Wednesday, June 27, 2012

First US BSE Case Since 2006 Underscores Need for Vigilance Neurology Today 21 June 2012

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










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



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