Monday, February 24, 2014

UPDATE ON CREUTZFELDT–JAKOB DISEASE Masters et al Australia

UPDATE ON CREUTZFELDT–JAKOB DISEASE Masters et al Australia
 
 
UPDATE ON CREUTZFELDT–JAKOB DISEASE

 

Colin L. Masters

 

Mental Health Research Institute, Florey Institute of Neuroscience and Mental Health, the University of Melbourne, Vic, Australia

 

Creutzfeldt–Jakob disease (CJD) is a transmissible spongiform encephalopathy caused by the accumulation of an abnormal conformer of the prion protein (PrPCJD). The abnormally folded protein has the autocatalytic capacity for converting natural conformers of PrP, and this property underlies its infectivity/transmissibility. Closely related to the causal mechanism of bovine spongiform encephalopathy (BSE), the zoonotic spread of BSE to humans has proven to be a major threat to the public health system. Infection control guidelines have been updated. New sensitive and specific diagnostic assays based on protein-misfolding amplification are being developed which also have the capacity to screen for the safety of the blood supply.

 

The spectrum of prion-like diseases has emerged with the recognition that Ab, tau and a-synuclein also have the capacity to spread within the human brain to cause Alzheimer’s disease, frontotemporal dementia and Parkinson’s disease.

 

PDF

 

http://pdfs.journals.lww.com/pathologyrcpa/2014/02001/Update_on_Creutzfeldt_Jakob_Disease.92.pdf?token=method|ExpireAbsolute;source|Journals;ttl|1393261976602;payload|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;hash|vc6+RCn4HF2d0BFMF46X7A==

 

 

Aggregate-Depleted Brain Fails to Induce Aβ Deposition in a Mouse Model of Alzheimer's Disease

 

Claudia Duran-Aniotz, Affiliations: Mitchell Center for Alzheimer's Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas, United States of America, Universidad de los Andes, Facultad de Medicina, Santiago, Chile

 

X Rodrigo Morales, Affiliation: Mitchell Center for Alzheimer's Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas, United States of America

 

X Ines Moreno-Gonzalez, Affiliation: Mitchell Center for Alzheimer's Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas, United States of America

 

X Ping Ping Hu, Affiliations: Mitchell Center for Alzheimer's Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas, United States of America, Education Ministry Key Laboratory on Luminescence and Real-Time Analysis, College of Life Sciences, Southwest University, Chongqing, China

 

X Joseph Fedynyshyn, Affiliation: Novartis Vaccines and Diagnostics, Emeryville, California, United States of America

 

X Claudio Soto mail * E-mail: Claudio.Soto@uth.tmc.edu

 

Affiliation: Mitchell Center for Alzheimer's Disease and Related Brain Disorders, Department of Neurology, University of Texas Houston Medical School, Houston, Texas, United States of America

 

X Published: February 12, 2014 DOI: 10.1371/journal.pone.0089014

 

Abstract Recent studies in animal models of Alzheimer's disease (AD) show that amyloid-beta (Aβ) misfolding can be transmissible; however, the mechanisms by which this process occurs have not been fully explored. The goal of this study was to analyze whether depletion of aggregates from an AD brain suppresses its in vivo “seeding” capability. Removal of aggregates was performed by using the Aggregate Specific Reagent 1 (ASR1) compound which has been previously described to specifically bind misfolded species. Our results show that pre-treatment with ASR1-coupled magnetic beads reduces the in vivo misfolding inducing capability of an AD brain extract. These findings shed light respect to the active principle responsible for the prion-like spreading of Alzheimer's amyloid pathology and open the possibility of using seeds-capturing reagents as a promising target for AD treatment.

 


 

 

Wednesday, May 16, 2012

 

Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?

 

Proposal ID: 29403

 


 

 

Wednesday, September 21, 2011

 

PrioNet Canada researchers in Vancouver confirm prion-like properties in Amyotrophic Lateral Sclerosis (ALS)

 


 

 

Sunday, February 10, 2013

 

Parkinson's Disease and Alpha Synuclein: Is Parkinson's Disease a Prion-Like Disorder?

 


 

 

Ann N Y Acad Sci. 1982;396:131-43.

 

Alzheimer's disease and transmissible virus dementia (Creutzfeldt-Jakob disease).

 

Brown P, Salazar AM, Gibbs CJ Jr, Gajdusek DC.

 

Abstract

 

Ample justification exists on clinical, pathologic, and biologic grounds for considering a similar pathogenesis for AD and the spongiform virus encephalopathies. However, the crux of the comparison rests squarely on results of attempts to transmit AD to experimental animals, and these results have not as yet validated a common etiology. Investigations of the biologic similarities between AD and the spongiform virus encephalopathies proceed in several laboratories, and our own observation of inoculated animals will be continued in the hope that incubation periods for AD may be even longer than those of CJD.

 


 

 

CJD1/9 0185 Ref: 1M51A

 

IN STRICT CONFIDENCE

 

Dr McGovern From: Dr A Wight Date: 5 January 1993 Copies: Dr Metters Dr Skinner Dr Pickles Dr Morris Mr Murray

 

TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES

 

1. CMO will wish to be aware that a meeting was held at DH yesterday, 4 January, to discuss the above findings. It was chaired by Professor Murray (Chairman of the MRC Co-ordinating Committee on Research in the Spongiform Encephalopathies in Man), and attended by relevant experts in the fields of Neurology, Neuropathology, molecular biology, amyloid biochemistry, and the spongiform encephalopathies, and by representatives of the MRC and AFRC. 2. Briefly, the meeting agreed that:

 

i) Dr Ridley et als findings of experimental induction of p amyloid in primates were valid, interesting and a significant advance in the understanding of neurodegenerative disorders;

 

ii) there were no immediate implications for the public health, and no further safeguards were thought to be necessary at present; and

 

iii) additional research was desirable, both epidemiological and at the molecular level. Possible avenues are being followed up by DH and the MRC, but the details will require further discussion. 93/01.05/4.1

 


 

 

BSE101/1 0136

 

IN CONFIDENCE

 

5 NOV 1992 CMO From: Dr J S Metters DCMO 4 November 1992

 

TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES

 

1. Thank you for showing me Diana Dunstan's letter. I am glad that MRC have recognized the public sensitivity of these findings and intend to report them in their proper context. This hopefully will avoid misunderstanding and possible distortion by the media to portray the results as having more greater significance than the findings so far justify.

 

2. Using a highly unusual route of transmission (intra-cerebral injection) the researchers have demonstrated the transmission of a pathological process from two cases one of severe Alzheimer's disease the other of Gerstmann-Straussler disease to marmosets. However they have not demonstrated the transmission of either clinical condition as the "animals were behaving normally when killed'. As the report emphasizes the unanswered question is whether the disease condition would have revealed itself if the marmosets had lived longer. They are planning further research to see if the conditions, as opposed to the partial pathological process, is transmissible. What are the implications for public health?

 

3. The route of transmission is very specific and in the natural state of things highly unusual. However it could be argued that the results reveal a potential risk, in that brain tissue from these two patients has been shown to transmit a pathological process. Should therefore brain tissue from such cases be regarded as potentially infective? Pathologists, morticians, neuro surgeons and those assisting at neuro surgical procedures and others coming into contact with "raw" human brain tissue could in theory be at risk. However, on a priori grounds given the highly specific route of transmission in these experiments that risk must be negligible if the usual precautions for handling brain tissue are observed.

 

92/11.4/1-1 BSE101/1 0137

 

4. The other dimension to consider is the public reaction. To some extent the GSS case demonstrates little more than the transmission of BSE to a pig by intra-cerebral injection. If other prion diseases can be transmitted in this way it is little surprise that some pathological findings observed in GSS were also transmissible to a marmoset. But the transmission of features of Alzheimer's pathology is a different matter, given the much greater frequency of this disease and raises the unanswered question whether some cases are the result of a transmissible prion. The only tenable public line will be that "more research is required" before that hypothesis could be evaluated. The possibility on a transmissible prion remains open. In the meantime MRC needs carefully to consider the range and sequence of studies needed to follow through from the preliminary observations in these two cases. Not a particularly comfortable message, but until we know more about the causation of Alzheimer's disease the total reassurance is not practical.

 

JS METTERS Room 509 Richmond House Pager No: 081-884 3344 Callsign: DOH 832 121/YdeS 92/11.4/1.2

 


 

 

BSE101/1 0136

 

IN CONFIDENCE

 

CMO

 

From: Dr J S Metters DCMO

 

4 November 1992

 

TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES

 


 

 

CJD1/9 0185

 

Ref: 1M51A

 

IN STRICT CONFIDENCE

 

From: Dr. A Wight Date: 5 January 1993

 

Copies:

 

Dr Metters Dr Skinner Dr Pickles Dr Morris Mr Murray

 

TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES

 


 

 

Wednesday, January 5, 2011

 

ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011 Prions

 

David W. Colby1,* and Stanley B. Prusiner1,2

 


 


 

Friday, September 3, 2010

 

Alzheimer's, Autism, Amyotrophic Lateral Sclerosis, Parkinson's, Prionoids, Prionpathy, Prionopathy, TSE

 


 


 

 

SCENARIO 3: ‘THE THIN STEMMED GLASS’

 

... a TSE is found that is linked to Alzheimer’s disease.

 


 

 

Tuesday, November 26, 2013

 

Transmission of multiple system atrophy prions to transgenic mice

 


 

Transmission of Prions and Alzheimer’s disease Abeta Amyloid

 

Claudio Soto, PhD

 

Mitchell Center for Alzheimer’s disease and Related Brain Disorders, Dept of Neurology

 

University of Texas Medical School at Houston

 


 

 

Tuesday, October 4, 2011

 

Molecular Psychiatry

 

advance online publication 4 October 2011; doi: 10.1038/mp.2011.120

 

De novo induction of amyloid-ß deposition in vivo

 

Our results suggest that some of the typical brain abnormalities associated with AD can be induced by a prion-like mechanism of disease transmission through propagation of protein misfolding. These findings may have broad implications for understanding the molecular mechanisms responsible for the initiation of AD, and may contribute to the development of new strategies for disease prevention and intervention. Keywords: amyloid; prion; protein misfolding; disease transmission

 


 

 

see more here ;

 


 


 

 

Friday, January 31, 2014

 

***Confidentiality in preclinical Alzheimer disease studies

 


 

Tuesday, February 11, 2014

 

Novant Health Forsyth Medical Center Information on potential CJD exposure

 


 

I suppose one of the most disturbing studies I have ever read, was the one of Gibbs et al, way back, with electrodes that caused CJD, again, and again.

 

I am not posting this to scare folks, so be it if it does, but I am posting this for you to see what you are dealing with. ...this study still amazes me. read it more than once.

 

please see ;

 

1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8

 

*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.

 

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

 

Laboratory of Central Nervous System Studies, National Institute of

 

Neurological Disorders and Stroke, National Institutes of Health,

 

Bethesda, MD 20892.

 

*** Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

 

PMID: 8006664 [PubMed - indexed for MEDLINE]

 


 

New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication

 


 

Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production

 


 

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

 


 

A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing

 


 

 Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals

 


 

PPo4-4:

 

Survival and Limited Spread of TSE Infectivity after Burial

 


 

*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.

 


 

Wednesday, April 24, 2013

 

Chimpanzees Released After 30 Years Of Testing, Brace Yourself For Smiles

 


 

see full text ;

 

Saturday, November 16, 2013

 

Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

 

Infect Control Hosp Epidemiol.

 


 

Thursday, February 06, 2014

 

*** Commons Science and Technology Committee announce new inquiry on blood, tissue and organ screening Parliament exposure vcjd and blood risk while still ignoring recent risks factors of sporadic CJD

 


 

Wednesday, February 12, 2014

 

USDA/APHIS NOTICE: Final Rule Regarding Imports and BSE Effective March 4, 2014

 

LOL !!! Laughing out loud

 


 

Sunday, January 19, 2014

 

National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014

 


 

Wednesday, January 15, 2014

 

*** INFECTION PREVENTION AND CONTROL OF CJD, VCJD AND OTHER HUMAN PRION DISEASES IN HEALTHCARE AND COMMUNITY SETTINGS Variably Protease-Sensitive Prionopathy (VPSPr) January 15, 2014

 


 

Monday, January 13, 2014

 

Prions in Variably Protease-Sensitive Prionopathy: An Update Pathogens 2013

 

Pathogens 2013, 2, 457-471; doi:10.3390/pathogens2030457

 


 

Friday, January 10, 2014

 

*** vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???

 


 

Friday, January 10, 2014

 

*** vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???

 


 

 

Monday, February 24, 2014

 

Sporadic Fatal Insomnia in an Adolescent

 


 

 

 

CJD QUESTIONNAIRE USA

 


 


 

CJD VOICE

 


 


 


 


 


 


 


 


 


 


 

 

 layperson

 

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

 

 

posted by Terry S. Singeltary Sr. at 12:15 PM

Friday, February 14, 2014

Creutzfeldt-Jakob disease (CJD) biannual update (February 2014), with briefing on novel human prion disease National CJD Research and Surveillance Unit NCJDRSU





This six-monthly report provides an update on the enhanced surveillance of potential iatrogenic (healthcare-acquired) exposures to Creutzfeldt-Jakob Disease (CJD). The data is correct as at 31 December 2013. For numbers of CJD case reports, readers should consult data provided by the National CJD Research and Surveillance Unit (NCJDRSU, http://www.cjd.ed.ac.uk/data.html).

A briefing on a recently-identified human prion disease – Variably Protease-Sensitive Prionopathy – is also presented below.

Monitoring of patients 'at increased risk' of CJD

Individuals who have been identified as at increased risk of CJD as a consequence of their medical care are informed of their exposure and asked to follow public health precautions to avoid potentially transmitting the infection to others. They are also followed-up to help determine the risks of CJD transmission to patients through different routes and to ascertain whether any people who may have been exposed to increased CJD risks go on to develop CJD.

Public health follow-up activities include clinical monitoring, general practitioner (GP) updates, and post mortem investigations to determine whether asymptomatic individuals in these groups have been infected with the CJD agent. Some individuals also provide blood or tissue specimens for research purposes. A number of different organisations are involved in these activities: Public Health England (formerly the Health Protection Agency), Health Protection Scotland (HPS), UCL Institute of Child Health/Great Ormond Street Hospital (ICH), NHS Blood and Transplant (NHSBT), National CJD Research and Surveillance Unit (NCJDRSU), National Prion Clinic (NPC), and the UK Haemophilia Centre Doctors' Organisation (UKHCDO).

The PHE CJD Section coordinates the collation of data on individuals identified as at increased risk of CJD, and who have been informed of this. These individuals are followed up through public health monitoring and research activities by different organisations (table 1).

The PHE CJD Section currently holds data on the following groups of ‘at risk' patients:

  • recipients of blood components from donors who subsequently developed vCJD
  • blood donors to individuals who later developed vCJD
  • other recipients of blood components from these blood donors
  • recipients of certain plasma products between 1990 and 2001 (non-bleeding disorder patients)
  • certain surgical contacts of patients diagnosed with CJD
  • highly transfused recipients.

Data on the following risk groups are not held by PHE, but are held by other organisations:

  • bleeding disorder patients who received plasma products between 1990 and 2001 (UKHCDO)
  • recipients of human derived growth hormone before 1985 (ICH)
  • patients who could have received a dura mater graft before August 1992 (data not currently collected)
  • people who have been treated with gonadotrophin sourced from humans before 1973 (data not currently collected)
  • family risk of genetic prion disease (NPC).

The data from the UKHCDO are likely to be an underestimate of the true number of ‘at risk' patients with bleeding disorders who received UK-sourced clotting factors, as there was incomplete reporting of identified ‘at risk' patients by haemophilia centres to the UKHCDO database. Notified ‘at risk' patients are given the option of removing their details from the UKHCDO database, and are then removed from the ‘at risk' totals.

The data on ‘at risk' patients who received human-derived human growth hormone held by the ICH is a slight underestimate of the total as a small number of these patients are not included in the ICH follow-up.

Table 1. Summary of all ‘at risk’ groups on which data are collected (as at 31 December 2013)

 

'At risk' Group
Identified as 'at risk'
Number notified as being 'at risk'
Cases
Asymptomatic infections [b]
All Alive
Recipients of blood from who later developed vCJD
67
27
15
3
1

Blood donors to who later developed vCJD
112
107
104
–
–

Other recipients of blood components from these donors
34
32 [c]
19 [c]
–
–

Plasma product recipients (non-bleeding disorders) who received UK sourced plasmsa products 1980-2001
11
10
4
–
–

Certain surgical contacts of patients diagnosed witih CJD
154
129 [d]
113 [e]
–
–

Highly transfused patients
11
10
6
–
–

Total for 'at risk' groups where PHE holds data
389
315 [f]
261 [f]
3
1

Patients with bleeding disorders who received UK-sourced plasma products 1980-2001 [a]
3,875
National information incomplete
National information incomplete
–
1

Recipients of human-derived growth hormone [a]
1,883
1,883
1,504
75
–

Total for all 'at risk' groups [a]
6147
At least
2198
At least 1765
78
2

a. These are minimum figures. Central reporting for bleeding disorder patients is incomplete, and seven patients have opted out of the central UK Haemophilia Centre Doctors' Organisation database. A small number of ‘at risk’ growth hormone recipients are not included in the Institute of Child Health study. Not all of ‘at risk’ growth hormone recipients have been notified. There is no central record of who has been informed.
b. An unsymptomatic infection is when an individual does not exhibit any of the signs and symptoms of CJD in life but abnormal prion protein indicative of CJD infection has been found in tissue obtained from them. In these cases the abnormal prion protein was identified during post mortem after the individuals had died of other causes.
c. One patient notified by proxy.
d. Four of these notified by proxy.
e. Two of these notified by proxy.
f. Includes patients notified by proxy.

 

Variably Protease-Sensitive Prionopathy
 
Professor James W Ironside and Dr Mark W Head

The National CJD Research and Surveillance Unit, University of Edinburgh.
Variably protease-sensitive prionopathy (VPSPr) is the most recently identified human prion disease, first described in the USA by Gambetti et al. in 2008 as “a novel human disease with abnormal prion protein sensitive to protease” [1]. Since then, similar cases have been identified in other countries; the National CJD Research and Surveillance Unit has identified nine cases in the UK, three of which have been identified retrospectively and the others prospectively from samples and data collected since 1991 [2-6]. Other candidate cases are currently under investigation.
 
Patients with VPSPr have no identified risk factors for acquired human prion disease and no associated mutations in the prion protein gene (PRNP) coding sequence have been found. In the original description a proportion of the patients had family histories of ill-defined dementia, but this has not been a feature in more recently identified cases [1,2,6]. VPSPr affects patients in the same age range as sporadic Creutzfeldt-Jakob disease (sCJD), occurring mostly in patients over the age of 60. The clinical features are more varied than in sCJD and include movement abnormalities, cognitive decline and unsteadiness while walking. The clinical illness is longer than for sCJD; most patients survive for over a year before succumbing to the illness. Diagnostic clinical criteria are therefore difficult to establish, and further work is required on this topic since this disease is likely to be under-ascertained [2,6].
 
Like sCJD, VPSPr occurs in all genetic groups defined by the polymorphism at codon 129 in the PRNP gene, ie MM, MV and MV. Unlike sCJD, there is a preponderance of the codon 129-V haplotype. VPSPr has distinctive neuropathological features, the most typical of which are microplaques that occur in a target-like arrangement and are particularly common in the cerebellum. These microplaques show differential staining with a panel of different anti-PrP antibodies, allowing a distinction from both the common sCJD VV2 and the rare sCJD VV1 subtypes [1,2,5,6]. The most distinctive and defining feature of VPSPr is the biochemistry of the abnormal prion protein in the brain, which is only poorly resistant to proteolytic digestion, yielding a low abundance, truncated 8kDa (approx) band in Western blot assays [1]. This fragment is often accompanied by a faint ladder of bands extending into the 18-30kDa range [1,2]. Some cases of VPSPr also show a sCJD-like pattern on Western blot analysis for abnormal prion protein, often in the cerebellum, suggesting molecular overlaps between VPSPr and sCJD [6,7].
 
Further work is required to fully establish the epidemiology, clinical and pathological diagnostic criteria and transmission characteristics of VPSPr. The Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy (ACDP TSE) Subgroup concluded that until further research can demonstrate how transmissible VPSPr may be, it would be advisable to add this novel form of human prion disease to the infection control guidance for CJD and other related disorders.
 
References

1. Gambetti P, Dong, Yuan J, et al. A novel human disease with abnormal prion protein sensitive to protease. Ann Neurol 2008; 63: 697-708.

2. Zou WQ, Puoti G, Xiao X, et al. Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein. Ann Neurol 2010; 68: 162-72.
3. Head MW, Knight R, Zeidler M, et al. A case of protease sensitive prionopathy in a patient in the United Kingdom. Neuropathol Appl Neurobiol 2009; 35: 628-32.

4. Jansen C, Head MW, van Gool WA, et al. The first case of protease-sensitive prionopathy (PSPr) in The Netherlands: a patient with an unusual GSS-like clinical phenotype. J Neurol Neurosurg Psychiatry 2010; 81: 1052-5.

5. Head MW, Lowrie S, Chohan G, et al. Variably protease-sensitive prionopathy in a PRNP codon 129 heterozygous UK patient with co-existing tau, a synclein and Aß pathology. Acta Neuropathol 2010: 120: 821-3.

6. Head MW, Yull HM, Ritchie DL, et al. Variably protease-sensitive prionopathy in the UK: a retrospective review 1991-2008. Brain 2013; 136: 1102-15.

7. Rodriguez-Martinez AB, de Munain AL, Ferrer I, et al. Coexistence of protease sensitive and resistant prion protein in 129 VV homozygous sporadic Creutzfeldt-Jakob disease. J Med Case Rep 2012; 6: 348

 

http://www.hpa.org.uk/hpr/infections/ei_cjd.htm#cjd

 

Wednesday, March 28, 2012

 

VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $

 

OR-10: Variably protease-sensitive prionopathy is transmissible in bank voles

 

Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1 Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome, Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna, Italy; 3Case Western Reserve University; Cleveland, OH USA

 

 Background. Variably protease-sensitive prionopathy (VPSPr) is a recently described “sporadic”neurodegenerative disease involving prion protein aggregation, which has clinical similarities with non-Alzheimer dementias, such as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is the electrophoretic pattern of PrPSc after digestion with proteinase K (PK). After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern similar to that described in GSS cases. The clinical and pathological features of VPSPr raised the question of the correct classification of VPSPr among prion diseases or other forms of neurodegenerative disorders. Here we report preliminary data on the transmissibility and pathological features of VPSPr cases in bank voles.

 

 Materials and Methods. Seven VPSPr cases were inoculated in two genetic lines of bank voles, carrying either methionine or isoleucine at codon 109 of the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical diagnosis in voles was confirmed by brain pathological assessment and western blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.

 

 Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission in BvM109. Overall, 3 voles were positive with survival time between 290 and 588 d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form of the typical PrP27–30, which was indistinguishable to that previously observed in BvM109 inoculated with sCJDMM1 cases.

 

 In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until now. Overall, 5 voles were positive with survival time between 281 and 596 d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern, characterized by low molecular weight PrPres. These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus and the N-terminus. Second passages are in progress from these first successful transmissions.

 

 Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.

 

 The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.

 


 

 Wednesday, March 28, 2012

 

 VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $

 


 

 *** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.

 

 
SEE FULL TEXT AND MORE HERE ;

 

Wednesday, March 28, 2012

 

VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $

 


 

 Friday, August 16, 2013

*** Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and Contaminated blood products induce a highly atypical prion disease devoid of PrPres in primates
http://creutzfeldt-jakob-disease.blogspot.com/2013/08/creutzfeldt-jakob-disease-cjd-biannual.html

 

Volume 4 Number 7 Published on: 19 February 2010

 

Emerging infections/CJD Creutzfeldt-Jakob disease (CJD) biannual update (2010/1)

 

http://www.hpa.org.uk/hpr/archives/2010/hpr0710.pdf

 

Saturday, March 23, 2013

 

$$$ CJD Incidents Panel to be disbanded $$$

 


 

ALL iatrogenic cjd is, is sporadic cjd, until route and source is documented, confirmed, and put in the academic and public domain, which very seldom happens. that's why 85%+ of all human TSE prion disease is sporadic CJD, they like to keep it that way$$$ ...just saying$$$

I suppose one of the most disturbing studies I have ever read, was the one of Gibbs et al, way back, with electrodes that caused CJD, again, and again.

I am not posting this to scare folks, so be it if it does, but I am posting this for you to see what you are dealing with. ...this study still amazes me. read it more than once.

please see ;

1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8

Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of

Neurological Disorders and Stroke, National Institutes of Health,

Bethesda, MD 20892.

Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

PMID: 8006664 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract



New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication

http://www.pnas.org/content/97/7/3418.full


Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/



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

http://www.landesbioscience.com/journals/prion/NicholsPRION3-3.pdf



A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing

http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract


Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals

http://transmissiblespongiformencephalopathy.blogspot.com/2013/07/rapid-assessment-of-bovine-spongiform.html



PPo4-4:

Survival and Limited Spread of TSE Infectivity after Burial

http://www.prion2010.org/bilder/prion_2010_program_latest_w_posters_4_.pdf?139&PHPSESSID=a30a38202cfec579000b77af81be3099



*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.

http://cdmrp.army.mil/prevfunded/nprp/NPRP_Summit_Final_Report.pdf



Saturday, November 16, 2013

Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

Infect Control Hosp Epidemiol.

http://creutzfeldt-jakob-disease.blogspot.com/2013/11/management-of-neurosurgical-instruments.html

 

Tuesday, May 28, 2013

Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance
http://creutzfeldt-jakob-disease.blogspot.com/2013/05/late-in-life-surgery-associated-with.html



Tuesday, February 11, 2014

Novant Health Forsyth Medical Center Information on potential CJD exposure

http://creutzfeldt-jakob-disease.blogspot.com/2014/02/novant-health-forsyth-medical-center.html
 

Wednesday, January 15, 2014

 

*** INFECTION PREVENTION AND CONTROL OF CJD, VCJD AND OTHER HUMAN PRION DISEASES IN HEALTHCARE AND COMMUNITY SETTINGS Variably Protease-Sensitive Prionopathy (VPSPr) January 15, 2014

 
http://transmissiblespongiformencephalopathy.blogspot.com/2014/01/infection-prevention-and-control-of-cjd.html

 

Wednesday, November 27, 2013

 

NHS failed to sterilise surgical instruments contaminated with 'mad cow' disease

 


 

 
Friday, January 10, 2014

 

*** vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???

 


 

 
Thursday, January 23, 2014

 

Medical Devices Containing Materials Derived from Animal Sources (Except for In Vitro Diagnostic Devices) [Docket No. FDA–2013–D–1574]

 


 

 
Saturday, November 16, 2013

 

Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

 

Infect Control Hosp Epidemiol.

 


 

 
Thursday, November 14, 2013

 

Prion diseases in humans: Oral and dental implications

 


 

 
Saturday, November 2, 2013

 

Recommendation of the Swiss Expert Committee for Biosafety on the classification of activities using prion genes and prion protein January 2013

 


 

 
Thursday, January 16, 2014

 

The Anspach Effort, Inc. RECALL FDA Blackmax motor had been used in a case where the patient was diagnosed with Creutzfeldt-Jacob Disease (CJD) MARYLAND HOSTPITAL

 


 

 
WHAT about the sporadic CJD TSE proteins ?

 

WE now know that some cases of sporadic CJD are linked to atypical BSE and atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all it’s sub-types $$$

 

Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013

 

*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010 ***

 


 

 
Sunday, October 13, 2013

 

*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012

 


 

 
Sunday, January 19, 2014

 

National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014

 


 

 
Thursday, January 17, 2013

 

TSE guidance, surgical, dental, blood risk factors, Part 4 Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings (updated January 2013)

 


 

 
Tuesday, September 24, 2013

 

NORDION (US), INC., AND BIOAXONE BIOSCIENCES, INC., Settles $90M Mad Cow TSE prion Contamination Suit Cethrin(R)

 

Case 0:12-cv-60739-RNS Document 1 Entered on FLSD Docket 04/26/2012 Page 1 of 15

 


 

 
Tuesday, February 11, 2014

Novant Health Forsyth Medical Center Information on potential CJD exposure
http://creutzfeldt-jakob-disease.blogspot.com/2014/02/novant-health-forsyth-medical-center.html

 
Thursday, September 05, 2013

 

Possible Patient Exposure to Creutzfeldt-Jakob Disease Announced New Hampshire DHHS

 

Press Release

 


 

 
Friday, July 19, 2013

 

Beaumont Hospital in Dublin assessing patients for CJD

 


 

 
Tuesday, July 31, 2012

 

11 patients may have been exposed to fatal disease Creutzfeldt-Jakob Disease CJD Greenville Memorial Hospital

 


 

 
Thursday, August 02, 2012

 

CJD case in Saint John prompts letter to patients Canada CJD case in Saint John prompts letter to patients

 


 

 
Saturday, February 12, 2011

 

Another Pathologists dies from CJD, another potential occupational death ?

 

another happenstance of bad luck, a spontaneous event from nothing, or friendly fire ???

 


 

 
Monday, April 15, 2013

 

Dr. Stephen B. Thacker Director Centers for Disease Control and Prevention′s Office of Science, Epidemiology and Laboratory Services (OSELS) dies from Creutzfeldt Jakob Disease CJD

 


 

 
Thursday, April 12, 2012

 

Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010

 

Eurosurveillance, Volume 17, Issue 15, 12 April 2012

 

Research articles

 


 

 
Wednesday, February 16, 2011

 

IN CONFIDENCE SCRAPIE TRANSMISSION TO CHIMPANZEES IN CONFIDENCE

 


 

 
Sunday, December 12, 2010

 

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

 


 

 
Sunday, April 18, 2010

 

SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010

 


 

 
Sunday, February 2, 2014

 

The Presence of Disease-Associated Prion Protein in Skeletal Muscle of Cattle Infected with Classical Bovine Spongiform Encephalopathy

 

NOTE Pathology

 


 

 
Monday, February 10, 2014

 

Enhanced Virulence of Sheep-Passaged Bovine Spongiform Encephalopathy Agent Is Revealed by Decreased Polymorphism Barriers in Prion Protein Conversion Studies

 


 

 
Wednesday, December 11, 2013

 

*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease ***

 


 

Wednesday, April 24, 2013

Chimpanzees Released After 30 Years Of Testing, Brace Yourself For Smiles

http://transmissiblespongiformencephalopathy.blogspot.com/2013/04/chimpanzees-released-after-30-years-of.html

 

layperson

 

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

posted by Terry S. Singeltary Sr. at 1:26 PM

Tuesday, February 11, 2014

Novant Health Forsyth Medical Center Information on potential CJD exposure

February 11, 2014 UPDATE

Novant Health Forsyth Medical Center Information on potential CJD exposure

Information on potential CJD exposure

Novant Health Forsyth Medical Center is sharing information about the exposure of Creutzfeldt-Jakob disease, or CJD, to 18 patients who had neurosurgery at the hospital. According to the CDC, the risk of transmission from surgery is remote and the last reported case of surgical transmission was in 1976.

It is believed that CJD affects about one person in every one million people per year worldwide. In the United States there are about 300 cases per year. It is important to note that there are multiple variations of CJD and this case is not related to mad cow disease.

Although the risk of getting the disease is very low, Novant Health believes it is important to share this information with patients, family members, and the community. Patients who underwent neurosurgery and were exposed already have been contacted directly by Novant Health.

On January 18, 2014, a medical team at Novant Health Forsyth Medical Center performed a procedure on a patient with a neurological condition. Subsequently, the medical center was notified that lab results confirmed that the patient had CJD. Although the surgical instruments used during the patient’s surgery were sterilized using standard procedures for cleaning surgical instruments, they were not subjected to enhanced sterilization procedures necessary on instruments used in confirmed or suspected cases of CJD.

The patient had neurological symptoms that could have been attributed to CJD or another brain disease. Our standard procedure is to apply the enhanced sterilization process to surgical instruments that are used on any patient who is suspected or confirmed of having CJD in order to prevent possible transmission. There were reasons to suspect that this patient might have had CJD. As such, the extra precautions should have been taken, but were not.

Since then, Novant Health Forsyth Medical Center has identified 18 patients who were exposed to the disease during brain surgery. We believed it was our duty to inform those patients who were exposed during surgery.

"The risk to these individuals is remote," said Jim Lederer, MD, Novant Health VP clinical improvement. "However, we cannot say there is no risk and therefore it is our obligation to notify patients and provide them with information and support. Our first concern is for our patients who are recovering from surgery and may now need additional support.”

Following the CJD confirmation, Novant Health Forsyth Medical Center instituted the enhanced sterilization process on all surgical instruments used in brain surgery. In addition, the hospital is following CDC recommendations and guidelines and working with state and local health officials to ensure the facility is taking necessary precautions to prevent this in the future.

Questions about CJD and this incident may be directed to our toll-free number 877-463-4052.

 
News media inquiries, please call 336-287-4203.

 

 
Greetings Novant Health et al,

AS bad as this is, I applaud your efforts to contact the exposed, and help prevent further exposure. I only hope that you really consider disposable instruments for future use, when at all possible. I also hope that you offer free follow-up and trace forward for any further medical and dental procedures for these exposed, for the next 50 YEARS.
 
I wish to submit the following on the Creutzfeldt Jakob Disease CJD TSE prion disease and the iatrogenic aspects of it i.e. friendly fire.

I am a layperson, I lost my mother to the Heidenhain Variant of Creutzfeldt Jakob Disease hvCJD December 14, 1997, and just made a promise, never forget, and never let them forget.

here is the latest TSE prion science, please use as you wish.

good luck with this incident. ...

with sad regards, I am sincerely,

Terry S. Singeltary Sr.

 
Inactivation of the TSE Prion disease

Chronic Wasting Disease CWD, and other TSE prion disease, these TSE prions know no borders.

these TSE prions know no age restrictions.

The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit.

you cannot cook the TSE prion disease out of meat.

you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE.

Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well.

the TSE prion agent also survives Simulated Wastewater Treatment Processes.

IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades.

you can bury it and it will not go away.

The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area.

it’s not your ordinary pathogen you can just cook it out and be done with. that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent.

New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication

The infectious agents responsible for transmissible spongiform encephalopathy (TSE) are notoriously resistant to most physical and chemical methods used for inactivating pathogens, including heat. It has long been recognized, for example, that boiling is ineffective and that higher temperatures are most efficient when combined with steam under pressure (i.e., autoclaving). As a means of decontamination, dry heat is used only at the extremely high temperatures achieved during incineration, usually in excess of 600°C. It has been assumed, without proof, that incineration totally inactivates the agents of TSE, whether of human or animal origin.


 
Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production

Histochemical analysis of hamster brains inoculated with the solid residue showed typical spongiform degeneration and vacuolation. Re-inoculation of these brains into a new cohort of hamsters led to onset of clinical scrapie symptoms within 75 days, suggesting that the specific infectivity of the prion protein was not changed during the biodiesel process. The biodiesel reaction cannot be considered a viable prion decontamination method for MBM, although we observed increased survival time of hamsters and reduced infectivity greater than 6 log orders in the solid MBM residue. Furthermore, results from our study compare for the first time prion detection by Western Blot versus an infectivity bioassay for analysis of biodiesel reaction products. We could show that biochemical analysis alone is insufficient for detection of prion infectivity after a biodiesel process.


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

The data presented here demonstrate that sPMCA can detect low levels of PrPCWD in the environment, corroborate previous biological and experimental data suggesting long term persistence of prions in the environment2,3 and imply that PrPCWD accumulation over time may contribute to transmission of CWD in areas where it has been endemic for decades. This work demonstrates the utility of sPMCA to evaluate other environmental water sources for PrPCWD, including smaller bodies of water such as vernal pools and wallows, where large numbers of cervids congregate and into which prions from infected animals may be shed and concentrated to infectious levels.



A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing

Keywords:Abattoir;bovine spongiform encephalopathy;QRA;scrapie;TSE


In this article the development and parameterization of a quantitative assessment is described that estimates the amount of TSE infectivity that is present in a whole animal carcass (bovine spongiform encephalopathy [BSE] for cattle and classical/atypical scrapie for sheep and lambs) and the amounts that subsequently fall to the floor during processing at facilities that handle specified risk material (SRM). BSE in cattle was found to contain the most oral doses, with a mean of 9864 BO ID50s (310, 38840) in a whole carcass compared to a mean of 1851 OO ID50s (600, 4070) and 614 OO ID50s (155, 1509) for a sheep infected with classical and atypical scrapie, respectively. Lambs contained the least infectivity with a mean of 251 OO ID50s (83, 548) for classical scrapie and 1 OO ID50s (0.2, 2) for atypical scrapie. The highest amounts of infectivity falling to the floor and entering the drains from slaughtering a whole carcass at SRM facilities were found to be from cattle infected with BSE at rendering and large incineration facilities with 7.4 BO ID50s (0.1, 29), intermediate plants and small incinerators with a mean of 4.5 BO ID50s (0.1, 18), and collection centers, 3.6 BO ID50s (0.1, 14). The lowest amounts entering drains are from lambs infected with classical and atypical scrapie at intermediate plants and atypical scrapie at collection centers with a mean of 3 × 10−7 OO ID50s (2 × 10−8, 1 × 10−6) per carcass. The results of this model provide key inputs for the model in the companion paper published here.


 
PPo4-4:

Survival and Limited Spread of TSE Infectivity after Burial

Karen Fernie, Allister Smith and Robert A. Somerville The Roslin Institute and R(D)SVS; University of Edinburgh; Roslin, Scotland UK

Scrapie and chronic wasting disease probably spread via environmental routes, and there are also concerns about BSE infection remaining in the environment after carcass burial or waste 3disposal. In two demonstration experiments we are determining survival and migration of TSE infectivity when buried for up to five years, as an uncontained point source or within bovine heads. Firstly boluses of TSE infected mouse brain were buried in lysimeters containing either sandy or clay soil. Migration from the boluses is being assessed from soil cores taken over time. With the exception of a very small amount of infectivity found 25 cm from the bolus in sandy soil after 12 months, no other infectivity has been detected up to three years. Secondly, ten bovine heads were spiked with TSE infected mouse brain and buried in the two soil types. Pairs of heads have been exhumed annually and assessed for infectivity within and around them. After one year and after two years, infectivity was detected in most intracranial samples and in some of the soil samples taken from immediately surrounding the heads. The infectivity assays for the samples in and around the heads exhumed at years three and four are underway. These data show that TSE infectivity can survive burial for long periods but migrates slowly. Risk assessments should take into account the likely long survival rate when infected material has been buried.

 The authors gratefully acknowledge funding from DEFRA.



PPo3-22:

Detection of Environmentally Associated PrPSc on a Farm with Endemic Scrapie

Ben C. Maddison,1 Claire A. Baker,1 Helen C. Rees,1 Linda A. Terry,2 Leigh Thorne,2 Susan J. Belworthy2 and Kevin C. Gough3 1ADAS-UK LTD; Department of Biology; University of Leicester; Leicester, UK; 2Veterinary Laboratories Agency; Surry, KT UK; 3Department of Veterinary Medicine and Science; University of Nottingham; Sutton Bonington, Loughborough UK

Key words: scrapie, evironmental persistence, sPMCA

Ovine scrapie shows considerable horizontal transmission, yet the routes of transmission and specifically the role of fomites in transmission remain poorly defined. Here we present biochemical data demonstrating that on a scrapie-affected sheep farm, scrapie prion contamination is widespread. It was anticipated at the outset that if prions contaminate the environment that they would be there at extremely low levels, as such the most sensitive method available for the detection of PrPSc, serial Protein Misfolding Cyclic Amplification (sPMCA), was used in this study. We investigated the distribution of environmental scrapie prions by applying ovine sPMCA to samples taken from a range of surfaces that were accessible to animals and could be collected by use of a wetted foam swab. Prion was amplified by sPMCA from a number of these environmental swab samples including those taken from metal, plastic and wooden surfaces, both in the indoor and outdoor environment. At the time of sampling there had been no sheep contact with these areas for at least 20 days prior to sampling indicating that prions persist for at least this duration in the environment. These data implicate inanimate objects as environmental reservoirs of prion infectivity which are likely to contribute to disease transmission.


 

Wednesday, July 10, 2013

Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals

BMC Veterinary Research 2013, 9:134 doi:10.1186/1746-6148-9-134


 

now that I have your attention, please read on for the rest of this man made nightmare. ...tss

 

Thursday, February 06, 2014

Commons Science and Technology Committee announce new inquiry on blood, tissue and organ screening Parliament exposure vcjd and blood risk while still ignoring recent risks factors of sporadic CJD


 
Wednesday, January 15, 2014

*** INFECTION PREVENTION AND CONTROL OF CJD, VCJD AND OTHER HUMAN PRION DISEASES IN HEALTHCARE AND COMMUNITY SETTINGS Variably Protease-Sensitive Prionopathy (VPSPr) January 15, 2014


 
Wednesday, November 27, 2013

NHS failed to sterilise surgical instruments contaminated with 'mad cow' disease


 

Friday, January 10, 2014

vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type prion disease, what it ???


 
Thursday, January 23, 2014

Medical Devices Containing Materials Derived from Animal Sources (Except for In Vitro Diagnostic Devices) [Docket No. FDA–2013–D–1574]


 

Saturday, November 16, 2013

Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

Infect Control Hosp Epidemiol.


 

Thursday, November 14, 2013

Prion diseases in humans: Oral and dental implications


 

Saturday, November 2, 2013

Recommendation of the Swiss Expert Committee for Biosafety on the classification of activities using prion genes and prion protein January 2013


 

Thursday, January 16, 2014

The Anspach Effort, Inc. RECALL FDA Blackmax motor had been used in a case where the patient was diagnosed with Creutzfeldt-Jacob Disease (CJD) MARYLAND HOSTPITAL


 

WHAT about the sporadic CJD TSE proteins ?

WE now know that some cases of sporadic CJD are linked to atypical BSE and atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all it’s sub-types $$$

Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013

*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010 ***


 

Sunday, October 13, 2013

*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012


 

Sunday, January 19, 2014

National Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8, 2014


 
Thursday, January 17, 2013

TSE guidance, surgical, dental, blood risk factors, Part 4 Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings (updated January 2013)


 

Tuesday, September 24, 2013

NORDION (US), INC., AND BIOAXONE BIOSCIENCES, INC., Settles $90M Mad Cow TSE prion Contamination Suit Cethrin(R)

Case 0:12-cv-60739-RNS Document 1 Entered on FLSD Docket 04/26/2012 Page 1 of 15


 

Thursday, September 05, 2013

Possible Patient Exposure to Creutzfeldt-Jakob Disease Announced New Hampshire DHHS

Press Release


 

Friday, July 19, 2013

Beaumont Hospital in Dublin assessing patients for CJD


 

Tuesday, July 31, 2012

11 patients may have been exposed to fatal disease Creutzfeldt-Jakob Disease CJD Greenville Memorial Hospital


 

Thursday, August 02, 2012

CJD case in Saint John prompts letter to patients Canada CJD case in Saint John prompts letter to patients


 

Saturday, February 12, 2011

Another Pathologists dies from CJD, another potential occupational death ?

another happenstance of bad luck, a spontaneous event from nothing, or friendly fire ???


 

Thursday, April 12, 2012

 
Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010

 
Eurosurveillance, Volume 17, Issue 15, 12 April 2012

 

Research articles

 
http://creutzfeldt-jakob-disease.blogspot.com/2012/04/health-professions-and-risk-of-sporadic.html

 

Wednesday, February 16, 2011

IN CONFIDENCE SCRAPIE TRANSMISSION TO CHIMPANZEES IN CONFIDENCE


 

Sunday, December 12, 2010

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


 

Sunday, April 18, 2010

SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010


 

Sunday, February 2, 2014

The Presence of Disease-Associated Prion Protein in Skeletal Muscle of Cattle Infected with Classical Bovine Spongiform Encephalopathy

NOTE Pathology


 

Monday, February 10, 2014

Enhanced Virulence of Sheep-Passaged Bovine Spongiform Encephalopathy Agent Is Revealed by Decreased Polymorphism Barriers in Prion Protein Conversion Studies


 

Thursday, August 12, 2010

Seven main threats for the future linked to prions

First threat

The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.

***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.

Second threat

snip...


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

 
This is an interesting editorial about the Mad Cow Disease debacle, and it's ramifications that will continue to play out for decades to come ;

Monday, October 10, 2011

 

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

 

snip...

 

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

 

snip...

 


 


 

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

 


 

 I remember a case of Creutzfeldt-Jakob disease: implications for gastroenterology & CJD 38 years after _diagnostic_ use of hGH (Iatrogenic CJDs & sporadic CJDs) (see below);

 

We describe the second patient with hGH related CJD in the Netherlands. The patient developed the disease 38 years after hGH injections. To our knowledge, this is the longest incubation period described for any form of iatrogentic CJD. Furthermore, our patient was _not_ treated with hGH, but only received a _low_ dose as part of a diagnostic procedure. (see full text below).

 


 

 

 

Published March 26, 2003

 

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

 

Terry S. Singeltary, retired (medically)

 

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

 

Published March 26, 2003

 


 

 

Letters

 

JAMA. 2001;285(6):733-734. doi: 10.1001/jama.285.6.733

 

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

 

Terry S. Singeltary, Sr Bacliff, Tex

 

Since this article does not have an abstract, we have provided the first 150 words of the full text.

 

KEYWORDS: creutzfeldt-jakob disease, diagnosis. To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

 

References 1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.

 


 

 

 

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.

 

 


 

 

 

CJD Singeltary submission to PLOS ;

 

No competing interests declared.

 

see full text ;

 


 


 


 

 

Wednesday, May 16, 2012

 

Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?

 

Background

 

Alzheimer’s disease and Transmissible Spongiform Encephalopathy disease have both been around a long time, and was discovered in or around the same time frame, early 1900’s. Both diseases are incurable and debilitating brain disease, that are in the end, 100% fatal, with the incubation/clinical period of the Alzheimer’s disease being longer (most of the time) than the TSE prion disease. Symptoms are very similar, and pathology is very similar.

 

Methods

 

Through years of research, as a layperson, of peer review journals, transmission studies, and observations of loved ones and friends that have died from both Alzheimer’s and the TSE prion disease i.e. Heidenhain Variant Creutzfelt Jakob Disease CJD.

 

Results

 

I propose that Alzheimer’s is a TSE disease of low dose, slow, and long incubation disease, and that Alzheimer’s is Transmissible, and is a threat to the public via the many Iatrogenic routes and sources. It was said long ago that the only thing that disputes this, is Alzheimer’s disease transmissibility, or the lack of. The likelihood of many victims of Alzheimer’s disease from the many different Iatrogenic routes and modes of transmission as with the TSE prion disease.

 

Conclusions

 

There should be a Global Congressional Science round table event set up immediately to address these concerns from the many potential routes and sources of the TSE prion disease, including Alzheimer’s disease, and a emergency global doctrine put into effect to help combat the spread of Alzheimer’s disease via the medical, surgical, dental, tissue, and blood arena’s. All human and animal TSE prion disease, including Alzheimer’s should be made reportable in every state, and Internationally, WITH NO age restrictions. Until a proven method of decontamination and autoclaving is proven, and put forth in use universally, in all hospitals and medical, surgical arena’s, or the TSE prion agent will continue to spread. IF we wait until science and corporate politicians wait until politics lets science _prove_ this once and for all, and set forth regulations there from, we will all be exposed to the TSE Prion agents, if that has not happened already.

 

 

end...tss

 

 

SEE FULL TEXT AND SOURCE REFERENCES ;

 

Wednesday, May 16, 2012

 

Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion disease, Iatrogenic, what if ?

 

Proposal ID: 29403

 


 

Friday, January 31, 2014

 

Confidentiality in preclinical Alzheimer disease studies

 


 

Wednesday, December 11, 2013

 

*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease ***

 


 

 

Monday, February 10, 2014

 

18 Forsyth Medical Center patients exposed to CJD; apology issued...OOOPS, SORRY, TOO BAD $$$

 


 

 

 

layperson

 

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518


 

never break a promise to your mom...tss

posted by Terry S. Singeltary Sr. at 12:57 PM