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

Infection reports

 

Volume 7 Number 33 Published on: 16 August 2013

 

HIV/CJD

 

Creutzfeldt-Jakob disease (CJD) biannual update August 2013

 

This six-monthly report provides an update on the enhanced surveillance of potential iatrogenic (healthcareacquired) exposures to Creutzfeldt-Jakob Disease (CJD). The data is correct as of 30 June 2013. The report also includes an update on the CJD Incidents Panel (CJDIP). For numbers of CJD case reports, readers should consult data provided by the National CJD Research and Surveillance Unit (NCJDRSU) [1]. The latest analysis of variant CJD (vCJD) reports (onsets and deaths) is also available from the NCJDRSU website [2].

 

Dissolution of the CJD Incidents Panel, 31 March 2013

 

The CJDIP was an independent expert advisory committee established in 2000 on behalf of the UK Chief Medical Officers (CMOs). It advised on the management of incidents involving the potential transmission of CJD between patients. CJD incidents arise when there is potential transmission of any form of CJD between patients through clinical interventions, including via surgical instruments, tissues, organs or blood. The panel gave advice on a case by case basis. By the time the panel was dissolved in March 2013 a significant amount of this advice was based on precedent built up from cases discussed over the preceding 13 years.

 

Following the dissolution of the panel, the following arrangements apply:

 

Responsibility for investigating, assessing and managing CJD incidents (and where appropriate notifying patients) rests with local trusts, health boards and health protection teams in the same way as most other incidents that place patients at infection risk. National guidance on CJD incident management, drawing on the CJDIP precedents, is available to support this [3].

 

Long term public health surveillance of CJD exposures will continue and trusts, health boards and health protection teams are asked to continue reporting the occurrence of incidents to the CJD Section of Public Health England, in particular if they involve a patient notification exercise.

 

Novel issues that arise with respect to CJD risk management and infection control, or difficulties with interpretation of current guidance, can be referred to the CJD Section at Public Health England. If necessary advice may be sought from the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy (ACDP TSE) Risk Management Subgroup.

 

Potential iatrogenic exposures to CJD via surgery: 2000 to 31 March 2013

 

A surgical incident occurs when a patient with or at ‘increased risk’ of CJD has undergone surgery without the appropriate infection control guidance being followed [4]. This could occur if an asymptomatic patient undergoes surgery during the incubation period of CJD, or because information for those at an increased risk of CJD is not available at the time of surgery. If this happens, surgical instruments may be contaminated with the infectious agent that causes CJD. These instruments could then pose a transmission risk when they are re-used on other patients.

 

Table 1 shows the number of CJD surgical incidents and reports notified to the panel by the diagnosis of the index patient from 2000 to 31 March 2013.

 

Health Protection Report Vol. 7 No. 33 - 16 August 2013

 

Table 1. Number of CJD Surgical Incidents/Reports Notified to the CJD Incidents Panel: 2000 - 31 March 2013

 

Index patient status

 

2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 a 2011 2012 b 2013c Total I (% of total) TotalR (% of I R I R I R I R total)

 

Sporadic (possible, probable or definite)

 

7 19 22 24 16 18 31 17 21 15 5 4 2 23 – 17 – 1 197 (43) 45 (64)

 

vCJD (possible,

 

probable or definite) 6 14 22 5 4 1 2 – 1 1 – – 1 1 – – – – 57 (13) 1 (1)

 

Familial (including ‘at risk familial) – 2 2 7 1 3 7 – 2 3 2 – – 2 – – – – 29 (6) 2 (3)

 

‘At risk’ vCJD blood component recipient – – – – 4 10 5 1 – – 2 – 1 – – – – – 23 (5) –

 

‘At risk’ vCJD plasma product recipient – 1 2 – 10 18 9 8 6 9 3 - 8 2 6 – – – 80 (18) 2 (3)

 

‘At risk’ other – – 2 2 1 2 5 – – 1 7 – – 9 – 3 – – 20 (4) 12 (17)

 

CJD type unclear/ CJD unlikely 1 1 – 4 1 1 2 – – – – – – 1 – – – – 10 (2) 1 (1)

 

Not CJD 2 1 4 7 7 1 1 – 3 – 1 – 1 1 1 2 – – 29 (6) 3 (4)

 

Other – – 1 1 1 2 1 – – – 1 – – 1 – 1 – – 7 (2) 2 (3)

 

No longer considered ‘at risk’ – – 1 – – – – 1 – – 2 – – – – 2 – – 4 (1) 2 (3)

 

TOTAL 16 38 56 50 45 56 63 27 33 29 23 4 13 40 7 25 – 1 456 70

 

a. In June 2010 a distinction was made between surgical incidents and CJD reports. Only CJD cases (or patients at ‘increased risk’ of CJD) who have undergone surgical procedures which are thought to pose a possible transmission risk (i.e. within the likely infectious incubation period, and involving medium or high risk procedures) are categorised as 'surgical incidents'. Other procedures, either earlier in the incubation period, or involving low infectivity tissues, are categorised as 'CJD reports'.

 

b. There may be an additional surgical incident to report for 2012, investigation is still under way.

 

c. 2013 represents only the first quarter of the year, 1 January to 31 March.

 

Monitoring of patients 'at increased risk' of CJD

 

After a risk assessment has been carried out following a surgical incident, it may be necessary to contact and inform patients of their possible exposure to CJD. These patients should be considered 'at risk of CJD for public health purposes' and are asked to take certain precautions (i.e. not to donate blood, other tissues or organs, and to inform their medical and dental carers prior to any invasive procedures) in order to reduce the risk of transmitting the CJD agent.

 

The diagnosis of the index patient; the timing of the procedure relative to the development of clinical CJD; the tissue with which instruments were in contact during the procedure on the index patient; and the number of cycles of re-use and decontamination the instruments have been through following the procedure on the index case – all influence the possible risk to subsequent patients.

 

The CJD Incidents Panel and the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy Risk Management Subgroup (formerly the ACDP TSE Working Group) identified a range of individuals and groups who may have been exposed to an increased risk of CJD as a consequence of their medical care (see table 2 below). The risks of iatrogenic CJD transmission to these different individuals are very uncertain Health Protection Report Vol. 7 No. 33 - 16 August 2013 and low, but potentially devastating. The CJD Incidents Panel advised that these individuals should be informed of their risk and asked to follow public health precautions to avoid transmitting the infection to others.

 

It is important to follow up these individuals to help determine the risks of CJD spreading to patients through different routes. Follow up involves a range of activities and is carried out by different organisations. The aim is to ascertain whether any people who may have been exposed to an increased risk of contracting CJD go on to develop the disease.

 

Table 2. Summary of all ‘at risk’ groups on which data are collected (Data correct as at 30 June 2013)

 

‘At risk’ Group Identified as ‘at risk’ a Number notified as being ‘at risk’ Clinical cases Asymptomatic infectionsb All Alive

 

Recipients of blood from vCJD cases 67 27 15 3 1

 

Blood donors to vCJD cases 112 107 104 – –

 

Other recipients of blood donors to vCJD cases 34 32c 20c – –

 

Plasma product recipients (all except one are non-bleeding disorders) 11 10 4 – –

 

Surgical contacts of all CJD cases 154 129 d 115e – –

 

Highly transfused patients (recipients of blood from over 80 donors identified at pre-surgical assessment before January 2013) 11 10 7 – –

 

Total for ‘at risk’ groups where HPA holds data 389 315f 265f 3 1

 

Patients with bleeding disorders who received UK sourced plasma products a 3,862 National information incomplete National information incomplete – 1

 

Recipients of human derived growth hormone a 1,883 1,883 1,505 73 –

 

Total for all ‘at risk’ groups a 6,134 >2,198 >1,770 76 2

 

a. These are minimum figures. Central reporting for bleeding disorder patients is incomplete, and seven patients have opted out of the central UKHCDO 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 asymptomatic 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.

 

References

 

1. The National Creutzfeldt-Jakob Disease Research and Surveillance Unit, The University of Edinburgh. CJD statistics. Available at: http://www.cjd.ed.ac.uk/data.html

 

2. The National Creutzfeldt-Jakob Disease Research and Surveillance Unit, The University of Edinburgh. Incidence of variant Creutzfeldt-Jakob disease onsets and deaths in the UK January 1994 - May 2011. Edinburgh: NCJDSU, 18 May 2011. Available at: http://www.cjd.ed.ac.uk/documents/report20.pdf

 

3. CJD Guidance and Advice CJD website, Public Health England (2013)

 

4. Transmissible spongiform encephalopathy agents: safe working and the prevention of infection. The ACDP TSE Risk Management Subgroup.


 

 

 


 

 

 

Prion2013

 

 

Oral.05: Contaminated blood products induce a highly atypical prion disease devoid of PrPres in primates

 

Emmanuel Corney,1 Nina Jaffre,1 Jacqueline Mikol,1 Valerie Durand,1 Christelle Jas-Duval,1,2 Sophie Luccantoni-Freire,1 Evelyne Correia,1 Nathalie Lescoutra-Etcheqaray,3 Nathalie Streichenberqer,4 Stephane Haik,5 Chryslain Sumian,3 Paul Brown1 and Jean-Philippe Deslys1

 

1Commissariat a l'Energie Atomique; Institute of Emerging Diseases and Innovative Therapies (iMETI); Division of Prions and Related Diseases (SEPIA); Fontenay-aux- Roses, France; 2EFS·Nord de France; Lille, France; 3MacoPharm; Tourcoing, France; 4Hospices Civils de Lyon; Prion Unit; Neurobiology Department; Bron, France; 5Inserm; U 975·CNRS; UMR 7225 - Universite Pierre et Marie Curie; Paris, France

 

Background, Concerns about the blood-borne risk of prion infection have been confirmed by the occurrence in the UK of four transfusion-related infections of vCJD and an apparently silent infection in an hemophiliac patient. Asymptomatic incubation periods in prion diseases can extend over decades in humans. We present here unexpected results of experiments evaluating blood transmission risk in a non-human primate model.

 

Material and Methods, Cynomolgus macaques were inoculated with brain or blood specimens from vCJD infected humans or monkeys. Neuropathological and biochemical findings were obtained using current methods used for human patients.

 

Results, Thirteen out of 23 primates exposed to various human or macaque blood products exhibited a previously undescribed myelopathic syndrome, devoid of the classical features of prion disease, notably abnormal prion protein (PrPres) deposition, whereas the 14 corresponding brain-inoculated donor animals and 1 transfused animal exhibited the classical vCJD pattern. In passage experiments, plasma transfusion induced the same atypical phenotype after two years (again, with no detectable PrPres), whereas the intracerebral inoculation of spinal cord led to a typical prion disease with cerebral spongiosis and PrPres accumulation in the brain of the primate recipient. Interestingly, passage experiments in transgenic mice were largely unsuccessful.

 

In another experiment designed to test the efficacy of antiprion filters, three recipients of filtered red blood cells suspended in plasma are still healthy 4.5 y after transfusion whereas the recipients of unfiltered inocula died after 2.5 y with the atypical neurological profile.

 

Conclusion. We describe a new fatal neurological myelopathic syndrome in monkeys exposed to various vCJD/BSE-infected blood components.

 

Secondary transmission in primates confirms

 

(I) the transmissibility of this myelopathy, and

 

(2) its prion origin which could not be diagnosed as such in the first recipients.

 

This myelopathy might be compared in some respects to certain forms of human lower motor neuron disease, including neuromyelitis optica, the flail arm syndrome of amyotrophic lateral sclerosis (ALS), and the recently described FOSMN (facial onset sensory and motor neuronopathy) syndrome.

 

 


 


 

 

 

AD.56: The emergence of novel BSE prions by serial passages of H-type BSE in bovinized mice

 

Kentaro Masujin, Naoko Tabeta, Ritsuko Miwa, Kohtaro Miyazawa, Hiroyuki Okada, Shirou Mohri and Takashi Yokoyama

 

National Institute of Animal Health; Tsukuba, Japan

 

H-type bovine spongiform encephalopathy (BSE) is an atypical form of BSE, and has been detected in several European countries, and North America. Transmission studies of H-type BSE led to the emergence of the classical BSE (C-BSE) phenotypes during passages in inbred wild type and bovinized PrP-overexpressing transgenic mice. In this study, we conducted serial passages of Canadian H-type BSE isolate in bovinized PrP-overexpressing transgenic mice (TgBoPrP). H-type BSE isolate was transmitted to TgBoPrP with incubation periods of 320 ± 12.2 d at primary passage. The incubation period of 2nd and 3rd passage were constant (~= 220 d), no clear differences were observed in their biological and biochemical properties. However, at the forth passage, 2 different BSE phenotypes were confirmed; one is shorter survival times (109 ± 4 d) and the other is longer survival times. TgBoPrP mice with longer incubation period showed the H-type phenotype of PrPsc profile and pathology. However, those of shorter incubation period were different phenotypes from previously existed BSE prions (C-BSE, L-type BSE, and H-type BSE). *** This study imply the possibility that the novel BSE prions with high virulence in cattle will be emerged during intraspecies transmission.

 

 


 


 

 

 

 

see also ;

 

 

 

Thursday, August 15, 2013

 

The emergence of novel BSE prions by serial passages of H-type BSE in bovinized mice

 


 

 

 

PRION2013

 

 

Oral.11: Variant CJD 17 years on

 

Jean C. Manson,1 Matthew Bishop,2 Abigail B. Diack,1 Diane Ritchie,2 Sandra McCutcheon, Richard Alejo Blanco,1 Pedro Piccardo,1,3 James ironside2 and Robert Will2 1The Roslin Institute & R(D)SVS; Easter Bush, UK; 2National CJD Research and Surveillance Unit; University of Edinburgh; Edinburgh, UK; 3Center for Biologics Evaluation and Research; Food and Drug Administration; Rockville, MD USA

 

It is now 17 years since the first identification of a case of vCJD in the UK. Since that time there has been much speculation over how vCJD might impact on human health. To date there have been 176 cases reports in the UK and a further 51 cases worldwide in 11 different countries. It has been important to establish

 

(1) if all worldwide cases represent the same strain of agent;

 

(2) the potential for human to human transmission;

 

(3) if the strain of agent will adapt to the human host and become more virulent; and

 

(4) the extent to which asymptomatic infection may impact on human health.

 

We have now established by examining a number of worldwide vCJD cases that there is broad similarity to UK vCJD cases on first passage to mice. Transgenic mouse studies have indicated that all codon 129 genotypes are susceptible to vCJD and that genotype may influence whether disease appears in a clinical or asymptomatic form, supported by the appearance of the first case of asymptomatic vCJD infection in a PRNP 129MV patient. We have established, by transmission to RIII mice, that there is little evidence for strain modification following human to human transmission of vCJD by blood transfusion of an MM case to an MM or MV recipient. Some differences in incubation times in VM mice observed in these transmissions are now being assessed on second passage to establish if alterations represent differences in strain characteristics or are due to species barrier effects in primary passage.

 

Following evidence of blood transfusion as a route of transmission, we have ascertained that all blood components and leucoreduced blood, in a sheep model of vCJD have the ability to transmit disease.

 

Importantly, we have recently established that a blood recipient with an asymptomatic infection with limited PrPsc deposition in the spleen could readily transmit disease into mice, demonstrating the potential for peripheral infection in the absence of clinical disease. This, along with the recent appendix survey which identified 16 positive appendices in a study of 32,441 cases, underlines the importance of continued CJD surveillance and maintaining control measures already in place to protect human health.

 

References

 

1. Bishop MT, Hart P, Airchison L, Baybutt HN, Plinston C. Thomson V, er al. Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lance. Neurol 2006; 5:393·8; PMID: 16632309; http://dx.doi.org/10.1016/S1474-4422(06)70413-6 2. McCutcheon S, AJejo Blanco AR, Houston EF, de Wolf C. Tan BC, Smith A, et al. All clinically-relevant blood components transmit prion disease following a single blood transfusion: a sheep model of vCJD. PLoS One 2011; 6:e23169; PMID:21858015; http://dx.doi.org/10.1371/journal.pone.0023169

 

3. Diack AB, Ritchie D, Bishop M, Pinion V, Brandel JP, Haik S, e. al. Constant trans- mission properties of variant Creutzfeldt-jakob disease in 5 countries. Emerg Infect Dis 2012; 18: 1574-9; PMID:23017202; http://dx.doi.org/10.3201/eid1810.120792.

 

4. HPA. Health Protection Report 2012; 6(32).

 

5. Bishop MT, Diack AB, Ritchie DL, Ironside JW, Will RG, Manson JC. Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt- Jakob disease. Brain 2013; 136:1139-45; PMID:23449776; http://dx.doi.org/10.1093/brain/awt032

 

 

 

Oral.12: Preclinical detection of variant CJD and BSE prions in blood

 

Caroline Lacroux,1 Jean Yves Douet,1 Emmanuel Corney,2 Hugh Simmons,3 Vincent Beringue,4 Jean Philippe Deslys,2 Didier Vilette1 and Olivier Andreoletti1 1INRA; Toulouse, France; 2CEA; Fontenay aux roses, France; 3AHVLA; Weybridge, UK; 4INRA VIM; Jouy en Josas, France

 

The emergence of variant Creutzfeldt Jakob Disease (vCJD) is considered a likely consequence of human dietary exposure to Bovine Spongiform Encephalopathy (BSE) agent. More recently, secondary vCJD cases were identified in patients transfused with blood products prepared from apparently healthy donors who later went on to develop the disease. As there is no validated assay for detection of vCJD/BSE infected individuals the prevalence of the disease in the population remains uncertain. In that context the risk of vCJD blood borne transmission is considered as a serious concern by health authorities.

 

In this study, appropriate conditions and substrates for highly efficient and specific in vitro amplification of vCJD/BSE agent using Protein Misfolding Cyclic Assay (PMCA) were first identified. This showed that whatever the origin (species) of vCJD/BSE agent, the ovine Q171 PrP substrates provided the best amplification performances. These results indicate that the homology of PrP amino-acid sequence between the seed and the substrate is not the crucial determinant of the vCJD agent in vitro propagation.

 

The ability of this method to detect endogenous vCJD/BSE agent in the blood was then defined. In both sheep and primate models of the disease, the assay enabled the identification of infected individuals in the early preclinical stage of the incubation period. Finally, blood from two vCJD affected patients and 135 healthy controls were tested. The assay detected the presence of the vCJD case within a pool of several dozens of human blood samples. The equivalent 0.05 uL of whole blood from the vCJD affected patient was sufficient for amplifying PrPres. These results open new possibilities for vCJD screening and prevention of its iatrogenic transmission.

 

 

 

Oral.13: Prion detection in urine of patients with variant Creutzfeldt-Jakob disease

 

Fabio Moda,1 Silvio Notari,2 Pierluigi Gambetti,2 Valeria Fuqnanesi,3 Kyung-Won Park,1 Michela Morbin,3 Silvia Suardi,3 Fabrizio Tagliavini3 and Claudio Soto1 1Mitchell Center for Alzheimer's Disease and Related Brain Disorders; University of Texas Medical School; Houston, TX USA; 2Case Western Reserve University; Cleveland, OH USA; 3Carlo Besta Neurological Institute; Milan, Italy

 

Definitive diagnosis of prion disease can only be made by tissue examination and relies on the detection of misfolded prion protein (PrPSc) and associated histopathological changes in the brain. PrPSc is the main component of the infectious agent and is the only validated surrogate marker for the disease. The unique mechanism of prion transmission and the appearance of a new variant form of CJD (vCJD), which has been linked to the consumption of meat from BSE-affected cattle, have raised concerns for public health. Recently, four cases of vCJD have been associated with blood transfusion from asymptomatic donors who subsequently died from vC]D. This suggests that prions exist in the blood of individuals silently incubating the disease. It is likely that minute amount of prions can be present in urine of vCJD patients, as product of blood filtration, but no evidences of prion detection in urine of humans with CJD have been provided so far. With the advent of PMCA (protein mysfolding cyclic amplification) several groups have been able to detect infectious prion in urine of cervids infected by chronic wasting disease (CWD), sheep infected by Scrapie and rodents (mouse and hamsters) infected with different prion agents. Taking advantage of the extreme sensitivity of PMCA, we have analyzed urine from several patients suffering from different forms of CJD (variant, sporadic and genetic). Of all the cases analyzed, only those affected by vCJD were found to contain PrPSc that can be amplified to obtain a signal that has the typical electrophoretic profile of PrPSc associated to vCJD. Many controls including urine from patients affected by other neurological diseases (Alzheimer disease, Frontotemporal dementia, Parkinson disease, progressive supranuclear palsy), as well as healthy control subjects were all found to be negative for PrPSc in urine. To the best of our knowledge, this is the first report showing the presence of prions in human urine. Due to the limited number of vCJD urine samples examined, we cannot definitively establish whether PrPSc presence is common in all vCJD patients or depends on the existence of other pathologies (e.g., renal malfunction). The detection of prions in human urine may be utilized as a non-invasive diagnostic test of vCJD, and also uncovers a possible risk related with the use of urinary-derived products (hormones, enzymes and other proteins) as well as the collection and disposal of urine from vCJD patients.

 

Acknowledgments. This research was partially fund by NIH grants R42NS079060, P01AI077774 and P01AG14359 to CS and P01AG14359 and Charles S. Britton Fund to PG.

 

 


 


 

 

 

 

RE-Oral.13: Prion detection in urine of patients with variant Creutzfeldt-Jakob disease



ahhh, the fruits of Harash Narang's work still paying off. ...


 

 

1996 NARANG URINE TEST

http://collections.europarchive.org/tna/20081106082948/http://www.bseinquiry.gov.uk/files/yb/1996/02/09003001.pdf


http://collections.europarchive.org/tna/20080102200511/http://www.bseinquiry.gov.uk/files/yb/1996/02/21004001.pdf


http://collections.europarchive.org/tna/20081106042311/http://www.bseinquiry.gov.uk/files/yb/1996/02/21005001.pdf


http://web.archive.org/web/20030527133551/http://www.bseinquiry.gov.uk/files/ws/s113.pdf

http://web.archive.org/web/20010305030338/http://www.bseinquiry.gov.uk/report/volume11/chaptec3.htm



5.289 We have concluded, for the reasons given above, that Dr Narang's work received fair consideration by MAFF scientists. While we would pay tribute to Dr Narang's dedication to research into TSEs, we feel that he had a fair opportunity to demonstrate the validity of his work but did not succeed in doing so.


8. I was in receipt of no extra funds beyond those provided by the NHS and the University of London to run my laboratories and pay my salary as a senior lecturer/honorary Consultant and I suffered no constraints over my publications, lectures to my students, or statements to the media. However, I became increasingly aware after 1988 that questioning official dogma about BSE brought difficulties to one’s career. I was myself about to retire from the Charing Cross Hospital, where I worked as a Consultant Neuropathologist, but I observed with horror that the good reputations of dissenting scientists in the field, not least Dr Stephen Dealler and especially Dr Harash Narang were systematically undermined.
http://collections.europarchive.org/tna/20080102135133/http://www.bseinquiry.gov.uk/files/ws/s410.pdf

 

snip...see more here ;

 

 

Thursday, September 30, 2010

 

Characterization of the Prion Protein in Human Urine*

 


 

 

 

Sunday, February 10, 2013

Creutzfeldt-Jakob disease with unusually extensive neuropathology in a child treated with native human growth hormone
http://creutzfeldt-jakob-disease.blogspot.com/2013/02/creutzfeldt-jakob-disease-with.html



Tuesday, March 5, 2013

Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION)

FDA believes current regulation protects the public from BSE but reopens comment period due to new studies
http://transmissiblespongiformencephalopathy.blogspot.com/2013/03/use-of-materials-derived-from-cattle-in_6452.html




 

 

 

 

greetings,

 

 

just my opinion, but some times I think they are making these different TSE prion disease names up as they go along.

 

we have a TSE prion disease i.e. vCJD in humans, caused by BSE TSE prion disease in the bovine, and now we have an entirely different disease or diseases named as the by-product of the vCJD.

 

now instead of a encephalopathy, we now have a myelopathy, last it was a prionpathy, the prionopathy, then proteinopathy?  

 

seems they are really grasping for straws now, trying to eliminate any link to any other TSE in livestock, or friendly fire therefrom, and now they are going to change transmission there from to a different disease entity altogether.

 

changing the names of all the different strains of the TSE prion disease, does not change the science, and the facts, of the transmission of the different TSE prion strains, it only makes one wonder, why this ? ($$$)

 

 

 

different strains (of same disease), different species (of same disease), different routes of infection (of same disease), different infectivity levels (dose rate) of the (same disease), different age groups, different human and animal genetics, = different symptoms, different lengths of illness from 1st onset of illness to death, (of the same disease) = different strains (of same disease)...TSS

 

 

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

 

The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why???? than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!!

 

Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....

 

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

 

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

 

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

 

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

 

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

 

 

 

Sunday, August 09, 2009

 

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

 


 

 

 

Tuesday, August 18, 2009

 

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

 


 

 

 

 

see more from Prion2013 below ;

 

 

 

Sunday, August 11, 2013

 

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 (Prion2013)

 


 

 

 

*** 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.

 


 

 

Friday, July 19, 2013

 

Beaumont Hospital in Dublin assessing patients for CJD

 


 

 

Saturday, July 6, 2013

 

Small Ruminant Nor98 Prions Share Biochemical Features with Human Gerstmann-Sträussler-Scheinker Disease and Variably Protease-Sensitive Prionopathy

 

Research Article

 


 

 

Monday, May 6, 2013

 

Warning of mad cow disease threat to blood transfusions

 


 

 

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, May 28, 2013

 

Late-in-life surgery associated with Creutzfeldt-Jakob disease: a methodological outline for evidence-based guidance

 


 

 

Sunday, June 9, 2013

 

TSEAC March 14, 2013: Transmissible Spongiform Encephalopathies Advisory Committee Meeting Webcast

 


 

 

Tuesday, May 21, 2013

 

CJD, TSE, PRION, BLOOD Abstracts of the 23rd Regional Congress of the International Society of Blood Transfusion, Amsterdam, The Netherlands, June 2-5, 2013

 


 

 

Tuesday, March 5, 2013

 

Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION)

 

FDA believes current regulation protects the public from BSE but reopens comment period due to new studies

 


 

 

Friday, June 29, 2012

 

Highly Efficient Prion Transmission by Blood Transfusion

 


 

 

Thursday, October 25, 2012

 

Current limitations about the cleaning of luminal endoscopes and TSE prion risk factors there from

 

Article in Press

 


 

 

Saturday, January 16, 2010

 

Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al

 

Evidence For CJD/TSE Transmission Via Endoscopes

 

From Terry S. Singletary, Sr flounder@wt.net 1-24-3

 


 

 

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

 


 

 

Friday, February 10, 2012

 

Creutzfeldt-Jakob disease (CJD) biannual update (2012/1) potential iatrogenic (healthcare-acquired) exposure to CJD, and on the National Anonymous Tonsil Archive

 


 

 

Monday, November 26, 2012

 

Aerosol Transmission of Chronic Wasting Disease in White-tailed Deer

 


 

 

Thursday, December 29, 2011

 

Aerosols An underestimated vehicle for transmission of prion diseases?

 

PRION www.landesbioscience.com

 

please see more on Aerosols and TSE prion disease here ;

 


 

 

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 ???

 


 

 

Tuesday, December 14, 2010

 

Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings part 4, Annex A1, Annex J,

 

UPDATE DECEMBER 2010

 


 

 

Tuesday, September 14, 2010

 

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

 


 

 

Thursday, September 02, 2010

 

NEUROSURGERY AND CREUTZFELDT-JAKOB DISEASE Health Law, Ethics, and Human Rights The Disclosure Dilemma

 


 

 

Thursday, August 12, 2010

 

USA Blood products, collected from a donor who was at risk for vCJD, were distributed July-August 2010

 


 

 

Sunday, August 01, 2010

 

Blood product, collected from a donors possibly at increased risk for vCJD only, was distributed USA JULY 2010

 


 


 

 

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

 


 

 

Thursday, July 08, 2010

 

GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010

 


 

 

Wednesday, June 02, 2010

 

CJD Annex H UPDATE AFTER DEATH PRECAUTIONS Published: 2 June 2003 Updated: May 2010

 


 

 

Tuesday, May 11, 2010

 

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

 


 

 

Tuesday, May 04, 2010

 

Review of the Human Pituitary Trust Account and CJD Issue 20 January 2010

 


 

 

Tuesday, March 16, 2010

 

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

 


 

 

Monday, August 17, 2009

 

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J,K, AND D Published: 2009

 


 

 

Monday, July 20, 2009

 

Pre-surgical risk assessment for variant Creutzfeldt-Jakob disease (vCJD) risk in neurosurgery and eye surgery units

 


 

 

Friday, July 17, 2009

 

Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units Volume 3 No 28; 17 July 2009

 


 

 

Sunday, May 10, 2009

 

Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

 


 

 

Thursday, January 29, 2009

 

Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008 (WARNING TO Neurosurgeons and Ophthalmologists) Volume 15, Number 2-February 2009 Research

 


 

 

Wednesday, August 20, 2008

 

Tonometer disinfection practice in the United Kingdom: A national survey

 


 

 

Tuesday, August 12, 2008

 

Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)

 


 

 

Monday, December 31, 2007

 

Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation

 


 

 

Subject: CJD: update for dental staff

 

Date: November 12, 2006 at 3:25 pm PST

 

1: Dent Update. 2006 Oct;33(8):454-6, 458-60.

 

CJD: update for dental staff.

 


 

 

Saturday, March 23, 2013

CJD Incidents Panel to be disbanded
http://creutzfeldt-jakob-disease.blogspot.com/2013/03/cjd-incidents-panel-to-be-disbanded.html



Thursday, February 21, 2013

National Prion Disease Pathology Surveillance Center Cases Examined January 16, 2013
http://prionunitusaupdate2008.blogspot.com/2013/02/national-prion-disease-pathology.html



16 YEAR OLD SPORADIC FFI ?



Monday, January 14, 2013

Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe
http://transmissiblespongiformencephalopathy.blogspot.com/2013/01/gambetti-et-al-usa-prion-unit-change.html


Monday, December 31, 2012

Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012
http://creutzfeldt-jakob-disease.blogspot.com/2012/12/creutzfeldt-jakob-disease-and-human-tse.html


Tuesday, December 25, 2012

CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25, 2012
http://creutzfeldt-jakob-disease.blogspot.com/2012/12/creutzfeldt-jakob-tse-prion-disease.html


Tuesday, June 26, 2012

Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012

type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA
http://creutzfeldt-jakob-disease.blogspot.com/2012/06/creutzfeldt-jakob-disease-human-tse.html


Wednesday, June 13, 2012

MEXICO IS UNDER or MIS DIAGNOSING CREUTZFELDT JAKOB DISEASE AND OTHER PRION DISEASE SOME WITH POSSIBLE nvCJD
http://creutzfeldt-jakob-disease.blogspot.com/2012/06/mexico-is-under-or-mis-diagnosing.html



*** 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.



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.


http://www.landesbioscience.com/journals/prion/01-Prion6-2-OralPresentations.pdf?nocache=1216084967


Wednesday, March 28, 2012

VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $
http://prionopathy.blogspot.com/2012/03/variably-protease-sensitve-prionopathy.html



Thursday, April 4, 2013

Variably protease-sensitive prionopathy in the UK: a retrospective review 1991–2008

Brain (2013) 136 (4): 1102-1115. doi: 10.1093/brain/aws366
http://prionopathy.blogspot.com/2013/04/variably-protease-sensitive-prionopathy.html


Sunday, March 31, 2013

Creutzfeldt Jakob Disease CJD worlds youngest documented victim, 11 years old, shall we pray
http://creutzfeldt-jakob-disease.blogspot.com/2013/03/creutzfeldt-jakob-disease-cjd-worlds.html



Saturday, April 20, 2013

Insight into the frequent occurrence of dura mater graft-associated Creutzfeldt-Jakob disease in Japan
http://creutzfeldt-jakob-disease.blogspot.com/2013/04/insight-into-frequent-occurrence-of.html


Sunday, May 19, 2013

CJD BLOOD SCREENING, DONORS, AND SILENT CARRIERS House of Commons Written Answers 16 May 2013
http://creutzfeldt-jakob-disease.blogspot.com/2013/05/cjd-blood-screening-donors-and-silent.html

 

 

Sunday, February 10, 2013

Creutzfeldt-Jakob disease (CJD) biannual update (February 2013) Infection report/CJD
http://creutzfeldt-jakob-disease.blogspot.com/2013/02/creutzfeldt-jakob-disease-cjd-biannual.html

 

 

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 ;

 


 

 

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...

 


 

 

Saturday, June 25, 2011

 

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

 

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

 


 

 

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...

 


 

 

Monday, September 26, 2011

 

L-BSE BASE prion and atypical sporadic CJD

 


 

 

 

 

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