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

Posted Feb 11 2011 7:28pm
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 .

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 .

3. HPA. Biannual CJD update (2009/1). Health Protection Report [serial online] 2009; 3(27): Emerging Infections/CJD. Available at .

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 .

PLEASE notice where in 1990 in the UK, the sporadic CJD cases went from 28, to 88 cases of sporadic CJD in 2008, a steady increase.

CJD Figures These figures show the number of suspect cases referred to the CJD surveillance unit in Edinburgh, and the number of deaths of definite and probable cases in the UK, from 1 January 1990 up to 8th February 2011


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.

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.


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)

Tuesday, December 14, 2010

Infection control of CJD, vCJD and other human prion diseases in healthcare and community settings part 4, Annex A1, Annex J, UPDATE DECEMBER 2010

Saturday, June 13, 2009

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

Saturday, January 2, 2010

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

my comments to PLosone here ;

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


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.


12 years independent research of available data


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.


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.

Seven main threats for the future linked to prions

The NeuroPrion network has identified 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


Wednesday, March 31, 2010

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.


please see all seven threats listed in the USA, and more...FULL TEXT ;

Thursday, August 12, 2010

Seven main threats for the future linked to prions

Friday, February 11, 2011

AN EPIDEMIOLOGIC CRITIQUE OF CREUTZFELDT-JAKOB DISEASE Vol. 2, 1980 Paul Brown vs Zohreh Davanipour and Scrapie


Thursday, February 10, 2011

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31

Saturday, January 29, 2011

Atypical L-Type Bovine Spongiform Encephalopathy (L-BSE) Transmission to Cynomolgus Macaques, a Non-Human Primate

Jpn. J. Infect. Dis., 64 (1), 81-84, 2011

Friday, January 21, 2011

Strain-Specific Barriers against Bovine Prions in Hamsters

Tuesday, January 25, 2011

Generation of a new form of human PrPSc in vitro by inter-species transmission from cervids prions

Wednesday, January 19, 2011

EFSA BIOHAZ Scientific Opinion on the revision of the quantitative risk assessment (QRA) of the BSE risk posed by processed animal proteins (PAPs)

EFSA Journal 2011;9(1):1947

Monday, January 17, 2011

MAD COW Update on Feed Enforcement Activities to Limit the Spread of BSE January 13, 2011

January 2011

Thursday, November 18, 2010

Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep

Tuesday, January 18, 2011

Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease

Thursday, December 23, 2010

Molecular Typing of Protease-Resistant Prion Protein in Transmissible Spongiform Encephalopathies of Small Ruminants, France, 2002-2009 Volume 17, Number 1-January 2011

Wednesday, January 19, 2011

EFSA and ECDC review scientific evidence on possible links between TSEs in animals and humans Webnachricht 19 Januar 2011

Friday, January 21, 2011

Strain-Specific Barriers against Bovine Prions in Hamsters

Saturday, December 18, 2010

OIE Global Conference on Wildlife Animal Health and Biodiversity - Preparing for the Future (TSE AND PRIONS) Paris (France), 23-25 February 2011

Monday, November 30, 2009


Tuesday, November 02, 2010

BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only) diagnostic criteria CVL 1992

Friday, February 11, 2011

Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues

Friday, February 04, 2011

NMLB and USDA allow scrapie prion infected mutton to enter food chain on the Navajo Reservation in New Mexico

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

From: Terry S. Singeltary Sr.

To: President.BenShelly

Cc: sroanhorse ; opvp.nelson ; alaughing; georgehardeen; pressoffice

Sent: Thursday, February 03, 2011 12:15 PM

Subject: NMLB and USDA allow scrapie prion infected mutton to enter food chain on the Navajo Reservation in New Mexico

Greetings Honorable People of the Great Navajo Nation, and the Honorable President Ben Shelly,

I send this to you with great concern. ...

Thursday, February 10, 2011

CWD ILLINOIS UPDATE FEBRUARY 2011 Locations of CWD-Positive Deer - Updated 2/07/2011

Thursday, February 10, 2011

Chronic Wasting Disease Found In A White-Tailed Deer In Maryland

Tuesday, January 25, 2011

Minnesota, National Veterinary Services Laboratory in Ames, Iowa, has confirmed CWD case near Pine Island

Tuesday, January 25, 2011

Generation of a new form of human PrPSc in vitro by inter-species transmission from cervids prions

Monday, February 7, 2011

FDA's Currently-Recommended Policies to Reduce the Possible Risk of Transmission of CJD and vCJD by Blood and Blood Products 2011 ??


Friday, November 30, 2007


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