Friday, February 19, 2010

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

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 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.

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

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: http://www.hpa.org.uk/web/HPAweb&Page&HPAwebAutoListName/Page/1204031511121.


5. Spongiform Encephalopathy Advisory Committee. Combining evidence from tissue surveys to estimate the prevalence of subclinical vCJD. London: SEAC, 2008. Available at: http://www.seac.gov.uk/papers/paper100-2.pdf.


Health Protection Report Vol 4 No. 7 - 19 February 2010


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





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 ...


http://bse-atypical.blogspot.com/2010/01/14th-international-congress-on.html




Saturday, January 16, 2010

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


http://creutzfeldt-jakob-disease.blogspot.com/2010/01/evidence-for-cjd-tse-transmission-via.html




Monday, July 20, 2009

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


http://vcjdtransfusion.blogspot.com/2009/07/pre-surgical-risk-assessment-for.html




[2] UK: SEAC position statement on dentistry Date: Sat 30 Jun 2007 Source: Position Statement vCJD and Dentistry, Spongiform Encephalopathy Advisory Committee (SEAC) Update, June 2007 [edited]





Position Statement vCJD and Dentistry ------------------------------------- Issue ----- 1. The Department of Health (DH) asked SEAC to advise on the findings of preliminary research aimed at informing estimates of the risk of variant Creutzfeldt-Jakob Disease (vCJD) transmission via dentistry.

Background ---------- 2. Prions are more resistant than other types of infectious agents to the conventional cleaning and sterilization practices used to decontaminate dental instruments (1). Appreciable quantities of residual material may remain adherent to the surface after normal cleaning and sterilization (2). Therefore, if dental tissues are both infectious and susceptible to infection, the dental instruments are a potential mechanism for the secondary transmission of vCJD. Dentistry could be a particularly significant route of transmission for the population as a whole, due to the large number of routine procedures undertaken and also because dental patients have a normal life expectancy.

This is in contrast with other transmission routes, such as blood transfusion and neurosurgery, where procedures are often carried out in response to some life-threatening condition. Additionally, the ubiquity of dental procedures and the lack of central records on dental procedures means that should such transmission occur, then it would be difficult to detect and control.

3. No cases of vCJD transmission arising from dental procedures have been reported to date (3). Previous DH risk assessments (4,5) have focused on 2 possible mechanisms for the transfer of vCJD infectivity via dental instruments; accidental abrasion of the lingual tonsil and endodontic procedures that involve contact with dental pulp. In considering these assessments, SEAC agreed that the risk of transmission via accidental abrasion of the lingual tonsil appears very low. However, the risk of transmission via endodontic procedures may be higher and give rise to a self sustaining vCJD epidemic under circumstances where (i) dental pulp is infective, (ii) transmission via endodontic instruments is efficient and (iii) a large proportion of vCJD infections remain in a subclinical carrier state (SEAC 91, February 2006). In light of this, SEAC advised that restricting endodontic files and reamers to single use be considered (6). SEAC recommended reassessment of these issues as new data emerge.

New research ------------ 4. Preliminary, unpublished results of research from the Health Protection Agency, aimed at addressing some of the uncertainties in the risk assessments, were reviewed by SEAC (SEAC 97, May 2007). The prion agent used in these studies is closely related to the vCJD agent. This research, using a mouse model, shows that following inoculation of mouse-adapted bovine spongiform encephalopathy (BSE) directly into the gut, infectivity subsequently becomes widespread in tissues of the oral cavity, including dental pulp, salivary glands and gingiva, during the preclinical as well as clinical stage of disease.

5. It is not known how closely the level and distribution of infectivity in the oral cavity of infected mice reflects those of humans infected with vCJD, as there are no comparable data from oral tissues, in particular dental pulp and gingiva, from human subclinical or clinical vCJD cases (7). Although no abnormal prion protein was found in a study of human dental tissues, including dental pulp, salivary glands and gingiva from vCJD cases, the relationship between levels of infectivity and abnormal prion protein is unclear (8). Infectivity studies underway using the mouse model and oral tissues that are presently available from human vCJD cases will provide some comparable data. On the basis of what is currently known, there is no reason to suppose that the mouse is not a good model for humans in respect to the distribution of infectivity in oral tissues. Furthermore, the new data are consistent with published results from experiments using a hamster scrapie model (9).

6. A 2nd set of experiments using the same mouse model showed that non-invasive and transient contact between gingival tissue and fine dental files contaminated with mouse-adapted BSE brain homogenate transmits infection very efficiently. It is not known how efficient gingival transmission would be if dental files were contaminated with infectious oral tissues and then subsequently cleaned and sterilized, a situation which would more closely model human dental practice. Further studies using the mouse model that would be more representative of the human situation, comparing oral tissues with a range of doses of infectivity, cleaned and sterilized files and the kind of tissue contact with instruments that occurs during dentistry, should be considered.

7. SEAC considered that the experiments appear well designed and the conclusions justified and reliable, while recognizing that the research is incomplete and confirmatory experiments have yet to be completed. It is recommended that the research be completed, submitted for peer-review and widely disseminated as soon as possible so others can consider the implications. Nevertheless, these preliminary data increase the possibility that some oral tissues of humans infected with vCJD may potentially become infective during the preclinical stage of the disease. In addition, they increase the possibility that infection could potentially be transmitted not only via accidental abrasion of the lingual tonsil or endodontic procedures but a variety of routine dental procedures.

Implications for transmission risks ----------------------------------- 8. The new findings help refine assumptions made about the level of infectivity of dental pulp and the stage of incubation period when it becomes infective in the risk assessment of vCJD transmission from the reuse of endodontic files and reamers (10). For example, if one patient in 10 000 were to be carrying infection (equivalent to about 6000 people across the UK, the best current estimate (11), the data suggest that in the worst case scenario envisaged in the risk assessment, reuse of endodontic files and reamers might lead to up to 150 new infections per annum. It is not known how many of those infected would go on to develop clinical vCJD. In addition, transmission via the reuse of endodontic files and reamers could be sufficiently efficient to cause a self-sustaining vCJD epidemic arising via this route.

9. These results increase the importance of obtaining reliable estimates of vCJD infection prevalence. Data that will soon be available from the National Anonymous Tonsil Archive may help refine this assessment and provide evidence of the existence and extent of subclinical vCJD infection in tonsillectomy patients. Further data, such as from post mortem tissue or blood donations, will be required to assess prevalence in the general UK population (12).

10. Recent guidance issued by DH to dentists to ensure that endodontic files and reamers are treated as single use (13) is welcomed and should, as long as it is effectively and quickly implemented, prevent transmission and a self-sustaining epidemic arising via this route. However, the extent and monitoring of compliance with this guidance in private and National Health Service dental practice is unclear.

11. The new research also suggests that dental procedures involving contact with other oral tissues, including gingiva, may also be capable of transmitting vCJD. In the absence of a detailed risk assessment examining the potential for transmission via all dental procedures, it is not possible to come to firm conclusions about the implications of these findings for transmission of vCJD. However, given the potential for transmission by this route, serious consideration should be given to assessing the options for reducing transmission risks, such as improving decontamination procedures and practice or the implementation of single use instruments.

12. The size of the potential risk from interactions between the dental and other routes of secondary transmission, such as blood transfusion and hospital surgery, to increase the likelihood of a self-sustaining epidemic is unclear.

13. It is likely to be difficult to distinguish clinical vCJD cases arising from dietary exposure to BSE from secondary transmissions via dental procedures, should they arise, as a large proportion of the population is likely both to have consumed contaminated meat and undergone dentistry.

However, an analysis of dental procedures by patient age may provide an indication of the age group in which infections, if they occur, would be most likely to be observed. Should the incidence of clinical vCJD cases in this age group increase significantly, this may provide an indication that secondary transmission via dentistry is occurring. Investigation of the dental work for these cases may provide supporting data. There is no clear evidence, to date, based on surveillance or investigations of clinical vCJD cases, that any vCJD cases have been caused by dental procedures, but this possibility cannot be excluded.

Conclusions ----------- 14. Preliminary research findings suggest that the potential risk of transmission of vCJD via dental procedures may be greater than previously anticipated. Although this research is incomplete, uses an animal model exposed to relatively high doses of infectivity, and there are no data from infectivity studies on human oral tissues, these findings suggest an increased possibility that vCJD may be relatively efficiently transmitted via a range of dental procedures. Ongoing infectivity studies using human oral tissues and the other studies suggested here will enable more precise assessment of the risks of vCJD transmission through dental procedures.

15. Guidance was issued to dentists earlier this year [2007] recommending that endodontic files and reamers be treated as single use, which, provided this policy is adhered to, will remove any risk of a self-sustaining epidemic arising from reuse of these instruments. To minimize risk, it is critical that appropriate management and audit is in place, both for NHS and private dentistry.

16. It is also critical that a detailed and comprehensive assessment of the risks of all dental procedures be conducted as a matter of urgency. While taking into account the continuing scientific uncertainties, this will allow a more thorough consideration of the possible public health implications of vCJD transmission via dentistry and the identification of possible additional precautionary risk reduction measures. The assessment will require continued updating as more evidence becomes available on the transmissibility of vCJD by dental routes, and on the prevalence of infection within the population. A DH proposal to convene an expert group that includes dental professionals to expedite such an assessment is welcomed. Given the potential for transmission via dentistry, consideration should be given to the urgent assessment of new decontamination technologies which, if proven robust and effective, could significantly reduce transmission risks.

References ---------- (1) Smith et al. (2003) Prions and the oral cavity. J. Dent. Res. 82, 769-775. (2) Smith et al. (2005) Residual protein levels on reprocessed dental instruments. J. Hosp. Infect. 61, 237-241. (3) Everington et al. (2007) Dental treatment and risk of variant CJD - a case control study. Brit. Den. J. 202, 1-3. (4) Department of Health. (2003) Risk assessment for vCJD and dentistry. (5) Department of Health (2006) Dentistry and vCJD: the implications of a carrier-state for a self-sustaining epidemic. Unpublished. (6) SEAC (2006) Position statement on vCJD and endodontic dentistry . (7) Head et al. (2003) Investigation of PrPres in dental tissues in variant CJD. Br. Dent. J. 195, 339-343. (8) SEAC 90 reserved business minutes. (9) Ingrosso et al. (1999) Transmission of the 263K scrapie strain by the dental route. J. Gen. Virol. 80, 3043-3047. (10) Department of Health (2006) Dentistry and vCJD: the implications of a carrier-state for a self-sustaining epidemic. Unpublished. (11) Clarke & Ghani (2005) Projections of future course of the primary vCJD epidemic in the UK: inclusion of subclinical infection and the possibility of wider genetic susceptibility R. J. Soc. Interface. 2, 19-31. (12) SEAC Epidemiology Subgroup (2006) position statement of the vCJD epidemic . (13) DH (2007) Precautionary advice given to dentists on re-use of instruments .

-- Communicated by Terry S. Singletary, Sr.

******


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Monday, December 31, 2007

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


http://creutzfeldt-jakob-disease.blogspot.com/2007_12_01_archive.html





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.



http://seac992007.blogspot.com/2008/06/seac-2008-one-hundredth-meeting-of.html






Friday, July 24, 2009

UW Hospital and Clinics Addresses Creutzfeldt-Jakob Disease Risk

PLEASE SEE ;

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

Hospitals should already be using a questionnaire in Annex J of the ACDP TSE Working Group Infection Control guidance to find out whether any patients who are about to undergo any surgery or endoscopy may be at increased risk of being infected with CJD. If a patient is found to have an increased risk of CJD prior to their surgery or endoscopy then special infection control precautions may need to be taken.

Annex J of the TSE Infection Control guidance has recently been revised, and now advises that patients who are due to have high risk surgery [1] or neuro-endoscopy should be asked an additional question: whether they have received transfusions of blood or blood components from 80 or more donors since 1980. This is because these patients may have an increased risk of being infected with variant CJD (vCJD).

On 16 July 2009 the HPA wrote to the chief executives of NHS trusts asking them to ensure that the guidance is implemented. Detailed information and tools for implementing the guidance can be downloaded from the links below.

If you have any queries about the implementation of the guidance, please contact the HPA Centre for Infections CJD Section at mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000260/!x-usc:mailto:cjd@hpa.org.uk or on 020 8327 6074/6411.

Background information on this new pre-surgical assessment is contained in this Letter to chief executives - July 2009 (PDF, 73 KB) written to all hospitals in England.

The new version of Annex J of the TSE Infection Control Guidance contains new question for patients undergoing high risk surgery and neuro-endoscopy. These questions should be used to assess patients' CJD risk factors.

Clinicians carrying out the new pre-surgical assessment should read Pre-surgical assessment Information for healthcare staff - July 2009 (PDF, 163 KB) This vCJD Algorithm for per-surgical roles - July 2009 (PDF, 28 KB) shows suggested roles and responsibilities for infection control teams, surgical teams and blood transfusion specialists.

Information on patients' transfusion histories should be collected using the Highly transfused vCJD risk assessment form - July 2009 (Word Document, 328 KB) This form is also available as a vCJD risk assessment spreadsheet - July 2009 (Excel Spreadsheet, 2.7 MB). This may help calculate the number of blood donors to a patient. The form may be posted or emailed to the HPA Centre for Infections CJD Section mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000260/!x-usc:mailto:cjd@hpa.org.uk.

Blood transfusion laboratories may wish to use this draft Letter to other blood laboratories - July 2009 (Word Document, 31 KB) when collecting transfusion information from other hospitals.

Pre-surgical assessment teams and patients may wish to read this vCJD Information for presurgical patients - July 2009 (PDF, 29 KB) about this new pre-surgical assessment.

[1] High risk surgery is defined as surgery involving any of the following organs or tissues (high risk tissues): brain, spinal cord, cranial nerves (specifically the entire optic nerve and only the intercranial components of the other cranial nerves), cranial nerve ganglia, posterior eye (specifically the posterior hyaloid face, retina, retinal pigment epithelium, choroid, subretinal fluid, optic nerve) and pituitary gland.

Letter to chief executives - July 2009 (PDF, 73 KB) Added/updated: 16 July 2009


http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469060207




Pre-surgical assessment Information for healthcare staff - July 2009 (PDF, 163 KB) Added/updated: 16 July 2009


http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469061870




vCJD Algorithm for per-surgical roles - July 2009 (PDF, 28 KB) Added/updated: 16 July 2009


http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469062057




Highly transfused vCJD risk assessment form - July 2009 (Word Document, 328 KB) Added/updated: 16 July 2009


http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469062225




Letter to other blood laboratories - July 2009 (Word Document, 31 KB) Added/updated: 16 July 2009


http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469062420




vCJD Information for presurgical patients - July 2009 (PDF, 29 KB) Added/updated: 16 July 2009


http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469062586




vCJD risk assessment spreadsheet - July 2009 (Excel Spreadsheet, 2.7 MB) Added/updated: 16 July 2009


http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247728926790




full text ;



http://www.hpa.org.uk/webw/HPAweb&Page&HPAwebAutoListName/Page/1247469069188




http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247728926790




see also ;

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


http://creutzfeldt-jakob-disease.blogspot.com/2009/07/revision-to-pre-surgical-assessment-of.html




Tuesday, August 12, 2008

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


http://creutzfeldt-jakob-disease.blogspot.com/2008/08/biosafety-in-microbiological-and.html




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


http://creutzfeldt-jakob-disease.blogspot.com/2009/01/medical-procedures-and-risk-for.html




SEE FULL TEXT ;


Friday, July 24, 2009

UW Hospital and Clinics Addresses Creutzfeldt-Jakob Disease Risk


http://creutzfeldt-jakob-disease.blogspot.com/2009/07/uw-hospital-and-clinics-addresses.html



http://creutzfeldt-jakob-disease.blogspot.com/2009/07/uw-hospital-warning-53-patients-about.html




Sunday, January 17, 2010

CJD Following up: Patients never contracted brain disorder UW Hospital patients


http://creutzfeldt-jakob-disease.blogspot.com/2010/01/cjd-following-up-patients-never.html




Friday, February 05, 2010


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


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





CJD


http://creutzfeldt-jakob-disease.blogspot.com/





Sunday, February 14, 2010


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



http://bseusa.blogspot.com/2010/02/docket-no-fsis-2006-0011-fsis-harvard.html




Friday, January 29, 2010


14th International Congress on Infectious Diseases H-type and L-type Atypical BSE January 2010 (special pre-congress edition)


http://bse-atypical.blogspot.com/2010/01/14th-international-congress-on.html





TSS

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Thursday, January 28, 2010

Multiorgan Detection and Characterization of Protease-Resistant Prion Protein in a Case of Variant CJD Examined in the United States

PLoS One. 2010; 5(1): e8765. Published online 2010 January 19. doi: 10.1371/journal.pone.0008765. PMCID: PMC2808239

Copyright Notari et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Multiorgan Detection and Characterization of Protease-Resistant Prion Protein in a Case of Variant CJD Examined in the United States

Silvio Notari,#1 Francisco J. Moleres,#1 Stephen B. Hunter,2 Ermias D. Belay,3 Lawrence B. Schonberger,3 Ignazio Cali,1 Piero Parchi,4 Wun-Ju Shieh,3 Paul Brown,5 Sherif Zaki,3 Wen-Quan Zou,1 and Pierluigi Gambetti1* 1Institute of Pathology, Case Western Reserve University, Cleveland, Ohio, United States of America 2Department of Pathology and Laboratory Medicine, Emory University Hospital, Atlanta, Georgia, United States of America 3National Center for Zoonotic, Vector-Borne, and Enteric Diseases, Center for Disease Control and Prevention, Atlanta, Georgia, United States of America 4Dipartimento di Scienze Neurologiche, Universita' di Bologna, Italy 5CEA/DSV/iMETI/SEPIA, France Delia Goletti, Editor National Institute for Infectious Diseases L. Spallanzani, Italy #Contributed equally. * E-mail: pxg13@case.edu Conceived and designed the experiments: SN FJM PG. Performed the experiments: SN FJM IC. Analyzed the data: SN FJM PB WQZ PG. Contributed reagents/materials/analysis tools: SBH WJS SZ PG. Wrote the paper: SN FJM SBH EDB LBS IC PP WJS PB SZ WQZ PG. Received October 19, 2009; Accepted December 18, 2009.

ABSTRACT

Background Variant Creutzfeldt–Jakob disease (vCJD) is a prion disease thought to be acquired by the consumption of prion-contaminated beef products. To date, over 200 cases have been identified around the world, but mainly in the United Kingdom. Three cases have been identified in the United States; however, these subjects were likely exposed to prion infection elsewhere. Here we report on the first of these subjects.

Methodology/Principal Findings Neuropathological and genetic examinations were carried out using standard procedures. We assessed the presence and characteristics of protease-resistant prion protein (PrPres) in brain and 23 other organs and tissues using immunoblots performed directly on total homogenate or following sodium phosphotungstate precipitation to increase PrPres detectability. The brain showed a lack of typical spongiform degeneration and had large plaques, likely stemming from the extensive neuronal loss caused by the long duration (32 months) of the disease. The PrPres found in the brain had the typical characteristics of the PrPres present in vCJD. In addition to the brain and other organs known to be prion positive in vCJD, such as the lymphoreticular system, pituitary and adrenal glands, and gastrointestinal tract, PrPres was also detected for the first time in the dura mater, liver, pancreas, kidney, ovary, uterus, and skin.

Conclusions/Significance Our results indicate that the number of organs affected in vCJD is greater than previously realized and further underscore the risk of iatrogenic transmission in vCJD.

snip...

The oral route of prion infection in vCJD raised the possibility that tissues and organs, beside the central nervous system (CNS), might also be affected. To date, PrPres has been reported in several tissues and organs outside the CNS of vCJD patients (Table 1) [14–19, P. Brown, unpublished data].

Although the amount of PrPres in non-neural tissues is small compared to that in the brain, the risk posed by the spread of even small amounts of PrPres has been underscored by the iatrogenic transmission of vCJD from blood donors in the preclinical phase of the disease [20].

We examined the main characteristics and tissue distribution of PrPres in a case of vCJD, in which the disease was most likely acquired in the UK but which is officially referred to as an American case because illness onset occurred in the US [21]. In an extensive autopsy examination, sodium phosphotungstate (NaPTA) precipitation, a highly sensitive method of PrPres detection [14,22], was used to establish the presence and estimate the relative amounts of PrPres in several organs and tissues made available to the National Prion Disease Pathology Surveillance Center (NPDPSC).

snip...

Results Clinical History Clinical data on the present patient have been reported in detail [21]. Briefly, the patient lived in Britain until the age of 13 and immigrated to the US in 1992. In early November 2001, at the age of 22 years, the patient was evaluated for depression, emotional instability and memory loss, followed one month later by involuntary movements, gait disturbances and incontinence. During the ensuing three months, the patient’s motor and cognitive deficits worsened, and confusion, hallucination, dysarthria, bradykinesia, and spasticity also occurred. The diagnosis of vCJD was made following brain magnetic resonance imaging and confirmed by immunoblot and immunohistochemistry of tonsil tissue. She received an experimental treatment with quinacrine for 3 months, but showed only minimal and transitory improvement. The patient died in June 2004, 32 months after the clinical onset.

snip...

Discussion Our study confirms the diagnosis of vCJD in the present case, based on the characteristics of the PrPres and the methionine homozygosity at codon 129 of the PrP gene, the last feature being invariably present in vCJD [32]. However, we also observed two unusual features in this case. The first is the long disease duration of 32 months, which is more than twice the 14 month mean duration of the British cases of vCJD [3]. However, cases of up to 40 months duration after the diseases onset have been reported [3,33]. The second unusual feature is the absence of typical spongiform degeneration which likely stemmed from the long duration of the disease. The long disease duration likely led to extensive loss of neurons, in which most of the vacuoles are formed, with ensuing astroglial scar [34].

As previously reported [21], the BSE exposure most likely occurred between the early eighties, when the BSE epidemic emerged in the UK, and 1992, when the patient immigrated to the US. This assumption is consistent with an incubation period of 9 to 21 years, which correlates well with the medium incubation period of 17 years estimated for the UK cases of vCJD [35]. The brain PrPres of the present case displayed the glycoform ratio and electrophoretic mobility characteristic of the PrPres associated with vCJD [5]. One exception is the cerebellum where the monoglycosylated and unglycosylated PrPres isoform migrated slightly faster than the PrPres from other brain regions and resolved in three bands. The variation in PrPres electrophoretic characteristics between the cerebellum and the cerebral cortex is not surprising for it has also been observed in sCJD [36]. Yet to our knowledge it has never been reported in vCJD. Finally, contrary to previous reports [29], PrPres type 1 did not co-occur with type 2. This discrepancy might stem from our rigorous PrP digestion with PK and from the use of different antibodies, an approach that rules out the possibility that partially cleaved fragments derived by the incomplete digestion of PrPSc be misinterpreted as the type 1 fragment [30,37].

The major finding of the present study is the demonstration that PrPres is present in a number of non-CNS tissues and organs which previous studies had reported as free of PrPres (Table 1 and 2) [14– 19, P. Brown, unpublished data]. These tissues include the dura mater, skin, liver, kidney, pancreas, descending colon, uterus and ovary (Table 2 and Fig. 3). The use of NaPTA, along with the long disease duration, may both have contributed to the undisputed detection of PrPres in these organs in this case. The glycoform ratio of the brain PrPres was not retained in every peripheral organ examined (Fig. 4). In the pituitary gland and the skin the diglycosylated and monoglycosylated PrPres isoforms were about equally represented thus the diglycosylated isoform was not dominant. On the other hand, electrophoretic mobility appeared to match that of the brain. Variations in the glycoform ratio could be assessed only on the TH because the glycoform ratio, as well the electrophoretic mobility, is affected by NaPTA enrichment [14].

The presence of prion in the human dura mater is not surprising because sCJD has been transmitted following transplantation of dura obtained from sCJD-affected cases [38]. However, to our knowledge this is the first immunoblot demonstration of PrPres in the dura mater in any prion disease. The detection of relatively large amounts of PrPres in the dura mater raises the possibility of contamination with brain tissue at autopsy. Although this possibility cannot be completely ruled out, extensive rinses in PBS were performed before homogenization in some experiments without observing a reduction in the amount of the PrPres detected.

Prion infectivity of kidney and liver has been demonstrated by bioassay in other human prion diseases [39], and PrPres has been observed in the kidney of scrapie infected sheep [40]. The presence of PrPres has also been reported in kidney, liver and pancreas of scrapie infected mice in association with lymphofollicular proliferation [41]. This last finding is relevant to the present case in which multiple lymphocytic infiltrates with follicular pattern were present in the kidney. However, contrary to this report, we observed no significant inflammatory reaction in any of the other tissues which contained PrPres. A puzzling finding of our study is the presence of PrPres albeit in small amounts in the kidney but not in the urinary bladder. This apparent discrepancy is relevant to the recent demonstrations of prion infectivity in urine

of animals carrying experimental or naturally occurring prion diseases [42–46]. It would indicate that prion infectivity in urine is acquired from the kidney while the urinary bladder acts as a bystander. However the amount of PrPres we observed in the kidney was minimal, and might have not been sufficient to infect the urine and to propagate to the bladder in detectable amounts. Indeed we failed to demonstrate PrPres in the urine in the present case even after hundred-fold urine concentration (data not shown). Obviously more studies are needed to clarify this issue. The present study also demonstrates for the first time the presence of PrPres in the skin in a human prion disease. Previously, PrPres has been detected in the skin from animals with experimental or naturally occurring scrapie [47] as well as in the antler velvet of elk affected by CWD [48].

Furthermore, it is remarkable that we observed PrPres in the uterus and the ovary, a finding which implicates the reproductive system, thereby raising the possibility of maternal transmission of vCJD. Vertical transmissibility of prion infection has been demonstrated in transgenic mice infected with BSE [49]. Related literature on human prion diseases is very scanty. Pregnancy completed to delivery has been reported in sCJD, iatrogenic CJD and vCJD [50,51]; however, transmission to the progeny has not been examined in detail or confirmed in any of these cases. The first detailed determination of PrPC and PrPres in the reproductive and gestational tissues from a sCJD patient has been carried out only recently [51]. Although this study failed to detect PrPres, remarkably it showed that, in uterine tissue obtained at biopsy, most of the PK-sensitive PrP is truncated at the N-terminus and matches the C-terminal PrPC fragment C1 which is generated during normal PrPC metabolism [51]. Similarly, in the present case we observed that the C1-like fragment was largely predominant over the full-length PrPC in the uterus, and it was easily digested by PK but it was present along with a significant amount of characteristic vCJD PrPres (Fig. 4). Since the Nterminus of the PrPres type 2 associated with vCJD is at residues 92–99, the uterine PrPres must have formed from the full length PrPC rather than from C1, the N-terminus of which is at residues 111–112 [31,52]. These findings raise the question of the origin of the PrPres found in the uterus, a question that is currently unanswered. A similar question may be raised for the urine, in

which although the prion infectivity has been demonstrated in animals by bioassay [42–46], the only detected form of PrP under normal condition in animals and humans, is a fragment matching the C1 [53, 54, Notari et al., unpublished data].

All these considerations notwithstanding, the widespread presence of PrPres in visceral organs that we observed in the present case further reinforces the concerns over iatrogenic transmission of vCJD. These concerns are already compelling given the multiple reports of vCJD transmission by blood transfusion.


http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2808239/pdf/pone.0008765.pdf





Wednesday, January 27, 2010


Fast, broad-range disinfection of bacteria, fungi, viruses and prions


http://creutzfeldt-jakob-disease.blogspot.com/2010/01/fast-broad-range-disinfection-of.html




Saturday, January 23, 2010


Experimental Verification of a Traceback Phenomenon in Prion Infection


http://creutzfeldt-jakob-disease.blogspot.com/2010/01/experimental-verification-of-traceback.html




Sunday, January 17, 2010


CJD Following up: Patients never contracted brain disorder UW Hospital patients


http://creutzfeldt-jakob-disease.blogspot.com/2010/01/cjd-following-up-patients-never.html




Sunday, January 17, 2010


Human tissue, recovered from a donor history indicated increased risk factors for Creutzfeldt-Jacob disease Lions Eye Bank


http://creutzfeldt-jakob-disease.blogspot.com/2010/01/human-tissue-recovered-from-donor.html




Saturday, January 16, 2010


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


http://creutzfeldt-jakob-disease.blogspot.com/2010/01/evidence-for-cjd-tse-transmission-via.html




Sunday, August 09, 2009

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


http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html



Tuesday, August 18,

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


http://madcowusda.blogspot.com/2009/08/bse-untold-story-joe-gibbs-and.html



R.I.P. MOM hvCJD confirmed DECEMBER 14, 1997

Monday, December 14, 2009

Similarities between Forms of Sheep Scrapie and Creutzfeldt-Jakob Disease Are Encoded by Distinct Prion Types


http://nor-98.blogspot.com/2009/12/similarities-between-forms-of-sheep.html



Saturday, January 2, 2010

Human Prion Diseases in the United States January 1, 2010 ***FINAL***


http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html


my comments to PLosone here ;


http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd



TSS

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Wednesday, September 23, 2009

Updated clinical diagnostic criteria for sporadic Creutzfeldt Jakob disease

Brain Advance Access published online on September 22, 2009 Brain, doi:10.1093/brain/awp191



The Author(s) 2009. Published by Oxford University Press on behalf of Brain. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/2.5/uk/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Updated clinical diagnostic criteria for sporadic Creutzfeldt Jakob disease


I. Zerr1, K. Kallenberg2, D. M. Summers3, C. Romero4, A. Taratuto4, U. Heinemann1, M. Breithaupt1, D. Varges1, B. Meissner1, A. Ladogana5, M. Schuur6, S. Haik7, S. J. Collins8, Gerard H. Jansen9, G. B. Stokin10, J. Pimentel11, E. Hewer12, D. Collie3, P. Smith13, H. Roberts8, J. P. Brandel7, C. van Duijn6, M. Pocchiari5, C. Begue4, P. Cras14, R. G. Will3 and P. Sanchez-Juan15 1 National TSE Reference Center, Department of Neurology, Georg-August University Goettingen, Goettingen, Germany 2 Department of Neuroradiology, Georg-August University Goettingen, Goettingen, Germany 3 CJD Surveillance Unit, Western General Hospital, Edinburgh, UK 4 Departamento de Neuropatologia, Centro de Referencia de Encefalopatias Espongiformes Transmisibles, Instituto de Investigaciones Neurologicas/FLENI Montañeses 2325 C1428AQK, Buenos Aires, Argentina 5 Istituto Superiore di Sanità, Department of Cell Biology and Neurosciences, Roma, Italy 6 Genetic Epidemiology Unit, Department of Epidemiology, Biostatistics and Clinical Genetics, Erasmus Medical Center, Rotterdam, The Netherlands 7 INSERM UMRS 975, Hôpital de la Salpêtrière Cellule Nationale de référence des maladies de Creutzfeldt-Jakob Paris, F-75013, France 8 Australian National Creutzfeldt-Jakob Disease Registry, Department of Pathology, the University of Melbourne, Parkville, Australia 3010 9 Creutzfeldt-Jakob Disease Surveillance System, Prion Diseases Program, Public Health Agency of Canada, Ottawa, Canada 10 Division of Neurology, University Medical Center and Gerontopsychiatric Unit, University Psychiatric Hospital, SI-1000 Ljubljana, Slovenia 11 Department of Neurology, Laboratory of Neuropathology, Institute of Molecular Medicine, Hospital de Santa Maria, Lisbon Faculty of Medicine, Lisbon, Portugal 12 Institute of Neuropathology, University Hospital Zurich, Zurich, Switzerland 13 Clinic Director, Mercy Private Radiology, Grey st, East Melbourne, 3002, Australia 14 Laboratory of Neurobiology, Department of Neurology, Born Bunge Institute, University of Antwerp, Belgium 15 Fundación ‘Marqués de Valdecilla’ IFIMAV and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED). Santander, Spain

Correspondence to: Corresponding author: Inga Zerr, MD, National TSE Reference Center, Department of Neurology, Georg-August University Goettingen, Robert-Koch-Str. 40, 37075 Goettingen, Germany E-mail: epicjd@med.uni-goettingen.de

Several molecular subtypes of sporadic Creutzfeldt–Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt–Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt–Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt–Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease. Patients with sporadic Creutzfeldt–Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as ‘suspected sporadic Creutzfeldt–Jakob disease’ but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt–Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt–Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic resonance imaging was positive in 83% of cases. In all definite cases, the amended criteria would cover the vast majority of suspected cases, being positive in 98%. Cerebral cortical signal increase and high signal in caudate nucleus and putamen on fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging are useful in the diagnosis of sporadic Creutzfeldt–Jakob disease. We propose an amendment to the clinical diagnostic criteria for sporadic Creutzfeldt–Jakob disease to include findings from magnetic resonance imaging scans.

Key Words: CJD; MRI; FLAIR; DWI; molecular subtypes; dementia

Abbreviations: CSF, cerebrospinal fluid; DWI, diffusion-weight imaging; EEG, electroencephalography; FLAIR, fluid attenuated inversion recovery; IgG, immunglobulin G; MRI, magnetic resonance imaging; sCJD, sporadic Creutzfeldt–Jakob disease; PRNP, prionprotein gene; PrPSc, pathogenic Prion protein; SREAT, steroid responsive encephalitis associated with autoimmune thyroiditis

Received March 26, 2009. Revised June 12, 2009. Accepted June 14, 2009.



http://brain.oxfordjournals.org/cgi/content/abstract/awp191v1?papetoc



Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html


Tuesday, August 11, 2009

Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants

Brian S. Appleby, MD; Kristin K. Appleby, MD; Barbara J. Crain, MD, PhD; Chiadi U. Onyike, MD, MHS; Mitchell T. Wallin, MD, MPH; Peter V. Rabins, MD, MPH

Background: The classic Creutzfeldt-Jakob disease (CJD), Heidenhain, and Oppenheimer-Brownell variants are sporadic CJD (sCJD) phenotypes frequently described in the literature, but many cases present with neuropsychiatric symptoms, suggesting that there may be additional sCJD phenotypes.

Objective: To characterize clinical, diagnostic, and molecular features of 5 sCJD variants.

Design: Retrospective analysis.

Setting: The Johns Hopkins and Veterans Administration health care systems.

Participants: Eighty-eight patients with definite or probable sCJD.

Main Outcome Measures: Differences in age at onset, illness progression, diagnostic test results, and molecular subtype.

Results: The age at onset differed among sCJD variants (P=.03); the affective variant had the youngest mean age at onset (59.7 years). Survival time (P.001) and the time to clinical presentation (P=.003) differed among groups. Patients with the classic CJD phenotype had the shortest median survival time from symptom onset (66 days) and those who met criteria for the affective sCJD variant had the longest (421 days) and presented to clinicians significantly later (median time from onset to presentation, 92 days; P=.004). Cerebrospinal fluid analyses were positive for 14-3-3 protein in all of the affective variants, regardless of illness duration. Periodic sharp-wave complexes were not detected on any of the electroencephalography tracings in the Oppenheimer-Brownell group; basal ganglia hyperintensity was not detected on brain magnetic resonance imaging in this group either. All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.

Conclusions: The classic CJD phenotype and the Heidenhain, Oppenheimer-Brownell, cognitive, and affective sCJD variants differ by age at disease onset, survival time, and diagnostic test results. Characteristics of these 5 phenotypes are provided to facilitate further clinicopathologic investigation that may lead to more reliable and timely diagnoses of sCJD.

Arch Neurol. 2009;66(2):208-215

snip...

COMMENT

snip...see full text ;


http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html



Sunday, September 6, 2009

MAD COW USA 1997 VIDEO

SEE ANOTHER VIDEO THAT SHOWED IN CANADA, BUT NOT USA, ABOUT ANOTHER USA TSE COVER-UP

MORE BRAINS NOT TESTED PROPERLY, key brain parts missing. ...

http://madcowusda.blogspot.com/2009/09/mad-cow-usa-1997-video.html



SEE THIS DAMNING VIDEO AT BOTTOM OF ;

Monday, July 27, 2009

U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ?

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html




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

''nobody has ever ask''
''they dont want our comment''
''they don't want to know, the don't care''
''i have tried repeatedly''
''level of absolute ignorance''
''Entire policy was driven...heard from mr. laycraft, so now, after time has passed, it's ok for Canada, cattle under 30 month, to the USA, THAT'S ALL THAT MATTERED!
PRUSINER ASKED : IF FROM YOUR TESTIMONY, A DEMONSTRATED THREAT TO PUBLIC HEATH ?
''yes, i think prions are bad to eat, and you can die from them''


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





TSS

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Monday, February 02, 2009

Netherlands reports third human death from mad cow disease

Voor derde keer patiënt aan variant Creutzfeldt-Jakob overleden 2 februari 2009 In Nederland is voor de derde keer de ziekte variant Creutzfeldt-Jakob vastgesteld. De betreffende patiënt overleed begin januari. Na onderzoek in het UMC Utrecht en het Nederlandse CJD-expertisecentrum in Rotterdam is bevestigd dat het gaat om variant Creutzfeldt-Jakob. Deze zeldzame ziekte kan worden overgedragen door het eten van met BSE besmette vleesproducten. BSE is ook wel bekend onder de naam gekkekoeienziekte.

naar persbericht informatie over variant Creutzfeldt-Jakob


http://www.rivm.nl/



2009

Voor derde keer patiënt aan variant Creutzfeldt-Jakob overleden 2 februari 2009 In Nederland is voor de derde keer de ziekte variant Creutzfeldt-Jakob (vCJD) vastgesteld. De betreffende patiënt overleed begin januari. Na onderzoek in het UMC Utrecht en het Nederlandse CJD-expertisecentrum in Rotterdam is bevestigd dat het gaat om variant Creutzfeldt-Jakob. Deze zeldzame ziekte kan worden overgedragen door het eten van met BSE besmette vleesproducten. BSE is ook wel bekend onder de naam gekkekoeienziekte.

Op dit moment wordt onderzocht hoe groot de kans is dat de patiënt andere mensen heeft besmet. Het RIVM coördineert dit onderzoek. De gegevens die tot dusver zijn verzameld tonen aan dat deze kans uiterst klein is. De overdracht van mens op mens kan plaatsvinden via besmet bloed of weefsel.

Variant Creutzfeldt-Jakob De variant Creutzfeldt-Jakob is een zeer zeldzame, door prionen veroorzaakte hersenziekte. De overdracht van variant Creutzfeldt-Jakob op mensen vindt in de meeste gevallen plaats door de consumptie van besmette vleesproducten. Het rundvlees in Nederland is nu veilig, omdat sinds 2001 runderen bij de slacht op BSE worden getest. Ook worden sinds 1997 bij de slacht de meest infectieuze organen, de hersenen en het ruggenmerg van de runderen verzameld en vernietigd om overdracht te voorkomen.

Het is de derde keer dat een patiënt aan variant Creutzfeldt-Jakob is overleden in Nederland. In 2005 en 2006 werd bij twee andere patiënten in Nederland variant Creutzfeldt-Jakob vastgesteld. In de gehele wereld zijn tenminste 200 personen bekend die aan de ziekte zijn overleden, waarvan 167 in Engeland. De overige slachtoffers kwamen uit Frankrijk, Ierland, Italië, Canada, Amerika, Japan, Spanje, Saudi-Arabië en Portugal.

EINDE BERICHT

Noot voor de redactie: Informatie over variant Creutzfeldt-Jakob kunt u vinden op de website van het RIVM.

Voor meer informatie kunt u contact opnemen met: - RIVM, Afdeling Communicatie, Marieke Timmer, (030) 274 40 49. - Nederlands CJD-Expertise Centrum Rotterdam, Erasmus MC, afdeling communicatie, David Drexhage, 010 - 703 55 25.

Er worden geen persoonsgegevens verstrekt over de patiënt.



http://www.rivm.nl/persberichten/2009/variant_creutzfeldt_jakob_2009.jsp



02/02/2009

Netherlands reports third human death from mad cow disease

The third person in the Netherlands died of the human variant of mad cow disease, Creutzfeldt-Jakob, in the beginning of January.

THE HAGUE-A third person in four years has died from the human form of mad cow disease in the Netherlands, the national health research agency RIVM said Monday.

"The patient died at the beginning of January," it announced in a press statement. "An investigation has confirmed that the patient died of the variant form of Creutzfeldt-Jakob disease."

The incurable disease is contracted from eating beef infected with BSE, also known as mad cow disease-caused by a rogue protein that proliferates in the brain and turns it spongy.

The RIVM did not say how, when or where the patient contracted the disease, but pointed out that the incubation period, from infection to the display of symptoms, could last decades.

Investigations were ongoing into the "very rare chance" that the patient may have infected other people.

The statement stressed that Dutch beef was safe, explaining that all slaughtered cows are since 2001 tested for BSE.

The RIVM said two other people have died of the disease in the Netherlands, one diagnosed in 2005 and the other in 2006.

"About 200 people have died of the disease in the world, 167 of them in Britain," said the statement.

AFP/Expatica



http://www.expatica.com/nl/news/local_news/Netherlands-reports-third-human-death-from-mad-cow-disease_49203.html



First case of Creutzfeldt-Jakob Disease in the Netherlands The first probable case of variant Creutzfeldt-Jakob (vCJD) disease in a patient in the Netherlands was reported on April 22nd 2005 by the Dutch Ministry of Health, Welfare and Sport. The patient ... Newsitem April 26, 2005 Source: PG

Patient in the Netherlands diagnosed with variant Creutzfeldt-Jakob Disease A patient at the Mesos Medisch Centrum in Utrecht was diagnosed today with variant Creutzfeldt-Jakob Disease (vCJD), the human form of mad cow disease (BSE). Investigations into the contraction of ... Press release April 22, 2005 Source: PG



http://www.expatica.com/nl/news/local_news/Netherlands-reports-third-human-death-from-mad-cow-disease_49203.html



Wednesday, December 10, 2008

Evaluation of FSIS Management Controls Over Pre-Slaughter Activities (Audit Report 24601-7-KC) USDA CERTIFIED DEADSTOCK DOWNER COW SCHOOL LUNCH PROGRAM



http://downercattle.blogspot.com/2008/12/evaluation-of-fsis-management-controls.html



Wednesday, August 20, 2008 Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?



http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html



A New Prionopathy OR more of the same old BSe and sporadic CJD



http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html



Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]



http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html



http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963



There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.



http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm



http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf



sporadic Fatal Familial Insomnia



http://sporadicffi.blogspot.com/



THE PATHOLOGICAL PROTEIN

Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.



http://www.thepathologicalprotein.com/



The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003

doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI

Tracking spongiform encephalopathies in North America

Original TextXavier Bosch



http://www.thelancet.com/journals/laninf/article/PIIS1473-3099(03)00715-1/fulltext



http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/%20fulltext



http://www.ncbi.nlm.nih.gov/pubmed/12906010



http://infection.thelancet.com/journal/journal.isa



JOURNAL OF NEUROLOGY

MARCH 26, 2003

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

disease in the United States

Email Terry S. Singeltary:

mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000046/!x-usc:mailto:flounder@wt.net

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



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



Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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

Terry S. Singeltary, Sr Bacliff, Tex

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



http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT



http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT



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



Creutzfeldt Jakob Disease



http://creutzfeldt-jakob-disease.blogspot.com/



USA PRION UNIT BLOG



http://prionunitusaupdate2008.blogspot.com/



Sunday, April 20, 2008

Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;



http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html



CJD TEXAS (cjd clusters)



http://cjdtexas.blogspot.com/



USA WRITTEN CJD QUESTIONNAIRE ???



http://cjdquestionnaire.blogspot.com/



The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.



http://www.cjdfoundation.org/fact.html



Friday, December 12, 2008

Creutzfeldt-Jakob disease (CJD) update report Emerging Infections/CJD Published on: 12 December 2008



http://creutzfeldt-jakob-disease.blogspot.com/2008/12/creutzfeldt-jakob-disease-cjd-update.html



Friday, August 29, 2008

CREEKSTONE VS USDA COURT OF APPEALS, BUSH SAYS, NO WAY, NO HOW



http://madcowtesting.blogspot.com/2008/08/creekstone-vs-usda-court-of-appeals.html



Sunday, March 16, 2008

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE



http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html



HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008

snip...

Tissue infectivity and strain typing of the many variants Manuscript of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...

snip...



http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html



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

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

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

FINAL AUTOPSY DIAGNOSIS

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



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



TSS

Monday, February 02, 2009 Netherlands reports third human death from mad cow disease

http://creutzfeldt-jakob-disease.blogspot.com/2009/02/netherlands-reports-third-human-death.html

---------- https://lists.aegee.org/cjd-l.html ----------

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Monday, September 01, 2008

Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008

Eurosurveillance, Volume 13, Issue 15, 10 April 2008 Rapid communications Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008 J de Pedro Cuesta ()1 Instituto de Salud Carlos III, Department of Applied Epidemiology, National Centre of Epidemiology, Madrid, Spain

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

Citation style for this article: de Pedro Cuesta J. Two cases of variant Creutzfeldt-Jakob disease reported in Spain in 2007 and 2008. Euro Surveill. 2008;13(15):pii=18831. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=18831

Date of submission: 09 April 2008

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In 2005, the first case of variant Creutzfeldt-Jakob disease (vCJD) was reported in Spain, in a woman born in 1978 with clinical onset of symptoms in 2004 [1]. She subsequently died in 2005.

Recently, two more laboratory-confirmed vCJD cases were reported to the Spanish CJD state registry. In February 2006, a woman born in 1957 developed progressive cognitive deterioration, and died in December 2007 with suspected sporadic CJD (typical EEG in October 2007) MM at codon 129 and no mutations in PRPN gene. A man born in 1967 had onset in May 2007 with psychiatric symptoms, and after several months developed progressive cognitive decline with dementia, typical MRI, MM at codon 129, no mutations in PRPN gene. He died in February 2008. Post-mortem, neuropathology with histochemistry confirmed vCJD in both cases. No clear specific dietary habits, blood donations or reception were recorded. Neither case appears to have visited the United Kingdom before 2004.

The latest two cases were resident in the same region of the country, Castilla y Leon, but no link between them was established.

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

References

Centro Nacional de Epidemiología, Instituto de Salud Carlos III. First case of vCJD reported in Spain. Euro Surveill. 2005;10(8):E050804.1. Available from: http://www.eurosurveillance.org/ew/2005/050804.asp#1


http://www.eurosurveillance.org/images/dynamic/EE/V13N15/art18831.pdf


Scrapie

http://www.mapa.es/en/ganaderia/pags/sanidad_ganadera/caprino/caprino.htm


Programa de vigilancia, control y erradicación de las EET1 Año 2007 EET en pequeños rumiantes 1 Encefalopatía

http://www.mapa.es/ganaderia/pags/sanidad_ganadera/caprino/Programa%20EET%20Pequeños%20rumiantes%202007%20comisión.pdf


BSE in Spain

2008/01 Castilla-Leon Zamora Tapioles January - 08 20-01-98

2008/10 Castilla-Leon Leon Villaquejida June-08 05-07-02

2008/12 Castilla-Leon Leon Cistierna July-08 26-11-98

http://www.eeb.es/pags/espana.htm


Spain Awaits Lab Result to Confirm Fourth Mad Cow Disease Case

Spanish health authorities said they were looking into the possibility that a woman who died last week at a hospital in Leon suffered from the human variant of mad cow disease.

If laboratory tests confirm the 64-year-old had Creutzfeldt-Jakob Disease (CJD), the human variant of bovine spongiform encephalopathy (BSE) or mad cow disease, it will be the fourth death from the disease in Spain.

"We are currently carrying out tests at a laboratory in Alcorcon to determine if the disease is Creutzfeldt-Jakob or not," a health ministry spokesman told AFP.

Regional newspaper Diario de Leon said the woman was the mother of the last person to die in Spain from the brain-wasting disease, a 41-year-old man who died in February 2008.

The health ministry and regional authorities could not confirm the parental link between the two.

Spain recorded its first human death from mad cow disease in June 2005 when a 26-year-old woman succumbed to it in Madrid.

More than 200 people around the world are suspected to have died, most of them in Britain, from the human variant of the disease, which was first described in 1996.

Scientists believe the disease was caused by using infected parts of cattle to make feed for other cattle.

Authorities believe eating meat from infected animals can trigger the human variant of the fatal brain-wasting disease.

The 27-member EU, of which Spain is part, has banned high-risk materials such as spinal cord from use in feed and stricter labelling was also introduced.

Source-AFP SRM

http://www.medindia.net/news/Spain-Awaits-Lab-Result-to-Confirm-Fourth-Mad-Cow-Disease-Case-41110-1.htm


Two people die from vCJD in Spain Two people have died in Spain after contracting the human form of "mad cow disease", variant Creutzfeldt-Jakob Disease (vCJD) that is is thought to be transmitted in infected meat and bone. Since 2000, more than 800 Spanish have suffered from different types of vCJD.

Variant Creutzfeldt-Jakob Disease (vCJD) was first found in humans in 1995 and is thought to be transmitted in infected meat and bone. Since 2000, more than 800 Spanish have suffered from different types of vCJD but the only case of a death in the country was in July 2005.

Recently, however, two people have died in Spain after contracting this human form of "mad cow disease". The woman, 50 years old, died in December 2007 while the man, 41 years old, died in February 2008.

Elena Espinosa, from the Spanish Ministry of Health, has asked the consumers and the farmer sector to "be calmed". Javier Castrodeza, Public Health General Director of the Regional Government of Castilla Leon, where the two people died, declared that this facts don’t have any "relevant health consequences" and "don’t involve a public health problem" at all.

For further information

Creutzfeldt-Jakob Disease (vCJD) Spanish Ministry of Health. Regional Government of Castilla Leon El Pais. "Dos muertes por vacas locas"(Spanish). El Mundo. "Los dos casos de ’vacas locas’ traen a la memoria la crisis ganadera de 2001"(Spanish)

EPHA related articles

The EU’s activities in health at a glance **Updated** Romania and Bulgaria join the EU in January 2007 MEPs call for stronger sanctions on food safety WTO Agreements and public health - briefing of a joint study

Last modified on May 6 2008.

http://www.epha.org/a/2983


Eurosurveillance, Volume 10, Issue 31, 04 August 2005 Articles Centro Nacional de Epidemiología, Instituto de Salud Carlos III1

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

Citation style for this article: Centro Nacional de Epidemiología, Instituto de Salud Carlos III. First case of vCJD reported in Spain. Euro Surveill. 2005;10(31):pii=2764. Available online: http://www.eurosurveillance.org/ViewArticle.aspx?ArticleId=2764 Date of submission: --------------------------------------------------------------------------------

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

First case of vCJD reported in Spain

Centro Nacional de Epidemiología, Instituto de Salud Carlos III, Madrid, Spain (http://cne.isciii.es/)

The Spanish Ministry of Health has reported the detection of a case of variant Creutzfeldt-Jakob disease (vCJD) in Spain [1]. The patient was a 26 year old woman in Madrid who died on 10 July 2005. She had no specific risk factors for vCJD (no prior blood transfusions or visits to the United Kingdom).

The patient’s symptoms began in November 2004, with rapid progression to dementia, normal MRI and EEG, and 14-3-3 protein found in cerebral spinal fluid. Other neurological manifestations including ataxia, dysarthria, apraxia and myoclonus appeared in early 2005. The first abnormal MRI was observed in April 2005. The patient was methionine homozygous at codon 129 of the prion protein gene (PRPN). There were no identified PRPN mutations or family history of CJD.

The case was notified to the Spanish CJD state registry in May 2005 and initially fitted the criteria for both probable sporadic CJD (sCJD) and possible vCJD; it was registered as probable sCJD. Brain neuropathology results of tests conducted at Fundación Hospital de Alcorcón at Madrid subsequently confirmed vCJD. Materials have been sent to the European reference centre at the United Kingdom’s National CJD Surveillance Unit in Edinburgh for further study, and results are expected later in August.

References: Ministerio de Sanidad y Consumo. La Comunidad de Madrid comunica al Ministerio de Sanidad la probable detección del primer caso de la variante de la enfermedad de Creutzfeldt-Jakob en España. Press release 29 July 2005. (http://www.msc.es/gabinetePrensa/notaPrensa/desarrolloNotaPrensa.jsp?id=385)

http://www.eurosurveillance.org/ViewArticle.aspx?PublicationType=W&Volume=10&Issue=31&OrderNumber=1


see rise in sporadic CJD cases in Spain ;

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


Sunday, March 16, 2008 MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE March 16, 2008

http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html


Sunday, August 10, 2008

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

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


Thursday, July 10, 2008 A New Prionopathy update July 10, 2008

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


MY COMMENTS, for whatever they are worth ;

A New Prionopathy OR more of the same old BSe and sporadic CJD

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html


Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html


http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963


There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf


JOURNAL OF NEUROLOGY

MARCH 26, 2003

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

disease in the United States

Email Terry S. Singeltary:

flounder@wt.net

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

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


THE PATHOLOGICAL PROTEIN

Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam

CHAPTER 14 LAYING ODDS

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

http://www.thepathologicalprotein.com/


Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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

Terry S. Singeltary, Sr Bacliff, Tex

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

http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT


http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT


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


Creutzfeldt Jakob Disease

http://creutzfeldt-jakob-disease.blogspot.com/


http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html


http://creutzfeldt-jakob-disease.blogspot.com/2008/01/risk-factors-for-sporadic-creutzfeldt.html


http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html


http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html


http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html


http://creutzfeldt-jakob-disease.blogspot.com/2006/11/on-question-of-sporadic-or-atypical.html


USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/


Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html


CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/


USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/


The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html


Tuesday, August 19, 2008

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html


Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?

August 20, 2008

http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html


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

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