Friday, August 13, 2010

Creutzfeldt-Jakob disease (CJD) biannual update 13 August 2010 UK Iatrogenic CJD Incidents Report

Emerging Infections/CJD Published on: 13 August 2010

Next update: 12 February 2011

Last updated: 13 August 2010, Volume 4 No 32 (PDF file, 280 KB)

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Emerging Infections Emerging Infections Summary (July 2010)

CJD Creutzfeldt-Jakob disease (CJD) biannual update (2010/2)

Emerging Infections Summary (July 2010)

The Emerging Infections and Zoonoses Section (EIZ) at the HPA Centre for Infections uses an integrated horizon scanning approach which combines information on both human and animal health, in order to identify and assess outbreaks and incidents of new and emerging infectious diseases reported nationally and internationally. A wide variety of sources are scanned and the information gathered is logged daily. This is then used to produce a monthly Emerging Infections Summary that covers those incidents or events which might pose a public health threat to the UK population.

Recent topics have included polio resurgence in Tajikistan, Q fever in the Netherlands, Hantavirus in Germany, and a newly emerging disease of uncertain aetiology in cattle, bovine neonatal pancytopenia, in Europe.

The summary is produced for the Chief Medical Officer's National Expert Panel for New and Emerging Infections and, in addition to panel members, is circulated within the HPA, to the Department of Health, and to animal health colleagues. The summaries are published each month on the HPA website [1].

References 1. Emerging Infections Monthly Summaries, HPA website: Home › Topics › Infectious Diseases › Infections A-Z › Emerging Infections ›Emerging Infections Monthly Summaries.

Creutzfeldt-Jakob disease (CJD) biannual update (2010/2)

This six-monthly report provides an update on reports of incidents of potential iatrogenic (healthcare-acquired) exposure to CJD, and on the National Anonymous Tonsil Archive. The data are correct as of 13 July 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 30 June 2010

A surgical incident occurs when a patient with or at risk of CJD has undergone surgery without the appropriate infection control guidance being followed [3]. This could happen if a patient undergoes surgery during the incubation period of CJD, or because information about CJD risk factors is not available at the time of surgery. If this happens, surgical instruments that may be contaminated with the infectious agent that causes CJD, could pose a transmission risk when they are reused on other patients.

Advice has been issued for the six surgical incidents that were reported to the CJD Incidents Panel in the first six months of 2010 (01/01/2010 to 30/06/2010). Table 1 shows the number of CJD surgical incidents reported to the CJD Incidents Panel from 2000 to 30 June 2010 by the diagnosis of the index patient. As shown in the table, 46% of surgical incidents result from surgery on index cases diagnosed with sporadic CJD.

Information about the CJD Incidents Panel can be found on the HPA website [4].

Table 1: CJD Surgical Incidents (n=417) reported to the CJD Incidents Panel (which have been closed, or where advice has been issued) by diagnosis of index patient: 2000 to 30 June 2010 Incident type 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 First half 2010 Total % of total incidents

Sporadic (possible, probable or definite) 7 19 22 24 16 18 31 17 21 14 2 191 46%

vCJD (possible, probable or definite) 6 14 22 5 4 1 2 – 1 – 56 13%

Familial (including 'at risk' familial) – 2 7 1 3 7 – 2 1 27 6%

'At risk' vCJD blood component recipient – 3 10 6 1 – 20 5%

'At risk' - vCJD plasma product recipient – 1 2 – 10 18 9 8 6 8 1 63 15%

Asymptomatic vCJD infection – 1 – 1 – 2 <1%

At risk' - other – 2 1 2 4 – 1 13 3%

CJD type unclear/ CJD unlikely 1 – 4 1 2 – 10 2%

Not CJD 2 1 4 7 1 – 3 – 26 6% Other – 1 2 1 – 1 7 2% No longer considered 'at-risk' – 1 – 1 – 2 <1%%

Total 16 38 56 50 45 56 63 27 33 27 6 417 100%

If the investigation of a surgical incident identifies any instruments that are considered to be potentially contaminated with the infectious agent, and that could still pose an infection risk to other patients, the Panel advises that these instruments should be removed from general use or refurbished. These instruments may be quarantined, kept for exclusive use on the index patient, refurbished (endoscopes only), destroyed, or sent for research (to the Surgical Instruments Store held by the Health Protection Agency, Porton Down).

Since 2000 there have been 83 incidents in which instruments have been permanently removed from general use or refurbished (endoscopes only). In the first half of 2010, four incidents have resulted in the Panel advising Trusts to remove instruments from general use (either destroying them, keeping them for sole use on the index patient or sending them to the instrument research repository) or to refurbish them (endoscopes only).

Surgical incidents resulting in At Risk patients

The Panel may advise contacting and informing patients of their possible exposure to CJD following a surgical incident. These patients should be considered ‘at-risk of CJD for public health purposes' and are asked to take certain precautions (ie not to donate blood, other tissues or organs, and to inform their medical and dental carers prior to any invasive procedures) in order to reduce the risk of transmitting the CJD agent.

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

The threshold level of risk at which patients are considered to be ‘at risk' of CJD is 1%, in addition to the background risk experienced by the UK population. This risk is based on risk assessment models, using precautionary assumptions. The 1% threshold level is used as a cut off for implementing public health precautions, and is not intended to be a precise measure of an individual patient's risk. A similar threshold is used for identifying other patients who have been exposed to possible CJD risks following surgical, blood, plasma and tissue incidents.

Since 2000 to 30 June 2010, there have been 24 surgical incidents, in which the Panel has advised that 156 patients should be considered to have an increased risk of CJD.

Patient denotifications

Following changes in the assessment of tissue infectivity, the Panel has advised that 38 patients in 14 surgical incidents who were originally considered (and notified) as being at risk of CJD should no longer be considered at risk, and should be denotified. In November 2005, gastrointestinal endoscopies without invasive procedures were reclassified as low risk procedures, and advice was issued to denotify two patients in one surgical incident. In 2006, anterior eye was reclassified as a ‘medium low' risk tissue. This led to a change in advice as only the first patient on whom instruments were used following an anterior eye procedure was to be considered as having an increased risk of CJD. Previously this had applied to the first two patients exposed to such instruments. This resulted in the Panel advising that 16 patients in 7 incidents should be denotified. In 2009, the anterior eye was further reclassified as a low risk tissue. Following this change, the Panel advised that 20 patients should be denotified.

There are eleven remaining surgical incidents in which 118 patients are still considered to be at risk of CJD. Currently, 57 of these at risk patients have been notified that they are at risk of CJD. Local decisions have been taken not to notify 4 patients in these incidents. The Panel is currently advising on two other incidents in which approximately 40 at risk patients are to be advised that they have an increased risk of CJD (these two incidents are not included in Table 2).

Table 2: Surgical At Risk patients still identified as being at risk by the Panel by procedure on the index patient Diagnosis of index patient Procedure on index patient Number of Incidents Patients identified as ‘at increased risk' Patients who died before being notified Local decision not to notify patient Notified patients

Sporadic Brain biopsy 2 28 2 1 25

Variant Appendectomy 1 2 – 2 –

Variant Endoscopy 1 – 1 –

Asymptomatic infected vCJD Endoscopy 1 4 1 – 3

At risk Variant Endoscopy 3 12 4 – 8

At risk Familial Neurosurgery 1 31 10 – 21

Total 9 78 17 4 57

Reports of Blood Incidents 1 January 2010 to 30 June 2010:

The diagnosis in 2009 of a vCJD case, who had previously received blood transfusions, resulted in 10 individuals being identified as alive and at risk of vCJD. Two of these individuals had donated blood to the case, and eight others had received blood components from these two donors. Nine of these patients were notified of their exposure and asked to follow public health advice. A local decision was taken not to notify the 10th recipient.

Six monthly update on the National Anonymous Tonsil Archive: End of June 2010

The National Anonymous Tonsil Archive (NATA) continues to receive approximately 250 tonsil pairs per week (Figure 1). The archive had received a total of 87, 721 tonsil pairs up to the end of June 2010 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 90,721. The number of collection forms that were completed but no tonsil tissue collected was 2,480 (1,625 due to patient objection and 855 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 to Q2 2010)

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 to June 2010)

Figure 3: NHS Trusts and Scottish Hospitals currently collecting and sending tonsil tissue to the archive June 2010


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 20. Available at:

3. Transmissible spongiform encephalopathy agents: safe working and the prevention of infection. The ACDP TSE Working Group.

4. HPA CJD Incidents Panel [online]. London : HPA. Available at:

Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

Thursday, August 12, 2010

Seven main threats for the future linked to prions

Friday, November 30, 2007


Thursday, August 12, 2010

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

Saturday, July 17, 2010

Variant Creutzfeldt-Jakob disease Ironside JW., Haemophilia. 2010 Jul;16 Suppl 5:175-80 REVIEW ARTICLE

Thursday, July 08, 2010

Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions Public release date: 8-Jul-2010

Tuesday, March 16, 2010

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

Thursday, January 28, 2010

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

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],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.

PLEASE SEE AND LISTEN TO VIDEO WAY BACK, toward the bottom of this url ; Monday, July 27, 2009 U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? Jeff Schwann 26 year old cjd victim USA, and please tell me why USA media did not pick this video up and run it on every evening broadcast across the USA $$$


>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<

Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas

Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas. She left 6 Kids and a Husband.The Purpose of this web is to give information in Spanish to the Hispanic community, and to all the community who want's information about this terrible disease.-

Physician Discharge Summary, Parkland Hospital, Dallas Texas

Admit Date: 12/29/2009

Discharge Date: 1/20/2010 Attending Provider: Greenberg, Benjamin Morris;

General Neurology Team: General Neurology Team

Linda was a Hispanic female with no past medical history presents with 14 months of incresing/progressive altered mental status, generalized weakness, inability to walk, loss of appetite, inability to speak, tremor and bowel/blader incontinence.She was, in her usual state of health up until February, 2009, when her husbans notes that she began forgetting things like names and short term memories. He also noticed mild/vague personality changes such as increased aggression. In March, she was involved in a hit and run MVA,although she was not injured. The police tracked her down and ticketed her. At that time, her son deployed to Iraq with the Army and her husband assumed her mentation changes were due to stress over these two events. Also in March, she began to have weakness in her legs, making it difficult to walk. Over the next few months, her mentation and personality changes worsened, getting to a point where she could no longer recognized her children. She was eating less and less. She was losing more weight. In the last 2-3 months, she reached the point where she could not walk without an assist, then 1 month ago, she stopped talking, only making grunting/aggressive sounds when anyone came near her. She also became both bowel and bladder incontinent, having to wear diapers. Her '"tremor'" and body jerks worsened and her hands assumed a sort of permanent grip position, leading her family to put tennis balls in her hands to protect her fingers. The husband says that they have lived in Nebraska for the past 21 years. They had seen a doctor there during the summer time who prescribed her Seroquel and Lexapro, Thinking these were sx of a mood disorder. However, the medications did not help and she continued to deteriorate clinically.

Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. The husband says that he does not know any fellow workers with a similar illness. He also says that she did not have any preceeding illness or travel.

>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<

please see full text ;

Monday, March 29, 2010

Irma Linda Andablo CJD Victim, she died at 38 years old on February 6, 2010 in Mesquite Texas


Archive Number 20100405.1091 Published Date 05-APR-2010 Subject PRO/AH/EDR> Prion disease update 1010 (04)


[Terry S. Singeltary Sr. has added the following comment:

"According to the World Health Organisation, the future public health threat of vCJD in the UK and Europe and potentially the rest of the world is of concern and currently unquantifiable. However, the possibility of a significant and geographically diverse vCJD epidemic occurring over the next few decades cannot be dismissed.

The key word here is diverse. What does diverse mean? If USA scrapie transmitted to USA bovine does not produce pathology as the UK c-BSE, then why would CJD from there look like UK vCJD?",F2400_P1001_PUB_MAIL_ID:1000,82101

Sunday, July 11, 2010

CJD or prion disease 2 CASES McLennan County Texas population 230,213 both cases in their 40s

Tuesday, June 1, 2010

USA cases of dpCJD rising with 24 cases so far in 2010

Friday, February 05, 2010

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

Saturday, January 2, 2010

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

my comments to PLosone here ;

Saturday, June 13, 2009

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

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

Saturday, January 16, 2010

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

Evidence For CJD/TSE Transmission Via Endoscopes

From Terry S. Singletary, Sr 1-24-3

I have researched human/animal TSEs now for over 5 years due to the death of my Mother from the Heidenhain Variant Creutzfeldt Jakob disease, one of six - known - variants of the infamous 'sporadic' CJD.

I did a little survey several years ago about CJD and ENDOSCOPY in 2001, and then went there again when another article was released recently. However, they seemed to only be concerned with the vCJD strain and risk from endoscopy equipment.

My concerns are if vCJD can be transmitted by blood, and there are now 6 variants of the infamous sporadic CJDs that they are documenting to date, how do they know that none of these 6 variants will not transmit the agent (prion) via blood?...especially since the sporadic CJDs are the only ones documented to date to transmit via the surgical arena and now that the CWD is spreading more and more, who knows about the cattle?

I would always read this study and it would bring me back to reality as to how serious/dangerous this agent is in the surgical/medical arena. You might want to read this short abstract from the late, great Dr. Gibbs twice, and let it really sink in. And please remember while reading some of these transmission studies, that most all, if not ALL these agents transmit freely to primates. Humans, of course, are primates.

Regarding claims that:

'Well, it has never been documented to transmit to humans."

There are two critical factors to think about:

A. CJD/TSEs in the USA are NOT reportable in most states and there is NO CJD/TSE questionnaire for most victims and their families, and the one they are now issuing asks absolutely nothing about route and source of the (prion) agent, only how the disease was diagnosed. Furthermore, the elderly are only very rarely autopsied, ie looking for Alzheimer's or 'FAST Alzheimer's' OR prion disease-related factors and phenomena, such as heart failure caused by disease.

B. It is unethical and against the law to do transmission studies of TSEs to humans, they are 100% FATAL.

I suggest you read these case studies about medical arena CJD transmission very carefully:

snip...see full text ;

Saturday, January 16, 2010

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

Evidence For CJD/TSE Transmission Via Endoscopes

From Terry S. Singletary, Sr 1-24-3


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