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

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
 
 
 
Published: Jan 13, 2013 05:36 PM Modified: Jan 13, 2013 05:38 PM
 
 
 
At last, answers to a mother’s grief Cary teen died of extremely rare disease
 
 
 
By Andrew Kenney, akenney@newsobserver.com
 
 
 
By Andrew Kenney Cary News
 
 
 
It wasn’t bacteria or a virus that plagued young Michael Mendy’s body and mind. He did not inherit his symptoms from his mother or father. Nothing toxic was in his blood. And while he was sick, Michael’s parents had no explanation.
 
 
 
“I had to figure it out. I had to find an answer. I had to find a doctor that could help him,” said Michael’s mother, Kathleen Mendy, who lives in western Cary. “You don’t think you’re going to come across something that nobody’s ever seen.”
 
 
 
But they had. Dozens of specialists and three years of suffering brought no diagnosis. Michael died a year ago, at age 16. And only then did the explanation and the terrible significance of his case emerge.
 
 
 
Doctors estimate that fewer than one in 100 million young people will share Michael’s journey. The sports nut and East Cary Middle School student was killed by a disease that mostly afflicts the elderly.
 
 
 
Michael was a heart-wrenching outlier, apparently among the youngest ever to suffer a spontaneous ravaging of the brain.
 
 
 
The onset
 
 
 
Kathleen Mendy thinks it started on Michael’s 13th birthday in 2009.
 
 
 
On that January day, another boy knocked Michael’s head to the court during a basketball game. He was playing again a minute later, his mother watching with a tinge of worry.
 
 
 
The symptoms of a years-long illness crept in that weekend, during a mother-son Super Bowl trip to Florida. That’s where she first saw Michael’s confusion, his unsure movements and his inexplicable crying.
 
 
 
It seemed at first like the troubling wake of the teen’s second concussion in three months, but that theory would erode and change. Across the next three years, in a nightmare that kept unfolding, the brawny teenager would drop almost half his body weight, spend months in the hospital, lose his speech and lay debilitated by simple infections.
 
 
 
The printed record of Michael’s hospital visits and test appointments is three and a half pages long. It documents an increasingly desperate search, listing 140 days in the hospital, 91 visits with doctors and 426 therapy appointments from 2009 to 2012.
 
 
 
“I always thought he would get better,” Kathleen Mendy said. “I used to always tell him, ‘Michael, one day you’re just going to run out of your bedroom, and you’re going to come running downstairs, and you’re going to be all better.’ ”
 
 
 
The turning point wouldn’t come.
 
 
 
While his friends went on to high school, Michael was confined to a wheelchair and fed through a tube. His care grew so intense that his mother brought on a full-time medical aide.
 
 
 
His father, divorced from his mother, drove in from Florida each time he entered the hospital, and Kathleen Mendy’s family often visited from New York.
 
 
 
Each treatment was more esoteric than the last. By the end of 2011, the Mendys had seen more than 30 doctors, medical specialists, faith healers and alternative practitioners.
 
 
 
“I tried chiropractors, reflexology, myofacial therapy,” Kathleen Mendy said. “I tried everything.”
 
 
 
‘I’m so glad I didn’t know’
 
 
 
The realization came to Kathleen Mendy on the last night of January 2012, the 22nd day that Michael spent in a UNC hospital bed.
 
 
 
He’d been kept alive in an intensive-care unit by a breathing machine while an infection took hold of his lungs. The mysterious disorder had left his body unable to respond.
 
 
 
The memory shakes Kathleen Mendy to tears. The scene sticks in her mind.
 
 
 
“Not until the night before he died, is when, honestly, it hit me,” she recalled.
 
 
 
The doctors laid a choice before his parents that night. Michael could go home with a tracheotomy and a ventilator, but they believed he’d live just a few months longer. Or doctors could remove him from life support.
 
 
 
Michael’s parents didn’t want him to suffer anymore. He died on Feb. 1, 2012.
 
 
 
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.
 
 
 
“I’m so glad I didn’t know what it was” before Michael died, Kathleen Mendy said. “Because then I wouldn’t have had hope.”
 
 
 
‘A descent into hell’
 
 
 
“This disease is a descent into hell,” said Florence Kranitz, president of The Creutzfeldt-Jakob Disease Foundation in New York City.
 
 
 
She saw her own husband die in 2001 of an ailment similar to Michael’s. Since then she has heard the stories of many of the 300 CJD victims her organization identifies each year, including cases of fatal insomnia.
 
 
 
“We get this phone call, and tragically it’s the same phone call over and over again,” Kranitz said. “They’ve never heard of this disease.”
 
 
 
The story she heard from Kathleen Mendy fit the profile, with one beguiling exception. Almost everyone afflicted by CJD subtypes are older than 45, except those who contract a variant of the disease genetically or through contaminated beef, which Michael had not.
 
 
 
Michael’s case quickly drew the attention of national experts, including Pierluigi Gambetti, director of the National Prion Disease Pathology Surveillance Center in Ohio. Gambetti, a pioneering researcher, examined Michael’s brain and in April identified his disease as sporadic fatal insomnia.
 
 
 
He’d later take hours to talk with Kathleen Mendy about her son’s death. Sporadic fatal insomnia and CJD, he explained, are part of the still-mysterious field of neurodegenerative diseases, including Alzheimer’s and Parkinson’s.
 
 
 
Some of these ravages of the brain, such as so-called “Mad Cow,” begin with an infection of prions, or pathogenic proteins, from the outside. CJD in the young also can be caused by prions, often transmitted during surgery.
 
 
 
Like a virus, a prion can essentially “breed.” The virus hijacks human cells, and the prion reshapes other proteins into its own mutated form. And when a prion or virus propagates enough, it can destroy its host.
 
 
 
But there’s a crucial difference: The prion also can come from within. Gambetti believes that Michael’s disease began when the boy’s brain misfolded a protein, creating a prion instead. The defect may then have multiplied out of control and ruined the delicate balance of the body.
 
 
 
It’s not uncommon for the body to make mistakes. Neurons and other cells normally catch and eliminate prions before they replicate. These defensive systems may grow weaker with age; some people may also inherit weaker defenses.
 
 
 
But Michael was a teenager, with no apparent family history of neurodegenerative diseases. Gambetti put the odds of such a case at one in 100 million in the general population; another doctor said it was one in 600 million.
 
 
 
In fact, Michael may be among the youngest ever to be affected by a neurodegenerative disease without an inherited or outside cause.
 
 
 
“We just haven’t seen this disease affect someone this young,” said John Trojanowski, a professor of geriatric medicine at the University of Pennsylvania.
 
 
 
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.
 
 
 
“The bodies of all animals are a marvel of things, in positive and negative,” Gambetti said. “They can do things striking for the good, but also for the bad.”
 
 
 
Looking to others
 
 
 
Nearly a year after Michael’s death, Kathleen Mendy finds love and support from family, friends and Compassionate Friends, a local group. But the extreme rarity of Michael’s case is isolating.
 
 
 
When she attended a CJD conference last summer with her twin sister, they met the families of people who had mostly died in middle and old age.
 
 
 
Some nights she goes up to her only child’s room. It’s lined with dozens of sports team caps and trophies. Athletes’ names are still painted on the blades of his ceiling fan, and the UNC comforter is still on his bed.
 
 
 
All that’s new is the shrine on the desk, where Michael’s photo stands near a glazed statue of praying hands.
 
 
 
“Sometimes I think I’m OK, and other times it’s like it just happened last night. It’s like a rollercoaster,” Kathleen Mendy said.
 
 
 
She may have as many logical answers now as she’ll ever get – a medical, if not a spiritual description of why Michael died.
 
 
 
She still doesn’t know what it was that made her son vulnerable. She believes Michael’s concussions triggered his illness, but his doctors haven’t confirmed the idea.
 
 
 
“I’m a little bit resolved that I’ll never hear the answer,” she said. “It would be nice to know, but if I don’t know it, it’s not what matters now.”
 
 
 
She finds hope instead in the idea that she could help others; she’s thinking of writing a book and becoming a public advocate for those who suffer with CJD.
 
 
Meanwhile, as Michael’s birthday and the first anniversary of his death approach, Gambetti and one of Michael’s former doctors are preparing to present his case to their respective medical communities. As painful as the case is, “its rarity may contribute to expand the knowledge on this terrible disease,” according to Gambetti.
 
 
 
He hopes his research will one day allow much earlier diagnosis and treatment of fatal insomnia. Such a breakthrough could key medical progress across the spectrum of prion-related diseases, which are fatal in practically all cases.
 
 
 
Gambetti’s research into Michael’s case will soon yield a more immediate result too: He’ll be able to tell caregivers that sporadic fatal insomnia can strike not just the mature, but perhaps people who are just beginning their lives.

 
And with Michael’s story spreading, the next stricken family may at least know the harrowing path ahead.
 
 
 
Kenney: 919-460-2608 or twitter.com/KenneyOnCary
 
 
 
 
 
 
 
 
sporadic FFI, really $$$

 
HOW can a genetic disease, one NOT tied to any family member, how can this be a genetic disease ?
 
 
 
easy, they simply change the name to another stupid name of the same damn disease.
 
 
 
Gambetti et al must be getting paid very well for all this junk science on CJD and human TSE in the USA $$$
 
 
 
 
 
Wednesday, November 09, 2011
 
 
 
Case report Sporadic fatal insomnia in a young woman: A diagnostic challenge: Case Report TEXAS
 
 
 
HOW TO TURN A POTENTIAL MAD COW VICTIM IN THE USA, INTO A HAPPENSTANCE OF BAD LUCK, A SPONTANEOUS MUTATION FROM NOTHING.
 
 
 
OR WAS IT $$$
 
 
 
 
 
 
snip...
 
 
 
AND THAT MY FRIENDS, IS HOW YOU EXPLAIN SOMETHING AWAY INTO NOTHING. IT'S THE USDA ET AL MAD COW WAY $$$
 
 
 
how many times have we seen this happen? time and time again.
 
 
 
sporadic FFI or nvCJD Texas style ???
 
 
 
DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)
 
 
 
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....
 
 
 
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!
 
 
 
And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...
 
 
 
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"
 
 
 
again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
 
 
 
You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
 
 
 
END...TSS
 
 
 
 
snip...see full text ;
 
 
 
 
 
 
 
U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001
 
 
 
Subject: BSE--U.S. 50 STATE CONFERENCE CALL Jan. 9, 2001
 
 
 
Date: Tue, 9 Jan 2001 16:49:00 –0800
 
 
 
From: "Terry S. Singeltary Sr."
 
 
 
Reply-To: Bovine Spongiform Encephalopathy
 
 
 
 
 
 
######### Bovine Spongiform Encephalopathy #########
 
 
 
 
 
 
 
Here we go folks. AS predicted. THIS JUST OUT ! as i predicted, more BSe. $$$
 
 
 
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 ;
 
 
 
 
 
 
 
 
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 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
 
 
 
 
 
 
 
 
PPS POLITICAL PRION SCIENCE $$$
 
 
 
 
Creutzfeldt-Jakob Disease Surveillance in Texas
 
 
 
 
 
 
 
 
Sunday, July 11, 2010

 
CJD or prion disease 2 CASES McLennan County Texas population 230,213 both cases in their 40s
 
 
 
 
 
 
 
 
 
 
see the continuing rise of sporadic CJD in Texas here ;
 
 
 
 
 
 
 
 
 
Saturday, March 5, 2011
 
 
 
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA

 
 
 
 
 
 
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)

 
 
 
 
 
 
Thursday, August 4, 2011
 
 
 
Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis, Date aired: 27 Jun 2011
 
 
 
 
 
 
 
Monday, December 31, 2012
 
 
 
Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012
 
 
 
 
 
 
 
 
Tuesday, December 25, 2012
 
 
 
CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25, 2012

 
 
 
 
 
 
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’

 
 
 
 
 
 
Friday, November 23, 2012

 
sporadic Creutzfeldt-Jakob Disease update As at 5th November 2012 UK, USA, AND CANADA
 
 
 
 
 
 
 
 
Saturday, January 05, 2013
 
 
 
Immunohistochemical Detection of Disease- Associated Prion Protein in the Peripheral Nervous System in Experimental H-Type Bovine Spongiform Encephalopathy
 
 
 
 
 
 
 
 
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...
 
 
 
 
 
 
 
 
 
 
 
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...
 
 
 
 
 
 
 
IT is of my opinion, that the OIE and the USDA et al, are the soul reason, and responsible parties, for Transmissible Spongiform Encephalopathy TSE prion diseases, including typical and atypical BSE, typical and atypical Scrapie, and all strains of CWD, and human TSE there from, spreading around the globe.
 
 
 
I have lost all confidence of this organization as a regulatory authority on animal disease, and consider it nothing more than a National Trading Brokerage for all strains of animal TSE, just to satisfy there commodity. AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization.
 
 
 
JUST because of low documented human body count with nvCJD and the long incubation periods, the lack of sound science being replaced by political and corporate science in relations with the fact that science has now linked some sporadic CJD with atypical BSE and atypical scrapie, and the very real threat of CWD being zoonosis, I believed the O.I.E. has failed terribly and again, I call for this organization to be dissolved. ...
 
 
 
 
 
Tuesday, July 17, 2012

 
O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th General Session, 20 - 25 May 2012

 
 
 
 
 
 
Saturday, October 6, 2012
 
 
 
TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES 2011 Annual Report
 
 
 
 
 
 
 
 
re-Human Prion Diseases in the United States Posted by flounder on 01 Jan 2010 at 18:11 GMT
 
 
 
I kindly disagree with your synopsis for the following reasons ;

 
 
 
 
 
 
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.
 
 
 
 
 
 
 
 
Views & Reviews
 
 
 
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States
 
 
 
Ermias D. Belay, MD, Ryan A. Maddox, MPH, Pierluigi Gambetti, MD and Lawrence B. Schonberger, MD
 
 
 
+ Author Affiliations
 
 
 
From the Division of Viral and Rickettsial Diseases (Drs. Belay and Schonberger and R.A. Maddox), National Center for Infectious Diseases, Centers for Disease Control and Prevention, Atlanta, GA; and National Prion Disease Pathology Surveillance Center (Dr. Gambetti), Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, OH.

 
Address correspondence and reprint requests to Dr. Ermias D. Belay, 1600 Clifton Road, Mailstop A-39, Atlanta, GA 30333.
 
 
 
 
 
 
 
 
26 March 2003
 
 
 
Terry S. Singeltary, retired (medically) CJD WATCH

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



JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

 
 
 
Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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

 
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
 
 
 
 
 
 
 
2 January 2000
 
 
 
British Medical Journal
 
 
 
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

 
 
 
 
 
15 November 1999
 
 
 
British Medical Journal
 
 
 
vCJD in the USA * BSE in U.S.
 
 
 
 
 
 
 
 
 
Saturday, January 2, 2010
 
 
 
Human Prion Diseases in the United States January 1, 2010 ***FINAL***

 
 
 
 
 
14th ICID International Scientific Exchange Brochure -
 
 
 
Final Abstract Number: ISE.114
 
 
 
Session: International Scientific Exchange

 
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
 
 
 
T. Singeltary
 
 
 
Bacliff, TX, USA
 
 
 
Background:

 
An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
 
 
 
Methods:
 
 
 
12 years independent research of available data
 
 
 
Results:
 
 
 
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.


 
Conclusion:
 
 
 
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
 
 
 
 
 
 
 
 
 
re-Human Prion Diseases in the United States Posted by flounder on 01 Jan 2010 at 18:11 GMT
 
 
 
I kindly disagree with your synopsis for the following reasons ;
 
 
 
 
 
 
 
Wednesday, May 16, 2012

 
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?
 
 
 
Proposal ID: 29403

 
 
 
 
 
 
Monday, August 20, 2012

 
CASE REPORTS CREUTZFELDT-JAKOB DISEASE: AN UNDER-RECOGNIZED CAUSE OF DEMENTIA
 
 
 
 
 
 
 
 
Friday, October 05, 2012
 
 
 
Differential Diagnosis of Jakob-Creutzfeldt Disease
 
 
 
 
 
 
 
 
see the Duke, Pa, Yale, and Mexican study here, showing the misdiagnosis of CJD TSE prion disease as Alzheimers ;
 
 
 
 
 
 
 
 
Monday, July 23, 2012
 
 
 
The National Prion Disease Pathology Surveillance Center July 2012
 
 
 
 
 
 
 
 
TSS
layperson mom dod 12/14/97 confirmed hvCJD...

Saturday, January 12, 2013

Exposure of RML scrapie agent to a sodium percarbonate-based product and sodium dodecyl sulfate renders PrPSc protease sensitive but does not eliminate infectivity

Exposure of RML scrapie agent to a sodium percarbonate-based product and sodium dodecyl sulfate renders PrPSc protease sensitive but does not eliminate infectivity


BMC Veterinary Research 2013, 9:8 doi:10.1186/1746-6148-9-8 Jodi D Smith (jodi.smith@ars.usda.gov) Eric M Nicholson (eric.nicholson@ars.usda.gov) Gregory H Foster (ghfoster@mac.com) Justin J Greenlee (justin.greenlee@ars.usda.gov) ISSN 1746-6148


Article type Research article Submission date 18 September 2012 Acceptance date 8 January 2013 Publication date 11 January 2013 Article URL http://www.biomedcentral.com/1746-6148/9/8


BMC Veterinary Research


© 2013 Smith et al.



Exposure of RML scrapie agent to a sodium percarbonate-based product and sodium dodecyl sulfate renders PrPSc protease sensitive but does not eliminate infectivity



Jodi D Smith1 Email: jodi.smith@ars.usda.gov Eric M Nicholson1 Email: eric.nicholson@ars.usda.gov Gregory H Foster1 Email: ghfoster@mac.com Justin J Greenlee1* * Corresponding author Email: justin.greenlee@ars.usda.gov


1 Virus and Prion Research Unit, National Animal Disease Center, USDA, Agricultural Research Service, 1920 Dayton Ave, Ames, IA 50010, USA



Abstract



Background



Prions, the causative agents of the transmissible spongiform encephalopathies, are notoriously difficult to inactivate. Current decontamination recommendations by the World Health Organization include prolonged exposure to 1 N sodium hydroxide or > 20,000 ppm sodium hypochlorite, or autoclaving. For decontamination of large stainless steel surfaces and equipment as in abattoirs, for example, these methods are harsh or unsuitable. The current study was designed to evaluate the effectiveness of a commercial product containing sodium percarbonate to inactivate prions. Samples of mouse brain infected with a mouse-adapted strain of the scrapie agent (RML) were exposed to a sodium percarbonate-based product (SPC-P). Treated samples were evaluated for abnormal prion protein (PrPSc)- immunoreactivity by western blot analysis, and residual infectivity by mouse bioassay.



Results



Exposure to a 21% solution of SPC-P or a solution containing either 2.1% or 21% SPC-P in combination with sodium dodecyl sulfate (SDS) resulted in increased proteinase K sensitivity of PrPSc. Limited reductions in infectivity were observed depending on treatment condition. A marginal effect on infectivity was observed with SPC-P alone, but an approximate 2–3 log10 reduction was observed with the addition of SDS, though exposure to SDS alone resulted in an approximate 2 log10 reduction.



Conclusions



This study demonstrates that exposure of a mouse-adapted scrapie strain to SPC-P does not eliminate infectivity, but does render PrPSc protease sensitive.



Keywords Inactivation, Prion, Scrapie, Sodium dodecyl sulfate, Sodium percarbonate



SNIP...



A major finding of this study was the increased sensitivity of PrPSc to PK by the SPC-based product without (SPC-PH only) or with SDS at room temperature, as judged by immunoblotting after exposure of the samples to limited proteolysis. Based on the loss of detectable PrPSc immunoreactivity after incubation at pH 11, it appears this effect may be largely pH-dependent. It is well established that prion infectivity is reduced under extremely basic conditions, such as exposure to NaOH (pH 12–14) [19-21]. While the pH generated by SPC-P is lower at 11, it appears to be a favorable characteristic of the compound with regard to PrPSc protease sensitivity. However, a solely pH-dependent effect does not explain why SPC-PL treatment alone (pH 11) did not yield similar WB results. One possible explanation is that a lower concentration of the product may have contained diminished buffering capacity resulting in a drop in pH as treatment proceeded, but serial pH evaluation of treated brain homogenate at 30, 90, and 180 min revealed that the pH remained above 10.7. Although treatment with the SPC product did render PrPSc sensitive to digestion by proteinase K, it did not eliminate infectivity. Recent studies examining prion infectivity in infected tissue and cell cultures have also demonstrated loss of detectable PrPSc on western blot, but residual infectivity [22,23]. Our results support the inference that biochemical analysis alone is insufficient for determination of prion infectivity. The observed PrPSc/infectivity mismatch in this study and in others warrants a number of considerations including WB sensitivity, epitope disruption by inactivation treatments, and alternative infectious agents to PrPSc, such as PK-sensitive forms of PrP or viruses. It is possible the amount of residual PrPSc in our treated samples was below the detection limit of our WB (0.025 mg equivalents of brain tissue for this particular inoculum [24]), or it may be that a true dissociation of PrPSc and TSE infectivity exists supporting the actuality of alternative infectious agents to PrPSc [25]. A recent study has demonstrated poor correlation between infectivity and WB results for sheep scrapie and sheep BSE [26] in line with observations that PK-sensitive PrP particles are associated with disease [27,28].



The bioassay results we present indicate that exposure to the selected SPC-based product alone or in combination with 2.5% SDS is not a viable option for the inactivation of prions. No decrease in infectivity was observed using the SPC-PL solution alone, and a modest 1 log10 reduction was achieved with the SPC-PH solution. However, recent investigations have demonstrated differential susceptibility of distinct prion strains to the same inactivation procedure [29]; therefore, we are currently investigating the efficacy of these treatment conditions in an ovine scrapie model. It should also be acknowledged that chemical treatment of the scrapie agent has been shown to delay the dose–response relationship [30,31] resulting in prolonged incubation times without a change in calculable titer. It is possible our results could be reflecting this phenomenon, but without bioassay data from serial dilutions of treated brain homogenate this cannot be definitively determined. Some caution may therefore be warranted when interpreting these results. The addition of 2.5% SDS to the SPC-P solutions resulted in a 2–3 log10 reduction in infectivity, but exposure to SDS alone resulted in an approximate 2 log10 reduction. This suggests much of the observed combinatorial effect was due to SDS. Prior studies using SDS have demonstrated minimal effects on CJD infectivity [16], but up to a 3 log10 reduction on scrapie infectivity [17]. Exposure of hamster-adapted Sc237 scrapie to room temperature SDS at pH values of ≤4.5 or ≥10 resulted in increased PK sensitivity of PrPSc, and exposure to acidic SDS resulted in decreased infectivity [11]. Since SDS at room temperature is an effective denaturant at a pH ≥10, this could have contributed to the loss of detectable PrPScimmunoreactivity we observed after proteolysis in samples treated with SPC-P and SDS. There was also enhanced reduction in infectivity with the combination of SPC-PL and SDS. This may be indicative of an enhanced effect of SDS under basic conditions or a two-step mechanism whereby denaturation of PrPSc by the relatively high pH of the solution and/or SDS is followed by exposure of sites sensitive to oxidative damage. Alternatively, the two treatment components could be acting on different PrPSc fractions in the inoculum resulting in an additive effect since the combination of SPC-PL and SDS was roughly equivalent to slightly greater than the sum of the effects of each individual component. The combination of SPC-PH and SDS did not provide an equivalent or better increase in survival time than the combination of SPC-PL and SDS. While we are confident in this result, we cannot definitively explain this observation. Perhaps disease in this group was exacerbated by oxidative damage induced by the introduction of treated brain samples containing a greater concentration of sodium percarbonate. Oxidative stress, whether a cause or consequence of disease progression, is considered an important contributor to prion neuropathology [32-34]. It is also possible that the SPC-P solution at higher concentration may somehow be interfering with the denaturing action of SDS. SDS action may be enhanced when combined with lower concentrations of SPC-P for longer exposure times, but restricted by higher concentrations, perhaps via chemical modification of SDS binding sites on the protein.



Oxidizing agents have been used with variable success in prion inactivation studies. Exposure of prions to halogens such as sodium hypochlorite at ≥ 20,000 ppm is an accepted means of decontamination [8], but chlorine dioxide is much less effective at inactivating hamster-adapted 263 K scrapie [35]. Peroxygens such as liquid hydrogen peroxide [13,35,36] and peracetic acid [37] also promote limited inactivation. However, recent studies using vaporized hydrogen peroxide to decontaminate stainless steel surfaces have demonstrated significant reductions in infectivity for hamster-adapted 263 K scrapie and mouse-adapted BSE [13,15]. A protective effect from oxidation by peracetic acid has been demonstrated with the ME7 scrapie agent and attributed to prion aggregation [37]. Peracetic acid at 2% was effective at inactivating the ME7 scrapie agent in intact brain tissue, but not homogenized tissue. Samples in the current study were homogenized, which may have imparted a degree of protection from oxidation and contributed to the ineffectiveness of SPC-P alone at decreasing infectivity. We propose that the addition of SDS would have decreased aggregation of cell membranes to which infectivity is bound, thus enhancing the activity of SPC-P and perhaps contributing to the increased survival observed with the combination.



Conclusions



This study demonstrates that exposure of the RML scrapie agent to an SPC-containing product alone or in combination with SDS does not eliminate prion infectivity, but does render PrPSc sensitive to proteinase K. Because of this, it is interesting to consider the potential viability of a combination of SPC and SDS, even at relatively low concentrations and mild temperatures, concomitant with or followed by a protease for prion decontamination. Also, because the SPC product we used contains additional proprietary ingredients, we cannot rule-out contributions to increased PK-sensitivity or increased survival by other components of the product. Studies in our laboratory are currently underway examining exposure of prions to chemical grade SPC with or without SDS followed by exposure to a protease.










Tuesday, December 25, 2012


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






remember, all iatrogenic CJD is, is sporadic CJD until route and source is confirmed for iatrogenic transmission. ...




Tuesday, December 18, 2012


Bioassay Studies Support the Potential for Iatrogenic Transmission of Variant Creutzfeldt Jakob Disease through Dental Procedures







Thursday, October 25, 2012



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



Article in Press










Friday, August 24, 2012



Iatrogenic prion diseases in humans: an update










Monday, August 13, 2012



Summary results of the second national survey of abnormal prion prevalence in archived appendix specimens August 2012










Friday, August 10, 2012



Incidents of Potential iatrogenic Creutzfeldt-Jakob disease (CJD) biannual update (July 2012)










Friday, June 29, 2012



Highly Efficient Prion Transmission by Blood Transfusion









Thursday, May 17, 2012



Iatrogenic Creutzfeldt-Jakob Disease, Final Assessment



Volume 18, Number 6—June 2012









Tuesday, December 25, 2012



CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25, 2012









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









Sunday, December 2, 2012



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










Monday, December 31, 2012



Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012









Tuesday, November 6, 2012



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








Saturday, January 05, 2013



Immunohistochemical Detection of Disease- Associated Prion Protein in the Peripheral Nervous System in Experimental H-Type Bovine Spongiform Encephalopathy








2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006








Comments on technical aspects of the risk assessment were then submitted to FSIS.




Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.



This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:









Owens, Julie



From: Terry S. Singeltary Sr. [flounder9@verizon.net]



Sent: Monday, July 24, 2006 1:09 PM



To: FSIS RegulationsComments



Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)



Page 1 of 98









FSIS, USDA, REPLY TO SINGELTARY










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












2012 atypical L-type BSE BASE California reports




Saturday, August 4, 2012



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








SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012



Summary Report BSE 2012



Executive Summary








Saturday, August 4, 2012



Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation










Thursday, March 29, 2012



atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012



NIAA Annual Conference April 11-14, 2011San Antonio, Texas








Sunday, April 18, 2010



SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010









Wednesday, February 16, 2011



IN CONFIDENCE



SCRAPIE TRANSMISSION TO CHIMPANZEES IN CONFIDENCE








Tuesday, November 02, 2010



IN CONFIDENCE



The information contained herein should not be disseminated further except on the basis of "NEED TO KNOW".



BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992















Wednesday, May 16, 2012



Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?



Proposal ID: 29403











TSS

Tuesday, December 25, 2012

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

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


Amie Adkin1,*, Neil Donaldson1, Louise Kelly1,2Article first published online: 24 DEC 2012


DOI: 10.1111/j.1539-6924.2012.01922.x


© 2012 Society for Risk Analysis




Keywords:Abattoir;bovine spongiform encephalopathy;QRA;scrapie;TSE


In this article the development and parameterization of a quantitative assessment is described that estimates the amount of TSE infectivity that is present in a whole animal carcass (bovine spongiform encephalopathy [BSE] for cattle and classical/atypical scrapie for sheep and lambs) and the amounts that subsequently fall to the floor during processing at facilities that handle specified risk material (SRM). BSE in cattle was found to contain the most oral doses, with a mean of 9864 BO ID50s (310, 38840) in a whole carcass compared to a mean of 1851 OO ID50s (600, 4070) and 614 OO ID50s (155, 1509) for a sheep infected with classical and atypical scrapie, respectively. Lambs contained the least infectivity with a mean of 251 OO ID50s (83, 548) for classical scrapie and 1 OO ID50s (0.2, 2) for atypical scrapie. The highest amounts of infectivity falling to the floor and entering the drains from slaughtering a whole carcass at SRM facilities were found to be from cattle infected with BSE at rendering and large incineration facilities with 7.4 BO ID50s (0.1, 29), intermediate plants and small incinerators with a mean of 4.5 BO ID50s (0.1, 18), and collection centers, 3.6 BO ID50s (0.1, 14). The lowest amounts entering drains are from lambs infected with classical and atypical scrapie at intermediate plants and atypical scrapie at collection centers with a mean of 3 × 10−7 OO ID50s (2 × 10−8, 1 × 10−6) per carcass. The results of this model provide key inputs for the model in the companion paper published here.









it would have been interesting if CWD would have been included in this study. ...tss





Saturday, December 15, 2012



Bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle -- an update 5 December 2012









Detection of Protease-Resistant Prion Protein in Water from a CWD-Endemic Area


65


Tracy A. Nichols*1,2, Bruce Pulford1, Christy Wyckoff1,2, Crystal Meyerett1, Brady Michel1, Kevin Gertig3, Jean E. Jewell4, Glenn C. Telling5 and M.D. Zabel1 1Department of Microbiology, Immunology and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO 80523, USA 2National Wildlife Research Center, Wildlife Services, United States Department of Agriculture, Fort Collins, Colorado, 80521, USA 3Fort Collins Water and Treatment Operations, Fort Collins, Colorado, 80521, USA 4 Department of Veterinary Sciences, Wyoming State Veterinary Laboratory, University of Wyoming, Laramie, Wyoming, 82070, USA 5Department of Microbiology, Immunology, Molecular Genetics and Neurology, Sanders Brown Center on Aging, University of Kentucky, Lexington, Kentucky, 40536, USA * Corresponding author- tracy.a.nichols@aphis.usda.gov


Chronic wasting disease (CWD) is the only known transmissible spongiform encephalopathy affecting free-ranging wildlife. Experimental and epidemiological data indicate that CWD can be transmitted horizontally and via blood and saliva, although the exact mode of natural transmission remains unknown. Substantial evidence suggests that prions can persist in the environment, implicating it as a potential prion reservoir and transmission vehicle. CWD- positive animals can contribute to environmental prion load via biological materials including saliva, blood, urine and feces, shedding several times their body weight in possibly infectious excreta in their lifetime, as well as through decomposing carcasses. Sensitivity limitations of conventional assays hamper evaluation of environmental prion loads in water. Here we show the ability of serial protein misfolding cyclic amplification (sPMCA) to amplify minute amounts of CWD prions in spiked water samples at a 1:1 x106 , and protease-resistant prions in environmental and municipal-processing water samples from a CWD endemic area. Detection of CWD prions correlated with increased total organic carbon in water runoff from melting winter snowpack. These data suggest prolonged persistence and accumulation of prions in the environment that may promote CWD transmission.


snip...


The data presented here demonstrate that sPMCA can detect low levels of PrPCWD in the environment, corroborate previous biological and experimental data suggesting long term persistence of prions in the environment2,3 and imply that PrPCWD accumulation over time may contribute to transmission of CWD in areas where it has been endemic for decades. This work demonstrates the utility of sPMCA to evaluate other environmental water sources for PrPCWD, including smaller bodies of water such as vernal pools and wallows, where large numbers of cervids congregate and into which prions from infected animals may be shed and concentrated to infectious levels.



snip...end...full text at ;

















PLoS ONE. 2008; 3(8): e2969.



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



The epidemic of bovine spongiform encephalopathy (BSE) has led to a world-wide drop in the market for beef by-products, such as Meat-and-Bone Meal (MBM), a fat-containing but mainly proteinaceaous product traditionally used as an animal feed supplement. While normal rendering is insufficient, the production of biodiesel from MBM has been suggested to destroy infectivity from transmissible spongiform encephalopathies (TSEs). In addition to producing fuel, this method simultaneously generates a nutritious solid residue. In our study we produced biodiesel from MBM under defined conditions using a modified form of alkaline methanolysis. We evaluated the presence of prion in the three resulting phases of the biodiesel reaction (Biodiesel, Glycerol and Solid Residue) in vitro and in vivo. Analysis of the reaction products from 263K scrapie infected MBM led to no detectable immunoreactivity by Western Blot. Importantly, and in contrast to the biochemical results the solid MBM residue from the reaction retained infectivity when tested in an animal bioassay. Histochemical analysis of hamster brains inoculated with the solid residue showed typical spongiform degeneration and vacuolation. Re-inoculation of these brains into a new cohort of hamsters led to onset of clinical scrapie symptoms within 75 days, suggesting that the specific infectivity of the prion protein was not changed during the biodiesel process. The biodiesel reaction cannot be considered a viable prion decontamination method for MBM, although we observed increased survival time of hamsters and reduced infectivity greater than 6 log orders in the solid MBM residue. Furthermore, results from our study compare for the first time prion detection by Western Blot versus an infectivity bioassay for analysis of biodiesel reaction products. We could show that biochemical analysis alone is insufficient for detection of prion infectivity after a biodiesel process.







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



Abstract



One-gram samples from a pool of crude brain tissue from hamsters infected with the 263K strain of hamster-adapted scrapie agent were placed in covered quartz-glass crucibles and exposed for either 5 or 15 min to dry heat at temperatures ranging from 150°C to 1,000°C. Residual infectivity in the treated samples was assayed by the intracerebral inoculation of dilution series into healthy weanling hamsters, which were observed for 10 months; disease transmissions were verified by Western blot testing for proteinase-resistant protein in brains from clinically positive hamsters. Unheated control tissue contained 9.9 log10LD50/g tissue; after exposure to 150°C, titers equaled or exceeded 6 log10LD50/g, and after exposure to 300°C, titers equaled or exceeded 4 log10LD50/g. Exposure to 600°C completely ashed the brain samples, which, when reconstituted with saline to their original weights, transmitted disease to 5 of 35 inoculated hamsters. No transmissions occurred after exposure to 1,000°C.



see full text:








Friday, December 14, 2012


Susceptibility of domestic cats to chronic wasting disease







Friday, December 14, 2012


Susceptibility Chronic Wasting Disease (CWD) in wild cervids to Humans 2005 - December 14, 2012







Friday, December 14, 2012


DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012







Tuesday, December 18, 2012


A Growing Threat How deer breeding could put public trust wildlife at risk







Wednesday, March 28, 2012


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







Tuesday, December 18, 2012


Bioassay Studies Support the Potential for Iatrogenic Transmission of Variant Creutzfeldt Jakob Disease through Dental Procedures








Wednesday, May 16, 2012


Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?


Proposal ID: 29403






















































RIP MOM 12/14/97 confirmed hvCJD...TSS December 25, 2012

Thursday, December 20, 2012

OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe WITH BOVINE MAD COW DISEASE

A_Annex_VII_A_AHG_Report_Meeting_Final



Scrapie – The disease does not show significant morbidity (2-30% within-flock morbidity) or mortality and is not zoonotic. However, the Group noted the difficulty in evaluating the level of morbidity for diseases with a long incubation period such as scrapie. The Group recommended that the disease be delisted.







Chapter 1.6.


PROCEDURES FOR SELF DECLARATION AND FOR OFFICIAL RECOGNITION BY the OIE


Article 1.6.1.


[No change]


Article 1.6.2.


[No change]


Article 1.6.3.


Questionnaire on bovine spongiform encephalopathy






SNIP...




Article 11.5.29.


Conclusions of the risk assessment


The overall risk of BSE in the cattle population of a country or zone is proportional to the level of known or potential exposure to BSE infectivity and the potential for recycling and amplification of the infectivity through livestock feeding practices. For the risk assessment to conclude that the cattle population of a country or zone is free from BSE risk, it should have demonstrated that appropriate measures have been taken to manage any risks identified.














BOUGHT AND PAID FOR BY YOUR LOCAL CATTLE DEALERS AND LOBBYIEST EVERYWHERE...




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,







i pulled this comment off another board about the OIE and all it's lobby groups...tss



Having been to their offices in Paris and talked personally with the Head of the Animal Test Section, you would choke if you knew how many lobby groups attend that office daily. There is a steady stream of paid lobby groups that have one goal in life and that is to sway the Section Heads of each department within the OIE to suit the needs of different juristictions around the world, which curiously enough, also includes the USA and Canada. Anyone can go there and chat with them - providing they can privide valid cause to be let in. To say that the only goal of the OIE is animal health is actually only part of their function. They are more than that and my discussions with Dr. Diaz there has showed me that. But to blindly make a statement regarding what they do when you have no idea what they actually do is like eating the skin of the orange and not knowing what is actually under. Interstingly you state that the US Government applied pressure (to the OIE) I assume and that is a great example of the lobby groups doing their job. So, at the end of the day, one can safely assume that it is the pressure applied by certain influential lobby groups that will determine a likely aoutcome to an apparent OIE directive. Man alive, isn't it great to live in a democracy wherein the people get to make the choices and not just some "other" interested party or group - say like........Cargyll or Tyson for example?



So, one last question, question?



Who wags the tail of that dog?? And for what reason other than one that is purely associated with trade and international agreements and greed?




Location: Edmonton, Alberta, Canada


Occupation: CEO of BSE Prion Solutions Inc.


Interests: Prion Diseases and Live Animal Testing


end... TSS





SEE MAD COW REPORTS AND TONNAGE OF MAD COW FEED IN COMMERCE ONE DECADE POST MAD COW PARTIAL AND VOLUNTARY FEED BAN AUGUST 4, 1997. ...TSS






Saturday, December 15, 2012


Bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle -- an update 5 December 2012







Friday, December 14, 2012


DEFRA U.K. What is the risk of Chronic Wasting Disease CWD being introduced into Great Britain? A Qualitative Risk Assessment October 2012






Sunday, December 2, 2012


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






SEE MORE MAD COW FEED BAN WARNINGS AND LETTERS HERE ;




Saturday, August 4, 2012


Final Feed Investigation Summary - California BSE Case - July 2012






Tuesday, July 17, 2012


O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th General Session, 20 - 25 May 2012







Wednesday, May 25, 2011


O.I.E. Terrestrial Animal Health Standards Commission and prion (TSE) disease reporting 2011


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


From: Terry S. Singeltary Sr.


To: BSE-L@LISTS.AEGEE.ORG


Cc: trade@oie.int ; oie@oie.int ; f.diaz@oie.int ; scientific.dept@oie.int ; cjdvoice@yahoogroups.com ; BLOODCJD@YAHOOGROUPS.COM


Sent: Tuesday, May 24, 2011 2:24 PM


Subject: O.I.E. Terrestrial Animal Health Standards Commission and prion (TSE) disease reporting 2011






Saturday, December 18, 2010


OIE Global Conference on Wildlife Animal Health and Biodiversity - Preparing for the Future (TSE AND PRIONS) Paris (France), 23-25 February 2011






Monday, November 23, 2009


BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E. COMMISSION DECISION of 11 November 2009 amending the Annex to Decision 2007/453/EC as regards the BSE status of Chile, Colombia and Japan (notified under document C(2009) 8590)






Tuesday, January 1, 2008


BSE OIE USDA


Subject: OIE BSE RECOMMENDATION FOR USA, bought and paid for by your local cattle dealers i.e. USDA


Date: May 14, 2007 at 9:00 am PST


OIE BSE RECOMMENDATION FOR USA, bought and paid for by your local cattle dealers i.e. USDA


STATEMENT BY DR. RON DEHAVEN REGARDING OIE RISK RECOMMENDATION


March 9, 2007







Tuesday, November 02, 2010


IN CONFIDENCE


The information contained herein should not be disseminated further except on the basis of "NEED TO KNOW".


BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992







2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006






Comments on technical aspects of the risk assessment were then submitted to FSIS.


Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.


This document provides itemized replies to the public comments received on the 2005 updated Harvard BSE risk assessment. Please bear the following points in mind:






Owens, Julie


From: Terry S. Singeltary Sr. [flounder9@verizon.net]


Sent: Monday, July 24, 2006 1:09 PM


To: FSIS RegulationsComments


Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)


Page 1 of 98







FSIS, USDA, REPLY TO SINGELTARY







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








2012 atypical L-type BSE BASE California reports


Saturday, August 4, 2012


Final Feed Investigation Summary - California BSE Case - July 2012






SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012


Summary Report BSE 2012


Executive Summary






Saturday, August 4, 2012


Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation






CENSORSHIP IS A TERRIBLE THING $$$




Canada has had a COVER-UP policy of mad cow disease since about the 17th case OR 18th case of mad cow disease. AFTER THAT, all FOIA request were ignored $$$


THIS proves there is indeed an epidemic of mad cow disease in North America, and it has been covered up for years and years, if not for decades, and it’s getting worse $$$




Thursday, February 10, 2011


TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31






Wednesday, August 11, 2010


REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA







Thursday, August 19, 2010


REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA







Friday, March 4, 2011


Alberta dairy cow found with mad cow disease







Reasons for the New Regulation Order No. 23 (as well as amending Order No. 149) of the State Committee for Veterinary Medicine name BSE as the reason for new import requirement. The legal title for Order No. 23 is "On Urgent Measures Aimed at Prevention and Elimination of BSE and Other Prion Infections in Cattle”. Neither Order explains how the threat of introduction of BSE can be addressed through the inspection of producers of all products of animal origin including fish, dairy products, poultry and pork. It is not clear what other concerns are addressed through the proposed inspections. Formal Notification of Trading Partners On August 3rd, Ukraine's Notification and Enquiry Point issued a legal Notification G/SPS/N/UKR/3/Rev.1 found on the Official WTO Website (Committee on Sanitary and Phytosanitary Measures)






Thursday, March 29, 2012


atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012


NIAA Annual Conference April 11-14, 2011 San Antonio, Texas







Monday, November 30, 2009


USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE







Monday, December 1, 2008


When Atypical Scrapie cross species barriers







EVIDENCE OF SCRAPIE IN SHEEP AS A RESULT OF FOOD BORNE EXPOSURE


This is provided by the statistically significant increase in the incidence of sheep scrape from 1985, as determined from analyses of the submissions made to VI Centres, and from individual case and flock incident studies. ........






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


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


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


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


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


PMID: 6997404








12/10/76


AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE


Office Note CHAIRMAN: PROFESSOR PETER WILDY


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A The Present Position with respect to Scrapie A] The Problem


Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries.


The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep.


It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible.


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


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


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76/10.12/4.6







Nature. 1972 Mar 10;236(5341):73-4.


Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). Gibbs CJ Jr, Gajdusek DC.


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


Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)


C. J. GIBBS jun. & D. C. GAJDUSEK


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


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








Epidemiology of Scrapie in the United States 1977











IT'S as obvious as day and night, either Larry, Curley, and Mo have been at the helm of the USDA/APHIS/FSIS/FDA/CDC/NIH et al for many many years, or the incompetence of these agencies are so inept, either through ignorance and or just too overweight with industry reps., they then should be all done away with and a single agency brought forth, and if not, how will you correct this ongoing problem ?








Friday, February 11, 2011


Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues








Sunday, December 12, 2010


EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010






Wednesday, January 18, 2012


Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie


Journal of Neuropathology & Experimental Neurology: February 2012 - Volume 71 - Issue 2 - p 140–147







Thursday, July 14, 2011


Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4)






Wednesday, January 18, 2012


BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE


February 1, 2012






Thursday, December 23, 2010


Molecular Typing of Protease-Resistant Prion Protein in Transmissible Spongiform Encephalopathies of Small Ruminants, France, 2002-2009


Volume 17, Number 1 January 2011








Thursday, November 18, 2010


Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep







Wednesday, February 16, 2011


IN CONFIDENCE


SCRAPIE TRANSMISSION TO CHIMPANZEES


IN CONFIDENCE







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



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



snip...



R. BRADLEY









Friday, February 11, 2011


Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues







Monday, April 25, 2011


Experimental Oral Transmission of Atypical Scrapie to Sheep


Volume 17, Number 5-May 2011







Sunday, April 18, 2010


SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010








Wednesday, March 28, 2012


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







Tuesday, November 6, 2012


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







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...










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


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IT is of my opinion, that the OIE and the USDA et al, are the soul reason, and responsible parties, for Transmissible Spongiform Encephalopathy TSE prion diseases, including typical and atypical BSE, typical and atypical Scrapie, and all strains of CWD, and human TSE there from, spreading around the globe.



I have lost all confidence of this organization as a regulatory authority on animal disease, and consider it nothing more than a National Trading Brokerage for all strains of animal TSE, just to satisfy there commodity. AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization.


JUST because of low documented human body count with nvCJD and the long incubation periods, the lack of sound science being replaced by political and corporate science in relations with the fact that science has now linked some sporadic CJD with atypical BSE and atypical scrapie, and the very real threat of CWD being zoonosis, I believed the O.I.E. has failed terribly and again, I call for this organization to be dissolved. ...




Tuesday, July 17, 2012


O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th General Session, 20 - 25 May 2012







TSS