vCJD USA THIRD CASE DOCUMENTED

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

2012-01-30T19:49:00.000-08:00

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...

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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:80/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

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

2010-02-05T13:45:00.000-08:00

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

vCJD USA THIRD CASE DOCUMENTED

2006-12-06T08:05:00.000-08:00

Wednesday, December 06, 2006 vCJD USA THIRD CASE DOCUMENTED

CJD (NEW VAR.) UPDATE 2006 (12) ******************************* A ProMED-mail post

ProMED-mail is a program of the International Society for Infectious Diseases

[The definition of the designations deaths, definite cases, probable vCJD cases, and the case definitions can be found by accessing the Department of Health website, or by reference to a previous ProMED-mail post in this thread (for example, CJD (new var.) - UK: update March 2002 20020305.3693).

Data on vCJD cases from other parts of the world are now included in these updates whenever available.

Also, data on other forms of CJD (sporadic, iatrogenic, familial and GSS) are now included when they have some relevance to the incidence and etiology of vCJD. - Mod.CP]

In this update:

[1] UK: Department of Health monthly vCJD and CJD statistics, Mon 4 Dec 2006 [2] EUROCJD data as of 31 October 2006 [3] National (US) Prion Disease Pathology Surveillance Center Data: 8 Nov 2006 [4] The Netherlands: 2nd vCJD death [5] USA: 3rd vCJD case (ex Saudi Arabia)

****** [1] UK: vCJD and CJD statistics Date: Mon 4 Dec 2006 From: ProMED-mail Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease Statistics, Mon 4 Dec 2006 [edited]

The Department of Health is today [Mon 4 Dec 2006] issuing the latest information about the numbers of known cases of Creutzfeldt-Jakob disease. This includes cases of variant Creutzfeldt-Jakob disease [abbreviated in ProMED-mail as CJD (new var.) or vCJD], the form of the disease thought to be linked to BSE (bovine spongiform encephalopathy).

Definite and probable CJD cases in the UK, as at Fri 1 Dec 2006

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

Summary of vCJD cases - deaths ------------------------------

Deaths from definite vCJD (confirmed): 112 Deaths from probable vCJD (without neuropathological confirmation): 46 Deaths from probable vCJD (neuropathological confirmation pending): 0 Number of deaths from definite or probable vCJD (as above): 158

Summary of vCJD cases - alive -----------------------------

Number of probable vCJD cases still alive: 6

Total ----- Number of definite or probable vCJD (dead and alive): 164

(The next table will be published on Mon 8 Jan 2007).

Since the previous monthly statistics were released on Mon 6 Nov 2006, the total number of deaths from definite vCJD remains unchanged and stands at 158. The overall total number of definite or probable vCJD cases (dead and alive) remains unchanged at 164.

These data are consistent with the view that the vCJD outbreak in the UK is in decline. The peak number of deaths was 28 in the year 2000, followed by 20 in 2001, 17 in 2002, 18 in 2003, 9 in 2004, and 5 in 2005. The number of deaths due to definite or probable vCJD in the UK during the first 11 months of 2006 remains at 5.

Totals for all types of CJD cases in the UK in 2005 and 2006

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

As of Fri 1 Dec 2006, in the UK in the year 2005, there were 123 referrals of suspected CJD, and there were 65 deaths from sporadic CJD, 7 from familial CJD, 3 from iatrogenic CJD, 6 GSS (Gerstmann-Straussler-Scheinker) syndrome cases, and 5 deaths from vCJD.

The corresponding figures so far for the 1st 11 months of 2006 are: 98 referrals, 53 deaths from sporadic CJD, 5 from vCJD, 5 from familial CJD, 3 from GSS and one from iatrogenic CJD.

During the period from 1995, when vCJD was first diagnosed, up to the present there have been 953 deaths from all forms of CJD including the 158 deaths attributable to definite or probable vCJD.

[These data are accessible via ]

-- ProMED-mail

****** [2] EU - EUROCJD Data Date: Tue 31 Oct 2006 From: ProMED-mail Source: EUROCJD 31 Oct 2006 [edited]

The European And Allied Countries Collaborative Study Group of CJD (EUCJD)

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

This web-site includes information from 2 projects funded by the European Commission. The EUROCJD project started in 1993 and compares data from national registries in Australia, Austria, Canada, France, Germany, Italy, the Netherlands, Slovakia, Spain, Switzerland and the UK. The NEUROCJD project started in 1998 after the European Union Council recommended that epidemiological surveillance of CJD should be extended to all member states. The member states involved in this project are Belgium, Denmark, Finland, Greece, Iceland, Ireland, Israel, Norway and Portugal. Both projects are coordinated from the U.K. National CJD Surveillance Unit based in Edinburgh.

Current data as at November 2006*

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

Country / Total No. of Primary cases (No. alive) / Cumulative residence in UK (>6 months) / Secondary transmission by blood transfusion

United Kingdom / 162 (6) / 164 / 2 (0) France / 21 (2) / 1 / 0 Republic of Ireland / 4 (1) / 2 / 0 Italy / 1 (0) / 0 / 0 USA / 3 (1*) / 2 / 0 / 0 Canada / 1 (0) / 1 / 0 Saudi Arabia / 1 (1) / 0 / 0 Japan / 1** (0) / 0 / 0 Netherlands*** / 2 (0) / 0 / 0 Portugal / 1 (1) / 0 / 0 Spain / 1 (0) / 0 / 0

Total / 197 (11) / - / 2

Footnotes ----------

* One case probably contracted in Saudi Arabia [see part (5) below], and 2 in U.K. ** Residence in the UK for 24 days *** Data for the Netherlands case (see part [4] below) was omitted in error from the corresponding table for October posted on 31 Oct 2006

-- ProMED-mail

****** [3] USA - CJD National Prion Disease Surveillance Data Date: Wed 8 Nov 2006 From: Terry S. Singeltary Sr Source: CJD Surveillance, National Prion Disease Pathology Surveillance Center, 8 Nov 2006 [edited]

Resources/Case Report ---------------------

Year / Referral / Total / Sporadic / Familial / Iatrogenic / vCJD

1997 / 104 / 60 / 54 / 6 / 0 / 0 1998 / 94 / 51 / 44 / 6 / 1 / 0 1999 / 114 / 74 / 65 / 9 / 0 / 0 2000 / 169 / 111 / 97 / 12 / 2 / 0 2001 / 2247 / 154 / 138 / 16 / 0 / 0 2002 / 265 / 151 / 127 / 22 / 1 / 0 2003 / 284 / 191 / 142 / 45 / 1 / 0 2004 / 360 / 202 / 167 / 21 / 0 / 1* 2005 / 357 / 201 / 144 / 39 / 0 / 0 2006 / 208 / 86 / 46 / 8 / 0 /1**

Total / 2202 / 1281 / 1024 / 184 / 5 / 2*

* Acquired in the United Kingdom ** Acquired in Saudi Arabia [see part (5) below]

[The gradual increase in sporadic and other forms of CJD in the USA throughout this period may merely reflect improvements in surveillance and diagnosis rather than a real increase in incidence. For example, according to the tables above, there were approximately 1 per million cases of sporadic CJD in the USA during 2005, compared with 2 cases per million in the UK during the same period. This difference could be indicative of a real difference in incidence or a difference in efficiency of diagnosis. - Mod.CP]

-- Terry S. Singeltary Sr

****** [4] The Netherlands - 2nd death Date: Thu 16 Nov 2006 From: Mary Marshall Source: iol.co.za Medical, Reuters report, Thu 16 Nov 2006 [edited]

A 2nd Dutch person has died from the human variant of mad cow disease following the death of a woman last year, health authorities said on Thu 16 Nov 2006. The Dutch Institute for Health and Environment (RIVM) give no details about the victim, but Dutch television stations said he was a 16-year-old boy.

A spokeswoman for the RIVM said the victim died from the brain-wasting [variant] Creutzfeldt-Jakob Disease (vCJD), the human form of Bovine Spongiform Encephalopathy (BSE) about 2 weeks ago. She did not explain why the RIVM had waited 2 weeks to confirm the death, which was reported in Dutch media on Thursday. The RIVM diagnosed the person with the human variant of mad cow [disease] in June and said at the time that the patient had most probably become infected by eating contaminated meat products. It was the 2nd Dutch death from the human variant of mad cow disease after a 26-year-old woman died in May 2005.

The disease is fatal and incurable. It is thought to be caused by eating food tainted with material from cattle with BSE, a progressive neurological disorder. More than 150 cases of vCJD have been reported around the world, mostly in Britain, but also in France, Ireland, Italy, Japan, Canada and the United States. Mad cow disease first emerged in Britain in the 1980s and has forced the destruction of millions of cattle.

-- ProMED-mail

****** [5] Date: Wed 29 Nov 2006 From: Terry S. Singeltary Sr. Source: CDC, National Center for Infectious Diseases, Wed 29 Nov 2006 [edited]

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 announced the recent confirmation of a vCJD case in a U.S. resident. This is the 3rd 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 2 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 scient! ific 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 neurological 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).

Reference --------- (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.

-- Terry S. Singeltary Sr.

[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]

[see also: CJD (new var.) update 2006 (11) 20061106.3190 CJD (new var.) update 2006 (10) 20061002.2820 CJD (new var.) update 2006 (09) 20060904.2519 CJD (new var.) update 2006 (08) 20060807.2207 CJD (new var.) update 2006 (07) 20060703.1831 CJD (new var.) - Netherlands: 2nd case 20060623.1741 CJD (new var.) update 2006 (06) 20060605.1566 CJD (new var.) update 2006 (05) 20060508.1332 CJD (new var.) update 2006 (04) 20060404.1005 CJD (new var.) update 2006 (03) 20060306.0728 CJD (new var.) - UK: 3rd transfusion-related case 20060209.0432 CJD (new var.) update 2006 (02) 20060206.0386 CJD (new var.) update 2006 (01) 20060111.0101 CJD (new var.) update 2006 20060111.0101 2005 ---- CJD (new var.) update 2005 (12) 20051209.3547 CJD (new var.) update 2005 (11) 20051108.3270 CJD (new var.) update 2005 (10) 20051006.2916 CJD (new var.) update 2005 (09) 20050905.2627 CJD (new var.) update 2005 (08) 20050801.2237 CJD (new var.) update 2005 (07) 20050703.1889 CJD (new var.) update 2005 (06) 20050607.1584 CJD (new var.) update 2005 (05) 20050505.1243 CJD (new var.) update 2005 (04) 20050405.0982 CJD (new var.) update 2005 (03) 20050308.0687 CJD (new var.) update 2005 (02) 20050211.0467 CJD (new var.) - UK: update 2005 (01) 20050111.0095 2004 ---- CJD, genetic susceptibility 20041112.3064 CJD (new var.) - UK: update 2004 (14) 20041206.3242 CJD (new var.) - UK: update 2004 (13) 20041103.2977 CJD (new var.) - UK: update 2004 (12) 20041023.2871 CJD (new var.) - UK: update 2004 (11) 20041008.2758 CJD (new var.) - UK: update 2004 (10) 20040909.2518 CJD (new var.) - UK: update 2004 (09) 20040809.2199 CJD (new var.) - UK: update 2004 (08) 20040806.2150 CJD (new var.) - UK: update 2004 (07) 20040706.1807 CJD (new var.) - UK: update 2004 (06) 20040608.1535 CJD (new var.) - UK: update 2004 (05) 20040510.1262 CJD (new var.) - UK: update 2004 (04) 20040406.0937 CJD (new var.) - UK: update 2004 (03) 20040314.0713 CJD (new var.) - UK: update 2004 (02) 20040202.0400 CJD (new var.) - UK: update 2004 (01) 20040106.0064 CJD (new var.) - France: 8th case 20041022.2864 CJD (new var.) - France: 9th case 20041123.3138 CJD (new var.), blood supply - UK 20040318.0758 CJD (new var.), carrier frequency study - UK 20040521.1365 2003 ---- CJD (new var.) - UK: update 2003 (13) 20031216.3072 CJD (new var.) - UK: update 2003 (01) 20030108.0057 2002 ---- CJD (new var.) - UK: update Dec 2002 20021207.5997 CJD (new var.) - UK: update Jan 2002 20020111.3223 2001 ---- CJD (new var.), incidence & trends - UK (02) 20011124.2875 CJD (new var.), incidence & trends - UK 20011115.2816 CJD (new var.) - UK: reassessment 20011029.2671 CJD (new var.) - UK: update Oct 2001 20011005.2419 CJD (new var.) - UK: regional variation (02) 20010907.2145 CJD (new var.) - UK: update Sep 2001 20010906.2134 CJD (new var.) - UK: update Aug 2001 20010808.1872 CJD (new var.) - UK: 9th Annual Report 20010628.1231 CJD (new var.) - UK: update June 2001 20010622.1188 CJD (new var.) - UK: update 3 Jan 2001 20010104.0025] ................cp/pg/mpp

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Volume 12, Number 12–December 2006

PERSPECTIVE

On the Question of Sporadic

or Atypical Bovine SpongiformEncephalopathy and

Creutzfeldt-Jakob Disease

Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§

Strategies to investigate the possible existence of sporadic

bovine spongiform encephalopathy (BSE) require

systematic testing programs to identify cases in countries

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

or to detect a stable occurrence of atypical cases in

countries in which orally acquired disease is disappearing.

To achieve 95% statistical confidence that the prevalence

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

annual incidence of sporadic Creutzfeldt-Jakob disease

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

randomly selected older cattle. A link between BSE and

sporadic CJD has been suggested on the basis of laboratory

studies but is unsupported by epidemiologic observation.

Such a link might yet be established by the discovery

of a specific molecular marker or of particular combinations

of trends over time of typical and atypical BSE and various

subtypes of sporadic CJD, as their numbers are influenced

by a continuation of current public health measures that

exclude high-risk bovine tissues from the animal and

human food chains.

SNIP...

Sporadic CJD

The possibility that at least some cases of apparently sporadic CJD might be due to infection by sporadic cases of BSE cannot be dismissed outright. Screening programs needed to identify sporadic BSE have yet to be implemented, and we know from already extant testing programs that at least a proportion of infected animals have no symptoms and thus would never be identified in the absence of systematic testing. Thus, sporadic BSE (or for that matter, sporadic disease in any mammalian species) might be occurring on a regular basis at perhaps the same annual frequency as sporadic CJD in humans, that is, in the range of 1 case per million animals.

Whether humans might be more susceptible to atypical forms of BSE cannot be answered at this time. Experimentally transmitted BASE shows shorter incubation periods than BSE in at least 1 breed of cattle, bovinized transgenic mice, and Cynomolgus monkeys (12,13). In humanized transgenic mice, BASE transmitted, whereas typical BSE did not transmit (13). Paradoxically, the other major phenotype (H) showed an unusually long incubation period in bovinized transgenic mice (12).

The limited experimental evidence bearing on a possible relationship between BSE and sporadic CJD is difficult to interpret. The original atypical BASE strain of BSE had a molecular protein signature very similar to that of 1 subtype (type 2 M/V) of sporadic CJD in humans (5). In another study, a strain of typical BSE injected into humanized mice encoding valine at codon 129 showed a glycopattern indistinguishable from the same subtype of sporadic CJD (15). In a third study, the glycopatterns of both the H and L strains of atypical BSE evidently did not resemble any of the known sporadic CJD subtypes (12).

To these molecular biology observations can be added the epidemiologic data accumulated during the past 30 years. The hypothesis that at least some cases of apparently sporadic CJD are due to unrecognized BSE infections cannot be formally refuted, but if correct, we might expect by now to have some epidemiologic evidence linking BSE to at least 1 cluster of apparently sporadic cases of CJD. Although only a few clusters have been found (and still fewer published), every proposed cluster that has been investigated has failed to show any common exposure to bovines. For that matter, no common exposure has been shown to any environmental vehicles of infection, including the consumption of foodstuffs from bovine, ovine, and porcine sources, the 3 livestock species known to be susceptible to transmissible spongiform encephalopathies. Additional negative evidence comes from several large case-control studies in which no statistically significant dietary differences were observed between patients with sporadic CJD and controls (16,17).

On the other hand, the difficulty of establishing a link between BSE and CJD may be compounded by our ignorance of the infectious parameters of a sporadic form of BSE (e.g., host range, tissue distribution of infectivity, route of transmission, minimum infectious dose for humans, whether single or multiple). Presumably, these parameters would resemble those of variant CJD; that is, high infectivity central nervous system and lymphoreticular tissues of an infected cow find their way into products consumed by humans. Transmissions that might have occurred in the past would be difficult to detect because meat products are generally not distributed in a way that results in detectable geographic clusters.

Barring the discovery of a specific molecular signature (as in variant CJD), the most convincing clue to an association will come from the observation of trends over time of the incidence of typical and atypical BSE and of sporadic and variant CJD. With 4 diseases, each of which could have increasing, unchanging, or decreasing trends, there could be 81 (34) possible different combinations. However, it is highly likely that the trends for typical BSE and variant CJD will both decrease in parallel as feed bans continue to interrupt recycled contamination. The remaining combinations are thus reduced to 9 (32), and some of them could be highly informative.

For example, if the incidence of atypical BSE declines in parallel with that of typical BSE, its candidacy as a sporadic form of disease would be eliminated (because sporadic disease would not be influenced by current measures to prevent oral infection). If, on the other hand, atypical BSE continues to occur as typical BSE disappears, this would be a strong indication that it is indeed sporadic, and if in addition at least 1 form of what is presently considered as sporadic CJD (such as the type 2 M/V subtype shown to have a Western blot signature like BASE) were to increase, this would suggest (although not prove) a causal relationship (Figure 5).

Recognition of the different forms of BSE and CJD depends upon continuing systematic testing for both bovines and humans, but bovine testing will be vulnerable to heavy pressure from industry to dismantle the program as the commercial impact of declining BSE cases ceases to be an issue. Industry should be aware, however, of the implications of sporadic BSE. Its occurrence would necessitate the indefinite retention of all of the public health measures that exclude high-risk bovine tissues from the animal and human food chains, whereas its nonoccurrence would permit tissues that are now destroyed to be used as before, once orally acquired BSE has disappeared.

SNIP...

PLEASE READ FULL TEXT ;

http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm?s_cid=eid06_0965_e

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

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve University

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

6:30 Close of Day One

http://www.healthtech.com/2007/tse/day1.asp

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

http://www.cjdsurveillance.com/resources-casereport.html

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

JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=dignosing+and+reporting+creutzfeldt+jakob+disease&searchid=1048865596978_1528&stored_search=&FIRSTINDEX=0&journalcode=jama

BRITISH MEDICAL JOURNAL

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2

BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1

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

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

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

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

THE SEVEN SCIENTIST REPORT ***

http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-EC244-Attach-1.pdf

PAUL BROWN M.D.

http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000490-vol40.pdf

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

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

http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000383-01-vol35.pdf

Embassy of Japan

http://www.fda.gov/ohrms/dockets/dockets/02n0273/02N-0273-EC240.htm

Dockets Entered on December 22, 2005

2005D-0330, Guidance for Industry and FDA Review Staff on Collection of Platelets

by Automated ... EC 203, McDonald's Restaurants Corporation, Vol #:, 34 ...

http://www.fda.gov/ohrms/dockets/dailys/05/Dec05/122205/122205.htm

03-025IF 03-025IF-631 Linda A. Detwiler [PDF] Page 1. 03-025IF 03-025IF-631 Linda A. Detwiler Page 2. Page 3. Page 4. Page 5. Page 6. Page 7. Page 8. Page 9. Page 10. Page 11. Page 12. http://www.fsis.usda.gov/OPPDE/Comments/03-025IF/03-025IF-631.pdf

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

http://www.fsis.usda.gov/OPPDE/Comments/03-025IF/03-025IF-634.pdf

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

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf

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

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-6.pdf

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

http://www.fsis.usda.gov/OPPDE/Comments/03-025IF/03-025IF-589.pdf

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