First US BSE Case Since 2006 Underscores Need for Vigilance
Neurology Today 21 June 2012; Volume 12(12); p 12–13
Samson, Kurt
Links • Download Article PDF
Outline • article in brief • variant cjd • what should neurologists be
looking for? • references:
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ARTICLE IN BRIEF
A case of bovine spongiform encephalopathy was identified in a dairy cow in
California, prompting new calls for strict ongoing monitoring of cattle, and
heightened awareness of variant Creutzfeldt-Jakob disease symptoms by
neurologists.
THE FOURTH CASE of bovine spongiform encephalopathy in the US was
identified in April. [ Click here to enlarge ] US health officials confirmed a
case of bovine spongiform encephalopathy (BSE), or mad cow disease, in April —
the first in six years — in a ten-year-old cow on a California dairy farm. It is
the fourth confirmed case of BSE in the US since surveillance began, but
according to the US Department of Agriculture, no products from the cow entered
the human food supply.
The US Department of Agriculture (USDA) stressed that the cow was never
presented for slaughter for human consumption, and therefore presented no risk
to human health in the United States. In 2011, there were only 29 worldwide
cases of BSE, a dramatic decline and 99 percent reduction since the peak in 1992
of 37,311 cases.
At press time, the USDA stated that one progeny born to the positive cow in
California in the last two years was stillborn, and another was identified in
another state; that animal was euthanized and its tissue was sampled and tested
negative for BSE. Dairies in California and at the site where the second calf
were found remain under quarantine.
If transmitted to humans, BSE, which is caused by misfolded prion proteins,
causes a rare but fatal neurodegenerative condition called variant
Creutzfeldt-Jakob disease (vCJD). The disease is transmitted by eating
contaminated cow brain, spinal cord, or digestive tract tissue. VARIANT CJD
Since an outbreak of BSE in Europe in the late 1980s, after which stringent
animal and human surveillance systems were put in place around the globe, more
than 200 cases of vCJD in humans have been linked to contaminated cattle; 22
cases were identified in North America, and three in the US.
In all three US cases of vCJD, the affected individuals were believed to
have contracted the disease outside the US, according to Pierluigi Gambetti, MD,
professor of neurology and pathology and director of the National Prion Disease
Pathology Surveillance Center at Case Western Reserve University in Cleveland.
The center, which was established by the Centers for Disease Control and
Prevention (CDC) in collaboration with the American Association of
Neuropathologists in 1997, performs diagnostic tests for prion diseases,
including postmortem tests for vCJD.
All three brains were examined at the surveillance center, Dr. Gambetti
explained, and the evidence suggests that two of the people with vCJD were
exposed to the BSE agent in the United Kingdom and the third while living in
Saudi Arabia.
“There is no evidence of any case of vCJD being acquired in the US, and
this is very comforting news,” he said.
But Dr. Gambetti said the discovery of the latest case of BSE in the
California animal points to the need for strict ongoing monitoring of cattle —
he noted that only one head of cattle for every thousand or more is currently
screened for the disease.
DR. PIERLUIGI GAMBETTI: “There is no evidence of any case of vCJD being
acquired in the US, and this is very comforting news.” [Click here to enlarge ]
Dr. Gambetti also stressed the need for heightened awareness of vCJD symptoms by
neurologists and other health practitioners.
Increasing the number of autopsies on patients with suspected prion
disease, including classic CJD — which is not linked to BSE and distinctly
different in clinical and pathologic presentation — will enhance efforts to
identify cases of vCJD, he said.
The origin of these diseases [in animals] is unclear, said Dr. Gambetti.
“We don't know how atypical prion diseases form in animals — if they are
spontaneous or related to exposure — and we don't know where they came from,” he
said. “We need to find the weak point in the transition of normal to abnormal
protein. Without it, treatment will be hard to find.”
The good news is that once found, treatments or treatment principles for
one form of disease may work for others as well because the same mechanisms are
involved, Dr. Gambetti said.
CLINICAL AND PATHOLOGIC CHARACTERISTICS DISTINGUISHING CLASSIC CJD FROM
VARIANT CJD [ Click here to enlarge ] WHAT SHOULD NEUROLOGISTS BE LOOKING FOR?
The CDC has asked for increased vigilance in monitoring and detecting other
possible prion diseases, including classic CJD, by increasing the number of
postmortem exams. And the hope is that with increased surveillance there will be
better detection of vCJD. But Michael D. Geschwind, MD, PhD, associate professor
of clinical neurology at the University of California, San Francisco, stressed
there are several important differences between classic CJD and variant
CJD.
For the median age of death for classic CJD patients is 68 years, with very
few cases in persons under 30 years of age. In contrast, the median age of death
of patients with vCJD is 28 years.
vCJD also has atypical clinical features, he said. These include prominent
progressive psychiatric and/or sensory symptoms, usually at the time of clinical
presentation, and delayed onset of neurologic abnormalities. For example, ataxia
may appear within weeks or months, and dementia and myoclonus in later stages of
the illness.
For a diagnosis, patients must have four of the following symptoms: ataxia,
depression, dementia, persistent painful sensations, and a movement disorder,
such as chorea, dystonia, or myoclonus.
The most important differences appear on fluid attenuated inversion
recovery (FLAIR) and diffusion-weighted imaging (DWI) sequences, said Dr.
Geschwind. Most patients have an abnormal MRI, such as a symmetrical high signal
in the posterior thalamus. The pattern of FLAIR/DWI hyperintensity and
restricted diffusion can differentiate CJD from other nonprion causes of rapidly
progressive dementia, he noted.
Cortical gyral grey matter hyperintensity (cortical ribboning) and other
gray matter hyperintensities are the most critical sign of CJD on FLAIR,
according to Dr. Geschwind. This is never restricted to just the limbic regions,
and is rarely seen in the precentral gyrus. With CJD cases with basal ganglia or
thalamic DWI hyperintensities, there is associated restricted diffusion not seen
in nonprion causes of rapidly progressive dementia, where isolated limbic
hyperintensities are common.
Other areas of the brain, such as the neocortex and the basal ganglia, may
also have a high signal, but what distinguishes vCJD is that on MRI the thalamus
is brighter than in other areas.
Unfortunately, many radiologists may not be aware of these differences. In
a study published last year Neurology, Dr. Geschwind and colleagues found that
one reader's sensitivity and specificity for CJD was 94 percent and 100 percent
while another's was 92 percent and 72 percent.
A more definitive diagnosis, he said, can be made by tonsil or brain biopsy
if there is evidence of prion protein.
Thomas Wisniewski, MD, professor of neurology, pathology, and psychiatry at
New York University School of Medicine, said he believes sporadic cases of BSE
will continue, and while the risk to humans “is very low,” health officials and
neurologists also need to be on guard for novel and unfamiliar prion
diseases.
In a telephone interview with Neurology Today, he said that while BSE and
vCJD are a concern that demands vigilance, similar new diseases have the
potential to emerge in the near future that might well carry greater risk to
humans.
“I think that the neurological community needs to be aware that other
infectious CJD-like diseases may start appearing in patients,” he said.
Of special concern is the risk of chronic wasting disease (CWD) jumping the
species barrier from wild deer and elk into the human reservoir, he noted.
Chronic wasting disease has yet to be reported in humans, but several factors
make it potentially more dangerous than BSE, Dr. Wisniewski said.
“In these animal populations it can be more prevalent than BSE, affecting
up to 80 percent of a herd population. Furthermore, CWD appears to be more
easily transmissible, including by an aerosol route, in the [deer] population.
We also know that it can be transmitted to non-human primates such as squirrel
monkeys.”
—Kurt Samson
REFERENCES: Back to top • Geschwind MD, Vitali P, Maccagnano E, et al.
Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other
rapid dementias. 2011;76:1711–1719.
• Belay ED, Schonberger LB.Variant Creutzfeldt-Jakob disease and bovine
spongiform encephalopathy. 2002;22(4):849–862.
• The National Prion Disease Pathology Surveillance Center:
www.cjdsurveillance.com
• CDC site on vCJD: http://www.cdc.gov/ncidod/dvrd/vcjd/index.htm
US health officials confirmed a case of bovine spongiform encephalopathy
(BSE), or mad cow disease, in April — the first in six years — in a ten-year-old
cow on a California dairy farm. It is the fourth confirmed case of BSE in the US
since surveillance began, but according to the US Department of Agriculture, no
products from the cow entered the human food supply.
The US Department of Agriculture (USDA) stressed that the cow was never
presented for slaughter for human consumption, and therefore presented no risk
to human health in the United States. In 2011, there were only 29 worldwide
cases of BSE, a dramatic decline and 99 percent reduction since the peak in 1992
of 37,311 cases. At press time, the USDA stated that one progeny born to the
positive cow in California in the last two years was stillborn, and another was
identifi ed in another state; that animal was euthanized and its tissue was
sampled and tested negative for BSE. Dairies in California and at the site where
the second calf were found remain under quarantine.
If transmitted to humans, BSE, which is caused by misfolded prion proteins,
causes a rare but fatal neurodegenerative condition called variant Creutzfeldt-
Jakob disease (vCJD). The disease is transmitted by eating contaminated cow
brain, spinal cord, or digestive tract tissue.
VARIANT CJD
Since an outbreak of BSE in Europe in the late 1980s, after which stringent
animal and human surveillance systems were put in place around the globe, more
than 200 cases of vCJD in humans have been linked to contaminated cattle; 22
cases were identifi ed in North America, and three in the US.
In all three US cases of vCJD, the affected individuals were believed to
have contracted the disease outside the US, according to Pierluigi Gambetti, MD,
professor of neurology and pathology and director of the National Prion Disease
Pathology Surveillance Center at Case Western Reserve University in Cleveland.
The center, which was established by the Centers for Disease Control and
Prevention (CDC) in collaboration with the American Association of
Neuropathologists in 1997, performs diagnostic tests for prion diseases,
including postmortem tests for vCJD.
All three brains were examined at the surveillance center, Dr. Gambetti
explained, and the evidence suggests that two of the people with vCJD were
exposed to the BSE agent in the United Kingdom and the third while living in
Saudi Arabia.
“There is no evidence of any case of vCJD being acquired in the US, and
this is very comforting news,” he said. But Dr. Gambetti said the discovery of
the latest case of BSE in the California animal points to the need for strict
ongoing monitoring of cattle — he noted that only one head of cattle for every
thousand or more is currently screened for the disease.
Dr. Gambetti also stressed the need for heightened awareness of vCJD
symptoms by neurologists and other health practitioners.
Increasing the number of autopsies on patients with suspected prion
disease, including classic CJD — which is not linked to BSE and distinctly
different in clinical and pathologic presentation — will enhance efforts to
identify cases of vCJD, he said.
The origin of these diseases [in animals] is unclear, said Dr. Gambetti.
“We don’t know how atypical prion diseases form in animals — if they are
spontaneous or related to exposure — and we don’t know where they came from,” he
said. “We need to fi nd the weak point in the transition of normal to abnormal
protein. Without it, treatment will be hard to fi nd.”
The good news is that once found, treatments or treatment principles for
one form of disease may work for others as well because the same mechanisms are
involved, Dr. Gambetti said. •
BSE Continued from page 12 CLINICAL AND PATHOLOGIC CHARACTERISTICS
DISTINGUISHING
CLASSIC CJD FROM VARIANT CJD Characteristic Classic CJD Variant CJD Median
age at death 68 years 28 years Median duration of illness 6 months 13-14 months
Clinical signs & symptoms Dementia; early neurologic signs Prominent
psychiatric/behavioral symptoms; painful dyesthesiasis; delayed neurologic signs
Periodic sharp waves on EEG Often present Often absent An abnormal signal in the
posterior thalami on T2- and DWI and FLAIR sequences on brain MRI Not reported
Present in more than 75% of cases Florid plaques on neuropathology Rare or
absent Present in large numbers Immunohistochemical analysis of brain tissue for
protease resistant prion protein Variable accumulation Marked accumulation of
protease-resistant prion protein Presence of prions in lymphoid tissue Not
readily detected Readily detected Increased glycoform ratio on immunoblot
analysis of protease-resistance prion protein Not reported Marked accumulation
of protease-resistance prion protein
Source: CDC, Adapted from Belay E, et al. Variant Creutzfeldt-Jakob
disease and bovine spongiform encephalopathy. Clin Lab Med 2002; 22:849-862. The
CDC has asked for increased vigilance in monitoring and detecting other possible
prion diseases, including classic CJD, by increasing the number of postmortem
exams. And the hope is that with increased surveillance there will be better
detection of vCJD. But Michael D. Geschwind, MD, PhD, associate professor of
clinical neurology at the University of California, San Francisco, stressed
there are several important differences between classic CJD and variant CJD.
For the median age of death for classic CJD patients is 68 years, with
very few cases in persons under 30 years of age. In contrast, the median age of
death of patients with vCJD is 28 years.
vCJD also has atypical clinical features, he said. These include prominent
progressive psychiatric and/or sensory symptoms, usually at the time of clinical
presentation, and delayed onset of neurologic abnormalities. For example, ataxia
may appear within weeks or months, and dementia and myoclonus in later stages of
the illness.
For a diagnosis, patients must have four of the following symptoms:
ataxia, depression, dementia, persistent painful sensations, and a movement
disorder, such as chorea, dystonia, or myoclonus.
The most important differences appear on fl uid attenuated inversion
recovery (FLAIR) and diffusion-weighted imaging (DWI) sequences, said Dr.
Geschwind. Most patients have an abnormal MRI, such as a symmetrical high signal
in the posterior thalamus. The pattern of FLAIR/DWI hyperintensity and
restricted diffusion can differentiate CJD from other nonprion causes of rapidly
progressive dementia, he noted. Cortical gyral grey matter hyperintensity
(cortical ribboning) and other gray matter hyperintensities are the most
critical sign of CJD on FLAIR, according to Dr. Geschwind. This is never
restricted to just the limbic regions, and is rarely seen in the precentral
gyrus. With CJD cases with basal ganglia or thalamic DWI hyperintensities, there
is associated restricted diffusion not seen in nonprion causes of rapidly
progressive dementia, where isolated limbic hyperintensities are common. Other
areas of the brain, such as the neocortex and the basal ganglia, may also have a
high signal, but what distinguishes vCJD is that on MRI the thalamus is brighter
than in other areas.
Unfortunately, many radiologists may not be aware of these differences. In
a study published last year Neurology, Dr. Geschwind and colleagues found that
one reader's sensitivity and specifi city for CJD was 94 percent and 100 percent
while another’s was 92 percent and 72 percent. A more defi nitive diagnosis, he
said, can be made by tonsil or brain biopsy if there is evidence of prion
protein. Thomas Wisniewski, MD, professor of neurology, pathology, and
psychiatry at New York University School of Medicine, said he believes sporadic
cases of BSE will continue, and while the risk to humans “is very low,” health
offi cials and neurologists also need to be on guard for novel and unfamiliar
prion diseases.
In a telephone interview with Neurology Today, he said that while BSE and
vCJD are a concern that demands vigilance, similar new diseases have the
potential to emerge in the near future that might well carry greater risk to
humans.
“I think that the neurological community needs to be aware that other
infectious CJD-like diseases may start appearing in patients,” he said.
Of special concern is the risk of chronic wasting disease (CWD) jumping
the species barrier from wild deer and elk into the human reservoir, he noted.
Chronic wasting disease has yet to be reported in humans, but several factors
make it potentially more dangerous than BSE, Dr. Wisniewski said.
“In these animal populations it can be more prevalent than BSE, affecting
up to 80 percent of a herd population. Furthermore, CWD appears to be more
easily transmissible, including by an aerosol route, in the [deer] population.
We also know that it can be transmitted to non-human primates such as squirrel
monkeys.”
—Kurt Samson
DR. PIERLUIGI GAMBETTI: “There is no evidence of any case of vCJD being
acquired in the US, and this is very comforting news.”
REFERENCES:
• Geschwind MD, Vitali P, Maccagnano E, et al. Diffusion-weighted MRI
hyperintensity patterns differentiate CJD from other rapid dementias. Neurology
2011;76:1711-1719.
• Belay ED, Schonberger LB.Variant Creutzfeldt-Jakob disease and bovine
spongiform encephalopathy. Clin Lab Med 2002;22(4): 849-862.
• The National Prion Disease Pathology Surveillance Center:
www.cjdsurveillance.com
• CDC site on vCJD
WHAT SHOULD NEUROLOGISTS BE LOOKING FOR?
The CDC has asked for increased vigilance in monitoring and detecting
other possible prion diseases, including classic CJD, by increasing the number
of postmortem exams. And the hope is that with increased surveillance there will
be better detection of vCJD. But Michael D. Geschwind, MD, PhD, associate
professor of clinical neurology at the University of California, San Francisco,
stressed there are several important differences between classic CJD and variant
CJD.
For the median age of death for classic CJD patients is 68 years, with very
few cases in persons under 30 years of age. In contrast, the median age of death
of patients with vCJD is 28 years.
vCJD also has atypical clinical features, he said. These include prominent
progressive psychiatric and/or sensory symptoms, usually at the time of clinical
presentation, and delayed onset of neurologic abnormalities. For example, ataxia
may appear within weeks or months, and dementia and myoclonus in later stages of
the illness. For a diagnosis, patients must have four of the following symptoms:
ataxia, depression, dementia, persistent painful sensations, and a movement
disorder, such as chorea, dystonia, or myoclonus.
The most important differences appear on fl uid attenuated inversion
recovery (FLAIR) and diffusion-weighted imaging (DWI) sequences, said Dr.
Geschwind. Most patients have an abnormal MRI, such as a symmetrical high signal
in the posterior thalamus. The pattern of FLAIR/DWI hyperintensity and
restricted diffusion can differentiate CJD from other nonprion causes of rapidly
progressive dementia, he noted. Cortical gyral grey matter hyperintensity
(cortical ribboning) and other gray matter hyperintensities are the most
critical sign of CJD on FLAIR, according to Dr. Geschwind. This is never
restricted to just the limbic regions, and is rarely seen in the precentral
gyrus. With CJD cases with basal ganglia or thalamic DWI hyperintensities, there
is associated restricted diffusion not seen in nonprion causes of rapidly
progressive dementia, where isolated limbic hyperintensities are common. Other
areas of the brain, such as the neocortex and the basal ganglia, may also have a
high signal, but what distinguishes vCJD is that on MRI the thalamus is brighter
than in other areas.
Unfortunately, many radiologists may not be aware of these differences. In
a study published last year Neurology, Dr. Geschwind and colleagues found that
one reader's sensitivity and specifi city for CJD was 94 percent and 100 percent
while another’s was 92 percent and 72 percent. A more defi nitive diagnosis, he
said, can be made by tonsil or brain biopsy if there is evidence of prion
protein. Thomas Wisniewski, MD, professor of neurology, pathology, and
psychiatry at New York University School of Medicine, said he believes sporadic
cases of BSE will continue, and while the risk to humans “is very low,” health
offi cials and neurologists also need to be on guard for novel and unfamiliar
prion diseases.
In a telephone interview with Neurology Today, he said that while BSE and
vCJD are a concern that demands vigilance, similar new diseases have the
potential to emerge in the near future that might well carry greater risk to
humans.
“I think that the neurological community needs to be aware that other
infectious CJD-like diseases may start appearing in patients,” he said.
Of special concern is the risk of chronic wasting disease (CWD) jumping the
species barrier from wild deer and elk into the human reservoir, he noted.
Chronic wasting disease has yet to be reported in humans, but several factors
make it potentially more dangerous than BSE, Dr. Wisniewski said.
“In these animal populations it can be more prevalent than BSE, affecting
up to 80 percent of a herd population. Furthermore, CWD appears to be more
easily transmissible, including by an aerosol route, in the [deer] population.
We also know that it can be transmitted to non-human primates such as squirrel
monkeys.” —Kurt Samson
CLINICAL AND PATHOLOGIC CHARACTERISTICS DISTINGUISHING CLASSIC CJD FROM
VARIANT CJD Characteristic Classic CJD Variant CJD Median age at death 68 years
28 years Median duration of illness 6 months 13-14 months Clinical signs &
symptoms Dementia; early neurologic signs Prominent psychiatric/behavioral
symptoms; painful dyesthesiasis; delayed neurologic signs Periodic sharp waves
on EEG Often present Often absent An abnormal signal in the posterior thalami on
T2- and DWI and FLAIR sequences on brain MRI Not reported Present in more than
75% of cases Florid plaques on neuropathology Rare or absent Present in large
numbers Immunohistochemical analysis of brain tissue for protease resistant
prion protein Variable accumulation Marked accumulation of protease-resistant
prion protein Presence of prions in lymphoid tissue Not readily detected Readily
detected Increased glycoform ratio on immunoblot analysis of protease-resistance
prion protein Not reported Marked accumulation of protease-resistance prion
protein Source: CDC, Adapted from Belay E, et al. Variant Creutzfeldt-Jakob
disease and bovine spongiform encephalopathy. Clin Lab Med 2002; 22:849-862.
see pdf file at link ;
From: Terry S. Singeltary Sr.
Sent: Tuesday, June 26, 2012 4:39 PM
To: BSE-L@LISTS.AEGEE.ORG
Subject: [BSE-L] Creutzfeldt Jakob Disease Human TSE report update North
America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012
type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the
rise in Canada and the USA
Creutzfeldt Jakob Disease Human TSE report update North America, Canada,
Mexico, and USDA PRION UNIT as of May 18, 2012
National Prion Disease Pathology Surveillance Center
Cases Examined1
(May 18, 2012)
Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD
1996 & earlier 50 32 28 4 0 0
1997 114 68 59 9 0 0
1998 88 52 44 7 1 0
1999 123 74 65 8 1 0
2000 145 103 89 14 0 0
2001 210 120 110 10 0 0
2002 248 149 125 22 2 0
2003 266 168 137 31 0 0
2004 326 187 164 22 0 13
2005 344 194 157 36 1 0
2006 382 196 166 28 0 24
2007 377 213 185 28 0 0
2008 396 232 206 26 0 0
2009 423 256 212 43 1 0
2010 413 257 216 41 0 0
2011 410 257 213 43 0 0
2012 153 82 51 15 0 0
TOTAL 44685 26406 2227 387 6 3
1 Listed based on the year of death or, if not available, on year of
referral;
2 Cases with suspected prion disease for which brain tissue and/or blood
(in familial cases) were submitted;
3 Disease acquired in the United Kingdom;
4 Disease was acquired in the United Kingdom in one case and in Saudi
Arabia in the other case;
5 Includes 14 cases in which the diagnosis is pending, and 18 inconclusive
cases;
6 Includes 17 (16 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 2118 cases of sporadic Creutzfeldt-Jakob disease (sCJD).
Rev 5/18/2012
> 6 Includes
> 17 (16 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 2118 cases
of sporadic Creutzfeldt-Jakob disease (sCJD).
WELL, it seems the USA mad cow strains in humans classified as type
determination pending tdpCJD, VPSPr, sFFI, and sCJD) have steadily increased
over the years, and the same old song and dance continues with sporadic CJD
cases $$$
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion
poker goes up again $
OR-10 15:25 - 15:40 VARIABLY PROTEASE-SENSITIVE PRIONOPATHY IS
TRANSMISSIBLE IN BANK VOLES Nonno
OR-10: Variably protease-sensitive prionopathy is transmissible in bank
voles
Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1
Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan
Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità ; Rome,
Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna,
Italy; 3Case Western Reserve University; Cleveland, OH USA
Background. Variably protease-sensitive prionopathy (VPSPr) is a recently
described “sporadic”neurodegenerative disease involving prion protein
aggregation, which has clinical similarities with non-Alzheimer dementias, such
as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in
Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the
prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is
the electrophoretic pattern of PrPSc after digestion with proteinase K (PK).
After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern
similar to that described in GSS cases. The clinical and pathological features
of VPSPr raised the question of the correct classification of VPSPr among prion
diseases or other forms of neurodegenerative disorders. Here we report
preliminary data on the transmissibility and pathological features of VPSPr
cases in bank voles.
Materials and Methods. Seven VPSPr cases were inoculated in two genetic
lines of bank voles, carrying either methionine or isoleucine at codon 109 of
the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases
selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical
diagnosis in voles was confirmed by brain pathological assessment and western
blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.
Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission
in BvM109. Overall, 3 voles were positive with survival time between 290 and 588
d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form
of the typical PrP27–30, which was indistinguishable to that previously observed
in BvM109 inoculated with sCJDMM1 cases. In BvI109, 3 VPSPr cases (2 VV and 1
MM) showed positive transmission until now. Overall, 5 voles were positive with
survival time between 281 and 596 d.p.i.. In contrast to what observed in
BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern,
characterized by low molecular weight PrPres. These PrPres fragments were
positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84,
suggesting that they are cleaved at both the C-terminus and the N-terminus.
Second passages are in progress from these first successful transmissions.
Conclusions. Preliminary results from transmission studies in bank voles
strongly support the notion that VPSPr is a transmissible prion disease.
Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of
voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109. The
discovery of previously unrecognized prion diseases in both humans and animals
(i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases
might be wider than expected and raises crucial questions about the epidemiology
and strain properties of these new forms. We are investigating this latter issue
by molecular and biological comparison of VPSPr, GSS and Nor98.
SOURCE PRION2012
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
THIS seems to raise more questions than answers, confusing the TSEs even
worse.
WHAT is sporadic CJD, and how many sub-types and atypical strains,
phenotypes etc. will there be, arising from nothing. a spontaneous happening of
sorts???
i think not. ...tss
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
snip...
Genetic findings
No mutations were found in the open reading frame after sequencing the
prion protein gene (PRNP). A heterozygosis methionine valine (MV) was observed
in codon 129.
snip...
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease
update July 10, 2008
Although several subjects had family histories of dementia, no mutations
were found in the PrP gene open reading frame.
Thursday, July 10, 2008
A New Prionopathy update July 10, 2008
***+++***
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease
update July 10, 2008 Friday, June 20, 2008
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
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
====================================
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 ;
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)
P.9.21
Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim
McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre,
Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of
Calgary, Canada
Background: Three BSE types (classical and two atypical) have been
identified on the basis of molecular characteristics of the misfolded protein
associated with the disease. To date, each of these three types have been
detected in Canadian cattle.
Objectives: This study was conducted to further characterize the 16
Canadian BSE cases based on the biochemical properties of there associated
PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and
relative proteinase K sensitivity of the PrPres from each of the 16 confirmed
Canadian BSE cases was determined using modified Western blot analysis.
Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type
and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and
changes in glycosylation similar to other atypical BSE cases. PK digestion under
mild and stringent conditions revealed a reduced protease resistance of the
atypical cases compared to the C-type cases. N terminal- specific antibodies
bound to PrPres from H type but not from C or L type. The C-terminal-specific
antibodies resulted in a shift in the glycoform profile and detected a fourth
band in the Canadian H-type BSE.
Discussion: The C, L and H type BSE cases in Canada exhibit molecular
characteristics similar to those described for classical and atypical BSE cases
from Europe and Japan. This supports the theory that the importation of BSE
contaminated feedstuff is the source of C-type BSE in Canada.
*** It also suggests a similar cause or source for atypical BSE in these
countries.
what about that ALABAMA MAD COW, AND MAD COW FEED THERE FROM IN THAT STATE
???
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)
BANNED MAD COW FEED IN COMMERCE IN ALABAMA
Date: September 6, 2006 at 7:58 am PST PRODUCT
a) EVSRC Custom dairy feed, Recall # V-130-6;
b) Performance Chick Starter, Recall # V-131-6;
c) Performance Quail Grower, Recall # V-132-6;
d) Performance Pheasant Finisher, Recall # V-133-6.
CODE None RECALLING FIRM/MANUFACTURER Donaldson & Hasenbein/dba J&R
Feed Service, Inc., Cullman, AL, by telephone on June 23, 2006 and by letter
dated July 19, 2006. Firm initiated recall is complete.
REASON
Dairy and poultry feeds were possibly contaminated with ruminant based
protein.
VOLUME OF PRODUCT IN COMMERCE 477.72 tons
DISTRIBUTION AL
______________________________
PRODUCT Bulk custom dairy pre-mixes,
Recall # V-120-6 CODE None RECALLING FIRM/MANUFACTURER Ware Milling Inc.,
Houston, MS, by telephone on June 23, 2006. Firm initiated recall is complete.
REASON Possible contamination of dairy animal feeds with ruminant derived meat
and bone meal.
VOLUME OF PRODUCT IN COMMERCE 350 tons
DISTRIBUTION AL and MS
______________________________
PRODUCT
a) Tucker Milling, LLC Tm 32% Sinking Fish Grower, #2680-Pellet, 50 lb.
bags, Recall # V-121-6;
b) Tucker Milling, LLC #31120, Game Bird Breeder Pellet, 50 lb. bags,
Recall # V-122-6;
c) Tucker Milling, LLC #31232 Game Bird Grower, 50 lb. bags, Recall #
V-123-6;
d) Tucker Milling, LLC 31227-Crumble, Game Bird Starter, BMD Medicated, 50
lb bags, Recall # V-124-6;
e) Tucker Milling, LLC #31120, Game Bird Breeder, 50 lb bags, Recall #
V-125-6;
f) Tucker Milling, LLC #30230, 30 % Turkey Starter, 50 lb bags, Recall #
V-126-6;
g) Tucker Milling, LLC #30116, TM Broiler Finisher, 50 lb bags, Recall #
V-127-6
CODE All products manufactured from 02/01/2005 until 06/20/2006 RECALLING
FIRM/MANUFACTURER Recalling Firm: Tucker Milling LLC, Guntersville, AL, by
telephone and visit on June 20, 2006, and by letter on June 23, 2006.
Manufacturer: H. J. Baker and Brothers Inc., Stamford, CT. Firm initiated recall
is ongoing.
REASON Poultry and fish feeds which were possibly contaminated with
ruminant based protein were not labeled as "Do not feed to ruminants".
VOLUME OF PRODUCT IN COMMERCE 7,541-50 lb bags
DISTRIBUTION AL, GA, MS, and TN
END OF ENFORCEMENT REPORT FOR AUGUST 9, 2006
###
Subject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125
TONS Products manufactured from 02/01/2005 until 06/06/2006
Date: August 6, 2006 at 6:16 pm PST PRODUCT
a) CO-OP 32% Sinking Catfish, Recall # V-100-6;
b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall #
V-101-6;
c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6;
d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6;
e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;
f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50
lb. bag, Recall # V-105-6;
g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%,
Recall # V-106-6;
h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to
20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall #
V-107-6;
i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall #
108-6;
j) CO-OP LAYING CRUMBLES, Recall # V-109-6;
k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall #
V-110-6;
l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6;
m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6 CODE
Product manufactured from 02/01/2005 until 06/06/2006
RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL,
by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is
complete.
REASON Animal and fish feeds which were possibly contaminated with ruminant
based protein not labeled as "Do not feed to ruminants".
VOLUME OF PRODUCT IN COMMERCE 125 tons
DISTRIBUTION AL and FL
END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006
###
MAD COW FEED RECALL USA EQUALS 10,878.06 TONS NATIONWIDE Sun Jul 16, 2006
09:22 71.248.128.67
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II
______________________________
PRODUCT
a) PRO-LAK, bulk weight, Protein Concentrate for Lactating Dairy Animals,
Recall # V-079-6;
b) ProAmino II, FOR PREFRESH AND LACTATING COWS, net weight 50lb (22.6 kg),
Recall # V-080-6;
c) PRO-PAK, MARINE & ANIMAL PROTEIN CONCENTRATE FOR USE IN ANIMAL FEED,
Recall # V-081-6;
d) Feather Meal, Recall # V-082-6 CODE
a) Bulk
b) None
c) Bulk
d) Bulk
RECALLING FIRM/MANUFACTURER H. J. Baker & Bro., Inc., Albertville, AL,
by telephone on June 15, 2006 and by press release on June 16, 2006. Firm
initiated recall is ongoing.
REASON
Possible contamination of animal feeds with ruminent derived meat and bone
meal.
VOLUME OF PRODUCT IN COMMERCE 10,878.06 tons
DISTRIBUTION Nationwide
END OF ENFORCEMENT REPORT FOR July 12, 2006
###
Saturday, July 23, 2011
CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK
MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE
Saturday, November 6, 2010
TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the
EU Berne, 2010 TAFS
INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a
non-profit Swiss Foundation
10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN
COMMERCE USA 2007
Date: March 21, 2007 at 2:27 pm PST
RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II
PRODUCT
Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried,
Recall # V-024-2007
CODE
Cattle feed delivered between 01/12/2007 and 01/26/2007
RECALLING FIRM/MANUFACTURER
Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007.
Firm initiated recall is ongoing.
REASON
Blood meal used to make cattle feed was recalled because it was cross-
contaminated with prohibited bovine meat and bone meal that had been
manufactured on common equipment and labeling did not bear cautionary BSE
statement. VOLUME OF PRODUCT IN COMMERCE 42,090 lbs. DISTRIBUTION WI
___________________________________
PRODUCT
Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot-
Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M
CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B
DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal,
JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT
Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral,
BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC
LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall #
V-025-2007
CODE
The firm does not utilize a code - only shipping documentation with
commodity and weights identified.
RECALLING FIRM/MANUFACTURER
Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm
initiated recall is complete.
REASON
Products manufactured from bulk feed containing blood meal that was cross
contaminated with prohibited meat and bone meal and the labeling did not bear
cautionary BSE statement.
VOLUME OF PRODUCT IN COMMERCE
9,997,976 lbs.
DISTRIBUTION
ID and NV
END OF ENFORCEMENT REPORT FOR MARCH 21, 2007
USDA TRIPLE BSE MAD COW FIREWALL, SRM, FEED, AND SURVEILLANCE
2012
***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...
MAD COW USDA ATYPICAL L-TYPE BASE BSE, the rest of the story...
***Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate
Model
***Infectivity in skeletal muscle of BASE-infected cattle
***feedstuffs- It also suggests a similar cause or source for atypical BSE
in these countries.
***Also, a link is suspected between atypical BSE and some apparently
sporadic cases of Creutzfeldt-Jakob disease in humans.
The present study demonstrated successful intraspecies transmission of
H-type BSE to cattle and the distribution and immunolabeling patterns of PrPSc
in the brain of the H-type BSE-challenged cattle. TSE agent virulence can be
minimally defined by oral transmission of different TSE agents (C-type, L-type,
and H-type BSE agents) [59]. Oral transmission studies with H-type BSEinfected
cattle have been initiated and are underway to provide information regarding the
extent of similarity in the immunohistochemical and molecular features before
and after transmission.
In addition, the present data will support risk assessments in some
peripheral tissues derived from cattle affected with H-type BSE.
Thursday, June 21, 2012
Clinical and Pathologic Features of H-Type Bovine Spongiform Encephalopathy
Associated with E211K Prion Protein Polymorphism
Justin J. Greenlee1*, Jodi D. Smith1, M. Heather West Greenlee2, Eric M.
Nicholson1
1 National Animal Disease Center, United States Department of Agriculture,
Agricultural Research Service, Ames, Iowa, United States of America, 2 Iowa
State University, Ames, Iowa, United States of America
Abstract
The majority of bovine spongiform encephalopathy (BSE) cases have been
ascribed to the classical form of the disease. Htype and L-type BSE cases have
atypical molecular profiles compared to classical BSE and are thought to arise
spontaneously. However, one case of H-type BSE was associated with a heritable
E211K mutation in the prion protein gene. The purpose of this study was to
describe transmission of this unique isolate of H-type BSE when inoculated into
a calf of the same genotype by the intracranial route. Electroretinograms were
used to demonstrate preclinical deficits in retinal function, and optical
coherence tomography was used to demonstrate an antemortem decrease in retinal
thickness. The calf rapidly progressed to clinical disease (9.4 months) and was
necropsied. Widespread distribution of abnormal prion protein was demonstrated
within neural tissues by western blot and immunohistochemistry. While this
isolate is categorized as BSE-H due to a higher molecular mass of the
unglycosylated PrPSc isoform, a strong labeling of all 3 PrPSc bands with
monoclonal antibodies 6H4 and P4, and a second unglycosylated band at
approximately 14 kDa when developed with antibodies that bind in the C-terminal
region, it is unique from other described cases of BSE-H because of an
additional band 23 kDa demonstrated on western blots of the cerebellum. This
work demonstrates that this isolate is transmissible, has a BSE-H phenotype when
transmitted to cattle with the K211 polymorphism, and has molecular features
that distinguish it from other cases of BSE-H described in the literature.
snip...
Most significantly it must be determined if the molecular phenotype of this
cattle TSE remains stable when transmitted to cattle without the E211K
polymorphism as several other isolates of atypical BSE have been shown to adopt
a molecular profile consistent with classical BSE after passage in transgenic
mice expressing bovine PrPC [40] or multiple passages in wild type mice [23].
Results of ongoing studies, namely passage of the E211K Htype isolate into
wild-type cattle, will lend further insight into what role, if any, genetic and
sporadic forms of BSE may have played in the origins of classical BSE. 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.
Saturday, May 26, 2012
Are USDA assurances on mad cow case 'gross oversimplification'?
SNIP...
What irks many scientists is the USDA’s April 25 statement that the rare
disease is “not generally associated with an animal consuming infected
feed.”
The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown,
one of the world’s experts on this type of disease who retired recently from the
National Institutes of Health. "(The agency) has no foundation on which to base
that statement.”
“We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an
official with the USDA during the Clinton Administration now at Mississippi
State.
In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows the
origins of atypical cases of BSE,” she said
The argument about feed is critical because if feed is the cause, not a
spontaneous mutation, the California cow could be part of a larger outbreak.
SNIP...
P.9.21
Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim
McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre,
Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of
Calgary, Canada
Background: Three BSE types (classical and two atypical) have been
identified on the basis of molecular characteristics of the misfolded protein
associated with the disease. To date, each of these three types have been
detected in Canadian cattle.
Objectives: This study was conducted to further characterize the 16
Canadian BSE cases based on the biochemical properties of there associated
PrPres. Methods: Immuno-reactivity, molecular weight, glycoform profiles and
relative proteinase K sensitivity of the PrPres from each of the 16 confirmed
Canadian BSE cases was determined using modified Western blot analysis.
Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type
and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and
changes in glycosylation similar to other atypical BSE cases. PK digestion under
mild and stringent conditions revealed a reduced protease resistance of the
atypical cases compared to the C-type cases. N terminal- specific antibodies
bound to PrPres from H type but not from C or L type. The C-terminal-specific
antibodies resulted in a shift in the glycoform profile and detected a fourth
band in the Canadian H-type BSE.
Discussion: The C, L and H type BSE cases in Canada exhibit molecular
characteristics similar to those described for classical and atypical BSE cases
from Europe and Japan. This supports the theory that the importation of BSE
contaminated feedstuff is the source of C-type BSE in Canada.
*** It also suggests a similar cause or source for atypical BSE in these
countries.
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...
in the url that follows, I have posted
SRM breaches first, as late as 2011.
then
MAD COW FEED BAN BREACHES AND TONNAGES OF MAD COW FEED IN COMMERCE up until
2007, when they ceased posting them.
then,
MAD COW SURVEILLANCE BREACHES.
Friday, May 18, 2012
Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy
(BSE) in the United States Friday May 18, 2012
Thursday, June 21, 2012
MEATINGPLACE.COM WAVES MAGIC WAND AND EXPECTS THE USDA MAD COW FOLLIES BSE
TO BE GONE
Thursday, June 14, 2012
R-CALF USA Calls USDA Dishonest and Corrupt; Submits Fourth Request for
Extension
R-CALF United Stockgrowers of America
Friday, May 25, 2012
R-CALF USDA’s New BSE Rule Eliminates Important Protections Needed to
Prevent BSE Spread
Monday, June 18, 2012
R-CALF Submits Incomplete Comments Under Protest in Bizarre Rulemaking
“Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products”
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE
RISE IN NORTH AMERICA
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 $$$
Sunday, February 12, 2012
National Prion Disease Pathology Surveillance Center Cases Examined1
(August 19, 2011) including Texas
snip...
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN
AND SPINAL CORD MATTER
Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010
in Mesquite Texas
Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010
in Mesquite Texas. She left 6 Kids and a Husband. The Purpose of this web is to
give information in Spanish to the Hispanic community, and to all the community
who want's information about this terrible disease.-
Physician Discharge Summary, Parkland Hospital, Dallas Texas
Admit Date: 12/29/2009 Discharge Date: 1/20/2010 Attending Provider:
Greenberg, Benjamin Morris; General Neurology Team: General Neurology Team
snip...
The husband says that they have lived in Nebraska for the past 21 years.
They had seen a doctor there during the summer time who prescribed her Seroquel
and Lexapro, Thinking these were sx of a mood disorder. However, the medications
did not help and she continued to deteriorate clinically. Up until about 6 years
ago, the pt worked at Tyson foods where she worked on the assembly line,
slaughtering cattle and preparing them for packaging. She was exposed to brain
and spinal cord matter when she would euthanize the cattle. The husband says
that he does not know any fellow workers with a similar illness. He also says
that she did not have any preceeding illness or travel.
snip...
>>> Up until about 6 years ago, the pt worked at Tyson foods where
she worked on the assembly line, slaughtering cattle and preparing them for
packaging. She was exposed to brain and spinal cord matter when she would
euthanize the cattle. <<<
SEE MORE HERE ;
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN
AND SPINAL CORD MATTER
Sunday, February 12, 2012
National Prion Disease Pathology Surveillance Center Cases Examined1
(August 19, 2011) including Texas
Tuesday, November 08, 2011
Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob
Disease Surveillance? A Study in France from 2000 to 2008 Vol. 37, No. 3-4,
2011
Original Paper
Conclusions:These findings raise doubt about the possibility of a reliable
CJD surveillance only based on mortality data.
price of prion poker goes up again $$$
Monday, June 11, 2012
Guidance for Industry Draft Guidance for Industry: Amendment to “Guidance
for Industry: Revised Preventive Measures to Reduce the Possible Risk of
Transmission of Creutzfeldt-Jakob Disease and Variant Creutzfeldt-Jakob Disease
by Blood and Blood Products”
Sunday, June 3, 2012
A new neurological disease in primates inoculated with prion-infected blood
or blood components
PLEASE REMEMBER ;
The Akron, Ohio-based CJD Foundation said the Center for Disease Control
revised that number in October of 2004 to about one in 9,000 CJD cases per year
in the population group age 55 and older.
HAVE YOU GOT YOUR CJD QUESTIONNAIRE ASKING REAL QUESTIONS PERTAINING TO
ROUTE AND SOURCE OF THE TSE AGENT THAT KILLED YOUR LOVED ONE ???
if not, why not...
Friday, November 30, 2007
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health
Crisis
full text with source references ;
Sunday, August 21, 2011
The British disease, or a disease gone global, The TSE Prion Disease (SEE
VIDEO)
U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? (see video at
bottom)
WHO WILL FOLLOW THE CHILDREN FOR CJD SYMPTOMS ???
Saturday, May 2, 2009
U.S. GOVERNMENT SUES WESTLAND/HALLMARK MEAT OVER USDA CERTIFIED DEADSTOCK
DOWNER COW SCHOOL LUNCH PROGRAM
OUR SCHOOL CHILDREN ALL ACROSS THE USA WERE FED THE MOST HIGH RISK CATTLE
FOR MAD COW DISEASE FOR 4 YEARS I.E. DEAD STOCK DOWNER CATTLE VIA THE USDA AND
THE NSLP.
WHO WILL WATCH OUR CHILDREN FOR THE NEXT 5+ DECADES ???
DID YOUR CHILD CONSUME SOME OF THESE DEAD STOCK DOWNER COWS, THE MOST HIGH
RISK FOR MAD COW DISEASE ???
you can check and see here ;
CANADA
TOO bad Canada’s policy on BSE aka mad cow type disease, and the reporting
there from of completed cases, have ceased to exist on the CFIA site for the
public to follow. you have to request a copy. CFIA ceased giving those copies
out to me. ...
•Request a copy of a completed BSE investigation report for a case after
January 2009
Sunday, May 27, 2012
CANADA PLANS TO IMPRISON ANYONE SPEAKING ABOUT MAD COW or ANY OTHER DISEASE
OUTBREAK, CENSORSHIP IS A TERRIBLE THING
PLEASE NOTE, type determination pending Creutzfeldt Jakob Disease (tdpCJD)
in Canada is also on a steady increase. please see ;
> 3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is
pending.
CJD Deaths Reported by CJDSS1, 1994-20122
As of May 31, 2012
Deaths of Definite and Probable CJD
Year Sporadic Iatrogenic Familial GSS FFI vCJD Total
1994 2 0 0 1 0 0 3
1995 3 0 0 0 0 0 3
1996 13 0 0 0 0 0 13
1997 16 0 1 1 0 0 18
1998 22 1 0 1 0 0 24
1999 26 2 2 1 0 0 31
2000 32 0 0 3 0 0 35
2001 27 0 2 1 0 0 30
2002 31 0 2 2 0 1 36
2003 27 1 1 0 0 0 29
2004 42 0 1 0 0 0 43
2005 42 0 0 2 0 0 44
2006 39 0 1 3 1 0 44 2007 35 0 0 4 0 0 39
2008 48 0 1 0 0 0 49
2009 48 0 3 2 0 0 53
2010 34 0 3 0 0 0 37
2011 37 0 2 1 0 1 41
2012 1 0 0 0 0 0 1
Total 525 4 19 22 1 2 573
1. CJDSS began in 1998
2. Data before 1998 are retrospective and partial, data from 1998 to 2008
are complete, and data for 2009 - 2012 are provisional
3. Final classification of 50 cases from 2009, 2010, 2011 and 2012 is
pending.
CJD Deaths Reported by CJDSS1, 1994-20122
As of May 31, 2012
CENSORSHIP IS A TERRIBLE THING $$$
Canada has had a COVER-UP policy of mad cow disease since about the 17th
case OR 18th case of mad cow disease. AFTER THAT, all FOIA request were ignored
$$$
THIS proves there is indeed an epidemic of mad cow disease in North
America, and it has been covered up for years and years, if not for decades, and
it’s getting worse $$$
Thursday, February 10, 2011
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011
and how to hide mad cow disease in Canada Current as of: 2011-01-31
Wednesday, August 11, 2010
REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM
ENCEPHALOPATHY (BSE) IN CANADA
Thursday, August 19, 2010
REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM
ENCEPHALOPATHY (BSE) IN CANADA
Friday, March 4, 2011
Alberta dairy cow found with mad cow disease
Monday, June 25, 2012
US Department of Agriculture ends funding for chronic wasting disease
CWD
for all those game farmers that thought the USDA was the save all to the
cervid game farming and ranching with CWD, instead of the DNR. please see ;
Thursday, March 29, 2012
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012
NIAA Annual Conference April 11-14, 2011San Antonio, Texas
Monday, June 11, 2012
another atypical Nor-98 Scrapie case documented in Canada for 2012
MEXICO
well, Mexico has not a clue about anything that has to do with
Transmissible Spongiform Encephalopathy in humans or animals, thanks to the OIE
and the USDA et al and their policy making $$$
Wednesday, June 13, 2012
MEXICO IS UNDER or MIS DIAGNOSING CREUTZFELDT JAKOB DISEASE AND OTHER PRION
DISEASE SOME WITH POSSIBLE nvCJD
previous USA PRION UNIT reports ;
Tuesday, June 26, 2012
Creutzfeldt Jakob Disease Human TSE report update North America, Canada,
Mexico, and USDA PRION UNIT as of May 18, 2012
type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the
rise in Canada and the USA
Wednesday, May 16, 2012
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion
disease, Iatrogenic, what if ?
Proposal ID: 29403
Published March 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease
in the United States
Terry S. Singeltary, retired (medically)
I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment
on the CDC's attempts to monitor the occurrence of emerging forms of CJD.
Asante, Collinge et al [1] have reported that BSE transmission to the
129-methionine genotype can lead to an alternate phenotype that is
indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD
and all human TSEs are not reportable nationally. CJD and all human TSEs must be
made reportable in every state and internationally. I hope that the CDC does not
continue to expect us to still believe that the 85%+ of all CJD cases which are
sporadic are all spontaneous, without route/source. We have many TSEs in the USA
in both animal and man. CWD in deer/elk is spreading rapidly and CWD does
transmit to mink, ferret, cattle, and squirrel monkey by intracerebral
inoculation. With the known incubation periods in other TSEs, oral transmission
studies of CWD may take much longer. Every victim/family of CJD/TSEs should be
asked about route and source of this agent. To prolong this will only spread the
agent and needlessly expose others. In light of the findings of Asante and
Collinge et al, there should be drastic measures to safeguard the medical and
surgical arena from sporadic CJDs and all human TSEs. I only ponder how many
sporadic CJDs in the USA are type 2 PrPSc?
Published March 26, 2003
Wednesday, April 25, 2012
USA MAD COW DISEASE AND CJD THERE FROM SINGELTARY ET AL 1999 – 2012
just made a promise to mom, there was no information for us back
then...
layperson
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
