Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases

Transmission of sporadic Creutzfeldt-Jakob disease by blood transfusion: risk factor or possible biases

Maria Puopolo,
Anna Ladogana,
Vito Vetrugno,
Maurizio Pocchiari

Article first published online: 7 JAN 2011



DOI: 10.1111/j.1537-2995.2010.03004.x

© 2010 American Association of Blood Banks



BACKGROUND: The occurrence of transfusion transmissions of variant Creutzfeldt-Jakob disease (CJD) cases has reawakened attention to the possible similar risk posed by other forms of CJD.



STUDY DESIGN AND METHODS: CJD with a definite or probable diagnosis (sporadic CJD, n = 741; genetic CJD, n = 175) and no-CJD patients with definite alternative diagnosis (n = 482) with available blood transfusion history were included in the study. The risk of exposure to blood transfusion occurring more than 10 years before disease onset and for some possible confounding factors was evaluated by calculating crude odds ratios (ORs). Variables with significant ORs in univariate analyses were included in multivariate logistic regression analyses.



RESULTS: In the univariate model, blood transfusion occurring more than 10 years before clinical onset is 4.1-fold more frequent in sporadic CJD than in other neurologic disorders. This significance is lost when the 10-year lag time was not considered. Multivariate analyses show that the risk of developing sporadic CJD after transfusion increases (OR, 5.05) after adjusting for possible confounding factors. Analysis conducted on patients with genetic CJD did not reveal any significant risk factor associated with transfusion.



CONCLUSION: This is the first case-control study showing a significant risk of transfusion occurring more than 10 years before clinical onset in sporadic CJD patients. It remains questionable whether the significance of these data is biologically plausible or the consequence of biases in the design of the study, but they counterbalance previous epidemiologic negative reports that might have overestimated the assessment of blood safety in sporadic CJD.





http://onlinelibrary.wiley.com/doi/10.1111/j.1537-2995.2010.03004.x/abstract;jsessionid=656FB07EE4507FB2632BA3E691D3B824.d03t02






Eurosurveillance, Volume 12, Issue 3, 18 January 2007

Articles

Editorial team1


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Citation style for this article: Editorial team. Fourth case of transfusion-associated vCJD infection in the United Kingdom. Euro Surveill. 2007;12(3):pii=3117. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=3117
Date of submission:


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Fourth case of transfusion-associated vCJD infection in the United Kingdom



Editorial team (eurosurveillance.weekly@hpa.org.uk), Eurosurveillance editorial office



A case of variant Creutzfeldt-Jakob disease (vCJD) has recently been diagnosed in a patient in the United Kingdom (UK), who received a blood transfusion from a donor who later developed vCJD [1]. This is the fourth case of probable transfusion transmission of vCJD infection in the UK. Three of the four recipients developed symptoms of vCJD.

The first symptomatic case of vCJD associated with blood transfusion was identified in December 2003. This individual developed vCJD six and a half years after transfusion of red cells donated by an individual who developed symptoms of vCJD three and a half years after donation.



A second case of vCJD 'infection' was identified a few months later in a person who had received red cells from a donor who developed symptoms of vCJD 18 months after donation. This patient (the second case) died from causes unrelated to vCJD five years after transfusion. Post-mortem investigations found abnormal prion protein in the spleen and a cervical lymph node., However, prion protein was not found in the brain, and no pathological features of vCJD were found.



A third case developed symptoms of vCJD six years after receiving a transfusion of red blood cells, and died two years and eight months later. The donor of the blood involved developed vCJD about 20 months after donating it.



These three cases have been published as case reports and in the findings of the ongoing collaborative study between the National Blood Services, the National CJD Surveillance Unit, and the Office for National Statistics. This study aims to collect evidence about transmission of CJD or vCJD via the blood supply [2,3,4,5].



The new, fourth case is in a patient who developed symptoms of vCJD eight and a half years after receiving a transfusion of red blood cells from a donor who developed vCJD about 17 months after this blood was donated [1]. The donor to this case also donated the vCJD-implicated blood transfused to the third case. As for all other reported clinical vCJD cases that have been tested for genotype, this patient is a methionine homozygote at codon 129 of the prion protein gene. The patient is currently alive.



All four cases had received transfusions of non-leucodepleted red blood cells between 1996 and 1999. Since October 1999, leucocytes have been removed from all blood used for transfusion in the UK. The effect of leucodepletion on the reduction of the risk of transmission of vCJD from an infective donation is uncertain.



This fourth case of vCJD infection associated with blood transfusion further increases the level of concern about the risk of vCJD transmission between humans by blood transfusion, although much remains unknown. This reinforces the importance of the existing precautions that have been introduced to reduce the risk of transmission of vCJD infection by blood and blood products [6]. No cases of vCJD have been associated with fractionated plasma products. The small group of living recipients of vCJD-implicated blood transfusion in the UK have been informed of their potential exposure to vCJD by blood transfusion, asked to take certain precautions to reduce the risk of onward person-to-person transmission of vCJD during health care, and offered specialist neurological evaluation and advice.



This article has been adapted from reference 1




References:

Health Protection Agency. Fourth case of variant CJD associated with blood transfusion (press release). Press release, 18 January 2007. (http://www.hpa.org.uk/hpa/news/articles/press_releases/2007/070118_vCJD.htm)
Llewelyn CA, Hewitt PE, Knight RSG, Amar K, Cousens S, Mackenzie J, et al. Possible transmission of variant CJD disease by blood transfusion. Lancet 2004; 363:417-21.
Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004 ; 364: 527-9.
Wroe SJ, Pal S, Siddique D, Hyare H, Macfarlane R, et al Clinical presentation and pre-mortem diagnosis of blood transfusion-associated variant CJD. Lancet 2006;368:2061-67.
Hewitt PE, Llewelyn CA, Mackenzie J, Will RG. Creutzfeldt-Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiology review study. Vox Sang. 2006;91(3):221-230.
Department of Health [London]. Further precautions to protect blood supply. Press release 2004/0104, 16 March 2004. (http://www.dh.gov.uk/PublicationsAndStatistics/PressReleases/PressReleasesNotices/fs/en?CONTENT_ID=4076608&chk=MTwE%2Bl)


http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=3117




Tuesday, September 28, 2010

Variant CJD: where has it gone, or has it?

Pract Neurol 2010; 10: 250–251


http://vcjdtransfusion.blogspot.com/2010/09/variant-cjd-where-has-it-gone-or-has-it.html




Tuesday, September 14, 2010

Transmissible Spongiform Encephalopathies Advisory Committee; Notice of Meeting October 28 and 29, 2010 (COMMENT SUBMISSION)

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

From: Terry S. Singeltary Sr.

To: william.freas@fda.hhs.gov Cc: rosanna.harvey@fda.hhs.gov ; Emery, Bryan (CBER)

SEE FULL TEXT ;

http://tseac.blogspot.com/2010/09/transmissible-spongiform_14.html



Wednesday, September 08, 2010

Emerging Infectious Diseases: CJD, BSE, SCRAPIE, CWD, PRION, TSE Evaluation to Implementation for Transfusion and Transplantation September 2010

http://vcjdtransfusion.blogspot.com/2010/09/emerging-infectious-diseases-cjd-bse.html



Saturday, July 17, 2010

Variant Creutzfeldt-Jakob disease Ironside JW., Haemophilia.

2010 Jul;16 Suppl 5:175-80 REVIEW ARTICLE

http://vcjdtransfusion.blogspot.com/2010/07/variant-creutzfeldtjakob-disease.html



http://vcjdtransfusion.blogspot.com/




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

From: Terry S. Singeltary Sr.

To: CJD-L@LISTS.AEGEE.ORG

Cc: BLOODCJD@YAHOOGROUPS.COM ; cjdvoice@yahoogroups.com

Sent: Friday, September 24, 2010 11:15 AM

Subject: [BLOODCJD] USA Blood products, collected from a donor who was at risk for vCJD, were distributed SEPTEMBER 2010

PRODUCT
Red Blood Cells. Recall # B-2300-10
CODE
Unit: W001607702825
RECALLING FIRM/MANUFACTURER
Recalling Firm: Department of the Air Force, Wright Patterson AFB, OH, by letter dated April 8, 2008.
Manufacturer: Depart of Air Force 88th Medical Group SGQC WPAFB, Wright Patterson AFB, OH. Firm initiated recall is complete.
REASON
Blood product, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), was distributed.
VOLUME OF PRODUCT IN COMMERCE
1 unit
DISTRIBUTION
Japan

___________________________________

PRODUCT
Recovered Plasma. Recall # B-2302-10
CODE
Units: R08951; P90041; P90041
RECALLING FIRM/MANUFACTURER
Blood Center of Northcentral Wisconsin, Inc., Wausau, WI, by fax on January 2, 2007. Firm initiated recall is complete.
REASON
Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
3 units
DISTRIBUTION
NY

___________________________________

PRODUCT
1) Red Blood Cells Leukocytes Reduced. Recall # B-2338-10;
2) Plasma Frozen. Recall # B-2339-10
CODE
1) and 2) Unit: 5039861
RECALLING FIRM/MANUFACTURER
Community Blood Center, Inc., Appleton, WI, by letter dated September 21, 2007 or by electronic notification on September 21, 2007. Firm initiated recall is complete.
REASON
Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
2 units
DISTRIBUTION
WI, Switzerland

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 22, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm227078.htm


PRODUCT

1) Cryoprecipitated AHF, Pooled. Recall # B-2155-10;

2) Recovered Plasma. Recall # B-2156-10

CODE

1) Unit: W036309907231;

2) Unit: W036309616077

RECALLING FIRM/MANUFACTURER

BloodCenter of Wisconsin, Inc., Milwaukee, WI, by fax and internet on May 5, 2010 and May 13, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

2 units

DISTRIBUTION

TX, Switzerland

___________________________________

PRODUCT

Red Blood Cells Leukocytes Reduced. Recall # B-2157-10

CODE

Unit: 6371718

RECALLING FIRM/MANUFACTURER

South Texas Blood & Tissue Center, San Antonio, TX, by telephone on January 23, 2010 and by fax on January 25, 2010. Firm initiated recall is complete.

REASON

Blood product, collected from a donor who failed to answer questions regarding risk for vCJD, was distributed.

VOLUME OF PRODUCT IN COMMERCE

1 unit

DISTRIBUTION

TX

___________________________________

PRODUCT

Source Plasma. Recall # B-2212-10

CODE

Units: 09FMOG6851; 09FMOG3410; 09FMOG2756; 09FMOG1418; 09FMOF6640; 09FMOF2642; 09FMOF1554; 09FMOD7746; 09FMOF0063; 09FMOF7599

RECALLING FIRM/MANUFACTURER

BioLife Plasma Service LP, Springfield, MO, by fax on April 1, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

10 units

DISTRIBUTION

CA

___________________________________

PRODUCT

1) Red Blood Cells Leukocytes Reduced. Recall # B-2213-10;

2) Recovered Plasma. Recall # B-2214

CODE

1) and 2) Unit: 6325245

RECALLING FIRM/MANUFACTURER

South Texas Blood and Tissue Center, San Antonio, TX, by facsimile on February 8, 2010. Firm initiated recall is complete.

REASON

Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.

VOLUME OF PRODUCT IN COMMERCE

2 units

DISTRIBUTION

FL, TX

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 15, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm225990.htm


PRODUCT
Source Plasma. Recall # B-2056-10
CODE
Units: FD0500537, FD0502880, FD0503259, FD0509894, FD0515518, FD0516063, FD0517957, FD0518606, FD0522255, FD0523346, FD0523544, FD0524204, FD0524698, FD0525142, FD0525845, FD0526653, FD0526878, FD0527579, FD0527845, FD0528519, FD0528827, FD0529544, FD0529761, FD0530471, FD0530712, FD0531425, FD0531801, FD0532483, FD0532869, FD0537501, FD0537687, FD0538370, FD0543210, FD0546250, FD0546632, FD0547328, FD0547832, FD0548286, FD0548743, FD0549325, FD0549840, FD0550427, FD0551448, FD0551572, FD0552307, FD0553173, FD0553418, FD0554063, FD0554834, FD0555041, FD0559685, FD0560235, FD0560592, FD0561168, FD0561786, FD0562212, FD0562883, FD0563248, FD0564435, FD0564723, FD0565467, FD0565880, FD0566540, FD0567053, FD0567723, FD0567965, FD0568941, FD0569180, FD0570057, FD0571177, FD0571477, FD0572411, FD0572818, FD0573582, FD0573871, FD0574531, FD0576955, FD0577140, FD0579983, FD0580403, FD0581156, FD0581623, FD0582680, FD0583090, FD0584073, FD0584500, FD0585410, FD0586089, FD0586790, FD0587500, FD0588791, FD0589023, FD0590248, FD0590600, FD0591592, FD0592445, FD0593277, FD0593712, FD0594626, FD0595049, FD0596132, FD0596519, FD0597701, FD0598681, FD0599198, FD0600210, FD0600690, FD0601755, FD0602401, FD0603415, FD0603985, FD0605122, FD0608608, FD0609074, FD0609979, FD0610508, FD0611469, FD0612006, FD0612905
RECALLING FIRM/MANUFACTURER
DCI Biologicals LLC, Farmington, NM, by facsimile on September 26, 2009 and electronic mail dated January 15, 2010. Firm initiated recall is complete.
REASON
Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
119 units
DISTRIBUTION
NY, UK

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 8, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm225223.htm


PRODUCT
Source Plasma. Recall # B-2134-10
CODE
Units: 3910020431, 3910019695, 3910018715, 3910018227, 3910017100, 3910016675, 3910015596, 3910015120, 3910014175, 3910013575, 3910012934, 3910012281, 3910010102, 3910009899, 3910007715, 3910007430
RECALLING FIRM/MANUFACTURER
Talecris Plasma Resources, Inc., N Las Vegas, NV, by fax on July 17, 2009. Firm initiated recall is complete.
REASON
Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
16 units
DISTRIBUTION
NC

___________________________________

PRODUCT
1) Red Blood Cells. Recall # B-2215-10;
2) Fresh Frozen Plasma. Recall # B-2216-10
CODE
1) and 2) Unit: 0951592
RECALLING FIRM/MANUFACTURER
Memorial Blood Centers, Saint Paul, MN, by letter on November 5, 2008. Firm initiated recall is complete.
REASON
Blood products, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed.
VOLUME OF PRODUCT IN COMMERCE
2 units
DISTRIBUTION
MN

___________________________________

PRODUCT
Recovered Plasma. Recall # B-2217-10
CODE
Unit: 0951592
RECALLING FIRM/MANUFACTURER
Memorial Blood Centers, Saint Paul, MN, by letter on November 5, 2008. Firm initiated recall is complete.
REASON
Blood product, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), was distributed.
VOLUME OF PRODUCT IN COMMERCE
1 unit
DISTRIBUTION
MN

___________________________________

END OF ENFORCEMENT REPORT FOR SEPTEMBER 1, 2010

http://www.fda.gov/Safety/Recalls/EnforcementReports/ucm224723.htm



TSS

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

Here we go folks. AS predicted. THIS JUST OUT !

kind regards, terry


Original Article

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

Wen-Quan Zou, MD, PhD 1 *, Gianfranco Puoti, MD, PhD 1, Xiangzhu Xiao, PhD 1, Jue Yuan, BA 1, Liuting Qing, PhD 1, Ignazio Cali, MSc 1, Miyuki Shimoji, PhD 1, Jan P.M. Langeveld, PhD 2, Rudy Castellani, MD 3, Silvio Notari, PhD 1, Barbara Crain, MD 4, Robert E. Schmidt, MD 5, Michael Geschwind, MD 6, Stephen J. DeArmond, MD, PhD 6, Nigel J. Cairns, MD 7, Dennis Dickson, MD 8, Lawrence Honig, MD 9, Juan Maria Torres, PhD 10, James Mastrianni, MD, PhD 11, Sabina Capellari, MD 12, Giorgio Giaccone, MD 13, Ermias D. Belay, MD 14, Lawrence B. Schonberger, MD, MPH 14, Mark Cohen, MD 1, George Perry, PhD 15, Qingzhong Kong, PhD 1, Piero Parchi, MD, PhD 12, Fabrizio Tagliavini, MD 13, Pierluigi Gambetti, MD 1 * 1Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH 2Central Veterinary Institute of Wageningen, Lelystad, the Netherlands 3Department of Neuropathology, University of Maryland Medical Center, Baltimore, MD 4Department of Neuropathology, Johns Hopkins University, Baltimore, MD 5Department of Neuropathology, Washington University, St. Louis, MO 6Department of Pathology, University of California at San Francisco, San Francisco, CA 7Departments of Neurology, Pathology, and Immunology, Washington University, St. Louis, MO 8Department of Neuropathology, Mayo Clinic-Jacksonville, Jacksonville, FL 9New York Presbyterian Hospital, Columbia University, New York, NY 10Centro de Investigación en Sanidad Animal, Madrid, Spain 11Department of Neurology, University of Chicago, Chicago, IL 12Department of Neurological Sciences, University of Bologna, Dipartimento di Scienze Neurologiche, Università di Bologna, Bologna, Italy 13IRCCS Foundation, National Neurological Institute, Instituto Nazionale Neurologico Carlo Besta, Milan, Italy 14Center of Investigation on Animal Health, Centers for Disease Control and Prevention, Atlanta, GA 15College of Science, University of Texas at San Antonio, San Antonio, TX

email: Wen-Quan Zou (wenquan.zou@case.edu) Pierluigi Gambetti (pierluigi.gambetti@case.edu)

*Correspondence to Wen-Quan Zou, Department of Pathology, National Prion Disease Pathology Surveillance Center, Case Western Reserve University, Cleveland, OH

*Correspondence to Pierluigi Gambetti, Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road, Cleveland, OH 44106

Potential Conflicts of Interest Nothing to report.

Funded by: NIH; Grant Number: NIA AG14359, AG08702 NINDS; Grant Number: R01NS062787 Centers for Disease Control and Prevention; Grant Number: CCU 515004 Britton Fund CJD Foundation Alliance BioSecure University Center on Aging and Health with the support of the McGregor Foundation and President's Discretionary Fund (Case Western Reserve University) National Institute on Aging; Grant Number: AG05681

Abstract

Objective: The objective of the study is to report 2 new genotypic forms of protease-sensitive prionopathy (PSPr), a novel prion disease described in 2008, in 11 subjects all homozygous for valine at codon 129 of the prion protein (PrP) gene (129VV). The 2 new PSPr forms affect individuals who are either homozygous for methionine (129MM) or heterozygous for methionine/valine (129MV).

Methods: Fifteen affected subjects with 129MM, 129MV, and 129VV underwent comparative evaluation at the National Prion Disease Pathology Surveillance Center for clinical, histopathologic, immunohistochemical, genotypical, and PrP characteristics.

Results: Disease duration (between 22 and 45 months) was significantly different in the 129VV and 129MV subjects. Most other phenotypic features along with the PrP electrophoretic profile were similar but distinguishable in the 3 129 genotypes. A major difference laid in the sensitivity to protease digestion of the disease-associated PrP, which was high in 129VV but much lower, or altogether lacking, in 129MV and 129MM. This difference prompted the substitution of the original designation with variably protease-sensitive prionopathy (VPSPr). None of the subjects had mutations in the PrP gene coding region.

Interpretation: Because all 3 129 genotypes are involved, and are associated with distinguishable phenotypes, VPSPr becomes the second sporadic prion protein disease with this feature after Creutzfeldt-Jakob disease, originally reported in 1920. However, the characteristics of the abnormal prion protein suggest that VPSPr is different from typical prion diseases, and perhaps more akin to subtypes of Gerstmann-Sträussler-Scheinker disease. ANN NEUROL 2010;68:162-172

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

Received: 9 March 2010; Revised: 5 May 2010; Accepted: 19 May 2010

Digital Object Identifier (DOI)

10.1002/ana.22094 About DOI


http://www3.interscience.wiley.com/journal/123598302/abstract?CRETRY=1&SRETRY=0




>>> Because 8 out of 10 patients had a positive family history of dementia in the original study, a genetic cause was suspected. Although all cases were homozygous for valine at codon 129 of the PrP gene, NO mutations were detected.<<<


Protease-sensitive prionopathy (PSPr) is a neurodegenerative disorder caused by an abnormal isoform of the prion protein. Contrary to the prions in Creutzfeldt-Jakob disease (CJD), the prions in this condition are sensitive to protease activity. PSPr was first described in an abstract for a conference on prions in 2006, and this study was published in a 2008 report on 11 cases. The study was conducted by Gambetti P. and coworkers from the United States National Prion Disease Pathology Surveillance Center.

[1] Symptoms of PSPr are behavioral and psychiatric abnormalities with progressive decline in cognitive and motor functions (including dementia, ataxia, parkinsonism, psychosis, aphasia and mood disorders). This is similar to what occurs in other spongiform encephalopathies, and the condition can be mistaken for Alzheimer's dementia. Contrary to what is the case in CJD, there were no cases with a positive 14-3-3 protein test in the cerebrospinal fluid, no periodic complexes on electroencephalography (EEG), and no pathognomonic changes on diffusion-weighted magnetic resonance images. The mean age of onset in the original 11 patients was 62 years. Patients progressed to death in 20 months on average. PSPr accounted for three percent of prion disease cases evaluated by the U.S. National Prion Disease Pathology Surveillance Center (prion diseases occur in the order of magnitude of 1 case per million people).

[2] The diagnosis can be made on pathological examination. There are unique microscopic and immunohistochemical features, and the prions cannot be digested using proteases. Because 8 out of 10 patients had a positive family history of dementia in the original study, a genetic cause was suspected. Although all cases were homozygous for valine at codon 129 of the PrP gene, no mutations were detected. How these protease-resistant prions lead to neurological disease remains incompletely understood. For instance, protein-resistant prions have been found in normal human brains.

[3] 1.^ Gambetti P, Dong Z, Yuan J, et al. (June 2008). "A novel human disease with abnormal prion protein sensitive to protease". Ann. Neurol. 63 (6): 697–708. doi:10.1002/ana.21420. PMID 18571782. 2.^ Will R, Head M (June 2008). "A new prionopathy". Ann. Neurol. 63 (6): 677–8. doi:10.1002/ana.21447. PMID 18570344. 3.^ Yuan J, Xiao X, McGeehan J, et al. (November 2006). "Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains". J. Biol. Chem. 281 (46): 34848–58. doi:10.1074/jbc.M602238200. PMID 16987816.


http://en.wikipedia.org/wiki/Protease-sensitive_prionopathy


no mad cow disease in the USA, no human TSE there from, it's all sporadic genetic now, with no family mutation link there from ???

p l e a s e !

the first 10 humans in the UK in 1995 = BSE to nvCJD in humans. now, 2010, similarly, the 10+ in the USA, = genetic make up same as the g-h-BSEalabama bovine case, but it's all a _sporadic_ genetic disease, that NO mutations were found in family members, BUT is not related to the g-h-BSEalabama case either, even though those mutations are the same? just happens out of thin air. my oh my, how political junk science works $$$

either the UK BSE nvCJD only theory was wrong, or Gambetti's theory is wrong.
which is it ? you can't have your cake and eat it too. just my opinion. ...tss



Genetic Creutzfeldt-Jakob disease associated with the E200K mutation: characterization of a complex proteinopathy

Journal Acta Neuropathologica Publisher Springer Berlin / Heidelberg ISSN 0001-6322 (Print) 1432-0533 (Online) Category Original Paper DOI 10.1007/s00401-010-0713-y Subject Collection Medicine SpringerLink Date Wednesday, June 30, 2010 Gabor G. Kovacs1, 2 , Jérémie Seguin3, Isabelle Quadrio3, Romana Höftberger1, István Kapás2, Nathalie Streichenberger3, Anne Gaëlle Biacabe4, David Meyronet3, Raf Sciot5, Rik Vandenberghe6, Katalin Majtenyi2, Lajos László7, Thomas Ströbel1, Herbert Budka1 and Armand Perret-Liaudet3 (1) Institute of Neurology, Medical University of Vienna, and Austrian Reference Center for Human Prion Diseases, AKH 4J, Währinger Gürtel 18-20, 1097 Vienna, Austria (2) Neuropathology and Prion Disease Reference Center, Hungarian Reference Center for Human Prion Diseases, Semmelweis University, Budapest, Hungary (3) Prion Disease Laboratory, Pathology and Biochemistry, Groupement Hospitalier Est, Hospices Civils de Lyon/Claude Bernard University, Lyon, France (4) Agence Française de Sécurité Sanitaire des AlimentsˆLyon, Unité ATNC, Lyon Cedex 07, France (5) Department of Pathology, University Hospital, Catholic University of Leuven, Leuven, Belgium (6) Neurology Department, University Hospital Gasthuisberg, Leuven, Belgium (7) Department of Anatomy, Cell and Developmental Biology, Eotvos Lorand University of Sciences, Budapest, Hungary Received: 1 April 2010 Revised: 10 June 2010 Accepted: 20 June 2010 Published online: 1 July 2010

Abstract

The E200K mutation is the most frequent prion protein gene (PRNP) mutation detected worldwide that is associated with Creutzfeldt-Jakob disease (CJD) and thought to have overlapping features with sporadic CJD, yet detailed neuropathological studies have not been reported. In addition to the prion protein, deposition of tau, a-synuclein, and amyloid-ß has been reported in human prion disease. To describe the salient and concomitant neuropathological alterations, we performed a systematic clinical, neuropathological, and biochemical study of 39 individuals carrying the E200K PRNP mutation originating from different European countries. The most frequent clinical symptoms were dementia and ataxia followed by myoclonus and various combinations of further symptoms, including vertical gaze palsy and polyneuropathy. Neuropathological examination revealed relatively uniform anatomical pattern of tissue lesioning, predominating in the basal ganglia and thalamus, and also substantia nigra, while the deposition of disease-associated PrP was more influenced by the codon 129 constellation, including different or mixed types of PrPres detected by immunoblotting. Unique and prominent intraneuronal PrP deposition involving brainstem nuclei was also noted. Systematic examination of protein depositions revealed parenchymal amyloid-ß in 53.8%, amyloid angiopathy (Aß) in 23.1%, phospho-tau immunoreactive neuritic profiles in 92.3%, neurofibrillary degeneration in 38.4%, new types of tau pathology in 33.3%, and Lewy-type a-synuclein pathology in 15.4%. TDP-43 and FUS immunoreactive protein deposits were not observed. This is the first demonstration of intensified and combined neurodegeneration in a genetic prion disease due to a single point mutation, which might become an important model to decipher the molecular interplay between neurodegeneration-associated proteins. Electronic supplementary material The online version of this article (doi:10.1007/s00401-010-0713-y) contains supplementary material, which is available to authorized users.

Keywords Alpha-synuclein - Amyloid-beta - Prion protein - Tau - Neurodegeneration


http://www.springerlink.com/content/p0236716117l0778/


P02.35

Molecular Features of the Protease-resistant Prion Protein (PrPres) in H-type BSE

Biacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron, TGM1 1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, Sweden

Western blot analyses of PrPres accumulating in the brain of BSE-infected cattle have demonstrated 3 different molecular phenotypes regarding to the apparent molecular masses and glycoform ratios of PrPres bands. We initially described isolates (H-type BSE) essentially characterized by higher PrPres molecular mass and decreased levels of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type is also distinct from another BSE phenotype named L-type BSE, or also BASE (for Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low representation of the diglycosylated PrPres band as well as a lower PrPres molecular mass. Retrospective molecular studies in France of all available BSE cases older than 8 years old and of part of the other cases identified since the beginning of the exhaustive surveillance of the disease in 20001 allowed to identify 7 H-type BSE cases, among 594 BSE cases that could be classified as classical, L- or H-type BSE. By Western blot analysis of H-type PrPres, we described a remarkable specific feature with antibodies raised against the C-terminal region of PrP that demonstrated the existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in cattle seems to by higher compared to the PrPres#1. Furthermore another PK–resistant fragment at about 7 kDa was detected by some more N-terminal antibodies and presumed to be the result of cleavages of both N- and C-terminal parts of PrP. These singular features were maintained after transmission of the disease to C57Bl/6 mice. The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band) reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.


FC5.5.1

BASE Transmitted to Primates and MV2 sCJD Subtype Share PrP27-30 and PrPSc C-terminal Truncated Fragments

Zanusso, G1; Commoy, E2; Fasoli, E3; Fiorini, M3; Lescoutra, N4; Ruchoux, MM4; Casalone, C5; Caramelli, M5; Ferrari, S3; Lasmezas, C6; Deslys, J-P4; Monaco, S3 1University of Verona, of Neurological and Visual Sciences, Italy; 2CEA, IMETI/SEPIA, France; 3University of Verona, Neurological and Visual Sciences, Italy; 4IMETI/SEPIA, France; 5IZSPLVA, Italy; 6The Scripps Research Insitute, USA

The etiology of sporadic Creutzfeldt-Jakob disease (sCJD), the most frequent human prion disease, remains still unknown. The marked disease phenotype heterogeneity observed in sCJD is thought to be influenced by the type of proteinase K-resistant prion protein, or PrPSc (type 1 or type 2 according to the electrophoretic mobility of the unglycosylated backbone), and by the host polymorphic Methionine/Valine (M/V) codon 129 of the PRNP. By using a two-dimensional gel electrophoresis (2D-PAGE) and imunoblotting we previously showed that in sCJD, in addition to the PrPSc type, distinct PrPSc C-terminal truncated fragments (CTFs) correlated with different sCJD subtypes. Based on the combination of CTFs and PrPSc type, we distinguished three PrPSc patterns: (i) the first was observed in sCJD with PrPSc type 1 of all genotypes,; (ii) the second was found in M/M-2 (cortical form); (iii) the third in amyloidogenic M/V- 2 and V/V-2 subtypes (Zanusso et al., JBC 2004) . Recently, we showed that sCJD subtype M/V-2 shared molecular and pathological features with an atypical form of BSE, named BASE, thus suggesting a potential link between the two conditions. This connection was further confirmed after 2D-PAGE analysis, which showed an identical PrPSc signature, including the biochemical pattern of CTFs. To pursue this issue, we obtained brain homogenates from Cynomolgus macaques intracerebrally inoculated with brain homogenates from BASE. Samples were separated by using a twodimensional electrophoresis (2D-PAGE) followed by immunoblotting. We here show that the PrPSc pattern obtained in infected primates is identical to BASE and sCJD MV-2 subtype. These data strongly support the link, or at least a common ancestry, between a sCJD subtype and BASE. This work was supported by Neuroprion (FOOD-CT-2004-506579)


FC5.5.2

Transmission of Italian BSE and BASE Isolates in Cattle Results into a Typical BSE Phenotype and a Muscle Wasting Disease

Zanusso, G1; Lombardi, G2; Casalone, C3; D’Angelo, A4; Gelmetti, D2; Torcoli, G2; Barbieri, I2; Corona, C3; Fasoli, E1; Farinazzo, A1; Fiorini, M1; Gelati, M1; Iulini, B3; Tagliavini, F5; Ferrari, S1; Monaco, S1; Caramelli, M3; Capucci, L2 1University of Verona, Neurological and Visual Sciences, Italy; 2IZSLER, Italy; 3IZSPLVA, Italy; 4University of Turin, Animal Pathology, Italy; 5Isituto Carlo Besta, Italy

The clinical phenotype of bovine spongiform encephalopathy has been extensively reported in early accounts of the disorder. Following the introduction of statutory active surveillance, almost all BSE cases have been diagnosed on a pathological/molecular basis, in a pre-symptomatic clinical stage. In recent years, the active surveillance system has uncovered atypical BSE cases, which are characterized by distinct conformers of the PrPSc, named high-type (BSE-H) and low-type (BSE-L), whose clinicopathological phenotypes remain unknown. We recently reported two Italian atypical cases with a PrPSc type similar to BSE-L, pathologically characterized by PrP amyloid plaques. Experimental transmission to TgBov mice has recently disclosed that BASE is caused by a distinct prion strain which is extremely virulent. A major limitation of transmission studies to mice is the lack of reliable information on clinical phenotype of BASE in its natural host. In the present study, we experimentally infected Fresian/Holstein and Alpine/Brown cattle with Italian BSE and BASE isolates by i.c. route. BASE infected cattle showed survival times significantly shorter than BSE, a finding more readily evident in Fresian/Holstein, and in keeping with previous observations in TgBov mice. Clinically, BSE-infected cattle developed a disease phenotype highly comparable with that described in field BSE cases and in experimentally challenged cattle. On the contrary, BASE-inoculated cattle developed an amyotrophic disorder accompanied by mental dullness. The molecular and neuropathological profiles, including PrP deposition pattern, closely matched those observed in the original cases. This study further confirms that BASE is caused by a distinct prion isolate and discloses a novel disease phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated cattle and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease.


P02.16

Analysis of Bovine Prion Protein Gene Sequence Variation in Animals with Classical and Atypical BSE

Polak, MP; Larska, M; Rola, J; Zmudzinski, JF National Veterinary Research Institute, Department of Virology, Poland B.

Variation within prion protein gene sequence have major impact on the susceptibility to prion diseases in humans and sheep. However no major differences between healthy cattle and bovine spongiform encephalopathy (BSE) affected individuals were identified. Recent studies indicate that susceptibility to bovine spongiform encephalopathy is associated with 23-base pair (bp) and 12-bp indel sequences. Identification of atypical BSE in older cattle in several countries pointed at the possibility of spontaneous origin of this new form of prion disease due to possible mutations within prion gene (PRNP) sequence. A./O. Therefore the aim of the study was to analyze and to compare prion protein gene sequences in animals showing classical and atypical BSE for any genetic traits differentating both forms of the disease. M. Analysis included: octapeptide-repeat polymorphism; sequence analysis of exon 3 region; deletion/insertion polymorphism within the promoter sequence (23-bp), intron 1 (12-bp) and 3’untranslated region - UTR (14-bp) of PRNP gene. R. No major differences were found as for the octapeptide-repeats. Most dominant genotype in both classical and atypical BSE involved 6/6 homozygous animals. Sequence comparison within exon 3 region also showed no differences. Results from indel sequence analysis within three regions of PRNP gene were also quite uniform between both forms of BSE. D. Therefore no genetic traits explaining the appearance of atypical BSE could be found. However, it is too early to reject the hypothesis that genetic makeup is not involved in atypical BSE. Further and more detailed studies including more cases of atypical BSE would be more reliable to draw such a conclusion.

http://www.neuroprion.com/pdf_docs/conferences/prion2007/abstract_book.pdf


g-h-BSEalabama


BSE Case Associated with Prion Protein Gene Mutation

Bovine spongiform encephalopathy (BSE) is a transmissible spongiform encephalopathy (TSE) of cattle and was first detected in 1986 in the United Kingdom. It is the most likely cause of variant Creutzfeldt-Jakob disease (CJD) in humans. The origin of BSE remains an enigma. Here we report an H-type BSE case associated with the novel mutation E211K within the prion protein gene (Prnp). Sequence analysis revealed that the animal with H-type BSE was heterozygous at Prnp nucleotides 631 through 633. An identical pathogenic mutation at the homologous codon position (E200K) in the human Prnp has been described as the most common cause of genetic CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. A recent epidemiological study revealed that the K211 allele was not detected in 6062 cattle from commercial beef processing plants and 42 cattle breeds, indicating an extremely low prevalence of the E211K variant (less than 1 in 2000) in cattle.

Author Summary Top

Bovine spongiform encephalopathy (BSE or Mad Cow Disease), a transmissible spongiform encephalopathy (TSE) or prion disease of cattle, was first discovered in the United Kingdom in 1986. BSE is most likely the cause of a human prion disease known as variant Creutzfeldt Jakob Disease (vCJD). In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in “the approximately 10-year-old cow” carrying the E221K mutation.


http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156


http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF


Archive Number 20100405.1091 Published Date 05-APR-2010

Subject PRO/AH/EDR> Prion disease update 1010 (04)

snip...

[Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.

The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"

http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101



Wednesday, July 9, 2008

10 people killed by new CJD-like disease USA

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/10-people-killed-by-new-cjd-like.html


Thursday, July 10, 2008

A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008

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


Thursday, July 10, 2008

A New Prionopathy update July 10, 2008

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html


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




some additional interesting studies ;


O.10.5

A novel human prion disease affecting subjects with the three prion protein codon 129 genotypes: could it be the sporadic form of Gerstmann-Straussler?

Pierluigi Gambetti Case Western Reserve University, USA

Background: We recently described a novel prion disease, named protease-sensitive prionopathy or PSPr, characterized by the presence of an abnormal prion protein (PrP) that was 60 fold less protease resistant than that of sporadic Creutzfeldt-Jakob disease (sCJD) and on immunoblot generated a distinct ladder-like profile. All affected subjects where homozygous for valine at codon 129 (VV) and had no mutation in the PrP gene.

Methods: We have characterized several new cases in our surveillance and received from Europe.

Results:

1) A disease overall similar to that reported in the 129VV subjects also affects subjects that are methionine/valine heterozygous (MV) and methionine homozygous (MM) at codon 129 and have no PrP gene mutation;

2) The clinical and histopathological features of the new MV and MM PSPr cases are similar but distinguishable from those of the original VV cases; 3) The electrophoretic profiles generated by the abnormal PrP isoforms associated with the MV and MM cases are similar to VV cases but show increasing levels of proteaseresistance; 3) abnormal tau is present in all three genotypic forms of PSPr with features apparently similar to those of primary tauopathies placing PSPr at the intersection of tauopathies and prion diseases.

Discussion: Will focus on: 1) the features of the abnormal PrP in the newly discovered 129MV and 129MM PSPr; 2) the effect of the 129 polymorphism on PSPr compared to that on sCJD; 3) the relationship of PSPr with tauopathies; 4) whether PSPr now with the three 129 genotypic forms is the long sought sporadic form of GSS. (Supported by NIH AG-14359, NS052319, CDC UR8/CCU515004).


http://www.neuroprion.org/resources/pdf_docs/conferences/prion2009/prion2009_bookofabstracts.pdf




Wednesday, September 3, 2008

Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series

Brain Advance Access published September 1, 2008

Phenotypic heterogeneity and genetic modification of P102L inherited prion disease in an international series

T. E. F.Webb,1,2 M. Poulter,1 J. Beck,1 J.Uphill,1 G. Adamson,1 T. Campbell,1 J. Linehan,1 C. Powell,1 S. Brandner,1,2 S. Pal,1,2 D. Siddique,1,2 J. D.Wadsworth,1 S. Joiner,1 K. Alner,2 C. Petersen,2 S. Hampson,2 C. Rhymes,2 C. Treacy,2 E. Storey,3 M. D.Geschwind,4 A. H. Nemeth,5 S.Wroe,1,2 J. Collinge1,2 and S. Mead1,2 1MRC Prion Unit and Department of Neurodegenerative Disease,UCL Institute of Neurology, 2National Prion Clinic and National Hospital for Neurology & Neurosurgery, Queen Square, London,WC1N 3BG, 3Department of Medicine (Neuroscience), Monash University, Melbourne, Australia, 4Department of Neurology,University of California, San Francisco (UCSF), San Francisco, CA,USA and 5Department of Clinical Genetics, Churchill Hospital and Weatherall Institute of Molecular Medicine, John Radcliffe Hospital,Oxford,OX3 9DU, UK Correspondence to: Prof. John Collinge, Department of Neurodegenerative Disease and MRC Prion Unit, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, LondonWC1N 3BG, UK E-mail: j.collinge@prion.ucl.ac.uk

The largest kindred with inherited prion disease P102L, historically Gerstmann-Stra«ussler-Scheinker syndrome, originates from central England, with e¤migre¤ s now resident in various parts of the English-speaking world. We have collected data from 84 patients in the large UK kindred and numerous small unrelated pedigrees to investigate phenotypic heterogeneity and modifying factors.This collection represents by far the largest series of P102L patients so far reported.Microsatellite and genealogical analyses of eight separate European kindreds support multiple distinct mutational events at a cytosine-phosphate diester-guanidine dinucleotide mutation hot spot. All of the smaller P102L kindreds were linked to polymorphic human prion protein gene codon 129M andwere not connected by genealogy ormicrosatellite haplotype background to the large kindred or each other. While many present with classical Gerstmann-Stra«ussler-Scheinker syndrome, a slowly progressive cerebellar ataxia with later onset cognitive impairment, there is remarkable heterogeneity. A subset of patients present with prominent cognitive and psychiatric features and some have met diagnostic criteria for sporadic Creutzfeldt-Jakob disease. We show that polymorphic human prion protein gene codon 129 modifies age at onset: the earliest eight clinical onsets were all MM homozygotes and overall age at onset was 7 years earlier for MM compared with MV heterozygotes (P=0.02).Unexpectedly, apolipoprotein E4 carriers have a delayed age of onset by10 years (P=0.02).We found a preponderance of female patients comparedwithmales (54 females versus 30 males,P=0.01), which probably relates to ascertainment bias.However, thesemodifiers had no impact on a semi-quantitative pathological phenotype in 10 autopsied patients.These data allow an appreciation of the range of clinical phenotype, modern imaging andmolecular investigation and should inform genetic counselling of at-risk individuals, with the identification of two genetic modifiers.

snip...

Discussion

snip...

The need for public health control measures, together with the evident diagnostic challenges that IPD heterogeneity causes, make a strong argument for including PRNP gene analysis in he list of investigations for suspected prion disease of any type and indeed of all undiagnosed familial dementia or cerebellar syndromes. Successful diagnosis allows clinicians to provide more accurate prognostic information to patients, to allow participation in the clinical trials and reduce the risk of iatrogenic transmission of disease. As a consequence of these geographically highly mobile ancestors, and the large number of untraced individuals in the nineteenth century who were clearly at risk of inheriting the P102L mutation, it remains likely that further patients and at-risk individuals exist who have yet to be identified. It is hoped that the data presented here will help to raise awareness of P102L IPD and its associated presentations.

snip...end...TSS

http://brain.oxfordjournals.org/cgi/reprint/awn202v2


please see also ;

http://sporadicffi.blogspot.com/


Sunday, August 24, 2008 Sporadic Fatal Insomnia with Unusual Biochemical and Neuropathological Findings

P03.121

Sporadic Fatal Insomnia with Unusual Biochemical and Neuropathological Findings

Giaccone, G1; Mangieri, M1; Priano, L2; Limido, L1; Brioschi, A2; Albani, G2; Pradotto, L2; Fociani, P3; Orsi, L4; Mortara, P4; Tagliavini, F1; Mauro, A2 1Fondazione IRCCS Istituto Neurologico Carlo Besta, Italy; 2IRCCS Istituto Auxologico Italiano, Italy; 3Università di Milano, Ospedale Luigi Sacco, Italy; 4Università di Torino, Dipartimento di Neuroscienze, Italy

Sporadic fatal insomnia (SFI) is a rare subtype of human prion disease, whose clinical and neuropathological phenotype is very similar to familial fatal insomnia (FFI). SFI patients reported until now were all homozygous for methionine at codon 129 of PRNP with deposition of type 2 PrPres (Parchi classification) in the brain. Here we describe a 56-year-old woman who died after a 10-month illness characterized by progressive drowsiness, cognitive deterioration, autonomic impairment and myoclonus. Polysomnography demonstrated a pattern similar to that described in FFI cases with loss of circadian pattern of sleep-wake cycle. A remarkable finding was that 20 years before the onset of symptoms, the patient had undergone surgery for a colloid cyst of the third ventricle, and two ventricular shunts were placed, one correctly in the left ventricle, while the second ended in the right thalamus. The PRNP gene showed no mutation and methionine homozygosity at codon 129. The neuropathologic examination revealed neuronal loss, gliosis, and spongiosis that were mild in the cerebral cortex, while relevant in the caudate nucleus, putamen, thalamus, hypothalamus and inferior olives. In the thalamus, the mediodorsal nuclei were more severely affected than the ventral ones. PrPres immunoreactivity was consistent in the striatum, thalamus and hypothalamus, patchy and of low intensity in the cerebral cortex and absent in the cerebellum. Western blot analysis confirmed this topographic distribution of PrPres. The bands corresponding to di- glycosylated, monoglycosylated and non-glycosylated PrPres were equally represented. The nonglycosylated PrPres band had an electrophoretic mobility identical to that of type 1 by Parchi classification, in the multiple cortical and subcortical regions examined. These findings demonstrate the existence of further rare molecular subtypes of human prion diseases, whose characterization may provide clues for the elucidation of the relation between biochemical characteristics of PrPres and clinico-pathological features of these disorders.

http://www.neuroprion.com/pdf_docs/conferences/prion2007/abstract_book.pdf



Greetings,

IT could also be that this sFFI is just another case of iCJD (via friendly fire from the surgery for a colloid cyst of the third ventricle, and two ventricular shunts were placed, one correctly in the left ventricle, while the second ended in the right thalamus), some 20 years before the onset of symptoms of this so called sFFI case, from some sub-type of sporadic CJD, now called sporadic FFI ???

I believe it was Gambetti et al that coined this term sporadic FFI, from some conspicuous sub-type of sporadic CJD possibly? seems they could not tie it to a true FFI by diagnostic standards to date, so it was then termed a sFFI, confusing matters even worse ;

A subtype of sporadic prion disease mimicking fatal familial insomnia

http://www.neurology.org/cgi/content/abstract/52/9/1757?ck=nck



THIS seems to raise more questions than answers, confusing the TSEs even worse.

WHAT is sporadic CJD, and how many sub-types and atypical strains, phenotypes etc. will there be, arising from nothing. a spontaneous happening of sorts???


http://sporadicffi.blogspot.com/2008/08/sporadic-fatal-insomnia-with-unusual.html



August 19, 2008, Publish Ahead of Print:

First Report of Creutzfeldt-Jakob Disease Occurring in 2 Siblings Unexplained by PRNP Mutation.

Original Article

Journal of Neuropathology & Experimental Neurology. POST EDITOR CORRECTIONS, 19 August 2008 Webb, Thomas E.F. MRCP; Pal, Suvankar MRCP; Siddique, Durrenajaf MRCP; Heaney, Dominic C. MRCP; Linehan, Jacqueline M. BSc; Wadsworth, Jonathan D.F. PhD; Joiner, Susan BSc; Beck, Jon BSc; Wroe, Stephen J. FRCP; Stevenson, Valerie MRCP; Brandner, Sebastian MRCPath; Mead, Simon PhD; Collinge, John FRS

Abstract: Sibling concurrence of pathologically confirmed prion disease has only been reported in association with pathogenic mutation of the prion protein gene (PRNP). Here, we report 2 siblings with classic neuropathologic features of sporadic Creutzfeldt-Jakob disease unexplained by PRNP mutation or known risk factors for iatrogenic transmission of prion infection. Possible explanations include coincidental occurrence, common exposure to an unidentified environmental source of prions, horizontal transmission of disease, or the presence of unknown shared genetic predisposition.

(C) 2008 American Association of Neurop

http://www.jneuropath.com/pt/re/jnen/abstract.00005072-900000000-99931.htm


GEN-07

SPORADIC FATAL INSOMNIA IN A FATAL FAMILIAL INSOMNIA PEDIGREE

S. Capellari1a, P. Cortelli1, P. Avoni1, G.P. Casadei2, A. Baruzzi1, E. Lugaresi1, M. Pocchiari3, P. Gambetti4, P. Montagna1, P. Parchi1. 1Department of Neurological Sciences, University of Bologna, Bologna, Italy; 2Department of Cell Biology and Neurosciences, ISS, Roma, Italy; 3Servizio di Anatomia Patologica, Ospedale Maggiore, Bologna, Italy, 4Division of Neuropathology, CWRU, Cleveland, OH, USA. a mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000208/!x-usc:mailto:capellari@neuro.unibo.it

We describe a case of sporadic fatal insomnia (sFI) occurring in a family in which several members carried the D178N mutation in the PRNP gene and died of fatal familial insomnia (FFI). A 43-year-old woman presented with an 11-month history of diplopia, withdrawal, confusion, memory loss, unsteady gait and inability to sleep with episodes of agitation and dream enactment. After a progressive course characterized by cognitive impairment, marked gait ataxia, signs of autonomic hyperactivity, and myoclonus the patient died 24 months after the onset of symptoms. The patient did not have any personal contact with FFI affected relatives and her closest one was a paternal uncle, the son of her grand-grand mother. Analyses of DNA from various tissues of endo- ecto- and meso-dermal origin, including 5 different regions of the CNS revealed no pathogenic mutations and methionine homozygosity at codon 129 of PRNP. Brain histopathology and PrPSc typing showed typical features of FI such as thalamic and olivary atrophy, focal spongiform degeneration limited to the cerebral cortex, relative sparing of basal ganglia and cerebellum, and relatively low amount of PrPSc type 2A accumulation. sFI represents the rarest among the sporadic human TSE subtypes described to date with less than twenty cases described worldwide and only three cases diagnosed in Italy since the establishment of TSE surveillance. Similarly, only six unrelated FFI families have been observed in Italy to date, making the probability of a chance association between sFI and FFI in the same family extremely low. Thus, we believe that our observation emphasizes the importance of undiscovered factors modulating the susceptibility to human prion diseases. Supported by the EU Network of Excellence "NeuroPrion" (FOOD-CT-2004-506579).



http://www.neuroprion.com/pdf_docs/conferences/prion2006/abstract_book.pdf



http://sporadicffi.blogspot.com/2008/08/sporadic-fatal-insomnia-with-unusual.html




Sunday, August 24, 2008

Sporadic Fatal Insomnia with Unusual Biochemical and Neuropathological Findings P03.121

snip...

for those interested, please see ;


http://sporadicffi.blogspot.com/2008/08/sporadic-fatal-insomnia-with-unusual.html



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


14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

update October 2009

T. Singeltary

Bacliff, TX, USA

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 fed 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.

http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf


International Society for Infectious Diseases Web: http://www.isid.org/


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



P26

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

Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University, Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous address: USDA National Animal Disease Center, Ames, IA 50010, USA

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

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

http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf


Wednesday, July 28, 2010

re-Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE UPDATE July 28, 2010

http://bse-atypical.blogspot.com/2010/07/re-freedom-of-information-act-project.html


Wednesday, March 31, 2010

Atypical BSE in Cattle North America

http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html


*****URGENT NOTE HERE ABOUT OIE AND ATYPICAL BSE*****


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


http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2


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


The familial mutations, Gajdusek proposed, lowered the barrier to such accidental conversion. "Thus," he wrote in 1996, "with these mutations, this ordinarily rare event becomes a ... dominant inherited trait." But Weissmann's qualification still remained to be refuted: the mutations might simply allow easier entry to a lurking virus. ...page 202 Deadly Feast


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


SO, cows can transmit BSE to humans as nvCJD in every country but the USA, but here in the USA, it's a new prionpathy that is genetic, not related to cows, even though one of the USA cows has the same type genetics, and even though Kong et al said that same type cow h-BSE is transmissible to humans. interesting isn't it?


IF you consider the many different TSE strains in different species in North America, and then think 'friendly fire' there from. For a few years now there seems to be a rise here in the U.S.A. of sporadic CJD strains of 'unknown phenotype', with ;

5 Includes 28 cases in which the diagnosis is pending, and 17 inconclusive cases;

6 Includes 28 (24 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded

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


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



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 TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER


>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<


http://creutzfeldt-jakob-disease.blogspot.com/2010/03/irma-linda-andablo-cjd-victim-she-died.html


CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER


http://cjdtexas.blogspot.com/2010/03/cjd-texas-38-year-old-female-worked.html


Creutzfeldt-Jakob Disease Surveillance in Texas

http://cjdtexas.blogspot.com/


Friday, February 05, 2010

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


http://vcjd.blogspot.com/2010/02/new-variant-creutzfelt-jakob-disease.html



Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary

Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.


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



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



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




Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

http://cjdquestionnaire.blogspot.com/




TSS

Second human madcow disease reported in Italy

Il portale informa - Notizie - Caso probabile di malattia di Creutzfeldt-Jakob variante, allevamenti sicuri


Non vi sono nuovi casi umani di "mucca pazza" in Italia e le attuali misure normative e di gestione vigenti sono idonee a garantire la sicurezza degli allevamenti italiani e dei consumatori. Come sottolineato nella nota del 21 giugno, le notizie stampa sulla persona ricoverata presso l’Ospedale di Livorno si riferiscono all’aggravamento del caso a cui la diagnosi di probabile malattia di Creutzfeldt-Jakob era stata effettuata nell’ottobre 2009. Si tratta del secondo caso riscontrato in Italia dopo il primo di diversi anni fa e si ritiene legato ad un’infezione occorsa prima dell’introduzione del divieto di utilizzo delle farine di carne per l’alimentazione dei bovini (dicembre 2000).

Redazione salute.gov.it - 22 luglio 2010


http://www.salute.gov.it/dettaglio/dettaglioNews.jsp?id=1129&tipo=new




Second human madcow disease reported in Italy (AFP) – 2 hours ago

ROME — A 42-year-old Italian woman was reported as the second ever case of madcow disease in humans in the country and is currently hospitalised in desperate conditions, the ANSA news agency said Wednesday.

The woman was diagnosed with a variant of the Creutzfeldt-Jakob disease (CJD) at a Milan neurological hospital in the past months and was then transferred to a hospital in Livorno, in western Tuscany, when she was already in a coma, ANSA said.

It is still unclear how the woman contracted the disease.

In 2009, Italy's health ministry had reported the woman's disease as a "likely variant of the CJD".

The only previous case of madcow disease in humans in Italy was reported in 2002 on the island of Sicily.

On Friday the European Union said it had nearly wiped out madcow disease in animals in Europe.

Only 67 positive cases of madcow disease were identified last year and the animals were old cows that could have been contaminated long ago, the EU said.

In the 1990s, panic erupted after Britain reported a link between madcow and a new form of Creutzfeldt-Jakob disease in humans.

http://www.google.com/hostednews/afp/article/ALeqM5i3cOap1PrjgW2aD_-vZBmUk1snBQ


Una livornese di 42 anni è ricoverata nell'hospice di cure palliative dell'ospedale di Livorno in coma, dopo avere contratto la variante della sindrome di Creutzfeldt-Jakob, il cosiddetto morbo della mucca pazza. La donna è in condizioni disperate ed è ricoverata nel reparto dell'ospedale livornese proprio per effettuare cure che l'aiutino ad affrontare con dignità la fase terminale della malattia. È il secondo caso della malattia registrato in Italia; il primo colpì una donna siciliana nel 2002.

COLDIRETTI: CARNE SICURA «Se confermato, è una eredità del lontano passato facilmente prevedibile, per i lunghi tempi di incubazione della malattia, che non ha nulla a che fare con il consumo della carne italiana, che è del tutto sicuro grazie ad un rigido sistema di controlli introdotto con successo nel 2001 per far fronte all'emergenza Bse». È questo il commento della Coldiretti in riferimento al caso probabile di morbo della "mucca pazza" contratto da una donna di Livorno. «La Bse è praticamente scomparsa da anni dagli allevamenti italiani per l'efficacia delle misure adottate per far fronte all'emergenza come - sottolinea la Coldiretti - il monitoraggio di tutti gli animali macellati sopra i 30 mesi, il divieto dell'uso delle farine animali nell'alimentazione del bestiame e l'eliminazione degli organi a rischio Bse dalla catena alimentare. Ma anche e soprattutto - prosegue la nota - l'introduzione, a partire dal primo gennaio 2002, di un sistema obbligatorio di etichettatura che consente di conoscere l'origine della carne acquistata con riferimento agli Stati di nascita, di ingrasso, di macellazione e di sezionamento, nonch‚ un codice di identificazione che rappresenta una vera e propria carta d'identit… del bestiame e consente di fare acquisti Made in Italy». Secondo la Coldiretti, «a dimostrare che nei bovini la malattia della mucca pazza è ormai quasi completamente debellata sono i numeri forniti dalla Commissione Ue: nell'unione Europea dai 37.000 animali ammalati del 1992 si è passati, nel 2009, a soli 67, dei quali appena due casi in Italia su oltre 450 mila test effettuati».

GIALLO SULL'ORIGINE DELLA MALATTIA Il caso era stato segnalato come «probabile variante della malattia di Cjd » nell'ottobre del 2009 dal ministero della salute. Secondo quanto riporta oggi il quotidiano Il Tirreno nei mesi scorsi la livornese aveva accusato progressivi disturbi neurologici e ha scelto di farsi curare presso l'Istituto neurologico milanese 'Besta' che le ha diagnosticato la malattia e l'ha sottoposta alle terapie senza riuscire ad arrestare l'avanzata del male. La paziente è dunque stata trasferita già in coma presso il reparto di cure palliative dell'ospedale di Livorno. Non è ancora chiaro invece in che modo la paziente abbia contratto la malattia.

LA SCHEDA DELLA MALATTIA Persone relativamente giovani, che presumibilmente hanno mangiato carne di bovini colpiti dalla malattia della «mucca pazza» e che mostrano disturbi psichiatrici, seguiti a breve distanza dalla comparsa di demenza e disturbi nei movimenti di braccia e gambe: sono queste le caratteristiche che permettono di distinguere le persone colpite dalla forma umana della malattia della mucca pazza e quelle colpite dalla classica malattia di Creutzfeldt-Jakob (Cjd). Quest'ultima colpisce infatti le persone anziane (in media 65 anni) e si manifesta con una demenza che peggiora progressivamente, accompagnata da disordini nei movimenti. La variante umana della malattia della mucca pazza è comparsa 14 anni fa in Gran Bretagna, dove è stata subito associata al consumo di carne di animali colpiti dall'Encefalopatia Spongiforme Bovina (Bse). La forma umana è considerata una variante della malattia di Creutzfeldt-Jakob (Cjd) ed il suo nome è stato ufficializzato come «nuova variante della malattia di Creutzfeldt-Jakob» (vCjd) nel 1997, da un articolo pubblicato sulla rivista The Lancet. A scatenare la malattia nell'uomo è l'alterazione di una proteina naturalmente presente nell'organismo, chiamata prione. Dopo il notevole aumento dei casi della malattia sia nei bovini che nell'uomo, concentrato soprattutto fra il 1996 e i primi anni 2000, da qualche anno si registra un notevole rallentamento nella diffusione della malattia sia negli allevamenti e altrettanto rari sono i casi della forma umana. Proprio nei giorni scorsi il commissario europeo alla Salute, John Dalli, ha dichiarato che l'Europa è uscita vincente dalla lotta contro la malattia, che nel 2009 ha fatto registrare 59 negli allevamenti degli Stati membri. L'ultimo caso di Bse in Italia risale al 2008, in Piemonte e l'unico decesso nell'uomo risale al 2002.

http://www.leggo.it/articolo.php?id=73638&sez=ITALIA


Eurosurveillance, Volume 6, Issue 6, 07 February 2002 Articles S Salmaso1

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

Citation style for this article: Salmaso S. First case of vCJD reported in Italy. Euro Surveill. 2002;6(6):pii=2022. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=2022 Date of submission: --------------------------------------------------------------------------------

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

First case of vCJD reported in Italy

The first case of variant Creutzfeld-Jakob Disease (vCJD) has been reported in Italy (1). The case from Sicily was diagnosed in Italy and Great Britain on the basis of clinical and instrumental tests and tonsillar biopsy. The Istituto Superiore di Sanità (ISS) has classified the case as probable, but since the patient is still alive, the ISS has not released any additional information. The surveillance and reporting of CJD has been mandatory in Italy since the beginning of 2002. A ban on feeding mammalian protein to ruminants was approved on 28 July 1984, and enforced from 15 September that year. The prevalence of indigenous cases of bovine spongiform encephalopathy per 10 000 tests was 1.03 for the year 2001 (2).

References : 1.Ministero della Salute. Segnalato caso variante malattia Creutzfeldt-Jakob. Press release 05/02/2002 N° 40, 5 February 2002. (http://www.sanita.interbusiness.it/comunicati_stampa/documenti/040-02.rtf) 2.Centro per lo Studio e le Ricerche sulle Encefalopatie Animali e Neuropatologie Comparate. Focolai BSE in Italia: 2001/2002. (http://www.to.izs.it) Reported by Stefania Salmaso (salmaso@iss.it), Istituto Superiore di Sanità, Italy.

http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=2022


sporadic CJD cases EU

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


For anyone that cares, from 1993 to 2005, a steady increase of sporadic CJD from 27 in 1993, to 104 in 2005, tapering off to 94 in 2006, and 88 in 2007, which seems to correlate to other BSE countries, which to me shows a relationship with human Sporadic CJD rising and falling along the same lines as the BSE cases. Course, nobody cares about that factor because of the UKBSEnvCJD only theory. nobody care that in fact what Collinge, Asante et al showed, that BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein, but who cares about sound science anymore $

http://www.nature.com/emboj/journal/v21/n23/full/7594869a.html


Saturday, July 17, 2010

TSE Road map 2 A Strategy paper on TSE, a road to no where

Brussels, 16.7.2010 COM(2010)384 final COMMUNICATION FROM THE COMMISSION TO THE EUROPEAN PARLIAMENT AND THE COUNCIL

http://transmissiblespongiformencephalopathy.blogspot.com/2010/07/tse-road-map-2-strategy-paper-on-tse.html


Thursday, July 08, 2010

GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/07/global-clusters-of-creutzfeldt-jakob.html


Saturday, July 17, 2010

Variant Creutzfeldt-Jakob disease Ironside JW., Haemophilia. 2010 Jul;16 Suppl 5:175-80

REVIEW ARTICLE

http://vcjdtransfusion.blogspot.com/2010/07/variant-creutzfeldtjakob-disease.html


TSS

GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010

----- Original Message -----
From: Terry S. Singeltary Sr.
To: CJD-L@LISTS.AEGEE.ORG
Cc: BSE-L@LISTS.AEGEE.ORG ; cjdvoice@yahoogroups.com ; BLOODCJD@YAHOOGROUPS.COM
Sent: Thursday, July 08, 2010 9:27 PM
Subject: GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010


CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010



Subject: J.P. MORGAN INVESTORS WARNED ABOUT POTENTIAL SPORADIC CJD AND BSE RELATION
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
Date: Mon, 19 Jan 2004 08:33:38 -0600 Content-Type: text/plain

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

North American Equity ResearchNew York13 January 2004

BSE (Mad Cow) Update: Do Reports of sCJD Clusters Matter?

· There have been seven cases of human sCJD clusters identified in the US in the last 15 years, in which people in a specific location were diagnosed with sCJD, resulting in rates between 2.1 and 8.4 deaths per million people for that specific location compared with the national average of one in 1 million. · There is no proven link between sCJD and BSE, and hence it is considered a different disease from vCJD (which has been linked to BSE). However, the existence of clusters raises the question of “contamination" or “infection”, and also raises the hypothesis that rather than cases of sCJD these might have been cases of vCJD. · Clusters are not spontaneous, they normally have a source. Moreover, some cases of sCJD may have been improperly diagnosed as Alzheimer's.· We continue to believe that as long as no further cases of BSE-positive cows are found in North America and the industry has respected the 1997 ban on animal feed for live cattle, beef consumption in the US will not suffer. · Moreover, due to political pressure we expect key overseas markets (Japan, South Korea, and Mexico) to open up to US beef in the next six months – the recent 20% drop in cattle prices can be attributed mainly to these import bans. · However, two concerns linger and should be kept in mind by investors, 1) Has the 1997 ban on animal feed for live cattle been honored by the beef industry? 2) Can clusters of cases of sporadic CJD (or sCJD) really be a variant of CJD and indeed be linked to BSE? In this note we focus on the issue of sCJD clusters, and the potential impact that the growing debate on clusters could have on beef consumption in the US. United StatesFoods Pablo E. Zuanic(1-212) 622-6744pablo.zuanic@jpmorgan.comChristopher M. Bledsoe(1-212) 622-6386christopher.m.bledsoe@jpmorgan.comDaniel Ogbonna(212) 622-6382daniel.c.ogbonna@jpmorgan.com

State of Our Views Regarding BSE in the US

We continue to believe that as long as no further cases of BSE-positive cows are found in North America and the industry has respected the 1997 ban on animal feed for live cattle, beef consumption in the US will not suffer. Moreover, due to political pressure we expect key overseas markets (Japan, South Korea, and Mexico) to open up to US beef in the next six months – the recent 20% drop in cattle prices can be attributed mainly to these import bans.

However, two concerns linger and should be kept in mind by investors, 1) Has the 1997 ban on animal feed for live cattle been honored by the beef industry? The government's General Accounting Office says it has not; 2) Can clusters of cases of sporadic CJD (or sCJD as it is commonly known) really be a variant of CJD and indeed be linked to BSE (vCJD is the scientific term for the disease linked to mad cow)?

In this note we focus on the issue of sCJD clusters.

Do sCJD Clusters Matter?

The media focus (and as a result, the consumer at large) since December 23, thus far, has been on the potential of new BSE-positive cows being found, and on the various initiatives the authorities are taking to prevent an outbreak of BSE. However, the apparent existence of sCJD clusters in the US has received little publicity. If sporadic (or spontaneous) CJD is really spontaneous, it should not be found in population clusters. The fact that it indeed has been found in clusters raises concerns. Understanding the "Difference" Between sCJD and vCJD

Prior to 1996 there was only one known type of CJD, and it was called “sporadic” or “spontaneous” because it was unclear where it came from, or how it was generated. In 1996 scientists in England "discovered" a "variant" of CJD (vCJD), which they indicated could be linked to the animal form of the disease (BSE or Mad Cow). Experts kept vCJD separate from sCJD because unlike the new vCJD the original sCJD could not be directly linked to BSE. However, not enough is known to be fully certain that sporadic CJD is truly spontaneous and has no external catalyst. The other notable difference between vCJD and sCJD is the incubation period. Whereas sCJD has an average incubation period of 40 years and is exceedingly rare in young people, vCJD can affect people of all ages and has a much shorter incubation period of just two to five years. An even more relevant difference is that sCJD is found in 1 out of 1 million people per annum, or 5,000 cases per year on a global basis, while only 180 human cases of vCJD (the type of CJD linked to BSE) have ever been reported.

Existence of Clusters of sCJD May Imply They Are Really Cases of vCJD There have been seven sCJD clusters identified in the US in the last 15 years, in which people in a specific location were diagnosed with sCJD, resulting in rates between 1.2 and 8.4 deaths per million people for that specific location compared with the national average of one in 1 million. The existence of clusters raises the question of “contamination" or “infection”, and also raises the hypothesis that rather than cases of sCJD these might have been cases of vCJD. Clusters are not spontaneous, they normally have a source.

A cluster consists of two statistical improbabilities: 1) multiple cases occurring in a relatively limited geographic area, and 2) multiple cases occurring within the same time period. The most recent cluster was found in Cherry Hill, New Jersey. The others have been found in Lehigh, Pennsylvania (1986-90), Allentown, Pennsylvania (1989-92), Tampa, Florida (1996-97), Oregon (2001-02), and Nassau County, New York (1999-2000). Given that sCJD occurs randomly in one out of one million cases, it is a statistical rarity to find an sCJD cluster – let alone six. The following tables highlight known clusters in the US. Table 1: Clustered sCJD Deaths

Local sCJD Deaths

Time Span State Local Area Pop. (MM) Period (mo.) Total Ann'lized

1986-1990 PA Lehigh Valley 0.5 48 18 4.5

1989-1992 PA Allentown 2.5 36 15 5.0

1996-1997 FL Tampa 2.2 18 13 8.7

1996-1999 TX Denton .01 38 4 1.3

1999-2000 NY Nassau County 1.3 12 7 7.0

2001-2002 OR Entire State 3.4 24 14 7.0

2000-2003* NJ Cherry Hill Area 1.7 36 12 4.0

Source: JPMorgan.

The second table, below, shows what portion of the state's total expected sCJD cases (as based on a one per million occurrence) were found in the local cluster, comparing the local cluster's portion of cases with the local area's portion of the state's total population. The greater the factor between the former and the latter suggests a higher statistical improbability that the cluster is spontaneous (sCJD).

Table 2: Clustered sCJD Deaths vs. Expected State Cases Annual Statewide Local Area (% of:)

Time Span State Local Area sCJD Deaths* exp. state cases state pop.

1986-1990 PA Lehigh Valley 11.9 37.8% 4.5%

1989-1992 PA Allentown 12.0 41.7% 20.8%

1996-1997 FL Tampa 14.1 61.5% 15.7%

1996-1999 TX Denton 20.9 6.1% .02%

1999-2000 NY Nassau County 18.1 38.7% 7.4%

2001-2002 OR Entire State 3.4 205.9% 100.0%

2000-2003* NJ Cherry Hill Area 8.0 50.0% 21.6%

* *State cases are extrapolated based on state population and the 1 per million national average. Source: JPMorgan.

The CDC Is Currently Investigating the New Jersey Cluster The US Center for Disease Control (CDC) has opened an investigation into a cluster of deaths in an area surrounding Cherry Hill, New Jersey. Specifically, after dismissing the case when it was first brought to their attention earlier in 2003, the agency has since reversed course and on December 31, 2003 sought out information from Janet Skarbek. Skarbek, a Cinnaminson, New Jersey CPA believes she has uncovered a common link between seven deaths in the local area and a restaurant at the now-closed Garden State Race Track. All of the deaths had first been identified as the randomly occurring (one out of one million) cases of sporadic Creutzfeldt-Jakob Disease (sCJD), and six of the deaths occurred between 2000 and 2003.

Science Expanding its Knowledge of CJDs

Have Cases of sCJD Been Overlooked?

Dr. Omar Bagasra believes that a 29 year old that died of presumably sCJD in the New Jersey cluster may have died from a new, mutated form of CJD since sCJD has a typical incubation period of 40 years and is limited to elderly patients in almost all cases. Moreover, he suspects that the link between the seven local deaths (clustered geographically and chronologically) indicates that the new form of the disease is caused by some external catalyst, unlike the randomly occurring sporadic CJD (sCJD). He adds, though, that there may actually have been other unreported CJD-related deaths in the area since the disease is often misdiagnosed as Alzheimer’s.

Diagnoses of Alzheimer’s Might Have Been Cases of CJD

Lawrence Schonberger, the CDC epidemiologist who contacted Janet Skarbek on December 31, is quoted separately as saying that sCJD is underreported on death certificates, and that about 14 percent of cases are missed. In fact, due to similarities between sCJD and Alzheimer’s disease, a 1998 Yale study found that as many as 13 percent of Alzheimer’s deaths are actually sCJD, but conservative estimates place this number closer to 1 percent. If we extrapolate this finding to the 50,000 Alzheimer’s deaths each year in the US, the number of actual sCJD deaths per year is somewhere between 500 and 6500. But, for us this raises additional questions, since at a rate of one per million, the US should not experience much more than 300 sCJD deaths in a single year. Furthermore, Alzheimer cases have grown 50-fold in the last 25 years from 857 cases in 1979 to 50,000 cases in today (albeit part of the increase could very well be attributed to improvements in reporting).

Can sCJD Be Caused by External Agents?

A recent study out of Imperial College in London has led some to believe that the same prions that cause the BSE-related vCJD may also cause a disease that manifests itself in a way that more closely resembles sporadic CJD. John Collinge, the scientist that conducted the experiment, is basing this assertion on findings in the study’s mice, which were injected with BSE prions. As expected, some of the mice developed symptoms from vCJD, but unexpectedly, others suffered from symptoms that more closely resembled sCJD. If true, the implications are significant, as it will force scientists to consider whether cases of sporadic CJD may actually have been caused by consumption of contaminated beef.

Does All This Matter?

For now we await the results of the CDC investigation of the New Jersey cluster. Previous investigations have found clusters to be just coincidences. While this may be the case, we believe that the media may start focusing more on the issue of clusters, and that the debate could raise consumers’ concerns about beef.

But even in the worst case scenario that these clusters were indeed linked to BSE, one could still make the argument that these cases were generated before the 1997 ban on animal feed for cattle was imposed, and that hence chances of contamination since then are unlikely. Still, the question lingers, has the 1997 ban been respected? Will consumers concerns increase as the discussion of CJD clusters hits the national media? Bottom Line: If no new cases of BSE-positive cows are found and the issue of CJD clusters is disregarded by consumers, then the effect on beef consumption will be negligible. On the other hand, new cases of infected cows and/or a wider debate of CJD clusters could indeed have an effect on beef sales.

Analyst Certification

The research analyst who is primarily responsible for this research and whose name is listed first on the front cover certifies (or in a case where multiple analysts are primarily responsible for this research, the analyst named first in each group on the front cover or named within the document individually certifies, with respect to each security or issuer that the analyst covered in this research) that: (1) all of the views expressed in this research accurately reflect his or her personal views about any and all of the subject securities or issuers; and (2) no part of any of the research analyst's compensation was, is, or will be directly or indirectly related to the specific recommendations or views expressed by the research analyst in this research.

JPMorgan Equity Research Ratings Distribution, as of December 31, 2003

Copyright 2003 J.P. Morgan Chase & Co.—All rights reserved. Additional information available upon request.

snip...

Revised December 12, 2003.

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https://lists.aegee.org/cgi-bin/wa?A2=ind0401&L=BSE-L&T=0&F=&S=&P=25337&X=14C6D9515C8B05410B



THE QUESTION OF CLUSTERING OF CREUTZFELDT-JAKOB DISEASE

RICHARD F. RAUBERTAS1, PAUL BROWN2,, FRANÇOISE CATHALA3 and IVOR BROWN4

1Division of Biostatistics, University of Rochester Rochester, NY 2Laboratoire of CNS Studies, NINCDS, NIH Bethesda, MD 3Laboratoire de Neurovirologie, Hópital de Ia Salpetriere Paris, France 4Engineering Division, H.R.B. Singer Co. Lanham, MD

Send reprint requests to: Dr. Paul Brown, Building 3G, Room 5B21, NINCDS, NIH, Bethesda, MD 20892.

Raubertas, R. F., P. Brown (NINCDS, NIH, Bethesda, MD 20892), F. Cathala, and I. Brown. The question of clustering of Creutzfeldt-Jakob disease. Am J Epidemiol 1989;129:146–54.

Clustering of Creutzfeldt-Jakob disease has been reported in several countries. The authors review these reports, and they describe their statistical analysis of clustering among 329 cases that died In France during 1968–1982. Paris was found to have a much higher case rate than the rest of France, white some large areas In the north and west had remarkably few cases relative to their populations. No rural clusters were identified. A number of explanations for regional variations in case rates are possible, including population characteristics and case finding artifacts. Based on their results and those of other studies, the authors conclude that the strongest evidence for clustering of Creutzfeldt-Jakob disease is familial and ethnic, rather than geographic.

Creufeldt-Jakob syndrome; space-time clustering

http://aje.oxfordjournals.org/cgi/content/abstract/129/1/146?ijkey=50059a12d834aa143ff774d15f38881ef24a2fcd&keytype2=tf_ipsecsha




Subject: cjd cluster IDAHO, another 'fluke', another mad cow cjd cover up in the USA

Date: March 11, 2006 at 8:30 pm PST

In Reply to: MAD COW USA JUNK SCIENCE PR COMING OUT BEFORE RECENT SUSPECT BSE CASE EVEN CONFIRMED i.e. preparing for the storm posted

by TSS on March 11, 2006 at 7:31 pm:

Subject: another cjd cluster, another 'fluke', another mad cow cjd cover up in the USA Date: August 16, 2005 at 6:23 am PST

Brain-disease deaths investigated Idaho seeks link in 5 rare cases By Adam Tanner, Reuters August 16, 2005

TWIN FALLS, Idaho -- In late May, Marjorie Skinner played golf well enough to place fourth in a Memorial Day weekend tournament. Yet within weeks, the previously vibrant retiree started losing her ability to speak.

By the time her family buried her Friday, she was the fifth suspected victim in the same sparsely populated area of Idaho of Creutzfeldt-Jakob disease, a rare brain-wasting disease that typically afflicts only one in a million people.

As word of the latest death spread yesterday, local and federal health specialists sifted through clues about an illness different from variant Creutzfeldt-Jakob disease, the human form of mad cow disease.

''Five [cases] in one valley is pretty serious," said Sue Skinner, Marjorie's daughter-in-law. ''It's a grave concern in our family."

The mystery has deepened in recent weeks. At the end of May, another elderly woman died of the incurable disease involving a malformed protein, or prion, that kills brain cells. After that, health officials learned of three other suspected cases, including one CJD death in February that was reported only last month.

''Is what is happening in Idaho an anomaly, a statistical fluke? That is possible," said Ermias Belay, a top CJD expert with the Centers for Disease Control and Prevention in Atlanta who is helping advise officials in Idaho. ''But once it exceeds 1.5 or 2 per million, you start asking questions."

''If they are all confirmed, it could be odd," he said.

In a year, the United States typically has fewer than 300 CJD cases, which mete out rapid death to the elderly, according to the Centers for Disease Control.

In Twin Falls, Cheryle Becker, epidemiology manager for Idaho's South Central District Health, is going to families with detailed questionnaires aimed at finding the roots of a disease that could date back 30 years.

She asks about past travels, unusual hobbies, and dietary habits, including eating organ meats, brain, and venison.

''We're asking them if they've consumed elk," Becker said, adding that many people hunt venison in Idaho. ''We're not having many people say that they have."

Specialists said they do not expect to find a link to eating meat, although locals are asking whether there is any connection to the human variant of mad cow disease. ''It's very frightening to the community," said Cheryl Juntunen, director of the South Central District Health.

Two confirmed US cases of mad cow disease, one in a Washington state dairy animal in 2003 and the other in a Texas beef cow this year, have further heightened concern.

Health specialists have found few parallels among the women, all of European heritage. Four were Idaho natives, all had children, and none had experienced neurological disease.

One had spent time in Britain before the outbreak of mad cow disease there, officials said. Several husbands were involved in farming, as is commonplace in a rural farmland region.

''There are things that lead you to believe this is not variant CJD," Becker said.

© Copyright 2005 Globe Newspaper Company.

http://www.boston.com/news/nation/articles/2005/08/16/brain_disease_deaths_investigated/?rss_id=Boston+Globe+--+National+News




Questions linger in U.S. CJD cases

Published: Oct. 21, 2005 at 9:49 PM By STEVE MITCHELL, Senior Medical Correspondent

SNIP...

The NPDPSC did not respond to UPI's phone call requesting comment about the Idaho cases. The CDC referred UPI to Idaho officials.

Of the nine Idaho cases, three people have tested positive for a CJD-like illness, but officials are conducting further tests to determine whether the disease is sCJD. Two others tested negative and four were buried without autopsies.

The cases could just be a statistical fluke, but the state averages about 1.2 sCJD cases per year and has never had more than three in a single year. The disease is rare and generally is thought to occur at the rate of one case per million people.

Several CJD clusters in other states have far exceeded that rate, however. These included:

--southern New Jersey (2000-2003),

--Lehigh, Pa. (1986-90),

--Allentown, Pa. (1989-92),

--Tampa, Fla. (1996-97),

--Oregon (2001-02), and

--Nassau County, N.Y. (1999-2000).

Some of the clusters involved as many as 18 deaths, and ranged from a rate of four to eight cases per million people.

A group of J.P. Morgan analysts issued an advisory last year on the impact the clusters could have on the beef industry, and said that some of the cases could be due to vCJD.

"The existence of clusters raises the question of 'contamination' or 'infection,' and also raises the hypothesis that rather than cases of sCJD, these might have been cases of vCJD," the advisory said. "Given that sCJD occurs randomly in one out of 1 million cases, it is a statistical rarity to find an sCJD cluster -- let alone six."

If that assessment is accurate, another cluster in Idaho would be even more unlikely.

Another possibility is some of the Idaho cases could be due to chronic wasting disease, which is similar to mad cow disease and currently is epidemic among deer and elk in several states, including Idaho's neighbors Wyoming and Utah.

No human cases of CWD have ever been confirmed, but the disease has been shown to infect human cells in a lab dish. Also, a team of researchers led by Jason Bartz of Creighton University in Omaha, Neb., report in the November issue of the Journal of Virology they had experimentally transmitted CWD to squirrel monkeys --the first reported transmission of CWD to primates.

If CWD is capable of infecting humans, it is unknown whether the resulting disease would resemble sCJD, vCJD or a novel disorder. If the disease looks like sCJD, cases could be going undetected or misdiagnosed.

SEE FULL TEXT ;

http://www.upi.com/Health_News/2005/10/21/Questions-linger-in-US-CJD-cases/UPI-65761129945790/




RE- TAMPA FLORIDA CJD CLUSTER 1996 - 1997


HOW can one be accurate about a cluster of CJD when you cut the age limit of cjd surveillance off at 55 ?

sporadic CJD, and all strains there from, will spread via the medical and surgical arenas, no matter how old you are. so why the age limit if the goal is to stop further spreading and deaths and exposure there from, IF that is what the goal of surveillance is $$$nvCJD$$$ i.e. UKBSEnvCJD only myth, bought and paid for by your local industries responsible. ...TSS


We cooperate with our federal partners at the Centers for Disease Control and Prevention (CDC) for closely investigating cases of CJD that occur in people less than 55 years of age. This age group is where experts feel nv-CJD is most likely to occur. We also take reports from medical providers and the public on clusters of disease.

http://www.doh.state.fl.us/disease_ctrl/epi/htopics/popups/cjd.htm




The Case of the Cherry Hill Cluster


By D.T. Max Published: March 28, 2004


http://artsci.wustl.edu/~anthro/articles/The%20Case%20of%20the%20Cherry%20Hill%20Cluster.htm



http://www.nytimes.com/2004/03/28/magazine/the-case-of-the-cherry-hill-cluster.html



results of state investigation = same old BSe


http://www.state.nj.us/health/eoh/cjd2004.pdf




Annals of Clinical & Laboratory Science, vol. 31, no. 2, 2001

Letter to the Editor:

A Cluster of Creutzfeldt-Jacob Disease Patients from Nassau County, New York, USA

Debasis Adikari and Peter Farmer Department of Pathology, North Shore University Hospital, Manhasset, New York (received 13 January 2001, accepted 18 January 2001)

Keywords: Creutzfeldt-Jacob disease, spongiform encephalopathy, prion, disease cluster Dear Editor

Creutzfeldt-Jacob disease (CJD) is a rare and transmissible neurological disorder, which has been increasing in frequency in the United States over the past two decades [1]. CJD is a spongiform encephalopathy characterized by a conformational change of prion protein [2]. The death rate for CJD in the USA is 0.9 per million [1].

We report 7 cases of CJD from North Shore University Hospital, a community-based teaching institution that serves Nassau County on Long Island, NY. These cases were diagnosed and died during the 12-mo period from mid-June 1999 to midJune 2000. The estimated population of Nassau County in 1997 was 1,281,500, according to the New York State Department of Health [3]. These data suggest a CJD death rate in Nassau County of 5.5 per million, which is approximately 6 times the national rate. During the previous 1-yr period, no case of CJD was diagnosed in our laboratory.

The diagnosis of CJD in the 7 cases was based on pathological examinations performed by at least 2 pathologists. Three cases were confirmed by brain biopsy and 4 were diagnosed at autopsy. In 1 case, CJD was not suspected during life. Pertinent data are summarized in Table 1. The patients’ clinical histories were significant for rapidly deteriorating higher cortical functions, with confusion, speech disorders, nystagmus, ataxia, and/or myoclonus. Only 1 patient had a family history of CJD.

The autopsied brains were grossly unremarkable. At microscopic examination, all specimens showed the pathological features of CJD, including spongy degeneration, loss of neurons, and gliosis in the cerebral cortex. The basal ganglia were also involved. No Kuru plaques or inflammatory changes were noted. The patients’ relatively advanced ages (median = 75 yr, range = 57 to 82 yr), the rapid couse of their disease (median = 8 wk; range = 2 to 10 wk), and the absence of Kuru plaques suggest that these cases represent classical CJD, as opposed to new variant CJD [2].

The authors are concerned that the number of CJD cases in our catchment area appears to have increased dramatically during the 12-mo period. There is no clustering of the patients in any particular neighborhood. While the causes for the apparent increase of CJD cases are unknown, enhanced alertness for the disease and early recognition of its symptoms may be possible explanations. The cluster of CJD patients ought to be investigated thoroughly, which is beyond the scope of this letter.

Address correspondence to Peter Farmer, M.D., Department of Pathology, North Shore University Hospital, 300 Community Drive, Manhasset, NY 11030, USA; tel 516 562 3676; fax 516 562 4591. 0091-7370/01/0200/0211 $0.50; © 2001 by the Association of Clinical Scientists, Inc.

http://www.annclinlabsci.org/cgi/reprint/31/2/211.pdf



J Ky Med Assoc. 2004 Apr;102(4):163-70.

Analysis for space-time clustering of CJD cases, Kentucky, 1988-1997. Groenewold MR.

Louisville Metro Health Department, 400 East Gray Street, Louisville, KY 40201, USA.

Abstract Creutzfeldt-Jakob Disease (CJD) is a transmissible spongiform encephalopathy of humans. It can be transmitted iatrogenically or inherited as an autosomal dominant trait. However, most cases (85%) of the disease are sporadic, occurring stochastically in approximately one person per million population. In 1996, a new, neuropathologically distinct variant of CJD was recognized in a group of unusually young patients who had apparently been infected by exposure to the agent of bovine spongiform encephalopathy (BSE) via contaminated beef products. This prompted many countries, including the United States, to renew their surveillance for CJD to assess the possibility of other variant cases. Spatiotemporal aggregation of cases may indicate a common source of infection or some other shared risk factor. In order to assess the possibility of variant cases, death certificate data was used to examine the spatiotemporal patterns of CJD mortality in Kentucky over a ten-year period. Space-by-time clustering was assessed using Cluster 3.1 software. The findings of this study produced some evidence for a cluster of six cases in central Kentucky. Overall, however, there was little or no evidence to suggest the occurrence of variant or unconventionally acquired cases.

PMID: 15125303 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/pubmed/15125303



Final CJD test results in Woman died from Scjd

Wed Feb 1, 2006 09:09 71.248.143.249

Final CJD test results in Woman died from classic form of brain disease By Sandy Miller Times-News writer

TWIN FALLS -- Final test results on brain tissue have confirmed another Idaho woman died from the classic form of Creutzfeldt-Jakob disease.

"Test results showed it was not the variant form of CJD," said Tom Shanahan, spokesman for the Idaho Department of Health and Welfare, on Tuesday. The variant form of CJD is caused by eating meat from a cow with bovine spongiform encephalopathy, commonly known as mad cow disease.

Since January 2005, the Idaho Department of Health and Welfare has received nine reports of people -- seven women and two men -- diagnosed with Creutzfeldt-Jakob disease -- or CJD -- a fatal brain-wasting disease carried by prions, an abnormal form of protein in the bloodstream. Prions cause folding of normal protein in the brain, leading to brain damage. Symptoms include dementia and other neurological signs. Its victims usually die within four or five months after onset of the disease, according to the Centers for Disease Control and Prevention.

The cases included four women from Twin Falls County, a woman from Minidoka County, a woman from Benewah County in northern Idaho, a woman from Bear Lake County in the southern corner of Idaho on the Utah border, a man from Elmore County and a man from Caribou County in southeastern Idaho.

Of the nine people in Idaho who have died, five had autopsies and their brain tissue was sent to the National Prion Disease Pathology Surveillance Center at Cleveland's Case Western Reserve University.

Of those five, three women -- two women from Twin Falls County and the woman from Benewah County -- tested positive for a prion disease, and final results on all three of them have now shown they died of classic CJD and not the variant form.

Two people, including the Elmore County man and a Twin Falls woman, tested negative for a prion disease.

Autopsies were not performed on the other four suspected CJD victims. However, a CDC neurologist has reviewed their medical records, Shanahan said.

The number of cases is highly unusual. Normally, there is one case of CJD per million people a year. Between 1984 and 2004, Idaho averaged 1.2 cases a year, Shanahan said. He said there was one year during that period when Idaho had three cases.

Because of their ages -- all of the victims except one were over the age of 60 -- health officials suspected they died of classic CJD and not the variant. However, the only way to confirm CJD is by testing brain tissue, according to the National Prion Disease Pathology Surveillance Center.

Times-News writer Sandy Miller can be reached at 735-3264 or by e-mail at smiller@magicvalley.com.

Story published at magicvalley.com on Wednesday, February 01, 2006

http://www.magicvalley.com/articles/2006/02/01/news_localstate/news_local_state.3.txt



Is There A CJD Cluster In Roane County, Tennessee?

Posted on March 16, 2009 by Mad Cow Email

The Tennessee Department of Health is investigating a case of Creutzfeldt-Jakob Disease, and saying in the meantime it is extremely unlikely that it is a human case of the disease known as 'mad cow.'

An early diagnosis of Creutzfeldt-Jakob has been made for one patient, one of 6-8 the state of Tennessee is likely to see this year.

The Centers for Disease Control identifies classic Creutzfeldt-Jakob as a degenerative disease of the nervous system, likely caused by normal prion proteins deforming into abnormal prion proteins. {The bovine spongiform encephalopathy (BSE) disease, more commonly known as 'mad cow' disease, is also a prion disease.}

The state's four cases of Creutzfeldt-Jakob last year were all the classic disease.

One of those commenting said:

My grandmother passed away Feb 4, 2009 at home with CJD. She spent 3 weeks in ParkWest hospital before we recevied her diagnoses. And passed away ten days from the date she was diagnosed. She was 69 years old and from Roane County. Our family did have an autopsy done on her and we have recieved the first phase of it confirming she did have CJD. We have learned of a Man in Rockwood having it and a lady from Kingson in ParkWest with it now. This is a very rare disease and I do believe their has to be a link in Roane County causing it. This disease is horrible and devasting to the family. There is no treatment for it and it progesses very rapid. There is no stopping it or slowing it down once the patient gets it. My mother has contacted the CDC this morning.

And another:

Actually, my father in Roane County was diagnosed with Creutzfeldt-Jakob last week. Our family was contacted by a lady whose mother passed away earlier this year of the same disease, also in Roane County. Furthermore, a family friend visited our house today and told us of yet another diagnosis of a citizen of Roane County, who is currently hospitalized in Knox County. This is devastating both for the families involved and for our county, and considering the statistical rarity of the disease, it seems very strange that three people residing within the same geographical area were diagnosed if the disease is, in fact, acting sporadically. This disease is a monster, and further investigation to identify a possible common link would be extremely beneficial.

Roane County is on I-40 in eastern Tennessee. Its farms and several small towns are home to about 52,000. Go here for the WBIR report.

http://www.madcowblog.com/2009/03/articles/mad-cow-updates/is-there-a-cjd-cluster-in-roane-county-tennessee/



NEWS

Scientists Try To Track Fatal Disease

International Expert Visits Area To Study Unusual Incidence Rate

by ANN WLAZELEK, The Morning Call September 27, 1990

The Lehigh Valley and northeastern Pennsylvania may not have an official "cluster" of the rare and fatal Creutzfeldt-Jakob disease, but an international authority believes the cases are worth studying regardless. Dr. Paul Brown, an epidemiologist with the National Institutes of Health who studied CJD the past 15 years, came to Allentown yesterday because local doctors reported at least 13 cases -- three to six times the normal incidence -- since 1985. That appeared to be an excess, according to Brown, yet whether they are statistically significant or not depends on the denominators of time, space and other factors.

http://articles.mcall.com/1990-09-27/news/2751251_1_brain-disease-cjd-creutzfeldt-jakob-disease



NEWS

Human form of mad cow killed at least 18

By Ann Wlazelek Of The Morning Call January 18, 2004

Government officials say there is only one case of the human form of mad cow disease in the United States -- a young woman living in Florida who probably became infected by eating contaminated beef in Britain, where she lived for the first 12 years of her life. But, 13 years ago, medical specialists in the Lehigh Valley reported a rare cluster of Creutzfeldt-Jakob disease, or the human form of mad cow, that killed as many as 18 residents of Lehigh, Northampton, Carbon, Monroe and Schuylkill counties between 1986 and 1990.

http://articles.mcall.com/2004-01-18/news/3527827_1_variant-cjd-cow-disease-form-of-mad-cow



CJD NE TEXAS CLUSTER

Julie Rawlings of the Texas Department of Health's main office in Austin said CJD was given "reportable" disease status by her agency in 1998, following "a cluster of CJD in northeast Texas" in 1997.

In '97, Rawlings said there were seven confirmed cases of CJD in her agency's Region IV, which extends to Oklahoma, Arkansas and Louisiana, while being connected by Paris, Palestine and Carthage.

"We never found anything," Rawlings said about the concentration of cases in northeast Texas. "We asked enough questions to fill a 70-page questionnaire ... You name it, we probably asked it. Again, we didn't come up with anything significant."

http://www.organicconsumers.org/madcow/palestine41201.cfm



Singeltary family experience with CJD Listserve 22 May 98 Hello, my name is Terry S. Singeltary Sr. and on 12-14-97 my mother died of heidenhan variant CJD, she died a very hidious death. Next, on 12-14-96 exactly one year earlier,my neighbors' mother died from C.J.D. Ii have autopsies to confirm both cases.not to long after my mother had passed away,my neighbor called me and said that I needed to see something. He had been going through a box that he had come across of his mothers. Inside was a bottle of nutritional supplements called IPLEX; INGREDIANTS;VACUUM DRIED BOVINE BRAIN,BONE MEAL,BOVINE EYE,VEAL BONE,BOVINE LIVER POWDER,BOVINE ADRENAL,VACUUM DRIED BOVINE KIDNEY,AND VACUUM DRIED PORCINE STOMACH, it's a cow in a pill. Now this woman taking these pills,died of C.J.D.there was a big article in the Galveston Daily News about all of this. I called the Texas Dept. Of Health and they came and got the pills the next day. Julie Rawlings at the texas dept. of health told me last week that the manufacturer has clammed up on them and will not cooperate anymore. They are referring all matters to their lawyers now.how can this be? Why doesn't the federal government intervEne?

Since the story came out in the galv. news on april 27,1998.a girl called me and told me of her father dying in late 94 or early 95 of C.J.D. in galveston. She told me that my mothers doctor was also her fathers doctor.now my mothers doctor would always mention the OTHER CASE but that's as far as it went.NOW i know why,her father was a BUTCHER AT A MEAT MARKET IN GALVESTON UNTIL HE RETIRED.

Makes me wonder? Did mom ever eat any beef that had come from that meat market in the last 30 or 40 years? MADCOW is here and you can call it whatever you want to. I saw it, my mother died from it. At times she would jerk so bad it would take 3 of us to hold her down.10 weeks start to finish,and she was gone.this disease that they claim is a different disease in younger folks (nvcjd) is the same damn thing. Just because it last longer and the plaques are a little more extreme,could it not be just a more extreme case of C.J.D. any young person with any disease will last longer than a older person with the same disease, because their body and organs are much younger and healthier.

The manufacturer of IPLEX is Standard Process Inc., Palmyra, Wisconsin.1-800-558-8740. I hope you find some interest in this.if you need more details,please don't hesitate to contact me."

With thanks,

Terry S. Singeltary

Texas cluster web site 21 May 98 Mark V. Gregg 512-458-7677 fax 512-458-7616 Director, Public Health Professional Education Texas Department of Health 1100 West 49th Street T-803 Austin, Texas 78756

"We've developed a CJD Web page here at the Texas Department of Health. In addition to some general information, the page links to the CDC's CJD page as well as a 1996 issue of our biweekly morbidity and mortality newsletter, the Disease Prevention News, which is also available on the Web. Our Infectious Disease Epidemiology and Surveillance (IDEAS) Division is currently investigating what we believe to be a cluster of CJD in a small area in East Texas. The Division's number is on the Web page if you wanted to follow up with specifics."

http://www.mad-cow.org/mid_may98.html#aaa



http://www.fortunecity.com/healthclub/cpr/798/terry.htm



Creutzfeldt-Jakob Disease in Northeast Texas,

J.A. Rawlings,*1 K.A. Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2, Texas Department of Health, 1Austin and 2Tyler, Texas

Creutzfeldt-Jacob Disease (CJD), a transmissible spongiform encephalopathy, is caused by prions composed of proteinaceous material devoid of nucleic acid. CJD occurs sporadically (generally 1 case/1,000,000 population per year) in older patients (average age of 65) and is characterized by rapidly progressive dementia, accompanied by severe muscle spasms and incoordination. Death usually occurs within 3 to 12 months (average 7 months). CJD activity in Texas, which has a population of nearly 19 million, appeared to be typical. The statewide death rate for 1995 and 1996 was just under 1/1,000,000. In April of 1997, the Texas Department of Health became aware of an increased number of possible CJD cases in a 23-county area of NE Texas with a population of just over one million. After review of medical and pathology records, four patients were identified with definite classic CJD and three were identified with probable CJD. Dates of death for the eight patients were from April, 1996 through mid-July 1997. The patients were from 46 through 65 years of age; four were male and three were female. A case-control study to identify risks for CJD in NE Texas has been initiated.

http://www.jifsan.umd.edu/tse/Rawlings.htm



North American Equity Research


New York

13 January 2004

BSE (Mad Cow) Update:

Do Reports of sCJD Clusters Matter?

SNIP...SEE FULL TEXT ;

http://cjdtexas.blogspot.com/2007/12/creutzfeldt-jakob-disease-surveillance.html



Friday, October 23, 2009

Creutzfeldt-Jakob Disease Surveillance Texas Data for Reporting Years 2000-2008


http://cjdtexas.blogspot.com/2009/10/creutzfeldt-jakob-disease-surveillance.html




POTENTIAL CJD CLUSTER IN 3 MANITOBANS, another coincidence of more spontaneous BSe

Date: December 8, 2006 at 7:29 am PST Curler among 3 Manitobans suspected of having CJD Last Updated: Friday, December 8, 2006 9:05 AM ET

CBC News Three Manitobans — including a prominent men's curler — are suspected of having the brain-wasting disease Creutzfeldt-Jakob.

Doctors from the Brandon Regional Health Authority have referred three suspected cases of CJD for further testing. They include Neil Andrews, 58, a two-time senior provincial curling champion from Brandon.

Dr. Charles Penner said he doesn't believe the cases are linked, but added that a cluster of cases is even rarer than CJD itself.

As of Nov. 1, six cases of CJD have been reported in Canada this year, says the Public Health Agency of Canada. Since 1997, 720 cases of the degenerative and fatal disease have been referred to the agency.

Andrews was diagnosed with a suspected case of CJD in September, after he had trouble keeping his balance while curling. Doctors at the Mayo Clinic in Rochester, Minn., later told him he likely had CJD.

Earlier this week, a hospital in London, Ont., suspended surgeries for two days when one neurosurgery patient was suspected of having CJD. Preliminary test results came back negative on Tuesday.

About 30 cases of "classical" CJD are diagnosed in Canada every year.

It's the more common, sporadic of two types of CJD, which occurs spontaneously, sometimes because the disease is hereditary. Less than one per cent of the time, it is contracted through hospital or medical procedures.

The second type, variant CJD, is associated with mad-cow disease.

With files from the Canadian Press

http://www.cbc.ca/sports/story/2006/12/08/mba-cjd.html



Human version of mad cow hits Manitoba Three cases suspected from same area in province

The Canadian Press Published: Friday, December 08, 2006 BRANDON, Man. - Three people in Manitoba, including prominent provincial curler Neil Andrews, are suspected of being infected with a degenerative and fatal brain disease, health officials confirmed Thursday.

Doctors from the Brandon Health Region said they have referred three suspected cases of Creutzfeld-Jakob disease, or CJD, for further testing.

CJD infects about one person in every million.

The facts on CJD Q&A about mad cow and CJD Mad cow protein may play role in diabetes More Body & Health news

Dr. Charles Penner, vice-president of medical services for the Brandon Regional Health Authority, doesn't believe the cases are linked, although a cluster of cases is even rarer than the disease.

"For the province to have three cases in one corner of the province, I suppose that would be unusual," he said.

Andrews, a two-time senior provincial men's curling champion, was actually diagnosed with a suspected case of CJD in September. Andrews, 58, noticed something was wrong when he had trouble keeping his balance during his curling delivery.

After a weeklong stay at the Mayo Clinic in Rochester, Minn., doctors told him he likely had CJD.

"(The curlers) knew I couldn't curl and some of them thought that before," he said, laughing, while at a curling club in Brandon in October. "And almost every guy took the time and stopped and talked to me and talked to me openly about the disease and that was good.

"They tried to understand. I'm one of the unfortunate ones who's going to die but they considered themselves lucky."

Typically, one in three suspected cases turns out to be classical CJD, which can lead to rapid brain deterioration, dementia and mobility trouble.

Unlike variant CJD, which has been linked to beef contaminated by mad cow disease, classical CJD cases most often appear sporadically and affect people between 45 and 75. A total of 14 confirmed cases has been reported in Manitoba in the last decade.

Doctors use MRI scans and spinal taps to test for the presence of an abnormal protein to help diagnose a suspected case. But physicians can't confirm anything until an autopsy on brain tissue is performed.

Most people infected with CJD die within a year of the onset of symptoms.

Dr. Michael Coulthard, director of host genetics and prion disease at the National Microbiology Lab in Winnipeg, said the results of the suspected case are still pending. He said there has only been one cluster of cases reported worldwide -- in Switzerland -- in the last five years.

© The Edmonton Journal 2006

http://www.canada.com/vancouversun/news/story.html?id=5ef5f015-0f75-45f1-aac7-c9dd939eb85a&k=66922



CJD Transmission in Canada

There is documentation of six cases in Ontario (five deaths and one diagnosed case) recorded between April 1989 and October 1990.

http://www.phac-aspc.gc.ca/publicat/ccdr-rmtc/96vol22/dr2208ea-eng.php



STATEMENT ON A VARIANT CJD FAMILY CLUSTER

Prof Robert G Will,NCJDSU 25th September 2008r.g.will@ed.a c.uk

Three cases of pathologically confirmed variant CJD have been identified in Spain in recent years, including a man in his early 40s who died earlier this year. The clinical illness in this individual was typical of variant CJD, including the appearances on the MRI brain scan.

A few months ago his mother, who was in her 60s, developed a rapidly progressive neurological illness and died about 5 months from the onset of this illness. An MRI brain scan showed appearances suggestive of variant CJD and preliminary results from post-mortem examination suggest that the suspected diagnosis of variant CJD is correct. Further results, which may confirm this diagnosis, should be available within a few days.

Since 1994 there have been 167 cases of variant CJD in the UK, 23 cases in France and 15 cases in other countries, excluding Spain. The occurrence of variant CJD in more than one member of the same family has not been seen before and it has been the general view that family members of variant CJD cases are not themselves at greater risk of developing this condition. This raises the question as to why two cases of variant CJD have now been found in a family in Spain. There is no evidence of a genetic form of CJD in these Spanish cases and preliminary investigation has not shown any risk of CJD through medical or surgical treatment.

There is no evidence of any risk of transmission of CJD through direct personal contact. The mother and son lived in an area of Spain in which BSE has been found and it is possible that direct consumption of material with high levels of BSE infection may have been the source of the infection. In the UK and other countries it is believed that processed bovine tissues were the most likely source of BSE infection and it is possible that different forms of exposure to BSE infection may explain the occurrence of variant CJD in two family members in Spain and not elsewhere.

This, however, is uncertain and public health policies in relation to variant CJD may have to be reviewed in the light of these two cases in Spain.

http://www.scribd.com/doc/6224550/Memorandum-on-a-Variant-CJD-Family-Cluster



Brain Advance Access originally published online on November 28, 2008 Brain 2009 132(2):493-501; doi:10.1093/brain/awn303

The Author (2008). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Enhanced geographically restricted surveillance simulates sporadic Creutzfeldt-Jakob disease cluster

Genevieve M. Klug1,5,*, Handan Wand2,*, Alison Boyd1,5, Matthew Law2, Scott Whyte3,4, John Kaldor2, Colin L. Masters1,5 and Steven Collins1,5

1 Australian National Creutzfeldt-Jakob Disease Registry, Department of Pathology, The University of Melbourne, Parkville, Victoria, Australia 2 National Centre in HIV Epidemiology and Clinical Research, The University of New South Wales, Sydney, New South Wales, Australia 3 North Sydney and Central Coast Area Health, New South Wales, Australia 4 The University of Newcastle, Newcastle, New South Wales, Australia 5 The Mental Health Research Institute of Victoria, Parkville, Victoria, Australia

Correspondence to: Steven J. Collins, Department of Pathology, The University of Melbourne, Victoria 3010, Australia E-mail: stevenjc@unimelb.edu.au

snip...

Although our strongest suspicion is that the managing clinicians in the Cluster 1 area were well-primed to investigate and confirm CJD cases, it remains possible that other factors unrelated to enhanced surveillance may have contributed to, or explain, the increased number of sCJD in the region. It is conceivable that the cluster could have occurred by chance alone, as apparent clusters are inherent in randomness. This has been seen previously in Australia (Collins et al., 2002a) and with variant CJD in the United Kingdom (Cousens et al., 1999). In Australia, a cluster of six cases in a rural city was identified between 1988 and 2000 and was concluded to have been a chance occurrence. Since this time, no further cases have been confirmed in the area (personal communication, S. Collins) which supports the initial conclusion. In this study, our analysis of case records did not support a definite or likely instance of causal transmission linkage between any cases; however, it cannot be completely dismissed that the Registry's case record details may be incomplete or influenced by recall error from those completing case questionnaires. This leaves the possibility that some of the excess cases may have arisen through covert transmission events. A further limitation of the cluster cohort is the low proportion of cases with complete PRNP genetic analysis, thereby limiting our ability to rule out the possibility of an underlying genetic aetiology in some cases. Finally, a comparison of the age groups of NSW and the Cluster 1 area revealed a small but significant difference between the age structures of the areas with a trend towards an older age population in Cluster 1. Since the disease is age-dependent, it could be speculated that the higher rate simply reflected the older age population. We are confident this is not the explanation given that age-adjustment did not alter the finding that there were significantly more cases of sCJD than expected in Cluster 1.

In conclusion, we believe that vigilant and motivated managing clinicians in a geographically circumscribed area of NSW evinced a sustained higher level of clinical awareness for the broad phenotypic spectrum of CJD with reliable referral of suspect cases for further investigation. In addition, these physicians established and maintained a well coordinated and active approach to suspect CJD autopsy. The combination of these factors translated into a higher intensity of surveillance at approximately twice the rate per population observed for the entire state, culminating in twice the incidence of sCJD at around 2.28 cases/million population/year. The hypothesis that intensity of surveillance for rare disorders such as sCJD can be quantified and correlates positively with disease incidence deserves further exploration and may prove to be an important insight into the varying incidence rates over periods of time within individual nations and between different countries, particularly in light of declining variant and iatrogenic CJD case numbers and a potential waning of awareness of CJD in the years to come. This type of improved understanding could provide strategies to maintain optimal national and potentially global surveillance.

snip....

full text ;

http://brain.oxfordjournals.org/cgi/content/full/132/2/493



Ann Neurol. 2002 Jul;52(1):115-8.

Creutzfeldt-Jakob disease cluster in an Australian rural city. Collins S, Boyd A, Fletcher A, Kaldor J, Hill A, Farish S, McLean C, Ansari Z, Smith M, Masters CL.

Australian National Creutzfeldt-Jakob Disease Registry and Department of Pathology, University of Melbourne, Victoria, Australia. stevencj@unimelb.edu.au

Abstract Through the Australian National Creutzfeldt-Jakob Disease Registry, 6 pathologically confirmed sporadic cases were recognized over a 13-year period in persons who had been long-term residents of a moderate-sized rural city, whereas the expected number was 0.923. An extensive investigation could not find any point-source or case-to-case transmission links. This occurrence is highly statistically significant (p = 0.0027) when viewed in isolation and remains significant (p < p =" 0.001)." view="long&pmid=" risk =" 2.28," p =" 0.021)." aktion="ShowPDF&ArtikelNr=" ausgabe="237323&ProduktNr=" filename="000126916.pdf">doi:10.1016/S0140-6736(05)74351-8Cite or Link Using DOI Cluster of vCJD cases in Kent and its importance Original TextR Salmon a, CEM Hillier a Sir S N Cousens and colleagues1 conclude that “the reported cluster of variant CJD cases in Kent is most probably a chance finding”. This conclusion is made on the basis that, despite a statistical excess of cases based on the assumption of a Poisson distribution, when the UK population is divided up into 36 equal groups of 1·5 million persons (the population of Kent), and 26 cases of variant CJD are allocated randomly to one of these 36 groups, over 10 000 computer simulations, four or more cases occurred within the same group in 18% of simulations. This methodology is a little like a roulette player who, having backed a given square to come up four or more times, expects to be paid if any single square comes up four or more times.

The justification would be that there is nothing special about this area (Kent), compared with any other. However, the initial epidemiological description of BSE gives the reported incidence as greater in southern England and greatest in Kent, leading Wilesmith and colleagues2 to suggest, as an explanation, “geographical variation in the market share of cattle feedstuffs between the compounders” and “a variation between companies in the use and inclusion rate of meat and bone meal”. Further, it was in Kent that some of the early cases of BSE among captive exotic ungulates occurred. Although Cousens and co-workers' conclusion that waterborne spread of infection is improbable is reasonable, it may be premature to rule out other factors that may have operated at a local level such as, for example, a locally distributed foodstuff. Only a local field investigation can elucidate this possibility. Furthermore, this needs to be an investigation tailored to the hypothesis under consideration at a local level, rather than, as seems to have been done, merely abstracting information from the national case control study of CJD. To expect a local consultant in communicable disease control to have the time or resources to undertake such a study may well be unrealistic. However, it would be an appropriate task for a national centre, such as the CJD Surveillance Unit, since any positive findings would have national implications and a negative investigation would offer some direct reassurance and complement the approach that Cousens and colleagues take.

http://www.lancet.com/journals/lancet/article/PIIS0140-6736(05)74351-8/fulltext?version=printerFriendly





I helped Jonathan Leaky of the Sunday London Times track down part of that Queniborough cluster source. ...TSS



Mad cow disease: Could it be here?

Man's stubborn crusade attracts experts notice

August 5, 2001T

he Houston Chronicle by Carol Christian

snip...

Science and environment writer Jonathan Leake of the Sunday Times in London said Singeltary has helped him track down families of people with CJD along with academic research papers.

"I strongly suspect he is right in thinking the USA has had BSE cases," Leake said by e-mail.

"The American government is making the same mistake as the British in putting the short-term commercial interests of its farmers before health considerations," he added.

"It should start formal and widespread testing of cattle plus compulsory autopsies for all human CJD victims at the state's expense. If there is BSE, then leaving it to spread will kill people -- and that would eventually destroy the industry, too."

snip...SEE FULL TEXT ;


http://www.organicconsumers.org/madcow/crusade8501.cfm




Knacker's yard link to Queniborough nvCJD cluster

Sun, 13 Aug 2000 Jonathan Leake and Dipesh Gadher

Sunday Times Additional reporting: Graham Hind

BRITAIN'S worst outbreak of the human form of mad-cow disease may be linked to a nearby knacker's yard that sold meat from diseased animals. The yard operated just eight miles from Queniborough, the Leicestershire village where health officials are investigating the first known cluster of CJD cases.

Three people who spent time in the village died from CJD in 1998, and a fourth person is suspected of having the degenerative brain disease. Another victim lived just three miles away.

The possible link to the knacker's yard - which recycled animals unfit for human consumption into pet food and other products - dates back 20 years, to about the time when scientists now believe the BSE epidemic may have begun.

Two meat traders from Bedfordshire were convicted in 1982 of buying unapproved beef from W E Mason & Sons of Wigston, near Leicester, and selling it to an unsuspecting butcher in Hertfordshire.

Last week officials seized council documents and court reports relating to the company to determine whether any unfit meat may have entered the human food chain locally.

"We have had a very useful series of conversations about this with Oadby and Wigston council," said Philip Monk, a consultant in communicable disease control at Leicestershire health authority, who is heading the Queniborough investigation. "I am ruling nothing in and nothing out. Anything we have that is potentially helpful in explaining local meat trading practices has to be examined."

The case heard by Leicester magistrates in 1982 was the culmination of Operation Meat Hook, a joint investigation between detectives and environmental health officers from three counties.

The teams covertly observed Peter Fletcher, a partner in a wholesale butcher's business near Dunstable, on four occasions in 1980 when he visited Leonard Mason, the yard's owner. He loaded beef carcasses from the yard into an un-marked van, which had been contaminated by a cow's head "fouled by stomach contents", according to evidence given in court. One of the carcasses was later found to have been infected with pleurisy.

Fletcher marked the meat with a fake inspector's stamp, and then left it with a retail butcher near Hemel Hempstead, Hertfordshire.

"A knacker's yard may, and frequently will, deal with diseased cattle," the prosecutor had told an earlier hearing. "Meat may be partly decomposed and contaminated. Disease is rife in such premises and could include anthrax and tuberculosis."

Fletcher was jailed for three months and fined ?500. His partner, Francis Fensome, received a suspended prison sentence. Mason was cleared after telling the court that he had been told the meat was to be used to feed animals at Whipsnade zoo [site of two cheetah BSE fatalities -- webmaster]

The knacker's yard, which had been run by the Mason family since 1947, was closed the same year and now stands derelict. Mason has since died.

Last week his brother, Jack Mason, said: "I am confident there is no connection with us and the outbreak in Queniborough. Most of the meat went to zoos. Any meat that was sold locally went to dog owners as pet food."

There is no proof that Mason dealt in cattle infected with BSE, which was not recognised at the time. But such yards commonly dealt in "downer" cows - those displaying symptoms of illness - so any animals that did have BSE were likely to have ended up in such places.

The Queniborough inquiry team is also examining slaughtering techniques at Leicestershire abattoirs and childhood eating habits of those who grew up in the village, although school meals have been ruled out as a possible cause of the CJD outbreak.

Arthur Beyless lost his daughter, Pamela, 24, a bank worker, to the disease after a two-year struggle for survival. Although the Beylesses live in nearby Glenfield, Pamela regularly visited her grandparents in Queniborough and the family often bought meat from Ian Bramley, the village butcher, in the late 1970s and early 1980s.

Beyless said: "On one occasion I was buying some meat when Ian told me he'd got it for 'a good deal'. It does make you wonder when you consider this theory about the knacker's yard. This disease is something that might never have happened if people weren't always grasping for that last penny."

The other two named victims with links to Queniborough are Stacey Robinson, 19, who lived there for 12 years before moving to another part of the county, and Glen Day, 34, who worked on a farm in the area. He regularly ate at the Horse and Groom pub, which was supplied with meat by Bramley.

Bramley died in a car crash. His stepmother, Hazel Bramley, said she knew nothing about Mason's yard. "We bought our meat directly from local farmers," she said. "The animals were slaughtered in Leicester and delivered to us. I don't know anything about this place in Wigston."


http://www.mad-cow.org/00/aug00_late_news.html#aaa



http://www.mad-cow.org/00/jul00_dont_eat_sheep.html#hhh




18 Jun 00 - CJD - Risk of CJD is higher in north Jonathan Leake

Sunday Times ... Sunday 18 June 2000

Northerners could be at several times more risk from variant CJD , the human form of "mad cow" disease, than those living in the Midlands and south, a study by government scientists has found, writes.

The research, carried out by the Creutzfeldt-Jakob disease Surveillance Unit, also shows that the rate of incidence of the disease, which is always fatal, is rising across Britain .

The figures remain too low to estimate accurately how many people will ultimately be affected. Estimates range from hundreds to many thousands .

Variations in the incidence of the disease are a matter of deep concern . In the north of England - north of Manchester and including Yorkshire and Humberside - there were 3.14 cases per million people over the five years to 1999. Scotland had the second highest rate at 2.98 cases per million .

The West Midlands emerged as the safest place with just 0.36 cases per million. East Anglia and the south experienced, respectively, 0.93 and 1.37 cases per


http://www.mad-cow.org/UKCJD/CJD_news8.html



http://www.mad-cow.org/UKCJD/CJD_news8.html

#18 Jun 00 - CJD - Risk of CJD is higher in north



http://www.mad-cow.org/UKCJD/CJD_news9.html



http://www.mad-cow.org/00/jul00_late_news.html#aaa




Knacker's yard link to Queniborough nvCJD cluster

Sun, 13 Aug 2000 Jonathan Leake and Dipesh Gadher Sunday Times Additional reporting: Graham Hind

BRITAIN'S worst outbreak of the human form of mad-cow disease may be linked to a nearby knacker's yard that sold meat from diseased animals. The yard operated just eight miles from Queniborough, the Leicestershire village where health officials are investigating the first known cluster of CJD cases. Three people who spent time in the village died from CJD in 1998, and a fourth person is suspected of having the degenerative brain disease. Another victim lived just three miles away.

The possible link to the knacker's yard - which recycled animals unfit for human consumption into pet food and other products - dates back 20 years, to about the time when scientists now believe the BSE epidemic may have begun.

Two meat traders from Bedfordshire were convicted in 1982 of buying unapproved beef from W E Mason & Sons of Wigston, near Leicester, and selling it to an unsuspecting butcher in Hertfordshire.

Last week officials seized council documents and court reports relating to the company to determine whether any unfit meat may have entered the human food chain locally.

"We have had a very useful series of conversations about this with Oadby and Wigston council," said Philip Monk, a consultant in communicable disease control at Leicestershire health authority, who is heading the Queniborough investigation. "I am ruling nothing in and nothing out. Anything we have that is potentially helpful in explaining local meat trading practices has to be examined."

The case heard by Leicester magistrates in 1982 was the culmination of Operation Meat Hook, a joint investigation between detectives and environmental health officers from three counties.

The teams covertly observed Peter Fletcher, a partner in a wholesale butcher's business near Dunstable, on four occasions in 1980 when he visited Leonard Mason, the yard's owner. He loaded beef carcasses from the yard into an un-marked van, which had been contaminated by a cow's head "fouled by stomach contents", according to evidence given in court. One of the carcasses was later found to have been infected with pleurisy.

Fletcher marked the meat with a fake inspector's stamp, and then left it with a retail butcher near Hemel Hempstead, Hertfordshire.

"A knacker's yard may, and frequently will, deal with diseased cattle," the prosecutor had told an earlier hearing. "Meat may be partly decomposed and contaminated. Disease is rife in such premises and could include anthrax and tuberculosis."

Fletcher was jailed for three months and fined ?500. His partner, Francis Fensome, received a suspended prison sentence. Mason was cleared after telling the court that he had been told the meat was to be used to feed animals at Whipsnade zoo [site of two cheetah BSE fatalities -- webmaster]

The knacker's yard, which had been run by the Mason family since 1947, was closed the same year and now stands derelict. Mason has since died.

Last week his brother, Jack Mason, said: "I am confident there is no connection with us and the outbreak in Queniborough. Most of the meat went to zoos. Any meat that was sold locally went to dog owners as pet food."

There is no proof that Mason dealt in cattle infected with BSE, which was not recognised at the time. But such yards commonly dealt in "downer" cows - those displaying symptoms of illness - so any animals that did have BSE were likely to have ended up in such places.

The Queniborough inquiry team is also examining slaughtering techniques at Leicestershire abattoirs and childhood eating habits of those who grew up in the village, although school meals have been ruled out as a possible cause of the CJD outbreak.

Arthur Beyless lost his daughter, Pamela, 24, a bank worker, to the disease after a two-year struggle for survival. Although the Beylesses live in nearby Glenfield, Pamela regularly visited her grandparents in Queniborough and the family often bought meat from Ian Bramley, the village butcher, in the late 1970s and early 1980s.

Beyless said: "On one occasion I was buying some meat when Ian told me he'd got it for 'a good deal'. It does make you wonder when you consider this theory about the knacker's yard. This disease is something that might never have happened if people weren't always grasping for that last penny."

The other two named victims with links to Queniborough are Stacey Robinson, 19, who lived there for 12 years before moving to another part of the county, and Glen Day, 34, who worked on a farm in the area. He regularly ate at the Horse and Groom pub, which was supplied with meat by Bramley.

Bramley died in a car crash. His stepmother, Hazel Bramley, said she knew nothing about Mason's yard. "We bought our meat directly from local farmers," she said. "The animals were slaughtered in Leicester and delivered to us. I don't know anything about this place in Wigston."

Bovine spongiform encephalopathy: Epidemiological studies


http://www.thesundaytimes.co.uk/sto/



http://www.mad-cow.org/00/aug00_late_news.html#aaa



http://archives.cnn.com/2001/WORLD/europe/03/21/uk.cjd/




The Queniborough CJD cluster

New claims link CJD to water supply


http://www.mad-cow.org/~tom/drink.html#water



http://www.mad-cow.org/~tom/drink.html



2009 UPDATE WATER AND PRIONS

Wednesday, October 14, 2009

Detection of protease-resistant cervid prion protein in water from a CWD-endemic area


http://chronic-wasting-disease.blogspot.com/2009/10/detection-of-protease-resistant-cervid.html




Eurosurveillance, Volume 5, Issue 12, 22 March 2001 Articles

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

Citation style for this article: Report on Leicestershire vCJD cluster published. Euro Surveill. 2001;5(12):pii=1785. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1785 Date of submission: --------------------------------------------------------------------------------

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

Report on Leicestershire vCJD cluster published

The inquiry team at Leicestershire Health Authority has reported on the results of the investigation into the geographical cluster of five cases of variant Creutzfeld-Jakob Disease (vCJD) around the village of Queniborough. The investigators have concluded that the purchase and consumption of beef in the early 1980’s from butcher’s shops where the meat could have been contaminated with brain tissue from cattle affected with bovine spongiform encephalopathy (BSE) provides a plausible explanation for the cluster (1). A case control study, in which relatives of the five cases and relatives of 30 age-matched controls were interviewed, found that cases were 15 times more likely than controls to have purchased and consumed beef from a butcher who removed brains from cattle (p = 0.0058, 95% C.I. for odds ratio 1.6 – 140). The two butchers linked to four of the five cases removed the brains from cattle that were slaughtered either by the butchers themselves or in a nearby small abattoir. Pithing rods were used during slaughtering, and the carcasses were cleaned by wiping rather than by hosing. Removal of the brain was difficult and messy and the meninges were often ruptured either at removal or by the pithing rod. This led to a risk of cross contamination of carcass meat with brain tissue. Reasons are also given as to why during the early 1980’s the cattle in mixed dairy-beef herds used for the local meat trade may have had higher levels of BSE agent at slaughter than cattle raised for beef alone.

The practice of removing and selling the brains of cattle as food was legal in the United Kingdom throughout the 1980’s. Since 1989 it has been illegal for cattle brains to be used for human consumption and since 1996 the whole head of cattle over six months must be disposed of in a slaughterhouse as specified risk material.

The current number of definite and probable cases of vCJD in the UK is 97 (2). Of these, seven are probable cases who are still alive. Although there are other geographical areas with more than one case, to date Queniborough is the only area where statistical analysis suggests the association between the cases is unlikely to have occurred by chance.

References : Bryant G, Monk P. Summary of the final report of the investigation into the North Leicestershire cluster of variant Creutzfeld-Jakob disease. Leicester: Leicestershire NHS Health Authority, 2001. Available online at . Queniborough vCJD cluster report - Department of Health statement [press release 2001/0141]. London: Department of Health, 21 March 2001. Available online at


http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1785



http://www.parliament.uk/documents/post/pn171.pdf



http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=1724




Tue, 8, Aug 2000 19:39:27 -0400

From: jonathan leake

Date: Tue, 8, Aug 2000 19:39:27 -0400

Subject: IN CONFIDENCE (I SMELL A STORY ......)

Sender: jonathan leake

To: BSE Terry Singletary

Message-ID: <200008081939_mc2-af13-1bc@compuserve.com> MIME-Version: 1.0 Content-Type: text/plain; charset=ISO-8859-1 Content-Disposition: inline Content-Transfer-Encoding: 8bit X-MIME-Autoconverted: from quoted-printable to 8bit by sys44.hou.wt.net id SAA15659 X-Mozilla-Status: 8007 X-Mozilla-Status2: 00000000 X-UIDL: ed0acd360d74370a3e06000000000000

Hi Terry - this is Jonathan Leake here.

we're thinking of doing a story on the knackers yard meat issue - is there a link to Queniborough? Would you mind resending any info you have on this - I may have lost some of the stuff you sent.

Cd you send it to

jonathan.leake@suandy-times.co.uk

AND TO

dipesh.gadher@sunday-times.co.uk

- HE'S RESEARCHING THIS STORY FOR ME AS I'M AT A CONFERENCE

MANY THANKS FOR YOUR HELP - AND FOR ALL THE GOOD WORK YOU'VE BEEN DOING

snip...end...TSS



Re: IN CONFIDENCE (I SMELL A STORY )

Subject: Re: IN CONFIDENCE (I SMELL A STORY )

Date: Tue, 08 Aug 2000 21:41:57 -0700

From: "Terry S. Singeltary Sr."

To: jonathan leake


Hello Jonathan,


yes, give me some time though. there is a shitstorm on CJD Voice, they let the Faillace's on the CJD Voice support group (TSE tainted sheep farmers) without telling anyone; and myself and other are pissed off to say the least. This was suppose to be a support group. i told them it would be like asking the Malboro Man on a Cancer List. But he is Dead. Maybe it struck a nerve.


Have you got the DFA 4, 5, and 7, i thought i read something about knackers or maybe babyfoods??? not sure. i can send to you. I am sure i have something in the GBR's for the states and the other countries, don't have time to read. you can read them at; http://europa.eu.int/comm/food/fs/sc/ssc/outcomeen.html#reports i will search as soon as i get time ....


kind regards, Terry



jonathan leake wrote:


Hi Terry - this is Jonathan Leake here. we're thinking of doing a story on the knackers yard meat issue - is there a link to Queniborough? Would you mind resending any info you have on this - I may have lost some of the stuff you sent. ...snip...END...TSS


Re: KNACKERS AND RENDERS

Subject: Re: KNACKERS AND RENDERS

Date: Thu, 10 Aug 2000 16:04:14 ·0700

From: "Terry S. Singeltary Sr."

To: jonathan.leake@sunday-times.co.uk, dipesh.gadher@Sunday-times.co.uk

do you have access to the;

The Veterinary-Record, December 20/27, 1997 Papers and Articles Effect of rendering procedures on the scrapie agent D. M. Taylor, S.L. Woodgate, A.J. Fleetwood, R.J.G. Cawthorne it's about 6 or 7 pages. i do not have it scanned and it's fairly fine print, however good print. also the report; The Veterinary Record, March 2, 1991 Papers and Articles Bovine Spongiform Encephalopathy: epidemiological studies on the origin there is a good section of rendering; Survey of rendering processes, solvents etc (very detailed on temps and processes) can scan copy correct and paste, but it will take some time, or fax COLLECT to you. I'm running out of quarters fast, nobody paying me to do this, and i am on disablility. so the fax will have to be collect ... regards, Terry 1 of 1 8/13/00 1 :06 PM

end...TSS



Date: Fri, 2 Mar 2001 23:27:10 +0000 (GMT)

From:

Subject: confidential

To: "Terry S. Singeltary Sr."

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.

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


snip...



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



http://collections.europarchive.org/tna/20080102161333/http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf



http://web.archive.org/web/20040823105233/www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf



http://collections.europarchive.org/tna/20080102172428/http://www.bseinquiry.gov.uk/files/sc/seac17/tab03.pdf



http://collections.europarchive.org/tna/20080102193705/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf




snip...


ANOTHER CJD CONFIRMED IN IDAHO CLUSTER (Scjd) Wed Feb 1, 2006 08:07 71.248.143.249


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



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



Thursday, July 08, 2010

Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions Public release date: 8-Jul-2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/07/nosocomial-transmission-of-sporadic.html



please note ;

Original Paper

Surgery and Risk of Sporadic Creutzfeldt-Jakob Disease in Denmark and Sweden: Registry-Based Case-Control Studies Ignacio Mahillo-Fernandeza, Jesús de Pedro-Cuestaa, Maria José Bledaa, Mabel Cruzb, Kåre Mølbakc, Henning Laursend, Gerhard Falkenhorstc, Pablo Martínez-Martína, Åke Sidenb, on behalf of the EUROSURGYCJD Research Group

aDepartment of Applied Epidemiology, National Center for Epidemiology, and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Carlos III Institute of Health, Madrid, Spain; bDepartment of Clinical Neurosciences, Neurology Division, Karolinska Institutet, Stockholm, Sweden; cDepartment of Epidemiology, Statens Serum Institute, and dNeuropathology Laboratory, Rigshospitalet, Copenhagen, Denmark

Address of Corresponding Author

Neuroepidemiology 2008;31:229-240 (DOI: 10.1159/000163097)

Abstract

Background: Epidemiologic evidence of surgical transmission of sporadic Creutzfeldt-Jakob disease (sCJD) remains controversial. Methods: From Danish and Swedish registries we selected 167 definite and probable sCJD cases (with onset between 1987 and 2003) and 3,059 controls (835 age-, sex-, and residence-matched, and 2,224 unmatched). Independent of case/control status, surgical histories were obtained from National Hospital Discharge Registries. Surgical procedures were categorized by body system group and lag time to onset of sCJD. Exposure frequencies were compared using logistic regression. Results: A history of any major surgery, conducted 20 years before sCJD onset, was more common in cases than both matched (OR = 2.44, 95% CI = 1.46-4.07) and unmatched controls (OR = 2.25, 95% CI = 1.48-3.44). This observation was corroborated by a linear increase in risk per surgical discharge (OR = 1.57, 95% CI = 1.13-2.18; OR = 1.50, 95% CI = 1.18-1.91). Surgery of various body systems, including peripheral vessels, digestive system and spleen, and female genital organs, was significantly associated with increased sCJD risk. Conclusions: A variety of major surgical procedures constitute a risk factor for sCJD following an incubation period of many years. A considerable number of sCJD cases may originate from health care-related accidental transmission.

Copyright © 2008 S. Karger AG, Basel

snip...

Discussion Overall, the present study indicates that a considerable proportion of sCJD may constitute a health care-related disorder, accidentally transmitted during surgery. While this has been suggested before [7–10] , the present study is unique because of the unbiased assessment of exposure histories for decades before disease onset, randomly chosen controls, and strict lag time measurement. The lack of surgical history data prior to the establishment of the National Hospital Discharge Registries in the early 1970s and low statistical power preclude the assessment of early- in-life surgery or specific infrequent procedures. The main findings were supported by analyses including both MCs and UMCs. The UMCs were not essential for this study, but it was reassuring to learn that a similar study could be undertaken in another setting where it was impossible to sample MCs as a reference group.

snip...

Conflicting reports from previously published casecontrol studies regarding the association between surgery and sCJD may partially be explained by variations in the type of control subjects used and in exposure assessment [31] . Even though we did not find a significant association between surgery performed at any time predating 1 1 year before onset, our results are consistent with positive results for lifetime surgical history found in recent, large studies using community controls [7, 9, 10] . Like us, one study reports dose-response effects [7] . Hence, in accordance with these studies and other reports suggesting that some types of surgery, e.g. cataract surgery [32, 33] , might be performed as a consequence of early sCJD manifestations, it appears that surgery may constitute: (1) a risk factor of sCJD with a considerable time lag, and (2) a risk indicator with yet unknown causal links for specific types of (recent) surgery, possibly including coronary surgery.

What then are the potential implications for public health? Based on period-specific ORs of 1.44 (10–19 years) and 2.44 ( 6 20 years), and 22 and 19% exposed, unrepeated cases for each window, we estimate the population- attributable proportion to be 18%. This figure may constitute a considerable underestimation of the effect of lifetime surgical history on sCJD incidence, because in our study the ascertainment of exposure to surgery before the middle of the 1970s was compromised by the incompleteness of hospital discharge registries. In the UK, a positive lifetime surgical history was recorded from 1980 onwards in 58 and 59% of vCJD cases and controls, with median ages of 26 and 33 years [34] . Since the

majority of surgical interventions at such ages was not captured in our study, and our results suggest that the lower the age at surgery, the higher the risk, the figure of 35% proposed by Ward et al. [9] for the population-attributable risk of sCJD due to lifetime surgery in the UK would appear to be a conservative estimate for Denmark and Sweden.

While negative results have been reported for clustered surgical chains [35] , studies supporting the hypothesis of a frequent surgical transmission of sCJD might be a French cluster of multioperated cases [36] ; the high sCJD incidence in regions with a high incidence of genetic transmissible spongiform encephalopathy, potentially acting as a focus for point source epidemics, such as in the Spanish Basque Country [37] (http://www.isciii. es/htdocs/pdf/DatosRegistroCreutzfeldJacob.ppt), and occasional increases in CJD incidence in countries where iatrogenic transmission has been mentioned as a possible cause [38] .

To conclude, we provide evidence to indicate that surgery, acting with long incubation periods, has constituted a risk factor for sCJD in Sweden and Denmark. The associations may have implications for precautionary measures and surveillance.


http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=000163097



http://content.karger.com/ProdukteDB/produkte.asp?Aktion=ShowPDF&ArtikelNr=000163097&Ausgabe=240333&ProduktNr=224263&filename=000163097.pdf



Cross infection control measures and the treatment of patients at risk of Creutzfeldt Jakob Disease in UK general dental practice

J. Bagg,1 C. P. Sweeney,2 K. M. Roy,3 T. Sharp,4 and A. Smith,5

Aims To determine the suitability of key infection control measures currently employed in UK dental practice for delivery of dental care to patients at risk of prion diseases.

Materials and methods Subjects: Five hundred dental surgeons currently registered with the General Dental Council of the UK.

Data collection: Structured postal questionnaire.

Analysis: Frequencies, cross-tabulations and chi-squared analysis. Results The valid response rate to the questionnaire was 69%. 33% of practices had no policy on general disinfection and sterilisation procedures. Only 10 of the 327 responding practices (3%) possessed a vacuum autoclave. 49% of dentists reported using the BDA medical history form but less than 25% asked the specific questions recommended by the BDA to identify patients at risk of iatrogenic or familial CJD. However, 63% of practitioners would refer such patients, if identified, to a secondary care facility. Of the 107 practitioners who were prepared to provide dental treatment, 75 (70%) would do so using routine infection control procedures.

Conclusions Most of the dental practices surveyed were not actively seeking to identify patients at risk of prion diseases. In many cases, recommended procedures for providing safe dental care for such patients were not in place.


http://eprints.gla.ac.uk/185/1/Bagg[1].pdf




AFTER consumption and or exposure, then the pass it forward and or friendly fire begins ;



Thursday, May 27, 2010 Guidance for Industry: Revised Preventive Measures to Reduce Possible Risk of Transmission of CJD and vCJD by blood and blood products; Availability

[Federal Register: May 27, 2010 (Volume 75, Number 102)] [Notices] [Page 29768-29769] From the Federal Register Online via GPO Access [wais.access.gpo.gov] [DOCID:fr27my10-66]

http://vcjdtransfusion.blogspot.com/2010/05/guidance-for-industry-revised.html



Tuesday, May 11, 2010

Current risk of iatrogenic Creutzfeld-Jakob disease in the UK: efficacy of available cleaning chemistries and reusability of neurosurgical instruments

http://creutzfeldt-jakob-disease.blogspot.com/2010/05/current-risk-of-iatrogenic.html



Tuesday, March 16, 2010

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Part 4 REVISED FEB. 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/03/transmissible-spongiform-encephalopathy.html




ATYPICAL BSE MORE VIRULENT THAN C-BSE, USDA AND OIE ARE POISONING THE GLOBE LIKE THE U.K. DID $$$


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

http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2



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



14th ICID International Scientific Exchange Brochure -

Final Abstract Number: ISE.114

Session: International Scientific Exchange

Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America

update October 2009

T. Singeltary

Bacliff, TX, USA

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 fed 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.


http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf



International Society for Infectious Diseases Web: http://www.isid.org/


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

P02.35

Molecular Features of the Protease-resistant Prion Protein (PrPres) in H-type BSE

Biacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron, TGM1 1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, Sweden

Western blot analyses of PrPres accumulating in the brain of BSE-infected cattle have demonstrated 3 different molecular phenotypes regarding to the apparent molecular masses and glycoform ratios of PrPres bands. We initially described isolates (H-type BSE) essentially characterized by higher PrPres molecular mass and decreased levels of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type is also distinct from another BSE phenotype named L-type BSE, or also BASE (for Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low representation of the diglycosylated PrPres band as well as a lower PrPres molecular mass. Retrospective molecular studies in France of all available BSE cases older than 8 years old and of part of the other cases identified since the beginning of the exhaustive surveillance of the disease in 20001 allowed to identify 7 H-type BSE cases, among 594 BSE cases that could be classified as classical, L- or H-type BSE. By Western blot analysis of H-type PrPres, we described a remarkable specific feature with antibodies raised against the C-terminal region of PrP that demonstrated the existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in cattle seems to by higher compared to the PrPres#1. Furthermore another PK–resistant fragment at about 7 kDa was detected by some more N-terminal antibodies and presumed to be the result of cleavages of both N- and C-terminal parts of PrP. These singular features were maintained after transmission of the disease to C57Bl/6 mice. The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band) reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.

http://www.neuroprion.com/pdf_docs/conferences/prion2007/abstract_book.pdf




Wednesday, March 31, 2010

Atypical BSE in Cattle

http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html



Tuesday, November 17, 2009

SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1

http://bse-atypical.blogspot.com/2009/11/seac-new-results-on-idiopathic.html




NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS "All of the 15 cattle tested showed that the brains had abnormally accumulated PrP" 2009

http://bse-atypical.blogspot.com/2009/02/new-results-on-idiopathic-brainstem.html




AND THE USDA ET AL KNEW IT TOO ;


""These 9,200 cases were different because brain tissue samples were preserved with formalin, which makes them suitable for only one type of test--immunohistochemistry, or IHC."

THIS WAS DONE FOR A REASON!

THE IHC test has been proven to be the LEAST LIKELY to detect BSE/TSE in the bovine, and these were probably from the most high risk cattle pool, the ones the USDA et al, SHOULD have been testing. ...TSS

USDA 2003

We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back. Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS.

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.

snip.............

Dr. Detwiler: It seems a good idea, but I'm not aware of it. Another important thing to get across to the public is that the negatives do not guarantee absence of infectivity. The animal could be early in the disease and the incubation period. Even sample collection is so important. If you're not collecting the right area of the brain in sheep, or if collecting lymphoreticular tissue, and you don't get a good biopsy, you could miss the area with the PRP in it and come up with a negative test. There's a new, unusual form of Scrapie that's been detected in Norway. We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS .

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.

snip...

Completely Edited Version PRION ROUNDTABLE

Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado

end...TSS


ONE FINAL COMMENT PLEASE, (i know this is long Dr. Freas but please bear with me)

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

These are the facts as i have come to know through daily and extensive research of TSE over 9 years, since 12/14/97. I do not pretend to have all the answers, but i do know to continue to believe in the ukbsenvcjd only theory of transmission to humans of only this one strain from only this one TSE from only this one part of the globe, will only lead to further failures, and needless exposure to humans from all strains of TSE, and possibly many more needless deaths from TSE via a multitude of proven routes and sources via many studies with primates and rodents and other species. ...

SNIP... SEE FULL TEXT

PAGE 1 STARTS ON PAGE 13, SKROLL TO PAGE 13...TSS

http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8



USA DEAD STOCK DOWNER COWS AND TME

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



PLEASE be aware, for 4 years, the USDA fed our children all across the Nation dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens. who will watch our children for CJD for the next 5+ decades ???

SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE

http://downercattle.blogspot.com/2009/05/who-will-watch-children.html



http://downercattle.blogspot.com/



WHAT MAD COW FEED BAN ???

Tuesday, March 2, 2010

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

http://madcowfeed.blogspot.com/2010/03/animal-proteins-prohibited-in-ruminant.html



Monday, March 1, 2010

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

http://madcowfeed.blogspot.com/2010/03/animal-protien-ie-mad-cow-feed-in.html



Terry S. Singeltary Sr. (Submitted question): Monday, April 5, 2010

Update on Feed Enforcement Activities to Limit the Spread of BSE April 5, 2010

http://madcowfeed.blogspot.com/2010/04/update-on-feed-enforcement-activities.html



Saturday, June 12, 2010

PUBLICATION REQUEST AND FOIA REQUEST Project Number: 3625-32000-086-05 Study of Atypical Bse

http://bse-atypical.blogspot.com/2010/06/publication-request-and-foia-request.html



Archive Number 20100405.1091 Published Date 05-APR-2010

Subject PRO/AH/EDR> Prion disease update 1010 (04)

snip...

[Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed

.

The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"

http://www.promedmail.org/pls/apex/f?p=2400:1001:568933508083034::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,82101



Saturday, June 5, 2010

Research Project: Transmissible Spongiform Encephalopathies: Identification of atypical scrapie in Canadian sheep

http://nor-98.blogspot.com/2010/06/research-project-transmissible.html



Heidenhain Variant Creutzfeldt Jakob Disease autopsy case report 'MOM'

DIVISION OF NEUROPATHOLOGY University of Texas Medical Branch 114 McCullough Bldg. Galveston, Texas 77555-0785

FAX COVER SHEET

DATE: 4-23-98

TO: Mr. Terry Singeltary @ -------

FROM: Gerald Campbell

FAX: (409) 772-5315 PHONE: (409) 772-2881

Number of Pages (including cover sheet):

Message:

*CONFIDENTIALITY NOTICE*

This document accompanying this transmission contains confidential information belonging to the sender that is legally privileged. This information is intended only for the use of the individual or entry names above. If you are not the intended recipient, you are hereby notified that any disclosure, copying distribution, or the taking of any action in reliances on the contents of this telefaxed information is strictly prohibited. If you received this telefax in error, please notify us by telephone immediately to arrange for return of the original documents. -------------------------- Patient Account: 90000014-518 Med. Rec. No.: (0160)118511Q Patient Name: POULTER, BARBARA Age: 63 YRS DOB: 10/17/34 Sex: F Admitting Race: C

Attending Dr.: Date / Time Admitted : 12/14/97 1228 Copies to:

UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 Pathology Report

FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858

Autopsy NO.: AU-97-00435

AUTOPSY INFORMATION: Occupation: Unknown Birthplace: Unknown Residence: Crystal Beach Date/Time of Death: 12/14/97 13:30 Date/Time of Autopsy: 12/15/97 15:00 Pathologist/Resident: Pencil/Fernandez Service: Private Restriction: Brain only

FINAL AUTOPSY DIAGNOSIS

I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.

http://creutzfeldt-jakob-disease.blogspot.com/2008/07/heidenhain-variant-creutzfeldt-jakob.html



Sent: Friday, April 16, 2010 11:38 AM Subject: PRO-MED ATYPICAL SCRAPIE

Background ----------- "Retrospective studies have identified cases predating the initial identification of this form of scrapie, and epidemiological studies have indicated that it does not conform to the behaviour of an infectious disease, giving rise to the hypothesis that it represents spontaneous disease. However, atypical scrapie isolates have been shown to be infectious experimentally, through intracerebral inoculation in transgenic mice and sheep. [Many of the neurological diseases can be transmitted by intracerebral inoculation, which causes this moderator to approach intracerebral studies as a tool for study, but not necessarily as a direct indication of transmissibility of natural diseases. - Mod.TG]

"The 1st successful challenge of a sheep with 'field' atypical scrapie from an homologous donor sheep was reported in 2007.

"Results -------- "This study demonstrates that atypical scrapie has distinct clinical, pathological, and biochemical characteristics which are maintained on transmission and sub-passage, and which are distinct from other strains of transmissible spongiform encephalopathies in the same host genotype.

"Conclusions ------------ Atypical scrapie is consistently transmissible within AHQ homozygous sheep, and the disease phenotype is preserved on sub-passage."

Lastly, this moderator wishes to thank Terry Singletary for some of his behind the scenes work of providing citations and references for this posting. - Mod.TG]

The HealthMap/ProMED-mail interactive map of Australia is available at . - Sr.Tech.Ed.MJ]


http://www.promedmail.org/pls/otn/f?p=2400:1001:962575216785367::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1000,81729



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



http://nor-98.blogspot.com/



Friday, May 14, 2010

Prion Strain Mutation Determined by Prion Protein Conformational Compatibility and Primary Structure

Published Online May 13, 2010 Science DOI: 10.1126/science.1187107 Science Express Index

http://chronic-wasting-disease.blogspot.com/2010/05/prion-strain-mutation-determined-by.html



Thursday, June 03, 2010

Prion Strain Mutation and Selection John Collinge

MEDICINE

http://chronic-wasting-disease.blogspot.com/2010/06/prion-strain-mutation-and-selection.html



TRADE, TRADE, TRADE, TO HELL WITH HUMAN HEALTH, and a disease that will kill you after your term is over, just pass it on. I have written to every President since this nightmare began. and that is what this disease does, you pass it forward via a multitude of proven routes and sources via the medical, dental, and surgical arena's once exposed. what the USA is doing now, is the same thing the UK did with their mad cow disease, when they poisoned the globe. it's all about money $$$

DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN

''they don't wanna know, the dont' care''

http://maddeer.org/video/embedded/prusinerclip.html



Saturday, June 19, 2010

U.S. DENIED UPGRADED BSE STATUS FROM OIE

see full text and reasons why here ;

http://usdameatexport.blogspot.com/2010/06/us-denied-upgraded-bse-status-from-oie.html



TSE

please read Senate 'Down Under' TSS SUBMISSION TO HANSARD SENATE VIA ABA, link at bottom of the following url ;

http://transmissiblespongiformencephalopathy.blogspot.com/



RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 26 March 2003

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



re-Human Prion Diseases in the United States

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


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



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


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



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



IF you consider the many different TSE strains in different species in North America, and then think 'friendly fire' there from. For a few years now there seems to be a rise here in the U.S.A. of sporadic CJD strains of 'unknown phenotype', with ;

5 Includes 28 cases in which the diagnosis is pending, and 17 inconclusive cases;

6 Includes 28 (24 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded

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



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



ATYPICAL BSE MORE VIRULENT TO HUMANS THAN UK STRAIN

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7 December 2006 are now available.

snip...

64. A member noted that at the recent Neuroprion meeting, a study was presented showing that in transgenic mice BSE passaged in sheep may be more virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A MUTATION FOUND IN CASES OF SPORADIC CJD.

snip...

http://www.seac.gov.uk/minutes/95.pdf



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 TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER



>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<


http://creutzfeldt-jakob-disease.blogspot.com/2010/03/irma-linda-andablo-cjd-victim-she-died.html



CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

http://cjdtexas.blogspot.com/2010/03/cjd-texas-38-year-old-female-worked.html



Creutzfeldt-Jakob Disease Surveillance in Texas

http://cjdtexas.blogspot.com/



Friday, February 05, 2010

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

http://vcjd.blogspot.com/2010/02/new-variant-creutzfelt-jakob-disease.html




Friday, November 30, 2007

CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION

http://cjdquestionnaire.blogspot.com/




IN CLOSING, I wish to say that if anyone still thinks that 85% to 90% of all sporadic CJD is a spontaneous happening without any route and source of the TSE agent, just a happen stance of bad luck, as some officials still claim today, as with every hospital that has a CJD exposure accident will tell you, if anyone still believes this, they should then go and resign from whatever scientific and or doctors field you practice in, because YOU are then partially responsible for the continued spread of this horrible disease around the Globe. For Pete's sake, if clusters happen with animal TSE, then why not humans? IF all these TSE transmit to many different animal species, both in the field and in the lab, what make's it so hard to believe that it will not transmit to humans? IF all these TSE in all these many different species, with all these many different strains now appearing, both typical and atypical, with over 20 strains in just typical scrapie alone, nor-98 atypical scrapie, with BSE freely transmitting to sheep as well, now 4 BSE strains in cattle i.e. c-BSE, l-BSE, h-BSE, and IBNC (prion gods will have to admit this is a prion TSE disease sooner or later), now 2 strains of CWD documented i.e. CWD-1 and CWD-2, and the TME with the drowsy TME strain and the hyper TME strain, if all this has been fed to humans and to livestock producing animals for human and animal consumption, then what would human TSE there from look like, either from consumption, or 2nd, 3rd, 4th passage via friendly fire i.e. via surgical, dental, blood, medical arenas, from humans exposed by consumption ? NOT to forget all the animal medical by-products there from too? BUT yet, officials will still try and have us believe that 85% to 95% of all human Transmissible Spongiform Encephalopathy TSE i.e. sporadic CJD, is a single strain, that just happens spontaneously, with no route and source from anything. P L E A S E, This is not rocket science. It's 2010, and the UKBSEnvCJD only theory should be put to rest once and for all. Iatrogenic CJD is spreading as we speak, there are many strains, they are becoming more virulent, and they came from some route and some source, and that could be many. North America is home to the most strains of documented natural field TSE in animals. These animals have been fed to both humans and animals for human consumption. The consumer there from are a source of TSE to the medical and surgical arena, and clusters there from are real, they are happening and there is a route and source. CJD and all human TSE prion disease must be made mandatory reportable, with NO age limits, with a CJD Questionnaire asking real questions pertaining to potential routes and sources going to all families of victims of this horrible disease. This must be done Nationally and Internationally immediately. We have floundered too long. ...


tarball, aka flounder, alias TSS, and for those that remember, madcowdeadmommadson


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