ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
SEAC
ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
The Spongiform Encephalopathy Advisory Committee held its 101st meeting in London on 15th October 2008, and discussed the following:
CURRENT ISSUES SEAC was informed about:
• A mother and son in Spain who had died of variant Creutzfeldt-Jakob Disease (vCJD). This is the first recorded instance of more than one case of vCJD within one family. Both the mother and son lived in a region of Spain with a history of BSE and had frequently shared meals of cattle brain. As no other risk factor has been identified, it seems most likely that both infections were acquired from dietary exposure.
• Results of tests on a single goat from a culled UK dairy herd with a large classical scrapie outbreak. On the basis of the results the presence of Bovine Spongiform Encephalopathy (BSE) cannot be excluded. Further testing by mouse bioassays, which may take at least two, if not more, years to complete, is required to make a definitive diagnosis.
UPDATE ON vCJD PREVALENCE STUDIES
SEAC was updated by the Health Protection Agency (HPA) about the progress of the National Anonymous Tonsil Archive (NATA), a proposed second retrospective survey of 30 000 stored appendix samples and a proposed post mortem tissue archive. These studies would provide data to estimate the prevalence of subclinical vCJD (vCJD infections that have yet to develop, or may never develop, into clinical disease).
Around 62 500 tonsil samples collected by NATA have been tested with no positive samples found. An application for the second retrospective survey of appendix samples is currently under consideration by a Research Ethics Committee. SEAC learned that the establishment of a post mortem tissue archive, which is dependent on the collection of samples from Coroners’ autopsies, does not have the support of Coroners needed to take it forward. SEAC is extremely disappointed about the lack of support from Coroners for the post mortem tissue archive. As SEAC has repeatedly stated, the archive is key to obtaining better estimates of the prevalence of subclinical vCJD. These estimates are vital to make meaningful assessments of the risks to public health from vCJD and of the effectiveness of current, and the need for further, very costly public health protection measures. SEAC acknowledged the strenuous efforts made by the HPA, the Department of Health (DH) and National Health Service Blood and Tissue to devise a system to collect samples that would have the least impact on the work of Coroners. SEAC remains strongly in favour of establishing the archive.
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PROTEASE SENSITIVE PRIONOPATHY
SEAC discussed with Dr Pierluigi Gambetti (US National Prion Disease Pathology Surveillance Center) his recently published report5 on the identification in the United States of America of a new human prion disease. SEAC agreed that there is considerable work to be done to characterise fully this new disease, its cause and whether it is infectious or not. As preliminary unpublished data were also presented, this issue was discussed in a reserved business session in accordance with the SEAC Code of Practice.
RESULTS ON HUMAN SCLERA
SEAC considered preliminary results provided by the HPA and National CJD Surveillance Unit from tests on eye tissue (sclera) from a vCJD case. The results suggest the presence of infectivity and, in contrast with previous testing of samples from other vCJD cases, abnormal prion protein in this tissue. However, as the sclera is very difficult to remove from surrounding eye tissues, which are themselves known to carry vCJD infectivity, the findings may have arisen as a result of contamination at autopsy. Nevertheless, even if the data are reliable, they indicate that there may only be a relatively low level of infectivity present in sclera. As preliminary unpublished data were considered, this issue was discussed in a reserved business session in accordance with the SEAC Code of Practice.
5 Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708. 5 © SEAC 2008
SEE FULL TEXT ;
SEAC 101st meeting on Wednesday 15th October 2008 AGENDA
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
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:
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?
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.
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
USA PRION UNIT BLOG
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)
USA WRITTEN CJD QUESTIONNAIRE ???
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.
Tuesday, August 19, 2008
Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate
Review on the epidemiology and dynamics of BSE epidemics
Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article
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And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
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Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.
And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.
full text 18 pages ;
please see full text ;
***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***
Progress Report from the National Prion Disease Pathology Surveillance Center
An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD
April 3, 2008
Sunday, March 16, 2008
MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE
Wednesday, October 08, 2008
Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008
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Tissue infectivity and strain typing of the many variants 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. ...
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