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

Posted Apr 09 2009 7:13pm
Emerging Infections/CJD Published on: 12 December 2008

Next update: 8 May 2009
Last updated: 12 December 2008,
Volume 2 No 50 (PDF file, xxx KB)

Creutzfeldt-Jakob disease (CJD) update report

This six-monthly report provides an update on reports of incidents of potential iatrogenic (healthcare-acquired) exposure to CJD via surgery, and on the National Anonymous Tonsil Archive. Data are correct as of 5 December 2008.

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: 1 January 2000 to 30 June 2008

There were a total of 350 incidents reported during this period (table 1). Twelve surgical incidents were reported between 1 January and 30 June 2008 (since the previous update report). 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 June 2008 by the diagnosis of the index patient.

Table 1. CJD Surgical Incidents (n=350) reported to the CJD Incidents Panel, by diagnosis of index patient: January 2000 to June 2008

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. In the second half of 2007, there were no 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, 20 incidents have given rise to such advice (table 2). One of these incidents was reported in the first half of 2008. The Panel has so far categorised 64 patients as “at-risk”; 13 of whom died before notification. Three patients have not been notified due to local, clinical decisions.

Table 2 Panel advice to inform patients that they are "at risk" of CJD/vCJD: 1 January 2000 to 31 June 2008

*The index patient was a blood component recipient with evidence of vCJD infection. Information about the CJD Incidents Panel can be found on the HPA website [3]. † For one incident, the total number of "at-risk" patients is still being determined.

National anonymous tonsil archive for studies of detectable abnormal prion protein

The National Anonymous Tonsil Archive (NATA) continues to receive approximately 400 tonsil pairs per week (figure 1). The archive had received a total of 67,696 tonsil pairs up to the end of October 2008 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 70,696. The number of collection forms that were completed but no tonsil tissue collected was 2,188 (1,426 due to patient objection and 762 due to clinical pathology being requested).

Fig 1 Number of tonsil pairs collected for NATA Quarterly: Q1 2004 to Q4 2008

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.

Fig 2. Tonsils collected by Strategic Health Authority, January 2005 to October 2008

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 3: NHS Trusts and Scottish Hospitals currently collecting and sending tonsil tissue to the archive October 2008

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) [4].

References 1. The National Creutzfeldt-Jakob Disease Surveillance Unit, The University of Edinburgh. CJD statistics. CJD figures. Edinburgh: NCJDSU, 3 May 2005. 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 – March 2005.Edinburgh: NCJDSU, 14 April 2005. Available at

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

4. Spongiform Encephalopathy Advisory Committee. Combining evidence from tissue surveys to estimate the prevalence of subclinical vCJD. SEAC, 2008. Available at

Thursday, November 13, 2008
SIXTEENTH ANNUAL REPORT 2007 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK Wednesday, August 20, 2008 Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?

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

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]

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.

sporadic Fatal Familial Insomnia


MARCH 26, 2003

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

disease in the United States

Email Terry S. Singeltary:


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?


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

June 2003

BY Philip Yam


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.

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

2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.

Creutzfeldt Jakob Disease


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 ;

CJD TEXAS (cjd clusters)


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.

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

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