Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012
Human Prion Disease in Washington, 2006–2011
Human Prion Diseases
Prion diseases are a group of rare brain and nervous system diseases that
affect humans and some kinds of animals. Creutzfeldt-Jakob disease (CJD) is by
far the most common human prion disease. It is a rare, fatal neurodegenerative
disease commonly characterized by rapidly progressing dementia, poor balance,
visual changes and/or muscle jerks. Sporadic CJD (sCJD) has no known cause and
accounts for about 85% of all CJD cases. It occurs worldwide at a rate of
approximately 1 case per million population per year(1), however, higher rates
have been reported(2). Most persons affected with sCJD are over 55 years old.
Familial CJD (fCJD) results from an inherited mutation and accounts for 10–15%
of cases. Rarely, CJD is acquired. It has been transmitted through certain
surgical procedures or human-derived growth hormone. In 1996, a new variant of
CJD (vCJD) recognized in the United Kingdom was associated with eating cattle
products from cows affected with bovine spongiform encephalopathy (“mad cow
disease”). To date, no cases of vCJD are thought to have been acquired in
Washington or the United States.
Surveillance Strategies in Washington State
In December 2003, the first known case of bovine spongiform encephalopathy
(i.e., “mad cow disease”) in the United States was diagnosed in an adult
Holstein cow from Washington State. Prior to the identification of this cow,
surveillance for human prion disease (including all types of CJD) in Washington
was performed through death certificate review. Beginning in 2004, the
Washington State Department of Health (DOH) enhanced efforts to identify and
confirm human prion disease by collaborating with the Centers for Disease
Control and Prevention (CDC) and the National Prion Disease Pathology
Surveillance Center (NPDPSC), and investigating Washington residents with
elevated 14-3-3 protein prior to death. In addition, healthcare providers in
Washington are required to report suspected human prion disease to the local
health jurisdiction where the patient resides.
Human Prion Disease in Washington State
During 2006–2011, 57 cases of CJD were detected in Washington; 44 (77%)
were tissue-confirmed by NPDPSC and 13 (23%) were clinically diagnosed. The
average yearly incidence rate over this time period was 1.4 cases per million
population per year based on population estimates provided by the Office of
Financial Management.
The following graph and table show the number of CJD cases by year of death
and the characteristics of CJD cases diagnosed in Washington during 2006–2011.
August 27, 2012
Number of CJD Cases by Year of Death, Washington State, 2006–2011
Characteristics of Definite or Probable CJD Cases, Washington State,
2006–2011 (n=57)
Characteristic No. Cases (%)
Male 26 (46)
Median age [range] 67.5 years [38–84 years old]
Western WA residents *41 (72)
Median duration of illness [range] 4 months [1–30 months]
Laboratory-confirmed 44 (77)
Sporadic CJD (sCJD) 38 (86)
Familial CJD (fCJD) 2 (5)
Sporadic or familial CJD 4 (9)
Variant CJD 0 (0)
*Western Washington is defined as living west of the Cascade Mountains. In
2010, 78% of the population lived in western Washington.
All clinically diagnosed patients had a clinical presentation consistent
with sCJD. Five patients were less than 55 years old at the time of death; 3
were confirmed sCJD, one was confirmed fCJD, and one was diagnosed clinically.
This last patient was 49 years old and developed symptoms 4 months prior to
death, suggestive of sCJD.
Summary
The incidence of CJD in Washington State is consistent with reported rates
worldwide. During 2006–2011, 77% of cases were tissue-confirmed. No cases of
vCJD were diagnosed or suspected. References
1. Holman RC, Belay ED, Christensen KY, et al. Human prion diseases in the
United States. PLoS One. 2010;5(1):e8521.
2. Ladogana A, Puopolo M, Croes EA, et al. Mortality from Creutzfeldt-Jakob
disease and related disorders in Europe, Australia, and Canada. Neurology
2005;64(9):1586-91.
August 27, 2012
>>> *** Five patients were less than 55 years old at the time of
death;
C. Human Prion Diseases in Washington State
Prior to 2005, surveillance for human prion diseases in Washington was
primarily conducted by death certificate review. This surveillance method
detected 3–9 cases of CJD per year. Since enhanced surveillance began in 2005,
7–17 cases have been reported per year (average 9 cases per year).
D. Reservoirs
It is unknown whether a reservoir exists for sporadic CJD.
3. CASE DEFINITIONS
A. Sporadic CJD* (2011)
1. Definite: Diagnosed by standard neuropathological techniques; and/or
immunocytochemically; and/or Western blot confirmed protease-resistant PrP; and
/or presence of scrapie-associated fibrils.
2. Probable:
• Rapidly progressive dementia; and
• At least two out of the following four clinical features: 1) myoclonus,
2) visual or cerebellar signs, 3) pyramidal/extrapyramidal signs, or 4) akinetic
mutism; and
• A positive result on at least one of the following laboratory tests;
and
1. A typical EEG (periodic sharp wave complexes) during an illness of any
duration;
2.A positive 14-3-3 cerebrospinal fluid (CSF) assay in patients with a
disease duration of less than 2 years; and/or
3.Magnetic resonance imaging (MRI) high signal abnormalities in caudate
nucleus and/or putamen on diffusion-weighted imaging (DWI) or fluid attenuated
inversion recovery (FLAIR); and
• The absence of an alternative diagnosis after routine investigation.
3. Possible:
• Progressive dementia; and
•At least two out of the following four clinical features: 1) myoclonus, 2)
visual or cerebellar signs, 3) pyramidal/extrapyramidal signs, or 4) akinetic
mutism; and
•The absence of a positive result for any of the three laboratory tests
that would classify a case as “probable” (see tests 1-3 above) and
•Duration of illness less than two years and
•The absence of an alternative diagnosis after routine investigation.
B. Iatrogenic CJD* (2011)
Progressive cerebellar syndrome in a recipient of human cadaveric-derived
pituitary hormone; or sporadic CJD with a recognized exposure risk, e.g.,
antecedent neurosurgery with dura mater implantation.
C. Familial CJD* (2011)
Definite or probable CJD plus definite or probable CJD in a first degree
relative; and/or neuropsychiatric disorder plus disease-specific PrP gene
mutation.
D. Variant CJD (2003)
1. Definite: Neuropathologic examination of brain tissue is required to
confirm a diagnosis of variant CJD. The following confirmatory features should
be present.
a. Numerous widespread kuru-type amyloid plaques surrounded by vacuoles in
both the cerebellum and cerebrum – florid plaques.
b. Spongiform change and extensive prion protein deposition shown by
immunohistochemistry throughout the cerebellum and cerebrum.
2. Suspected:
***a. Current age or age at death <55 a="a" all="all" autopsy="autopsy" brain="brain" cases="cases" cjd="cjd" div="div" for="for" however="however" is="is" physician-diagnosed="physician-diagnosed" recommended="recommended" years="years">
b. Psychiatric symptoms at illness onset and/or persistent painful sensory
symptoms (frank pain and/or dysesthesia).
c. Dementia, and development >4 months after illness onset of at least
two of the following five neurologic signs: poor coordination, myoclonus,
chorea, hyperreflexia, or visual signs. (If persistent painful sensory symptoms
exist, >4 months delay in the development of the neurologic signs is not
required).
d. A normal or an abnormal EEG, but not the diagnostic EEG changes often
seen in classic CJD.
e. Duration of illness of over 6 months.
f. Routine investigations of the patient do not suggest an alternative,
non-CJD diagnosis.
g. No history of receipt of cadaveric human pituitary growth hormone or a
dura mater graft.
h. No history of CJD in a first degree relative or prion protein gene
mutation in the patient.
NOTE
1. If a patient has the typical bilateral pulvinar high signal on MRI scan,
a suspected diagnosis of variant CJD requires the presence of a progressive
neuropsychiatric disorder, d, e, f and g of the above criteria, and four of the
following five criteria: 1) earlypsychiatric symptoms (anxiety, apathy,
delusions, depression, withdrawal); 2) persistent painful sensory symptoms
(frank pain and/or dysesthesia); 3) ataxia; 4) myoclonus or chorea or dystonia;
and 5) dementia.
2. A history of possible exposure to bovine spongiform encephalopathy (BSE)
such as residence or travel to a BSE-affected country after 1980 increases the
index of suspicion for a variant CJD diagnosis.
*These CDC diagnostic criteria for Creutzfeldt-Jakob Disease (2010) have
been adapted from: 1) Global Surveillance, diagnosis, and Therapy of Human
Transmissible spongiform Encephalopathies: Report of a WHO consultation,
February 9-11, 1998, Geneva, Switzerland; and 1) Zerr I, Kallenberg K, Summers
DM, et al. Brain 2009, 132; 2659-2668.
4. DIAGNOSIS AND LABORATORY SERVICES
SNIP...
A. Evaluate the Diagnosis
1. Determine the status (alive or deceased) of the patient. There is no
need to interview the next of kin unless variant CJD, iatrogenically-transmitted
CJD, a novel prion disease, or a CJD cluster is suspected.
SNIP...SEE FULL TEXT ;
Prion diseases in King County
Purpose of surveillance (reportable as a rare disease of public health
significance): •To detect cases of rare and emerging diseases •To understand the
epidemiology of rare and emerging diseases •To identify potentially exposed
persons
Local epidemiology:
In 2011, one confirmed case of sporadic CJD was reported in a King County
resident who expired.
In 2010, three cases of CJD were reported in King County residents. All
three persons died. Two were laboratory confirmed and likely sporadic cases, one
was a probable case. The cases ranged in age from 58 to 84 years of age.
From 2000 through 2010, Washington state received between 4 to 17 reports
each year.
>>>***Five patients were less than 55 years old at the time of
death;
>>> ***a. Current age or age at death <55 a="a" all="all" autopsy="autopsy" brain="brain" cases="cases" cjd="cjd" div="div" for="for" however="however" is="is" physician-diagnosed="physician-diagnosed" recommended="recommended" years="years">
>>> 1. Determine the status (alive or deceased) of the patient.
There is no need to interview the next of kin unless variant CJD,
iatrogenically-transmitted CJD, a novel prion disease, or a CJD cluster is
suspected.
THIS IS NOT SCIENTIFIC, and only promotes the further spreading of CJD via
the iatrogenic mode. TSE prion disease know no age limits. iatrogenic CJD
transmission KNOWS NO AGE GROUP. ALL AGE GROUPS SHOULD BE MANDATORY REPORTABLE
to prevent further transmission. to restrict reporting of TSE prion CJD victims
to the state only in the <55 and="and" blood="blood" dental="dental" div="div" exposure="exposure" further="further" iatrogenic="iatrogenic" medical="medical" promotes="promotes" surgical="surgical" the="the" transmission="transmission" tse.="tse." via="via">
ALSO, BY NOT interviewing the next of kin, you miss valuable information of
any potential route and source of the TSE prion agent, thus, you will not know
if it’s iCJD, or any other zoonotic human prion disease, or even information on
a potential cluster. so, by following these standards for CJD human TSE prion
disease surveillance, most all cases would then go down as simply sporadic CJD.
all iatrogenic CJD is, is sporadic CJD before route and source is documented.
looks to me as though officials simply don’t want to find any route and source
of the TSE prion agent. THE UKBSEnvCJD only theory lives on, corporate science
at it’s best, and the TSE prion agent will continue to spread because of this
bogus theory. it makes no sense, even less sense today. in my opinion, any
scientist that continues to believe in the UKBSEnvCJD only theory, and continues
to promote it, then they are part of the problem. ...tss
Saturday, December 29, 2012
MAD COW USA HUMAN TSE PRION DISEASE DECEMBER 29 2012 CJD CASE LAB REPORT
WASHINGTON STATE
A Tribute to Anna Henderson
32 year old victim in Washington
Woman’s brain tissue to be tested for disease
Pathology center to consider Creutzfeldt-Jakob disease
John Stucke The Spokesman-Review
September 28, 2012 - Updated: 9:12 a.m.
Disease investigators have sent a brain tissue sample of a deceased
32-year-old Spokane woman to a national research lab to be tested for
Creutzfeldt-Jakob disease, an incurable condition that has multiple variants,
including one called mad cow.
An autopsy of Amanda Greenwalt Wheaton noted that CJD was a potential
diagnosis. She died Aug. 24.
Officials with the Washington state Department of Health were notified of
the findings and sent a sample to the National Prion Disease Pathology
Surveillance Center, said department spokesman Donn Moyer.
The brain disease is rare. Of the cases that are confirmed, the most
frequent is a sporadic variant that can be hereditary.
A different, new variant of CJD transmitted to humans is called bovine
spongiform encephalopathy – or mad cow disease. No person is known to have
contracted the disease within the United States.
Mad cow disease created panic in the United Kingdom, where 180,000 cattle
became infected, leading to the eradication of 4.4 million, with carcasses
heaped into massive pyres.
More than 160 people in the United Kingdom have died from the
disease.
Friends and family of Wheaton said Thursday they were concerned about the
cause of her death. She gave birth to a daughter with her husband, Garick
Wheaton, last October.
“I just think the state needs to figure this out,” said her aunt, Debbie
Christie.
Moyer said the state investigates all suspected CJD cases that are
reported. There are several each year.
“People are concerned about this,” he said. The tissue samples sent to the
national lab help lead to an accurate diagnosis and also help researchers as
they attempt to track down an infection source and find a cure for all variants
of the disease.
Amanda Greenwalt Wheaton was the daughter of Ralph and Kay Greenwalt. She
worked at Deaconess Hospital as a pharmacy technician for several years and
later worked as a dental hygienist.
> Disease investigators have sent a brain tissue sample of a deceased
32-year-old Spokane woman to a national research lab to be tested for
Creutzfeldt-Jakob disease, an incurable condition that has multiple variants,
including one called mad cow.
> An autopsy of Amanda Greenwalt Wheaton noted that CJD was a potential
diagnosis. She died Aug. 24.
wonder what the USA Prion Unit Gambetti et al CWRU final diagnosis for this
case was, considering such a young age, and the fact the autopsy has already
noted CJD ?
no telling what this case will be. the last time a young case like this
happened in a mad cow state, there was a few cases, the final determination was
sporadic FFI, or anything unrelated to any mad cow $
look at the outbreak of CJD in King County way back in 1999, what’s up with
that, and could it be USA version of mad cow disease i.e. sporadic CJD, and see
how young the CJD victims were then ;
Briefly
Three Men Didn't Die From Mad-Cow Disease
Seattle Times Eastside Bureau
KING COUNTY
The mysterious deaths of three King County men were not caused by a brain
disorder linked to so-called mad-cow disease, health officials say.
Matthew Look, 42, of Issaquah, William Lapp, 41, of Carnation and Gary
Hollinquest, 43, of Kent had all suffered symptoms commonly associated with
Creutzfeldt-Jakob disease, which causes memory loss, dementia, loss of motor
function and eventually death.
In 1996 a variant of the disease was identified in England and linked to
mad-cow disease, possibly through beef consumption. At least 47 cases of the new
variant have been documented in Europe, but none in the United States.
The older strain generally strikes people over 55. The new strain can
strike younger people, so the three men's deaths raised the interest of health
officials. But University of Washington scientists proved last month that none
of the three men died of the new strain.
"We were not surprised by these results," said Dr. Ermias Belay, an
epidemiologist with the national Centers for Disease Control and
Prevention.
The cause of death for Lapp, who died in May, and Hollinquest was listed as
the old form of CJD. John Kobayashi, senior epidemiologist with the state Health
Department, said Look's cause of death last year hadn't been determined.
Copyright (c) 1999 Seattle Times Company, All Rights Reserved.
Wednesday, November 9, 2011 sporadic FFI or nvCJD in TEXAS ?
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 $$$
Greetings,
IT could also be that this sFFI is just another case of iCJD (via friendly
fire from the surgery for a colloid cyst of the third ventricle, and two
ventricular shunts were placed, one correctly in the left ventricle, while the
second ended in the right thalamus), some 20 years before the onset of symptoms
of this so called sFFI case, from some sub-type of sporadic CJD, now called
sporadic FFI ???
I believe it was Gambetti et al that coined this term sporadic FFI, from
some conspicuous sub-type of sporadic CJD possibly? seems they could not tie it
to a true FFI by diagnostic standards to date, so it was then termed a sFFI,
confusing matters even worse ;
A subtype of sporadic prion disease mimicking fatal familial insomnia
====================================
The familial mutations, Gajdusek proposed, lowered the barrier to such
accidental conversion. "Thus," he wrote in 1996, "with these mutations, this
ordinarily rare event becomes a ... dominant inherited trait." But Weissmann's
qualification still remained to be refuted: the mutations might simply allow
easier entry to a lurking virus. ...page 202 Deadly Feast
===================================
something to think about for sure.
but i interpret this as (1st not the gold standard, just my opinion;-), as
because of certain gene mutations, one or a family, would be more susceptible to
the many different strains of TSE, and the many different proven routes and
sources, (which will cause different symptoms, different incubation periods from
onset of clinical symptoms to death, different parts of the brain infected,
etc.). in other words, it's NOT the gene mutation that CAUSES the disease, but
the fact that it makes you more SUSCEPTIBLE, to the TSEs from the surrounding
environment, and PLUS accumulation, i think this plays a critical role. maybe
there is a one dose scenario, but i think there is more of the 'accumulators'
that go clinical, than the 'one dose'. and what is the threshold to sub-clinical
to clinical ?
anyway, just pondering out loud here.
also, for anyone interested, there are some studies with links to follow
here ;
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...
Rural and Regional Affairs and Transport References Committee
The possible impacts and consequences for public health, trade and
agriculture of the Government's decision to relax import restrictions on beef
Final report June 2010
2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr
Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric,
epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey
told the committee of his concerns regarding the lengthy incubation period for
transmissible spongiform encephalopathies, the inadequacy of current tests and
the limited nature of our current understanding of this group of diseases.49
2.66 Dr Fahey also told the committee that in the last two years a link has
been established between forms of atypical CJD and atypical BSE. Dr Fahey said
that: They now believe that those atypical BSEs overseas are in fact causing
sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad
sheep disease or a different form. If you look in the textbooks it looks like
this is just arising by itself. But in my research I have a summary of a
document which states that there has never been any proof that sporadic
Creutzfeldt-Jakob disease has arisen de novo-has arisen of itself. There is no
proof of that. The recent research is that in fact it is due to atypical forms
of mad cow disease which have been found across Europe, have been found in
America and have been found in Asia. These atypical forms of mad cow disease
typically have even longer incubation periods than the classical mad cow
disease.50
Atypical BSE in Cattle
To date the OIE/WAHO assumes that the human and animal health standards set
out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE
which include the H-type and L-type atypical forms. This assumption is
scientifically not completely justified and accumulating evidence suggests that
this may in fact not be the case. Molecular characterization and the spatial
distribution pattern of histopathologic lesions and immunohistochemistry (IHC)
signals are used to identify and characterize atypical BSE. Both the L-type and
H-type atypical cases display significant differences in the conformation and
spatial accumulation of the disease associated prion protein (PrPSc) in brains
of afflicted cattle. Transmission studies in bovine transgenic and wild type
mouse models support that the atypical BSE types might be unique strains because
they have different incubation times and lesion profiles when compared to C-type
BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian
hamster the resulting molecular fingerprint had changed, either in the first or
a subsequent passage, from L-type into C-type BSE.
In addition, non-human primates are specifically susceptible for atypical
BSE as demonstrated by an approximately 50% shortened incubation time for L-type
BSE as compared to C-type. Considering the current scientific information
available, it cannot be assumed that these different BSE types pose the same
human health risks as C-type BSE or that these risks are mitigated by the same
protective measures.
This study will contribute to a correct definition of specified risk
material (SRM) in atypical BSE. The incumbent of this position will develop new
and transfer existing, ultra-sensitive methods for the detection of atypical BSE
in tissue of experimentally infected cattle.
P.4.23
Transmission of atypical BSE in humanized mouse models
Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw
Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1
1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale,
Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research
Institute, Poland; 5Kansas State University (Previously at USDA National Animal
Disease Center), USA
Background: Classical BSE is a world-wide prion disease in cattle, and the
classical BSE strain (BSE-C) has led to over 200 cases of clinical human
infection (variant CJD). Atypical BSE cases have been discovered in three
continents since 2004; they include the L-type (also named BASE), the H-type,
and the first reported case of naturally occurring BSE with mutated bovine PRNP
(termed BSE-M). The public health risks posed by atypical BSE were largely
undefined.
Objectives: To investigate these atypical BSE types in terms of their
transmissibility and phenotypes in humanized mice. Methods: Transgenic mice
expressing human PrP were inoculated with several classical (C-type) and
atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation
time, characteristics and distribution of PrPSc, symptoms, and histopathology
were or will be examined and compared.
Results: Sixty percent of BASE-inoculated humanized mice became infected
with minimal spongiosis and an average incubation time of 20-22 months, whereas
only one of the C-type BSE-inoculated mice developed prion disease after more
than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse
brains was biochemically different from bovine BASE or sCJD. PrPSc was also
detected in the spleen of 22% of BASE-infected humanized mice, but not in those
infected with sCJD. Secondary transmission of BASE in the humanized mice led to
a small reduction in incubation time.*** The atypical BSE-H strain is also
transmissible with distinct phenotypes in the humanized mice, but no BSE-M
transmission has been observed so far.
Discussion: Our results demonstrate that BASE is more virulent than
classical BSE, has a lymphotropic phenotype, and displays a modest transmission
barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg
mice. The possibility of more than two atypical BSE strains will be discussed.
Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.
P26 TRANSMISSION OF ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN
HUMANIZED MOUSE MODELS
Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina
Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi
Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case
Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto
Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany;
4National Veterinary Research Institute, Poland; 5Kansas State University,
Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous
address: USDA National Animal Disease Center, Ames, IA 50010, USA
Classical BSE is a world-wide prion disease in cattle, and the classical
BSE strain (BSE-C) has led to over 200 cases of clinical human infection
(variant CJD). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have
been discovered in three continents since 2004. The first case of naturally
occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006
in the USA. The transmissibility and phenotypes of these atypical BSE
strains/isolates in humans were unknown. We have inoculated humanized transgenic
mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M
isolate. We have found that the atypical BSE-L strain is much more virulent than
the classical BSE-C.
*** The atypical BSE-H strain is also transmissible in the humanized
transgenic mice with distinct phenotype, but no transmission has been observed
for the BSE-M isolate so far.
III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE,
DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)
I ask Professor Kong ;
Thursday, December 04, 2008 3:37 PM Subject: RE: re--Chronic Wating Disease
(CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk
Assessment
''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE,
or less virulent than cBSE? just curious.....''
Professor Kong reply ; .....snip
''As to the H-BSE, we do not have sufficient data to say one way or
another, but we have found that H-BSE can infect humans. I hope we could publish
these data once the study is complete. Thanks for your interest.''
Best regards, Qingzhong Kong, PhD Associate Professor Department of
Pathology Case Western Reserve University Cleveland, OH 44106 USA
END...TSS
Thursday, December 04, 2008 2:37 PM
"we have found that H-BSE can infect humans."
personal communication with Professor Kong. ...TSS
BSE-H is also transmissible in our humanized Tg mice.
The possibility of more than two atypical BSE strains will be discussed.
Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.
Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate
Emmanuel E. Comoy1*, Cristina Casalone2, Nathalie Lescoutra-Etchegaray1,
Gianluigi Zanusso3, Sophie Freire1, Dominique Marcé1, Frédéric Auvré1,
Marie-Magdeleine Ruchoux1, Sergio Ferrari3, Salvatore Monaco3, Nicole Salès4,
Maria Caramelli2, Philippe Leboulch1,5, Paul Brown1, Corinne I. Lasmézas4,
Jean-Philippe Deslys1
1 Institute of Emerging Diseases and Innovative Therapies, CEA,
Fontenay-aux-Roses, France, 2 Istituto Zooprofilattico Sperimentale del
Piemonte, Turin, Italy, 3 Policlinico G.B. Rossi, Verona, Italy, 4 Scripps
Florida, Jupiter, Florida, United States of America, 5 Genetics Division,
Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts,
United States of America
Abstract
Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne
transmission of prions from slaughtered cattle with classical Bovine Spongiform
Encephalopathy (cBSE). Atypical forms of BSE, which remain mostly asymptomatic
in aging cattle, were recently identified at slaughterhouses throughout Europe
and North America, raising a question about human susceptibility to these new
prion strains.
Methodology/Principal Findings Brain homogenates from cattle with classical
BSE and atypical (BASE) infections were inoculated intracerebrally into
cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously
demonstrated to be susceptible to the original strain of cBSE. The resulting
diseases were compared in terms of clinical signs, histology and biochemistry of
the abnormal prion protein (PrPres). The single monkey infected with BASE had a
shorter survival, and a different clinical evolution, histopathology, and prion
protein (PrPres) pattern than was observed for either classical BSE or
vCJD-inoculated animals. Also, the biochemical signature of PrPres in the
BASE-inoculated animal was found to have a higher proteinase K sensitivity of
the octa-repeat region. We found the same biochemical signature in three of four
human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the
same country as the infected bovine.
Conclusion/Significance Our results point to a possibly higher degree of
pathogenicity of BASE than classical BSE in primates and also raise a question
about a possible link to one uncommon subset of cases of apparently sporadic
CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of
atypical strains should temper the urge to relax measures currently in place to
protect public health from accidental contamination by BSE-contaminated
products.
Citation: Comoy EE, Casalone C, Lescoutra-Etchegaray N, Zanusso G, Freire
S, et al. (2008) Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle
to a Primate. PLoS ONE 3(8): e3017. doi:10.1371/journal.pone.0003017 Editor:
Neil Mabbott, University of Edinburgh, United Kingdom
Received: April 24, 2008; Accepted: August 1, 2008; Published: August 20,
2008
Copyright: © 2008 Comoy et al. This is an open-access article distributed
under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the
original author and source are credited.
Funding: This work has been supported by the Network of Excellence
NeuroPrion.
Competing interests: CEA owns a patent covering the BSE diagnostic tests
commercialized by the company Bio-Rad.
* E-mail: emmanuel.comoy@cea.fr
snip...
In summary, we have transmitted one atypical form of BSE (BASE) to a
cynomolgus macaque monkey that had a shorter incubation period than monkeys
infected with classical BSE, with distinctive clinical, neuropathological, and
biochemical features; and have shown that the molecular biological signature
resembled that seen in a comparatively uncommon subtype of sporadic CJD. We
cannot yet say whether BASE is more pathogenic for primates (including humans)
than cBSE, nor can we predict whether its molecular biological features
represent a clue to one cause of apparently sporadic human CJD. However, the
evidence presented here and by others justifies concern about a potential human
health hazard from undetected atypical forms of BSE, and despite the waning
epizoonosis of classical BSE, it would be premature to abandon the precautionary
measures that have been so successful in reversing the impact of cBSE. We would
instead urge a gradual, staged reduction that takes into account the evolving
knowledge about atypical ruminant diseases, and both a permanent ban on the use
of bovine central nervous system tissue for either animal or human use, and its
destruction so as to eliminate any risk of environmental contamination.
Proc Natl Acad Sci U S A. 2004 March 2; 101(9): 3065–3070. Published online
2004 February 17. doi: 10.1073/pnas.0305777101 PMCID: PMC365745 Medical Sciences
Identification of a second bovine amyloidotic spongiform encephalopathy:
Molecular similarities with sporadic Creutzfeldt-Jakob disease
Cristina Casalone,*† Gianluigi Zanusso,†‡ Pierluigi Acutis,* Sergio
Ferrari,‡ Lorenzo Capucci,§ Fabrizio Tagliavini,¶ Salvatore Monaco,‡ and Maria
Caramelli*
Abstract
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are
mammalian neurodegenerative disorders characterized by a posttranslational
conversion and brain accumulation of an insoluble, protease-resistant isoform
(PrPSc) of the host-encoded cellular prion protein (PrPC). Human and animal TSE
agents exist as different phenotypes that can be biochemically differentiated on
the basis of the molecular mass of the protease-resistant PrPSc fragments and
the degree of glycosylation. Epidemiological, molecular, and transmission
studies strongly suggest that the single strain of agent responsible for bovine
spongiform encephalopathy (BSE) has infected humans, causing variant
Creutzfeldt-Jakob disease. The unprecedented biological properties of the BSE
agent, which circumvents the so-called ”species barrier” between cattle and
humans and adapts to different mammalian species, has raised considerable
concern for human health. To date, it is unknown whether more than one strain
might be responsible for cattle TSE or whether the BSE agent undergoes
phenotypic variation after natural transmission. Here we provide evidence of a
second cattle TSE. The disorder was pathologically characterized by the presence
of PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid
deposition in typical BSE cases, and by a different pattern of regional
distribution and topology of brain PrPSc accumulation. In addition, Western blot
analysis showed a PrPSc type with predominance of the low molecular mass
glycoform and a protease-resistant fragment of lower molecular mass than
BSE-PrPSc. Strikingly, the molecular signature of this previously undescribed
bovine PrPSc was similar to that encountered in a distinct subtype of sporadic
Creutzfeldt-Jakob disease.
snip...
Phenotypic Similarities Between BASE and sCJD. The transmissibility of CJD
brains was initially demonstrated in primates (27), and classification of
atypical cases as CJD was based on this property (28). To date, no systematic
studies of strain typing in sCJD have been provided, and classification of
different subtypes is based on clinical, neuropathological, and molecular
features (the polymorphic PRNP codon 129 and the PrPSc glycotype) (8, 9, 15,
19). The importance of molecular PrPSc characterization in assessing the
identity of TSE strains is underscored by several studies, showing that the
stability of given disease-specific PrPSc types is maintained upon experimental
propagation of sCJD, familial CJD, and vCJD isolates in transgenic PrP-humanized
mice (8, 29). Similarly, biochemical properties of BSE- and vCJD-associated
PrPSc molecules remain stable after passage to mice expressing bovine PrP (30).
Recently, however, it has been reported that PrP-humanized mice inoculated with
BSE tissues may also propagate a distinctive PrPSc type, with a
”monoglycosylated-dominant” pattern and electrophoretic mobility of the
unglycosylated fragment slower than that of vCJD and BSE (31). Strikingly, this
PrPSc type shares its molecular properties with the a PrPSc molecule found in
classical sCJD. This observation is at variance with the PrPSc type found in
M/V2 sCJD cases and in cattle BASE, showing a monoglycosylated-dominant pattern
but faster electrophoretic mobility of the protease-resistant fragment as
compared with BSE. In addition to molecular properties of PrPSc, BASE and M/V2
sCJD share a distinctive pattern of intracerebral PrP deposition, which occurs
as plaque-like and amyloid-kuru plaques. Differences were, however, observed in
the regional distribution of PrPSc. While in M/V2 sCJD cases the largest amounts
of PrPSc were detected in the cerebellum, brainstem, and striatum, in cattle
BASE these areas were less involved and the highest levels of PrPSc were
recovered from the thalamus and olfactory regions.
In conclusion, decoding the biochemical PrPSc signature of individual human
and animal TSE strains may allow the identification of potential risk factors
for human disorders with unknown etiology, such as sCJD. However, although BASE
and sCJD share several characteristics, caution is dictated in assessing a link
between conditions affecting two different mammalian species, based on
convergent biochemical properties of disease-associated PrPSc types. Strains of
TSE agents may be better characterized upon passage to transgenic mice. In the
interim until this is accomplished, our present findings suggest a strict
epidemiological surveillance of cattle TSE and sCJD based on molecular criteria.
Thursday, June 21, 2012
Clinical and Pathologic Features of H-Type Bovine Spongiform Encephalopathy
Associated with E211K Prion Protein Polymorphism
Atypical cases presumably of spontaneous or, in the case of E211K BSE-H,
genetic origins highlight that it may not be possible to eradicate BSE entirely
and that it would be hazardous to remove disease control measures such as
prohibiting the feeding of meat and bone meal to ruminants.
Thursday, June 21, 2012
Clinical and Pathologic Features of H-Type Bovine Spongiform Encephalopathy
Associated with E211K Prion Protein Polymorphism
Friday, May 11, 2012
Experimental H-type bovine spongiform encephalopathy characterized by
plaques and glial- and stellate-type prion protein deposits
***+++***
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease
update July 10, 2008 Friday, June 20, 2008
Here we go folks. AS predicted. THIS JUST OUT !
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. ...
Wednesday, October 27, 2010
A novel variant of human disease with a protease-sensitive prion protein
and heterozygosity methionine/valine at codon 129: Case report
Thursday, June 21, 2012
Clinical and Pathologic Features of H-Type Bovine Spongiform Encephalopathy
Associated with E211K Prion Protein Polymorphism
let's take a closer look at this new prionpathy or prionopathy, and then
let's look at the g-h-BSEalabama mad cow.
This new prionopathy in humans? the genetic makeup is IDENTICAL to the
g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like
this, ......wait, it get's better. this new prionpathy is killing young and old
humans, with LONG DURATION from onset of symptoms to death, and the symptoms are
very similar to nvCJD victims, OH, and the plaques are very similar in some
cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets
even better, the new human prionpathy that they claim is a genetic TSE, has no
relation to any gene mutation in that family. daaa, ya think it could be related
to that mad cow with the same genetic make-up ??? there were literally tons and
tons of banned mad cow protein in Alabama in commerce, and none of it
transmitted to cows, and the cows to humans there from ??? r i g h t $$$
ALABAMA MAD COW g-h-BSEalabama
In this study, we identified a novel mutation in the bovine prion protein
gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United
States of America. This mutation is identical to the E200K pathogenic mutation
found in humans with a genetic form of CJD. This finding represents the first
report of a confirmed case of BSE with a potential pathogenic mutation within
the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most
likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K
mutation.
her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS
Pathog. 4, e1000156; 2008).
This raises the possibility that the disease could occasionally be genetic
in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the
UK epidemic had most likely originated from such a mutation and argued against
the scrapierelated assumption. Such rare potential pathogenic PRNP mutations
could occur in countries at present considered to be free of BSE, such as
Australia and New Zealand. So it is important to maintain strict surveillance
for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many
countries still feed ruminant proteins to pigs). Removal of specified risk
material, such as brain and spinal cord, from cattle at slaughter prevents
infected material from entering the human food chain. Routine genetic screening
of cattle for PRNP mutations, which is now available, could provide additional
data on the risk to the public. Because the point mutation identified in the
Alabama animals is identical to that responsible for the commonest type of
familial (genetic) CJD in humans, it is possible that the resulting infective
prion protein might cross the bovine–human species barrier more easily. Patients
with vCJD continue to be identified. The fact that this is happening less often
should not lead to relaxation of the controls necessary to prevent future
outbreaks.
Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary
Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: maf12@cam.ac.uk Jürgen
A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier
Hall, Manhattan, Kansas 66506-5601, USA
NATURE|Vol 457|26 February 2009
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and
VPSPr PRIONPATHY (see mad cow feed in COMMERCE IN ALABAMA...TSS)
Tuesday, November 6, 2012
***Transmission of New Bovine Prion to Mice, Atypical Scrapie, BSE, and
Sporadic CJD, November-December 2012 update
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
Saturday, August 4, 2012
*** Final Feed Investigation Summary - California BSE Case - July 2012
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
Saturday, October 6, 2012
TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM
ENCEPHALOPATHIES 2011 Annual Report
Tuesday, November 02, 2010
*** BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex
only) diagnostic criteria CVL 1992
WASHINGTON STATE MAD COW DECEMBER 2003
February 17,2004
The Honorable Ann M. Veneman
Secretary of Agriculture
U.S. Department of Agriculture
1400 Independence Avenue, SW
Washington, DC 20250
Dear Madam Secretary:
We are writing with new information about the cow found to have Bovine
Spongiform Encephalopathy (BSE), commonly known as mad cow disease, in
Washington State in December 2003. Senior U.S. Department of Agriculture (USDA)
officials have repeatedly asserted that the BSE-infected cow was tested because
it was a "downer." We have now learned, however, that the co-manager of the
slaughter plant and two other eyewitnesses state that the cow stood and walked
on the day of slaughter. If this information is true, it could have serious
implications for both the adequacy of the national BSE surveillance system and
the credibility of the USDA.
In responding to the first case of mad cow disease in the United States,
USDA officials have emphasized that the USDA's BSE surveillance program
protected the public. This response was based on the assertion that the
BSE-infected cow could not walk, because the surveillance program is designed to
sample only downer cows and cows with symptoms of central nervous system
disease. You have said that the discovery of the cow was "a result of our
aggressive surveillance program" and "a clear indication that our surveillance
and detection program is working."
According to three individuals who actually observed the BSE-infected cow
the day it was slaughtered, however, the cow was not a downer. Contrary to
USDA's assertions, the cow appears to have been tested only because USDA had an
agreement to accept samples from nondowner cattle slaughtered at the Washington
plant.
We have reviewed (1) an affidavit from Thomas A. Ellestad, the co-manager
of Vern's Moses Lake Meats, Incorporated, which is the small facility where the
BSE-infected cow was slaughtered; (2) a declaration from Randy Hull, the hauler
who took the cow to slaughter; (3) the testimony of David Louthan, the employee
of Vern's Moses Lake Meats who says he killed the cow; (4) a USDA purchase order
for samples from Vern's Moses Lake Meats; and (5) other related documents. These
materials were provided to us by the Government Accountability Project (GAP), a
nonprofit organization that works with whistleblowers.
According to this information:
Three eyewitnesses say that the BSE-infected cow was not a downer. Hauler
Randy Hull states that the cow walked onto the hauling trailer at the fmth e day
of the slaughter. Plant co-manager Thomas Ellestad reports that after arriving
at his facility lying down, the cow stood up. Mr. Ellestad states, "the
BSE-infected cow was not a downer" and "efforts to portray our plant as a
'downer' plant could be considered a smokescreen." Plant employee David Louthan,
who recalls that he killed the cow, said, "That was a walking cow."
The BSE-infected cow was not tested because it was a downer cow. Vern's
Moses Lake Meats had a special contract with USDA under which USDA paid the
slaughterhouse to collect brain samples for testing from up to 1,000 cows
regardless of whether the cows were healthy, injured, or diseased. According to
Mr. Ellestad, his slaughterhouse did not test the cow as a downer.
USDA had information stating that the BSE-infected cow was not a downer. On
January 6,2004, Mr. Ellestad faxed a handwritten letter to USDA's Boulder
District office regarding his observations. Mr. Ellestad's fax disputed the
assertion that the BSE infected cow was sampled because it was a downer. The fax
stated, "the brain stem sample was not taken because this animal was
non-ambulatory." USDA has not released this information to Congress or the
public.
If the aforementioned is true, the information received from GAP could have
major ramifications. The information challenges a key principle guiding USDA's
surveillance program for BSE, which is that all infected cattle in the United
States will either be downer cows or show symptoms of central nervous system
disease. If the new information is accurate, USDA's surveillance program may
need to be significantly expanded.
The new information also raises questions about USDA's credibility. Above
all, the American people need to have confidence in what USDA reports about the
safety of the food supply. Contradictions between the new information and USDA's
statements can undermine that confidence. These contradictions require thorough
investigation and explanation.
The Honorable Ann M. Veneman February 17,2004 Page 3
Background
snip...see full text ;
WASHINGTON STATE MAD COW DECEMBER 2003
Summary Report
Epidemiological Investigation of Washington State BSE Case
March 2004
*** California BSE mad cow beef recall, QFC, CJD, and dead stock downer
livestock
snip...
QFC's Delayed Mad Cow Response Draws Lawsuit
... subsidiary of Kroger , claiming the grocery store chain should
... beef potentially tainted with "mad cow disease
... beef at approximately 40 stores across Washington.
snip...
SUPERIOR COURT OF THE STATE OF WASHINGTON FOR KING COUNTY
JILL CROWSON, ET AL., PLAINTIFFS
VS
QUALITY FOOD CENTERS, INC., an Ohio corporation Defendent
NO. 04-2-05608-0 SEA
snip...
The Court hereby GRANTS the defendant's motion to dismiss the plaintiff's
claims based on a manufacturer's strict liability (Counts I and II) and DENIES
the defendant's motion to dismiss the plaintiff's claim of negligence by a
product seller (Count III).
DATED this 14th day of June, 2004 snip...
Date Filed: March 5, 2004 Court: King County Superior Court (Washington)
Location: Seattle Ticker Symbol: NYSE:KR
Join This Suit Tell a Friend
Consumers filed a proposed class-action lawsuit against Quality Food
Centers (QFC), a subsidiary of Kroger (NYSE: KR), claiming the grocery store
chain should have used information gathered through its customer loyalty program
to warn those who purchased beef potentially tainted with ?mad cow disease.? The
USDA issued a recall notice for the meat on December 23, 2003. QFC sold the meat
through its approximately 40 stores across Washington.
The suit claims that even though QFC had the ability to quickly warn every
customer who purchased the potentially deadly meat if they used the QFC
Advantage Card at the time of purchase, the grocery store neglected to do so.
The suit seeks to represent every consumer in Washington state who
purchased the recalled meat from QFC.
Recent Updates
June 14, 2004 - the King County Superior Court gave the green light to a
suit claiming QFC didn't do enough to warn customers about beef potentially
tainted with 'mad cow disease,' finding enough questions about the beef and
QFC's responsibility to explore in the courtroom.
Read the court order.
QFC - 'Mad Cow' Frequently Asked Questions
The Suit
What is the key issue in this suit?
On December 23, 2003, the United States Department of Agriculture (USDA)
recalled more than 10,000 pounds of raw beef that could have been exposed to
bovine spongiform encephalopathy (BSE). Humans consuming BSE-tainted meat can
contract Creutzfeldt-Jakob Disease (vCJD), an always-fatal condition.
QFC sold this meat throughout its stores in Washington. Even though QFC had
the ability to quickly warn every customer who purchased the potentially deadly
meat if they used the QFC advantage card at the time of purchase, the grocery
store neglected to do so, the suit alleges.
Who does the suit seek to represent?
The suit seeks to represent all persons who purchased recalled meat from
any QFC store in the state of Washington.
Who are the defendants?
Quality Food Centers, or QFC. Once a local, Northwest company, QFC is now a
wholly owned subsidiary of the grocery chain giant, Kroger.
What does the suit seek?
The suit asks the court to order QFC to establish a medical monitoring fund
which would allow those who purchased and consumed the meat to seek medical
care, checking for, and if necessary, treating --- the infection of vCJD. The
suit also seeks the creation of a medical notification system, allowing those
who may have been exposed to the disease to receive periodic updates on research
and treatment of vCJD. The suit also seeks unspecified damages for the
plaintiffs. Does the suit claim QFC violated specific laws?
Yes. The lawsuit claims QFC violated the Washington Product Liability Act.
In addition, the suit claims QFC was negligent by not warning consumers of the
dangers associated with the affected meat. Where was the lawsuit filed?
The suit was filed in King County Superior Court on March 4, 2004.
How do I determine if I qualify to join the lawsuit?
If you have a QFC Advantage card and believe that you bought recalled meat
from a QFC store, you may be eligible to join the lawsuit. Click here to fill
out the sign-up request form, or you can contact Hagens Berman attorneys.
QFC
What is the QFC Advantage Card?
The Advantage Card is known in the grocery industry as a Customer Loyalty
Card. Customers who sign up for QFC's Advantage Card receive special discounts
on selected items, but gives the grocery store chain the ability to track
consumers purchases in order to enhance their marketing efforts. In addition,
grocery chains which offer affinity card programs often use the database and
shopping pattern data to send users coupons and other marketing material.
According to the complaint, QFC tracks every purchase made by consumers
presenting the Advantage Card, including product description, date of purchase,
store of purchase and the price, and saves that data with customer contact
information.
What was QFC's response to the meat recall?
On Dec. 23, 2003, QFC received notice from the U.S. Department of
Agriculture (USDA) of a recall of approximately 10,410 lbs. of raw meat that may
have been contaminated with the infectious agent that causes mad cow disease.
QFC did not act immediately on the recall notice but initially responded by
denying that it had any of the tainted meat. On December 24 QFC pulled the meat
from its shelves, but the company took no steps to directly warn consumers. It
was not until Dec. 27 that QFC posted small signs in its stores recalling the
tainted beef, according to the complaint. During that four day period when QFC
was silent hundreds of consumers may have eaten the meat.
Can QFC determine if an Advantage Card holder purchased the potentially
dangerous meat?
Yes. In fact, consumers can now contact QFC directly and the company will
provide information about meat purchases ? but only if you ask. Hundreds of
other consumers who purchased the meat and are unaware of the situation have not
heard from QFC, the complaint states.
Why was QFC sued even though they pulled the meat? Under Washington law
since QFC ground the meat it is deemed a manufacturer and is strictly liable for
any unsafe product. In addition QFC possessed specific and easily obtainable
information on which customers purchased the recalled meat, but did not act to
inform customers, the suit states. Considering the potential danger and risk of
worry for consumers, and the ease of contacting consumers using database
information, simply pulling the meat from the shelves and belatedly posting
small signs was not an adequate response, according to the complaint.
What information on customer purchases does QFC track with the Advantage
Card? QFC tracks every purchase that a customer with an Advantage Card makes,
regardless of whether discounts are offered or not, according to the complaint.
Does the recently announced larger-than-expected recall of beef affect the
lawsuit? No. Regardless of the size of the beef recall, attorneys believe the
facts in the case remain the same.
How can I find out if I bought recalled meat from QFC? If you believe that
you may have purchased recalled meat from a QFC store, and you have an Advantage
Card, you can contact QFC and ask if your record shows you purchased recalled
beef. You can contact QFC at 866-221-4141.
Isn't QFC prohibited by privacy laws from contacting consumers with
warnings like this? No ? the suit notes that the company will return car keys
returned to the store if the keys have an Advantage Card attached. According the
complaint, If QFC can return car keys by mail, why can't they send a notice
saying the meat a customer purchased in their store could cause an incurable,
fatal disease? Further privacy laws would prevent QFC from disclosing
information to third parties, disclosing the information to the customer whose
card it is does not violate privacy laws. For example, if a trade group wanted
to know the names of consumers who purchased a given drug sold at QFC,
disclosure of that private information might be a privacy concern. However,
disclosure to a consumer of his own records is not.
Mad Cow Disease
What is Mad Cow disease? In cows, mad cow disease is defined as bovine
spongiform encephalopathy (BSE), and is a progressive neurological disease. The
human disease variant is know as Creutzfeldt-Jakob Disease (vCJD), which is a
rare brain disorder that causes a rapid, progressive dementia and is always
fatal, according to the complaint.
Where can I get more information on Mad Cow disease? The USDA provides
information on the disease at www.usda.gov/.
What should I do if I believe that I've eaten recalled meat? According to
the complaint, no screening tests or treatments have been found for
Creutzfeldt-Jakob disease. Those who suspect they've eaten recalled meat should
contact their physician for more information.
Do Stores That Offer Loyalty Cards Have a Duty to Notify Customers of
Product Safety Recalls? A Recent Suit Raises This Novel Question By ANITA
RAMASASTRY ----
Thursday, Aug. 05, 2004
An interesting new Washington state court suit raises an important
question: If a retailer benefits from collecting personally identifiable
information about its customers, does it have a corresponding duty to use such
data to alert its customers that products they've bought have been recalled for
health or safety reasons? And if so, could turning over private data to
companies actually create benefits, as well as privacy risks, for the
consumer?
In the suit, consumer Jill Crowson is suing her grocery store -- Quality
Food Center (QFC), a subsidiary of Kroger -- for negligent infliction of
emotional distress and disregard of a "duty to warn" under the Washington
Product Liability Act. Crowson alleges in her complaint that QFC failed to alert
her family that ground beef it had sold them had been recalled in December's
mad-cow scare.
Yet, Crowson says, QFC easily could have done so through information it
maintained connected with her Advantage card - a "loyalty card" that meant QFC
had Crowson's name, address and purchasing information. According to her
complaint, QFC tracks every purchase made by consumers presenting the Advantage
Card, including product description, date of purchase, store of purchase and the
price, and saves that data alongside customer contact information. Now, Crowson
says, her family members "feel like walking time bombs" knowing they may be
infected with the human form of mad-cow disease which the complaint states may
have an up-to-30-year incubation period. And they are not the only ones: Crowson
is seeking class action status for herself and what she believes are "hundreds"
of similarly-situated Washington customers at QFC's approximately 40 stores in
the state.
Some lawyers think Crowson's suit is a stretch. Federal law does not impose
on companies a specific duty to notify consumers when tainted meat is recalled
under the direction of the U.S. Department of Agriculture (USDA), as was the
case here. Also, Crowson and her family, and the class she seeks to represent,
are suing based on fear (and possible future harm), not current illness.
Moreover, the chance they will actually get Mad Cow Disease some time in the
future are apparently remote.
Nevertheless, the lawsuit has strong intuitive appeal: QFC could have saved
the Crowsons and others like them a lot of worry, and perhaps sleepless nights,
with what appears would have been minimal effort, using information at its
digital fingertips. And the court has already once refused to dismiss it -
finding that there were sufficient factual questions about the beef and about
QFC's responsibility to the Crowsons, to merit further exploration of the
evidence, through discovery and in the courtroom.
Regardless of the outcome of Crowson's suit, it underscores the need for
retailers and policymakers to examine what sort of responsibilities come with
private data gathering under loyalty card schemes.
The Lawsuit: The Chronology of Facts Alleged, and the Loyalty Card at
Issue
On December 22 and 23, 2003, Crowson bought ground beef from a QFC store.
Also on December 23, 2003, the USDA recalled Washington beef after it confirmed
that a cow slaughtered in Washington had been infected with Mad Cow Disease. But
Crowson says QFC did not pull the affected meat from its shelves until December
24, and did not post signs in its stores announcing the recall until December
27. By then, the Crowson family had eaten the meat.
Crowson states that she only learned of the recall by reading an article in
her local newspaper. She said she subsequently called the supermarket chain,
then faxed QFC a letter asking that her purchase be traced through her QFC
Advantage card. On January 10, she was notified that her ground beef purchase
was indeed from the recalled batch.
Crowson says that what QFC allegedly did in response to the recall -
pulling the beef from shelves the next day, and posting signs three days after
that -- was far from enough. She says it should have immediately warned
customers who had bought possibly tainted meat through newspaper, radio and
television advertising -- and by contacting individually those who, like her,
had Advantage cards. Its failure to do so, she says, is what makes the company
liable to her and other shoppers.
The Advantage Card is known in the retail industry as a customer "loyalty
card" - providing discounts on specific items, in exchange for consumer
information that will aid in better tailoring the company's marketing efforts.
Combining the data from one's loyalty card application with data from other
commercial databases or public records (for examples, mortgage records, or court
filings) can often allow a very specific profile of each consumer. Some states
limit the types of information that a grocery store can collect from you when
you register for a loyalty card. For example, California state law prohibits a
grocery store from requiring that you turn over your social security or your
driver's license number.
Companies, of course, stress the potential savings that might result from
use of a loyalty card. Consider, for instance, the sales pitch on the QFC
website it reads: "If you don't have a QFC Advantage Card, you're missing out!
The Advantage Card is a powerful new way to save on the groceries you buy every
day. It gives you the best of all possible worlds: premium quality, superb
service and lower prices. That's something no other grocery store can match. So
make sure you take advantage of the big savings."
Privacy advocates complain that loyalty cards result in the improper use -
and, often, sale to third parties - of customers' private information. QFC
apparently doesn't sell customers' data to third parties, however. Its website
promises that "QFC will not release your name to any list service or
manufacturer, and that such information will be held in the strictest of
confidence-even within our company."
Privacy advocates also warn, however, that even if third-party sales of
data are not allowed, the data compiled can always be accessed with a subpoena
or warrant and used against the customer in court proceedings. Meanwhile,
consumer advocates claim that certain loyalty cards don't really offer the
savings they promise. Nevertheless, numerous stores employ loyalty cards.
Turning the Privacy Debate on Its Head: With Great Information, Comes Great
Responsibility?
The Crowson lawsuit turns the privacy debate on its head. Typically,
privacy advocates ask retailers to safeguard the personal information they
collect about their shoppers. In this case, in contrast, plaintiff is asking
that QFC delve into its database to notify her about a meat recall.
QFC does this very thing if a consumer loses his or her keys with an
Advantage Card attached to them - returning the keys free of charge. So
Crowson's attorney, Steve Berman, asks: "If they can contact you over a lost set
of car keys, why couldn't they contact you and tell you that the beef you
purchased could kill you?"
According to some news reports, QFC was reluctant to call customers
regarding the recall based on privacy concerns. But in this case, the concerns
seem misplaced. No privacy law is violated when a consumer communicates with the
customer herself regarding private information - indeed, every offer the
customer receives is, in a sense, this kind of communication. When the customer
is receiving personalized discounts based on her purchase history, why can't she
receive personalized health and safety warnings based on that history,
too?
Was There a Duty to Warn Here?
From the law's perspective, the question will be not whether QFC ideally
should have warned the Crowsons - of course it should have. The question will be
if it had a legal duty to do so. Such a duty would come from either the common
law of torts, which allows claims where there is a duty to behave reasonably to
prevent foreseeable harm to others. . Or it might come from the Washington
product liability statute - which, as noted above, creates a "duty to warn" in
certain situations.
And of course, if there is no current duty, the legislature may see fit to
pass a statute creating such a duty. :It may seem more prudent, however, for
retailers to voluntarily assume such a responsibility. When companies benefit
from collecting customer information, shouldn't they also assume a duty to
protect customers from known risks associated with that very information? Some
risks, of course, may be a matter of opinion. But this one was not: The fact of
the risk was acknowledged by the USDA recall of the meat. With this kind of
clear notice of the risk, it seems that QFC either does - or ought to - have a
duty to protect customers from this risk.
Of course, should a retailer not wish to take on this responsibility, it
can also change its loyalty program. QFC and other retailers could still track
consumer purchases without asking them for personally identifiable information.
FindLaw's Writ - Ramasastry: Mad Cow in the USA
http://writ.corporate.findlaw.com/ramasastry/20031230.html
http://writ.corporate.findlaw.com/ramasastry/20031230.html
Family to sue grocery chain
A Seattle family that ate beef linked to the US's only known case of BSE
has filed a classaction lawsuit against the grocery chain QFC, claiming the
company negligently exposed them and others to "highly hazardous" meat and did
not properly notify them that they had bought it.34 The suit contends that Jill
Crowson and her family bought and later ate ground beef from their local QFC
that was part of a batch processed at Vern's Moses Lake Meats on 9 December 2003
and included meat from the diseased Holstein. The beef was later shipped to
wholesalers and retailers in Washington, Oregon, California, Idaho, Montana and
Nevada.
After government scientists confirmed on 23 December that the Holstein was
infected with BSE, businesses began pulling potentially affected beef from store
shelves under a voluntary recall. But, the family's suit claims, although QFC
was aware of the recall, the store did not begin pulling the beef from about 40
of its stores until 24 December. The company also did not try to warn customers
about the recalled beef until 27 December – and only then with small,
inconspicuous signs inside the stores, the suit claims. The family only learned
QFC had sold any of the beef in question after reading a news story on 10
January about a man who discovered his family had eaten affected beef that he
bought at a local QFC store, Crowson said. She later called QFC and faxed the
company a signed letter asking that it track purchases made on her QFC Advantage
Card, and on 12 January the company notified Crowson that the beef she bought
and served to her family was, in fact, part of the recalled batch, she said.
The family seeks unspecified damages for emotional distress and medical
monitoring costs. Crowson said her reason for bringing the lawsuit is not about
money. "The more I've thought about this, the angrier I've gotten," she said.
Neither the company nor its parent corporation, Kroger, have commented.
snip...see full text and more here on this litigation ;
California BSE mad cow beef recall, QFC, CJD, and dead stock downer
livestock California BSE mad cow beef in commerce recall, QFC, and CJD
On January 6, 2004, over 2 weeks from recall initiation, USDA determined
that the beef went to only six states-Washington, Oregon, California, Nevada,
Idaho, and Montana-and that no beef went to Alaska, Hawaii, or Guam. To reach
that conclusion, USDA used the distribution lists, shipping records, and sales
invoices that it received from companies to piece together exactly where the
recalled beef may have been sent. The lists showed that 713 customers may have
received the recalled beef; 6 of those may have received beef from more than one
source. USDA determined that 176 customers on the lists did not actually receive
recalled beef, including the customers in Guam and Hawaii. USDA's review also
indicated that recalled beef was probably not shipped to Alaska or Utah, and
USDA checked 2 retailers in Alaska and 3 retailers in Utah to confirm that was
the case. In total, USDA conducted verification checks on 537 of the 713
customers on the lists. USDA's initial checks identified an additional 45
customers that may have received the recalled beef that were not included on the
distribution lists, for a total of 582 verification checks. Figure 4 summarizes
USDA's verification efforts during the recall.
SNIP...
USDA's press release stated that the recall involved 10,410 pounds of beef
products, and the USDA recall coordinator for this recall told us that
downstream processors mixed the recalled beef with nonrecalled beef, for a total
of more than 38,000 pounds of beef that was distributed at the secondary
customer level. According to USDA officials involved with the recall, the
precise amount of meat that was sold at the retail level is unknown because
retailers at the tertiary level further mixed nonrecalled meat with potentially
contaminated meat. USDA told us that more than 64,000 pounds of beef was
ultimately returned or destroyed by customers, and that, because of the mixing,
it was not able to determine how much of the original 10,410 pounds of recalled
beef was contained in the 64,000 pounds that were recovered.
Parts of the BSE-infected animal slaughtered on December 9, 2003, were not
used for food, but they were sent to renderers to be separated into raw
materials, such as proteins and blood. Rendered materials are used for many
purposes, including cosmetics and vaccines. FDA has jurisdiction over
renderers.
SNIP...
QFC sued over mad cow case
===============================================================
P03.141
Aspects of the Cerebellar Neuropathology in Nor98
Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National
Veterinary Insitute, Sweden; 2National Veterinary Institute,
Norway Nor98 is a prion disease of old sheep and goats. This atypical form
of scrapie was first described in Norway in 1998. Several features of Nor98 were
shown to be different from classical scrapie including the distribution of
disease associated prion protein (PrPd) accumulation in the brain. The
cerebellum is generally the most affected brain area in Nor98. The study here
presented aimed at adding information on the neuropathology in the cerebellum of
Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A
panel of histochemical and immunohistochemical (IHC) stainings such as IHC for
PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers
for phagocytic cells were conducted. The type of histological lesions and tissue
reactions were evaluated. The types of PrPd deposition were characterized. The
cerebellar cortex was regularly affected, even though there was a variation in
the severity of the lesions from case to case. Neuropil vacuolation was more
marked in the molecular layer, but affected also the granular cell layer. There
was a loss of granule cells. Punctate deposition of PrPd was characteristic. It
was morphologically and in distribution identical with that of synaptophysin,
suggesting that PrPd accumulates in the synaptic structures. PrPd was also
observed in the granule cell layer and in the white matter. The pathology
features of Nor98 in the cerebellum of the affected sheep showed similarities
with those of sporadic Creutzfeldt-Jakob disease in humans.
***The pathology features of Nor98 in the cerebellum of the affected sheep
showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
PR-26
NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS
R. Nonno1, E. Esposito1, G. Vaccari1, E. Bandino2, M. Conte1, B.
Chiappini1, S. Marcon1, M. Di Bari1, S.L. Benestad3, U. Agrimi1 1 Istituto
Superiore di Sanità, Department of Food Safety and Veterinary Public Health,
Rome, Italy (romolo.nonno@iss.it); 2 Istituto Zooprofilattico della Sardegna,
Sassari, Italy; 3 National Veterinary Institute, Department of Pathology, Oslo,
Norway
Molecular variants of PrPSc are being increasingly investigated in sheep
scrapie and are generally referred to as "atypical" scrapie, as opposed to
"classical scrapie". Among the atypical group, Nor98 seems to be the best
identified. We studied the molecular properties of Italian and Norwegian Nor98
samples by WB analysis of brain homogenates, either untreated, digested with
different concentrations of proteinase K, or subjected to enzymatic
deglycosylation. The identity of PrP fragments was inferred by means of
antibodies spanning the full PrP sequence. We found that undigested brain
homogenates contain a Nor98-specific PrP fragment migrating at 11 kDa (PrP11),
truncated at both the C-terminus and the N-terminus, and not N-glycosylated.
After mild PK digestion, Nor98 displayed full-length PrP (FL-PrP) and
N-glycosylated C-terminal fragments (CTF), along with increased levels of PrP11.
Proteinase K digestion curves (0,006-6,4 mg/ml) showed that FL-PrP and CTF are
mainly digested above 0,01 mg/ml, while PrP11 is not entirely digested even at
the highest concentrations, similarly to PrP27-30 associated with classical
scrapie. Above 0,2 mg/ml PK, most Nor98 samples showed only PrP11 and a fragment
of 17 kDa with the same properties of PrP11, that was tentatively identified as
a dimer of PrP11. Detergent solubility studies showed that PrP11 is insoluble in
2% sodium laurylsorcosine and is mainly produced from detergentsoluble,
full-length PrPSc. Furthermore, among Italian scrapie isolates, we found that a
sample with molecular and pathological properties consistent with Nor98 showed
plaque-like deposits of PrPSc in the thalamus when the brain was analysed by
PrPSc immunohistochemistry. Taken together, our results show that the
distinctive pathological feature of Nor98 is a PrP fragment spanning amino acids
~ 90-155. This fragment is produced by successive N-terminal and C-terminal
cleavages from a full-length and largely detergent-soluble PrPSc, is produced in
vivo and is extremely resistant to PK digestion.
*** Intriguingly, these conclusions suggest that some pathological features
of Nor98 are reminiscent of Gerstmann-Sträussler-Scheinker disease.
A newly identified type of scrapie agent can naturally infect sheep with
resistant PrP genotypes
Annick Le Dur*,?, Vincent Béringue*,?, Olivier Andréoletti?, Fabienne
Reine*, Thanh Lan Laï*, Thierry Baron§, Bjørn Bratberg¶, Jean-Luc Vilotte?,
Pierre Sarradin**, Sylvie L. Benestad¶, and Hubert Laude*,? +Author Affiliations
*Virologie Immunologie Moléculaires and ?Génétique Biochimique et Cytogénétique,
Institut National de la Recherche Agronomique, 78350 Jouy-en-Josas, France;
?Unité Mixte de Recherche, Institut National de la Recherche Agronomique-Ecole
Nationale Vétérinaire de Toulouse, Interactions Hôte Agent Pathogène, 31066
Toulouse, France; §Agence Française de Sécurité Sanitaire des Aliments, Unité
Agents Transmissibles Non Conventionnels, 69364 Lyon, France;
**Pathologie Infectieuse et Immunologie, Institut National de la Recherche
Agronomique, 37380 Nouzilly, France; and ¶Department of Pathology, National
Veterinary Institute, 0033 Oslo, Norway
***Edited by Stanley B. Prusiner, University of California, San Francisco,
CA (received for review March 21, 2005)
Abstract
Scrapie in small ruminants belongs to transmissible spongiform
encephalopathies (TSEs), or prion diseases, a family of fatal neurodegenerative
disorders that affect humans and animals and can transmit within and between
species by ingestion or inoculation. Conversion of the host-encoded prion
protein (PrP), normal cellular PrP (PrPc), into a misfolded form, abnormal PrP
(PrPSc), plays a key role in TSE transmission and pathogenesis. The intensified
surveillance of scrapie in the European Union, together with the improvement of
PrPSc detection techniques, has led to the discovery of a growing number of
so-called atypical scrapie cases. These include clinical Nor98 cases first
identified in Norwegian sheep on the basis of unusual pathological and PrPSc
molecular features and "cases" that produced discordant responses in the rapid
tests currently applied to the large-scale random screening of slaughtered or
fallen animals. Worryingly, a substantial proportion of such cases involved
sheep with PrP genotypes known until now to confer natural resistance to
conventional scrapie. Here we report that both Nor98 and discordant cases,
including three sheep homozygous for the resistant PrPARR allele (A136R154R171),
efficiently transmitted the disease to transgenic mice expressing ovine PrP, and
that they shared unique biological and biochemical features upon propagation in
mice. *** These observations support the view that a truly infectious TSE agent,
unrecognized until recently, infects sheep and goat flocks and may have
important implications in terms of scrapie control and public health.
Monday, December 1, 2008
When Atypical Scrapie cross species barriers
Authors
Andreoletti O., Herva M. H., Cassard H., Espinosa J. C., Lacroux C., Simon
S., Padilla D., Benestad S. L., Lantier F., Schelcher F., Grassi J., Torres, J.
M., UMR INRA ENVT 1225, Ecole Nationale Veterinaire de Toulouse.France;
ICISA-INlA, Madrid, Spain; CEA, IBiTec-5, DSV, CEA/Saclay, Gif sur Yvette cedex,
France; National Veterinary Institute, Postboks 750 Sentrum, 0106 Oslo, Norway,
INRA IASP, Centre INRA de Tours, 3738O Nouzilly, France.
Content
Atypical scrapie is a TSE occurring in small ruminants and harbouring
peculiar clinical, epidemiological and biochemical properties. Currently this
form of disease is identified in a large number of countries. In this study we
report the transmission of an atypical scrapie isolate through different species
barriers as modeled by transgenic mice (Tg) expressing different species PRP
sequence.
The donor isolate was collected in 1995 in a French commercial sheep flock.
inoculation into AHQ/AHQ sheep induced a disease which had all
neuro-pathological and biochemical characteristics of atypical scrapie.
Transmitted into Transgenic mice expressing either ovine or PrPc, the isolate
retained all the described characteristics of atypical scrapie.
Surprisingly the TSE agent characteristics were dramatically different
v/hen passaged into Tg bovine mice. The recovered TSE agent had biological and
biochemical characteristics similar to those of atypical BSE L in the same mouse
model. Moreover, whereas no other TSE agent than BSE were shown to transmit into
Tg porcine mice, atypical scrapie was able to develop into this model, albeit
with low attack rate on first passage.
Furthermore, after adaptation in the porcine mouse model this prion showed
similar biological and biochemical characteristics than BSE adapted to this
porcine mouse model. Altogether these data indicate.
(i) the unsuspected potential abilities of atypical scrapie to cross
species barriers
(ii) the possible capacity of this agent to acquire new characteristics
when crossing species barrier
These findings raise some interrogation on the concept of TSE strain and on
the origin of the diversity of the TSE agents and could have consequences on
field TSE control measures.
Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease
Are Encoded by Distinct Prion Types
Wiebke M. Wemheuer,* Sylvie L. Benestad,† Arne Wrede,* Ulf Schulze-Sturm,*
Wilhelm E. Wemheuer,‡ Uwe Hahmann,* Joanna Gawinecka,§ Ekkehard Schu¨ tz,‡ Inga
Zerr,§ Bertram Brenig,‡ Bjørn Bratberg,† Olivier Andre´ oletti,¶ and Walter J.
Schulz-Schaeffer*
From the Prion and Dementia Research Unit,* Department of Neuropathology,
and the National Transmissible Spongiform Encephalopathies Reference Center,§
Department of Neurology, University Medical Center Goettingen, Goettingen,
Germany; the Department of Pathology,† National Veterinary Institute, Oslo,
Norway; the Institute of Veterinary Medicine,‡ Faculty for Agricultural
Sciences, University of Goettingen, Goettingen, Germany; and Animal Health,¶
Interactions Hôte Agent Pathogène, Ecole Nationale Ve´te´rinaire de Toulouse,
Toulouse, France
Transmissible spongiform encephalopathies such as scrapie in sheep,
Creutzfeldt-Jakob disease (CJD) in humans, and bovine sporadic encephalopathy in
cattle are characterized by the accumulation of a misfolded protein: the
pathological prion protein. Ever since bovine sporadic encephalopathy was
discovered as the likely cause of the new variant of CJD in humans, parallels
between human and animal transmissible spongiform encephalopathies must be
viewed under the aspect of a disease risk for humans. In our study we have
compared prion characteristics of different forms of sheep scrapie with those of
different phenotypes of sporadic CJD. The disease characteristics of sporadic
CJD depend considerably on the prion type 1 or 2. Our results show that there
are obvious parallels between sporadic CJD type 1 and the so-called
atypical/Nor98 scrapie. These parelleles apply to the deposition form of
pathological prion protein in the brain, detected by the paraffin-embedded-
tissue blot and the prion aggregate stability with regard to denaturation by the
chaotropic salt guanidine hydrochloride. The same applies to sporadic CJD type 2
and classical scrapie. The observed parallels between types of sporadic CJD and
types of sheep scrapie demonstrate that distinct groups of prion disease exist
in different species. This should be taken into consideration when discussing
interspecies transmission. (Am J Pathol 2009, 175:2566–2573; DOI:
10.2353/ajpath.2009.090623)
snip...
Discussion
In humans, different prion types are linked with clinically and
neuropathologically distinct prion diseases.8 The present work emphasizes that
the differences in deposition characteristics and stability with regard to
denaturation between atypical/Nor98 and classical scrapie also account for
different prion types. Moreover, the two scrapie types that have been
characterized show a number of striking similarities with human PrPSc types in
sporadic CJD. Hence, we propose that the existence of different PrPSc types
might be a common denominator of prion diseases in humans and animals. Since
these two prion types show an across-the-species comparability with similar
biochemical and pathological
characteristics, it is most likely that they exist due to a different
conformational pattern of the disease-related prion protein.
snip...
Conclusion
As the prion protein is a highly conserved protein in terms of evolution,
parallels between characteristics of prion types in TSEs of different species
are of interest. In the present study, we report previously unknown similarities
between sheep scrapie forms and human sporadic CJD types. We propose that the
observed similarities between sheep scrapie and sporadic CJD in humans justify
new interspecies groups of prion diseases in which prion types, not prion
strains, are the major determinant for prion disease forms. While epidemiology
implies that classical scrapie is not related to human TSEs,47 the
atypical/Nor98 scrapie risk for human transmission has not yet been elucidated.
Currently there is no compelling evidence that sCJD has a different origin than
sporadic genesis. However, the finding of prion types with an across-the-species
comparability might provide further understanding of the pathogenesis in prion
diseases. Prion Types Encode Interspecies TSEs 2571 AJP December 2009, Vol. 175,
No. 6
why do we not want to do TSE transmission studies on chimpanzees $
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
BSE: TIME TO TAKE H.B. PARRY SERIOUSLY If the scrapie agent is generated
from ovine DNA and thence causes disease in other species, then perhaps, bearing
in mind the possible role of scrapie in CJD of humans (Davinpour et al, 1985),
scrapie and not BSE should be the notifiable disease. ...
Wednesday, February 16, 2011
IN CONFIDENCE
SCRAPIE TRANSMISSION TO CHIMPANZEES
IN CONFIDENCE
Sunday, April 18, 2010
SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010
Monday, April 25, 2011
Experimental Oral Transmission of Atypical Scrapie to Sheep
Volume 17, Number 5-May 2011
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
12/10/76
AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note CHAIRMAN: PROFESSOR PETER WILDY
snip...
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.
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
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).
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, January 22, 2012
Chronic Wasting Disease CWD cervids interspecies transmission
Friday, October 26, 2012
***CHRONIC WASTING DISEASE CWD PENNSYLVANIA GAME FARMS, URINE ATTRACTANT
PRODUCTS, BAITING, AND MINERAL LICKS
Friday, November 09, 2012
*** Chronic Wasting Disease CWD in cervidae and transmission to other
species
Friday, December 14, 2012
Susceptibility of domestic cats to chronic wasting disease
Tuesday, December 25, 2012
A Quantitative Assessment of the Amount of Prion Diverted to Category 1
Materials and Wastewater During Processing
Thursday, August 4, 2011
Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis,
Date aired: 27 Jun 2011 (SEE VIDEO)
Sunday, August 21, 2011
The British disease, or a disease gone global, The TSE Prion Disease (SEE
VIDEO)
Sunday, September 6, 2009
MAD COW USA 1997 (SEE SECRET VIDEO)
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE
RISE IN NORTH AMERICA
POLITICAL BSe and CJD and THE WOW FACTOR $$$
Monday, September 26, 2011
Variably Protease-Sensitive Prionopathy, Prionpathy, Prionopathy, FFI, GSS,
gCJD, hvCJD, sCJD, TSE, PRION, update 2011
Wednesday, March 28, 2012
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion
poker goes up again $
Tuesday, December 25, 2012
CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25,
2012
snip...
USA PRION UNIT LATEST HUMAN TSE PRION DISEASE UPDATE AUGUST 14, 2012
National Prion Disease Pathology Surveillance Center Cases Examined1
(August 14, 2012)
1996 & earlier 28 cases of sporadic CJD.
see steady increase to ;
2010 cases of sporadic CJD 216.
2011 cases of sporadic CJD 214.
snip...
1 Listed based on the year of death or, if not available, on year of
referral;
2 Cases with suspected prion disease for which brain tissue and/or blood
(in familial cases) were submitted;
3 Disease acquired in the United Kingdom;
4 Disease was acquired in the United Kingdom in one case and in Saudi
Arabia in the other case;
*** 5 Includes 8 cases in which the diagnosis is pending, and 18
inconclusive cases;
*** 6 Includes 10 (9 from 2012) cases with type determination pending in
which the diagnosis of vCJD has been excluded.
*** The Sporadic cases include 16 cases of sporadic Fatal Insomnia (sFI)
and 42 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 2224 cases
of sporadic Creutzfeldt-Jakob disease (sCJD).
snip...see full case reports here ;
please see full text for Texas and USA with more updated data on the TSE
BSE CWD Scrapie CJD prion disease outbreak in the USA here ;
Tuesday, December 25, 2012
CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25,
2012
Friday, August 10, 2012
Incidents of Potential iatrogenic Creutzfeldt-Jakob disease (CJD) biannual
update (July 2012)
Tuesday, December 18, 2012
Bioassay Studies Support the Potential for Iatrogenic Transmission of
Variant Creutzfeldt Jakob Disease through Dental Procedures
Sunday, December 9, 2012
Prions, prionoids and pathogenic proteins in Alzheimer disease
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
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
Thursday, December 20, 2012
OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe
WITH BOVINE MAD COW DISEASE
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
as a layperson, these are my opinions, from the sound science to date
documented here from, through daily investigation of the TSE prion science over
the last 15 years. ...
TSS
posted by Terry S. Singeltary Sr. at
11:06 AM
