Variant CJD is a rare, fatal brain disorder, first described in 1996 in the United Kingdom and associated with beef consumption overseas.
 
This is the fourth case ever reported in the United States. In each of the three previous cases, infection likely occurred outside the United States, including the United Kingdom and Saudi Arabia. The history of this fourth patient includes extensive travel to Europe and the Middle East, and infection likely occurred outside the United States. The CDC and DSHS continue to investigate the case.
 
There are no Texas public health concerns or threats associated with this case.
 
 
 
 
Last updated June 02, 2014
 
 
CONFIRMED HUMAN BSE AKA MAD COW DISEASE vCJD TEXAS USA
 
CONFIRMED HUMAN BSE AKA MAD COW DISEASE vCJD TEXAS USA
 
Monday, June 02, 2014 Confirmed Variant CJD Case in Texas
 
 
SO, 4 months after the fact and still no word on this case. no information what so ever. the silence is deafening $$$
 
Monday, November 3, 2014
 
The prion protein protease sensitivity, stability and seeding activity in variably protease sensitive prionopathy brain tissue suggests molecular overlaps with sporadic Creutzfeldt-Jakob disease
 
 
 
 
Saturday, August 14, 2010
 
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY
 
 
Monday, June 23, 2014
 
PRION 2014 TYPICAL AND ATYPICAL BSE AND CJD REPORT UPDATES
 
 
 Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014
 
Transmissible Spongiform Encephalopathy TSE Prion Disease have now been discovered in a wide verity of species across North America. typical C-BSE, atypical L-type BASE BSE, atypical H-type BSE, atypical H-G BSE, of the bovine, typical and atypical Scrapie strains, in sheep and goats, with atypical Nor-98 Scrapie spreading coast to coast in about 5 years. Chronic Wasting Disease CWD in cervid is slowly spreading without any stopping it in Canada and the USA and now has mutated into many different strains. Transmissible Mink Encephalopathy TME outbreaks. These Transmissible Spongiform Encephalopathy TSE Prion Disease have been silently mutating and spreading in different species in North America for decades.
 
The USDA, FDA, et al have assured us of a robust Triple BSE TSE prion Firewall, of which we now know without a doubt, that it was nothing but ink on paper. Since the 1997 mad cow feed ban in the USA, literally tons and tons of banned mad cow feed has been put out into commerce, never to return, as late as December of 2013, serious, serious breaches in the FDA mad cow feed ban have been documented. The 2004 enhanced BSE surveillance program was so flawed, that one of the top TSE prion Scientist for the CDC, Dr. Paul Brown stated ; Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.
 
see ;
 
 
The BSE surveillance and testing have also been proven to be flawed, and the GAO and OIG have both raised serious question as to just how flawed it has been (see GAO and OIG reports). North America has more documented TSE prion disease, in different documented species (excluding the Zoo BSE animals in the EU), then any other place on the Globe. This does not include the very likelihood that TSE prion disease in the domestic feline and canine have been exposed to high doses of the TSE prion disease vid pet food. To date, it’s still legal to include deer from cwd zone into pet food or deer food. Specified Risk Material i.e. SRM bans still being breach, as recently as just last month.
 
nvCJD or what they now call vCJD, another case documented in Texas last month, with very little information being released to the public on about this case? with still the same line of thought from federal officials, ‘it can’t happen here’, so another vCJD blamed on travel of a foreign animal disease from another country, while ignoring all the BSE TSE Prion risk factors we have here in the USA and Canada, and the time that this victim and others, do spend in the USA, and exposed to these risk factors, apparently do not count in any way with regard to risk factor. a flawed process of risk assessment.
 
sporadic CJD, along with new TSE prion disease in humans, of which the young are dying, of which long duration of illness from onset of symptoms to death have been documented, only to have a new name added to the pot of prion disease i.e. sporadic GSS, sporadic FFI, and or VPSPR. I only ponder how a familial type disease could be sporadic with no genetic link to any family member? when the USA is the only documented Country in the world to have documented two different cases of atypical H-type BSE, with one case being called atypical H-G BSE with the G meaning Genetic, with new science now showing that indeed atypical H-type BSE is very possible transmitted to cattle via oral transmission (Prion2014). sporadic CJD and VPSPR have been rising in Canada, USA, and the UK, with the same old excuse, better surveillance. You can only use that excuse for so many years, for so many decades, until one must conclude that CJD TSE prion cases are rising. a 48% incease in CJD in Canada is not just a blip or a reason of better surveillance, it is a mathematical rise in numbers. More and more we are seeing more humans exposed in various circumstance in the Hospital, Medical, Surgical arenas to the TSE Prion disease, and at the same time in North America, more and more humans are becoming exposed to the TSE prion disease via consumption of the TSE prion via deer and elk, cattle, sheep and goats, and for those that are exposed via or consumption, go on to further expose many others via the iatrogenic modes of transmission of the TSE prion disease i.e. friendly fire. I pondered this mode of transmission via the victims of sporadic FFI, sporadic GSS, could this be a iatrogenic event from someone sub-clinical with sFFI or sGSS ? what if?
 
Two decades have passed since Dr. Ironside first confirmed his first ten nvCJD victims in 1995. Ten years later, 2005, we had Dr. Gambetti and his first ten i.e. VPSPR in younger victims. now we know that indeed VPSPR is transmissible. yet all these TSE prion disease and victims in the USA and Canada are being pawned off as a spontaneous event, yet science has shown, the spontaneous theory has never been proven in any natural case of TSE prion disease, and scientist have warned, that they have now linked some sporadic CJD cases to atypical BSE, to atypical Scrapie, and to CWD, yet we don’t here about this in the public domain. We must make all human and animal TSE prion disease reportable in every age group, in ever state and internationally, we must have a serious re-evaluation and testing of the USA cattle herds, and we must ban interstate movement of all cervids. Any voluntary effort to do any of this will fail. Folks, we have let the industry run science far too long with regards to the TSE prion disease. While the industry and their lobbyist continues to funnel junk science to our decision policy makers, Rome burns. ...end
 
REFERENCES
 
Sunday, June 29, 2014
 
Transmissible Spongiform Encephalopathy TSE Prion Disease North America 2014
 
 
Tuesday, August 12, 2014
 
MAD COW USDA TSE PRION COVER UP or JUST IGNORANCE, for the record AUGUST 2014
 
 
Sunday, July 06, 2014
 
Dietary Risk Factors for Sporadic Creutzfeldt-Jakob Disease: A Confirmatory Case-Control Study
 
Conclusions—The a priori hypotheses were supported.
 
*Consumption of various meat products may be one method of transmission of the infectious agent for sCJD.
 
 
 PLEASE NOTE, all url links linked as ;
 
 
 have changed to ;
 
 
 Sunday, January 19, 2014
 
*** National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014
 
 
 Thursday, February 21, 2013
 
 *** National Prion Disease Pathology Surveillance Center Cases Examined January 16, 2013
 
 
 Monday, July 23, 2012
 
*** The National Prion Disease Pathology Surveillance Center July 2012
 
 
 Friday, April 15, 2011
 
*** PRION TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY PROJECTS, RESEARCH FUNDING, BSE VOLUNTARY TESTING UPDATE IN NORTH AMERICA 2011
 
 
Monday, August 9, 2010
 
*** National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)
 
 
Sunday, May 16, 2010
 
*** The Final Report of the National Prion Research Program 2007 (updated 2010)
 
 
Monday, April 5, 2010
 
*** UPDATE - CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER
 
 
Friday, March 12, 2010
 
*** CJD 2010 and the CJD Foundation Family Conference
 
 
 Saturday, January 2, 2010
 
 *** Human Prion Diseases in the United States January 1, 2010
 
 
Monday, April 20, 2009
 
*** National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)
 
 
Sunday, April 12, 2009
 
*** r-calf and the USA mad cow problem, don't look, don't find, and then blame Canada
 
 
 Saturday, April 11, 2009
 
*** CJD FOUNDATION SIDES WITH R-CALFERS NO BSE OR HUMAN TSE THERE OF IN USA 'don't be fooled'
 
 
 Sunday, April 20, 2008
 
*** Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008
 
 
 
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net
 
 
 
 
 
 
 
 
 
 
 




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Tuesday, January 21, 2014

PRION UNIT USA UPDATE URL CHANGES prionunitusaupdate2008.blogspot.com

PRION UNIT USA UPDATE URL CHANGES prionunitusaupdate2008.blogspot.com

 

 

PLEASE NOTE, all url links linked as ;

 


 

have changed to ;

 


 

 

 

FIRST LINK IS A GOOD LINK ;

 

 

Sunday, January 19, 2014

 

*** National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014

 


 

 

see history of links and changes as followed ;

 

 

Thursday, February 21, 2013

 

National Prion Disease Pathology Surveillance Center Cases Examined January 16, 2013

 


 

CHANGE TO ;

 

Thursday, February 21, 2013

 

*** National Prion Disease Pathology Surveillance Center Cases Examined January 16, 2013

 


 

 

Monday, July 23, 2012

 

The National Prion Disease Pathology Surveillance Center July 2012

 


 

CHANGE TO ;

 

Monday, July 23, 2012

 

*** The National Prion Disease Pathology Surveillance Center July 2012

 


 

 

Friday, April 15, 2011

 

PRION TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY PROJECTS, RESEARCH FUNDING, BSE VOLUNTARY TESTING UPDATE IN NORTH AMERICA 2011

 


 

CHANGE TO ;

 

Friday, April 15, 2011

 

*** PRION TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY PROJECTS, RESEARCH FUNDING, BSE VOLUNTARY TESTING UPDATE IN NORTH AMERICA 2011

 


 

 

Monday, August 9, 2010

 

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

 


 

CHANGE TO ;

 

Monday, August 9, 2010

 

*** National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

 


 

 

Sunday, May 16, 2010

 

The Final Report of the National Prion Research Program 2007 (updated 2010)

 


 

CHANGE TO ;

 

Sunday, May 16, 2010

 

*** The Final Report of the National Prion Research Program 2007 (updated 2010)

 


 

 

Monday, April 5, 2010

 

UPDATE - CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

 


 

CHANGE TO ;

 

Monday, April 5, 2010

 

*** UPDATE - CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

 


 

 

Friday, March 12, 2010

 

CJD 2010 and the CJD Foundation Family Conference

 


 

CHANGE TO ;

 

Friday, March 12, 2010

 

*** CJD 2010 and the CJD Foundation Family Conference

 


 

 

Saturday, January 2, 2010

 

Human Prion Diseases in the United States January 1, 2010

 


 

CHANGE TO ;

 

Saturday, January 2, 2010

 

*** Human Prion Diseases in the United States January 1, 2010

 


 

 

Monday, April 20, 2009

 

National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)

 


 

CHANGE TO ;

 

Monday, April 20, 2009

 

*** National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)

 


 

 

Sunday, April 12, 2009

 

r-calf and the USA mad cow problem, don't look, don't find, and then blame Canada

 


 

CHANGE TO ;

 

Sunday, April 12, 2009

 

*** r-calf and the USA mad cow problem, don't look, don't find, and then blame Canada

 


 

 

Saturday, April 11, 2009

 

CJD FOUNDATION SIDES WITH R-CALFERS NO BSE OR HUMAN TSE THERE OF IN USA 'don't be fooled'

 


 

CHANGE TO ;

 

Saturday, April 11, 2009

 

*** CJD FOUNDATION SIDES WITH R-CALFERS NO BSE OR HUMAN TSE THERE OF IN USA 'don't be fooled'

 


 

 

Sunday, April 20, 2008

 

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

 


 

CHANGE TO ;

 

Sunday, April 20, 2008

 

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

 


 

 

 

Terry S. Singeltary Sr.

Sunday, January 19, 2014

National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014

National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014

 

National Prion Disease Pathology Surveillance Center Cases Examined1 (January 8, 2014)

 

Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD

 

1996 & earlier 53 35 30 4 0 0

 

1997 114 68 59 9 0 0

 

1998 88 52 44 7 1 0

 

1999 123 74 65 8 1 0

 

2000 145 103 89 14 0 0

 

2001 212 120 110 10 0 0

 

2002 248 149 126 21 2 0

 

2003 266 168 137 31 0 0

 

2004 326 187 164 22 0 13

 

2005 343 194 157 36 1 0

 

2006 381 194 164 28 0 24

 

2007 377 213 185 28 0 0

 

2008 397 233 208 25 0 0

 

2009 422 256 213 42 1 0 2010 414 258 217 41 0 0

 

2011 411 257 215 42 0 0

 

2012 422 254 217 35 0 0

 

2013 395 246 167 54 1 0

 

TOTAL 51375 30616 2567 457 7 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 10 cases in which the diagnosis is pending, and 19 inconclusive cases;

 

6 Includes 27 (24 from 2013) cases with type determination pending in which the diagnosis of vCJD has been excluded.

 

The sporadic cases include 2500 cases of sporadic Creutzfeldt-Jakob disease (sCJD), 48 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 19 cases of sporadic Fatal Insomnia (sFI).

 


 

 

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

 

> The sporadic cases include 2500 cases of sporadic Creutzfeldt-Jakob disease (sCJD), ***48 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and *** 19 cases of sporadic Fatal Insomnia (sFI).

 

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

 

 

greetings TSE PRION WORLD,

 

i am reminded of a few things deep throat told me years ago;

 

 

*** The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people.........

 

Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie..... why???? than the UK... then would the same mechanisms that make different strains of scrapie here make different strains of BSE... if the patterns are different in sheep and mice for scrapie..... could not the BSE be different in the cattle, in the mink, in the humans....... I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........ bse..... scrapie

 

Scrape the damn slide and put it into mice..... wait..... chop up the mouse brain and and spinal cord........ put into some more mice..... dammit amplify the thing and start the damned research..... This is NOT rocket science... we need to use what we know and get off our butts and move.... the whining about how long everything takes..... well it takes a whole lot longer if you whine for a year and then start the research!!!

 

Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde..... for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year....... it is a big fat sponge... the agent continues to eat the brain ...... you can't make slides anymore because the agent has never stopped........ and the old slides that are stained with Hemolysin and Eosin...... they get holier and holier and degenerate and continue... what you looked at 6 months ago is not there........ Gambetti better be photographing every damned thing he is looking at.....

 

***Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........ if you want to move this thing along and shake the earth.... then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........ I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........ forget any action........ it is ALL gonna be sporadic!!! And, if there is a case....... there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats.... and this may be their biggest downfall...***

 

Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here.......... knocked me out of my chair........ you must keep pushing. If I was a power person.... I would be demanding that there be at least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the wood work as there is money to be made!!!

 

In short: "FIRE AT WILL"!!! for the very dumb.... who's "will"! "Will be the burden to bare if there is any coverup!"

 

again it was said years ago and it should be taken seriously.... BSE will NEVER be found in the US!

 

As for the BSE conference call... I think you did agreat service to freedom of information and making some people feign integrity... I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.

 

You need to watch your back........ but keep picking at them....... like a buzzard to the bone... you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)

 

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

 

 

greetings again,

 

 

then i remind everyone to read this;

 

'As implied in the Inset 25 we must not assume that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.'

 


 

 

see new url ;

 


 

 

*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***

 

Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014

 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

 

 

Wednesday, January 01, 2014

 

Molecular Barriers to Zoonotic Transmission of Prions

 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

 

 


 

 


 

 

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

 


 

 

Thursday, January 2, 2014

 

*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***

 


 

 

From: Terry S. Singeltary Sr.

 

Sent: Friday, January 10, 2014 10:12 PM

 

To: BSE-L BSE-L

 

Cc: journal@neurology.org ; pxg13@case.edu ; CJD-L ; CJDVOICE CJDVOICE ; bloodcjd bloodcjd Subject: vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???

 

Greetings Friends, Neighbors, and Colleagues,

 

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

 

Confucius is confused again.

 

I was just sitting and thinking about why there is no genetic link to some of these TSE prion sGSS, sFFi, and it’s really been working on my brain, and then it hit me today.

 

what if, vpspr, sgss, sffi, TSE prion disease, was a by-product from iatrogenic gss, ffi, familial type prion disease ???

 

it could explain the cases of no genetic link to the gss, ffi, familial type prion disease.

 

sporadic and familial is a red herring, in my opinion, and underestimation is spot on, due to the crude prehistoric diagnostic procedures and criteria and definition of a prion disease.

 

I say again, what if, iatrogenic, what if, with all these neurological disorders, with a common denominator that is increasingly showing up in the picture, called the prion.

 

I urge all scientist to come together here, with this as the utmost of importance about all these neurological disease that are increasingly showing up as a prion mechanism, to put on the front burners, the IATROGENIC aspect and the potential of transmission there from, with diseases/disease??? in question.

 

by definition, could they be a Transmissible Spongiform Encephalopathy TSE prion type disease, and if so, what are the iatrogenic chances of transmission?

 

this is very important, and should be at the forefront of research, and if proven, could be a monumental breakthrough in science and battle against the spreading of these disease/diseases.

 

the US National Library of Medicine National Institutes of Health pub-med site, a quick search of the word SPORADIC will give you a hit of 40,747. of those, there are a plethora of disease listed under sporadic. sporadic simply means (UNKNOWN).

 


 

 

the US National Library of Medicine National Institutes of Health pub-med site, a quick search of the word FAMILIAL will give you a hit of 921,815. of those, there are a plethora of disease listed under familial.

 


 

 

again, sporadic and familial is a red herring, in my opinion.

 

also, in my opinion, when you start have disease such as sporadic Fatal Familial Insomnia, (and or sporadic GSS, or the VPSPr type prion disease), and there is NO familial genetic linkage to the family of the diseased, I have serious questions there as to a familial type disease, and thus, being defined as such.

 

 because there is _NO_ genetic link to that particular family, the theory of a familial disease is out the door, in my opinion. there has to be a source, route, and mode of transmission, if there is no genetic link to the family.

 

SPONTANEOUS HAS NEVER EVER BEEN PROVEN, ESPECIALLY IN 85%+ OF ALL HUMAN TSE PRION CASES, AND THE FOLLOWING STATEMENT HAS NO SCIENTIFIC PROOF.

 

WHAT POLITICAL TSE PRION SCIENCE HAS DONE, IS TAKEN THE WORDS OF THEORY AND HYPOTHESIS FROM SCIENTIST, AND THEM PUT IT IN PRINT, MADE THEM FACT, a terrible unjust to society.

 

*** ''Gambetti, who played a key role in the discovery of fatal insomnia, theorizes that Michael’s illness was random, despite the odds. It may be that, by chance or some unknown factor, Michael’s brain perfectly bred its own pathogen.''

 

I ALSO STRONGLY DISAGREE WITH THE STATEMENT

 

'OR OUTSIDE CAUSE',

 

IF THERE IS NO

 

'WITHOUT AN INHERITED CAUSE',

 

THEN THERE MUST BE AN OUTSIDE CAUSE, AND I PROPOSED, IN SOME CASES, IT BE IATROGENIC.

 

 

VPSPR IS TRANSMISSIBLE

 

 

please see ;

 

Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.

 


 

 

GSS IS TRANSMISSIBLE

 

 

Acta Neuropathol. 1984;64(1):85-8.

 

Experimental transmission of human subacute spongiform encephalopathy to small rodents. IV. Positive transmission from a typical case of Gerstmann-Sträussler-Scheinker's disease.

 

Tateishi J, Sato Y, Nagara H, Boellaard JW.

 

Abstract

 

Spongiform encephalopathy was transmitted to mice from a patient belonging to the "Sch" family with Gerstmann-Sträussler-Scheinker's disease (GSS). Incubation periods in the first passage were much shorter than those in mice infected with Creutzfeldt-Jakob disease. Clinical and pathologic findings of mice infected with both diseases were almost identical. This is the first successful transmission from a typical GSS case without severe spongiform change which suggests the possible transmissible nature of this disorder.

 


 

 

FFI IS TRANSMISSIBLE

 

 

Nature 376, 434 - 435 (03 August 2002); doi:10.1038/376434a0

 

First experimental transmission of fatal familial insomnia

 

Jun Tateishi*, Paul Brown‡, Tetsuyuki Kitamoto*, Zahirul M. Hoque*, Raymond Roos§, Robert Wollman∥, Larisa Cervenàkovà ‡ & D. Carleton Gajdusek‡

 

* Department of Neuropathology, Neurological Institute, Kyushu University, Fukuoka 812, Japan ‡ Laboratory of CMS Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Mayland 20892, USA Departments of Neurology § and Pathology∥, University of Chicago Medical Center, Chicago, Illinois 60637, USA

 

ORIGINALLY described by Lugaresi et al. in 1986 (ref. 1), fatal familial insomnia (FFI) is a rare inherited neurological disease characterized by the subacute progression of intractable insomnia and other autonomic abnormalities, cerebellar and pyramidal signs, myoclonus and dementia; neuropathologically, the major feature is severe neuronal loss with associated gliosis in the ventral and mediodorsal thalamic nuclei. The disease has been related to the group of spongiform encephalopathies by virtue of the presence of low levels of proteinase-resistant amyloid protein (PrPres) in the brain2á¤-4, and of a pathogenic single-allele mutation at codon 178 of the PRNP gene that encodes PrF68 (refs 2, 5). Here we report the successful transmission of the disease to experimental animals, placing FFI within the group of infectious cerebral amyloidoses.

 


 

 

Wednesday, November 09, 2011

 

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

 


 

 

IATROGENIC

 

all iatrogenic cjd is, is sporadic CJD, until route and source of the iatrogenic event that took place, is detected, documented, placed in the academic domain as fact, and recorded, which happens very seldom due to a lot of factors, besides the incubation period, and that be mainly industry. kind of like asbestos and tobacco and the industry there from, they knew in the early 1900’s that they both were killing, and they both had long incubation, and somebody chose not to do anything about if for decades and decades. kind of like what we have here with the TSE prion disease. $$$

 

> In 12 of 15 hospitals with neurosurgical incidents, a decision was made to notify patients of their potential exposure.

 

SO, X number of patients, from 3 hospitals, where

 

''exposure to potentially CJD-contaminated instruments ''

 

took place on these patients, the final decision NOT to tell those folks about the potential exposure to the CJD TSE prion

 

insane, thus, the TSE prion agent continues to spread. ...

 

please see further comments here ;

 

Saturday, November 16, 2013

 

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

 


 

Monday, January 14, 2013

 

Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe

 


 

 

VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion poker goes up again $

 

OR-10 15:25 - 15:40 VARIABLY PROTEASE-SENSITIVE PRIONOPATHY IS TRANSMISSIBLE IN BANK VOLES Nonno

 


 

 

OR-10: Variably protease-sensitive prionopathy is transmissible in bank voles

 

Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1 Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome, Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna, Italy; 3Case Western Reserve University; Cleveland, OH USA

 

Background. Variably protease-sensitive prionopathy (VPSPr) is a recently described “sporadic”neurodegenerative disease involving prion protein aggregation, which has clinical similarities with non-Alzheimer dementias, such as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is the electrophoretic pattern of PrPSc after digestion with proteinase K (PK). After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern similar to that described in GSS cases. The clinical and pathological features of VPSPr raised the question of the correct classification of VPSPr among prion diseases or other forms of neurodegenerative disorders. Here we report preliminary data on the transmissibility and pathological features of VPSPr cases in bank voles.

 

Materials and Methods. Seven VPSPr cases were inoculated in two genetic lines of bank voles, carrying either methionine or isoleucine at codon 109 of the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical diagnosis in voles was confirmed by brain pathological assessment and western blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.

 

Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission in BvM109. Overall, 3 voles were positive with survival time between 290 and 588 d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form of the typical PrP27–30, which was indistinguishable to that previously observed in BvM109 inoculated with sCJDMM1 cases. In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until now. Overall, 5 voles were positive with survival time between 281 and 596 d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern, characterized by low molecular weight PrPres. These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus and the N-terminus. Second passages are in progress from these first successful transmissions.

 

Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109. The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.

 

SOURCE PRION2012

 


 

 

SNIP...

 

SEE FULL TEXT ;

 

 

Friday, January 10, 2014

 

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

 


 

 

Tuesday, October 29, 2013

 

VARIANT CJD PRESENTS DIFFERENTLY IN OLDER PATIENTS

 


 

 

Wednesday, October 09, 2013

 

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

 


 

 

Thursday, October 10, 2013

 

CJD REPORT 1994 increased risk for consumption of veal and venison and lamb

 


 

 

Friday, August 16, 2013

 

*** Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and Contaminated blood products induce a highly atypical prion disease devoid of PrPres in primates

 


 

 

WHAT about the sporadic CJD TSE proteins ?

 

WE now know that some cases of sporadic CJD are linked to atypical BSE and atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all it’s sub-types $$$

 

 

Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013

 

*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010 ***

 


 

 

Sunday, October 13, 2013

 

*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012

 


 

 

PRIONOPATHY OR PRIONOBALONEY $$$

 


 

 

Monday, September 26, 2011

 

Variably Protease-Sensitive Prionopathy, Prionpathy, Prionopathy, FFI, GSS, gCJD, hvCJD, sCJD, TSE, PRION, update 2011

 


 

 

Monday, June 27, 2011

 

Comparison of Sheep Nor98 with Human Variably Protease-Sensitive Prionopathy and Gerstmann-Sträussler-Scheinker Disease

 


 

 

*** vpspr - None of the subjects had mutations in the PrP gene coding region.

 


 

 

*** sporadic FFI - Genetic tests identified no prion protein (PrP) gene mutation

 


 

 

Monday, January 14, 2013

 

snip...

 

Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe

 

Only months later would Michael’s family learn the reason for his degradation and death. An autopsy showed that the teenager died of sporadic fatal insomnia, a subtype of Creutzfeldt-Jakob Disease.

 

In fact, Michael may be among the youngest ever to be affected by a neurodegenerative disease without an inherited or outside cause.

 

Gambetti, who played a key role in the discovery of fatal insomnia, theorizes that Michael’s illness was random, despite the odds. It may be that, by chance or some unknown factor, Michael’s brain perfectly bred its own pathogen.

 

snip...

 


 

 

sporadic GSS -

 

We report here an unusual sporadic case of Creutzfeldt-Jakob disease (CJD) characterized by an abundance of prion protein (PrP)-immunopositive kuru and multicentric but not florid plaques. Molecular genetic analysis of the PRNP open reading frame region spanning codons 8-221 was performed. Neither deletion nor insertion mutations were detected in the repeat area of the PRNP. No pathogenic mutation was found in the sequenced region between codon 108-221. Restriction analysis of the amplified fragment using restriction endonucleases DdeI, PvuII and AluI did not show any of the previously described pathogenic mutations at codon 102, 105, and 117 associated with Gerstmann-Sträussler-Scheinker (GSS). The patient was heterozygous for the methionine/valine coding triplet at polymorphic codon 129 of the PRNP gene by sequence, restriction endonuclease analysis and hybridization with allele-specific nucleotides. Furthermore, hybridization with 32P-labeled allele-specific oligonucleotides confirmed the absence of pathogenic mutations at codons 102, 200 and 178. Such a case may present a missing "link" between sporadic CJD and familial GSS.

 


 

 

Nor98 Prions Share Biochemical Features with Human Gerstmann-Sträussler-Scheinker Disease and Variably Protease-Sensitive Prionopathy

 


 


 

 

Tuesday, August 03, 2010

 

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

 


 

 

Tuesday, August 03, 2010

 

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

 


 

 

Monday, August 9, 2010

 

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?

 

snip...see full text ;

 


 


 

 

Thursday, July 10, 2008

 

A New Prionopathy update July 10, 2008

 


 

 

Thursday, July 10, 2008

 

A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 Friday, June 20, 2008

 


 

 

Sunday, August 10, 2008

 

A New Prionopathy OR more of the same old BSe and sporadic CJD

 


 

 

Sunday, August 24, 2008

 

Sporadic Fatal Insomnia with Unusual Biochemical and Neuropathological Findings

 


 

 

O.K. let's compare some recent cases of this prionpathy in other countries besides Gambetti's first 10 recently, that he claims is a spontaneous event, from a genetic disorder, that is not genetic, but sporadic, that is related to no animal TSE in North America, or the world. ...

 


 


 


 


 

 

Sunday, August 21, 2011

 

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

 

(see video here)

 


 

 

U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ?

 

(see video at bottom)

 


 

 

Sunday, September 6, 2009

 

MAD COW USA 1997

 

(SEE SECRET VIDEO)

 


 

 

Sunday, August 09, 2009

 

***CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009

 


 

 

Tuesday, August 18, 2009

 

BSE-The Untold Story - joe gibbs and singeltary 1999 – 2009 snip... Wed, 29 Nov 2000 14:14:18 -0500

 

a private email from the late Dr. Gibbs, a true pioneer in the research of human/animal TSEs and one that never wavered on helping the families and victims of this horrible disease, and one that helped me many times in trying to seek out the truth;

 

Subject: Re: Hello Dr. Gibbs...........

 

Date: Wed, 29 Nov 2000 14:14:18 –0500

 

From: "Clarence J. Gibbs, Jr., Ph.D."

 

To: "Terry S. Singeltary Sr." References: <3a254430 .9fb97284="" wt.net="">

 

Hi Terry:

 

326 E Stret N.E., Washington, D. C. 20002.

 

Better shrimp and oysters than cards!!!!

 

Have a happy holiday and thanks for all the information you bring to the screen.

 

Joe Gibbs

 

==========

 


 

2011

 

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

 

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

 

Monday, October 10, 2011

 

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

 

snip...

 

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

 

snip...

 


 


 

 

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

 


 

 

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...

 


 


 


 

WHAT about the sporadic CJD TSE proteins ?

 

WE now know that some cases of sporadic CJD are linked to atypical BSE and atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all it’s sub-types $$$

 

 

Monday, June 27, 2011

 

*** Comparison of Sheep Nor98 with Human Variably Protease-Sensitive Prionopathy and Gerstmann-Sträussler-Scheinker Disease

 


 

 

Monday, September 26, 2011

 

L-BSE BASE prion and atypical sporadic CJD

 


 

 

Thursday, August 4, 2011

 

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

 


 

 

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...

 


 

 

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.

 


 

 

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=====

 

 

 

CJD or prion disease 2 CASES McLennan County Texas population 230,213 both cases in their 40s 2 mysterious cases of disease in McLennan County a rarity, but no cause for alarm

 

By Cindy V. Culp Tribune-Herald staff writer

 

 

Friday July 9, 2010

 

Two likely cases of a mysterious, fatal brain disorder have been reported in McLennan County — a statistical anomaly considering that only one in 1 million people worldwide is affected by the condition in any given year.

 

Adding to the peculiarity is that the noncontagious disorder belongs to the same family as Creutzfeldt-Jakob disease.

 

One of its forms is believed to be triggered by people eating meat from cattle infected with mad cow disease.

 

As frightening as that might sound, officials said residents shouldn’t be alarmed.

 

One of the local cases definitely is not the type associated with mad cow disease, and there is no evidence the other one is, either. More importantly, the disorder cannot be transmitted from person to person, officials said.

 

“To have potentially two cases this close together is statistically unusual,” said Dr. Farley Verner, an infectious disease specialist who advises the Waco-McLennan County Public Health District. “But because of the type of disorder it is, and because of what we know about how it develops, it’s not a worrisome coincidence. It’s just a coincidence.”

 

Because of privacy laws, health officials can release only limited details about the local cases. Both were reported in May.

 

The first case involved a 49-year-old man from McGregor, Hammad Akram, the health district’s epidemiologist, said. The man has since died.

 

Initial results from an autopsy show he had some type of human prion disease, a family of diseases involving an abnormal protein.

 

Creutzfeldt-Jakob disease, or CJD, is the most common type of human prion disease. The autopsy ruled out CJD, however, Akram said.

 

The second case involves a Waco woman in her late 40s, Akram said. Her symptoms point to CJD, but since the only way to confirm the disease is to study brain tissue after death, that diagnosis is not confirmed, he said.

 

No apparent link

 

There is no apparent link between the two local victims, Akram said.

 

Prion disease usually occurs in people older than age 60.

 

Doctors give patients a “working diagnosis” of human prion disease based on certain symptoms, combined with results from a blood test, Farley said.

 

The symptoms are similar to those of other neurological conditions: confusion, difficulty remembering recent events, loss of feeling in certain body parts, balance problems, difficulty walking and muscle jerks and spasms.

 

If a physician rules out other causes for such symptoms, a blood test can be done that indicates whether the person has a genetic mutation associated with human prion disease. The test cannot confirm it, but positive results make the diagnosis more likely, Verner said.

 

The name of the disease category comes from a protein called a prion.

 

People have normal prions, which are concentrated in the brain. But in some instances, there is abnormal prion protein, which causes normal prions to be converted to abnormal form.

 

That destroys brain tissue and is eventually fatal. The process can take years, but most people die within three months to a year of having symptoms.

 

There are three main categories of human prion disease — sporadic, familial and acquired.

 

Sporadic cases start spontaneously, without a clearly identifiable cause. They account for about 85 percent of all human prion disease, according to the National Prion Disease Pathology Surveillance Center.

 

Familial cases are inherited and are caused by a defect in the prion protein gene, the center said.

 

Acquired cases are transmitted by infection, which can occur if a person receives a transplant infected with prion disease or undergoes surgery where contaminated instruments are used, according to the center.

 

Another avenue of infection is when someone eats contaminated beef, the center said. That’s where the connection to mad cow disease comes in.

 

Only three cases linked to contaminated beef have been found in the United States, according to health officials. In all three cases, the victims are thought to have been infected while living overseas.

 

In Texas, about 120 people died from human prion disease between 2000-08, according to state data.

 

Last year, there were 19 probable or confirmed cases of sporadic CJD and two familial CJD cases statewide.

 

McLennan County has not had any human prion disease cases in the past decade, according to state records. Verner said he can only recall two or three cases in the 25 years he has been here.

 

cculp@wacotrib.com

 

757-5744

 


 

 

> "It’s just a coincidence.”

 

 


 

 

 r i g h t. $$$

 

cjd = one-in-a-million ???

 

McLennan County, Texas population 2008 230,213

 


 

 

Thursday, July 08, 2010

 

GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010

 


 

 

Thursday, July 08, 2010

 

Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions Public release date: 8-Jul-2010

 


 

 

Wednesday, June 16, 2010

 

Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties

 


 

 

CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

 

 

>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<

 

 


 

 

>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<

 

 


 

 

CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

 


 

 

*** re-mad cow disease, the cow that stole Christmas December 23, 2003 10 years later, USA still feeding cows to cows in 2013.

 

 

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE

 

OAI 2012-2013

 

OAI (Official Action Indicated) when inspectors find significant objectionable conditions or practices and believe that regulatory sanctions are warranted to address the establishment’s lack of compliance with the regulation. An example of an OAI classification would be findings of manufacturing procedures insufficient to ensure that ruminant feed is not contaminated with prohibited material. Inspectors will promptly re-inspect facilities classified OAI after regulatory sanctions have been applied to determine whether the corrective actions are adequate to address the objectionable conditions.

 

 ATL-DO 1035703 Newberry Feed & Farm Ctr, Inc. 2431 Vincent St. Newberry SC 29108-0714 OPR DR, FL, FR, TH HP 9/9/2013 OAI Y

 

DET-DO 1824979 Hubbard Feeds, Inc. 135 Main, P.O. Box 156 Shipshewana IN 46565-0156 OPR DR, FL, OF DP 8/29/2013 OAI Y

 

ATL-DO 3001460882 Talley Farms Feed Mill Inc 6309 Talley Rd Stanfield NC 28163-7617 OPR FL, TH NP 7/17/2013 OAI N

 

NYK-DO 3010260624 Sherry Sammons 612 Stoner Trail Rd Fonda NY 12068-5007 OPR FR, OF NP 7/16/2013 OAI Y

 

DEN-DO 3008575486 Rocky Ford Pet Foods 21693 Highway 50 East Rocky Ford CO 81067 OPR RE, TH HP 2/27/2013 OAI N

 

CHI-DO 3007091297 Rancho Cantera 2866 N Sunnyside Rd Kent IL 61044-9605 OPR FR, OF HP 11/26/2012 OAI Y

 

*** DEN-DO 1713202 Weld County Bi Products, Inc. 1138 N 11th Ave Greeley CO 80631-9501 OPR RE, TH HP 10/12/2012 OAI N

 

Ruminant Feed Inspections Firms Inventory (excel format)

 


 

 

Sunday, December 15, 2013

 

*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2013 UPDATE

 


 

 

However, a BSE expert said that consumption of infected material is the only known way that cattle get the disease under natural conditons.

 

 

*** “In view of what we know about BSE after almost 20 years experience, contaminated feed has been the source of the epidemic,” said Paul Brown, a scientist retired from the National Institute of Neurological Diseases and Stroke. BSE is not caused by a microbe. It is caused by the misfolding of the so-called “prion protein” that is a normal constituent of brain and other tissues. If a diseased version of the protein enters the brain somehow, it can slowly cause all the normal versions to become misfolded. It is possible the disease could arise spontaneously, though such an event has never been recorded, Brown said.

 


 

 

*** 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.”

 


 

 

2012 ATYPICAL L-TYPE BSE BASE CALIFORNIA ‘confirmed’ Saturday, August 4, 2012

 

*** Final Feed Investigation Summary - California BSE Case - July 2012

 


 

 

Saturday, August 14, 2010

 

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

 

(see mad cow feed in COMMERCE IN ALABAMA...TSS)

 


 

 

Friday, January 17, 2014

 

Annual report of the Scientific Network on BSE-TSE EFSA, Question No EFSA-Q-2013-01004, approved on 11 December 2013

 


 

 

Saturday, December 21, 2013

 

**** Complementary studies detecting classical bovine spongiform encephalopathy infectivity in jejunum, ileum and ileocaecal junction in incubating cattle ****

 


 

 

Wednesday, December 4, 2013

 

*** Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products; Final Rule Federal Register / Vol. 78 , No. 233 / Wednesday, December 4, 2013

 


 

 

Saturday, November 2, 2013

 

*** APHIS Finalizes Bovine Import Regulations in Line with International Animal Health Standards while enhancing the spread of BSE TSE prion mad cow type disease around the Globe

 


 


 

 

*** Saturday, November 2, 2013 ***

 

Exploring the risks of a putative transmission of BSE to new species

 


 

 

Friday, January 17, 2014

 

*** Annual report of the Scientific Network on BSE-TSE EFSA, Question No EFSA-Q-2013-01004, approved on 11 December 2013

 

TECHNICAL REPORT

 


 

 

Monday, December 02, 2013

 

*** A parliamentary inquiry has been launched today into the safety of blood, tissue and organ screening following fears that vCJD – the human form of ‘mad cow’ disease – may be being spread by medical procedures

 


 

 

Wednesday, December 11, 2013

 

*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease

 


 

 

Thursday, January 2, 2014

 

*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***

 


 

 

Friday, January 10, 2014

 

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

 


 

 

Wednesday, January 15, 2014

 

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

 


 

 

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

 

[host Richard Barns] and now a question from Terry S. Singeltary of CJD Watch.

 

[TSS] yes, thank you, U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?

 

[no answer, you could hear in the back ground, mumbling and 'we can't. have him ask the question again.]

 

[host Richard] could you repeat the question?

 

[TSS] U.S. cattle, what kind of guarantee can you give for serum or tissue donor herds?

 

[not sure whom ask this] what group are you with?

 

[TSS] CJD Watch, my Mom died from hvCJD and we are tracking CJD world-wide.

 

[not sure who is speaking] could you please disconnect Mr. Singeltary

 

 

SNIP...SEE FULL TEXT ;

 

 


 

 

Saturday, March 5, 2011

 

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

 


 

 

Thursday, February 21, 2013

 

National Prion Disease Pathology Surveillance Center Cases Examined January 16, 2013

 


 

 

Sunday, February 12, 2012

 

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

 


 

 

CJD QUESTIONNAIRE

 


 


 

 

CJD VOICE

 


 


 


 


 


 


 


 


 


 


 


 

 

TSS