Creutzfeldt-Jakob disease (CJD) biannual update (2012/1) potential iatrogenic (healthcare-acquired) exposure to CJD, and on the National Anonymous Tonsil Archive
From 2000 to 31 December 2011, there have been 26 surgical incidents in which the Panel has advised that 190 patients should be considered to have an increased risk of CJD.
There are 13 surgical incidents in which 152 patients are still considered to be at increased risk of CJD. Currently, 119 of these 'at risk' patients have been notified that they are at increased risk of CJD. Local decisions have been taken not to notify four patients in these incidents.
Creutzfeldt-Jakob disease (CJD) biannual update (2012/1)
This six-monthly report provides an update on reports of incidents of potential iatrogenic (healthcare-acquired) exposure to CJD, and on the National Anonymous Tonsil Archive. The data are correct as of 3 February 2012.
For numbers of CJD case reports, readers should consult data provided by the National CJD Research and Surveillance Unit (NCJDRSU), Edinburgh [1]. The latest yearly analysis of vCJD reports (onsets and deaths) is also available from the NCJDRSU website [2].
Reports of incidents of potential iatrogenic exposure to CJD via surgery: 2000 to 31 December 2011.
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 [3]. This could happen if a patient undergoes surgery during the incubation period of CJD, or because information about CJD risk factors is not available at the time of surgery. If this happens, surgical instruments that may be contaminated with the infectious agent that causes CJD, could pose a transmission risk when they are re-used on other patients.
In June 2010 the CJD Incidents Panel changed its protocol for reporting surgical incidents, and a new reporting algorithm was published on the HPA CJD Section website. Under the new protocol only CJD cases (or patients at increased risk of CJD) who have undergone surgical procedures which are thought to pose a possible transmission risk (ie 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'.
Table 1 shows the number of CJD surgical incidents reported to the CJD Incidents Panel from 2000 to 31 December 2011 by the diagnosis of the index patient. Advice has been issued for five surgical incidents and 37 CJD reports that were reported to the CJD Incidents Panel in 2011.
Information about the CJD Incidents Panel can be found on the HPA website [4].
Table 1: CJD surgical incidents (n=437) reported to the CJD Incidents Panel (which have been closed, or where advice has been issued) by diagnosis of index patient: 2000 to 30 June 2011
Index patient status | '00 | '01 | '02 | '03 | '04 | '05 | '06 | '07 | '08 | '09 | '10 | '11 | Total incidents (% of total) | Total CJD reports | ||
| Incid'ts | Rep'ts | Incid'ts | Rep'ts | |||||||||||||
Sporadic (possible, probable or definite) | 7 | 19 | 22 | 24 | 16 | 18 | 31 | 17 | 21 | 15 | 5 | 4 | 1 | 22 | 196 (44%) | 26 (63%) |
vCJD (possible, probable or definite) | 6 | 14 | 22 | 5 | 4 | 1 | 2 | – | 1 | 1 | – | – | – | 1 | 56 (13%) | 1 (2%) |
Familial including 'at risk' familial | – | 2 | 2 | 7 | 1 | 3 | 7 | – | 2 | 3 | 2 | – | – | 1 | 29 (7%) | 1 (2%) |
'At risk' vCJD blood component recipient | – | – | – | – | 4 | 10 | 5 | 1 | – | – | 2 | – | – | – | 22 (5%) | – |
'At risk' - vCJD plasma product recipient | – | 1 | 2 | – | 10 | 18 | 9 | 8 | 6 | 9 | 3 | – | 4 | 2 | 70 (16%) | 2 (5%) |
'At risk' - other | – | – | 2 | 2 | 1 | 2 | 5 | – | – | 1 | 7 | – | - | 9 | 20 (5%) | 9 (22%) |
CJD type unclear/ CJD unlikely | 1 | 1 | – | 4 | 1 | 1 | 2 | – | – | – | – | – | – | 1 | 10 (2%) | 1 (2%) |
Not CJD | 2 | 1 | 4 | 7 | 7 | 1 | 1 | – | 3 | – | 1 | – | – | – | 27 (6%) | – |
Other | – | – | 1 | 1 | 1 | 2 | 1 | – | – | – | 1 | – | – | 1 | 7 (2%) | 1 (2%) |
No longer considered 'at-risk' | – | – | 1 | – | – | – | – | 1 | – | – | 2 | – | – | – | 4 (1%) | – |
Total – | 16 | 38 | 56 | 50 | 45 | 56 | 63 | 27 | 33 | 29 | 23 | 4 | 5 | 37 | 441 | 41 |
If the investigation of a surgical incident identifies any instruments that are considered to be potentially contaminated with the infectious agent, and that could still pose an infection risk to other patients, the Panel advises that these instruments should be removed from general use or refurbished. These instruments may be quarantined, kept for exclusive use on the index patient, refurbished (endoscopes only) or destroyed.
Since 2000 there have been 86 incidents in which instruments have been permanently removed from general use or refurbished (endoscopes only).
Surgical incidents resulting in ‘at risk’ patients
The Panel may advise contacting and informing patients of their possible exposure to CJD following a surgical incident. These patients should be considered 'at-risk of CJD for public health purposes' and are asked to take certain precautions (ie 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 that instruments were in contact with 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 threshold level of risk at which patients are considered to be ‘at increased risk’ of CJD is 1%, in addition to the background risk in the UK population. This risk threshold is based on risk assessment models, using precautionary assumptions. The 1% threshold level is used as a cut off for implementing public health precautions and is not intended to be a precise measure of an individual patient's risk. A similar threshold is used for identifying other patients who have been exposed to possible CJD risks following surgical, blood, plasma and tissue incidents.
From 2000 to 31 December 2011, there have been 26 surgical incidents in which the Panel has advised that 190 patients should be considered to have an increased risk of CJD.
Patient denotifications
Following changes in the assessment of tissue infectivity, the Panel has advised that 38 patients in 14 surgical incidents who were originally considered (and notified) as being ‘at risk' of CJD should no longer be considered ‘at risk', and should be denotified. In November 2005, gastrointestinal endoscopies without invasive procedures were reclassified as low risk procedures, and advice was issued to denotify two patients in one surgical incident. In 2006, anterior eye was reclassified as a ‘medium low' infectivity tissue. This led to a change in advice as only the first patient on whom instruments were used following an anterior eye procedure was to be considered as having an increased risk of CJD. Previously this had applied to the first two patients exposed to such instruments. This resulted in the Panel advising that 16 patients in seven incidents should be denotified. In 2009, the anterior eye was further reclassified as a low infectivity tissue. Following this change, the Panel advised that 20 patients should be denotified.
As of 31 December 2011, the Panel has received confirmation that of the 33 patients originally notified of their exposure (out of the 38 originally considered to be ‘at risk'), 25 patients have been informed that they are no longer considered ‘at risk' and eight patients died before they could be denotified.
Current 'at risk' patients resulting from surgical instruments
There are 13 surgical incidents in which 152 patients are still considered to be at increased risk of CJD. Currently, 119 of these 'at risk' patients have been notified that they are at increased risk of CJD. Local decisions have been taken not to notify four patients in these incidents.
Table 2: Surgical ‘at risk’ patients still identified as being ‘at increased risk of CJD’ by the Panel by procedure on the index patient
Diagnosis of index patient | Procedure on index patient | Number of incidents | Patients identified as 'at risk' | Patients who died before being notified | Local decision not to notify patient | Notified patients |
| Sporadic | Brain biopsy | 2 | 28 | 2 | 1 | 25 |
| Variant | Appendectomy | 1 | 2 | – | 2 | – |
| Variant | Endoscopy | 1 | 1 | – | 1 | – |
| Asymptomatic infected vCJD | Endoscopy | 1 | 4 | 1 | – | 3 |
| At risk variant | Endoscopy | 5 | 36 | 4 | – | 32 |
| At risk familial | Neurosurgery | 1 | 31 | 10 | – | 21 |
| At risk familial | Ophthalmic surgery | 1 | 39 | 1 | – | 38 |
| At risk sporadic | Ophthalmic surgery | 1 | 11* | 2 | – | – |
| Total | 13 | 152 | 20 | 4 | 119 |
Monitoring of patients 'at increased risk' of CJD
The CJD Incidents Panel and the Advisory Committee on Dangerous Pathogens Transmissible Spongiform Encephalopathy Risk Management Subgroup (formerly the ACDP TSE Working Group) have 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 3 below). The risks of iatrogenic CJD transmission to these different individuals are very uncertain, but potentially devastating. The CJD Incidents Panel has 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. At core, follow up aims to ascertain whether any people who may have been exposed to increased CJD risks go on to develop CJD.
Table 3. Individuals at increased risk of CJD up to 31 December 2011
'At risk' Group | Identified as 'at risk' | Ever notified as being 'at risk' | Alive and Notified | Cases | Asymptomatic infections |
| Recipients of blood from vCJD cases | 67 | 27 | 18 | 3 | 1 |
| Blood donors to vCJD cases | 112 | 107 | 104 | – | – |
| Other recipients from blood donors to vCJD cases | 34 | 32 | 23 | – | – |
| Plasma product recipients (all except one have non-bleeding disorders) | 11 | 10 | 4 | – | – |
| Surgical contacts of all CJD cases | 141 | 119 | 110 | – | – |
| Highly transfused patients (recipients of blood from ≥80 donors identified at pre-surgical assessment) | 9 | 7 | 6 | – | – |
| Total for at risk groups where HPA holds data | 374 | 302 | 265 | 3 | 1 |
| Patients with bleeding disorders who received UK sourced plasma products [a] | 3,868 | n/k | n/k | – | 1 |
| Recipients of human derived growth hormone [b] | 1,883 | 1,883 | 1,521 | 67 | – |
| Total for all 'at risk' groups [c] | 6,125 | At least 2,184 | At least 1,786 | 70 | 2 |
Update on summary of abnormal prion prevalence
The National Anonymous Tonsil Archive (NATA) was set up in 2004 to prospectively collect 100,000 tonsils pairs obtained after routine tonsillectomies in England and Scotland and to test these samples for abnormal prion protein. Only tissues not required for patient care, which would normally be discarded, were collected.
The NATA work is now finished with recruitment and testing of specimens stopped. Up to the end of September 2011, NATA received a total of 95,672 tonsil pairs from hospitals in England and Scotland, about 18,003 of which are from the birth cohort in which most vCJD cases have arisen (1961-1985) (figure 1). A further 3,010 tonsil pairs were received from the Medical Research Council Prion Unit at the UCL Institute for Neurology, National Hospital for Neurology and Neurosurgery. Therefore the total number of tonsil pairs in the archive is 98,682. The number of collection forms that were completed but where no tonsil tissue was collected was 2,557 (1,671 due to patient objection and 886 due to clinical pathology being requested for the sample).
Figure 1. Number of tonsil pairs collected by birth cohort: January 2004 to September 2011
Of the 100 NHS Hospital Trusts that perform over 200 tonsillectomies per year in England, 91 were recruited into the study and sent tonsil pairs to NATA on a regular basis. There were 120 hospital sites within these trusts taking part in NATA. During the seven year period, approximately 50,000 tonsillectomies were performed annually in England. Figure 2 shows the number of tonsil pairs received from each Strategic Health Authority area.
Figure 2. Tonsil pairs collected by Strategic Health Authority: January 2004 to September 2011 3. NHS Trusts and Scottish hospitals currently collecting and sending tonsil tissue to the archive July 2011
The tonsils have been screened for abnormal prion protein by two enzyme immunoassays (EIAs) and a small proportion selected for additional investigative analytical tests such as immunohistochemical testing (IHC) and Western blot tests. No positives were found.
An earlier study of appendix and some tonsil tissue, from operations conducted between 1995 and 1999, found three positive samples out of 12,674 screened for abnormal prion protein using the IHC method [5]. The prevalence estimate calculated from this study was 237 per million overall (95% CI: 49-692 per million) – or 380 per million (95% CI=80-1120 per million) in those born between 1961 and 1985. This prevalence estimate equals to one infection per 4,000 of the population in the 1961 to 1985 birth cohort, the cohort in which most vCJD cases have arisen.
In 2008, the Spongiform Encephalopathy Advisory Committee (SEAC) considered the available NATA data [6]. At that time no abnormal prion protein positive samples had been found in nearly 55,000 samples, analysed by the high throughput dual EIA screening technique, including about 11,000 samples from the 1961 to 1985 birth cohort [7]. This translated to a prevalence estimate of zero per million (95% CI=0-324 per million) in this birth cohort. Statistical analysis of the data from the previous appendix survey and NATA showed the prevalence rates were consistent with each other.
To further investigate the prevalence of abnormal prion protein in the NATA samples from patients in the 1961 to 1985 birth cohort, the IHC technique was applied to screen 9,160 samples within that cohort; these samples which had already been shown to be negative when screened by the dual EIA technique [8]. One specimen showed a single strongly positive follicle. The specimen was negative when further investigated by EIA, IHC, and immunoblotting. This finding of 1 in 9,160 gives a prevalence estimate of 109 infections per million (95% CI: 3-608 per million) in those born between 1961 and 1985 which is not statistically different from the prevalence estimate based on the previous appendix study (exact p =0.64).
The results of NATA and the “IHC screening” sub-study were considered by SEAC in 2010 [9]. The committee agreed that “even though the positive sample suggested a possible prevalence of 1:10,000, the result from the Hilton data of 1:4,000 would still remain the most precautionary figure for risk management purposes”.
The Health Protection Agency is also coordinating a second Appendix Survey in order to test for abnormal prion protein in archived appendix tissues in two birth cohorts of individuals[10]. The study is an unlinked anonymous survey testing approximately 20,000 samples from individuals born between 1961 and 1985 and 10,000 from those born between 1941 and 1960. The study is continuing but the interim data shows that four out of 13,878 suitable samples across both birth cohorts have tested positive for disease associated prion protein (PrP CJD ). This translates to a prevalence estimate of 288 per million (95% CI=79-738 per million). Two of the four positive samples were from the 1941-1960 birth cohort resulting in a prevalence estimate of 641 per million (95% CI=78-2314 per million) compared to 186 per million (95% CI=23-671 per million) in the younger cohort of individuals. The interim data has been reviewed by the ACDP TSE Risk Assessment Sub-Group [11].
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/figures.htm.
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/cjdq68.pdf.
3. Transmissible spongiform encephalopathy agents: safe working and the prevention of infection. The ACDP TSE Risk Management Subgroup. http://www.dh.gov.uk/ab/ACDP/TSEguidance/index.htm.
4. HPA CJD Incidents Panel [online]. Available at: http://www.hpa.org.uk/web/ CJDIncidentsPanel.
5. Hilton DA, Ghani AC, Conyers L, Edwards P, McCardle L, Ritchie D, et al. Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol 2004; 203: 733-9.
6. Spongiform Encephalopathy Advisory Committee (SEAC). Prevalence of subclinical variant Creutzfeldt-Jakob Disease infections. August 2008, SEAC position statement; de Marco MF, Linehan J, Gill ON, Clewley JP, Brander S. Large Scale immunohistochemical examination for lymphoreticular prion protein in tonsil specimens collected in Britain. J Pathol 2010; 222: 380-7.
7. Clewley J, Kelly CM, Andrews N, Vogliqi K, Mallinson G, Kaisar M, et al. Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey. BMJ 2009; 338: b1442.
8. de Marco MF, Linehan J, Gill ON, Clewley JP, Brander S. Large Scale immunohistochemical examination for lymphoreticular prion protein in tonsil specimens collected in Britain. J Pathol 2010; 222: 380-7.
9. Spongiform Encephalopathy Advisory Committee (SEAC). Minutes of the 104th Meeting held on 5 March 2010.
10. HPA Health Protection Report. Interim data from the current national survey of abnormal prion prevalence in archived appendix specimens. September 2011. Volume 5 No 36. Available at: http://www.hpa.org.uk/hpr/archives/2011/news3611.htm#cjd.
11. Advisory Committee on Dangerous Pathogens TSE Risk Assessment SubGroup. Minutes 14 July 2011. ACDP TSE RA Minutes.
Emerging infections/CJD
Creutzfeldt-Jakob disease (CJD) biannual update (2010/1)
This six-monthly report provides an update on reports of incidents of potential iatrogenic (healthcareacquired)
exposure to CJD via surgery, and on the National Anonymous Tonsil Archive. Data are correct as
of 26 January 2010.
For numbers of CJD case reports, readers should consult data provided by the national CJD Surveillance
Unit (NCJDSU), Edinburgh [1]. The latest yearly analysis of vCJD reports (onsets and deaths) is also
available from the NCJDSU website [2].
Reports of incidents of potential iatrogenic exposure to CJD via surgery:
2000 to 31 December 2009
Since the previous update report [3], 19 surgical incidents were reported – between 1 July and 31 December 2009 – bringing the total number reported since 2000 to 407 (table 1). A surgical incident occurs when a patient undergoes surgery but is only identified as having CJD or being at risk of CJD at a later date. This means that the ACDP TSE Working Group infection control guidelines would not have been followed. The surgery carried out on an index patient with, or at risk of CJD, may result in contamination of the instruments with abnormal prion protein. Table 1 shows the number of CJD surgical incidents reported to the CJD Incidents Panel from January 2000 to 31st December 2009 by the diagnosis of the index patient. Information about the CJD Incidents Panel can be found on the HPA website [4].
Table 1. Closed CJD Surgical Incidents (n=407) reported to the CJD Incidents Panel, by diagnosis of
index patient: 1 January 2000 to 30 June 2009
Incident type 2000 2001 2002 2003 2004 2005 2006 2007 2008
First
half
2009
Total
1. Sporadic (possible, probable
or definite)
7 19 22 24 16 18 31 17 21 11 186(46%)
2. vCJD (possible, probable or
definite)
6 14 22 5 4 1 2 – 1 1 56(14%)
3. Familial including 'at risk'
familial
– 2 2 7 1 3 7 – 2 2 26(6%)
4. 'At risk' vCJD blood
component recipient
– – – – 4 10 6 1 – – 21(5%)
5. 'At risk' - vCJD plasma product
recipient
– 1 2 – 10 18 9 8 6 8 62(15%)
6. 'At risk' - other – – 2 2 1 2 4 – – 1 12(3%)
7. CJD type unclear/ CJD unlikely 1 1 4 1 1 2 – – – 10(2%)
8. Not CJD 2 1 4 7 7 1 1 – 3 – 26(6%)
9.Other – – 1 1 1 2 1 – – – 6(1%)
10. No longer considered 'at-risk' – – 1 – – – – 1 – – 2(0%)
Total 16 38 56 50 45 56 63 27 33 23 407(100%)
Investigation of surgical incidents may result in advice to remove surgical instruments from clinical use (to quarantine, destroy, or donate for research). Such advice is generally only given for instruments considered to be potentially contaminated with the CJD agent that have not undergone a certain number of cycles of use and decontamination since their use on an index patient. Hospitals are asked to consider sending any instruments to be permanently removed from use to the Surgical Instrument Store (held by the Health Protection Agency, Porton Down) for research. Since 2000, there have been 46 incidents in which instruments were permanently removed from use.
The Panel may advise contacting and informing some patients of their possible exposure to CJD in a surgical incident. Such advice is generally only given for patients who have definitely been exposed to
Health Protection Report Vol 4 No. 7 - 19 February 2010
potentially contaminated instruments which have been used on risk tissues in certain index patients. The Panel may advise that some of these patients should be considered „at-risk of CJD for public health purposes' and asked to take certain precautions (ie, not to donate blood or other tissues and to inform their medical and dental carers prior to any invasive procedures) in order to reduce the risk of transmitting the CJD agent further. Since 2000, 22 incidents have given rise to such advice. There are currently nine incidents in which 77 patients have been categorised as „at-risk' by the Panel, according to the current risk assessment. Seven of these patients died before notification. A total of 31 patients are currently notified of their „at-risk' status. Notifications are pending for another 31 patients. Three patients have not been notified due to local, clinical decisions.
The Panel has revised its advice on endoscopy and anterior eye patients. This has led to patients being denotified in 2006 and 2009. This resulted in reclassification of 38 patients from the „at-risk' category; 32 in anterior eye surgery, two in invasive endoscopy.
Table 2. Panel advice to inform patients that they are ‘at-risk' of CJD/vCJD: 1 January 2000 to 30 June
2009
Diagnosis
of index
patient
Procedure on
index patient
Number
of
Incidents
Alive 'at-risk'
Died before
notification
Total
Notified
Not
notified
Total
Sporadic CJD Brain biopsy 2 20 1* 21 2 28
vCJD Appendectomy 1 – 2* 2 – 2
Endoscopy and GI
surgery
2† 3 1 ** 4 1 5
'At risk' vCJD
Endoscopy and GI
surgery
4 8 30** 38 4 42
Total – 9 31 34 65 7 77
*Local decision not to notify.
† The index patient in one of these incidents was a haemophiliac plasma product recipient with evidence of vCJD infection.
** Notification pending.
National anonymous tonsil archive for studies of detectable abnormal prion protein
The National Anonymous Tonsil Archive (NATA) continues to receive approximately 250 tonsil pairs per week (figure 1). The archive had received a total of 81,604 tonsil pairs up to the end of December 2009 from hospitals in England and Scotland. A further 3,000 tonsil pairs have been received from the Medical Research Council Prion Unit at the Institute for Neurology, National Hospital for Neurology and Neurosurgery. Therefore the total number of tonsil pairs in the archive was 84,604. The number of collection forms that were completed but no tonsil tissue collected was 2,395 (1,565 due to patient objection and 830 due to clinical pathology being requested).
Out of the 100 NHS Hospital Trusts that perform over 200 tonsillectomies per year in England, 91 have been recruited and are currently sending tonsil pairs to NATA on a regular basis. There are 120 hospitals sites within these trusts taking part in NATA. At present, approximately 50,000 tonsillectomies are performed annually in England. Figure 2 shows the number of tonsil pairs received from each Strategic Health Authority.
Health Protection Report Vol 4 No. 7 - 19 February 2010
Figure 1. Number of tonsil pairs collected for NATA quarterly: Q1 2004 – Q4 2009
Just over 5,000 tonsillectomies are performed in Scotland each year. The project in Scotland, where there are 14 hospitals that each carry out more than 200 tonsillectomies per year, is being coordinated by Health Protection Scotland. All fourteen of these hospitals have been recruited and are collecting tonsils for NATA. The tonsil tissue is being transported to the Health Protection Agency in Colindale for inclusion in the archive. Figure 3 shows all hospitals in England and Scotland currently recruited in the study.
Figure 2. Tonsils pairs collected by Strategic Health Authority, January 2004 - December 2009
Health Protection Report Vol 4 No. 7 - 19 February 2010
Figure 3. NHS Trusts and Scottish Hospitals currently collecting and sending tonsil tissue to the archive December
2009
Testing of homogenates of the tonsil tissue from the archive began at the end of January 2007. Two enzyme immunoassays (EIAs) are being used for the initial screening of the homogenates for the presence of abnormal prion protein. These EIAs allow the identification of any tonsils that need to be investigated further by the more specific tests of Western blotting (WB) and immunohistochemistry (IHC) [5].
References
1. The National Creutzfeldt-Jakob Disease Surveillance Unit, The University of Edinburgh. CJD statistics.
Available at: http://www.cjd.ed.ac.uk/figures.htm.
2.The National Creutzfeldt-Jakob Disease Surveillance Unit, The University of Edinburgh. Incidence of variant Creutzfeldt-Jakob Disease Onsets and Deaths in the UK January 1994 - December 2006. Edinburgh:
NCJDSU, 2 February 2007. Available at: http://www.cjd.ed.ac.uk/vcjdqdec06.htm.
3. HPA. Biannual CJD update (2009/1). Health Protection Report [serial online] 2009; 3(27): Emerging
Infections/CJD. Available at: http://www.hpa.org.uk/hpr/archives/2009/hpr2709.pdf.
4. HPA. CJD Incidents Panel [online]. London: HPA, 2010. Available at:
5. Spongiform Encephalopathy Advisory Committee. Combining evidence from tissue surveys to estimate the prevalence of subclinical vCJD. London: SEAC, 2008. Available at:
Greetings,
to bad this kind of report does not come out in the USA. no telling what they might find, if they looked. considering all iatrogenic CJD is, is sporadic CJD, until source and route is documented, and considering the total TSE prion exposure rate to humans in North America from cattle, sheep, goat, deer, elk, mink, and that’s just the documented species, and TSE prion disease there from, i.e., consider the risk factor there from to the medical, surgical, blood, tissue. I cannot stress enough that all human and animal TSE prion disease, OF ALL AGE GROUPS, be made to be mandatory reportable immediately ASAP. I have said it for 14 years, that the UKBSEnvCJD only theory only helps the spreading of all human and animal TSE. ...
Atypical BSE in Cattle
To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE.
In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.
This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.
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...
Rural and Regional Affairs and Transport References Committee
The possible impacts and consequences for public health, trade and agriculture of the Government's decision to relax import restrictions on beef Final report June 2010
2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49
2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo-has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50
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 ;
[Terry S. Singeltary Sr. has added the following comment:
"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.
Terry S. Singeltary Sr. has added the following comment:
"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.
The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?"
When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.
This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.
The conclusions state that, at present, the only TSE agent demonstrated to be zoonotic is the classical BSE agent. Active screening has allowed the identification of 3 new forms of animal TSEs (H-type atypical BSE, L-type atypical BSE, and atypical scrapie), but the information obtained has major limitations due to the unknown sensitivity of the current monitoring system for these TSEs. There is no epidemiological evidence to suggest that classical scrapie is zoonotic. The epidemiological data are too limited to conclude whether the atypical scrapie agent has a zoonotic potential. Transmission experiments to human PrP transgenic mice or primates suggest that some TSE agents other than the classical BSE agent in cattle (namely L-type atypical BSE, classical BSE in sheep, TME, CWD agents) might have zoonotic potential and indicate that that of the L-type atypical BSE agent appears similar or even higher than that of the classical BSE agent. A single study reported efficient transmission of a natural sheep classical scrapie isolate to primates.
Commentary ---------- Following to a request from the European Commission, the Panel on Biological Hazards (BIOHAZ) and the European Centre for Disease Prevention and Control (ECDC) were asked to deliver a scientific opinion on any possible epidemiological or molecular association between transmissible spongiform encephalopathies (TSEs) in animals and humans. The opinion reviews and discusses the existing scientific evidence that links animal and human TSEs currently known.
The opinion first considers the definition of zoonoses and the principles for the identification of zoonotic diseases, which can be based on evidence gathered from both epidemiological and laboratory studies. The opinion describes the challenges involved in identifying TSEs as zoonoses, due to the specific characteristics of TSE infections/diseases, such as the nature of TSE agents, the occurrence of animal and human TSEs, and the type of monitoring applied, the long incubation period of TSEs etc. The example of the process that led to establishing a link between bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) is reviewed. The epidemiological and laboratory criteria that can be used to investigate such a link are described in detail, since those criteria might be useful for the identification of links between other animal and human TSEs.
The opinion discusses the strain diversity of the TSE agents described in sheep, goats, cattle, cervids, and humans, based on the current knowledge, which highlights that multiple TSE agents exist in each species. The factors influencing the capacity of TSE agents to cross the species transmission barrier are then considered in detail, including the variability in host and donor PrP gene and protein, the TSE strain type involved and its interaction with the host PrP, and the route of infection.
The opinion critically assesses the tools and methodologies currently available to study and evaluate the possible association between animal and human TSEs. The use of epidemiology is discussed for TSEs in both animals and humans, and the possibility to compare the 2 sources of information is presented as a possible method to study the possible links.
Both in vivo and in vitro laboratory methods are considered and discussed, including neuropathology, transmission experiments involving different animal models (wild type and transgenic mice, primates and other species), biochemical methods, cell-free conversion assays, protein misfolding cyclic amplification (PMCA), and cell culture assays. Characteristics, advantages, and disadvantages of the different methods are reviewed, including the opportunity to collate data from different types of experiments for the study of potential associations between animal and human TSEs.
The opinion then reviews the scientific evidence currently available for the different animal and human TSEs, including classical BSE, atypical BSE (H-type and L-type), classical scrapie, atypical scrapie, chronic wasting disease (CWD), transmissible mink encephalopathy (TME), and human TSEs. In particular, the following aspects are systematically discussed for each TSE agent: epidemiology, pathogenesis, and in vivo and in vitro transmission experiments.
The opinion concludes that, at present, the only TSE agent demonstrated to be zoonotic is the classical BSE agent. With regard to human TSEs, detected cases of sporadic CJD are randomly distributed in time and geographical location. These observations have been interpreted as a supportive argument that sporadic CJD is not environmentally acquired. However, the epidemiological evidence in relation to sporadic CJD cannot be regarded as definitive, and the possibility that a small proportion of cases are zoonotic cannot be excluded.
It also concludes that a series of uncertainties in relation to the epidemiological patterns of animal and human TSEs indicate that even a rough comparison of the present epidemiological patterns of human and animal TSEs other than classical BSE is unlikely to be informative. Because of these uncertainties, it is an imperative to continue to carry out systematic surveillance of human TSE diseases, and to continue and improve the surveillance of animal TSE diseases.
The opinion highlights that the active screening has allowed the identification of 3 new forms of animal TSEs (L-type atypical BSE, H-type atypical BSE, and atypical scrapie), but that the information obtained has major limitations due to the unknown sensitivity of the current monitoring system for these TSEs.
There is no epidemiological evidence to suggest that classical scrapie is zoonotic. The epidemiological data are too limited to conclude whether the atypical scrapie agent has a zoonotic potential.
Transmission experiments to human PrP transgenic mice suggest that some TSE agents other than the classical BSE agent in cattle (namely L-type atypical BSE and classical BSE in sheep agents) might have zoonotic potential, whereas for other agents there is no evidence provided of a zoonotic potential (H-type atypical BSE and CWD), or no published studies are available (classical and atypical scrapie). In addition, transmission experiments to primates suggest that some TSE agents other than the classical BSE agent in cattle (namely L-type atypical BSE, classical BSE in sheep, TME, CWD agents) might have zoonotic potential. In particular, primates are highly permissive to L-type atypical BSE, even by the oral route.
The opinion emphasizes that laboratory transmission experiments indicate that the L-type atypical BSE agent has a significant zoonotic potential, which appears similar or even higher than that of the classical BSE agent. While transmission data for evaluating the zoonotic potential of classical scrapie in primates and human PrP transgenic mice are extremely limited or not yet available, a single study reported efficient transmission of a natural sheep classical scrapie isolate to primates.
The opinion concludes that human PrP transgenic mice and primates are currently the most relevant models for investigating the human transmission barrier, but the extent to which such models are informative for measuring the zoonotic potential of an animal TSE under field exposure conditions is unknown. It is unpredictable whether a TSE agent will transmit to a new host, and if the transmission principally occurs, what the transmission rate will be.
Based on the results obtained with in vitro conversion assays, the opinion concludes that there is probably no absolute molecular barrier to transmission of TSE agents between mammalian species. Results also suggest that these assays may be developed as a tool for quantifying the transmission barriers between species for different TSE agent strains; however, there is no means at the moment to transpose in vitro results into the likelihood of in vivo interspecies transmission.
-- Communicated by: Terry S Singeltary Sr
[ProMED-mail thanks Terry S Singeltary Sr for drawing attention to this comprehensive document which provides a current evaluation of experimental work designed to explore the zoonotic potential of the various recently recognised TSEs of domestic and other animals.
It is concluded that at present the only TSE agent demonstrated to be zoonotic is the classical BSE agent. Nor can it be entirely excluded at the present time that a small proportion of cases of sporadic CJD may be environmentally acquired. - Mod.CP]
******
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.
Monday, May 23, 2011
Atypical Prion Diseases in Humans and Animals 2011
Top Curr Chem (2011)
DOI: 10.1007/128_2011_161
# Springer-Verlag Berlin Heidelberg 2011
Michael A. Tranulis, Sylvie L. Benestad, Thierry Baron, and Hans Kretzschmar
Abstract
Although prion diseases, such as Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the "scrapie form" (PrPSc), are used as disease markers, and for taxonomic purposes, in correlation with clinical, pathological, and genetic data. In humans, prion diseases can arise sporadically (sCJD) or genetically (gCJD and others), caused by mutations in the PrP-gene (PRNP), or as a foodborne infection, with the agent of bovine spongiform encephalopathy (BSE) causing variant CJD (vCJD). Person-to-person spread of human prion disease has only been known to occur following cannibalism (kuru disease in Papua New Guinea) or through medical or surgical treatment (iatrogenic CJD, iCJD). In contrast, scrapie in small ruminants and chronic wasting disease (CWD) in cervids behave as infectious diseases within these species. Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents. This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans.
M.A. Tranulis (*)
Norwegian School of Veterinary Science, Oslo, Norway
e-mail: Michael.Tranulis@nvh.no
S.L. Benestad
Norwegian Veterinary Institute, Oslo, Norway
T. Baron
Agence Nationale de Se´curite´ Sanitaire, ANSES, Lyon, France
H. Kretzschmar
Ludwig-Maximilians University of Munich, Munich, Germany
Keywords Animal Atypical Atypical/Nor98 scrapie BSE-H BSE-L Human Prion disease Prion strain Prion type
snip...SEE MORE HERE ;
Sunday, September 25, 2011
Clinical Heidenhain Variant Of Sporadic Creutzfeldt-Jakob Disease (CJD) With Co-occurrence Of Prion Protein Types 1 and 2
Thursday, June 23, 2011
Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits
Thursday, July 21, 2011
A Second Case of Gerstmann-Sträussler-Scheinker Disease Linked to the G131V Mutation in the Prion Protein Gene in a Dutch Patient
Journal of Neuropathology & Experimental Neurology:
August 2011 - Volume 70 - Issue 8 - pp 698-702
Risk.12:
Transmission of Atypical Italian sCJD Case to Humanized Mice Reveals a Novel Infectious Strain
Roberta Galeno,1,† Marco Sbriccoli,1 Loredana Ingrosso,1 Silvia Graziano,1 Angelina Valanzano,1 Anna Poleggi,1 Angela De Pascalis,1 Anna Ladogana,1 Franco Cardone,1 Maria Puopolo,1 Gianluigi Zanusso2 and Maurizio Pocchiari1
1Istituto Superiore di Sanità; Rome, Italy; 2University of Verona; Verona, Italy†Presenting author; Email: roberta.galeno@iss.it
Sporadic Creutzfeldt-Jakob disease (sCJD) is a neurodegenerative prion disorder with uncertain etiology characterized by a typical combination of clinical symptoms, neuropathological lesions, and by the deposition of the pathological protein PrPTSE in the brain.
The vast majority of patients affected by sCJD can be categorized according to the genotype at the polymorphic position www.landesbioscience.com Prion 127
129 of PrP (methionine or valine) and to the molecular mass of PrPTSE (type 1 or 2, corresponding to 21 or 19 kDa), yielding six possible combinations (MM1, MM2, VV1, VV2, MV1, and MV2) that associate with five clinico-pathological variants. Transmission studies of these sCJD subtypes into transgenic mice expressing the human prion protein allowed to identify four different infectious strains, which can partly explain the heterogeneity observed in sCJD patients.1
We recently described a novel molecular and pathological phenotype of sCJD (MV at position 129 of PrP), associated with an unprecedented electrophoretic pattern of PrPTSE characterized by the absence of the highly glycosylated isoform. In this work, we sought to characterize the prion strain associated with this atypical case by intracerebral inoculation into gene-targeted transgenic mice (HuTg) carrying the human PRNP gene with the three 129 genotype combinations. For comparison, three Italian sCJD cases heterozygous at position 129 of the prion protein, belonging to different subtypes (MV1, MV1/2, MV2), were transmitted to the same panel of transgenic mice. Survival times, attack rates, lesion profiles, and molecular analysis of the PrPTSE type recovered from mouse brains injected with the atypical case were compared with data from control animals. Mice inoculated with the atypical case displayed a restricted host tropism, with only a small number of VV animals that resulted PrPTSE-positive after an exceedingly long survival time. Interestingly, PrPTSE accumulated in brains from these mice lacks the diglycosylated band similar to that in sCJD inoculum, yet dissimilar to any other PrPTSE observed in HuTg mice by us and by other authors.1,2 Overall, these results strongly indicate that our atypical case associates with a new infectious strain of sCJD. Further investigations are needed to understand the possible connection with other human and animal prion diseases.
References
1. Bishop MT, Will RG, Manson JC. Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci USA 2010; 107:12005-10.
2. Bishop MT, Hart P, Aitchison L, Baybutt HN, Plinston C, Thomson V, et al. Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lancet Neurol 2006; 5:393-8.
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"
Sunday, June 26, 2011
Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque
Thursday, January 26, 2012
The Risk of Prion Zoonoses
Science 27 January 2012: Vol. 335 no. 6067 pp. 411-413 DOI: 10.1126/science.1218167
http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/risk-of-prion-zoonoses.html
Thursday, January 26, 2012
Facilitated Cross-Species Transmission of Prions in Extraneural Tissue
Science 27 January 2012:
Vol. 335 no. 6067 pp. 472-475
DOI: 10.1126/science.1215659
Sunday, January 22, 2012
Chronic Wasting Disease CWD cervids interspecies transmission
Thursday, February 09, 2012
50 GAME FARMS IN USA INFECTED WITH CHRONIC WASTING DISEASE
SNIP...
Research initiatives
Dr. Michael Coulthart, et al, Public Health Agency of Canada
- although not occurring to date, he estimates that report of just one probable case of human CWD could trigger a public health crisis in North America.
- epidemiological studies so far indicate the probability is very slight, however, prion agents and their transmission properties are highly mutable and adaptable and the possibility can not be ruled out.
- suggests those involved in human prion disease surveillance should consider the possibility of human CWD and develop a readiness to deal with it.
SNIP...SEE FULL TEXT ;
THE CDC DID NOT PUT THIS WARNING OUT FOR THE WELL BEING OF THE DEER AND ELK ;
Thursday, May 26, 2011
Travel History, Hunting, and Venison Consumption Related to Prion Disease Exposure, 2006-2007
FoodNet Population Survey
Journal of the American Dietetic Association Volume 111, Issue 6 , Pages 858-863, June 2011.
http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/travel-history-hunting-and-venison.html
NOR IS THE FDA recalling this CWD positive elk meat for the well being of the dead elk ;
Wednesday, March 18, 2009
Noah's Ark Holding, LLC, Dawson, MN RECALL Elk products contain meat derived from an elk confirmed to have CWD NV, CA, TX, CO, NY, UT, FL, OK RECALLS AND FIELD CORRECTIONS: FOODS CLASS II
http://chronic-wasting-disease.blogspot.com/2009/03/noahs-ark-holding-llc-dawson-mn-recall.html
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease
2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.
snip...
*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,
snip... full text ;
http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html
From: TSS (216-119-163-189.ipset45.wt.net)
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST
From: "Belay, Ermias"
To:
Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Dear Sir/Madam,
In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.
That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.
Ermias Belay, M.D. Centers for Disease Control and Prevention
-----Original Message-----
From:
Sent: Sunday, September 29, 2002 10:15 AM
To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV
Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
Sunday, November 10, 2002 6:26 PM
......snip........end..............TSS
http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html
O.K. THE PRION GODS AT THE CDC SAY NO _STRONG_ EVIDENCE FOR CWD TO HUMANS YET ???
HOWEVER, COLORADO OFFICIALS CLAIM NO CASUAL RELATIONSHIP BETWEEN CWD AND HUMAN HEALTH PROBLEM ???
an oxymoron or what ???
To date, ongoing investigations by state and federal public health officials have shown no causal relationship between CWD and human health problems, for more information see CWD and Potential Transmission to Humans .
http://wildlife.state.co.us/hunting/biggame/cwd/Pages/CWDHome.aspx
Monday, November 14, 2011
WYOMING Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011
http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/wyoming-creutzfeldt-jakob-disease-cwd.html
Wednesday, November 16, 2011
Wisconsin Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011
http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/wisconsin-creutzfeldt-jakob-disease-cwd.html
Sunday, November 13, 2011
COLORADO CWD CJD TSE PRION REPORTING 2011
http://transmissiblespongiformencephalopathy.blogspot.com/2011/11/colorado-cwd-cjd-tse-prion-reporting.html
PLEASE SEE THE LETTER THE British Deer Farmers Association October 1994
CJD9/10022
October 1994
Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ
Dear Mr Elmhirst,
CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT
Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.
The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.
The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.
The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.
I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.
http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
Wednesday, February 1, 2012
CJD and PLASMA / URINE PRODUCTS EMA Position Statements Alberto Ganan Jimenez, European Medicines Agency PDA TSE Safety Forum, 30 June 2011
PIG IN A POKE $$$
Sunday, January 29, 2012
Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety Dr. Detwiler
Dr. Detwiler published Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety Forum\Presentations TSE\ Page 33 and 34 of 44 ;
Wednesday, January 4, 2012
A Bovine Prion Acquires an Epidemic Bovine Spongiform Encephalopathy Strain-Like Phenotype on Interspecies Transmission
http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/bovine-prion-acquires-epidemic-bovine.html
Full Text
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=dignosing+and+reporting+creutzfeldt+jakob+disease&searchid=1048865596978_1528&stored_search=&FIRSTINDEX=0&journalcode=jama
http://jama.ama-assn.org/content/285/6/733.extract
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
Singeltary submission to PLOS ;
No competing interests declared.
see full text ;
http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd
Tuesday, November 08, 2011
Can Mortality Data Provide Reliable Indicators for Creutzfeldt-Jakob Disease Surveillance? A Study in France from 2000 to 2008 Vol. 37, No. 3-4, 2011 Original Paper
Conclusions:These findings raise doubt about the possibility of a reliable CJD surveillance only based on mortality data.
http://creutzfeldt-jakob-disease.blogspot.com/2011/11/can-mortality-data-provide-reliable.html
Saturday, January 2, 2010
Human Prion Diseases in the United States January 1, 2010 ***FINAL***
http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html
14th ICID International Scientific Exchange Brochure -
Final Abstract Number: ISE.114
Session: International Scientific Exchange
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
T. Singeltary
Bacliff, TX, USA
Background:
An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
Methods:
12 years independent research of available data
Results:
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.
Conclusion:
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
http://ww2.isid.org/Downloads/14th_ICID_ISE_Abstracts.pdf
CANADA CJD UPDATE 2011
CJD Deaths Reported by CJDSS1, 1994-20112 As of January 31, 2011
3. Final classification of 49 cases from 2009, 2010, 2011 is pending.
snip...
http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/stats_0111-eng.pdf
USA 2011
USA
National Prion Disease Pathology Surveillance Center
Cases Examined1
(November 1, 2010)
Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD
1996 & earlier 51 33 28 5 0 0
1997 114 68 59 9 0 0
1998 87 51 43 7 1 0
1999 121 73 65 8 0 0
2000 146 103 89 14 0 0
2001 209 119 109 10 0 0
2002 248 149 125 22 2 0
2003 274 176 137 39 0 0
2004 325 186 164 21 0 13
2005 344 194 157 36 1 0
2006 383 197 166 29 0 24
2007 377 214 187 27 0 0
2008 394 231 205 25 0 0
2009 425 258 215 43 0 0
2010 333 213 158 33 0 0
TOTAL 38315 22656 1907 328 4 3
1 Listed based on the year of death or, if not available, on year of referral;
2 Cases with suspected prion disease for which brain tissue and/or blood (in familial cases) were submitted;
3 Disease acquired in the United Kingdom;
4 Disease was acquired in the United Kingdom in one case and in Saudi Arabia in the other case;
5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;
6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.
http://www.cjdsurveillance.com/pdf/case-table.pdf
Please notice where sporadic CJD cases in 1996 went from 28 cases, to 215 cases in 2009, the highest recorded year to date. sporadic CJD is on a steady rise, and has been since 1996.
I also urge you to again notice these disturbing factors in lines 5 and 6 ;
5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;
6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.
========end=====tss=====2011
Monday, August 9, 2010
National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)
(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)
http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html
THE steady rise of sporadic CJD cases in Canada AND USA, with many unusual cases of ''PENDING CLASSIFICATIONS" which have been pending now FOR 3 YEARS. HOW long can this cover-up continue $$$
The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.
http://www.oie.int/boutique/extrait/06heim937950.pdf
SEE FULL TEXT AND MORE HERE ;
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA
http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html
Wednesday, February 1, 2012
Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety Update on CJD and VCJD Transmission RG Will
snip...
Greetings sporadic CJD victims et al in the UK, and around the globe.
Sadly, once again, the UK Prion Gods and nvCJD victims groups in the UK and EU have alienated the sporadic CJD victims and their families, and nothing says it more than RG Will, and his report to the Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety. even though the report title states UPDATE ON CJD AND VCJD TRANSMISSION RG WILL, they play down to low key, with very little data at all on the recent science about sporadic CJD. you would think that with scientist around the globe saying that atypical BSE is now linked with some cases (if not all) of the sporadic CJD cases i.e. 85%+ of all human TSE, you would think that RG Will et al, and the victims group for nvCJD would all put their arms around these victims. BUT NO, instead, they alienate them as a happenstance of bad luck, and refuse to acknowledge updated science, instead going by old and outdated science i.e. the UKBSEnvCJD only theory. I was hoping to finally see RG Will et al finally to speak about sporadic CJD and it’s links as a zoonosis disease, instead, we got nothing. IN this report to the industry pharmaceutical groups at the Prion Disease Risks in the 21st Century 2011 PDA European Virus-TSE Safety by RG Will titled : Update on CJD and VCJD Transmission RG Will, sporadic CJD was mentioned only ONE TIME, with a title to a paper trying once again to alienate sporadic CJD as being tied to blood even though the Baxter study DID tie GSS to blood ;
Transmission of sporadic Creutzfeldt Jakob Disease by blood transfusion: risk factor or possible biases
NO where else was sporadic CJD mentioned in this report. NO WHERE !
Its very sad that not only scientist like RG Will, but nvCJD victims groups in the UK and EU still to this day in 2012, still they all refuse to wrap their arms around these victims to of sporadic CJD. maybe it’s the risk of loosing any compensation, or maybe afraid if they mingle with the 85%+, they would loose out on compensation, even though I know that some refused this blood money, they still refuse to wrap their arms around the sporadic CJD victms, and stand up for them in public.
it’s sad when you can be bought off, or manipulated $$$
some day will come, when people like RG Will and his UKBSEnvCJD only theory will have to eat crow, and I hope I am still alive to see it. ...
Update on CJD and VCJD Transmission
RG Will
National CJD Research and Surveillance Unit
PLEASE REMEMBER ;
The Akron, Ohio-based CJD Foundation said the Center for Disease Control revised that number in October of 2004 to about one in 9,000 CJD cases per year in the population group age 55 and older.
HAVE YOU GOT YOUR CJD QUESTIONNAIRE ASKING REAL QUESTIONS PERTAINING TO ROUTE AND SOURCE OF THE TSE AGENT THAT KILLED YOUR LOVED ONE ???
if not, why not...
Friday, November 30, 2007
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html
http://cjdquestionnaire.blogspot.com/
http://cjdquestionnaire.blogspot.com/
Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis
full text with source references ;
Wednesday, November 30, 2011
First iCJD Death Confirmed in Korea
http://creutzfeldt-jakob-disease.blogspot.com/2011/11/first-icjd-death-confirmed-in-korea.html
Monday, December 12, 2011
Second iatrogenic CJD case confirmed Korea
http://creutzfeldt-jakob-disease.blogspot.com/2011/12/second-iatrogenic-cjd-case-confirmed.html
Second iatrogenic CJD case confirmed Korea
http://creutzfeldt-jakob-disease.blogspot.com/2011/12/second-iatrogenic-cjd-case-confirmed.html
Monday, December 26, 2011
Prion Uptake in the Gut: Identification of the First Uptake and Replication Sites
Thursday, December 22, 2011
Risk of Prion Disease Transmission through Bovine-Derived Bone Substitutes: A Systematic Review
Clin Implant Dent Relat Res. 2011 Dec 15. doi: 10.1111/j.1708-8208.2011.00407.x. [Epub ahead of print]
Saturday, December 3, 2011
Candidate Cell Substrates, Vaccine Production, and Transmissible Spongiform Encephalopathies
Volume 17, Number 12—December 2011
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
http://creutzfeldt-jakob-disease.blogspot.com/2010/01/evidence-for-cjd-tse-transmission-via.html
Sunday, February 5, 2012
February 2012 Update on Feed Enforcement Activities to Limit the Spread of BSE
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/february-2012-update-on-feed.html
LAYPERSON
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
Labels: and on the National Anonymous Tonsil Archive, Creutzfeldt-Jakob disease (CJD) biannual update (2012/1) potential iatrogenic (healthcare-acquired) exposure to CJD
posted by Terry S. Singeltary Sr. at
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