Science and Technology Committee Oral evidence: Blood, tissue and organ screening, HC 990 Wednesday 5 March 2014 SPORADIC CJD
Science and Technology Committee Oral evidence: Blood, tissue and organ
screening, HC 990 Wednesday 5 March 2014 SPORADIC CJD
Science and Technology Committee
Oral evidence: Blood, tissue and organ screening, HC 990 Wednesday 5 March
2014
Ordered by the House of Commons to be published on 5 March 2014.
Written evidence from witnesses:
– Prometic BioSciences Ltd
– Terumo BCT
– Prionics AG
– DuPont Chemicals and Fluoroproducts
Watch the meeting
Members present: Andrew Miller (Chair); Jim Dowd; David Heath; Stephen
Metcalfe; David Morris; Stephen Mosley; Pamela Nash; Sarah Newton; Graham
Stringer; David Tredinnick
snip...
Professor Collinge: I think there is some misunderstanding there. The
apparent incidence of sporadic CJD is increasing. There are about a hundred new
cases a year at the moment, and that has gone up substantially from the start of
surveillance. That is thought, principally, to reflect better diagnosis of the
disease, although that may not be all the explanation. It is possible, and we
can talk about that, that some of that may be BSE-related. However, I think that
the specific confusion there is that people talk about sporadic CJD occurring at
1 per million. That is not your individual risk. Your risk is 1 per million
every year. Actually, it is nearer 2 per million per year of the population will
develop sporadic CJD, but your lifetime risk of developing sporadic CJD is about
1 in 30,000. So that has not really changed. When people talk about 1 per
million, often they interpret that as thinking it is incredibly rare. They think
they have a 1-in-a-million chance of developing this disease. You haven’t.
You’ve got about a 1-in-30,000 chance of developing it.
snip...
Q112 Graham Stringer: Professor Collinge, the last time you were here you
said that you believed that the risk of transmission of vCJD via surgical
instruments was a significant problem. If that is the case, why have no cases
been reported where vCJD has been transmitted in that particular way?
Professor Collinge: What I was trying to say is that there is clearly a
risk there. We have not been able to quantify that risk. It is correct that we
don’t know of any cases of secondary vCJD that can be attributed to surgical
instruments at this point in time, but it can’t be excluded that some of the
cases we have seen so far of vCJD have been related to that. It is a question of
how you would tell. That really comes from epidemiological studies. Since a
history of prior surgery—particularly a history of prior dental surgery—is so
common, and many patients you see will have had prior surgery and certainly
virtually all of them will have had dentistry, it is hard to make that link. The
link has been made with sporadic CJD where there have been clearly documented
instances of transmission of prions by surgical instruments, by neurosurgical
instruments. There is also epidemiological evidence from several countries now
that patients developing classical CJD are more likely to have had abdominal
surgery beforehand, for example. There are sufficient numbers where an
epidemiological link has been shown. You are right—we don’t have definite
evidence that a surgically transmitted case of vCJD has occurred, but that is,
perhaps, not surprising at this point in time.
What we do have is a great deal of scientific evidence that, first of all,
the infective agent in vCJD is much more widely distributed in the body than it
is in classical CJD, so there is more opportunity for it to contact surgical and
medical instruments. Of course, as you heard from the HPA study, there are,
potentially, quite a lot of people carrying the infection around. We also know,
experimentally, that it is relatively easy to transmit prions by binding them to
metal surfaces. In fact, it is something that we use experimentally now. It is a
very efficient way of transmitting the disease. Indeed, the blood test that we
talked about earlier exploits the affinity of prions on metal surfaces as a way
of concentrating the infective agent prior to detection. There is plenty of
scientific evidence that this would be an efficient route of transmission. There
is plenty of evidence that there are many people in the UK incubating the
disease and that the infection would be in tissues that come into contact with
surgical instruments. But you are right: so far, there is not a documented
example of that happening with vCJD.
Wednesday, December 11, 2013
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic
Creutzfeldt-Jakob Disease ***
Sunday, March 09, 2014
*** A Creutzfeldt-Jakob Disease (CJD) Lookback Study: Assessing the Risk of
Blood Borne Transmission of Classic Forms of Creutzfeldt-Jakob Disease
FDA TSEAC CIRCUS AND TRAVELING ROAD SHOW FOR THE TSE PRION DISEASES
Thursday, January 2, 2014
*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant
Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***
Thursday, February 20, 2014
*** Unnecessary precautions BSE MAD COW DISEASE Dr. William James FSIS VS
Dr. Linda Detwiler 2014
Saturday, February 01, 2014
*** vCJD With Extremely Low Lymphoreticular Deposition of Prion Protein MAY
NOT HAVE BEEN DETECTABLE
Monday, October 14, 2013
*** Researchers estimate one in 2,000 people in the UK carry variant CJD
proteins ***
Wednesday, December 11, 2013
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic
Creutzfeldt-Jakob Disease ***
Thursday, March 6, 2014
*** TEXAS RECALL LIST MASSIVE FROM DEAD STOCK DOWNER CANCER COWS OFFAL from
Class I Recall 002-2014 and 013-2014 Health Risk: High Jan 13, 2014 and Feb 8,
2014 shipped to Texas, Florida, and Illinois UPDATE FEBRUARY 14, 2014 ***
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
Friday, January 10, 2014
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type
prion disease, what 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
snip...
*** 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.
Monday, March 10, 2014
Docket No. 00-108-10 Chronic Wasting Disease Herd Certification Program and
Interstate Movement of Farmed or Captive Deer, Elk, and Moose; Program Standards
Singeltary Submission
BSE prions propagate as either variant CJD-like or sporadic CJD-like prion
strains in transgenic mice expressing human prion protein
Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only
in individuals homozygous for methionine at PRNP codon 129. Here we show that
transgenic mice expressing human PrP methionine 129, inoculated with either
bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the
neuropathological and molecular phenotype of vCJD, consistent with these
diseases being caused by the same prion strain. Surprisingly, however, BSE
transmission to these transgenic mice, in addition to producing a vCJD-like
phenotype, can also result in a distinct molecular phenotype that is
indistinguishable from that of sporadic CJD with PrPSc type 2. These data
suggest that more than one BSE-derived prion strain might infect humans; it is
therefore possible that some patients with a phenotype consistent with sporadic
CJD may have a disease arising from BSE exposure.
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
TO ALL IMPORTING COUNTRIES THAT IMPORTS FROM THE USA, BE WARNED, NEW MAD
COW BSE REGULATIONS USDA, AND OIE, not worth the paper the regulations were
wrote on, kind of like the mad cow feed ban of August 1997, nothing but ink on
paper $$$
full text ;
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
U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001
Aug. 5, 2001, 12:25AM
Mad cow disease: Could it be here?
Man's stubborn crusade attracts experts' notice
By CAROL CHRISTIAN Copyright 2001 Houston Chronicle
Like Paul Revere with e-mail, Terry Singeltary Sr. is on a mission to sound
an alarm: Beware of mad cow disease.
As is true of many crusaders, however, his pleas often fall on deaf ears.
Health officials here and abroad insist that bovine spongiform encephalopathy --
popularly known as mad cow disease, a fatal brain disorder that can make cows
shake uncontrollably -- has been kept out of this country through surveillance
of the cattle industry.
But since his mother's death in December 1997, the Galveston County man has
been obsessed with possible connections between her deadly brain disorder,
sporadic Creutzfeldt-Jakob Disease, and mad cow disease.
And after much persistence on his part, people are taking notice of this
former machinist and high school dropout who jokes that he has a Ph.D. -- a Pool
Hall Degree.
"They called me Chicken Little for four years," he said. "Now they're
calling back, asking for more information."
For the past year he has been U.S. co-coordinator of an international
monitoring group called CJD Watch. He regularly gets e-mail from scientists and
journalists around the world.
Debora MacKenzie, a reporter for the British magazine New Scientist,
described Singeltary, 47, as a "dogged unearther and tabulator of government
documents." Singeltary monitors "every word written about CJD/BSE," said Anita
Manning of USA Today, also by e-mail.
"He's passionate, opinionated and not always tactful, although I like him
because he's such a character and he is so transparent," Manning said. "He is
what he appears to be."
Science and environment writer Jonathan Leake of the Sunday Times in London
said Singeltary has helped him track down families of people with CJD along with
academic research papers.
"I strongly suspect he is right in thinking the USA has had BSE cases,"
Leake said by e-mail.
"The American government is making the same mistake as the British in
putting the short-term commercial interests of its farmers before health
considerations," he added.
"It should start formal and widespread testing of cattle plus compulsory
autopsies for all human CJD victims at the state's expense. If there is BSE,
then leaving it to spread will kill people -- and that would eventually destroy
the industry, too."
Texas Department of Health epidemiologist Julie Rawlings said Singeltary's
careful monitoring of the disease had proven useful.
"Terry has been helpful in providing contact information regarding suspect
CJD cases so that the Health Department can initiate case investigations and
learn more about CJD in Texas," she said.
Noting that the department cannot release records on individual patients,
she added, "I think we learn more from him than he does from us."
Mad cow disease surfaced in England in 1986 and quickly became an epidemic.
It since has been reported in 15 European countries, most recently Greece on
July 2, and the Czech Republic on June 14. Two German-born cows tested positive
for BSE in November.
Singeltary said he became convinced that BSE is here as he watched his
mother, Barbara Poulter of Crystal Beach, dying of sporadic Creutzfeldt-Jakob
Disease. The rare, fatal brain disease is sometimes accompanied by severe
jerking.
"She would jerk so bad at times, it would take three of us to hold her
down," Singeltary said. "They can call it whatever they want, but I know what I
saw, and what she went through. `Sporadic' simply means they don't know."
Poulter, a retired telephone-company field worker, had a form of sporadic
CJD -- Haidenhain variant -- that is even less common than the typical sporadic
case. One of its first symptoms is loss of vision.
She started seeing brown spots in September 1997 and was virtually blind
within two weeks. By the eighth week of the illness Poulter was bedridden, and
in the 10th week she died. Before that she had been in good health.
In many countries and most U.S. states, physicians are not required to
report CJD cases to health officials. Texas made the disease reportable in 1998.
Through 2000, there were 17 probable or confirmed cases, according to the Texas
Department of Health.
In mid-June, a case of sporadic CJD was confirmed through brain biopsy at
Christus Spohn Hospital Shoreline in Corpus Christi, said Jane Bakos, hospital
vice president. The patient has since died, the hospital reported.
CJD and mad cow disease leave their victims' brains full of holes like a
sponge.
Although not contagious, the illnesses are thought to be transmissible
through prions, or nearly indestructible abnormal proteins.
Because the prion protein is not killed by standard sterilization, sporadic
CJD can be spread by contaminated surgical instruments.
In March 1996, the British government announced the discovery of a new
variant of CJD, most likely explained by exposure to bovine spongiform
encephalopathy.
Through June, 101 cases of new-variant CJD have been reported in the United
Kingdom, three in France and one in Ireland. In contrast to sporadic CJD, the
new variant usually affects younger patients and lasts longer.
No cases of new-variant CJD or BSE have been reported in the United States.
No relationship has been shown between sporadic CJD and mad cow disease.
There is no indication that new-variant CJD can be spread through blood
transfusions, but a U.S. Food and Drug Administration advisory committee voted
in June to broaden the categories for excluding potential donors. The
recommendations have not yet been approved by the FDA.
The American Red Cross has announced that on Sept. 17 it will begin
rejecting potential blood donors who, since 1980, have spent at least three
months in the United Kingdom or at least six months in any European country or
combination of countries. Those who have received a blood transfusion in Britain
since 1980 also will be rejected.
The primary collector of local blood donations is the Gulf Coast Regional
Blood Center, which will follow the FDA's guidelines, said Bill Teague,
president and chief executive officer.
Singeltary said it's naive to think that U.S. prevention efforts have kept
mad cow and new-variant CJD out of the United States.
"They haven't found it," he said, "because they haven't looked."
For one thing, he said, too few cows are tested for the disease. In the
first six months of this year, the European Union tested more than 3.2 million
cows, David Byrne of the European Commission said in a speech last month.
By contrast, it took the U.S. Department of Agriculture nearly 10 years to
analyze about 13,000 cow brains, according to the department's Web site.
With more than 68 million cattle slaughtered since 1990 in the United
States, according to the USDA, checking about 13,000 falls far short, Singeltary
said.
Though not a scholar, Singeltary has collected voluminous material on mad
cow and CJD. Disabled from a neck injury, Singeltary never used a computer until
1998.
He now spends hours each day on the Internet while his wife, Bonnie
Singeltary, runs a flower shop in their home in Bacliff, in north Galveston
County.
His challenge to the CJD/BSE establishment is courageous and refreshing,
said Dr. Lynette Dumble, former visiting professor of surgery at University of
Texas Medical School at Houston and a former senior research fellow in the
history and philosophy of science at the University of Melbourne in
Australia.
"I certainly have no problem with Terry's ideas on BSE/CJD," said Dumble,
who coordinates the Global Sisterhood Network, a computer service that posts
media reports on developments affecting women. "His research skills are
excellent, and he is abreast of each and every development in the field."
Among Singeltary's worries now, he said, are widespread violations of an
August 1997 ban on feeding animal products to U.S. cattle. The FDA reported in
January that hundreds of feed manufacturers were not complying with regulations
designed to keep BSE out of this country.
(That same month, a Purina Mills feedlot near San Antonio told the FDA that
a "very low level" of cow parts had been found in cattle feed. The company
voluntarily removed 1,222 animals who had been fed the prohibited
materials.)
He obtained copies of FDA letters to various feed mills that had been found
in violation of the regulations and immediately sent them by e-mail to hundreds
of people around the world.
Singeltary might not be so zealous in getting the word out if he weren't
convinced that someone is covering up the truth.
"They used to say BSE would never transmit to humans," he said, "and it
has. They lied about the feed ban being in place.
"I've lost faith in the whole process. I've discovered too many things."
Sporadic CJD type 1 and atypical/ Nor98 scrapie are characterized by fine
(reticular) deposits,
see also ;
All of the Heidenhain variants were of the methionine/ methionine type 1
molecular subtype.
also, see relations from atypical h-type BSE and Heidenhain Variant of
Creutzfeldt Jakob Disease here ;
Wednesday, February 26, 2014
GERMANY REPORTS ANOTHER CASE OF ATYPICAL BSE (H-TYPE)
Tuesday, July 29, 2008
Heidenhain Variant Creutzfeldt Jakob Disease Case Report
snip...
Heidenhain Variant Creutzfeldt Jakob Disease autopsy case report
'MOM'
DIVISION OF NEUROPATHOLOGY University of Texas Medical Branch 114
McCullough Bldg. Galveston, Texas 77555-0785
FAX COVER SHEET
DATE: 4-23-98
TO: Mr. Terry Singeltary @ -------
FROM: Gerald Campbell
FAX: (409) 772-5315 PHONE: (409) 772-2881
Number of Pages (including cover sheet):
Message:
*CONFIDENTIALITY NOTICE*
This document accompanying this transmission contains confidential
information belonging to the sender that is legally privileged. This information
is intended only for the use of the individual or entry names above. If you are
not the intended recipient, you are hereby notified that any disclosure, copying
distribution, or the taking of any action in reliances on the contents of this
telefaxed information is strictly prohibited. If you received this telefax in
error, please notify us by telephone immediately to arrange for return of the
original documents. -------------------------- Patient Account: 90000014-518
Med. Rec. No.: (0160)118511Q Patient Name: POULTER, BARBARA Age: 63 YRS DOB:
10/17/34 Sex: F Admitting Race: C
Attending Dr.: Date / Time Admitted : 12/14/97 1228 Copies to:
UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409)
772-1238 Fax (409) 772-5683 Pathology Report
FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858
Autopsy NO.: AU-97-00435
AUTOPSY INFORMATION: Occupation: Unknown Birthplace: Unknown Residence:
Crystal Beach Date/Time of Death: 12/14/97 13:30 Date/Time of Autopsy: 12/15/97
15:00 Pathologist/Resident: Pencil/Fernandez Service: Private Restriction: Brain
only
FINAL AUTOPSY DIAGNOSIS
I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.
snip...see full text ;
P.5.21
*** Parallels between different forms of sheep scrapie and types of
Creutzfeldt-Jakob disease (CJD)
Wiebke M. Wemheuer1, Sylvie L. Benestad2, Arne Wrede1, Wilhelm E.
Wemheuer3, Tatjana Pfander1, Bjørn Bratberg2, Bertram Brenig3,Walter J.
Schulz-Schaeffer1 1University Medical Center Goettingen, Germany; 2Institute of
Veterinary Medicine Oslo, Norway; 3Institute of Veterinary Medicine Goettingen,
Germany
Background: Scrapie in sheep and goats is often regarded as the archetype
of prion diseases. In 1998, a new form of scrapie - atypical/Nor98 scrapie - was
described that differed from classical scrapie in terms of epidemiology, Western
blot profile, the distribution of pathological prion protein (PrPSc) in the body
and its stability against proteinase K. In a similar way, distinct disease types
exist in sporadic Creutzfeldt-Jakob disease (CJD). They differ with regard to
their clinical outcome, Western blot profile and PrPSc deposition pattern in the
central nervous system (CNS).
Objectives: The comparison of PrPSc deposits in sheep scrapie and human
sporadic CJD.
Methods: Tissues of the CNS of sheep with classical scrapie, sheep with
atypical/Nor98 scrapie and 20 patients with sporadic CJD were examined using the
sensitive Paraffin Embedded Tissue (PET) blot method. The results were compared
with those obtained by immunohistochemistry. With the objective of gaining
information on the protein conformation, the PrPSc of classical and
atypical/Nor98 sheep scrapie and sporadic CJD was tested for its stability
against denaturation with guanidine hydrochloride (GdnHCl) using a Membrane
Adsorption Assay.
Results: The PrPSc of atypical/Nor98 scrapie cases and of CJD prion type 1
patients exhibits a mainly reticular/synaptic deposition pattern in the brain
and is relatively sensitive to denaturation with GdnHCl. In contrast classical
scrapie cases and CJD prion type 2 patients have a more complex PrPSc deposition
pattern in common that consists of larger PrPSc aggregates and the PrPSc itself
is comparatively stable against denaturation.
Discussion: The similarity between CJD types and scrapie types indicates
that at least two comparable forms of the misfolded prion protein exist beyond
species barriers and can elicit prion diseases. It seems therefore reasonable to
classify classical and atypical/Nor98 scrapie - in analogy to the existing CJD
types - as different scrapie types.
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
why do we not want to do TSE transmission studies on chimpanzees $
snip...
5. A positive result from a chimpanzee challenged severly would likely
create alarm in some circles even if the result could not be interpreted for
man. I have a view that all these agents could be transmitted provided a large
enough dose by appropriate routes was given and the animals kept long enough.
Until the mechanisms of the species barrier are more clearly understood it might
be best to retain that hypothesis.
snip...
R. BRADLEY
1: J Infect Dis 1980 Aug;142(2):205-8
Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to
nonhuman primates.
Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep
and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were
exposed to the infectious agents only by their nonforced consumption of known
infectious tissues. The asymptomatic incubation period in the one monkey exposed
to the virus of kuru was 36 months; that in the two monkeys exposed to the virus
of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the
two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively.
Careful physical examination of the buccal cavities of all of the monkeys failed
to reveal signs or oral lesions. One additional monkey similarly exposed to kuru
has remained asymptomatic during the 39 months that it has been under
observation.
snip...
The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie
by natural feeding to squirrel monkeys that we have reported provides further
grounds for concern that scrapie-infected meat may occasionally give rise in
humans to Creutzfeldt-Jakob disease.
PMID: 6997404
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
Recently the question has again been brought up as to whether scrapie is
transmissible to man. This has followed reports that the disease has been
transmitted to primates. One particularly lurid speculation (Gajdusek 1977)
conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and
transmissible encephalopathy of mink are varieties of a single "virus". The U.S.
Department of Agriculture concluded that it could "no longer justify or permit
scrapie-blood line and scrapie-exposed sheep and goats to be processed for human
or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is
emphasised by the finding that some strains of scrapie produce lesions identical
to the once which characterise the human dementias"
Whether true or not. the hypothesis that these agents might be
transmissible to man raises two considerations. First, the safety of laboratory
personnel requires prompt attention. Second, action such as the "scorched meat"
policy of USDA makes the solution of the acrapie problem urgent if the sheep
industry is not to suffer grievously.
snip...
76/10.12/4.6
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).
Re: vCJD in the USA * BSE in U.S. 15 November 1999 Terry S Singeltary, NA
CWD is just a small piece of a very big puzzle. I have seen while deer
hunting, deer, squirrels and birds, eating from cattle feed troughs where they
feed cattle, the high protein cattle by products, at least up until Aug. 4,
1997. So why would it be so hard to believe that this is how they might become
infected with a TSE. Or, even by potentially infected land. It's been well
documented that it could be possible, from scrapie.
It was proven in Oprah Winfrey's trial, that Cactus Cattle feeders, sent
neurologically ill cattle, some with encephalopathy stamped on the dead slips,
were picked up and sent to the renders, along with sheep carcasses.
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as
well...
2 January 2000 Terry S Singeltary
The exact same recipe for B.S.E. existed in the U.S. for years and years.
In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25
page report by the USDA:APHIS:VS. It could have been done in one page. The first
page, fourth paragraph says it all;
"Similarities exist in the two countries usage of continuous rendering
technology and the lack of usage of solvents, however, large differences still
remain with other risk factors which greatly reduce the potential risk at the
national level."
Then, the next 24 pages tries to down-play the high risks of B.S.E. in the
U.S., with nothing more than the cattle to sheep ratio count, and the
geographical locations of herds and flocks. That's all the evidence they can
come up with, in the next 24 pages.
Something else I find odd, page 16;
"In the United Kingdom there is much concern for a specific continuous
rendering technology which uses lower temperatures and accounts for 25 percent
of total output. This technology was _originally_ designed and imported from the
United States. However, the specific application in the production process is
_believed_ to be different in the two countries."
A few more factors to consider, page 15;
"Figure 26 compares animal protein production for the two countries. The
calculations are based on slaughter numbers, fallen stock estimates, and product
yield coefficients. This approach is used due to variation of up to 80 percent
from different reported sources. At 3.6 million tons, the United States produces
8 times more animal rendered product than the United Kingdom."
"The risk of introducing the BSE agent through sheep meat and bone meal is
more acute in both relative and absolute terms in the United Kingdom (Figures 27
and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61
thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in
the United States. For sheep greater than 1 year, this is less than one-tenth of
one percent of the United States supply."
"The potential risk of amplification of the BSE agent through cattle meat
and bone meal is much greater in the United States where it accounts for 59
percent of total product or almost 5 times more than the total amount of
rendered product in the United Kingdom."
Considering, it would only take _one_ scrapie infected sheep to contaminate
the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug.
1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful
of scrapie infected material is lethal to a cow. Considering all this, the sheep
to cow ration is meaningless. As I said, it's 24 pages of B.S.e.
To be continued...
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA
Competing interests: None declared
Letters
JAMA. 2001;285(6):733-734. doi: 10.1001/jama.285.6.733
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr Bacliff, Tex
Since this article does not have an abstract, we have provided the first
150 words of the full text.
KEYWORDS: creutzfeldt-jakob disease, diagnosis. 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.
References 1. Gibbons RV, Holman RC, Belay ED, Schonberger LB.
Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA.
2000;284:2322-2323.
Published March 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease
in the United States
Terry S. Singeltary, retired (medically)
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?
Published March 26, 2003
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.
The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003
doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI
Tracking spongiform encephalopathies in North America
Original
Xavier Bosch
“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever
since. What I have found is that we have not been told the truth. CWD in deer
and elk is a small portion of a much bigger problem.” 49-year—old Singeltary is
one of a number of people who have remained largely unsatisfied after being told
that a close relative died from a rapidly progressive dementia compatible with
spontaneous Creutzfeldt—Jakob ...
SEE FULL TEXT ;
-------- Original Message --------
Subject: Tracking spongiform encephalopathies in North America LANCET
INFECTIOUS DISEASE Volume 3, Number 8 01 August 2003
Date: Tue, 29 Jul 2003 17:35:30 –0500
From: "Terry S. Singeltary Sr." Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@uni-karlsruhe.de
Volume 3, Number 8 01 August 2003
Previous
Next
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever
since. What I have found is that we have not been told the truth. CWD in deer
and elk is a small portion of a much bigger problem.
49-year-old Singeltary is one of a number of people who have remained
largely unsatisfied after being told that a close relative died from a rapidly
progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease
(CJD). So he decided to gather hundreds of documents on transmissible spongiform
encephalopathies (TSE) and realised that if Britons could get variant CJD from
bovine spongiform encephalopathy (BSE), Americans might get a similar disorder
from chronic wasting disease (CWD)the relative of mad cow disease seen among
deer and elk in the USA. Although his feverish search did not lead him to the
smoking gun linking CWD to a similar disease in North American people, it did
uncover a largely disappointing situation.
Singeltary was greatly demoralised at the few attempts to monitor the
occurrence of CJD and CWD in the USA. Only a few states have made CJD
reportable. Human and animal TSEs should be reportable nationwide and
internationally, he complained in a letter to the Journal of the American
Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue
to expect us to still believe that the 85% plus of all CJD cases which are
sporadic are all spontaneous, without route or source.
Until recently, CWD was thought to be confined to the wild in a small
region in Colorado. But since early 2002, it has been reported in other areas,
including Wisconsin, South Dakota, and the Canadian province of Saskatchewan.
Indeed, the occurrence of CWD in states that were not endemic previously
increased concern about a widespread outbreak and possible transmission to
people and cattle.
To date, experimental studies have proven that the CWD agent can be
transmitted to cattle by intracerebral inoculation and that it can cross the
mucous membranes of the digestive tract to initiate infection in lymphoid tissue
before invasion of the central nervous system. Yet the plausibility of CWD
spreading to people has remained elusive.
Part of the problem seems to stem from the US surveillance system. CJD is
only reported in those areas known to be endemic foci of CWD. Moreover, US
authorities have been criticised for not having performed enough prionic tests
in farm deer and elk.
Although in November last year the US Food and Drug Administration issued a
directive to state public-health and agriculture officials prohibiting material
from CWD-positive animals from being used as an ingredient in feed for any
animal species, epidemiological control and research in the USA has been quite
different from the situation in the UK and Europe regarding BSE.
Getting data on TSEs in the USA from the government is like pulling teeth,
Singeltary argues. You get it when they want you to have it, and only what they
want you to have.
Norman Foster, director of the Cognitive Disorders Clinic at the University
of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion
disease in people in the USA is inadequate to detect whether CWD is occurring in
human beings; adding that, the cases that we know about are reassuring, because
they do not suggest the appearance of a new variant of CJD in the USA or
atypical features in patients that might be exposed to CWD. However, until we
establish a system that identifies and analyses a high proportion of suspected
prion disease cases we will not know for sure. The USA should develop a system
modelled on that established in the UK, he points out.
Ali Samii, a neurologist at Seattle VA Medical Center who recently reported
the cases of three hunterstwo of whom were friendswho died from pathologically
confirmed CJD, says that at present there are insufficient data to claim
transmission of CWD into humans; adding that [only] by asking [the questions of
venison consumption and deer/elk hunting] in every case can we collect suspect
cases and look into the plausibility of transmission further. Samii argues that
by making both doctors and hunters more aware of the possibility of prions
spreading through eating venison, doctors treating hunters with dementia can
consider a possible prion disease, and doctors treating CJD patients will know
to ask whether they ate venison.
CDC spokesman Ermias Belay says that the CDC will not be investigating the
[Samii] cases because there is no evidence that the men ate CWD-infected meat.
He notes that although the likelihood of CWD jumping the species barrier to
infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that
CWD does not exist in humans& the data seeking evidence of CWD transmission
to humans have been very limited.
LANCET SINGELTARY ET AL CWD TSE PRION NORTH AMERICA
http://download.thelancet.com/pdfs/journals/laninf/PIIS1473309903007151.pdf?id=baa1CkXPkhI3Ih_Vlh6ru
Singeltary submission to PLOS ;
No competing interests declared.
see full text ;
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
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
Monday, March 10, 2014
Investigators study silent variant of mad cow disease Galveston Daily News
March 4, 2014
Wednesday, May 16, 2012
*** Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion
disease, Iatrogenic, what if ?
Proposal ID: 29403
CJD QUESTIONNAIRE USA
CJD VOICE
Thank You,
Respectfully,
I am sincerely,
layperson
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
flounder9@verizon.net
MOM DOD 12/14/97 confirm ‘hvCJD’ just made a promise to mom, NEVER FORGET!
and never let them forget. ...
posted by Terry S. Singeltary Sr. at
11:08 AM
<< Home