Sunday, May 6, 2012

Bovine Spongiform Encephalopathy Mad Cow Disease, BSE May 2, 2012 IOWA State University OIE




Bovine Spongiform Encephalopathy Mad Cow Disease, BSE May 2, 2012


Importance


Bovine spongiform encephalopathy (BSE) is a fatal neurodegenerative disease, caused by a prion, that mainly affects cattle. Other ruminant species, cats, non-human primates and humans are occasionally affected; this disease is called feline spongiform encephalopathy (FSE) in cats, and variant Creutzfeldt–Jakob disease (vCJD) in people. BSE is a relatively new disease that was first reported in the United Kingdom in the 1980s. It is spread by ingestion; animals or humans become infected when they eat prion-containing tissues from an infected animal. Cooking and standard disinfection procedures do not destroy this agent. Infected animals or people do not become ill for years; however, the disease is always progressive and fatal once the symptoms develop.


The origins of BSE are unknown; however, the recycling of ruminant proteins in ruminant feed amplified this prion and caused an explosive epidemic in the U.K in the 1980s and 1990s. This epidemic peaked in 1992, with almost 1,000 new cases diagnosed each week. Although cases continue to be detected, control measures have greatly decreased their prevalence; fewer than 15 bovine cases were reported annually in the U.K. during 2009-2011. BSE also spread to many European countries, North America, parts of Asia, and possibly other areas of the world. The presence of BSE in a country can result in trade sanctions, as well as increased public concern about meat safety. Many nations, including the U.S., conduct control and surveillance programs. Many countries have also passed new regulations to prevent BSE-containing tissues from entering human or animal food supplies.


As a result of increased surveillance, BSE prions that differ from the prion causing ‘classical’ BSE have been identified at very low levels in cattle populations. Currently, it is thought that these “atypical” prions may represent a spontaneous form of prion disease. Some experiments suggest that an atypical prion might have given rise to the BSE epizootic when it was amplified in cattle feed.


Etiology


BSE is a member of the transmissible spongiform encephalopathies (TSEs), a group of neurodegenerative disorders caused by unconventional disease agents. These agents are resistant to the treatments that ordinarily destroy bacteria, spores, viruses, and fungi. They are generally thought to be prions, although a minority opinion suggests that TSEs may be caused by virinos or retroviruses. Prions are infectious proteins that appear to replicate by converting a normal cellular protein into copies of the prion. The cellular protein, which is called PrPc, is found on the surface of neurons. Pathogenic isoforms of PrPc are designated PrPres; PrPSc or PrPTSE are other names for this protein. Prions that cause different diseases (e.g. BSE or scrapie) are considered to be different strains of PrPres.


In addition to the ‘classical’ BSE prion, at least two atypical BSE prions can be found in cattle. One has higher molecular mass fragments than classical BSE and is called ‘H-type’ BSE or H-BSE; the other has a lower molecular mass and is called ‘L-type’ BSE or L-BSE. Some authors call the disease caused by the latter organism ‘bovine amyloidotic spongiform encephalopathy (BASE).’ Atypical BSE prions are thought to represent additional strains of BSE. Currently, the most likely hypothesis is that these prions arise spontaneously in cattle, similarly to some prion diseases in other species (e.g., spontaneous Creutzfeldt–Jakob disease in humans). Atypical L-BSE has been reported to change to a classical BSE phenotype on transmission to inbred mice or to some transgenic mice. Similarly, H-BSE developed features of classical BSE in some wild type mice. This has led to the suggestion that one of these prions may have originally given rise to the BSE epidemic after amplification through the food chain.


Geographic Distribution


Cases of BSE have been reported in indigenous cattle in most European countries, Canada, the U.S., Israel, and Japan. This disease was seen in imported cattle in the Falkland Islands and Oman. Some countries including Iceland, Australia, and New Zealand appear to be free of BSE. The presence or absence of this disease cannot be determined in countries without adequate surveillance programs.


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Atypical BSE prions have been reported in Europe, the U.S., Canada, and Japan, as the result of surveillance programs for BSE. They are also likely to exist in other countries.


Transmission


BSE is usually transmitted when an animal or human ingests tissues containing the BSE prion. Young animals may be particularly susceptible to infection; some studies suggest that most cattle become infected with BSE during the first six months of life. The prions are thought to replicate initially in the Peyer’s patches of the ileum, then are transported via the peripheral nerves to the central nervous system (CNS). In cattle, prions can accumulate in the brain as early as 24 months after infection. The risks of transmission from various tissues are still incompletely understood; however, the highest prion concentration occurs in the CNS and ileum. In naturally infected cattle, BSE prions have been found mainly in the brain, spinal cord, retina, and distal ileum, but more sensitive techniques have recently detected this agent in the dorsal root ganglia, peripheral nerves (including the optic, facial and sciatic nerves), and adrenal glands. In experimentally infected cattle, it has been reported from the CNS, dorsal root ganglia, trigeminal ganglion, thoracic ganglia, some peripheral nerves, distal ileum (particularly in the Peyer’s patches), jejunum, ileocecal junction, cecum, colon, myenteric plexus of the intestines, adrenal glands, tonsils, and bone marrow. In one animal, immunostaining detected prions in the macrophages of the subiliac lymph nodes but not other lymph nodes tested. In this animal, very weak immunostaining was also detected in the renal tubular epithelial cells, the thymus, and the islets of Langerhans. Using a very sensitive system (transgenic mouse bioassay), infectivity was recently detected in the tongue and nasal mucosa of cattle in the terminal stages of the disease, although no prions could be found by immunoblotting or the protein misfolding cyclic amplification (PMCA) method. Unpublished data suggested that BSE prions might occur in the lymphoid tissues of nictitating membranes, but no evidence to confirm this has been published.


In some tissues, the quantity of prions may be low or the incidence rare, and the risk of transmission is uncertain. Some tissues may contain prions only in the late stages of the disease. For example, the accumulation of these agents in the peripheral nerves and adrenal gland seems to coincide with or follow prion accumulation in the CNS. Classical BSE has not been found in muscle, except in one sample tested by mouse bioassay, where infectivity was thought to be associated with the endings of the sciatic nerve. However, meat could become contaminated with CNS tissues during slaughter or processing. For this reason, high-risk slaughter and processing techniques have been banned in many nations (see ‘Prevention’). Epidemiological evidence and transmission studies suggest that BSE is not transmitted in milk, semen, or embryos.


There is no evidence that BSE is transmitted horizontally between cattle; however, there is an unexplained increase in the risk of BSE among the offspring of infected animals. In one study, the risk that a calf would develop BSE appeared to be higher when the dam was in the later stages of infection (i.e., nearer to the onset of clinical signs). These observations have led to speculation that vertical transmission might be possible. If it occurs, vertical transmission seems to be rare, and the route is unknown. One model suggested that the cumulative risk of BSE transmission from dam to offspring is about 2%; however, the confidence interval included zero.


BSE transmission in experimentally infected sheep resembles transmission in cattle, but the prions are more widely disseminated in the body, and additional routes of transmission may occur. In sheep inoculated orally, BSE prions are readily found in many lymphoid tissues including the spleen, lymph nodes, and gut-associated lymphoid tissue (GALT), as well as in the CNS. Blood-borne transmission has been demonstrated in this species. Transmission from two ewes to their lambs occurred in an experimental flock; it is not known whether this event took place in utero or soon after birth.


Atypical BSE


In cattle, some studies report that the tissue distribution of atypical L-BSE and H-BSE seems to resemble that of classical BSE, with prions detected mainly in the CNS. (There are, however, some differences in the pattern of distribution within the brain.) H-BSE and L-BSE have also been found in peripheral nerves and sensory receptors (muscle spindles) and the trigeminal ganglion in some studies, and L-BSE was detected in the adrenal gland. In a recently published study, PrPres was reported to occur in the muscles of L-BSE infected cattle by immunostaining, and infectivity was found in muscle homogenates using a transgenic mouse bioassay.


Very little is known about the potential for vertical transmission. One calf born to a cow in the late stages of infection with L-BSE did not have any evidence of infection.


Transmission to humans and iatrogenic spread


In humans, variant Creutzfeldt-Jakob disease usually results from the ingestion of BSE prions. Based on studies in humanized transgenic mice, some authors have suggested that BSE isolates from sheep and goats might be more readily transmitted to humans than isolates from cattle. Iatrogenic transmission has also been seen. Probable person-to-person spread was reported in several patients who received blood transfusions from asymptomatically infected individuals. There is also potential for transmission by routes such as transplantation or the use of prion-contaminated Bovine Spongiform Encephalopathy


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equipment during surgeries. Prions can be found in the brain, spinal cord, dorsal root ganglia, trigeminal ganglia, retina, optic nerves, and lymphoid tissues of humans with vCJD. Although prions are particularly common in the spleen, tonsils, appendix and other gut-associated lymphoid tissues (GALT), they can also be found in lymph nodes throughout the body. Prions have been found in the appendix as early as two years before the onset of clinical disease. They have not been demonstrated in human blood, but this may be due to the insensitivity of the assays used to detect these agents. Person-to-person transmission of vCJD does not occur during casual contact.


Origins of the BSE epidemic


The origins of BSE are not well understood. This disease was first reported in the 1980s, but it was probably present in cattle since the 1970s or earlier. The two most popular hypotheses are that BSE originated as a spontaneous PrPc mutation in cattle, or that it came from a mutated scrapie prion that contaminated ruminant feed. Other sources suggest that BSE might have originated from a wildlife population or a human TSE agent. Once the BSE agent entered cattle populations, it was amplified by recycling tissues from infected cattle into ruminant feed supplements, mainly as meat-and-bone meal (MBM). MBM is a rendered concentrate derived from animal offal and carcasses. Rendering cannot completely inactivate prions, but the epidemic may have been facilitated by changes in rendering practices that allowed more prions to survive.


Banning ruminant tissues from ruminant feed has significantly reduced the number of new cases of BSE, but cases have been reported in cattle born after these regulations came into effect (“born-after-the-ban” cases). These cases might be caused by the use of imported feed components produced under inadequate quality controls, illegal feeding of ruminant proteins, or cross-contamination of cattle feed with swine or poultry feed. Theoretical possibilities include inadequate heating of bone meal or tallow used in concentrates and milk replacers, horizontal transmission, or environmental reservoirs. Current diagnostic techniques are not sensitive enough to detect very low levels of prions, and there is little information on prion survival in the environment; however, hamster-adapted scrapie prions have been shown to survive in the soil for at least three years.


Disinfection


Prions can differ in their resistance to inactivation, and one study found that BSE prions from cattle were more resistant than prions from human spontaneous CJD, mouse-passaged BSE prions, or hamster prions.


Decontamination of prion-contaminated tissues, surfaces, and environments is difficult. These agents are highly resistant to most disinfectants (including formalin), heat, ultraviolet radiation, and ionizing radiation, particularly when they are protected in organic material or preserved with aldehyde fixatives, or when the prion titer is high. Prions can bind tightly to some surfaces, including stainless steel and plastic, without losing infectivity. Prions bound to metal seem to be highly resistant to decontamination. Few effective decontamination techniques have been published. A 1-2 N sodium hydroxide solution, or a sodium hypochlorite solution containing 2% available chlorine, has traditionally been recommended for equipment and surfaces. Surfaces should be treated for more than 1 hour at 20°C (68°F). Overnight disinfection is recommended for equipment. Cleaning before disinfection removes organic material that may protect prions. In experiments, milder treatments including a phenolic disinfectant, an alkaline cleaner (KOH with detergents), and an enzymatic cleaner combined with vaporized hydrogen peroxide were shown to inactivate scrapie prions. The alkaline cleaner and phenolic disinfectant were also effective against BSE and vCJD prions. New commercial decontaminants have been developed for prions. In one experiment, the most effective commercial reagents, using a rodent prion on stainless steel wires, were those that contained proteolytic agents. One of these solutions was prepared in an 2 M NaOH alkali carrier, which may have contributed to its effectiveness. A commercial alkali/detergent reagent was unable to completely decontaminate the wires.


Physical inactivation of prions can be carried out by porous load autoclaving at 134-138°C (273-280°F) for 18 minutes at 30 lb/in2, but residual infectivity has been demonstrated in some studies. Autoclaving items in water is more effective than autoclaving without immersion. Dry heat is less effective; hamster-adapted scrapie prions can survive dry heat at temperatures as high as 360°C (680°F) for an hour. A combination of chemical and physical decontamination can be more effective than either procedure alone; chemical disinfection should be carried out first, then the items should be rinsed and autoclaved. Anecdotal evidence suggests that decontamination of contaminated facilities is very difficult. Even the harshest combination of chemical and physical disinfection is not guaranteed to destroy all prions. In experiments, a stainless-steel wire remained infectious after cleaning with sodium hydroxide and autoclaving. Surgical instruments that have undergone repeated cycles of cleaning and disinfection have transmitted the sporadic (genetic) form of CJD iatrogenically. For this reason, disposable equipment and instruments may be recommended instead of disinfection during some medical procedures.


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Infections in Humans


Incubation Period


The incubation period for vCJD is difficult to establish with certainty; however, the average incubation period is estimated to be 11 to 12 years, and incubation periods up to 16 years have been reported. In three cases transmitted in blood transfusions, the incubation period was 6 to 8.5 years. For comparison, some other human prion diseases have similar median incubation periods, but have been reported up to 40 years after exposure.


Clinical Signs


The symptoms of vCJD are broadly similar to the sporadic (genetic) form of CJD, but usually appear in younger patients. The median age of onset is 26 years (range 12 to 74 years). The first signs are usually psychiatric symptoms, such as anxiety, depression, insomnia, social withdrawal, and/or persistent painful sensory symptoms. In most patients, frank neurological signs such as gait disturbances, ataxia, incoordination, memory loss, slurring of speech, and tremor appear a few months later; however, neurological signs coincide with or precede psychiatric symptoms in a minority of patients. Cognitive function gradually deteriorates. Chorea, dystonia, myoclonus, visual disturbances, and dementia typically develop late in the course of disease. Most patients die in six months to two years.


Communicability


Person-to-person transmission of vCJD does not occur during casual contact. Probable human-to-human spread has been reported in several patients who received blood transfusions from asymptomatically infected individuals. Other iatrogenic routes of transmission may be possible, including transmission in transplants or by contaminated equipment during surgeries. In humans, prions can be found in the CNS and many lymphoid tissues including the tonsils. Prions have been found in the appendix as early as two years before the onset of clinical disease.


Diagnostic Tests


A tentative diagnosis can be made before death by the history, clinical signs, and cortical atrophy on magnetic resonance imaging (MRI) of the brain. The electroencephalogram (EEG) is sometimes normal during the early stages of disease, but later develops characteristic abnormalities. A definitive diagnosis can be made if the abnormal prion protein is found in tonsil biopsies by immunoblot (Western blot) or immunohistochemistry. In other cases, the diagnosis is made by microscopic examination of brain tissue, usually at necropsy. Numerous amyloid plaques surrounded by vacuoles are found in vCJD; such plaques are seen in only 5-10% of cases of sporadic (genetic) CJD. Large amounts of prion protein can be found around the plaques by immunohistochemistry.


Treatment


No treatment is available, other than supportive care.


Prevention


Variant Creutzfeldt-Jakob disease can usually be avoided by not eating tissues from BSE-infected cattle. Some nations conduct active surveillance of cattle at slaughter (using rapid tests) to detect cases of BSE. Active surveillance for BSE has been conducted in the E.U. since 2001; however, the age limits have increased since the programs began. As of 2011, most E.U. member states must test cattle over the age of 48 months that die, undergo emergency slaughter, are killed for reasons other than human consumption, or display certain abnormalities at antemortem inspection. Healthy cattle over the age of 72 months and intended for human consumption must also be tested. Lower age limits apply to cattle from a few E.U. member states and some other areas. Japan conducts unusually rigorous testing. At one time, the Japanese government required all slaughtered cattle to be tested for BSE. Since 2005, it has required only cattle that are 21 months of age or older to be tested; however, there has been public resistance to relaxing the testing requirements, and local authorities have continued to test all slaughtered cattle regardless of age. Some countries with a low incidence of disease, including the U.S., test only a percentage of cattle at slaughter. In the U.S., surveillance is targeted particularly at high risk cattle such as nonambulatory animals and those with neurological disease. These animals cannot be used in human food, and the carcass is held until testing is complete. Because BSE has been detected in goats, and sheep are also susceptible to experimental infection, some countries may also conduct BSE surveillance in small ruminants. Tissues that have a high risk of transmitting BSE have been banned from human food in many countries. In the U.S., prohibited tissues include the brain, skull, eyes, trigeminal ganglia, dorsal root ganglia, spinal cord, and most of the vertebrae from cattle 30 months of age and older. The tonsils and distal ileum from all cattle are also banned. In the E.U., banned tissues include the skull (including the brain and eyes but not the mandible) and spinal cord in cattle over 12 months of age, and the spinal column in cattle over 30 months of age. The tonsils, entire intestines, and mesentery are not allowed from any cattle. Slaughter and processing techniques that have a high risk of contaminating muscle tissues with CNS have been prohibited in many countries, including the U.S.


Person-to-person transmission of vCJD can be reduced by the use of disposable surgical instruments in high risk surgeries, when this disease is suspected. Because prions can be found in the tonsils, some authors also suggest that disposable equipment always be used during tonsillectomies, in countries with a significant risk


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of this disease. Transmission in blood cannot be completely prevented with current techniques; however, many countries do not allow people who have spent time in the U.K. and/or other European countries to be blood donors. Some countries have taken other measures, such as universal leucodepletion of blood, to reduce the risk of vCJD. Prion filters have been developed to reduce infectivity in plasma, but are still being evaluated and are not in wide use. Some countries import fresh frozen plasma from low-risk countries for patients without dietary exposure to BSE (e.g., patients born after 1996 in the U.K.). Although laboratory or abattoir-related cases have not been reported, veterinarians and laboratory workers should always take precautions when conducting necropsies on BSE suspects or handling tissues; BSL-3 is the recommended level of protection. Standard precautions include the use of protective clothing and the avoidance of penetrating injuries, contamination of abraded skin, and ingestion. A negative pressure laminar flow hood should be used for tissue manipulations whenever possible. Because prions may be able to survive in the environment for years and are difficult to disinfect, precautions should be taken to avoid contamination of surfaces and equipment. Disposable plastic-coated paper sheets can be used to protect tables and other surfaces. Disposable instruments and work clothing can also be used. No vaccine is available.


Morbidity and Mortality


The prevalence of vCJD is unknown. Most cases have been seen in people who lived in either the U.K. or France during the peak of the BSE epidemic. As of April 2012, 176 cases of vCJD had been reported in the U.K. The incidence peaked in 2000, when 28 cases were diagnosed, and gradually fell to five cases per year in 2005. Between 2006 and 2011, two to five cases were reported each year. As of April 2012, 25 cases had been reported from France, as well as four from the Republic of Ireland, five from Spain, three each from the United States and the Netherlands, and two from Canada, Portugal, and Italy. Japan, Saudi Arabia, and Taiwan have each reported one case. To date, all cases of vCJD in the U.S. seem to have been acquired in other countries. The number of people who are infected but asymptomatic is unknown. Based on the pattern of infection in the U.K, some sources suggest that, at most, 70 additional cases can be expected; however, surveillance conducted on appendectomy samples in the U.K. suggested a prevalence of 237 cases per million population, with 95% confidence intervals of 49-692. Another study, based on samples from tonsils, estimated a prevalence of 1 case per 10,000 population.


Variant Creutzfeldt–Jakob disease is usually seen in young patients. The reason is unknown, but it is possible that children and adolescents are more susceptible to infection than adults. The median age of onset is 26 years for vCJD (range 12 to 74 years); in contrast, it is 65 years (range 15 to 94 years) in the sporadic (genetic) form of Creutzfeldt–Jakob disease. People who are homozygous for methionine at codon 129 in the PrPC protein have an increased risk of developing vCJD. All clinical cases have occurred in people with this genotype. One infection was reported in a person who was heterozygous for methionine/valine at this codon, but did not develop vCJD symptoms. This person became infected in a blood transfusion and died of unrelated causes after five years. It is not known whether people with resistant genotypes (valine/valine or methionine/ valine) are completely resistant to the development of disease, or simply have a longer incubation period. Once the symptoms of vCJD develop, this disease is always fatal.


As of 2012, no human infections have been reported with atypical BSE prions, but these prions are also likely to be of zoonotic concern. In particular, L-BSE seems to be more virulent than classical BSE in intracerebrally inoculated macaques and humanized transgenic mice, with a shorter incubation period and more rapid progression. There is currently no evidence that this is the case for H-BSE; however, H-BSE (like L-BSE) is capable of changing to resemble classical BSE in transgenic mice.


Infections in Animals


Species Affected


BSE mainly occurs in cattle, but the host range of this prion is unusually broad compared to most prions. BSE has been reported from exotic ruminants in zoos; affected species include nyala (Tragelaphus angasi), kudu (Tragelaphus strepsiceros), gemsbok (Oryx gazella), eland (Taurotragus oryx), Arabian oryx (Oryx leucoryx), scimitar-horned oryx (Oryx dammah), ankole cattle, and bison (Bison bison). Rare field cases have been documented in goats, and experimental infections have been reported in both sheep and goats. European red deer (Cervus elaphus elaphus) are susceptible to oral exposure at a high dose, as well as to intracerebral inoculation, and develop neurological signs. BSE prions have also caused disease in various felids including housecats, cheetahs (Acinonyx jubatus), pumas (Felis concolor), ocelots (Felis pardalis), tigers (Panthera tigris), and Asian golden cats (Catopuma temminckii). (See the feline spongiform encephalopathy factsheet for details on infections in felids.) Two lemurs at a French zoo were apparently infected in contaminated feed. In addition, the BSE agent has been experimentally transmitted to mink, mice, marmosets, squirrel monkeys (Saimiri sciureus) and cynomolgus macaques (Macaca fascicularis). Pigs could be infected by the intracranial, intravenous, and intraperitoneal routes, but short-term feeding trials did not cause disease. One study reported that sea bream (Sparus aurata) might be susceptible to infection, although they did not develop clinical signs.


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L-BSE can infect cynomolgus macaques by intracerebral inoculation. It has also been transmitted to lemurs by the oral route, with the development of neurological signs. L-BSE and H-BSE can infect mice by intracerebral inoculation. Incubation Period


The incubation period for classical BSE is estimated to be 2 to 8 years in cattle. The peak incidence of disease occurs in four to five year old animals. Atypical BSE is usually detected in cattle that are at least eight years of age. Some research suggests that the incubation period might be shorter for atypical L-BSE than classical BSE; however, this is based on comparisons in intracerebrally inoculated cattle and bovinized transgenic mice. The incubation period in experimentally infected sheep varies with the animal’s age and genetic susceptibility, and the route of exposure and dose. In genetically susceptible sheep, the incubation period was 21 to 38 months for animals inoculated orally at six months of age, and 18 to 24 months in lambs inoculated orally at two weeks of age. In genetically resistant (ARR/ARR) sheep, the incubation period was approximately 3 to 5 years. One European red deer challenged by the oral route developed clinical signs after approximately 4 years, 9 months, while four other deer were still healthy more than 5 years after challenge. In experimentally infected macaques inoculated orally, the incubation period was 3.6 to 5 years.


Clinical Signs


Cattle with classical BSE


Bovine spongiform encephalopathy is a neurological disease that usually has an insidious onset in cattle. The symptoms may include gait abnormalities (particularly hindlimb ataxia), hyperresponsiveness to stimuli, tremors, and behavioral changes such as aggression, nervousness or apprehension, changes in temperament, and even frenzy. The combination of behavioral changes, hyperreactivity to stimuli, and gait abnormalities is highly suggestive of BSE, but some animals exhibit only one category of neurological signs. Pacing, a modified gait in which the legs move in lateral pairs, occurred in 25% of the cattle with BSE in one study, and may be suggestive of this disease. Intense pruritus is not usually seen in cattle, but some animals may lick or rub persistently. Nonspecific signs include loss of condition, weight loss, teeth grinding (possibly due to visceral pain or neurological disease), and decreased milk production. Decreased rumination, bradycardia, and altered heart rhythms have also been reported. The symptoms of BSE usually worsen gradually over a few weeks to six months, but rare cases can develop acutely and progress rapidly. Rapid, acute onset neurological disease seems to be particularly common in exotic ruminants in zoos. Once the symptoms appear, BSE is always progressive and fatal. The final stages are characterized by recumbency, coma, and death. Cattle with atypical BSE


The features of atypical BSE in cattle are still incompletely understood. Most atypical strains have been found in asymptomatic cattle during routine surveillance, in fallen stock (‘downer’ cattle), or at emergency slaughter. However, H-BSE associated with neurological signs was reported in a 19-year-old zebu bull (Bos indicus) at a zoo. Experiments (all using intracerebrally inoculated cattle) have reported varying clinical signs, with some researchers concluding that L-BSE can be distinguished clinically from classical BSE, and others reporting that the spectrum of clinical signs overlaps to a greater or lesser extent between all forms of BSE.


In Friesian and Alpine brown cattle, one group of researchers reported that an Italian isolate of L-BSE mainly causes a form characterized by inactivity, mental dullness, and muscle atrophy, which could be distinguished from classical BSE. The early clinical signs included muscle fasciculations, a dull coat, decreased alertness, low carriage of the head, and mild kyphosis. These signs progressed to muscle atrophy, which began in the gluteal region and progressed to involve other areas, with relative sparing of forelimb muscles. Although a “downer” cow was reported in this study, other animals did not develop ataxia or difficulty rising. However, sudden falls were seen. The animals in this study were reported to be hyperresponsive to tactile facial stimuli, but not to light or sound. In this experiment, the same breeds inoculated with classical BSE prions developed behavioral changes including aggressiveness, bellowing and head shaking, as well as postural abnormalities and hyperresponsiveness to stimuli.


Another group reported that, in Holstein–Friesian cattle inoculated with German isolates of H-BSE and L-BSE, the first signs were weight loss and loss of condition. Animals tended to separate from the herd and carried their head low. However, these cattle were hyperresponsive to acoustic and visual stimuli as well as tactile facial stimuli, similarly to cattle with classical BSE. Ataxia and difficulty rising were also reported in this experiment. These researchers concluded that, although the initial signs appear to be more nonspecific and subtle in atypical BSE, the differences are not sufficient to unambiguously distinguish these forms from classical BSE.


Another experiment used Danish Holstein/ Aberdeen Angus crosses inoculated with an Italian L-BSE strain and an H-BSE strain. Both “dull” and “nervous” forms of the illness were reported in this study. Behavioral, sensory, and motor signs were all seen, and cattle infected with H-BSE and L-BSE had similar clinical signs. Low head carriage and separation from the herd did not occur consistently, and most of the animals had no signs of


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dullness. Instead, many animals became hyperreactive to external stimuli including tactile and facial stimuli. No cattle developed tremors. In this study, the cattle tended to develop dysmetria and have difficulty in rising, early in the course of the disease, but none progressed to permanent recumbency (unlike animals with classical BSE that develop ataxia).


A study that used a Japanese L-BSE isolate in Holstein cattle reported decreased activity, hyperresponsiveness to stimuli, ataxia mainly of the hindlegs, difficulty rising, and little aggression.


Sheep with classical BSE


Various neurological signs have been reported in experimentally infected sheep. In one study, Cheviot sheep mainly developed ataxia with minimal pruritus, and died in a few days to a week. In indigenous French breeds, the symptoms included ataxia and intense pruritus with loss of fleece. These animals deteriorated slowly and died in approximately three months. A third study mainly used ARQ homozygous Suffolk and Romney sheep, but also included a few individuals of other breeds, and reported that the clinical signs were similar in all animals. Pruritus was detected in all clinically affected sheep in this study (however, it should be noted that this sign was also reported in 29% of the sheep that did not have evidence of BSE at slaughter). Other signs in some animals included behavioral changes, teeth grinding, movement abnormalities including tremor and ataxia, hypperresponsiveness to auditory stimuli or decreased menace response in a few animals, and weight loss and loss of body condition. Altered behavior combined with ataxia and pruritus was detected in 40% of these sheep. The course of the illness lasted 16 to 20 weeks before animals were culled due to the progression of neurological signs.


Goats with classical BSE


The few BSE cases that have been reported in naturally infected goats were discovered during routine surveillance at slaughter. One goat was reported to be a scrapie suspect. Neurological signs have been reported in experimentally infected animals. In one study, the disease was characterized by ataxia and tremors, and progressed rapidly in intracerebrally inoculated goats; however, the signs in orally inoculated goats were mainly lethargy and weight loss, which progressed to recumbency over three weeks. Ataxia was not seen in orally inoculated goats, and neither intracerebrally nor orally inoculated goats had signs of pruritus. In another study, intracerebrally inoculated Saanen goats developed abnormalities in movement (e.g., ataxia, tremors, postural deficits, and especially hypermetria) and hyperresponsiveness to stimuli. Over the course of the experiment, sniffing and nibbling of the animal handlers and instruments changed to aversive behavior, including head tossing or shaking, or kicking, and these signs became more pronounced with time. One goat carried its head low when undisturbed and was inappetent. Other signs in some animals included pruritus, an absent menace response, teeth grinding, and weight loss.


Post Mortem Lesions


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Gross lesions are not found in BSE, with the exception of nonspecific signs, such as emaciation or wasting. The histopathologic lesions are confined to the CNS. Neuronal vacuolation and non-inflammatory spongiform changes in the gray matter are characteristic of the disease in cattle. These lesions are usually but not always bilaterally symmetrical. Amyloid plaques are not typical of classical BSE or infection with H-BSE, but are associated with L-BSE prions. Similar spongiform changes occur in experimentally infected sheep and macaques.


Communicability


There is no evidence that the BSE agent is transmitted horizontally between cattle; however, the offspring of infected animals have an increased risk of developing this disease. If vertical transmission is possible, the route is unknown. Diagnostic Tests


There is no live animal test for BSE. This disease is usually diagnosed by detecting prions (PrPres) in the CNS. Accumulations of prions can be found in unfixed brain extracts by immunoblotting, and in fixed brains by immunohistochemistry. In addition, several rapid diagnostic tests based on enzyme-linked immunosorbent assays (ELISAs), automated immunoblotting (Western blotting) and lateral flow devices (LFD) are available. Rapid tests allow large numbers of samples to be screened, and are often used in surveillance and slaughter testing. Positive samples in rapid tests are traditionally confirmed with more specific assays such as immunohistochemistry or immunoblotting. However, the OIE now states that confirmation of positive results with a second rapid test is acceptable under some circumstances. Preferred test combinations, which are not in danger of producing false positives by the use of shared reagents, are listed on the OIE website. Two rapid tests can only be used to confirm a BSE case; a negative result on the confirmatory test is not adequate to rule out BSE, and should be investigated with other assays. A diagnosis of BSE may also be confirmed by finding characteristic prion fibrils called scrapie-associated fibrils (SAF) with electron microscopy in brain extracts. Some of these tests can be used on frozen or autolyzed brains. Techniques used diagnostically to detect prions are relatively insensitive compared to assays for other types of pathogens; prions cannot usually be detected in the brain until 3 to 6 months before the onset of disease.


Atypical prions can be detected with the same tests, including rapid tests, in animals infected with H-BSE or


Bovine Spongiform Encephalopathy


Last Updated: April 2012 © 2012 CFSPH page 8 of 13


L-BSE. A complete evaluation of the rapid tests has not been done for these prions. The distribution patterns of H-BSE and L-BSE in the brain differ somewhat from that of classical BSE, as well as from each other; however, all three prions can be detected in the obex. Atypical prions can be differentiated from classical BSE prions by their biochemical properties, for example by immunoblotting. H-BSE has higher molecular mass fragments than classical BSE. It also reacts with a monoclonal antibody to an N-terminal epitope that is not found in classical BSE after proteinase K cleavage. L-BSE has a lower molecular mass than classical BSE prions. Its glycosylation pattern differs from classical BSE, and it has an unusual deposition pattern characterized by amyloid plaques.


Histological examination of the brain can be very helpful in diagnosis, but some animals in early stages of infection have few or no spongiform changes. In addition, BSE can be detected by transmission studies in mice; however, an incubation period of several months makes this technique impractical for routine diagnosis. Serology is not useful for diagnosis, as antibodies are not made against the BSE agent.


Treatment


There is no treatment for BSE. Suspect animals are usually euthanized for testing.


Prevention


BSE can be prevented by not feeding ruminant tissues that may contain prions to susceptible species. Complete avoidance is generally necessary, as cooking or rendering cannot completely inactivate prions. Many nations have now banned the use of either ruminant or mammalian proteins, with certain exceptions such as milk and blood, in livestock feed. The specific bans, and protein sources prohibited, vary with the country. In some countries, bans also apply to other animal feeds, or even to fertilizer. The latter measures can help prevent cross-contamination and accidental exposure of cattle to BSE prions. Preventing prions from re-entering the ruminant food chain can interrupt transmission and control BSE epidemics; however, due to the long incubation period, the number of BSE cases may not decline for some time. In addition, countries may place trade bans on the importation of live cattle and certain ruminant proteins from affected countries.


BSE suspects are usually euthanized for testing. These carcasses cannot be used as food and must be destroyed. In the U.K., BSE carcasses are rendered at 133°C (3 bar pressure) for at least 20 minutes. Surveillance can help prevent infected animals from being used in food. Some nations conduct active surveillance of cattle at slaughter (using rapid tests) to detect cases of BSE. Active surveillance for BSE has been conducted in the E.U. since 2001; however, the age limits have increased since the programs began. As of 2011, cattle that must be tested (with rapid tests) in most E.U. member states include animals over the age of 48 months that die, undergo emergency slaughter, are killed for reasons other than human consumption, or display certain abnormalities at ante-mortem inspection. Healthy cattle over the age of 72 months and intended for human consumption must also be tested. Lower age limits apply to cattle from some other areas. Japan has unusually strict requirements. At one time, the Japan government required all cattle to be tested for BSE. Since 2005, only cattle that are 21 months of age or older must be tested; however, there has been public resistance to relaxing testing requirements, and local authorities have continued to test all slaughtered cattle regardless of age. Some countries may also conduct BSE surveillance in small ruminants.


Some countries with a low incidence of disease, including the U.S., test only a percentage of cattle at slaughter. In the U.S., surveillance is targeted particularly at high risk cattle such as nonambulatory animals and those with neurological disease. These animals cannot be used in human food, and the carcass is held until testing is complete. The U.S. also conducts passive surveillance for BSE. When an infected animal is identified, the affected herd is quarantined, and the source of the infection is investigated. Due to the increased risk of BSE in the offspring of infected cattle, they are usually traced and euthanized.


Tissues that have a high risk of transmitting BSE have been banned from human food in many countries. In the U.S., prohibited tissues include the brain, skull, eyes, trigeminal ganglia, dorsal root ganglia, spinal cord, and most of the vertebrae from cattle 30 months of age and older. The tonsils and distal ileum from all cattle are also banned. In the E.U., banned tissues include the skull (including the brain and eyes but not the mandible) and spinal cord in cattle over 12 months of age, and the spinal column in cattle over 30 months of age. The tonsils, entire intestine, and mesentery are not allowed from any cattle. Slaughter and processing techniques that have a high risk of contaminating muscle tissues with CNS have been prohibited in many countries, including the U.S.


Morbidity and Mortality


Classical BSE is seen most often in four to five year old cattle, particularly dairy animals. This disease is always fatal once the symptoms appear. The prevalence of BSE varies widely. At one time, the estimated prevalence in various countries ranged from more than 100 cases per million cattle to fewer than two cases per million. The latter are defined as World Organization for Animal Health (OIE) ‘minimal risk’ countries for BSE. Control measures have greatly decreased the prevalence in the most severely affected nations since that time.


BSE epidemics were reported in several European countries. The first outbreak occurred in the U.K., where more than 180,000 cases have been confirmed since the 1980s. The U.K. epidemic peaked in 1992, with nearly 1,000 new cases confirmed each week. At the time, the annual incidence in affected herds was approximately 2-


Bovine Spongiform Encephalopathy


Last Updated: April 2012 © 2012 CFSPH page 9 of 13


3%. As a result of control measures (particularly feed bans), the incidence declined to approximately 5-10 new cases per week in 2004. This number continued to drop, with the annual incidence decreasing to 99 confirmed cases in 2006, 35 cases in 2008, and 7 to 11 cases each year between 2009 and 2011.The peak of the epidemic curve occurred later in countries where feed bans were established more recently. In the U.S., only four cases of BSE have been reported. One case occurred in an animal imported from Canada. Three additional cases have been reported in indigenous cattle; one was caused by the H-form of atypical BSE. The most recent case (April 2012) was infected by an L-BSE prion, but the specific form has not yet been specified.


As of 2012, approximately 60 cases of L-BSE or H-BSE have been identified worldwide as a result of surveillance for classical BSE. The incidence of atypical BSE appears to be much lower than classical BSE. Its prevalence among cattle in France and Germany may be as low as 1 case per 3 million adult cattle. Nearly all L-BSE and H-BSE prions have been detected in cattle over the age of 8 years, with the exception of an L-BSE prion reported from a 23-month old steer in Japan.


Cases of BSE are very rarely reported in goats. Infections have not been seen in sheep or deer other than experimentally infected animals. Surveillance conducted in Europe suggests that the prevalence of BSE is very low in sheep, if it occurs at all. Estimates of the maximum proportion of sheep TSE cases that could be BSE range from 0.7% to 5%. Experimentally infected sheep that are genetically resistant to scrapie seem to have some resistance to BSE, but are not immune to infection or disease.


Internet Resources


snip...see full text with resources ;




COMMENT


please note Senior TSE PRION Scientist Paul Brown comments on spontaneous TSE ; How the California cow got the disease remains unknown. Government officials expressed confidence that contaminated food was not the source, saying the animal had atypical L-type BSE, a rare variant not generally associated with an animal consuming infected feed...


However, a BSE expert said that consumption of infected material is the only known way that cattle get the disease under natural conditons. “In view of what we know about BSE after almost 20 years experience, contaminated feed has been the source of the epidemic,” said Paul Brown, a scientist retired from the National Institute of Neurological Diseases and Stroke.


BSE is not caused by a microbe. It is caused by the misfolding of the so-called “prion protein” that is a normal constituent of brain and other tissues. If a diseased version of the protein enters the brain somehow, it can slowly cause all the normal versions to become misfolded.


It is possible the disease could arise spontaneously, though such an event has never been recorded, Brown said.






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


From: Terry S. Singeltary Sr.


Sent: Friday, May 04, 2012 9:59 AM


To: promedNOREPLY@promed.isid.harvard.edu ; promed-ahead@promedmail.org


Cc: ProMed ProMed ; Dawn Looken ; Craig Pringle ; joseph@promedmail.org


Subject: Re: PRO/AH> BSE, bovine - USA (05): (CA)


Greetings Promed, Dr. Detwiler et al,


If I am not mistaken, I believe that Promed et al and Dr. Detwiler overlooked Kong et al's finding that ;


"The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice"


P.4.23 Transmission of atypical BSE in humanized mouse models


Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University (Previously at USDA National Animal Disease Center), USA


Background: Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Atypical BSE cases have been discovered in three continents since 2004; they include the L-type (also named BASE), the H-type, and the first reported case of naturally occurring BSE with mutated bovine PRNP (termed BSE-M). The public health risks posed by atypical BSE were largely undefined.


Objectives: To investigate these atypical BSE types in terms of their transmissibility and phenotypes in humanized mice. Methods: Transgenic mice expressing human PrP were inoculated with several classical (C-type) and atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation time, characteristics and distribution of PrPSc, symptoms, and histopathology were or will be examined and compared. Results: Sixty percent of BASE-inoculated humanized mice be came infected with minimal spongiosis and an average incubation time of 20-22 months, whereas only one of the C-type BSE-inoculated mice developed prion disease after more than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse brains was biochemically different from bovine BASE or sCJD. PrPSc was also detected in the spleen of 22% of BASE-infected humanized mice, but not in those infected with sCJD. Secondary transmission of BASE in the humanized mice led to a small reduction in incubation time.*** The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice, but no BSE-M transmission has been observed so far.


Discussion: Our results demonstrate that BASE is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg mice. The possibility of more than two atypical BSE strains will be discussed.


Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.




SEE NEW LINK ;




P26 TRANSMISSION OF ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN HUMANIZED MOUSE MODELS


Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University, Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA.


*Previous address: USDA National Animal Disease Center, Ames, IA 50010, USA


Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have been discovered in three continents since 2004. The first case of naturally occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006 in the USA. The transmissibility and phenotypes of these atypical BSE strains/isolates in humans were unknown. We have inoculated humanized transgenic mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M isolate. We have found that the atypical BSE-L strain is much more virulent than the classical BSE-C.*** The atypical BSE-H strain is also transmissible in the humanized transgenic mice with distinct phenotype, but no transmission has been observed for the BSE-M isolate so far.


III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE, DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)






I ask Professor Kong ;


Thursday, December 04, 2008 3:37 PM


Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment


''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....''


Professor Kong reply ;


.....snip


''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete. Thanks for your interest.''


Best regards,


Qingzhong Kong,


PhD Associate Professor Department of Pathology Case Western Reserve


University Cleveland, OH 44106 USA


END...TSS


Tuesday, November 02, 2010


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




Thursday, December 04, 2008 2:37 PM


"we have found that H-BSE can infect humans."


personal communication with Professor Kong. ...TSS


BSE-H is also transmissible in our humanized Tg mice.


The possibility of more than two atypical BSE strains will be discussed.




Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.








"The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice"


a very important factor we cannot ignore. ...


kind regards,


terry


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


From: ProMED-mail Sent: Friday, May 04, 2012 8:25 AM


To: promed-ahead@promedmail.org


Subject: PRO/AH> BSE, bovine - USA (05): (CA)


BSE, BOVINE - USA (05): (CALIFORNIA)


************************************


A ProMED-mail post




ProMED-mail is a program of the International Society for Infectious Diseases http://www.isid.org


[1]


Date: 3 May 2012


From: Linda Detwiler [edited]


The moderator commented about my statements in the Nature News Blog [ProMED archive 20120501.1119136]. As I read the post, it appears to me that the reporter characterized my comments correctly. I have included the transmissions mentioned and references for these. This is not meant to be all-inclusive, and it does not explain the complexities of these studies.


- L-type BSE inoculated intracerebrally and intraperitoneally transmitted to transgenic mice overexpressing bovine PrP on a murine PrP knockout background (Tgbov XV) (Capobianco et al., 2007)


- L-type BSE inoculated intracerebrally transmitted to transgenic ovinized mice expressing the VRQ allele (Beringue et al., 2007).


- L-type BSE inoculated intracerebrally transmitted to mice transgenic for human PrP Met129 (Beringue et. al., 2008 - In this study H type was reported not to show evidence of transmission)


- L-type BSE transmitted to cynomolgus macaque intracerebrally (Comoy et. al., 2008)


- L-type BSE transmitted to mouse lemurs both intracerebrally and orally (Mestre-Frances et. al., 2012)


- L-type and H-type transmitted to wild-type mice intracerebrally (Wilson et.al. 2012)


- H-type BSE has transmitted intracerebrally to transgenic mice expressing ovine and bovine PrP (Beringue et. al., 2006; Buschmann et.al., 2006; Torres et. al., 2011)


It should be noted that the studies listed above represent atypical BSE isolates transmitted to a range of models including wild-type mice, transgenic mice overexpressing PrP from different species at different levels, on a variety of genetic backgrounds, and nonhuman primates. A very recent project (Wilson et. al., 2012) performed a comparison of the neuropathological and molecular properties of all 3 BSE agents (BASE, BSE-C and BSE-H) upon transmission into the same gene-targeted transgenic mouse line expressing the bovine prion protein (Bov6) and a wild-type control of the same genetic background. The results differed from observations made with the other models. Care must be taken in interpreting all of these findings. To repeat another statement I made, it is important for regulatory authorities to keep current with research and epidemiological findings and modify current policies as needed.


--


Linda A. Detwiler, DVM


lavet22@aol.com


Department of Pathobiology and Population Medicine


Mississippi State University


College of Veterinary Medicine


[It is completely agreed that interpretation of the research can be a challenge. Intracerebral modeling in other species, especially monogastric species (like mice) may have a different mechanism of prion development compared with oral ingestion by ruminant animals (e.g., cows). The inoculation mechanism (intracerebral vs. oral ingestion) as well as the physiologic differences between mice (or other monogastrastric species) and cattle (or other ruminant species) is also not completely comparable.


It should never be assumed that the interpretation of data in non-human primates is the same as for humans. However, non-human primates are closely related to humans and the physiological leap is not as far as the example in the previous paragraph. We appreciate Dr. Detwiler providing the additional summation and references. - Mod.TG]


******


snip...


A HealthMap/ProMED-mail map can be accessed at: <http://healthmap.org/r/1hcv>.] [see also: BSE, bovine - USA (04): (CA) 20120501.1119136 BSE, bovine - USA (03): (CA) 20120429.1117352 BSE, bovine - USA (02): (CA) OIE 20120428.1116584 BSE, bovine - USA: (CA) 4th animal confirmed 20120425.1113102 2006 ---- BSE, bovine - USA: feed recall 20060621.1718 BSE, bovine - USA: atypical strain (02) 20060607.1588 BSE, bovine - USA: atypical strain 20060601.1525 BSE, bovine - USA (AL)(02) 20060503.1280 BSE, bovine - USA (AL): conf. 20060313.0788 2005 ---- BSE - USA: Not 20050803.2261 BSE, bovine - USA (TX)(11): testing 20050710.1963 BSE, bovine - USA (10): Texas 20050701.1867 BSE, bovine - USA (10): Texas 20050701.1867 BSE, bovine - USA (09) 20050629.1837 BSE, bovine - USA (08) 20050628.1826 BSE, bovine - USA (07) 20050626.1806 BSE, bovine - USA (06): confirmed 20050624.1779 BSE - Austria (02): 2nd case (OIE) 20050623.1762 BSE - Austria: 2nd case 20050621.1739 BSE, bovine - USA (05): susp. 20050614.1664 BSE, bovine - USA (04): susp. 20050613.1657 BSE, bovine - USA (03): susp. 20050612.1637 BSE, bovine - USA: susp. 20050611.1625 BSE, bovine - USA: susp. (02): tests 20050611.1626 BSE - USA: policy change considered (03) 20050422.1119 BSE policy - USA: change considered (02) 20050420.1105 BSE policy - USA: change considered 20050418.1094 2004 ---- BSE, bovine - USA: susp (03) 20041118.3096 BSE, bovine - USA: susp. (02) 20040627.1720 BSE, bovine - USA: susp. 20040626.1713 BSE surveillance - USA (05) 20040603.1511 BSE surveillance - USA (TX) 20040507.1246 BSE, bovine - USA (WA) (19): tests, corr. 20040416.1047 BSE, bovine - USA (WA) (19): tests 20040414.1011 BSE update 2004 (03) 20040330.0866 BSE, bovine - USA (WA)(18) 20040323.0810 BSE, bovine - USA (WA)(05) 20040106.0058 BSE, bovine - USA (WA) 20040101.0004 2003 ---- BSE, bovine - USA (WA) (09): new regulations 20031230.3172 BSE, bovine - USA (WA) (08): recall 20031230.3166 BSE, bovine - USA (WA) (05) 20031226.3136 BSE, bovine - USA (WA) 20031223.3119] .................................................lm/tg/lm/msp/lm *##########################################################* ************************************************************ ProMED-mail makes every effort to verify the reports that are posted, but the accuracy and completeness of the information, and of any statements or opinions based thereon, are not guaranteed. The reader assumes all risks in using information posted or archived by ProMED-mail. ISID and its associated service providers shall not be held responsible for errors or omissions or held liable for any damages incurred as a result of use or reliance upon posted or archived material. ************************************************************ Donate to ProMED-mail. Details available at: <http://www.isid.org/ProMEDMail_Donations.shtml> ************************************************************ Visit ProMED-mail's web site at <http://www.promedmail.org>. Send all items for posting to: promed@promedmail.org (NOT to an individual moderator). If you do not give your full name name and affiliation, it may not be posted. You may unsub- scribe at <http://www.isid.org/promedmail/subscribe.lasso>. For assistance from a human being, send mail to: . ############################################################ ############################################################




May 2, 2012: Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States


Contact: Lyndsay Cole (970) 494-7410 Lawrence Hawkins (916) 930-5509


Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States


On April 24, USDA's Animal and Plant Health Inspection Service confirmed the nation's 4th case of Bovine Spongiform Encephalopathy (BSE) in an animal that was sampled for the disease at a rendering facility in central California. This animal was never presented for slaughter for human consumption, so at no time presented a risk to the food supply, or to human health in the United States.


Through its continuing epidemiological investigation, APHIS--in collaboration with the California Department of Food and Agriculture (CDFA)--has identified that one progeny born to the positive cow in the last 2 years was stillborn, and another has been located on a site in another state. That animal has been appraised, humanely euthanized, and sampled for BSE at the National Veterinary Services Laboratories in Ames, Iowa. Test results for that animal are negative for BSE. No birth cohort cattle have been located through the investigation.


A hold order has been placed on all cattle at a second dairy (dairy 2) that is associated with the dairy of the initial positive cow (also called the index dairy). Both dairies remain under quarantine. Inventories of both the index dairy and dairy 2 have been completed by CDFA. Records are still being matched and validated to determine if any at-risk cattle may be present.


In addition, a calf ranch where the initial positive cow was raised 10 years ago is being investigated.


The Food and Drug Administration and CDFA continue the investigation of feed records at the index dairy, rendering facility and calf ranch. To date, 10 feed firms have been identified as suppliers for the index dairy during the time period of interest. At the rendering facility, feed investigators confirmed that all domestic distribution of meat and bone meal meets federal labeling requirements.


USDA will continue to work closely with CDFA and FDA to provide additional information as it is available.


The United States has a longstanding system of three interlocking safeguards against BSE that protects public and animal health in the United States, the most important of which is the removal of specified risk materials - or the parts of an animal that would contain BSE should an animal have the disease - from all animals presented for slaughter in the United States. The second safeguard is a strong feed ban that protects cattle from the disease. The third safeguard - which led to this detection - is our ongoing BSE surveillance program that allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population.


#


Note to Reporters: USDA news releases, program announcements and media advisories are available on the Internet and through Really Simple Syndication (RSS) feeds. Go to the APHIS news release page at www.aphis.usda.gov/newsroom and click on the RSS feed link. USDA is an equal opportunity provider, employer and lender. To file a complaint of discrimination, write: USDA, Director, Office of Civil Rights, 1400 Independence Ave., SW., Washington, DC 20250-9410 or call (800) 795-3272 (voice) or (202) 720-6382 (TDD).




Friday, May 4, 2012


May 2, 2012: Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States






The United States has a longstanding system of three interlocking safeguards against BSE that protects public and animal health in the United States, the most important of which is the removal of specified risk materials - or the parts of an animal that would contain BSE should an animal have the disease - from all animals presented for slaughter in the United States.




Thursday, July 14, 2011


Valley Farm Meats (DBA Strasburg Provision, Inc) Issues Precautionary Recall for Beef Products Due to Possible Contamination with Prohibited Materials SRM Ohio Department of Agriculture and Ohio Department of Health




YET, FSIS seems to find it important enough to list this recall from Ohio ;


Ohio Firm Recalls Various Beef Jerky Products due to Misbranding and Undeclared Allergens


Recall Release CLASS II RECALL FSIS-RC-053-2011 HEALTH RISK: LOW


Congressional and Public Affairs (202) 720-9113 Adam Tarr


WASHINGTON, July 22, 2011 –




HOWEVER, look at the recalls of the past (see below), the first two were other voluntary recalls from other Companies, which i am using as an example (and see others that follow), but my question, WHY has the FSIS et al apparently chosen NOT to announce this recall on their website here ;


Valley Farm Meats (DBA Strasburg Provision, Inc) Issues Precautionary Recall for Beef Products Due to Possible Contamination with Prohibited Materials SRM Ohio Department of Agriculture and Ohio Department of Health




I have written both the FSIS and MEATINGPLACE.COM/, both of which have failed to report this important, life long exposure and human health risk factor for TSE from this voluntary recall, and all it got me was being banned from meatingplace.com/ again, and this time i did not even post anything, just sent them a kind note ;


see full text ;




SEE MORE SRM VIOLATIONS HERE ;




North Dakota Firm Recalls Whole Beef Head Products That Contain Prohibited Materials


Recall Release CLASS II RECALL FSIS-RC-023-2010 HEALTH RISK: LOW


Congressional and Public Affairs (202) 720-9113 Catherine Cochran


WASHINGTON, April 5, 2010 - North American Bison Co-Op, a New Rockford, N.D., establishment is recalling approximately 25,000 pounds of whole beef heads containing tongues that may not have had the tonsils completely removed, which is not compliant with regulations that require the removal of tonsils from cattle of all ages, the U.S. Department of Agriculture's Food Safety and Inspection Service (FSIS) announced today.


Tonsils are considered a specified risk material (SRM) and must be removed from cattle of all ages in accordance with FSIS regulations. SRMs are tissues that are known to contain the infective agent in cattle infected with Bovine Spongiform Encephalopathy (BSE), as well as materials that are closely associated with these potentially infective tissues. Therefore, FSIS prohibits SRMs from use as human food to minimize potential human exposure to the BSE agent.


The product subject to recall includes: Various weight cases of "Beef Heads KEEP FROZEN." Each case bears the establishment number "EST. 18859" inside the USDA mark of inspection and a case code number "16999." "North Dakota Natural Beef" is printed in the bottom left-hand corner of each label.


The recalled products were produced between June 25, 2009, and February 19, 2010. These products were shipped to distribution centers in Md., Mich., and Minn. for further sale.


The problem was discovered during FSIS inspection activities at the establishment. FSIS routinely conducts recall effectiveness checks to verify recalling firms notify their customers of the recall and that steps are taken to make certain that the product is no longer available to consumers.


Media with questions about the recall should contact Philip Wicke, Vice President of Operations, at (701) 356-7723. Consumers with questions about the recall should contact Jeremy Anderson, Director of Customer Service, at (952) 545-2495.


Consumers with food safety questions can "Ask Karen," the FSIS virtual representative available 24 hours a day at AskKaren.gov. The toll-free USDA Meat and Poultry Hotline 1-888-MPHotline (1-888-674-6854) is available in English and Spanish and can be reached from l0 a.m. to 4 p.m. (Eastern Time) Monday through Friday. Recorded food safety messages are available 24 hours a day. #




Missouri Firm Recalls Cattle Heads That Contain Prohibited Materials


Recall Release CLASS II RECALL FSIS-RC-021-2008 HEALTH RISK: LOW


Congressional and Public Affairs (202) 720-9113 Amanda Eamich


WASHINGTON, June 26, 2008 – Paradise Locker Meats, a Trimble, Mo., establishment, is voluntarily recalling approximately 120 pounds of fresh cattle heads with tonsils not completely removed, which is not compliant with regulations that require the removal of tonsils from cattle of all ages, the U.S. Department of Agriculture’s Food Safety and Inspection Service announced today.


Tonsils are considered a specified risk material (SRM) and must be removed from cattle of all ages in accordance with FSIS regulations. SRMs are tissues that are known to contain the infective agent in cattle infected with BSE, as well as materials that are closely associated with these potentially infective tissues. Therefore, FSIS prohibits SRMs from use as human food to minimize potential human exposure to the BSE agent.


The products subject to recall include: Boxes of “BEEF HEAD, PARADISE LOCKER MEATS.” Each shipping package bears the establishment numbers “EST. 31865” inside the USDA mark of inspection.


These products were sent to retail establishments and restaurants in the Kansas City, Kansas, area.


The problem was discovered through routine FSIS inspection that verified there had been incomplete removal of the tonsils by the recalling establishment.


Media and consumers with questions about the recall should contact company Production Supervisor Louis Fantasma at (816) 370-6328.


Consumers with food safety questions can “Ask Karen,” the FSIS virtual representative available 24 hours a day at AskKaren.gov. The toll-free USDA Meat and Poultry Hotline 1-888-MPHotline (1-888-674-6854) is available in English and Spanish and can be reached from l0 a.m. to 4 p.m. (Eastern Time) Monday through Friday. Recorded food safety messages are available 24 hours a day. #




HAS the greed and money gotten so bad that the FSIS, USDA, APHIS, OIE et al, just decided that not only to exempt the atypical Scrapies and apparently now the BSE's, exempt them all, and just agreed to choose to not even speak about it anymore. i mean...really, the USDA and OIE have systematically covered up mad cow disease i.e. they call it SSS policy. where is USA burying them all at ? i do not accept the star trek like cloaking device that appears to be the only thing left that could be protecting the USA from mad cow disease....really. sadly, Canada has now taken the same low road as the USA in regards to discussing and making public documents on there mad cow cases. all this, 2011, with the science mounting, still follow the global myth of the UKBSEnvCJD only theory, and that all the sporadic CJDs (85%+ of all human TSE) are a mear happenstance of bad luck, when North America is plum full of different strains of the Transmissible Spongiform Encephalopathy in different species, all of which over a period of time, decades, were rendered and fed to food producing animals for human and animal food...really. i really just don't buy it...tss


some history on SRM's IN COMMERCE ;


SEE FULL TEXT HERE ;


Tuesday, July 1, 2008


Missouri Firm Recalls Cattle Heads That Contain Prohibited Materials SRMs




Sunday, October 18, 2009


Wisconsin Firm Recalls Beef Tongues That Contain Prohibited Materials SRM WASHINGTON, October 17, 2009




Thursday, October 15, 2009


Nebraska Firm Recalls Beef Tongues That Contain Prohibited Materials SRM WASHINGTON, Oct 15, 2009




Thursday, June 26, 2008


Texas Firm Recalls Cattle Heads That Contain Prohibited Materials




Friday, August 8, 2008


Texas Firm Recalls Cattle Heads That Contain Prohibited Materials SRMs 941,271 pounds with tonsils not completely removed




Saturday, April 5, 2008


SRM MAD COW RECALL 406 THOUSAND POUNDS CATTLE HEADS WITH TONSILS KANSAS




Wednesday, April 30, 2008


Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings




Wednesday, April 30, 2008


Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings




Friday, October 15, 2010


BSE infectivity in the absence of detectable PrPSc accumulation in the tongue and nasal mucosa of terminally diseased cattle




SPECIFIED RISK MATERIALS SRMs








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




Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR> Prion disease update 2010 (11)


PRION DISEASE UPDATE 2010 (11)




Tuesday, May 3, 2011


PRION, TSE, typical, atypical BSE, aka mad cow disease, spray dried blood, feed, and a recipe for disaster






============================================




The second safeguard is a strong feed ban that protects cattle from the disease.






LET’S see how that mad cow triple firewall aka mad cow feed ban is working out $$$


*** BANNED MAD COW FEED IN THE USA IN COMMERCE TONS AND TONS


THIS is just ONE month report, of TWO recalls of prohibited banned MBM, which is illegal, mixed with 85% blood meal, which is still legal, but yet we know the TSE/BSE agent will transmit blood. we have this l-BSE in North America that is much more virulent and there is much concern with blood issue and l-BSE as there is with nvCJD in humans. some are even starting to be concerned with sporadic CJD and blood, and there are studies showing transmission there as well. ... this is one month recall page, where 10 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE, TO BE FED OUT. very little of the product that reaches commerce is ever returned via recall, very, very little. this was 2007, TEN YEARS AFTER THE AUGUST 4, 1997, PARTIAL AND VOLUNTARY MAD COW FEED BAN IN THE USA, that was nothing but ink on paper. i have listed the tonnage of mad cow feed that was in ALABAMA in one of the links too, this is where the infamous g-h-BSEalabama case was, a genetic relation matching the new sporadic CJD in the USA. seems this saga just keeps getting better and better.......$$$


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




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


###




please see full text ;




Tuesday, March 2, 2010


Animal Proteins Prohibited in Ruminant Feed/Adulterated/Misbranded Rangen Inc 2/11/10 USA




Monday, March 1, 2010


ANIMAL PROTEIN I.E. MAD COW FEED IN COMMERCE A REVIEW 2010




Tuesday, September 14, 2010


Feed Safety and BSE/Ruminant Feed Ban Support Project (U18)




Friday, October 8, 2010


Scientific reasons for a feed ban of meat-and-bone meal, applicable to all farmed animals including cattle, pigs, poultry, farmed fish and pet food






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




Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR>


Prion disease update 2010 (11) PRION DISEASE UPDATE 2010 (11)




Sunday, February 5, 2012


February 2012 Update on Feed Enforcement Activities to Limit the Spread of BSE




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.




==============================================




The third safeguard - which led to this detection - is our ongoing BSE surveillance program that allows USDA to detect the disease if it exists at very low levels in the U.S. cattle population.




How the California cow got the disease remains unknown. Government officials expressed confidence that contaminated food was not the source, saying the animal had atypical L-type BSE, a rare variant not generally associated with an animal consuming infected feed.


However, a BSE expert said that consumption of infected material is the only known way that cattle get the disease under natural conditons.


“In view of what we know about BSE after almost 20 years experience, contaminated feed has been the source of the epidemic,” said Paul Brown, a scientist retired from the National Institute of Neurological Diseases and Stroke.


BSE is not caused by a microbe. It is caused by the misfolding of the so-called “prion protein” that is a normal constituent of brain and other tissues. If a diseased version of the protein enters the brain somehow, it can slowly cause all the normal versions to become misfolded.


It is possible the disease could arise spontaneously, though such an event has never been recorded, Brown said.




counterpart: Creutzfeldt-Jakob disease (CJD).


In a statement released on 24 April, Karen Ross, Secretary of the California Department of Food and Agriculture said, “The detection of BSE shows that the surveillance program in place in California and around the country is working.” Food safety advocates such as Yonkers, New York, -based Consumers Union say it’s a warning sign that surveillance is inadequate and needs to be stepped up.


Ross’s statement also makes a point of noting a key feature of this particular case: The infected cow carried what is known as ‘L-type’ BSE, a version of the disease that has not been detected before in the US and has so far not been associated with transmission through animal feed. As the policy debate over testing rumbles on, here is a short guide to what is known and not known about this rare strain and its unexpected appearance.




STATEMENT FROM CDFA SECRETARY KAREN ROSS ON USDA ANNOUNCEMENT OF DETECTION OF BSE Release #12-016 Print This Release


SACRAMENTO, April 24, 2012 – CDFA Secretary Karen Ross issued this statement following the USDA’s announcement of the detection of atypical bovine spongiform encephalopathy (BSE) in a California dairy cow:


“The detection of BSE shows that the surveillance program in place in California and around the country is working. Milk and beef remain safe to consume. The disease is not transmitted through milk. Because of the strength of the food protection system, the cow did not enter the food or feed supply. There are numerous safeguards in place to prevent BSE from entering the food chain.


“The atypical BSE designation is important because this is a very rare form of BSE not generally associated with an animal consuming infected feed. CDFA veterinarians are working with the USDA to investigate this case and to identify whether additional cows are at risk. Feed restrictions in place in California and around the country for the last 15 years minimize that risk to the greatest degree possible. We will provide additional information about this case as it becomes available.”






CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...




CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006


In an article today for United Press International, science reporter Steve Mitchell writes:


Analysis: What that mad cow means


By STEVE MITCHELL UPI Senior Medical Correspondent


WASHINGTON, March 15 (UPI) -- The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.


The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.


These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.


"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."


Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.


USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.


"Everything they did on the Texas cow makes everything they did before 2005 suspect," Brown said.


Despite this, Brown said the U.S. prevalence of mad cow, formally known as bovine spongiform encephalopathy, or BSE, did not significantly threaten human or cattle health.


"Overall, my view is BSE is highly unlikely to pose any important risk either in cattle feed or human feed," he said.


However, Jean Halloran of Consumers Union in Yonkers, N.Y., said consumers should be troubled by the USDA's secrecy and its apparent plan to dramatically cut back the number of mad cow tests it conducts.


"Consumers should be very concerned about how little we know about the USDA's surveillance program and the failure of the USDA to reveal really important details," Halloran told UPI. "Consumers have to be really concerned if they're going to cut back the program," she added.


Last year the USDA tested more than 300,000 animals for the disease, but it has proposed, even in light of a third case, scaling back the program to 40,000 tests annually.


"They seem to be, in terms of actions and policies, taking a lot more seriously the concerns of the cattle industry than the concerns of consumers," Halloran said. "It's really hard to know what it takes to get this administration to take action to protect the public."


The USDA has insisted that the safeguards of a ban on incorporating cow tissue into cattle feed (which is thought to spread the disease) and removal of the most infectious parts of cows, such as the brain and spinal cord, protect consumers. But the agency glosses over the fact that both of these systems have been revealed to be inadequately implemented.


The feed ban, which is enforced by the Food and Drug Administration, has been criticized by the Government Accountability Office in two reports, the most recent coming just last year. The GAO said the FDA's enforcement of the ban continues to have weaknesses that "undermine the nation's firewall against BSE."


USDA documents released last year showed more than 1,000 violations of the regulations requiring the removal of brains and spinal cords in at least 35 states, Puerto Rico and the Virgin Islands, with some plants being cited repeatedly for infractions. In addition, a violation of similar regulations that apply to beef exported to Japan is the reason why Japan closed its borders to U.S. beef in January six weeks after reopening them.


Other experts also question the adequacy of the USDA's surveillance system. The USDA insists the prevalence of mad cow disease is low, but the agency has provided few details of its surveillance program, making it difficult for outside experts to know if the agency's monitoring plan is sufficient.


"It's impossible to judge the adequacy of the surveillance system without having a breakdown of the tested population by age and risk status," Elizabeth Mumford, a veterinarian and BSE expert at Safe Food Solutions in Bern, Switzerland, a company that provides advice on reducing mad cow risk to industry and governments, told UPI.


"Everybody would be happier and more confident and in a sense it might be able to go away a little bit for (the USDA) if they would just publish a breakdown on the tests," Mumford added.


UPI requested detailed records about animals tested under the USDA's surveillance plan via the Freedom of Information Act in May 2004 but nearly two years later has not received any corresponding documents from the agency, despite a federal law requiring agencies to comply within 30 days. This leaves open the question of whether the USDA is withholding the information, does not have the information or is so haphazardly organized that it cannot locate it.


Mumford said the prevalence of the disease in U.S. herds is probably quite low, but there have probably been other cases that have so far gone undetected. "They're only finding a very small fraction of that low prevalence," she said.


Mumford expressed surprise at the lack of concern about the deadly disease from American consumers. "I would expect the U.S. public to be more concerned," she said.


Markus Moser, a molecular biologist and chief executive officer of Prionics, a Swiss firm that manufactures BSE test kits, told UPI one concern is that if people are infected, the mad cow pathogen could become "humanized" or more easily transmitted from person to person.


"Transmission would be much easier, through all kinds of medical procedures" and even through the blood supply, Moser said.


© Copyright 2006 United Press International, Inc. All Rights Reserved






PAUL BROWN COMMENT TO ME ON THIS ISSUE


Tuesday, September 12, 2006 11:10 AM


"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency." ...END...TSS


IN A NUT SHELL ;


(Adopted by the International Committee of the OIE on 23 May 2006)


11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to then Central Bureau............




Audit Report Animal and Plant Health Inspection Service Bovine Spongiform Encephalopathy (BSE) Surveillance Program ­ Phase II and Food Safety and Inspection Service


Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III


Report No. 50601-10-KC January 2006


Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain




Report to Congressional Requesters:


February 2005:


Mad Cow Disease:


FDA's Management of the Feed Ban Has Improved, but Oversight Weaknesses Continue to Limit Program Effectiveness:








January 2002 MAD COW DISEASE Improvements in the Animal Feed Ban and Other Regulatory Areas Would Strengthen U.S. Prevention Efforts GAO-02-183






FOR IMMEDIATE RELEASE Statement May 4, 2004 Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA


Statement on Texas Cow With Central Nervous System Symptoms


On Friday, April 30 th , the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed.


FDA, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.


FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.


Cattle with central nervous system symptoms are of particular interest because cattle with bovine spongiform encephalopathy or BSE, also known as "mad cow disease," can exhibit such symptoms. In this case, there is no way now to test for BSE. But even if the cow had BSE, FDA's animal feed rule would prohibit the feeding of its rendered protein to other ruminant animals (e.g., cows, goats, sheep, bison).


FDA is sending a letter to the firm summarizing its findings and informing the firm that FDA will not object to use of this material in swine feed only. If it is not used in swine feed, this material will be destroyed. Pigs have been shown not to be susceptible to BSE. If the firm agrees to use the material for swine feed only, FDA will track the material all the way through the supply chain from the processor to the farm to ensure that the feed is properly monitored and used only as feed for pigs.


To protect the U.S. against BSE, FDA works to keep certain mammalian protein out of animal feed for cattle and other ruminant animals. FDA established its animal feed rule in 1997 after the BSE epidemic in the U.K. showed that the disease spreads by feeding infected ruminant protein to cattle.


Under the current regulation, the material from this Texas cow is not allowed in feed for cattle or other ruminant animals. FDA's action specifying that the material go only into swine feed means also that it will not be fed to poultry.


FDA is committed to protecting the U.S. from BSE and collaborates closely with the U.S. Department of Agriculture on all BSE issues. The animal feed rule provides crucial protection against the spread of BSE, but it is only one of several such firewalls. FDA will soon be improving the animal feed rule, to make this strong system even stronger.


####




Wednesday, August 20, 2008


Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?






Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model


Nadine Mestre-Francés, Simon Nicot, Sylvie Rouland, Anne-Gaëlle Biacabe, Isabelle Quadrio, Armand Perret-Liaudet, Thierry Baron, and Jean-Michel Verdier


We report transmission of atypical L-type bovine spongiform encephalopathy to mouse lemurs after oral or intracerebral inoculation with infected bovine brain tissue. After neurologic symptoms appeared, transmissibility of the disease by both inoculation routes was confirmed by detection of disease-associated prion protein in samples of brain tissue.


snip...


Conclusions


We demonstrated that the agent of L-BSE can be transmitted by the oral route from cattle to mouse lemurs. As expected, orally inoculated animals survived longer than IC-inoculated animals. Orally inoculated lemurs had less severe clinical signs and symptoms, with no evidence of motor dysfunction. It was previously suggested that the agent of L-BSE might be involved in the foodborne transmission of a prion disease in mink (11,12), a species in which several outbreaks of transmissible mink encephalopathy had been identified, notably in the United States (13).


Our study clearly confirms, experimentally, the potential risk for interspecies oral transmission of the agent of L-BSE. In our model, this risk appears higher than that for the agent of classical BSE, which could only be transmitted to mouse lemurs after a first passage in macaques (14). We report oral transmission of the L-BSE agent in young and adult primates. Transmission by the IC route has also been reported in young macaques (6,7). A previous study of L-BSE in transgenic mice expressing human PrP suggested an absence of any transmission barrier between cattle and humans for this particular strain of the agent of BSE, in contrast to findings for the agent of classical BSE (9). Thus, it is imperative to maintain measures that prevent the entry of tissues from cattle possibly infected with the agent of L-BSE into the food chain.






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.




Subject: Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate


Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate


Emmanuel E. Comoy1*, Cristina Casalone2, Nathalie Lescoutra-Etchegaray1, Gianluigi Zanusso3, Sophie Freire1, Dominique Marcé1, Frédéric Auvré1, Marie-Magdeleine Ruchoux1, Sergio Ferrari3, Salvatore Monaco3, Nicole Salès4, Maria Caramelli2, Philippe Leboulch1,5, Paul Brown1, Corinne I. Lasmézas4, Jean-Philippe Deslys1


1 Institute of Emerging Diseases and Innovative Therapies, CEA, Fontenay-aux-Roses, France, 2 Istituto Zooprofilattico Sperimentale del Piemonte, Turin, Italy, 3 Policlinico G.B. Rossi, Verona, Italy, 4 Scripps Florida, Jupiter, Florida, United States of America, 5 Genetics Division, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts, United States of America


Abstract Top Background


Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform Encephalopathy (cBSE). Atypical forms of BSE, which remain mostly asymptomatic in aging cattle, were recently identified at slaughterhouses throughout Europe and North America, raising a question about human susceptibility to these new prion strains.


Methodology/Principal Findings


Brain homogenates from cattle with classical BSE and atypical (BASE) infections were inoculated intracerebrally into cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously demonstrated to be susceptible to the original strain of cBSE. The resulting diseases were compared in terms of clinical signs, histology and biochemistry of the abnormal prion protein (PrPres). The single monkey infected with BASE had a shorter survival, and a different clinical evolution, histopathology, and prion protein (PrPres) pattern than was observed for either classical BSE or vCJD-inoculated animals. Also, the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine.


Conclusion/Significance


Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products.


Citation: Comoy EE, Casalone C, Lescoutra-Etchegaray N, Zanusso G, Freire S, et al. (2008) Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate. PLoS ONE 3(8): e3017. doi:10.1371/journal.pone.0003017


Editor: Neil Mabbott, University of Edinburgh, United Kingdom


Received: April 24, 2008; Accepted: August 1, 2008; Published: August 20, 2008


Copyright: © 2008 Comoy et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


Funding: This work has been supported by the Network of Excellence NeuroPrion.


Competing interests: CEA owns a patent covering the BSE diagnostic tests commercialized by the company Bio-Rad.


* E-mail: emmanuel.comoy@cea.fr


snip...


In summary, we have transmitted one atypical form of BSE (BASE) to a cynomolgus macaque monkey that had a shorter incubation period than monkeys infected with classical BSE, with distinctive clinical, neuropathological, and biochemical features; and have shown that the molecular biological signature resembled that seen in a comparatively uncommon subtype of sporadic CJD. We cannot yet say whether BASE is more pathogenic for primates (including humans) than cBSE, nor can we predict whether its molecular biological features represent a clue to one cause of apparently sporadic human CJD. However, the evidence presented here and by others justifies concern about a potential human health hazard from undetected atypical forms of BSE, and despite the waning epizoonosis of classical BSE, it would be premature to abandon the precautionary measures that have been so successful in reversing the impact of cBSE. We would instead urge a gradual, staged reduction that takes into account the evolving knowledge about atypical ruminant diseases, and both a permanent ban on the use of bovine central nervous system tissue for either animal or human use, and its destruction so as to eliminate any risk of environmental contamination.




Wednesday, March 31, 2010


Atypical BSE in Cattle


To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.


This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.




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...




October 2009 O.11.3


Infectivity in skeletal muscle of BASE-infected cattle


Silvia Suardi1, Chiara Vimercati1, Fabio Moda1, Ruggerone Margherita1, Ilaria Campagnani1, Guerino Lombardi2, Daniela Gelmetti2, Martin H. Groschup3, Anne Buschmann3, Cristina Casalone4, Maria Caramelli4, Salvatore Monaco5, Gianluigi Zanusso5, Fabrizio Tagliavini1 1Carlo Besta" Neurological Institute,Italy; 2IZS Brescia, Italy; 33FLI Insel Riems, D, Germany; 4CEA-IZS Torino, Italy; 5University of Verona, Italy


Background: BASE is an atypical form of bovine spongiform encephalopathy caused by a prion strain distinct from that of BSE. Upon experimental transmission to cattle, BASE induces a previously unrecognized disease phenotype marked by mental dullness and progressive atrophy of hind limb musculature. Whether affected muscles contain infectivity is unknown. This is a critical issue since the BASE strain is readily transmissible to a variety of hosts including primates, suggesting that humans may be susceptible.


Objectives: To investigate the distribution of infectivity in peripheral tissues of cattle experimentally infected with BASE. Methods: Groups of Tg mice expressing bovine PrP (Tgbov XV, n= 7-15/group) were inoculated both i.c. and i.p. with 10% homogenates of a variety of tissues including brain, spleen, cervical lymph node, kidney and skeletal muscle (m. longissimus dorsi) from cattle intracerebrally infected with BASE. No PrPres was detectable in the peripheral tissues used for inoculation either by immunohistochemistry or Western blot.


Results: Mice inoculated with BASE-brain homogenates showed clinical signs of disease with incubation and survival times of 175±15 and 207±12 days. Five out of seven mice challenged with skeletal muscle developed a similar neurological disorder, with incubation and survival times of 380±11 and 410±12 days. At present (700 days after inoculation) mice challenged with the other peripheral tissues are still healthy. The neuropathological phenotype and PrPres type of the affected mice inoculated either with brain or muscle were indistinguishable and matched those of Tgbov XV mice infected with natural BASE.


Discussion: Our data indicate that the skeletal muscle of cattle experimentally infected with BASE contains significant amount of infectivity, at variance with BSE-affected cattle, raising the issue of intraspecies transmission and the potential risk for humans. Experiments are in progress to assess the presence of infectivity in skeletal muscles of natural BASE.




MAD COW USDA ATYPICAL L-TYPE BASE BSE


***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.




full text ;


atypical L-type BASE BSE




Tuesday, May 1, 2012


BSE MAD COW LETTERS TO USDA (Tom Vilsack, Secretary of Agriculture) and FDA (Magaret Hamburg, Commissioner of FDA) May 1, 2012




Wednesday, May 2, 2012


ARS FLIP FLOPS ON SRM REMOVAL FOR ATYPICAL L-TYPE BASE BSE RISK HUMAN AND ANIMAL HEALTH




Subject: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68


Comment from Terry Singeltary


Document ID: APHIS-2008-0010-0008 Document Type: Public Submission


This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products


Docket ID: APHIS-2008-0010 RIN:0579-AC68


Topics: No Topics associated with this document


View Document:


More


Document Subtype: Public Comment Status: Posted Received Date: March 22 2012, at 12:00 AM Eastern Daylight Time Date Posted: March 22 2012, at 12:00 AM Eastern Daylight Time Comment Start Date: March 16 2012, at 12:00 AM Eastern Daylight Time Comment Due Date: May 15 2012, at 11:59 PM Eastern Daylight Time Tracking Number: 80fdd617 First Name: Terry Middle Name: S. Last Name: Singeltary City: Bacliff Country: United States State or Province: TX Organization Name: CJD TSE PRION Submitter's Representative: CONSUMERS


Comment:


comment submission Document ID APHIS-2008-0010-0001 Greetings USDA, OIE et al, what a difference it makes with science, from one day to the next. i.e. that mad cow gold card the USA once held. up until that fateful day in December of 2003, the science of BSE was NO IMPORTS TO USA FROM BSE COUNTRY. what a difference a day makes$ now that the shoe is on the other foot, the USDA via the OIE, wants to change science again, just for trade $ I implore the OIE decision and policy makers, for the sake of the world, to refuse any status quo of the USA BSE risk assessment. if at al, the USA BSE GBR should be raise to BSE GBR IV, for the following reasons. North America is awash with many different TSE Prion strains, in many different species, and they are mutating and spreading. IF the OIE, and whatever policy makers, do anything but raise the risk factor for BSE in North America, they I would regard that to be highly suspicious. IN fact, it would be criminal in my opinion, because the OIE knows this, and to knowingly expose the rest of the world to this dangerous pathogen, would be ‘knowingly’ and ‘willfully’, just for the almighty dollar, once again. I warned the OIE about all this, including the risk factors for CWD, and the fact that the zoonosis potential was great, way back in 2002. THE OIE in collaboration with the USDA, made the legal trading of the atypical Nor-98 Scrapie a legal global commodity. yes, thanks to the OIE and the USDA et al, it’s now legal to trade the atypical Nor-98 Scrapie strain all around the globe. IF you let them, they will do the same thing with atypical BSE and CWD (both strains to date). This with science showing that indeed these TSE prion strains are transmissible. I strenuously urge the OIE et al to refuse any weakening to the USA trade protocols for the BSE TSE prion disease (all strains), and urge them to reclassify the USA with BSE GBR IV risk factor.


SEE REFERENCE SOURCES IN ATTACHMENTS


SEE Terry S. Singeltary Sr. Attachment WORD FILE ;






Sunday, March 11, 2012


APHIS Proposes New Bovine Spongiform Encephalopathy Import Regulations in Line with International Animal Health Standards Proposal Aims to Ensure Health of the U.S. Beef Herd, Assist in Negotiations






Owens, Julie


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


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


To: FSIS RegulationsComments


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


Page 1 of 98




FSIS RFEPLY TO TSS ;


Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 INTRODUCTION The United States Department of Agriculture’s Food Safety and Inspection Service (FSIS) held a public meeting on July 25, 2006 in Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October 31, 2005 (report and model located on the FSIS website:




Comments on technical aspects of the risk assessment were then submitted to FSIS. Comments were received from Food and Water Watch, Food Animal Concerns Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S. Singeltary.


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






Wednesday, April 4, 2012


Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68




Friday, May 4, 2012


May 2, 2012: Update from APHIS Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States




Wednesday, July 28, 2010


Atypical prion proteins and IBNC in cattle DEFRA project code SE1796 FOIA Final report




IN CONFIDENCE


BSE ATYPICAL LESION DISTRIBUTION




Tuesday, November 02, 2010


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




Wednesday, February 16, 2011


IN CONFIDENCE


SCRAPIE TRANSMISSION TO CHIMPANZEES


IN CONFIDENCE




Sunday, April 18, 2010


SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010




Monday, April 25, 2011


Experimental Oral Transmission of Atypical Scrapie to Sheep


Volume 17, Number 5-May 2011




Monday, November 30, 2009


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




I strenuously urge the USDA and the OIE et al to revoke the exemption of the legal global trading of atypical Nor-98 scrapie TSE. ...TSS


Friday, February 11, 2011


Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues




Wednesday, January 18, 2012


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


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




Thursday, November 18, 2010


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




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




Saturday, June 19, 2010


U.S. DENIED UPGRADED BSE STATUS FROM OIE




Friday, August 20, 2010


USDA: Animal Disease Traceability August 2010




Friday, November 18, 2011


country-of-origin labeling law (COOL) violates U.S. obligations under WTO rules WT/DS384/R WT/DS386/R






FARM TO FORK TRACEABILITY, not in your lifetime $$$


Wednesday, May 25, 2011


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


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


From: Terry S. Singeltary Sr.


To: BSE-L@LISTS.AEGEE.ORG


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


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


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




Saturday, December 18, 2010


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




Monday, November 23, 2009


BSE GBR RISK ASSESSMENTS UPDATE NOVEMBER 23, 2009 COMMISSION OF THE EUROPEAN COMMUNITIES AND O.I.E. COMMISSION DECISION of 11 November 2009


amending the Annex to Decision 2007/453/EC as regards the BSE status of Chile, Colombia and Japan (notified under document C(2009) 8590)






Greetings USDA, OIE et al,


what a difference it makes with science, from one day to the next. i.e. that mad cow gold card the USA once held. up until that fateful day in December of 2003, the science of BSE was NO IMPORTS TO USA FROM BSE COUNTRY.


what a difference a day makes$ now that the shoe is on the other foot, the USDA via the OIE, wants to change science again, just for trade $


I implore the OIE decision and policy makers, for the sake of the world, to refuse any status quo of the USA BSE risk assessment. if at al, the USA BSE GBR should be raise to BSE GBR IV, for the following reasons.


North America is awash with many different TSE Prion strains, in many different species, and they are mutating and spreading.


IF the OIE, and whatever policy makers, do anything but raise the risk factor for BSE in North America, they I would regard that to be highly suspicious.


IN fact, it would be criminal in my opinion, because the OIE knows this, and to knowingly expose the rest of the world to this dangerous pathogen, would be ‘knowingly’ and ‘willfully’, just for the almighty dollar, once again.


I warned the OIE about all this, including the risk factors for CWD, and the fact that the zoonosis potential was great, way back in 2002.


THE OIE in collaboration with the USDA, made the legal trading of the atypical Nor-98 Scrapie a legal global commodity. yes, thanks to the OIE and the USDA et al, it’s now legal to trade the atypical Nor-98 Scrapie strain all around the globe. IF you let them, they will do the same thing with atypical BSE and CWD (both strains to date). This with science showing that indeed these TSE prion strains are transmissible.


I strenuously urge the OIE et al to refuse any weakening to the USA trade protocols for the BSE TSE prion disease (all strains), and urge them to reclassify the USA with BSE GBR IV risk factor.


SOURCE REFERENCES AS FOLLOWS ;


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




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




Friday, March 4, 2011


Alberta dairy cow found with mad cow disease




Saturday, June 19, 2010


U.S. DENIED UPGRADED BSE STATUS FROM OIE




Comment from Terry S Singletary Sr


Document ID: APHIS-2006-0041-0006 Document


Type: Public Submission This is comment on Proposed


Rule: Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Docket ID: APHIS-2006-0041








Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL


IMPORTS FROM CANADA




PLEASE SEE FULL TEXT HERE ;


Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA




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


Subject: US SENATOR AND STAN THE MAN SLAM USDA ''DAMNING TESTIMONY''


Date: Wed, 3 Mar 2004 15:15:24 –0600


From: "Terry S. Singeltary Sr." flounder@wt.net


Reply-To: Bovine Spongiform Encephalopathy BSE-L@uni-karlsruhe.de


To: BSE-L@uni-karlsruhe.de


######## Bovine Spongiform Encephalopathy #########


Greetings List members,


damning testimony below. be sure to _first_ open up real player competely, then paste your url in there. this worked best for me.........TSS


US SENATOR AND STAN THE MAN SLAM USDA ''DAMNING TESTIMONY''


Senator Michael Machado from California


''USDA does not know what's going on''.


''USDA is protecting the industry''.


''SHOULD the state of California step in''


Stanley Prusiner


''nobody has ever ask us to comment''


''they don't want us to comment''


''they never ask''


i tried to see Venemon, after Candian cow was discovered with BSE. went to see lyle. after talking with him...


absolute ignorance...


then thought I should see Venemon...


it was clear his entire policy was to get cattle bonless beef prods across the border...


nothing else mattered...


his aids confirmed this...


5 times i tried to see Venemon, never worked...


eventually met with carl rove the political...


he is the one that arranged meeting with Venemon...


just trying to give you a sense of the distance...


healh public safety...


was never contacted...


yes i believe that prions are bad to eat and you can die from them...


END


PLEASE NOTE THESE VIDEOS HAVE BEEN REMOVED FROM THE INTERNET $$$


Dr. Stan bashing Ann Veneman - 3 minutes




Recall Authority and Mad Cow Disease: Is the Current System Good for Californians?


Tuesday, February 24, 2004


JOINT HEARING


AGRICULTURE AND WATER RESOURCES HEALTH AND HUMAN SERVICES AND SELECT COMMITTEE ON GOVERNMENT OVERSIGHT - MACHADO, ORTIZ, and SPEIER, Chairs


Choose a RealPlayer video --->


Selected excerpts:


Opening Statement by Senator Michael Machado




Elisa Odibashian - Consumers Union




Anthony Iton - Alemeda County Health




USDA's "memorandum of understanding"




Dave Louthan - Killed the Mad Cow




Dennis Laycraft - Canadian Cattlemen's Association




Stanley Prusiner - Discoverer of Prions




Steven DeArmond - Professor of Neuropathology




Entire 5 hour hearing - The California Channel


(scroll down to "022404 Senate Info-Hearing")




PLEASE NOTE THIS HEARING IN CALIFORNIA, THE VIDEOS HAVE BEEN REMOVED FROM THE INTERNET IN BOTH THE OFFICIAL, AND UNOFFICIAL WEBSITES I.E. MADDEER.ORG AND CALIFORNIA SENATE SITE WHERE THEY ONCE WERE...TSS


Sunday, November 13, 2011


California BSE mad cow beef recall, QFC, CJD, and dead stock downer livestock




Thursday, January 5, 2012


Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93


Subject: Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION




Saturday, April 10, 2010


TOYOTA VS MAD COW DISEASE USA OIE BSE MRR IMPORT AND EXPORT TRADE WARS






Comments »




Terry S. Singeltary Sr. wrote on Jun 24, 2010 9:57 AM:


" Saturday, June 19, 2010


U.S. DENIED UPGRADED BSE STATUS FROM OIE


Greetings,


IF the truth were known (and it's not like I have not been trying), the USA, Canada, and Mexico (there are other Countries too), should all be listed in this new TSE prion trader friendly atmosphere as ''undetermined risk''. Because USDA et al have absolutely no idea. The ideology of only the UK BSE theory and there from only imported MBM and feed, to ignore the fact that the continuous rendering technology was developed and the USDA got the UK to use it first, some five years before the USA started using the same technology, and then the fact of all the different TSE in different species here in North America, and different strains there from, to continue to believe in only the imported factor of feed and animals, and not take seriously the _home grown_ factor, from tainted _home grown TSE tainted feed_, from the same type rendering technology, is like sticking your head in a hole in the ground and hoping for the best. kind of like what BP did in the Gulf of Mexico. But for the OIE to continue to go by this decades old science on BSE, and continue to ignore the risk factors from other strains of BSE, and other TSE in other species, when scientist from around the globe continue to wave flags of concern, to continue this ignorance is dangerous for human and animal health. But typical for the OIE and the USDA in reference to the Transmissible Spongiform Encephalopathy disease. Both the USDA and the OIE have ignored these documented risk factors for years, even decades, simply for trading purposes. The USDA et al until 2003 when the first documented case of c-BSE was documented in Washington State, the USDA had nothing to do with countries that had BSE. Until that cow old Luther capped in Washington, then the shoe was on the other foot. The USDA and the OIE after that literally changed the rules and regulations on BSE that had been in place for almost a decade trying to eradicate it all around the globe before it mutated, by doing away with the BSE GBR risk assessments and ignoring them, and implementing the infamous force fed USDA BSE MRR policy (all this is explained below in the source reference). But two mad cows sat on ice while all this political science was taking place for 7 months. One finally confirmed thanks to the OIG and the Honorable Phyllis Fong, and the other could not be confirmed due to the fact in had been improperly stored for 4 MONTHS, before testing. Kind of like the other stumbling and staggering mad cow in Texas that got away, went straight to be rendered for pet food, without NO TSE prion test at all. I could go on, about the healthy brains, from healthy cows, cows they knew did not have BSE, submitted for the infamous 2004 Enhanced BSE surveillance and testing program, or the other 9,200 brains they only used IHC testing, the least likely to find BSE. Sadly, once they did start documenting BSE back to back, they shut it down, said that was enough, let's cancel this right here in it's tracks, and we have heard nothing since, like the USA has now become immune to any TSE in any bovine. ;




When the OIE and the USDA et al collaborated to make legal the trading of Transmissible Spongiform Encephalopathy, when they did away with the BSE GBR risk assessments, where the USA, Canada, and Mexico were categorized as BSE GBR III. please see ;




EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases.




snip...




Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission (EC) to provide an up-to-date scientific report on the GBR in the United States of America, i.e. the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, in USA. This scientific report addresses the GBR of USA as assessed in 2004 based on data covering the period 1980-2003. The BSE agent was probably imported into USA and could have reached domestic cattle in the middle of the eighties. These cattle imported in the mid eighties could have been rendered in the late eighties and therefore led to an internal challenge in the early nineties. It is possible that imported meat and bone meal (MBM) into the USA reached domestic cattle and leads to an internal challenge in the early nineties. A processing risk developed in the late 80s/early 90s when cattle imports from BSE risk countries were slaughtered or died and were processed (partly) into feed, together with some imports of MBM. This risk continued to exist, and grew significantly in the mid 90’s when domestic cattle, infected by imported MBM, reached processing. Given the low stability of the system, the risk increased over the years with continued imports of cattle and MBM from BSE risk countries.


EFSA concludes that the current GBR level of USA is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent. As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (pre-clinically or clinically) infected with the BSE-agent persistently increases. Key words: BSE, geographical risk assessment, GBR, USA, third countries












5. CONCLUSION ON THE GEOGRAPHICAL BSE-RISK


5.1 The current GBR as function of the past stability and challenge


• The current geographical BSE risk (GBR) level is III, i.e. it is likely but not confirmed that domestic cattle are (clinically or pre-clinically) infected with the BSE-agent.


Note1: It is also worth noting that the current GBR conclusions are not dependent on the large exchange of imports between USA and Canada. External challenge due to exports to the USA from European countries varied from moderate to high. These Annex to the EFSA Scientific Report (2004) 3, 1-17 on the Assessment of the Geographical BSE Risk of USA


- 16 -


challenges indicate that it was likely that BSE infectivity was introduced into the North American continent.


Note2: This assessment deviates from the previous assessment (SSC opinion, 2000) because at that time several exporting countries were not considered a potential risk. 5.2 The expected development of the GBR as a function of the past and present stability and challenge


• As long as there are no significant changes in rendering or feeding, the stability remains extremely/very unstable. Thus, the probability of cattle to be (preclinically or clinically) infected with the BSE-agent persistently increases.


• Since recent improvements in the safety of MBM production in many countries or significant recent reductions in the incidence of BSE are not taken into account for the assessment of the external challenge, the external challenge assessed after 2001 could be overestimated and is the worst case assumption. However all current GBR conclusions are not dependent on these assumptions in any of the countries assessed. For future assessments and when the impact of the production, surveillance and true incidence changes have been fully quantified, these developments should be taken into account.






Annex to the EFSA Scientific Report (2004) 3, 1-17 on the Assessment of the Geographical BSE Risk of USA


please see full text ;








YET, in 2010, tons and tons of banned mad cow protein are still in commerce here in the USA, scientific studies are being misconstrued and manipulated by ARS USDA, which are still going by TSE science that is decades old, while refusing to acknowledge new scientific studies, and FOIA requests are still being held up by the USDA et al on these urgent matters (see source related materials below). CJD of unknown phenotype, in victims that are getting younger, with longer clinical course from first onset of symptoms to death are occurring, in fact, sporadic CJD is still rising, where the TSEs in the different species are mutating here in the USA, and we still have this same dog and pony show by the OIE and USDA et al. IF you go back and look at the Countries that went by these OIE BSE guidelines, most all came down with BSE. I have said it before, I was say it again now, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization. ...TSS




see full text and reasons why here ;













Geographical BSE risk assessment and its impact on disease detection and dissemination


Original Research Article


Preventive Veterinary Medicine, Available online 1 February 2012,


Mo Salman, Vittorio Silano, Dagmar Heim, Joachim Kreysa


Preventive Veterinary Medicine


1 February 2012


Geographical BSE risk assessment and its impact on disease detection and dissemination


Salman M, Silano V, Heim D, Kreysa J.


Source


Campus Stop 1644, Animal Population Health Institute, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO 80523-1644, USA.


Abstract


Bovine Spongiform Encephalopathy (BSE) rapidly evolved into an issue of major public concern particularly when, in 1996, evidence was provided that this disease had crossed the species barrier and infected humans in the UK with what has become known as "variant Creutzfeldt Jakob Disease" (vCJD). The aim of this paper is to describe the European Geographical BSE risk assessment (GBR) that was successfully used for assessing the qualitative likelihood that BSE could be present in a country where it was not yet officially recognized. It also discusses how this can lead to risk-based and therefore preventive management of BSE at national and international levels. The basic assumption of the GBR method is that the BSE agent is initially introduced into a country's domestic cattle production system through the importation of contaminated feedstuffs or live cattle. This is referred to as an "external challenge". The ability of the system to cope with such a challenge is, in turn, referred to as its "stability": a stable system will not allow the BSE agent to propagate and amplify following its introduction, while an unstable system will. The BSE-status of a country assessed by this system was used by the European Commission as the basis for trade legislation rules for cattle and their products. The GBR was an invaluable tool in evaluating the potential global spread of BSE as it demonstrated how a disease could be transferred through international trade. This was shown to be a critical factor to address in reducing the spread and amplification of BSE throughout the world. Furthermore, GBR resulted in the implementation of additional measures and management activities both to improve surveillance and to prevent transmission within the cattle population.


Copyright © 2012 Elsevier B.V. All rights reserved.




see more here ;




IN SHORT, AND IN A NUT SHELL ;


(Adopted by the International Committee of the OIE on 23 May 2006)


11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries. The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to the Central Bureau,






layperson


Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder9@verizon.net





Comment from Terry S Singletary Document ID: APHIS-2006-0026-0012 Document Type: Public Submission This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Minimal-Risk Regions, Identification of Ruminants and Processing and Importation of Commodities Docket ID: APHIS-2006-0026 RIN:Not Assigned


Topics: No Topics associated with this document View Document: More Document Subtype: Public Comment Status: Posted Received Date: November 22 2006, at 04:07 PM Eastern Standard Time Date Posted: November 28 2006, at 12:00 AM Eastern Standard Time Comment Start Date: August 09 2006, at 07:46 AM Eastern Daylight Time Comment Due Date: November 24 2006, at 11:59 PM Eastern Standard Time Tracking Number: 801e47e1 First Name: Terry Middle Name: S Last Name: Singeltary City: Bacliff Country: United States State or Province: TX Organization Name: CJD Submitter's Representative: NA


Comment:


I IMPLORE, that due to the lack of surveillance and proper BSE/TSE protocols for testing and surveillance, the fact that sporadic CJD in the USA has tripled over the last few years or so, the new findings of BASE, the fact that CWD and Scrapie are out of control in the USA, I IMPLORE that the BSE MRR policy be repealed and the BSE GBR risk assessments revised to address all TSEs i.e. TSE GBR, for the following reasons ;







PUBLIC SUBMISSION As of: 5/5/12 5:34 PM Tracking No. 801e47e1 Comments Due: November 24, 2006


Docket: APHIS-2006-0026 Bovine Spongiform Encephalopathy; Animal Identification and Importation of Commodities


Comment On: APHIS-2006-0026-0001 Bovine Spongiform Encephalopathy; Minimal-Risk Regions, Identification of Ruminants and Processing and Importation of Commodities


Document: APHIS-2006-0026-0012 Comment from Terry S Singletary


--------------------------------------------------------------------------------


Submitter Information Name: Terry S Singeltary Address: Bacliff, TX,


Submitter's Representative: NA Organization: CJD


--------------------------------------------------------------------------------


General Comment






besides the other tons and tons of recall of potential BSE/BASE/TSE feed in the USA in 2006;



















> the need not to overstate risk in countries with a low BSE prevalence but large cattle


> populations;




what about understating the risk???




PERSPECTIVE


On the Question of Sporadic


or Atypical Bovine SpongiformEncephalopathy and


Creutzfeldt-Jakob Disease


Paul Brown,* Lisa M. McShane,? Gianluigi Zanusso,? and Linda Detwiler?


Strategies to investigate the possible existence of sporadic


bovine spongiform encephalopathy (BSE) require


systematic testing programs to identify cases in countries


considered to have little or no risk for orally acquired disease,


or to detect a stable occurrence of atypical cases in


countries in which orally acquired disease is disappearing.


To achieve 95% statistical confidence that the prevalence


of sporadic BSE is no greater than 1 per million (i.e., the


annual incidence of sporadic Creutzfeldt-Jakob disease


[CJD] in humans) would require negative tests in 3 million


randomly selected older cattle. A link between BSE and


sporadic CJD has been suggested on the basis of laboratory


studies but is unsupported by epidemiologic observation.


Such a link might yet be established by the discovery


of a specific molecular marker or of particular combinations


of trends over time of typical and atypical BSE and various


subtypes of sporadic CJD, as their numbers are influenced


by a continuation of current public health measures that


exclude high-risk bovine tissues from the animal and


human food chains. ......



PLEASE READ FULL TEXT ;






3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit Hall


3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain discovered recently in Italy, and similar or different atypical BSE cases were also reported in other countries. The infectivity and phenotypes of these atypical BSE strains in humans are unknown. In collaboration with Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have inoculated transgenic mice expressing human prion protein with brain homogenates from BASE or BSE infected cattle. Our data shows that about half of the BASE-inoculated mice became infected with an average incubation time of about 19 months; in contrast, none of the BSE-inoculated mice appear to be infected after more than 2 years. These results indicate that BASE is transmissible to humans and suggest that BASE is more virulent than classical BSE in humans.


6:30 Close of Day One






SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype of 'UNKNOWN' strain growing. ...






There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.


He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.










[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle






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






THE SEVEN SCIENTIST REPORT ***






PAUL BROWN M.D.






9 December 2005 Division of Dockets Management (RFA-305)


SEROLOGICALS CORPORATION James J. Kramer, Ph.D. Vice President, Corporate Operations






Embassy of Japan






Dockets Entered on December 22, 2005 2005D-0330, Guidance for Industry and FDA Review Staff on Collection of Platelets by Automated ... EC 203, McDonald's Restaurants Corporation, Vol #:, 34 ...






03-025IF 03-025IF-631 Linda A. Detwiler [PDF] Page 1. 03-025IF 03-025IF-631 Linda A. Detwiler Page 2. Page 3. Page 4. Page 5. Page 6. Page 7. Page 8. Page 9. Page 10. Page 11. Page 12.






03-025IF 03-025IF-634 Linda A. Detwiler [PDF] Page 1. 03-025IF 03-025IF-634 Linda A. Detwiler Page 2. Page 3. Page 4. Page 5. Page 6. Page 7. Page 8.






Page 1 of 17 9/13/2005 [PDF] ... 2005 6:17 PM To: fsis.regulationscomments@fsis.usda.gov Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food ...






03-025IFA 03-025IFA-6 Jason Frost [PDF] ... Zealand Embassy COMMENTS ON FEDERAL REGISTER 9 CFR Parts 309 et al [Docket No. 03- 025IF] Prohibition of the Use of Specified Risk Materials for Human Food and ...






In its opinion of 7-8 December 2000 (EC 2000), the SSC ... [PDF] Page 1. Linda A. Detwiler, DVM 225 Hwy 35 Red Bank, New Jersey 07701 Phone: 732-741-2290 Cell: 732-580-9391 Fax: 732-741-7751 June 22, 2005 FSIS Docket Clerk US ...







your only fooling yourselves with this stupid ukbsenvcjd only theory, and the BSE methology of the OIE. most any coutnry that went by those same OIE BSE guidelines all went down with BSE.




THE OIE has now shown they are nothing more than a National Trading Brokerage for all strains of animal TSE.




AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization. ...









Terry S. Singeltary SR. P.O. Box 42 Bacliff, Texas USA 77518




C:\Users\flounder\Downloads\APHIS-2006-0026-0012 (2).html




Comment from Terry S Singletary Document ID: APHIS-2006-0041-0028 Document Type: Public Submission This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Minimal-Risk Regions; Importation of Live Bovines and Products Derived From Bovines Docket ID: APHIS-2006-0041 RIN:0579-AC01




Topics: No Topics associated with this document View Document: More Document Subtype: Public Comment Status: Posted Received Date: January 28 2007, at 10:02 PM Eastern Standard Time Date Posted: January 29 2007, at 12:00 AM Eastern Standard Time Comment Start Date: January 09 2007, at 12:00 AM Eastern Standard Time Comment Due Date: March 12 2007, at 11:59 PM Eastern Daylight Time Tracking Number: 801f8151 First Name: Terry Middle Name: S Last Name: Singeltary City: Bacliff Country: United States State or Province: TX




Comment: 2006-2007 USA AND OIE POISONING GLOBE WITH BSE MRR POLICY




THE USA is in a most unique situation, one of unknown circumstances with human and animal TSE. THE USA has the most documented TSE in different species to date, with substrains growing in those species (BSE/BASE in cattle and CWD in deer and elk, there is evidence here with different strains), and we know that sheep scrapie has over 20 strains of the typical scrapie with atypical scrapie documented and also BSE is very likely to have passed to sheep. all of which have been rendered and fed back to animals for human and animal consumption, a frightening scenario. WE do not know the outcome, and to play with human life around the globe with the very likely TSE tainted products from the USA, in my opinion is like playing Russian roulette, of long duration, with potential long and enduring consequences, of which once done, cannot be undone. These are the facts as I have come to know through daily and extensive research of TSE over 9 years, since 12/14/97.




I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species.




MY personal belief, since you ask, is that not only the Canadian border, but the USA border, and the Mexican border should be sealed up tighter than a drum for exporting there TSE tainted products, until a validated, 100% sensitive test is available, and all animals for human and animal consumption are tested. all we are doing is the exact same thing the UK did with there mad cow poisoning when they exported it all over the globe, all the while knowing what they were doing. this BSE MRR policy is nothing more than a legal tool to do just exactly what the UK did, thanks to the OIE and GW, it's legal now. and they executed Saddam for poisoning ???




go figure. ...






Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518











Comment from Terry S Singletary Document ID: APHIS-2006-0026-0012 Document Type: Public Submission This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Minimal-Risk Regions, Identification of Ruminants and Processing and Importation of Commodities Docket ID: APHIS-2006-0026 RIN:Not Assigned




Topics: No Topics associated with this document View Document: Less Document Subtype: Public Comment Status: Posted Received Date: November 22 2006, at 04:07 PM Eastern Standard Time Date Posted: November 28 2006, at 12:00 AM Eastern Standard Time Comment Start Date: August 09 2006, at 07:46 AM Eastern Daylight Time Comment Due Date: November 24 2006, at 11:59 PM Eastern Standard Time Tracking Number: 801e47e1 First Name: Terry Middle Name: S Last Name: Singeltary City: Bacliff Country: United States State or Province: TX Organization Name: CJD Submitter's Representative: NA









SADLY, it seems that the OIE is more worried only about the animal health issues that only relates to trade and the almighty dollar, instead of animal disease that relates to human health factors.




also, in relations to human health and the TSE prion diseases. the OIE will be responsible for refusing to address all TSE disease, and floundering too long, when science and politics allow the many different sporadic CJD strains to animal disease. there is as much science today showing the atypical BSE is related to some strains of sporadic CJD, as there was when Ironside presented his first 10 nvCJD victims. however the OIE refuses to act. CWD ? only time will tell, but the OIE floundered as well there. Scrapie, same there, scrapie transmits to primate. nor-98 atypical scrapie, science mounting there too that the nor-98 is tied to sporadic CJD cases, and what did the OIE DO. COLLABORATE WITH THE USDA ET AL to make the Nor-98 atypical scrapie disease exempt from regulations, and made it LEGAL to trade the Nor-98 atypical scrapie disease a tradable disease $$$ the OIE, USDA et al chose to expose humans and animals to the prion TSE agent around the globe, thus, you will expose not only humans and animals, but the environment as well. ...






HOW ABOUT THAT TEXAS MAD COW THAT WAS COVERED UP FOR 7 MONTHS...




BIO-RAD BSE TEST POLITICAL REPLY TO TSS


Subject: FSIS NOTICE SAMPLE COLLECTION FROM CATTLE UNDER THE BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) ONGOING SURVEILLANCE PROGRAM From: "Terry S. Singeltary Sr." Reply-To: Sustainable Agriculture Network Discussion Group Date: Fri, 2 Feb 2007 17:32:58 -0600


Subject: Re: USDA/APHIS JUNE 2004 'ENHANCED' BSE/TSE COVER UP UPDATE DECEMBER 19, 2004 USA Date: Thu, 30 Dec 2004 12:27:06 -0600 From: "Terry S. Singeltary Sr.


BSE-L


snip...


> > OH, i did ask Bio-Rad about this with NO reply to date; > > > -------- Original Message -------- > Subject: USA BIO-RADs INCONCLUSIVEs > Date: Fri, 17 Dec 2004 15:37:28 -0600 > From: "Terry S. Singeltary Sr." > To: susan_berg@bio-rad.com > > > > Hello Susan and Bio-Rad, > > Happy Holidays! > > I wish to ask a question about Bio-Rad and USDA BSE/TSE testing > and there inconclusive. IS the Bio-Rad test for BSE/TSE that complicated, > or is there most likely some human error we are seeing here? > > HOW can Japan have 2 positive cows with > No clinical signs WB+, IHC-, HP- , > BUT in the USA, these cows are considered 'negative'? > > IS there more politics working here than science in the USA? > > What am I missing? > > > > -------- Original Message -------- > Subject: Re: USDA: More mad cow testing will demonstrate beef's safety > Date: Fri, 17 Dec 2004 09:26:19 -0600 > From: "Terry S. Singeltary Sr." > snip...end > > > Experts doubt USDA's mad cow results


snip...END


WELL, someone did call me from Bio-Rad about this, however it was not Susan Berg. but i had to just about take a blood oath not to reveal there name. IN fact they did not want me to even mention this, but i feel it is much much to important. I have omitted any I.D. of this person, but thought I must document this ;


Bio-Rad, TSS phone conversation 12/28/04


Finally spoke with ;


Bio-Rad Laboratories 2000 Alfred Nobel Drive Hercules, CA 94547 Ph: 510-741-6720 Fax: 510-741-5630 Email: XXXXXXXXXXXXXXXXXX


at approx. 14:00 hours 12/28/04, I had a very pleasant phone conversation with XXXX XXXXX about the USDA and the inconclusive BSE testing problems they seem to keep having. X was very very cautious as to speak directly about USDA and it's policy of not using WB. X was very concerned as a Bio-Rad official of retaliation of some sort. X would only speak of what other countries do, and that i should take that as an answer. I told X I understood that it was a very loaded question and X agreed several times over and even said a political one.


my question;


Does Bio-Rad believe USDA's final determination of False positive, without WB, and considering the new atypical TSEs not showing positive with -IHC and -HP ???


ask if i was a reporter. i said no, i was with CJD Watch and that i had lost my mother to hvCJD. X did not want any of this recorded or repeated.


again, very nervous, will not answer directly about USDA for fear of retaliation, but again said X tell me what other countries are doing and finding, and that i should take it from there. "very difficult to answer"


"very political"


"very loaded question"


outside USA and Canada, they use many different confirmatory tech. in house WB, SAF, along with IHC, HP, several times etc. you should see at several talks meetings (TSE) of late Paris Dec 2, that IHC- DOES NOT MEAN IT IS NEGATIVE. again, look what the rest of the world is doing. said something about Dr. Houston stating; any screening assay, always a chance for human error. but with so many errors (i am assuming X meant inconclusive), why are there no investigations, just false positives? said something about ''just look at the sheep that tested IHC- but were positive''. ...


TSS





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


Subject: Your questions


Date: Mon, 27 Dec 2004 15:58:11 –0800


From: To: flounder@wt.net


Hi Terry:


............................................


snip


Let me know your phone number so I can talk to you about the Bio-Rad BSE test. Thank you


Regards


Bio-Rad Laboratories 2000 Alfred Nobel Drive Hercules, CA 94547 Ph: 510-741-6720 Fax: 510-741-5630 Email:


=================================


snip...end...TSS




TSS REPORT ON 2ND TEJAS MAD COW


Mon, 22 Nov 2004 17:12:15 -0600 (the one that did NOT get away, thanks to the Honorable Phyllis Fong)




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


Subject: Re: BSE 'INCONCLUSIVE' COW from TEXAS ??? Date: Mon, 22 Nov 2004 17:12:15 -0600 From: "Terry S. Singeltary Sr." To: Carla Everett References: <[log in to unmask]> <[log in to unmask] us>


Greetings Carla,still hear a rumor;


Texas single beef cow not born in Canada no beef entered the food chain?


and i see the TEXAS department of animal health is ramping up forsomething, but they forgot a url for update?I HAVE NO ACTUAL CONFIRMATION YET...can you confirm???


terry




==============================




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


Subject: Re: BSE 'INCONCLUSIVE' COW from TEXAS ???


Date: Fri, 19 Nov 2004 11:38:21 -0600 From: Carla Everett


To: "Terry S. Singeltary Sr." References: <[log in to unmask]>


The USDA has made a statement, and we are referring all callers to the USDA web site. We have no informationabout the animal being in Texas. CarlaAt 09:44 AM 11/19/2004, you wrote:>Greetings Carla,>>i am getting unsubstantiated claims of this BSE 'inconclusive' cow is from>TEXAS. can you comment on this either way please?>>thank you,>Terry S. Singeltary Sr.>>


===================




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


Subject: Re: BSE 'INCONCLUSIVE' COW from TEXAS ???


Date: Mon, 22 Nov 2004 18:33:20 –0600


From: Carla Everett


To: "Terry S. Singeltary Sr." References: <[log in to unmask]> <[log in to unmask] us> <[log in to unmask]> <[log in to unmask] us> <[log in to unmask]>




our computer department was working on a place holder we could postUSDA's announcement of any results. There are no results to be announced tonightby NVSL, so we are back in a waiting mode and will post the USDA announcementwhen we hear something.



At 06:05 PM 11/22/2004, you wrote:


>why was the announcement on your TAHC site removed?


>>Bovine Spongiform Encephalopathy:>November 22: Press Release title here


>>star image More BSE information


>>>>terry


>>Carla Everett wrote:


>>>no confirmation on the U.S.' inconclusive test...


>>no confirmation on location of animal.


>>>>>>


==========================




THEN, 7+ MONTHS OF COVER-UP BY JOHANN ET AL! no doubt about it now $$$




NO, it's not pretty, hell, im not pretty, but these are the facts, take em or leave em, however, you cannot change them.





with kindest regards,


I am still sincerely disgusted and tired in sunny Bacliff, Texas USA 77518


Terry S. Singeltary Sr.





FULL 130 LASHINGS TO USDA BY OIG again







tss

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