VARIANT CJD (vCJD) or nvCJD

New Variant Creutzfeldt Jakob Disease nvCJD, was linked to young people and BSE in the U.K., aka mad cow disease...

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Location: BACLIFF, Texas, United States

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Monday, January 30, 2012

The First Report of a Patient with Probable Variant Creutzfeldt-Jakob Disease in Turkey

Dement Geriatr Cogn Dis Extra. 2011 Jan-Dec; 1(1): 429–432. Published online 2011 December 24. doi: 10.1159/000332024 PMCID: PMC3265806



Copyright© 2011 by S. Karger AG, Basel



The First Report of a Patient with Probable Variant Creutzfeldt-Jakob Disease in Turkey



Demet Özbabalık Adapınar,a* Suzan Saylısoy,b Çınar Yenilmez,c Hüseyin Aslan,d Bengü Ertan,a Sevilhan Artan,d Gülcan Güleç,c Çiğdem Susuz,a and Baki Adapınarb


aDepartment of Neurology, Eskisehir Osmangazi University Medical Faculty, Eskisehir, Turkey


bDepartment of Radiology, Eskisehir Osmangazi University Medical Faculty, Eskisehir, Turkey


cDepartment of Psychiatry, Eskisehir Osmangazi University Medical Faculty, Eskisehir, Turkey


dDepartment of Medical Genetics, Eskisehir Osmangazi University Medical Faculty, Eskisehir, Turkey


*Demet Özbabalık Adapınar, Eskisehir Osmangazi University, TR–26480 Meselik/Eskisehir (Turkey), E-Mail demetg@ogu.edu.tr



This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License (http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com/ and the terms of this license are included in any shared versions.



Abstract



Variant Creutzfeldt-Jakob disease (vCJD) was first reported in the UK in 1996. Here, we report the first Turkish case of vCJD. A 47-year-old man, who has never lived outside of Turkey and had had no transfusion, was admitted to the University Hospital with speech disorder, cognitive decline and ataxia following depression, irritability, and personality change. The immunoassay of the 14-3-3 protein in the cerebrospinal fluid was negative. Brain magnetic resonance imaging revealed high-signal lesions involving the bilateral caudate and lentiform nucleus on T2- and diffusion-weighted imaging. The patient developed akinetic mutism 10 months after disease onset. The clinical presentation and neuroimaging findings were compatible with the vCJD cases reported since 1996 and met the World Health Organization's case definition for probable vCJD.Key Words: Magnetic resonance imaging, Prion, Variant Creutzfeldt-Jakob disease



Other Sections▼ Abstract Introduction Case Report Discussion References



Introduction



Creutzfeldt-Jakob disease (CJD), which is characterized by progressive dementia with a fatal and incurable course, is the most common human prion disease. There are 4 types of this disease: the sporadic (85%), familial (10–15%), iatrogenic (1%), and variant types [1]. Variant Creutzfeldt-Jakob disease (vCJD) was first reported in 1996 in the United Kingdom and has been causally linked to the consumption of cattle products contaminated with the bovine spongiform encephalopathy (BSE) agent [2]. To date, more than 215 cases of vCJD have been identified worldwide, including in the UK, France, Ireland, Italy, the USA, Canada, Saudi Arabia, Japan, The Netherlands, Portugal, Spain, and Taiwan. In the present paper, we report the clinical and radiological data of the first Turkish case of vCJD.



Other Sections▼ Abstract Introduction Case Report Discussion References



Case Report



A 47-year-old man, who was previously healthy and had no history of psychiatric or neurological disorders before the onset of this disease, presented with a 6-month history of progressive behavioral and personality changes, depression, and cognitive decline. His relatives reported that personality changes were his first symptoms. He had been a rigorous, disciplined, and frugal person, who was dependable at work and a valued member of his family; however, he became aggressive, extravagant, foul-mouthed, sexually disinhibited, and angry. His wife reported that the patient would have sudden outbursts of agitation, and 2 months later, these outbursts were followed by paranoid behaviors and possessiveness. Due to the psychiatric nature of his complaints, he was admitted to a psychiatry clinic, where he was diagnosed as having a manic disorder and was given atypical antipsychotic drugs. Three months later, the patient exhibited gait changes, ataxia and dysarthria, severe forgetfulness, difficulties in swallowing and eating, and incontinence. The patient developed involuntary movements in both of his feet, with dystonic aversion-inversion posturing and occasional erratic movements. He became dependent and apathetic and exhibited regressive behaviors. The patient also exhibited visual hallucinations, during which he reported seeing animals. Some of these complaints may have been side effects from his medication and, consequently, several of the patient's medications were stopped or changed.



As a result of the progressive deterioration of the patient's general status, his relatives transferred him to the Psychiatry Department of our University Hospital. After the neurological examination conducted at the Psychiatry Clinic, he was diagnosed as having rapid progressive dementia with early onset and was hospitalized at the Neurology Clinic. As reported in the patient's medical history, he had never been exposed to cadaveric pituitary hormones, had never undergone a neurosurgical procedure, organ or tissue grafts, or a blood transfusion, and had never travelled to the UK or to any country with reported incidences of BSE.



The neurological examination revealed that the patient was disorientated in place and time. In addition, he was mute. Nystagmus and conjugate gaze dysfunction were present, as were cerebellar ataxia, dysmetria, and dysdiadochokinesia. The patient's tone was slightly increased in his lower limbs, and his plantar responses were extensor.



The level of protein in his cerebrospinal fluid was increased, and no 14-3-3 protein was detected. An electroencephalogram showed a generalized slowing of wave, which was more evident in the left hemisphere, but did not have any periodic complexes. He was referred to the Radiology Department for cerebral MRI. On T2-weighted images and fluid-attenuated inversion recovery (FLAIR) images, hyperintense signal changes in the bilateral caudate nuclei (white arrows) and the lentiform nucleus (black arrows) were seen. In addition, hyperintensity in the bilateral thalamic region was less prominent than in the previously described areas (fig. 1a–c). Cortical hyperintensity was noted on diffusion-weighted imaging.



Fig. 1 MRI shows hyperintensity in the bilateral caudate (white arrows) and bilateral lentiform nucleus (black arrows) as well as in the bilateral thalamus (curved arrow) on axial T2-weighted image (a) and coronal FLAIR image (b). Ribbon-shaped hyperintense (more ...)



Genotyping of the prion protein gene (PRNP) identified a P102L mutation and heterozygosity for methionine at codon 129.The patient did not want to undergo a brain biopsy, and we continued to follow his progress at his home. At the time this report was written, the patient was alive, mute, and on bed rest. Other Sections▼ Abstract Introduction Case Report Discussion References



Discussion



The patient described in the present report was the first probable case of vCJD in Turkey. The clinical features of this patient are consistent with the vCJD cases that have been identified in the UK and France, including psychiatric manifestations at the disease onset, a delayed occurrence of neurological signs, ataxia, and dementia [3]. In addition to the pulvinar sign that was present on the MRI and EEG, the patient fulfilled the WHO case definition for probable vCJD, the specificity of which is 100% [4]. Of note, a tonsil biopsy is not necessary if the clinical features and the MRI findings are compatible with vCJD, as the pulvinar sign is highly characteristic [5].



Sequencing analysis revealed that the patient had two different nucleotide changes in the coding region of the PRNP gene. The first one is the M129V polymorphism, and our patient was heterozygous for this alteration. Interestingly, all the patients who have undergone genotyping up to now have been homozygous for methionine at codon 129. This polymorphism is associated with susceptibility to prion diseases [6]. The second one is the P102L mutation, which was first identified in affected members of two unrelated families with Gerstmann-Sträussler disease. P102L is one of the most common PRNP mutations and also related to CJD.



This case highlights the difficulties in achieving an early diagnosis of vCJD. At the initial presentation, a variety of diagnoses were proposed, but vCJD was not considered in this patient. Instead, he was diagnosed as having an affective disorder at the psychiatry clinic. The delayed neurological signs in this patient pointed to the possibility of progressive dementia, which is not surprising given the frequency of psychiatric features that are observed by primary care physicians. These clinical features are often misleading. Almost half of the cases of vCJD were reviewed by a psychiatrist prior to the patients’ neurological referral [7]. A neurological etiology was usually suspected promptly after the patients developed objective neurological features, which resulted in a neurological referral in all of the cases. The single most important determinant of early diagnosis was the presence of objective neurological features. For this reason, all physicians must be careful when diagnosing rapidly progressing dementia that begins at a young age.



The results presented in this report indicate that patients with vCJD are still seen in the medical community. In the present study, we have reported the first Turkish vCJD case, which appeared 15 years after the first case in the UK.



Other Sections▼ Abstract Introduction Case Report Discussion References



References



1. Johnson RT. Prion diseases. Lancet Neurol. 2005;4:635–642.[PubMed]



2. Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A, Poser S, Pocchiari M, Hofman A, Smith PG. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet. 1996;347:92.[PubMed]



3. Brandel JP, Heath CA, Head MW, Levavasseur E, Knight R, Laplanche JL, Langeveld JP, Ironside JW, Hauw JJ, Mackenzie J, Alpérovitch A, Will RG, Haïk S. Variant CJD in France and the United Kingdom: evidence for the same agent strain. Ann Neurol. 2009;65:233–235. [PMC free article][PubMed]



4. World Health Organization: The revision of the surveillance case definition for variant Creutzfeldt-Jakob disease (vCJD). WHO/CDS/CSR/EPH/2001.5, 2002. Report of a WHO consultation.



5. Collie DA, Summers DM, Sellar RJ, Ironside JW, Cooper S, Zeidler M, Knight R, Will RG. Diagnosing variant Creutzfeldt-Jakob disease with the pulvinar sign: MR imaging findings in 86 neuropathologically confirmed cases. Am J Neuroradiol. 2003;24:1560–1569.[PubMed]



6. Lukic A, Beck J, Joiner S, Fearnley J, Sturman S, Brandner S, Wadsworth JD, Collinge J, Mead S. Heterozygosity at polymorphic codon 219 in variant Creutzfeldt-Jakob disease. Arch Neurol. 2010;67:1021–1023.[PubMed]



7. Lépine J, Gastpar M, Mendlwicz J, Tylee A. Depression in the community: the first pan-European study DEPRES (Depression Research in European Society. Int Clin Psychopharmacol. 1997;12:19–29.[PubMed]



http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3265806/?tool=pubmed






European Journal of Human Genetics (2001) 2001 Nature Publishing Group




Distribution of the M129V polymorphism of the prion protein gene in a Turkish population suggests a high risk for Creutzfeldt-Jakob disease




Nihan Erginel-Unaltuna1, Katell Peoc'h2, Evrim Komurcu1, Tufan Tevfik Acuner3, Halim Issever4 and Jean-Louis Laplanche*,2 1Department of Genetics, Institute for Experimental Medical Research, Istanbul University, Istanbul, Turkey; 2Service de Biochimie et Biologie MoleÂculaire, Association Claude Bernard, HoÃpital LariboisieÁre, Paris, France; 33rd Neurology Clinic, Turkish Ministry of Health Bakirkoy Hospital for Psychiatric and Neurological Diseases, Istanbul, Turkey; 4Division of Biostatistics and Demography, Department of Public Health, Istanbul Medical School, Istanbul University, Istanbul, Turkey



A polymorphism (M129V) at codon 129 of the prion protein gene (PRNP) results in either a methionine residue (Met) or a valine residue (Val) and is known to determine susceptibility for the development of sporadic or acquired Creutzfeldt-Jakob disease (CJD). The distributions of M129V genotypes and alleles in various general populations have been reported and there are clear differences between Western Europeans and East Asians. We analysed the coding sequence of the PRNP gene in 100 healthy Turkish subjects to determine whether the distributions of the M129V genotypes and alleles or other PRNP gene variants in the Turkish population differ from those in other normal populations. Three known polymorphisms but no other gene variants were detected in the PRNP coding sequence of the Turkish individuals. Genotype frequencies at codon 129 were 57% Met/Met, 34% Met/Val and 9% Val/Val, with an allele frequency of 0.740 : 0.260 Met:Val. These distributions are considerably different from those reported for other normal populations residing in Western Europe and East Asia, except in Crete. The higher frequency of 129 Met-homozygotes in Turkey than in Western Europe suggests that the Turkish are at greater risk of developing CJD. European Journal of Human Genetics (2001) 9, 965 ± 968.



Keywords: Creutzfeldt-Jakob disease; prion; gene; PRNP; polymorphism; Turkey; population; genetic




snip...




Consequently, the distributions of the M129V genotypes and alleles in the Turkish population differ considerably from those reported for other normal populations residing in either Western Europe or East Asia, with the notable exception of Cretan natives. A recent report19 found that the high rate of PRNP 129Met homozygosity in Crete was associated with a local increase in the incidence of sporadic CJD. As homozygosity at PRNP codon 129 is a recognized risk factor for sporadic and acquired CJD in Caucasians5,21 and heterozygosity is protective,2 ± 4,21 the higher frequency of 129Met-homozygotes in Turkey than in Western Europe would also suggest that the Turkish are at increased risk of developing CJD.




see full report ;




http://www.nature.com/ejhg/journal/v9/n12/pdf/5200754a.pdf







Prp Gene Based Scrapie Susceptibility in Native Turkish Sheep: Do We Need To Introduce a Breeding Program to Select For Resistance to Scrapie in Turkey?




Emel OZKAN*, M. İhsan SOYSAL, Begüm UZUN, Ebru GOKALP, İnci TOGAN


Two studies on scrapie in Turkey (published)




http://www.rarebreedsinternational.org/turkey/Ozkan%208th%20RBI%20Global%20Conference.pdf






“ Although not commented on in the Report, it is intriguing that the sampled Turkish population has the second highest polymorphic frequency of 129V (0.48), which is close to the that of the Fore population (0.55), and even closer to the expected equilibrium frequency of 0.45 (1). Given that there are no records of cannibalism in Turkish history, and the impossibility of the existence of undetected cannibalism of the scale observed in the Fore, there must be a different explanation for this high frequency. Sheep and cow brains are delicacies in Turkey, and certain popular restaurants (kelle pa�a) specialize in sheep brains and heads. The brains of sheep, cattle, pigs, primates, and other mammals are esteemed dietary items by people in many parts of the world, including the Middle East; north and central Africa; some Caribbean islands; central, south, and southeast Asia; “






Prion diseases and a penchant for brains




CONTRIBUTORS: Author: Sekercioglu, Cagan H. (Stanford University) JOURNAL: Science, 305(5682), 342 - 343. YEAR: 2004 PUB TYPE: Journal Article SUBJECT(S): Prions, kuru, Fore, Turks, cannibalism, scrapie, mad-cow disease, kelle pa�a, kokore�, balancing selection, epidemics, bovine spongiform encephalopathy, BSE, Creutzfeldt-Jakob disease, vCJD, brain consumption, public health DISCIPLINE: Medicine HTTP: http://www.sciencemag.org/cgi/content/full/305/5682/342 LANGUAGE: English PUB ID: 103-405-161 (Last edited on 2004/07/20 18:41:32 GMT-6) SPONSOR(S):




ABSTRACT:




In their Report "Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics," S. Mead et al. provide an interesting and persuasive argument on how kuru transmitted by endocannibalism resulted in balancing selection at codon 129 of human prion protein gene in the Fore linguistic group of Papua New Guinea (25 Apr. 2003, p. 640). I am not convinced, however, that acquired prion disease causing the selective pressure results mainly from cannibalism in other populations around the world. Although not commented on in the Report, it is intriguing that the sampled Turkish population has the second highest polymorphic frequency of 129V (0.48), which is close to the that of the Fore population (0.55), and even closer to the expected equilibrium frequency of 0.45 (1). Given that there are no records of cannibalism in Turkish history, and the impossibility of the existence of undetected cannibalism of the scale observed in the Fore, there must be a different explanation for this high frequency. Sheep and cow brains are delicacies in Turkey, and certain popular restaurants (kelle pa�a) specialize in sheep brains and heads. The brains of sheep, cattle, pigs, primates, and other mammals are esteemed dietary items by people in many parts of the world, including the Middle East; north and central Africa; some Caribbean islands; central, south, and southeast Asia; Russia; Iceland; southern Europe; and North and Latin America. In Mead et al.'s Table 1, the samples from many of these groups also show relatively high M129V frequencies. In cases of bovine spongiform encephalopathy (BSE) and scrapie, prion diseases found in cattle and sheep, the prions are concentrated in the brain and other nervous tissues (2), and the high frequency of M129V in many groups can also be explained by regular exposure to prion diseases as a result of frequent consumption of animal brains. Even though Mead et al. mention the possibility of animal prion disease as an explanation for the observed pattern, they clearly favor cannibalism as the main cause, citing the evidence for prehistoric cannibalism in certain human populations. However, variant Creutzfeldt-Jakob disease (vCJD) has been transmitted from BSE-infected cattle to humans (3); the BSE agent can infect various animals (such as cattle, goats, pigs, and sheep) that are often consumed by people (4); the significant increase in vCJD incidence in Leicestershire, England, was a result of the contamination of cow meat with central nervous system tissue (2); the high incidence of CJD in Libyan Jews (5) and Slovakian herdsmen (6) correlates with a high preference for sheep brains; and in rural Kentucky, a fondness for squirrel brains suggests a similar connection (7). An important reason for the dietary preference for brain tissue is its high fat content. This can also be seen in wild chimpanzees feeding on monkeys (8), and it is likely to have been present in prehistoric human populations feeding on a diversity of animals. That 85% of human transmissible spongiform encephalopathies are sporadic and have no known etiologies (2); that many people regularly consume ruminant intestinal tissue, which also has a high concentration of BSE prions (9); and that other possible vectors of human prion diseases include rodents (10) and flies (11) necessitate further caution in assigning cannibalism as the predominant cause of balancing selection at the prion protein gene in human populations worldwide. Prion diseases in people, especially outside the developed world, are likely to be misdiagnosed and underrecorded. To the extent possible, consumption of mammal brains, intestines, and other highly infective tissue should be evaluated as a potential causal factor in any study of human prion diseases.





http://www.getcited.org/pub/103405161






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



Subject: SCRAPIE, BRAIN CONSUMPTION AND CJD



Date: Wed, 31 Mar 2004 10:01:26 –0600



From: "Terry S. Singeltary Sr."



Reply-To: Bovine Spongiform Encephalopathy



To: BSE-L@uni-karlsruhe.de



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




THE LIBYAN JEWISH FOCUS OF CREUTZFELDT-JAKOB DISEASE:



A SEARCH FOR THE MODE OF NATURAL TRANSMISSION1 Harvey Goldberg2, Milton Alter3, and Esther Kahana4 ABSTRACT In 1974, we reported chat Creutzfeldt-Jakob disease (CJD), a fatal slow virus disease of man, occurred at least thirty tines more commonly In Libyan Jewish immigrants to Israel than all other groups studied. The mode of natural transmission of CJD Is unknown. This report describes genetic and environ- mental factors Investigated In an effort to explain the focus of CJD among Libyan Jews. A total-of 14 Libyan and 11 non-Libyan cases of CJD were studied. Of these, three were diagnosed after completion of our original report and two In the original report did not participate in this study. Among the 14 Libyans with CJD diagnosed between 1963 and 1974 and available for this study, two definite and three possible familial cases were identi- fied. Parents were first cousins in three of 14 (21%) cases among the Libyans, but 18% of a control sample of Tunisians also report first cousin marriages between parents. Non-Libyan patients had no familial cases but the holocaust and dispersion of families made collection of reliable pedigrees difficult. Because CJD can be transmitted experimentally by inoculation of susceptible animals with brain tissue, dietary habits were investi- gated. Over 80% of Libyan patients ate brain but they did not appear to differ in this respect from controls. Libyan cases resembled controls also In source of meat and frequency of meat consumption. However, Libyan Jews preferred quick-grilled brain while Tunisians preferred fried and simmered brain. In their occupations, Libyans 1This work was supported In part by grants from the National Institutes of Health (NS13766-02) and from the Medical Research Council of Israel. 2Department of Sociology and Social Anthropology, Hebrew University of Jerusalem, Jerusalem, Israel. 3Department of Neurology, Temple University, School of Medicine, 3401 N. Broad Street, Philadelphia. PA 19140. 4Uri Leibowicz Neuroepidemiology Unit, Department of Neurology, Hadassah Hebrew University Hospital, Jerusalem, Isr snip...




Harvey Golberg et al.



regularly by residents of rural towns; the rural population ate goat as well. Chicken was eaten frequently by everyone, turkey much less frequently. There was no systematic differ- ence among the patients and the three control groups in the type of meat eaten or in the frequency of meat consumption.




Table 3 contains more detailed information on the con- sumption of brain. The percentages of those eating brain sometimes ranged from 79 to 92 for the four groups. The patient group was not consistently higher than any of the control groups. A higher percentage of Libyans ate brain in our small sample than in Bobowick's series (8). The percent- age eating spinal cord was consistently lower than that of brain consumers, but many people said that some spinal cord may have been found in bones purchased for soup, and the actual consumption of spinal cord may have been higher than that shown in Table 3.




Brain was commonly prepared in stew (m'chuma) and in a patty (m'akod). The number of people who reported eating these dishes at least once every two months was low (10-25%), and was not different in patients compared to controls.




Brain was also prepared by grilling, typically while the brain was wrapped in heavy paper, so that the full effect of the heat did not reach the brain. Usually the grilling was for a brief period of time, often less than five minutes. The number of people who reported eating grilled brain which was on the fire less than twenty minutes is shown in Table 3. More than one-third of the patients were described as having eaten brain prepared in this manner, but each of the control groups reported a higher precentage.




The Libyans also reported eating eyeballs, although not with great frequency. This question was introduced midway through the research so that only some people were queried. The small number of cases interviewed did not suggest any difference between patients and controls.




Reviewing the dietary data presented in Tables 2 and 3, it appears that meat consumption was relatively high among Libyan Jews, that brain was eaten by most people at some time, and that brain was eaten by many people several times a year. Patients, however, did not appear different from controls in most of the variables studied. Thus, there is an overall consistency in the answers among the four groups of Libyan respondents. While this was encouraging so far as the quality of the data is concerned, it did not support the hypothesis that (diseased) brain consumption set patients apart from other Libyans. It is still conceivable that high consumption of brain places the Libyan population at high risk with regard to CJD, but that another mechanism, perhaps genetic, makes some individuals more vulnerable than others...




snip...END




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1: Eur J Epidemiol 1991 Sep;7(5):520-3




"Clusters" of CJD in Slovakia: the first laboratory evidence of scrapie.



Mitrova E, Huncaga S, Hocman G, Nyitrayova O, Tatara M.



Institute of Preventive and Clinical Medicine, Bratislava.



Epidemic-like occurrence of Creutzfeldt-Jakob disease was observed in 1987 in Slovakia (Orava). Search for the cause of CJD focus indicated a coincidence of genetic and environmental risks in clustering patients. Since Spongiform Encephalopathies might be transmitted orally, (Bovine Spongiform Encephalopathy), the possibility of zoonotic source of CJD cases in Orava was also considered. A deficient knowledge about the occurrence of scrapie in Slovakia stimulated an examination of sheep with signs of CNS disorders in two flocks of Valasky breed in Orava. In one flock, neurohistopathological examination revealed in sheep brains lesions characteristic for scrapie. Frozen brain tissue of these animals were used for the detection of scrapie associated fibrils. They were found in 2 animals from the same flock. This is the first laboratory confirmation of scrapie in Czecho-Slovakia. The possible epidemiological and economical implications are emphasized.




http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1761109&dopt=Abstract





Scrapie to Humans USA?




1: Neuroepidemiology. 1985;4(4):240-9. Related Articles,




Sheep consumption: a possible source of spongiform encephalopathy in humans.




Davanipour Z, Alter M, Sobel E, Callahan M.




A fatal spongiform encephalopathy of sheep and goats (scrapie) shares many characteristics with Creutzfeldt-Jakob disease (CJD), a similar dementing illness of humans. To investigate the possibility that CJD is acquired by ingestion of contaminated sheep products, we collected information on production, slaughtering practices, and marketing of sheep in Pennsylvania. The study revealed that sheep were usually marketed before central nervous system signs of scrapie are expected to appear; breeds known to be susceptible to the disease were the most common breeds raised in the area; sheep were imported from other states including those with a high frequency of scrapie; use of veterinary services on the sheep farms investigated and, hence, opportunities to detect the disease were limited; sheep producers in the area knew little about scrapie despite the fact that the disease has been reported in the area, and animal organs including sheep organs were sometimes included in processed food. Therefore, it was concluded that in Pennsylvania there are some 'weak links' through which scrapie-infected animals could contaminate human food, and that consumption of these foods could perhaps account for spongiform encephalopathy in humans. The weak links observed are probably not unique to Pennsylvania.




PMID: 3915057 [PubMed - indexed for MEDLINE]




http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=3915057&dopt=Abstract






NEW SCIENTIST MAGAZINE 4/02/01



NEW SCIENTIST EDITORIAL PAGE 3



MAD SHEEP DISEASE?



IF THERE is one categorical pronouncement you can safely make about prion diseases like BSE or CJD, it is that one should not make categorical pronouncements. "British beef is safe" and "there is no BSE in Germany" come to mind. Now there are two more: "scrapie is safe", and "people don't catch sporadic CJD". Scrapie is the most widespread prion disease, infecting untold numbers of sheep worldwide. Sporadic CJD is the old-fashioned pre-BSE kind that is supposed to happen spontaneously in unlucky people. But a surprise observation in France suggests some sCJD cases--though by no means all--may be linked to scrapie after all (see p 4).




For years, British authorities asserted that BSE was harmless because it was a form of scrapie. In fact, the only evidence scrapie is safe is some broad-brush epidemiology, good as far as it goes but unable to reveal occasional risks for some people from some sheep. Alarm bells should have rung in 1980 when researchers gave monkeys scrapie by feeding them infected brains. But that research, like so much other work on prion diseases, was never followed up. We still have little idea what BSE does in pigs and chickens. The Queniborough vCJD outbreak (see p 5) would be easier to understand if we knew how much brain we must eat to be infected. As for scrapie, it shouldn't take a chance finding to tell us that there may be dangerous sheep out there.




Suspect symptoms




What if you can catch old-fashioned CJD by eating meat from a sheep infected with scrapie?





28 Mar 01




Like lambs to the slaughter




31 March 2001




by Debora MacKenzie




Magazine issue 2284.





FOUR years ago, Terry Singeltary watched his mother die horribly from a degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.




Most doctors believe that sCJD is caused by a prion protein deforming by chance into a killer. But Singeltary thinks otherwise. He is one of a number of campaigners who say that some sCJD, like the variant CJD related to BSE, is caused by eating meat from infected animals. Their suspicions have focused on sheep carrying scrapie, a BSE-like disease that is widespread in flocks across Europe and North America.




Now scientists in France have stumbled across new evidence that adds weight to the campaigners' fears. To their complete surprise, the researchers found that one strain of scrapie causes the same brain damage in mice as sCJD.




"This means we cannot rule out that at least some sCJD may be caused by some strains of scrapie," says team member Jean-Philippe Deslys of the French Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses, south-west of Paris. Hans Kretschmar of the University of Göttingen, who coordinates CJD surveillance in Germany, is so concerned by the findings that he now wants to trawl back through past sCJD cases to see if any might have been caused by eating infected mutton or lamb.




Scrapie has been around for centuries and until now there has been no evidence that it poses a risk to human health. But if the French finding means that scrapie can cause sCJD in people, countries around the world may have overlooked a CJD crisis to rival that caused by BSE.




Deslys and colleagues were originally studying vCJD, not sCJD. They injected the brains of macaque monkeys with brain from BSE cattle, and from French and British vCJD patients. The brain damage and clinical symptoms in the monkeys were the same for all three. Mice injected with the original sets of brain tissue or with infected monkey brain also developed the same symptoms.




As a control experiment, the team also injected mice with brain tissue from people and animals with other prion diseases: a French case of sCJD; a French patient who caught sCJD from human-derived growth hormone; sheep with a French strain of scrapie; and mice carrying a prion derived from an American scrapie strain. As expected, they all affected the brain in a different way from BSE and vCJD. But while the American strain of scrapie caused different damage from sCJD, the French strain produced exactly the same pathology.



"The main evidence that scrapie does not affect humans has been epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute for Animal Health in Edinburgh, who was a member of the same team as Deslys. "You see about the same incidence of the disease everywhere, whether or not there are many sheep, and in countries such as New Zealand with no scrapie." In the only previous comparisons of sCJD and scrapie in mice, Bruce found they were dissimilar.




But there are more than 20 strains of scrapie, and six of sCJD. "You would not necessarily see a relationship between the two with epidemiology if only some strains affect only some people," says Deslys. Bruce is cautious about the mouse results, but agrees they require further investigation. Other trials of scrapie and sCJD in mice, she says, are in progress.




People can have three different genetic variations of the human prion protein, and each type of protein can fold up two different ways. Kretschmar has found that these six combinations correspond to six clinical types of sCJD: each type of normal prion produces a particular pathology when it spontaneously deforms to produce sCJD.




But if these proteins deform because of infection with a disease-causing prion, the relationship between pathology and prion type should be different, as it is in vCJD. "If we look at brain samples from sporadic CJD cases and find some that do not fit the pattern," says Kretschmar, "that could mean they were caused by infection."




There are 250 deaths per year from sCJD in the US, and a similar incidence elsewhere. Singeltary and other US activists think that some of these people died after eating contaminated meat or "nutritional" pills containing dried animal brain. Governments will have a hard time facing activists like Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have insisted.




Deslys's work on macaques also provides further proof that the human disease vCJD is caused by BSE. And the experiments showed that vCJD is much more virulent to primates than BSE, even when injected into the bloodstream rather than the brain. This, says Deslys, means that there is an even bigger risk than we thought that vCJD can be passed from one patient to another through contaminated blood transfusions and surgical instruments.





http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html





“The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate.”





Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt– Jakob disease: Implications for human health




Corinne Ida Lasmézas*†, Jean-Guy Fournier*, Virginie Nouvel*, Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp‡, Jean-Jacques Hauw§, James Ironside¶, Moira Bruce‖, Dominique Dormont*, and Jean-Philippe Deslys*




+ Author Affiliations




*Commissariat à l'Energie Atomique, Service de Neurovirologie, Direction des Sciences du Vivant/Département de Recherche Medicale, Centre de Recherches du Service de Santé des Armées 60-68, Avenue du Général Leclerc, BP 6, 92 265 Fontenay-aux-Roses Cedex, France; ‡Hôpital Neurologique Pierre Wertheimer, 59, Boulevard Pinel, 69003 Lyon, France; §Laboratoire de Neuropathologie, Hôpital de la Salpêtrière, 83, Boulevard de l'Hôpital, 75013 Paris, France; ¶Creutzfeldt–Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, United Kingdom; and ‖Institute for Animal Health, Neuropathogenesis Unit, West Mains Road, Edinburgh EH9 3JF, United Kingdom




Edited by D. Carleton Gajdusek, Centre National de la Recherche Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000 (received for review October 16, 2000)




Abstract




There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt–Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.




snip...



http://www.pnas.org/cgi/content/full/041490898v1






1: J Infect Dis 1980 Aug;142(2):205-8




Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.




Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.




Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.




snip...




The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.



PMID: 6997404




http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract





12/10/76




AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE




Office Note CHAIRMAN: PROFESSOR PETER WILDY




snip...




A The Present Position with respect to Scrapie A] The Problem Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries. The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep. It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible. Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates.




One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.




snip...



76/10.12/4.6




http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf






Nature. 1972 Mar 10;236(5341):73-4.



Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).



Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0



Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)



C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland



SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).




http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html






Wednesday, February 16, 2011



IN CONFIDENCE



SCRAPIE TRANSMISSION TO CHIMPANZEES



IN CONFIDENCE




http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html






Sunday, April 18, 2010



SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010




http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html






Monday, April 25, 2011



Experimental Oral Transmission of Atypical Scrapie to Sheep




Volume 17, Number 5-May 2011



http://nor-98.blogspot.com/2011/04/experimental-oral-transmission-of.html





Wednesday, January 18, 2012



BSE IN GOATS CAN BE MISTAKEN FOR SCRAPIE



February 1, 2012





http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/bse-in-goats-can-be-mistaken-for.html





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




http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/selection-of-distinct-strain-phenotypes.html





Thursday, July 14, 2011



Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4)




http://transmissiblespongiformencephalopathy.blogspot.com/2011/07/histopathological-studies-of-ch1641.html






Monday, June 27, 2011



Comparison of Sheep Nor98 with Human Variably Protease-Sensitive Prionopathy and Gerstmann-Sträussler-Scheinker Disease




http://prionopathy.blogspot.com/2011/06/comparison-of-sheep-nor98-with-human.html





Wednesday, January 11, 2012



Bucks for brains on offer to cattle and sheep producers Queensland TSE PRION TESTING




http://usdameatexport.blogspot.com/2012/01/bucks-for-brains-on-offer-to-cattle-and.html






BSE: TIME TO TAKE H.B. PARRY SERIOUSLY



If the scrapie agent is generated from ovine DNA and thence causes disease in other species, then perhaps, bearing in mind the possible role of scrapie in CJD of humans (Davinpour et al, 1985), scrapie and not BSE should be the notifiable disease. ...




http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.gov.uk/files/yb/1988/06/08004001.pdf






Thursday, November 18, 2010



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




http://bse-atypical.blogspot.com/2010/11/increased-susceptibility-of-human-prp.html






Sunday, December 12, 2010



EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010




http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/efsa-reviews-bsetse-infectivity-in.html






Monday, November 22, 2010



Atypical transmissible spongiform encephalopathies in ruminants: a challenge for disease surveillance and control



REVIEW ARTICLES




http://transmissiblespongiformencephalopathy.blogspot.com/2010/11/atypical-transmissible-spongiform.html






Wednesday, January 19, 2011



EFSA and ECDC review scientific evidence on possible links between TSEs in animals and humans Webnachricht 19 Januar 2011




http://transmissiblespongiformencephalopathy.blogspot.com/2011/01/efsa-and-ecdc-review-scientific.html






RISK TO PHARMACEUTICALS



Prion Disease Risks in the 21st Century 2011



PDA European Virus-TSE Safety Dr. Detwiler



Dr. Detwiler published Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety Forum\Presentations TSE\ Page 33 and 34 of 44 ;




http://www.pda.org/




http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/prion-disease-risks-in-21st-century.html





Thursday, January 26, 2012



The Risk of Prion Zoonoses



Science 27 January 2012: Vol. 335 no. 6067 pp. 411-413 DOI: 10.1126/science.1218167



http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/risk-of-prion-zoonoses.html




Thursday, January 26, 2012



Facilitated Cross-Species Transmission of Prions in Extraneural Tissue



Science 27 January 2012: Vol. 335 no. 6067 pp. 472-475 DOI: 10.1126/science.1215659



http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/facilitated-cross-species-transmission.html






TSS






*** UPDATE MARCH 20, 2012 ***



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

From: Terry S. Singeltary Sr.

Sent: Tuesday, March 20, 2012 12:14 PM

To: CJD-L@LISTS.AEGEE.ORG

Subject: Re: [CJD-L] The First Report of a Patient with Probable Variant Creutzfeldt-Jakob Disease in Turkey




IN reply to ;



Response to the Article by Adanipar et al.:



The First Report of a Patient with Probable Variant Creutzfeldt-Jakob Disease in Turkey, Dement Geriatr Cogn Disord Extra 2011;1:429–432



Dear Editor



It is essential that all cases of variant Creutzfeldt-Jakob disease (vCJD) are reported as the incidence and geographic distribution of cases inform public health policy. Validated criteria for the classification of cases of suspected vCJD [1] have been agreed on internationally and contain obligatory preconditions, the presence of specific clinical features, and the results of specialist investigations, including the ‘pulvinar sign’ on MRI brain scan. A definitive diagnosis of vCJD requires neuropathological confirmation, either at brain biopsy or autopsy.



The case described by Adanipar et al. [2] has been reported as a case of ‘probable variant Creutzfeldt-Jakob disease’. However, although the clinical features would be compatible with this condition, other characteristics of the case make this diagnosis unlikely. One of the preconditions for the diagnosis of vCJD is that there should be ‘no evidence of a familial form of TSE’, but the authors have identified a P102L mutation, an abnormality associated with the familial prion disease Gerstmann-Straussler disease [3] . The codon 129 genotype was heterozygous, which has not been identified, to date, in any definite or probable case of vCJD. The MRI scan images published in the article show high signal in the caudate and lentiform nuclei, appearances seen in sporadic CJD [4] , and not the ‘pulvinar sign’ which is characteristic of vCJD.



The case reported is of significant interest, but the suggested diagnosis of vCJD cannot be regarded as correct unless there is neuropathological verification of this diagnosis.



Yours sincerely,



The EUROCJD Group



R.G. Will This is an Open Access article licensed under the terms of the Creative Commons Attribution- NonCommercial-NoDerivs 3.0 License (www.karger.com/OA-license), applicable to the online version of the article only. Distribution for non-commercial purposes only. National CJD Research and Surveillance Unit, Western General Hospital Edinburgh EH4 2XU (UK) Tel. +44 131 537 2085, E-Mail r.g.will@ed.ac.uk www.karger.com/dee DOI: 10.1159/000337504



References 1 Heath CA, Cooper SA, Murray K, Lowman A, Henry C, Macleod MA, Stewart GE, Zeidler M, Mackenzie JM, Ironside JW, Summers DM, Knight RS, Will RG: Validation of diagnostic criteria for variant Creutzfeldt-Jakob disease. Ann Neurol 2010; 67: 761–770. 2 Adapınar DÖ, Saylısoy S, Yenilmez Ç, Aslan H, Ertan B, Artan S, Güleç G, Susuz Ç, Adapınar B: The first report of a patient with probable variant Creutzfeldt-Jakob disease in Turkey. Dement Geriatr Cogn Disord Extra 2011; 1: 429–432. 3 Kovacs GG, Trabattoni G, Hainfellner JA, Ironside JW, Knight RSG, Budka H: Mutations of the prion protein gene: phenotypic spectrum. J Neurol 2002; 249: 1567–1582. 4 Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemenn U, Breithaupt M, Varges D, Meissner B, Ladogana A, Schuur M, Haik S, Collins SJ, Jansen GH, Stokin GB, Pimentel J, Hewer E, Collie D, Smith P, Roberts H, Brandel JP, van Duijn C, Pocchiari M, Begue C, Cras P, Will RG, Sanchez- Juan P: Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain 2009; 132: 2659–2668.



http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowPDF&ArtikelNr=337504&Ausgabe=256812&ProduktNr=254733&filename=337504.pdf







I kindly submit the following;




THE UKBSEnvCJD only theory, is wrong, and only enhances the spread of other zoonosis strains of human and animal Transmissible Spongiform Encephalopathy TSE prion disease around the globe.

THE OIE and USDA stance on the BSE MRR policy, and disregard from their own science on atypical BSE, also, enhances the spread of human and animal Transmissible Spongiform Encephalopathy TSE prion disease around the globe.





Monday, October 10, 2011


EFSA Journal 2011 The European Response to BSE: A Success Story


snip...



EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.



snip...



http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1





http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf






see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;



http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html








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...



http://www.neuroprion.org/en/np-neuroprion.html





Rural and Regional Affairs and Transport References Committee




The possible impacts and consequences for public health, trade and agriculture of the Government's decision to relax import restrictions on beef Final report June 2010



2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49



2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo-has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50



http://www.aph.gov.au/senate/committee/rrat_ctte/mad_cows/report/report.pdf






snip...see full text and more here ;




Thursday, March 01, 2012


Variant Creutzfeldt-Jakob disease Fact sheet N°180 Revised February 2012 W.H.O.


http://vcjd.blogspot.com/2012/03/variant-creutzfeldt-jakob-disease-fact.html






***** Monday, March 19, 2012 *****




Infectivity in Skeletal Muscle of Cattle with Atypical Bovine Spongiform Encephalopathy




PLoS One. 2012; 7(2): e31449.





http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/infectivity-in-skeletal-muscle-of.html







Friday, March 09, 2012




Experimental H-type and L-type bovine spongiform encephalopathy in cattle: observation of two clinical syndromes and diagnostic challenges




Research article




http://bse-atypical.blogspot.com/2012/03/experimental-h-type-and-l-type-bovine.html






Saturday, June 25, 2011




Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque


"BSE-L in North America may have existed for decades"


http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/transmissibility-of-bse-l-and-cattle.html






Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.


snip...


The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...


http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf







USDA OIE ON HOW TO TRADE MAD COW DISEASE GLOBALLY $$$



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



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, then 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 FULL TEXT AND SOURCE REFERENCES AS FOLLOWS ;



http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/aphis-proposes-new-bovine-spongiform.html





Saturday, March 5, 2011



MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA



http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html







Sunday, February 12, 2012



National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas



http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/national-prion-disease-pathology.html







DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)



The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....



Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!



And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...


Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"



again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.



You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)



END...TSS





IN CONFIDENCE



Perceptions of unconventional slow virus in the USA



GAH WELLS



Report of a visit to the U.S.A. April-May 1989



3. Prof. A Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed.



Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fantical incident to be avoided in the USA AT ALL COSTS.



http://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf









Saturday, June 13, 2009



Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009



http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html







re-Human Prion Diseases in the United States



Posted by flounder on 01 Jan 2010 at 18:11 GMT



I kindly disagree with your synopsis for the following reasons ;



http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd







Saturday, January 2, 2010



Human Prion Diseases in the United States January 1, 2010 ***FINAL***



http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html







GOVERNMENT REMOVED MY SUBMISSION RECENTLY ;



Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.



http://www.regulations.gov/fdmspublic/ContentViewer?objectId=090000648027c28e&disposition=attachment&contentType=pdf





http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html







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 8/3/2006



Greetings FSIS, I would kindly like to comment on the following ;



http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf





http://www.scribd.com/doc/1490709/USDA-200600111







Response to Public Comments on the 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: http://www.fsis.usda.gov/Science/Risk_Assessments/index.asp).




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:



http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Comments.pdf





Suppressed peer review of Harvard study October 31, 2002.



October 31, 2002 Review of the Evaluation of the Potential for Bovine Spongiform Encephalopathy in the United States Conducted by the Harvard Center for Risk Analysis, Harvard School of Public Health and Center for Computational Epidemiology, College of Veterinary Medicine, Tuskegee University Final Report Prepared for U.S. Department of Agriculture Food Safety and Inspection Service Office of Public Health and Science Prepared by RTI Health, Social, and Economics Research Research Triangle Park, NC 27709 RTI Project Number 07182.024



http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf






Sunday, February 14, 2010



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



http://bseusa.blogspot.com/2010/02/docket-no-fsis-2006-0011-fsis-harvard.html





http://www.scribd.com/doc/1490059/USDA-03025IFA2





http://docket-aphis-2006-0041.blogspot.com/







Sunday, August 09, 2009



CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009



http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html







Tuesday, August 18, 2009



BSE-The Untold Story - joe gibbs and singeltary 1999 - 2009



http://madcowusda.blogspot.com/2009/08/bse-untold-story-joe-gibbs-and.html







kind regards, terry

layperson

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



END...TSS

Labels: , , , ,

Friday, February 05, 2010

New Variant Creutzfelt Jakob Disease case reports United States 2010 A Review

New Variant Creutzfelt Jakob Disease case reports United States 2010 A Review

ALL is well in the USA, no mad cows, and no human mad cow there from. it's all the Canadians fault, the U.K.'s fault, and or Saudi Arabia's fault, even though Saudi Arabia has never documented mad cow disease in their cows. and if you ask the USDA, they too will tell you it was all Canada's fault, Saudi Arabia's and or the UK's, and or a spontaneous event of genetic differences, but no mad cows and or human mad cow there from. RIGHT $$$

I propose that this third case of nvCJD documented in the USA has as much, or even more of a chance of being an indigenous home grown nvCJD case in the USA, as that of being exposed the short time this person was in Saudi Arabia, a country that has never had a case of mad cow disease documented. THE USA import of highly infectious materials from documented BSE countries, including the U.K. was phenomenal, let alone it's own TME exposed MBM from right here in the USA. Strange there is no case history of this supposedly Saudi Arabia source case and history of time in the USA compared to time in Saudi Arabia, food habits, and such? It would be nice to compare and try and figure up incubation time periods with this third case, age, symptoms, beginning clinical signs to death, etc, a good case study in other words, where is it ???

let's look at a few factors ;

Incubation period: The incubation period in vCJD is difficult to establish, but is estimated to be around 10 years. The incubation period in transfusion transmitted vCJD has been between 6.5 and 8 years.


http://ecdc.europa.eu/en/healthtopics/Documents/2018_ECDC_Variant_Creutzfeldt_FACTSHEET.pdf


The exact incubation period for foodborne vCJD is unknown. However, a range of possible incubation periods was estimated for 4 vCJD patients who likely acquired the disease during their residence in the United Kingdom and for 5 vCJD patients reported as part of a cluster in Leicestershire, England (Table). The median of the estimated range of incubation periods for these 9 vCJD patients was 13 years. ...

In June 2005 the US Department of Agriculture confirmed BSE in an = 12-year-old cow born and raised in Texas. This is the first time an indigenous BSE case was detected in the United States. A previous BSE-positive cow identified in Washinton State was imported from Canada. ...

http://www.mhlw.go.jp/shingi/2006/01/dl/s0126-10c45.pdf


ACTUALLY, another highly suspect mad cow was detected in Texas, and was covered-up. nobody likes to speak of this mad cow too much ;

Statement on Texas cow with central nervous system symptoms

Date: 05 May 2004 - 0:00 PDT

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.

Source: FDA http://www.fda.gov/bbs/topics/news/2004/NEW01061.html


Actually, it's been one blunder after another blunder with the BSE surveillance and BSE feed ban of August 4, 1997. you can it documented here ;

http://madcowusda.blogspot.com/ and http://madcowtesting.blogspot.com/


ONE must consider, North America has documented c-BSE, atypical h-BSE, and atypical l-BSE (BASE). Also documented, two strains of CWD in deer and elk, i.e. typical CWD and the Wisconsin strain, to date. Also documented, many many typical strains of Scrapie in sheep and goats, with the atypical Nor-98 scrapie also being documented, to date. Also, two strains of TME in mink, the hyper and the drowsy TME. Who knows about feline and canine TSE, but over the years, decades, at some point, all of these species have been rendered and fed back to food producing animals for humans and animals. This is fact.

WE ALSO SEE atypical sporadic CJD showing up in young and old people in the USA, some of long duration. incubation and symptoms different in some cases, some pathology difference, SO WHY is it impossible to believe that a sub-type of the sporadic CJDs could be from consumption of these atypical animal TSE cases and or iCJD there from, and just not look like the typical UK c-BSE human TSE i.e. vCJD aka nvCJD ??? friendly fire i.e. pass it forward i.e. iatrogenic Creutzfeldt Jakob Disease there from, could play a bigger role in the transmission in consumption itself, and be simply hiding itself as a sporadic CJD. ...

CJD USA RISING, with UNKNOWN PHENOTYPE ;

5 Includes 41 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 46 cases with type determination pending in which the diagnosis of vCJD has been excluded.

http://www.cjdsurveillance.com/pdf/case-table.pdf


let's look at the three case reports of nvCJD in the USA, and lets see what the incubation periods were ;


Variant Creutzfeldt-Jakob Disease Death, United States: 1st Case Report


The only variant Creutzfeldt-Jakob disease (vCJD) patient identified in the United States died in 2004, and the diagnosis was confirmed by analysis of autopsy tissue. The patient likely acquired the disease while growing up in Great Britain before immigrating to the United States in 1992. Additional vCJD patients continue to be identified outside the United Kingdom, including 2 more patients in Ireland, and 1 patient each in Japan, Portugal, Saudi Arabia, Spain and the Netherlands. The reports of bloodborne transmission of vCJD in 2 patients, 1 of whom was heterozygous for methionine and valine at polymorphic codon 129, add to the uncertainty about the future of the vCJD outbreak.

snip...

see case history on 1st nvCJD case in USA here ;

http://www.medscape.com/viewarticle/512024_2


A 22-year-old Florida resident became the first person in the USA to be diagnosed with probable variant Creutzfeldt-Jakob disease (vCJD), according to the US Centers for Disease Control and Prevention (CDC). Because the young woman was raised in the UK when the BSE outbreak was at its peak, officials believe that she contracted the disease there. The case report, which is published in Morbidity and Mortality Weekly Report (2002; 51: 927—29;

http://www.thelancet.com/journals/laninf/article/PIIS1473-3099(02)00474-7/fulltext


Probable Variant Creutzfeldt-Jakob Disease in a US Resident—Florida, 2002

JAMA. 2002;288:2965-2967.

MMWR. 2002;51:927-929

On April 18, 2002, the Florida Department of Health and CDC announced the occurrence of a likely case of variant Creutzfeldt-Jakob disease (vCJD) in a Florida resident aged 22 years. This report documents the investigation of this case and underscores the importance of physicians increasing their suspicion for vCJD in patients presenting with clinical features described in this report who have spent time in areas in which bovine spongiform encephalopathy (BSE) is endemic.

In early November 2001, the patient sought medical care for depression and memory loss that adversely affected the patient's work performance. The primary-care physician referred the patient to a psychologist. In early December 2001, the patient received a traffic ticket for failing to yield the right of way. In mid-December 2001, the patient had involuntary muscular movements, gait changes, difficulty dressing, and incontinence. In January 2002, the patient was evaluated in a local emergency department for these symptoms. A computerized tomography scan of the head revealed no abnormalities; a panic attack was diagnosed, and the patient was treated with an anti-anxiety medication.

In late January 2002, the patient's mother, a resident of the United Kingdom, took the patient to England, where medical evaluations were conducted during the next 3 months. During this period, the patient's memory loss and other neurologic symptoms worsened. The patient experienced falls with minor injuries, had difficulty taking a shower and dressing, and was unable to remember a home telephone number or to make accurate mathematical calculations. The patient subsequently became confused, hallucinated, and had speech abnormalities with lack of content, bradykinesia, and spasticity. The patient was referred to a neurologist, who suspected vCJD and subsequently referred the patient to the National Prion Clinic in the United Kingdom.

Medical evaluations at the National Prion Clinic included an electroencephalogram (EEG), which revealed a normal alpharhythm, and magnetic resonance imaging (MRI) studies, which revealed signal abnormalities in the pulvinar and metathalamus region that were suggestive of vCJD. The patient had a tonsil biopsy, and a Western blot analysis of the biopsy tissue demonstrated the presence of protease-resistant prion protein (PrP-res) with the characteristic pattern of vCJD; an immunohistochemical test for PrP-res also supported a diagnosis of vCJD. Analysis of the prion protein gene detected no mutation and showed methionine homozygosity at codon 129, consistent with all 105 vCJD patients tested in the United Kingdom (R. Will, Western General Hospital, Edinburgh, Scotland, personal communication, 2002).

The patient received experimental treatment with quinacrine for 3 months. As of late September 2002, the patient had become bedridden, experienced considerable weight loss requiring surgical insertion of a feeding tube, and was no longer communicating with family members. On the basis of a case definition developed in the United Kingdom, the patient's illness met criteria for a probable case of vCJD.1

The patient was born in the United Kingdom in 1979 and moved to Florida in 1992. The patient never had donated or received blood, plasma, or organs and never had received human growth hormone. There was no family history of CJD. In October 2001, before the onset of the illness, the patient's wisdom teeth were extracted, but there was no history of major surgery.

Reported by:

S Wiersma, MD, State Epidemiologist, Florida Dept of Health. S Cooper, MRCP, R Knight, FRCP, National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, Scotland; AM Kennedy, MD, National Prion Clinic, Dept of Neurology, St. Mary's Hospital, London; S Joiner, MSc, Medical Research Council Prion Unit, Dept of Neurodegenerative Disease, Institute of Neurology, London, United Kingdom. E Belay, MD, LB Schonberger, MD, Div of Viral and Rickettsial Diseases, National Center for Infectious Diseases, CDC.

CDC Editorial Note:

snip...full text ;


http://jama.ama-assn.org/cgi/content/full/288/23/2965


http://www2a.cdc.gov/han/ArchiveSys/ViewMsgV.asp?AlertNum=00085


http://www.cdc.gov/ncidod/EID/vol11no09/pdfs/05-0371.pdf


http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1919



Variant Creutzfeldt-Jakob Disease Death, United States: 2nd Case Report


vCJD (Variant Creutzfeldt-Jakob Disease)

Update: Variant Creutzfeldt-Jakob Disease in a U.K. Citizen Who Had Temporarily Resided in Texas, 2001-2005

In November 2005, the U.K. National Creutzfeldt-Jakob Disease (CJD) Surveillance Unit in Edinburgh, Scotland notified the Centers for Disease Control and Prevention (CDC) about a probable variant CJD diagnosis in a 30-year-old man who resided in Texas during 2001-2005. The patient had onset of symptoms in early 2005 while in Texas. He then returned to the United Kingdom, where his illness progressed, and a diagnosis of variant CJD was made. This diagnosis was confirmed neuropathologically after the patient's death.

The variant CJD diagnosis was initially based on typical clinical manifestations of the disease and demonstration of the characteristic “pulvinar sign” on magnetic resonance imaging of the brain. No biopsy tissues are available for pathologic confirmation of the diagnosis. While living in the United States, the patient had no history of hospitalization, of having invasive medical procedures, or of donation or receipt of blood and blood products.

The patient almost certainly acquired the disease in the United Kingdom. He was born in the United Kingdom and lived there throughout the defined period of risk (1980-1996) for human exposure to the agent of bovine spongiform encephalopathy (BSE, commonly known as “mad cow” disease). His stay in the United States was too brief relative to what is known about the incubation period for variant CJD. For additional information about the incubation period for variant CJD, see Belay ED, Sejvar JJ, Shieh WJ, et al. “Variant Creutzfeldt-Jakob Disease Death, United States,” Emerg Infect Dis 2005; available at

http://www.cdc.gov/ncidod/EID/vol11no09/05-0371.htm.


By convention, variant CJD cases are ascribed to the country of initial symptom onset, regardless of where the exposure occurred. Since variant CJD was first reported in 1996, a total of 195 patients with this disease from 11 countries have been identified. As of August 11, 2006, variant CJD cases have been reported from the following countries: 162 from the United Kingdom, 20 from France, 4 from Ireland, 2 from the United States (including the current case), and one each from Canada, Italy, Japan, Netherlands, Portugal, Saudi Arabia, and Spain. Similar to the two U.S. cases, two of the four cases from Ireland and the single cases from Canada and Japan were likely exposed to the BSE agent while residing in the United Kingdom.One of the 20 French cases may also have been infected in the United Kingdom. Strong scientific evidence indicates that variant CJD results from the transmission to humans of the agent that causes BSE in cattle. The BSE outbreak in cattle that was first detected in the 1980s in the United Kingdom has spread to many other European countries, and cases in cattle have been identified outside of Europe, in Canada, Israel, Japan, and the United States.

Date: August 14, 2006 Content source: National Center for Infectious Diseases

http://www.cdc.gov/ncidod/dvrd/vcjd/other/probablevcjd_texas2001_2005_111805.htm



Variant Creutzfeldt-Jakob Disease Death, United States: 3rd Case Report


NO DETAILED case report I could find on this very unusual and assumed case of nvCJD in the USA, but here is cdc short report ;

vCJD (Variant Creutzfeldt-Jakob Disease)

Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East

The Virginia Department of Health and the Centers for Disease Control and Prevention announce the recent confirmation of a vCJD case in a U.S. resident. This is the third vCJD case identified in a U.S. resident. This latest U.S. case occurred in a young adult who was born and raised in Saudi Arabia and has lived in the United States since late 2005. The patient occasionally stayed in the United States for up to 3 months at a time since 2001 and there was a shorter visit in 1989. In late November 2006, the Clinical Prion Research Team at the University of California San Francisco Memory and Aging Center confirmed the vCJD clinical diagnosis by pathologic study of adenoid and brain biopsy tissues. The two previously reported vCJD case-patients in U.S. residents were each born and raised in the United Kingdom (U.K.), where they were believed to have been infected by the agent responsible for their disease. There is strong scientific evidence that the agent causing vCJD is the same agent that causes bovine spongiform encephalopathy (BSE, commonly known as mad cow disease).

Variant CJD is a rare, degenerative, fatal brain disorder that emerged in the United Kingdom in the mid-1990s. Although experience with this new disease is limited, evidence to date indicates that there has never been a case transmitted from person-to-person except through blood transfusion. Instead, the disease is thought to result primarily from consumption of cattle products contaminated with the BSE agent. Although no cases of BSE in cattle have been reported in Saudi Arabia, potentially contaminated cattle products from the United Kingdom may have been exported to Saudi Arabia for many years during the large U.K. BSE outbreak.

The current case-patient has no history of receipt of blood, a past neurosurgical procedure, or residing in or visiting countries of Europe. Based on the patient's history, the occurrence of a previously reported Saudi case of vCJD attributed to likely consumption of BSE-contaminated cattle products in Saudi Arabia, and the expected greater than 7 year incubation period for food-related vCJD, this U.S. case-patient was most likely infected from contaminated cattle products consumed as a child when living in Saudi Arabia (1). The current patient has no history of donating blood and the public health investigation has identified no risk of transmission to U.S. residents from this patient.

As of November 2006, 200 vCJD patients were reported world-wide, including 164 patients identified in the United Kingdom, 21 in France, 4 in the Republic of Ireland, 3 in the United States (including the present case-patient), 2 in the Netherlands and 1 each in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain. Of the 200 reported vCJD patients, all except 10 of them (including the present case-patient) had resided either in the United Kingdom (170 cases) for over 6 months during the 1980-1996 period of the large UK BSE outbreak or alternatively in France (20 cases).

As reported in 2005 (1), the U.S. National Prion Disease Pathology Surveillance Center at Case Western Reserve University confirmed the diagnosis in the one previously identified case of vCJD in a Saudi resident. He was hospitalized in Saudi Arabia and his brain biopsy specimen was shipped to the United States for analysis. This earlier vCJD case-patient was believed to have contracted his fatal disease in Saudi Arabia (1).

1) Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P, Hunter S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis 2005, 11 (9):1351-1354.

Date: November 29, 2006

http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm


The third US resident with vCJD was born and raised in Saudi Arabia and beginning in 2001 he occasionally stayed in the United States for periods of up to 3 months duration [30], [31]. The patient relocated to the United States in 2005 where onset of vCJD symptoms was experienced in the spring of 2006. The diagnosis of vCJD was confirmed based on pathological study of adenoid and brain biopsy tissues in November 2006. The patient died later in 2006. The patient had no past history of neurosurgical procedures or visits to European countries. A previous case of vCJD attributed to consumption of BSE-contaminated cattle products had been reported in a Saudi Arabian resident [13].


http://www.plosone.org/article/info%3Adoi%2F10.1371%2Fjournal.pone.0008521?utm_source=feedburner&utm_medium=feed&utm_campaign=Feed%3A+plosone%2FPLoSONE+%28PLoS+ONE+Alerts%3A+New+Articles%29


re-Human Prion Diseases in the United States Posted by flounder on 01 Jan 2010 at 18:11 GMT

I kindly disagree with your synopsis for the following reasons ;


http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd


MORE BSe here from cdc about this 3rd case of vCJD in the USA ;


The Virginia Department of Health and the Centers for Disease Control and Prevention announce the recent confirmation of a case of variant Creutzfeldt-Jakob disease (vCJD) in a Virginia resident. There is no evidence to suggest that this case of vCJD was caused by anything the patient was exposed to while residing in the U.S. or that this situation represents a public health threat to any U.S. resident.

For more information on vCJD, visit CDC’s Web site at http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm.


Virginia CJD Epidemiology CJD is currently a reportable condition in Virginia only if the ill person is less than 55 years of age; therefore data on this condition are very limited.

snip...

One limitation to our understanding of CJD in Virginia and the US is that surveillance mainly uses death certificate data. However, it is very likely that some cases do not get recorded. CJD is an uncommon disease and physicians who are not familiar with the signs and symptoms may mistake it for other conditions. Evidence of the under-reporting of cases comes from some European countries, where enhanced surveillance has found higher than expected rates due to increased awareness and better diagnosis. 11 In addition, one small study reported that as many as 13% of patients diagnosed with Alzheimer’s disease were found upon autopsy to have actually had CJD.12 Therefore, it seems likely that the current data underestimate the true incidence of CJD in Virginia and in the US.

http://www.vdh.state.va.us/Epidemiology/Surveillance/Bulletin/pdf/vebjan05.pdf


Creutzfeldt-Jakob Disease (CJD) In 2006, a case of Creutzfeldt- Jakob Disease (CJD) in an individual less than 55 years of age was reported. Sporadic CJD occurs in Virginia; the age criteria is intended to screen for potential cases of new variant CJD (nvCJD). On further investigation, this individual was found to have new variant CJD. Epidemiologic investigation suggested that it was extremely unlikely that this individual acquired the infection in the U.S. However, this counted as only the third case of nvCJD ever diagnosed in the U.S. A high index of suspicion and early testing is important for diagnosing CJD, since arranging for brain biopsy or autopsy to confirm the diagnosis may be important for families and healthcare professionals.

http://www.vdh.virginia.gov/Epidemiology/Surveillance/Bulletin/pdf/vebjul07.pdf


From: TSS
Subject: Third case of vCJD reported in the United States
Date: December 7, 2006 at 11:08 am PST


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


Third case of vCJD reported in the United States Editorial Team (eurosurveillance.weekly@hpa.org.uk), Eurosurveillance editorial office

A clinical diagnosis of variant Creutzfeldt Jakob Disease (vCJD) was confirmed after brain biopsy investigations in a United States (US) resident and reported in November [1]. The patient is a young man who grew up in Saudi Arabia and lived in the US since late 2005. Before that he visited the US once in 1989 and several times after 2001. He has never visited any country in Europe or received a blood transfusion nor has he undergone any neurosurgical procedure. This vCJD case is the third in a US resident. The previous two patients both grew up in the United Kingdom (UK), and this is where they were believed to have been infected [2].

In Saudi Arabia, the first and only previous case of vCJD was reported in 2005. This was suspected to be related to consumption of meat contaminated with the prion agent which causes bovine spongiform encephalitis in cattle (BSE). The European Food Safety Authority (http://www.efsa.org) has not published a geographical BSE risk assessment for Saudi Arabia [3] and there have been no cases of BSE in cattle reported by Saudi Arabia to the World Organisation for Animal Health (http://www.oie.int). Although the UK is not the only potential beef exporter to have had a BSE epidemic, it remains plausible, subject to Saudi Arabia's import policy, that contaminated beef was inadvertently imported from the UK to Saudi Arabia in the period before 1996 (when the EU banned the export of UK beef and cattle).

Based on this patient's history, the occurrence of a previously reported case of vCJD in Saudi Arabia, and the expected length of the incubation period for food-related vCJD, the most likely source of infection is thought to be contaminated meat products the patient consumed as a child when living in Saudi Arabia. The patient has no known history of donating blood, and investigations have identified no risk of onwards transmission within the US.

Variant Creutzfeldt-Jakob disease was first identified in the United Kingdom in the mid-1990s. As of November 2006, worldwide there have been 200 vCJD cases: 164 patients in the United Kingdom, 21 in France, four in Ireland, three in the US (including the present case), two in the Netherlands and one each in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain [4]. All patients, except 10 (including the present case) had lived either in the United Kingdom (170 cases) or in France (20 cases). Evidence so far indicates that the most probable source of infection in most cases was consumption of meat products contaminated with the prion agent causing BSE.

References: Centers for Disease Control and Prevention.

Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East.

(http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm)


Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P, Hunter S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis 2005, 11 (9):1351-1354. European Food Safety Authority . Geographical BSE Risk (GBR) assessments covering 2000-2006. List of countries and their GBR level of risk as assessed by the Scientific Steering Committee and the (EFSA). 1 August 2006.

(http://www.efsa.europa.eu/etc/medialib/efsa/science/tse_assessments/gbr_assessments/summary_list_countries.Par.0001.File.dat/GBR_assessments_table_Overview_assessed_countries_2002-2006.pdf)



The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.

http://www.oie.int/boutique/extrait/06heim937950.pdf


Variant Creuzfeldt-Jakob disease. Current data – December 2006.

(http://www.cjd.ed.ac.uk/vcjdworld.htm)


http://www.eurosurveillance.org/ew/2006/061207.asp#2


>>>The patient is a young man who grew up in Saudi Arabia and lived in the US since late 2005. Before that he visited the US once in 1989 and several times after 2001. He has never visited any country in Europe or received a blood transfusion nor has he undergone any neurosurgical procedure.<<<


Heaven forbid anyone suggest that this unlucky Soul was contaminated in the USA from vCJD. This would just be preposterous, wouldn't it $$$ i am reminded of a few things deep throat told me years ago;


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



The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people.........Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why???? than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....


Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!


And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...


Thanks as always for your help.


(Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"


again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.


You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)


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


12/07/06


HOWEVER, if you ONLY consider an OUTSIDE source of mbm from the UK, you will see that indeed the UK did dump a great deal of mad cow poison on the middle east, compared to the amount they dumped on USA. comparing from my records from the U.K., the USA imported about 44 tons of UK mbm or greaves, Canada got 83 tons in 3 years, 1993, 1994, and 1995. compared to about 6,985 tons exported from the UK to Saudi Arabia over a period of about 20 years, with Saudi importing mbm from UK as late as 1995. ISRAEL ALSO IMPORTED A GREAT DEAL OF THIS POISON, 30,006 TONS of MBM FROM UK. A great deal was also imported to Asian Countries as well. HOWEVER, we cannot state that this is indeed a case of vCJD exported to the USA from Saudi with certainty. now we all know that the USDA will paint this pig with lipstick and take it to the dance, to the prom and anywhere else they can take it, but the fact still remains, they cannot state this with scientific proof. IF you look at the USA and it's TSE problem, the rendering industry, and the fact that the USA shipped the technology of continous rendering to the UK, only to start using 5 years later, then look at the 100s, if not thousands of tons of potentially and most likely TSE tainted feed used in the USA for the last 2 decades, the likelyhood of this being a USA source of vCJD, in my opinion, is possible as well. In 2006 alone, the amount of ruminant protein still being fed to USA cattle is not only phenominal, but also very very disturbing. ...TSS



----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Monday, December 04, 2006 10:55 AM
Subject: Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East



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



Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the United States in a Patient from the Middle East



The Virginia Department of Health and the Centers for Disease Control and Prevention announce the recent confirmation of a vCJD case in a U.S. resident. This is the third vCJD case identified in a U.S. resident. This latest U.S. case occurred in a young adult who was born and raised in Saudi Arabia and has lived in the United States since late 2005. The patient occasionally stayed in the United States for up to 3 months at a time since 2001 and there was a shorter visit in 1989. In late November 2006, the Clinical Prion Research Team at the University of California San Francisco Memory and Aging Center confirmed the vCJD clinical diagnosis by pathologic study of adenoid and brain biopsy tissues. The two previously reported vCJD case-patients in U.S. residents were each born and raised in the United Kingdom (U.K.), where they were believed to have been infected by the agent responsible for their disease. There is strong scientific evidence that the agent causing vCJD is the same agent that causes bovine spongiform encephalopathy (BSE, commonly known as mad cow disease).



Variant CJD is a rare, degenerative, fatal brain disorder that emerged in the United Kingdom in the mid-1990s. Although experience with this new disease is limited, evidence to date indicates that there has never been a case transmitted from person-to-person except through blood transfusion. Instead, the disease is thought to result primarily from consumption of cattle products contaminated with the BSE agent. Although no cases of BSE in cattle have been reported in Saudi Arabia, potentially contaminated cattle products from the United Kingdom may have been exported to Saudi Arabia for many years during the large U.K. BSE outbreak.



The current case-patient has no history of receipt of blood, a past neurosurgical procedure, or residing in or visiting countries of Europe. Based on the patient's history, the occurrence of a previously reported Saudi case of vCJD attributed to likely consumption of BSE-contaminated cattle products in Saudi Arabia, and the expected greater than 7 year incubation period for food-related vCJD, this U.S. case-patient was most likely infected from contaminated cattle products consumed as a child when living in Saudi Arabia (1). The current patient has no history of donating blood and the public health investigation has identified no risk of transmission to U.S. residents from this patient.



As of November 2006, 200 vCJD patients were reported world-wide, including 164 patients identified in the United Kingdom, 21 in France, 4 in the Republic of Ireland, 3 in the United States (including the present case-patient), 2 in the Netherlands and 1 each in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain. Of the 200 reported vCJD patients, all except 10 of them (including the present case-patient) had resided either in the United Kingdom (170 cases) for over 6 months during the 1980-1996 period of the large UK BSE outbreak or alternatively in France (20 cases).



As reported in 2005 (1), the U.S. National Prion Disease Pathology Surveillance Center at Case Western Reserve University confirmed the diagnosis in the one previously identified case of vCJD in a Saudi resident. He was hospitalized in Saudi Arabia and his brain biopsy specimen was shipped to the United States for analysis. This earlier vCJD case-patient was believed to have contracted his fatal disease in Saudi Arabia (1). 1) Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P, Hunter S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis 2005, 11 (9):1351-1354. Date: November 29, 2006 Content source: National Center for Infectious Diseases




http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm


The Virginia Department of Health and the Centers for Disease Control and Prevention announce the recent confirmation of a case of variant Creutzfeldt-Jakob disease (vCJD) in a Virginia resident. There is no evidence to suggest that this case of vCJD was caused by anything the patient was exposed to while residing in the U.S. or that this situation represents a public health threat to any U.S. resident. For more information on vCJD, visit CDC’s Web site at

http://www.cdc.gov/ncidod/dvrd/vcjd/other/vCJD_112906.htm.


http://www.vdh.virginia.gov/news/Alerts/vCJD.htm


TSS


#################### https://lists.aegee.org/bse-l.html ####################


http://cjdtexas.blogspot.com/



[There is inconsistency in the number of vCJD cases attributed to the United States. The Virginia Department of Health/CDC report cited in the above list 3 cases -- 2 cases probably contracted in the U.K. and the young adult described above who was born and raised in Saudi Arabia. The EUROCJD data for October 2006 in part (2) above also lists 2 U.S. cases, both assumed to have been contracted in the U.K. The U.S. National Prion Disease Pathology Surveillance Center Data published on 8 Nov 2006 (part 3 above), on the other hand, list only a single U.S. case believed to have been contracted in the U.K. - Mod.CP]



http://www.promedmail.org/pls/otn/f?p=2400:1001:19224::::F2400_P1001_BACK_PAGE,F2400_P1001_ARCHIVE_NUMBER,F2400_P1001_USE_ARCHIVE:1001,20061205.3431,Y



http://vcjd.blogspot.com/



Thursday, February 4, 2010

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft Minutes of the 103rd Meeting held on 24th November 2009


http://seac992007.blogspot.com/2010/02/spongiform-encephalopathy-advisory.html


Monday, February 01, 2010


Import Alert 17-04 BSE CJD HIGH RISK TISSUES, Nutritional Supplements and Cosmetics


http://creutzfeldt-jakob-disease.blogspot.com/2010/02/import-alert-17-04-bse-cjd-high-risk.html


Monday, February 01, 2010


Import Alert 57-20 and 84-03 Human Dura Mater and risk factors there from due to Creutzfeldt Jakob Disease (CJD)


http://creutzfeldt-jakob-disease.blogspot.com/2010/02/import-alert-57-20-and-84-03-human-dura.html


Wednesday, February 3, 2010


Import Alert 62-07 Sygen Injectable (Bovine-Extracted GMI Monosialoganglioside) manufactured from bovine brain starting material


http://bseusa.blogspot.com/2010/02/import-alert-62-07-sygen-injectable.html


Wednesday, February 3, 2010


Import Alert 71-02 Detention Without Physical Examination Of Animal Feeds And Feed Ingredients That May Contain Ingredients Of Animal Origin Import Alert 71-02


http://madcowfeed.blogspot.com/2010/02/import-alert-71-02-detention-without.html


Wednesday, February 3, 2010


Import Alert 99-25 Detention Without Physical Examination of Animal Feed...BSE...and Not the Subject of a Valid USDA Import Permit Import Alert 99-25


http://madcowfeed.blogspot.com/2010/02/import-alert-99-25-detention-without.html


BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein


Emmanuel A. Asante, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew F. Hill, Sarah E. Lloyd, Jonathan D.F. Wadsworth, and John Collinge1 MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK 1Corresponding author e-mail: j.collinge@prion.ucl.ac.ukReceived August 1, 2002; Revised September 24, 2002; Accepted October 17, 2002.


http://www.ncbi.nlm.nih.gov/pmc/articles/PMC136957/?tool=pubmed



USA sporadic CJD cases rising ; 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.


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf




CJD RISING



SWITZERLAND CJD is a predominantly sporadic disorder but can also occur as a dominantly inherited or infective condition. Only one of the 26 most recent confirmed cases was identified as carrying a disease related mutation of the PRNP gene, none had identifiable iatrogenic exposure, and none resembled variant CJD. Thus 25 of the 26 cases appear to be sporadic cases. Sporadic CJD is distributed worldwide with a reported incidence of about one in a million per year. Raised awareness of the disease in recent years could account for an increase in reported cases of CJD, although neither an increase in the average age of patients nor more frequent recognition of CJD amongst residents of nursing homes (where dementing illness is prevalent and misdiagnosis might be expected) were seen in the Swiss cases. Moreover, improved ascertainment as an explanation for the observed increase would imply levels of under-reporting in countries other than Switzerland, which appear implausible. The authors of the Lancet report suggest that the rise in cases might be due to some form of unidentified iatrogenic transmission or to exposure to a zoonotic source of infection, though cases do not resemble variant Creutzfeldt-Jakob disease (vCJD). The ongoing surveillance of CJD in Switzerland and the rest of Europe is essential to monitor the situation to see if this rise is sustained in Switzerland, and if a similar rise occurs in other countries (see http://www.eurocjd.ed.ac.uk/).


http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1921



Prion data suggest BSE link to sporadic CJD Declan Butler Predicting the number of cases of Creutzfeldt–Jakob disease (CJD) in people as a result of transmission of bovine spongiform encephalopathy (BSE) has just got more difficult.Whereas it was thought that BSE only caused a new form of the disease called variant CJD (vCJD), a study in mice from a team led by John Collinge at University College London suggests that it may also cause a disease indistinguishable from the commonest form of classical, or 'sporadic', CJD (E.


http://www.nature.com/nature/journal/v420/n6915/full/420450a.html



IF we look at sporadic incidence of CJD in UK from 1993 to 2003, the incidence rose from 37 in 1993 to 77 in 2003. THIS seems to show an increase to me? I do not understand the statement ; However, in the period following the first published description of vCJD in 1996, there was no increasing trend in the reported annual number of U.K. sporadic CJD deaths (52). IF we go further and look at some of the other documented BSE countries, you will the increase of sporadic CJD there as well ;

Canada from 2 to 25

France from 35 to 108

Germany 21+ to 96

Italy 27 to 76


http://www.eurocjd.ed.ac.uk/sporadic.htm


Switzerland sporadic CJD ; Swiss rise in CJD raises concerns over possible BSE link [LONDON] THE LANCET Plaque attack: Swiss patients have spongiform patterns in the brain typical of sporadic CJD. The number of people dying from Creutzfeldt-Jakob disease (CJD) has risen sharply in Switzerland -- sparking fears of a possible link with bovine spongiform encephalopathy (BSE). BSE is thought to be the cause of a distinctive form of the brain-wasting disease known as variant CJD. The Swiss cases, in contrast, are standard 'sporadic' CJD. Each year between 1997 and 2000, no more than 11 Swiss people developed CJD. But 19 cases were reported in 2001, and seven were recorded in the first quarter of this year. This is some four times higher than the incidence elsewhere, reports a team led by Adriano Aguzzi of the University Hospital Zurich (M. Glatzel et al. Lancet 360, 139-141; 2002). The increase could be a mere statistical blip, or it may be due to increased awareness of the disease leading to more diagnoses. More disturbing is the possibility that the cases are linked to the consumption of BSE-infected meat products -- which would mean that the BSE agent can cause two distinct forms of CJD. Possible links between the Swiss CJD cases and BSE will now be explored by strain-typing experiments in which the disease is transmitted to mice. These tests will take at least a year to complete. "It's the best way to establish or exclude any suspected link," says Moira Bruce of the UK Institute for Animal Health's Neuropathogenesis Unit in Edinburgh.


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


Experiences in England and Switzerland -- two countries that discovered mad cow disease in their cattle -- have heightened concerns about the possibility some cases of sporadic CJD are due to consuming mad-cow-tainted beef. Both countries have reported increases in sporadic CJD since mad cow was first detected in British herds in 1986. Switzerland discovered last year its CJD rate was twice that of any other country in the world. Switzerland had been seeing about eight to 11 cases per year from 1997 to 2000. Then the incidence more than doubled, to 19 cases in 2001 and 18 cases in 2002.


http://www.upi.com/view.cfm?StoryID=20030721-102924-4786r


Mouse model sheds new light on human prion disease

snip...

Professor John Collinge said We are not saying that all or even most cases of sporadic CJD are as a result of BSE exposure, but some more recent cases may be the incidence of sporadic CJD has shown an upward trend in the UK over the last decade. While most of this apparent increase may be because doctors are now more aware of CJD and better at diagnosing it, serious consideration should be given to a proportion of this rise being BSE-related. Switzerland, which has had a substantial BSE epidemic, has noted a sharp recent increase in sporadic CJD.


snip...


http://www.mrc.ac.uk/txt/index/public-interest/public-news-4/public-news_archive/public-news-archive_nov_dec_02/public-bse_and_sporadic_cjd.htm


Monday, May 19, 2008

SPORADIC CJD IN FARMERS, FARMERS WIVES, FROM FARMS WITH BSE HERD AND ABATTOIRS


http://bseinquiry.blogspot.com/


Sunday, August 10, 2008


A New Prionopathy OR more of the same old BSe and sporadic CJD


http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html


Epidemiologic implications of Creutzfeldt-Jakob disease in a 19 year-old girl

Journal European Journal of Epidemiology Publisher Springer Netherlands ISSN 0393-2990 (Print) 1573-7284 (Online) Issue Volume 1, Number 1 / March, 1985 P. Brown1, F. Cathala2, R. Labauge3, M. Pages3, J. C. Alary3 and H. Baron (1) Laboratory of CNS Studies, NINCDS, National Institutes of Health, 20205 Bethesda, Maryland, USA (2) Laboratoire de Neurovirologie, Hôpital de la Salpêtrière, Paris, France (3) Départment de Neurologie, Centre Hospitalier Universitaire, Montpellier, France


Abstract


A histopathologically-verified, clinically typical case of Creutzfeldt-Jakob disease (CJD) is described in a 19 year-old girl. Only 3 previous cases of CJD have been reported in adolescents, and one of these was iatrogenically transmitted, while another was familial. Epidemiologic investigation of the present case excluded a familial component, and provided no evidence for iatrogenic or natural case-to-case transmission, or of other environmental sources of viral contamination. Young patients such as this one serve to emphasize the obscurity that still sourrounds the epidemiology of CJD, and invite serious reconsideration of the possibilities of transmission by undetected virus carriers, or of the agent as a natural resident of human cells, replication of which might be triggered by non-infective (e.g., traumatic or mutational) environmental events. Key words Creutzfeldt-Jakob disease - Epidemiology P. Brown1, F. Cathala2, R. Labauge3, M. Pages3, J. C. Alary3 and H. Baron (1) Laboratory of CNS Studies, NINCDS, National Institutes of Health, 20205 Bethesda, Maryland, USA (2) Laboratoire de Neurovirologie, Hôpital de la Salpêtrière, Paris, France (3) Départment de Neurologie, Centre Hospitalier Universitaire, Montpellier, France Abstract A histopathologically-verified, clinically typical case of Creutzfeldt-Jakob disease (CJD) is described in a 19 year-old girl. Only 3 previous cases of CJD have been reported in adolescents, and one of these was iatrogenically transmitted, while another was familial. Epidemiologic investigation of the present case excluded a familial component, and provided no evidence for iatrogenic or natural case-to-case transmission, or of other environmental sources of viral contamination. Young patients such as this one serve to emphasize the obscurity that still sourrounds the epidemiology of CJD, and invite serious reconsideration of the possibilities of transmission by undetected virus carriers, or of the agent as a natural resident of human cells, replication of which might be triggered by non-infective (e.g., traumatic or mutational) environmental events. Key words Creutzfeldt-Jakob disease - Epidemiology


http://www.springerlink.com/content/j344470112792q50/


http://www.springerlink.com/content/j344470112792q50/fulltext.pdf

?page=1 2.



Sporadic CJD normally occurs in people in their 50s and 60s although it can occur more rarely in younger age groups. Until this year the youngest case of sporadic CJD in the UK had been in a 34 year old. Other countries, howver, have reported sporadic CJD in teenagers. Those we know about are;

* in the USA, a 16 year old in 1978;

* in France, a 19 year old in 1982;

* in Canada, a 14 year old of UK origin in 1988;

* in Poland cases in people aged 19, 23, and 27 were identified in a retrospective study (published 1991), having been originally misdiagnosed with a viral encephalitis;

* Creutzfeldt's first patient in 1920 was aged 23. full text ;


http://collections.europarchive.org/tna/20081106132604/http://www.bseinquiry.gov.uk/files/yb/1995/10/27013001.PDF


J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007. doi:10.1136/jnnp.2006.104570 © 2007 by BMJ Publishing Group Ltd Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4 and R G Will 4* 1 NationalCJD Surveillance Unit, United Kingdom 2 Neuropathogenesis Unit, United Kingdom 3 Walton Centre for Neurology and Neurosurgery, United Kingdom 4 National CJD Surveillance Unit, United Kingdom * To whom correspondence should be addressed. E-mail: r.g.will@ed.ac.uk. Accepted 15 April 2007


Abstract Background:


Sporadic Creutzfeldt-Jakob disease (CJD) is a condition predominantly affecting older age groups, with cases aged less than 45 years rare and an age at onset or death of less than 20 years exceptional. Methods: Data from the systematic study of sporadic CJD in the UK are available from 1970 onwards. Clinical and pathological data are reviewed in order to identify atypical cases, including those at the extremes of the age range of sporadic CJD. Detailed analysis of atypical cases is undertaken and in selected cases laboratory transmission studies are carried out in order to provide information on the characteristics of the infectious agent. Results: In the UK two cases of sporadic CJD in adolescents have been identified, dying aged 16 and 20 years. The first case predated the epidemic of bovine spongiform encephalopathy and the characteristics of the second case, including laboratory transmission studies, are consistent with a diagnosis of sporadic rather than variant CJD. Conclusion: The cases in this report indicate that sporadic CJD can develop at a very young age, that variant CJD is not the only form of CJD occurring in this age group and that neuropathological examination is essential to accurate diagnosis of human prion disease.

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1


http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html


http://stanford.wellsphere.com/cjd-article/a-novel-human-disease-with-abnormal-prion-protein-sensitive-to-protease-prionopathy/641206


WHERE IS IT WRITTEN, that scrapie and or atypical scrapie, CANNOT OR WILL NOT, infect humans ???


or rather, where has it been proven of this myth ???


fact is, there is more scientific evidence to support transmission of scrapie to humans, than there is, showing that it will not. ...


Monday, December 14, 2009


Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types


http://nor-98.blogspot.com/2009/12/similarities-between-forms-of-sheep.html


Thursday, January 07, 2010


Scrapie and Nor-98 Scrapie November 2009 Monthly Report Fiscal Year 2010 and FISCAL YEAR 2008


http://scrapie-usa.blogspot.com/2010/01/scrapie-and-nor-98-scrapie-november.html


TRANSMISSION STUDIES DO NOT LIE, only the politicians and the industry that put these politicians in office do ;


1: J Infect Dis 1980 Aug;142(2):205-8


Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.


Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.


Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation. snip... The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease. PMID: 6997404


http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract



12/10/76



AGRICULTURAL RESEARCH COUNCIL REPORT OF THE ADVISORY COMMITTE ON SCRAPIE


Office Note CHAIRMAN: PROFESSOR PETER WILDY


snip...


A The Present Position with respect to Scrapie A] The Problem


Scrapie is a natural disease of sheep and goats. It is a slow and inexorably progressive degenerative disorder of the nervous system and it ia fatal. It is enzootic in the United Kingdom but not in all countries.


The field problem has been reviewed by a MAFF working group (ARC 35/77). It is difficult to assess the incidence in Britain for a variety of reasons but the disease causes serious financial loss; it is estimated that it cost Swaledale breeders alone $l.7 M during the five years 1971-1975. A further inestimable loss arises from the closure of certain export markets, in particular those of the United States, to British sheep.


It is clear that scrapie in sheep is important commercially and for that reason alone effective measures to control it should be devised as quickly as possible.


Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"


Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously.


snip...


76/10.12/4.6


http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf



Nature. 1972 Mar 10;236(5341):73-4.


Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).


Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0
Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland


SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).


http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html



Epidemiology of Scrapie in the United States 1977


http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf


http://web.archive.org/web/20030513212324/http://www.bseinquiry.gov.uk/files/mb/m08b/tab64.pdf


HOUND STUDY AS implied in the Inset 25 we must not _ASSUME_ that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.


snip...


http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf


http://web.archive.org/web/20010305222642/www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf



2005 DEFRA Department for Environment, Food & Rural Affairs

Area 307, London, SW1P 4PQ Telephone: 0207 904 6000 Direct line: 0207 904 6287 E-mail: h.mcdonagh.defra.gsi.gov.uk


GTN: FAX:


Mr T S Singeltary P.O. Box 42 Bacliff Texas USA 77518


21 November 2001


Dear Mr Singeltary


TSE IN HOUNDS


Thank you for e-mail regarding the hounds survey. I am sorry for the long delay in responding.


As you note, the hound survey remains unpublished. However the Spongiform Encephalopathy Advisory Committee (SEAC), the UK Government's independent Advisory Committee on all aspects related to BSE-like disease, gave the hound study detailed consideration at their meeting in January 1994. As a summary of this meeting published in the BSE inquiry noted, the Committee were clearly concerned about the work that had been carried out, concluding that there had clearly been problems with it, particularly the control on the histology, and that it was more or less inconclusive. However was agreed that there should be a re-evaluation of the pathological material in the study.


Later, at their meeting in June 95, The Committee re-evaluated the hound study to see if any useful results could be gained from it. The Chairman concluded that there were varying opinions within the Committee on further work. It did not suggest any further transmission studies and thought that the lack of clinical data was a major weakness.


Overall, it is clear that SEAC had major concerns about the survey as conducted. As a result it is likely that the authors felt that it would not stand up to r~eer review and hence it was never published. As noted above, and in the detailed minutes of the SEAC meeting in June 95, SEAC considered whether additional work should be performed to examine dogs for evidence of TSE infection. Although the Committee had mixed views about the merits of conducting further work, the Chairman noted that when the Southwood Committee made their recommendation to complete an assessment of possible spongiform disease in dogs, no TSEs had been identified in other species and hence dogs were perceived as a high risk population and worthy of study. However subsequent to the original recommendation, made in 1990, a number of other species had been identified with TSE ( e.g. cats) so a study in hounds was less critical. For more details see-


http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf


http://web.archive.org/web/20030327015011/http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf



As this study remains unpublished, my understanding is that the ownership of the data essentially remains with the original researchers. Thus unfortunately, I am unable to help with your request to supply information on the hound survey directly. My only suggestion is that you contact one of the researchers originally involved in the project, such as Gerald Wells. He can be contacted at the following address.


Dr Gerald Wells, Veterinary Laboratories Agency, New Haw, Addlestone, Surrey, KT 15 3NB, UK


You may also wish to be aware that since November 1994 all suspected cases of spongiform encephalopathy in animals and poultry were made notifiable. Hence since that date there has been a requirement for vets to report any suspect SE in dogs for further investigation. To date there has never been positive identification of a TSE in a dog.


I hope this is helpful

Yours sincerely 4


HUGH MCDONAGH BSE CORRESPONDENCE SECTION


IN CONFIDENCE


CONCEPT NOT FOR FURTHER STUDY OF MATERIAL OBTAINED IN A SURVEY OF HOUNDS FOR EVIDENCE OF A SCRAPIE-LIKE SPONGIFORM ENCEPHALOPATHY (SE)


snip...


b) Fibrillar material closely similar to SAF, found in BSE/Scrapie, was observed in 19 (4.3%) cases, all of which were hounds > 7 years of age. 14/19 of these suspected SAF results correlated with cases in the unresolveable histopathological category.


snip...

The following proposals address the hypothesis that the hound survey observations represent a PrP related or scrapie-like disease of dogs in which the pathological response, and possible the spread of infectivity, is neuroanatomically localized. By inference this could also mean that the disorder is clinically silent and non-progressive.


http://www.bseinquiry.gov.uk/files/yb/1995/02/09001001.pdf



http://web.archive.org/web/20030907001733/www.bseinquiry.gov.uk/files/yb/1995/02/09001001.pdf



http://www.humanitarian.net/law/biodefense/bse_12004.html



http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html



2008 - 2010

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html



CJD USA RISING, with UNKNOWN PHENOTYPE ;

5 Includes 41 cases in which the diagnosis is pending, and 17 inconclusive cases; 6 Includes 46 cases with type determination pending in which the diagnosis of vCJD has been excluded.

http://www.cjdsurveillance.com/pdf/case-table.pdf



Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***


http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html



my comments to PLosone here ;


http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd




14th International Congress on Infectious Diseases H-type and L-type Atypical BSE January 2010 (special pre-congress edition)

18.173 page 189

Experimental Challenge of Cattle with H-type and L-type Atypical BSE

A. Buschmann1, U. Ziegler1, M. Keller1, R. Rogers2, B. Hills3, M.H. Groschup1. 1Friedrich-Loeffler-Institut, Greifswald-Insel Riems, Germany, 2Health Canada, Bureau of Microbial Hazards, Health Products & Food Branch, Ottawa, Canada, 3Health Canada, Transmissible Spongiform Encephalopathy Secretariat, Ottawa, Canada

Background: After the detection of two novel BSE forms designated H-type and L-type atypical BSE the question of the pathogenesis and the agent distribution of these two types in cattle was fully open. From initial studies of the brain pathology, it was already known that the anatomical distribution of L-type BSE differs from that of the classical type where the obex region in the brainstem always displays the highest PrPSc concentrations. In contrast in L-type BSE cases, the thalamus and frontal cortex regions showed the highest levels of the pathological prion protein, while the obex region was only weakly involved.

Methods:We performed intracranial inoculations of cattle (five and six per group) using 10%brainstemhomogenates of the two German H- and L-type atypical BSE isolates. The animals were inoculated under narcosis and then kept in a free-ranging stable under appropriate biosafety conditions.At least one animal per group was killed and sectioned in the preclinical stage and the remaining animals were kept until they developed clinical symptoms. The animals were examined for behavioural changes every four weeks throughout the experiment following a protocol that had been established during earlier BSE pathogenesis studies with classical BSE.

Results and Discussion: All animals of both groups developed clinical symptoms and had to be euthanized within 16 months. The clinical picture differed from that of classical BSE, as the earliest signs of illness were loss of body weight and depression. However, the animals later developed hind limb ataxia and hyperesthesia predominantly and the head. Analysis of brain samples from these animals confirmed the BSE infection and the atypical Western blot profile was maintained in all animals. Samples from these animals are now being examined in order to be able to describe the pathogenesis and agent distribution for these novel BSE types. Conclusions: A pilot study using a commercially avaialble BSE rapid test ELISA revealed an essential restriction of PrPSc to the central nervous system for both atypical BSE forms. A much more detailed analysis for PrPSc and infectivity is still ongoing.



http://www.isid.org/14th_icid/


http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf


http://www.isid.org/publications/ICID_Archive.shtml


From: xxxx To: Terry Singeltary Sent: Saturday, December 05, 2009 9:09 AM Subject: 14th ICID - abstract accepted for 'International Scientific Exchange'

Your preliminary abstract number: 670

Dear Mr. Singeltary,

On behalf of the Scientific Committee, I am pleased to inform you that your abstract

'Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009'

WAS accepted for inclusion in the INTERNATIONAL SCIENTIFIC EXCHANGE (ISE) section of the 14th International Congress on Infectious Diseases. Accordingly, your abstract will be included in the "Intl. Scientific Exchange abstract CD-rom" of the Congress which will be distributed to all participants.

Abstracts accepted for INTERNATIONAL SCIENTIFIC EXCHANGE are NOT PRESENTED in the oral OR poster sessions.

Your abstract below was accepted for: INTERNATIONAL SCIENTIFIC EXCHANGE

#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Author: T. Singeltary; Bacliff, TX/US

Topic: Emerging Infectious Diseases Preferred type of presentation: International Scientific Exchange

This abstract has been ACCEPTED.

#0670: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Authors: T. Singeltary; Bacliff, TX/US

Title: Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009

Body: Background

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and feed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods

12 years independent research of available data

Results

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries.

I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

Keywords: Transmissible Spongiform Encephalopathy Creutzfeldt Jakob Disease Prion


http://www.isid.org/14th_icid/


http://www.isid.org/publications/ICID_Archive.shtml


http://ww2.isid.org/Downloads/IMED2009_AbstrAuth.pdf


Monday, October 19, 2009

Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009

snip...

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

I look forward to further transmission studies, and a true ENHANCED BSE/atypical BSE surveillance program put forth testing all cattle for human and animal consumption for 5 years. a surveillance program that uses the most sensitive TSE testing, and has the personnel that knows how to use them, and can be trusted. I look forward to a stringent mad cow feed ban being put forth, and then strictly enforced. we need a forced, not voluntary feed ban, an enhanced feed ban at that, especially excluding blood. we need some sort of animal traceability. no more excuses about privacy. if somebody is putting out a product that is killing folks and or has the potential to kill you, then everybody needs to know who they are, and where that product came from. same with hospitals, i think medical incidents in all states should be recorded, and made public, when it comes to something like a potential accidental transmission exposure event. so if someone is out there looking at a place to go have surgery done, if you have several hospitals having these type 'accidental exposure events', than you can go some place else. it only makes sense. somewhere along the road, the consumer lost control, and just had to take whatever they were given, and then charged these astronomical prices. some where along the line the consumer just lost interest, especially on a long incubating disease such as mad cow disease i.e. Transmissible Spongiform Encephalopathy. like i said before, there is much more to the mad cow story than bovines and eating a hamburger, we must start focusing on all TSE in all species. ...TSS

http://bse-atypical.blogspot.com/2009/10/atypical-bse-bse-and-other-human-and.html


for those interested, please see full text ;


Friday, January 29, 2010 14th International Congress on Infectious Diseases H-type and L-type Atypical BSE January 2010 (special pre-congress edition)



http://bse-atypical.blogspot.com/2010/01/14th-international-congress-on.html



Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION


http://cjdquestionnaire.blogspot.com/



Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009


http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html



Sunday, January 17, 2010

BSE USA feed inspection violations 01/01/2009 to 01/17/2010 FDA BSE/Ruminant Feed Inspections Firms Inventory Report


http://madcowfeed.blogspot.com/2010/01/bse-usa-feed-inspection-violations.html



Tuesday, January 19, 2010

CVM's OR Develops New PCR-Based Method for Testing Animal Feed


http://madcowfeed.blogspot.com/2010/01/cvms-or-develops-new-pcr-based-method.html



Tuesday, January 26, 2010

Establishing a Fully Integrated National Food Safety System with Strengthened Inspection, Laboratory and Response Capacity Draft 09/24/09


http://fdafailedus.blogspot.com/2010/01/establishing-fully-integrated-national.html




WHO WILL WATCH THE CHILDREN FOR CJD OVER THE NEXT 5 + DECADES ???


For 4+ years, dead stock downer cows, the most high risk cattle for mad cow type disease, and other deadly pathogens, were fed to our kids, via the NSLP and the USDA all across our Nation.

Do you actually believe that the USDA et al jumped in on the law suit against Westland/Hallmark, at the time the largest beef recall in USA history, just because a few animals were abused on a video, or to cover their ass, for letting our children, from school district to school district, from state to state, be fed dead stock downer cows.


>>>In the papers, the government alleges the meatpacking plant slaughtered and processed downer cows for nearly four years — from January 2004 to September 2007 — at the average rate of one every six weeks... <<<



http://downercattle.blogspot.com/2009/09/suit-meatpacker-used-downer-cows-for-4.html



Do you actually believe all these schools recalled this meat because of a few cattle being abused, see list ; FNS All Regions Affected School Food Authorities By State United States Department of Agriculture Food and Nutrition Service National School Lunch Program March 24, 2008 School Food Authorities Affected by Hallmark/Westland Meat Packing Co. Beef Recall February 2006 - February 2008


http://www.fns.usda.gov/fns/safety/Hallmark-Westland_byState.pdf


IF url does not work above, go to this link to find out if any of your children and their school were part of this recall ; go to this site ;


http://www.fns.usda.gov/fns/


left hand corner search ; Hallmark/Westland Meat Packing Co. Beef Recall your should get this ;


http://65.216.150.153/texis/search?pr=FNS


1 through 1 of 1 matching documents, best matches first. sort by date 1: Hallmark - Westland SFA Reporting by State - 3-24-2008.xls Lunch Program March 24, 2008 School Food Authorities Affected by Hallmark/Westland Meat Packing Co. Beef Recall February 2006 - February 2008 The U.S. Department of Agriculture ...


http://www.fns.usda.gov/...ety/Hallmark-Westland_byState.pdf



PLEASE SEE ALSO ;


Members of The HSUS are also concerned about the meat products provided to their children through the National School Lunch Program. More than 31 million school children receive lunches through the program each school day. To assist states in providing healthful, low-cost or free meals, USDA provides states with various commodities including ground beef. As evidenced by the HallmarkNVestland investigation and recall, the potential for downed animals to make their way into the National School Lunch Program is neither speculative nor hypothetical.


http://biotech.law.lsu.edu/cases/FDA/hsus-v-schafer-usda-complaint.pdf



some history on dead stock downers in the USA ; Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. snip... The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...


http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf



TME


http://transmissible-mink-encephalopathy.blogspot.com/2009/07/transmissible-mink-encephalopathy.html



http://transmissible-mink-encephalopathy.blogspot.com/2007/12/phenotypic-similarity-of-transmissible.html



http://transmissible-mink-encephalopathy.blogspot.com/


CWD


http://chronic-wasting-disease.blogspot.com/




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

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