SIXTEENTH ANNUAL REPORT 2007 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK
13/11/2008 10:00
Department of Health (National)
(DH) National CJD Surveillance Unit publishes 16th Annual Report for 2007 and scientific report
The Sixteenth Annual Report of the National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) is published today. The report looks back over the period from May 1990 (when the Unit was set up) to 31 December 2007.
The report outlines the Unit's work in the clinical surveillance of variant (vCJD), sporadic and iatrogenic CJD. The key figures on the incidence of CJD are:
* between 1996 and 31 December 2007, a total of 166 cases of definite or probable vCJD had been identified in the UK;
* only one new probable or definite case of vCJD was reported in 2007;
* there were five deaths from vCJD in 2007, the same as in 2006;
* there were 56 deaths from sporadic CJD in 2007, compared to 65 in 2006. This is comparable to the mortality rates for sporadic CJD in most other countries.
We also welcome the publication of the Unit's scientific report, which provides details of the current, and planned future scientific research being undertaken by staff at the NCJDSU, in the context of the Unit's previous research and its on-going background surveillance.
Both reports are available on the NCJDSU website at http://www.cjd.ed.uk.
Notes for Editors
The NCJDSU is funded by the Department of Health and the Scottish Executive Health Department.
Please contact the Department of Health press office for further information on 020 7210 5221
COI ref 167527P
NATIONAL CJD SURVEILLANCE UNIT PUBLISHES 16TH ANNUAL REPORT FOR 2007 AND SCIENTIFIC REPORT
The Sixteenth Annual Report of the National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) is published today. The report looks back over the period from May 1990 (when the Unit was set up) to 31 December 2007.
The report outlines the Unit's work in the clinical surveillance of variant (vCJD), sporadic and iatrogenic CJD. The key figures on the incidence of CJD are:
" between 1996 and 31 December 2007, a total of 166 cases of definite or probable vCJD had been identified in the UK;
" only one new probable or definite case of vCJD was reported in 2007;
" there were five deaths from vCJD in 2007, the same as in 2006;
" there were 56 deaths from sporadic CJD in 2007, compared to 65 in 2006. This is comparable to the mortality rates for sporadic CJD in most other countries.
We also welcome the publication of the Unit's scientific report, which provides details of the current, and planned future scientific research being undertaken by staff at the NCJDSU, in the context of the Unit's previous research and its on-going background surveillance.
Both reports are available on the NCJDSU website at www.cjd.ed.uk.
Notes for Editors
The NCJDSU is funded by the Department of Health and the Scottish Executive Health Department.
Please contact the Department of Health press office for further information on 020 7210 5221
https://nds.coi.gov.uk/imagelibrary/downloadMedia.asp?MediaDetailsID=257247
SIXTEENTH ANNUAL REPORT 2007 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK
SUMMARY
The national surveillance programme for Creutzfeldt-Jakob disease (CJD) in the UK was initiated in May 1990. In 1999, the National CJD Surveillance Unit (NCJDSU) became a WHO Collaborative Centre for Reference and Research on the surveillance and epidemiology of human transmissible spongiform encephalopathies (TSEs). In September 2001 the National Care Team was formed, which currently comprises two care coordinators and a secretary. It is based within the NCJDSU and was formed in response to concerns regarding the care of CJD patients. For the first time, the Unit has prepared a Scientific Report, which is available on the Unit’s website (www.cjd.ed.ac.uk). The aim of the Scientific Report is to inform interested parties of details of the current and planned future, wide-ranging scientific research being undertaken by staff at the NCJDSU, in the context of the Unit’s previous research and its on-going core background surveillance. The Scientific Report complements this Annual Report, which provides a description of the clinicopathological epidemiology of CJD in the previous 12 months, reflecting the Unit’s core surveillance work. The NCJDSU Business Plan provides financial, structural and organisational information. The information provided in this Sixteenth Annual Report continues to indicate that the number of sporadic cases remains relatively stable (the data for 2007 may still be incomplete). Detailed clinical and epidemiological information has been obtained for the great majority of patients. There has been a lower number of referrals since 2003 but analysis suggests that much, if not all, of the decline is due to changes in the number of referrals who turn out not to be CJD cases. Although the post mortem rate for patients with suspected CJD has declined, in line with general autopsy rates in the UK, it remains high (around 60%). The number of brain specimens examined in the neuropathology laboratory for sporadic CJD declined from 32 in 2006 to 23 in 2007. In 1990-2007 mortality rates from sporadic CJD in England, Wales, Scotland and Northern Ireland were, respectively, 0.90, 1.01, 0.97 and 0.57/million/year. The differences between these rates are not statistically significant (p>0.5). The mortality rates from sporadic CJD in the UK are comparable to those observed in most other European countries and elsewhere in the world, including countries that are free of BSE. The highest and lowest mortality rates from sporadic CJD were observed in the South West (SMR=132) and Northern Ireland (SMR=76) respectively. The variation in the observed mortality rates between the different regions within the UK is not statistically significant (p>0.1). Up to 31 December 2007, there were 163 deaths from definite or probable variant CJD (vCJD) in the UK. Of these, 115 were confirmed by neuropathology. A further 3 probable cases were alive on 31st December 2007. The clinical, neuropathological and epidemiological features of these cases of vCJD are remarkably uniform and consistent with our previous descriptions. Risk factors for the development of vCJD include age, residence in the UK and methionine homozygosity at codon 129 of the prion protein gene - all 146 clinically affected cases of vCJD with available genetic analysis have been methionine homozygotes. The incidence of vCJD is higher in the north of the UK than in the south. Section 1 T Sixteenth Annual Report 2007 4 Analysis of the incidence of vCJD onsets and deaths from January 1994 to December 2007 indicates that a peak has passed. While this is an encouraging finding, the incidence of vCJD may increase again, particularly if different genetic subgroups with longer incubation periods exist. The identification of disease-related PrP in the spleen of a clinically unaffected blood recipient of PRNP-129 MV genotype is not inconsistent with such an hypothesis. This case, along with the report of the prevalence of abnormal PrP in the large study of appendix and tonsil tissues, suggests the possibility of a greater number of preclinical or subclinical cases in the population than might be indicated by the present numbers of confirmed clinical cases. The only statistically significant geographic cluster of vCJD cases in the UK was in Leicestershire. All geographically associated cases of vCJD are considered for investigation according to a protocol which involves the NCJDSU, colleagues at the HPA, HPS and local public health physicians. The activities of the NCJDSU are strengthened by collaboration with other surveillance projects, including the Transfusion Medicine Epidemiology Review and the study of Progressive Intellectual and Neurological Deterioration in Children. The collaboration of our colleagues in these projects is greatly appreciated; the effectiveness of this collaboration allowed the identification in 2003 of a case of vCJD associated with blood transfusion and the identification in 2004 of PrPres in the spleen of a recipient of blood donated by someone incubating vCJD. In 2006 a further two cases of vCJD associated with blood transfusion were identified. The success of the National CJD Surveillance Unit continues to depend on the extraordinary level of cooperation from the neuroscience community and other medical and paramedical staff throughout the UK. Ongoing support is provided by the Infectious Diseases Epidemiology Unit, London School of Hygiene and Tropical Medicine. We are also particularly grateful to the relatives of patients for their collaboration.
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Sporadic CJD
A recent publication describes the analysis of medical risk factors among 431 sCJD cases referred to the unit between 1998 and 2006 compared with 454 population controls. We also investigated possible geographical and temporal links between neurological and gynaecological operations in 857 sCJD cases referred to the unit between1990 and 2006 12. A reported history of ever having undergone surgery was associated with increased risk of sCJD (OR 2.0; 95% CIs 1.3, 2.1; p=0.003). Increased risk was not associated with surgical categories chosen a priori, but was confined to the residual category “other surgery”, covering a wide range of procedures from minor stitching of wounds to major cardiovascular procedures. Within the “other” category the increase in risk appeared most marked for 3 subcategories; skin stitches, nose/throat operations and removal of growths/cysts/moles. No convincing evidence was found of links (same hospital, within 2 years) between cases undergoing neurosurgery or gynaecological surgery. The conclusion of the paper was that it was unlikely that a high proportion of UK sCJD cases 10 National CJD Surveillance Unit 15th Annual Report, 2006. National CJD Surveillance Unit, Edinburgh, 2007. 11 Ward HJT et al. Risk factors for variant Creutzfeldt-Jakob disease: a case-control study. Ann Neurol 2006; 59: 111-120. 12 Ward HJT et al. Risk factors for sporadic Creutzfeldt-Jakob disease. Ann Neurol 2008; 63: 347- 354. Sixteenth Annual Report 2007 27 are the result of transmission during surgery, but we cannot exclude the possibility that such transmission occurs occasionally. To determine whether the increased risk associated with reported surgical history reflects a causal association or recall bias, a study based on accurate surgical histories obtained from medical records is required. As with variant CJD, we are in the process of obtaining medical risk factor data directly from primary care records (see below, section “2007 onwards” for more details).
2007 onwards
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2.5 Transfusion Medicine Epidemiology Review
The Transfusion Medicine Epidemiology Review (TMER) is a collaborative project between the UK NCJDSU and UK Blood Services (UKBS). The main purpose is to investigate whether there is any evidence that CJD or vCJD may have been transmitted via the blood supply. The following report is based on vCJD cases who donated or received blood and does not include data from the ongoing study of sporadic CJD. Methods vCJD cases (definite and probables) are notified to the UKBS by NCJDSU; a search establishes whether any have acted as donors. Donation records are checked and all components traced through hospital records. Details of all identified recipients are forwarded to NCJDSU for subsequent checking. In the reverse procedure, patients with vCJD reported to have received blood transfusions are identified by NCJDSU and notified to UKBS. Details of transfusions are traced through hospital records and relevant blood donors identified. The identity of donors is notified to NCJDSU for subsequent checking. 2 Swerdlow AJ, Higgins CD, Adlard P, Jones ME, Preece MA. Creutzfeldt-Jakob disease in United Kingdom patients treated with human pituitary growth hormone. Neurology 2003; 61: 783-91. 3 Heath CA, Barker RA, Esmonde TFG, Harvey P, Trend P, Head MW, Smith C, Bell JE, Ironside JW, Will RG, Knight RSG. Dura mater-associated Creutzfeldt-Jakob disease: experience from surveillance in the UK. JNNP 2006; 77: 880-2. Sixteenth Annual Report 2007 23 Results Thirty-one vCJD cases were reported to have been blood donors. Four additional cases who were not reported to have been blood donors were found to be registered with UKBTS. One of these cases was found to have been a blood donor while the other three cases were registered as a donor but never made any donations. Twenty-four of the cases have been traced at blood centres, including the four additional cases mentioned above. Components derived from donations made by 18 of these individuals were actually issued to hospitals. It has been established that 66 components were transfused to identified recipients. Four instances of probable transfusion transmitted infection have been identified. The first recipient (Case 1) developed symptoms of vCJD 6½ years after receiving a transfusion of red cells donated 3½ years before the donor (Donor 1) developed symptoms of vCJD4. The second recipient (Case 2) died from a non-neurological disorder 5 years after receiving blood from a donor (Donor 2) who subsequently developed vCJD5; protease-resistant prion protein (PrPres) was detected in the spleen but not in the brain. This is the first recorded case in the UK of autopsy detection of presumed pre- or subclinical vCJD infection. The third recipient (Case 3) developed symptoms of vCJD 7 years, 10 months after receiving a transfusion of red cells donated about 21 months before the donor (Donor 3) developed symptoms of vCJD6. The fourth recipient (Case 4) who also received a transfusion from the same donor as Case 3, developed symptoms of vCJD 8 years, 4 months after receiving a transfusion of red cells donated about 17 months before this donor (Donor 3) developed symptoms of vCJD7. In the reverse study, 14 vCJD cases were reported to have received blood transfusions in the past. A further case received a blood transfusion after onset of illness and is excluded from further discussion. Checks revealed that of these 14 cases, one was not transfused, 4 had transfusions which pre-dated available records (pre-1980), and 9 had records of transfusion which could be traced. These 9 individuals had received 207 donor exposures (with one patient given 103 components), which have been traced to 190 named donors (two of whom had vCJD as described above). No additional links between donors and recipients have been identified by the reverse study.
Conclusion
The identification of 3 cases of vCJD in the small cohort of known recipients of blood from persons incubating vCJD, together with the fact that 2 of the cases were associated with a common blood donor, establishes beyond reasonable doubt that blood transfusion is a transmission route for vCJD.
(Collaborators on this project: Dr P.E. Hewitt, Dr C.A. Llewelyn, Ms M Malfroy). 4 Llewelyn CA, Hewitt PA, Knight RSG, Amar K, Cousens S, Mackenzie J, Will RG. Possible transmission of variant Creutzfeldt- Jakob disease by blood transfusion. Lancet 2004; 363: 417-421. 5 Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004 364: 527-529. 6 Wroe SJ, Pal S, Siddique D, Hyare H, Macfarlane R, Joiner S, Linehan JM, Brandner S, Wadsworth JD, Hewitt P, Collinge J. Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet 2006; 368: 2061-2067. 7 Health Protection Agency. Fourth case of transfusion-associated variant-CJD. Health Protection Report 2007;1(3):
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Dentistry
For public health purposes, it is important to assess whether dental treatment is a potential source of iatrogenic transmission of vCJD in the UK. A risk assessment by the Department of Health (2006) concluded that, given the existence of a carrier state, a self- sustaining epidemic of vCJD via dentistry was feasible. In addition, preliminary findings from a mouse model (HPA) has lead SEAC to conclude that the potential risk of transmission of vCJD via a range of dental procedures may be greater than previously anticipated. However, to date there has been no evidence in humans of transmission of CJD by dental treatment. This study aims to investigate dental treatment as a possible risk factor for vCJD by examining the records of cases of vCJD and general population controls. A previous study found no significant associations between dental treatment and vCJD13. However, the study was limited to data reported from relatives, which may be unreliable. Therefore, there is a need to examine dental records of cases (and controls) directly to obtain more accurate information. A pilot study (funded by the Department of Health) has demonstrated the feasibility of collating information from dental records. Dental records will be traced with the assistance of the Dental Practice Boards for vCJD cases (n=161) and general population controls (up to 700) resident in England, Wales and Scotland. Details of dental treatment will be collected onto a standardised data collection form by a dental professional. Partial or unavailable dental histories will be investigated through NHS Dental payment schemes. Data will be examined to determine if two or more cases had dental treatment at the same practice within a similar time-frame. Statistical analysis will also be undertaken to determine if there is evidence of an association between dental treatment and vCJD. We aim to have completed gathering dental records by early 2009 and to have analysed the data by spring/ summer 2009.
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4.4 Molecular Genetics Familial CJD
Ninety-one cases of familial CJD (excluding cases of GSS) have been identified since 1970 by the NCJDSU (these data are incomplete as formal investigation of familial CJD in the UK is undertaken by the National Prion Clinic in London). Of the 91 cases, 82 were resident in England, 7 were resident in Wales and 2 were resident in Northern Ireland. Eighteen cases were still alive as at 31st December 2007. Forty-seven of the cases had insertions in the coding region of the PrP gene, 23 carried the mutation at codon 200 (Glu-Lys), 5 at codon 178 (Asp-Asn, with methionine at codon 129, ie FFI), 2 at codon 178 (MV), 2 at codon 210 (Val-Ile), one at codon D167G and one at codon V163STOP. The remaining 10 Sixteenth Annual Report 2007 33 were identified as familial on the basis of relatives known to have had CJD. The mean age at death was 55 years (range 31-77 years).
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Codon 129 distribution in sporadic CJD
The distribution of codon 129 genotypes in sporadic CJD has been analysed since the inception of the Unit in 1990. The overall distribution of codon 129 genotypes in sporadic CJD is 64% MM, 18% MV, 18% VV (see Table 9). There appears to be evidence (p=0.010) of a change in the codon 129 distribution in sporadic CJD between the periods 1990-1995 and 1996-2007. The explanation for this remains unclear and is being investigated further. It should be noted that not all cases are genotyped (data available on 64%) and, therefore, changes in codon 129 distribution may reflect changes in the way in which cases are selected for analysis. Table 9 Codon 129 genotypes of cases of sporadic CJD in the UK, 1990-2007 Deaths from sporadic CJD MM(%) MV(%) VV(%) Deaths from 1 May 1990 – 31 December 1995 97 (75) 15 (12) 17 (13) Deaths from 1 January 1996 – 31 December 2007 283 (61) 93 (20) 89 (19) Total 380 (64) 108 (18) 106 (18) Genotype distribution for the normal population Pooling data from five studies (39) (50) (11) Codon 129 distribution in vCJD All clinical cases for whom genetic data are available (n=146, 88%) were methionine homozygotes at codon 129 of the PrP gene. The genetic laboratory undertakes genetic analysis on a national and international basis. 4.5 CSF
snip...full text
http://www.cjd.ed.ac.uk/report16.pdf
Tuesday, November 11, 2008
SaBTO Summary of 1st Public Meeting - variant CJD and blood Tuesday 21st October 2008, 2pm-4pm
http://vcjdblood.blogspot.com/2008/11/sabto-summary-of-1st-public-meeting.html
Tuesday, November 11, 2008
Transmission of atypical bovine prions to mice transgenic for human prion protein
DOI: 10.3201/eid1412.080941
http://bse-atypical.blogspot.com/2008/11/transmission-of-atypical-bovine-prions.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
Wednesday, October 08, 2008
Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?
http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html
OIE Recognition of the BSE Status of Members RESOLUTION No. XXI (Adopted by the International Committee of the OIE on 27 May 2008)
snip...SEE FULL TEXT with facts and sources @ ;
http://usdavskorea.blogspot.com/2008/06/oie-recognition-of-bse-status-of.html
http://organicconsumers.org/forum/index.php?showtopic=1566
Friday, April 25, 2008
Substances Prohibited From Use in Animal Food or Feed [Docket No. 2002N-0273] (Formerly Docket No. 02N-0273) RIN 0910-AF46
http://madcowfeed.blogspot.com/2008/04/substances-prohibited-from-use-in.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 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
ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
Oct 23, 2008 at 9:00 AM
http://seac992007.blogspot.com/2008/10/one-hundred-and-first-meeting-of_23.html
http://flounder068.vox.com/library/post/one-hundred-and-first-meeting-of-the-spongiform-encephalopathy-advisory-committee.html
***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)
http://www.pnas.org/cgi/content/abstract/0502296102v1
Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA
http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html
NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 007
http://nor-98.blogspot.com/2008/04/seac-spongiform-encephalopathy-advisory.html
http://nor-98.blogspot.com/
SCRAPIE USA
http://scrapie-usa.blogspot.com/
Sunday, September 07, 2008
CWD LIVE TEST, and the political aspects or fallout of live testing for BSE in cattle in the USA
http://chronic-wasting-disease.blogspot.com/2008/09/cwd-live-test-and-political-aspects-or.html
Saturday, October 18, 2008 WYOMING STAR VALLEY MOOSE TESTS POSITIVE FOR CWD
http://chronic-wasting-disease.blogspot.com/2008/10/wyoming-star-valley-moose-tests.html
http://chronic-wasting-disease.blogspot.com/
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/
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/
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
TSS
Department of Health (National)
(DH) National CJD Surveillance Unit publishes 16th Annual Report for 2007 and scientific report
The Sixteenth Annual Report of the National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) is published today. The report looks back over the period from May 1990 (when the Unit was set up) to 31 December 2007.
The report outlines the Unit's work in the clinical surveillance of variant (vCJD), sporadic and iatrogenic CJD. The key figures on the incidence of CJD are:
* between 1996 and 31 December 2007, a total of 166 cases of definite or probable vCJD had been identified in the UK;
* only one new probable or definite case of vCJD was reported in 2007;
* there were five deaths from vCJD in 2007, the same as in 2006;
* there were 56 deaths from sporadic CJD in 2007, compared to 65 in 2006. This is comparable to the mortality rates for sporadic CJD in most other countries.
We also welcome the publication of the Unit's scientific report, which provides details of the current, and planned future scientific research being undertaken by staff at the NCJDSU, in the context of the Unit's previous research and its on-going background surveillance.
Both reports are available on the NCJDSU website at http://www.cjd.ed.uk.
Notes for Editors
The NCJDSU is funded by the Department of Health and the Scottish Executive Health Department.
Please contact the Department of Health press office for further information on 020 7210 5221
COI ref 167527P
NATIONAL CJD SURVEILLANCE UNIT PUBLISHES 16TH ANNUAL REPORT FOR 2007 AND SCIENTIFIC REPORT
The Sixteenth Annual Report of the National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) is published today. The report looks back over the period from May 1990 (when the Unit was set up) to 31 December 2007.
The report outlines the Unit's work in the clinical surveillance of variant (vCJD), sporadic and iatrogenic CJD. The key figures on the incidence of CJD are:
" between 1996 and 31 December 2007, a total of 166 cases of definite or probable vCJD had been identified in the UK;
" only one new probable or definite case of vCJD was reported in 2007;
" there were five deaths from vCJD in 2007, the same as in 2006;
" there were 56 deaths from sporadic CJD in 2007, compared to 65 in 2006. This is comparable to the mortality rates for sporadic CJD in most other countries.
We also welcome the publication of the Unit's scientific report, which provides details of the current, and planned future scientific research being undertaken by staff at the NCJDSU, in the context of the Unit's previous research and its on-going background surveillance.
Both reports are available on the NCJDSU website at www.cjd.ed.uk.
Notes for Editors
The NCJDSU is funded by the Department of Health and the Scottish Executive Health Department.
Please contact the Department of Health press office for further information on 020 7210 5221
https://nds.coi.gov.uk/imagelibrary/downloadMedia.asp?MediaDetailsID=257247
SIXTEENTH ANNUAL REPORT 2007 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK
SUMMARY
The national surveillance programme for Creutzfeldt-Jakob disease (CJD) in the UK was initiated in May 1990. In 1999, the National CJD Surveillance Unit (NCJDSU) became a WHO Collaborative Centre for Reference and Research on the surveillance and epidemiology of human transmissible spongiform encephalopathies (TSEs). In September 2001 the National Care Team was formed, which currently comprises two care coordinators and a secretary. It is based within the NCJDSU and was formed in response to concerns regarding the care of CJD patients. For the first time, the Unit has prepared a Scientific Report, which is available on the Unit’s website (www.cjd.ed.ac.uk). The aim of the Scientific Report is to inform interested parties of details of the current and planned future, wide-ranging scientific research being undertaken by staff at the NCJDSU, in the context of the Unit’s previous research and its on-going core background surveillance. The Scientific Report complements this Annual Report, which provides a description of the clinicopathological epidemiology of CJD in the previous 12 months, reflecting the Unit’s core surveillance work. The NCJDSU Business Plan provides financial, structural and organisational information. The information provided in this Sixteenth Annual Report continues to indicate that the number of sporadic cases remains relatively stable (the data for 2007 may still be incomplete). Detailed clinical and epidemiological information has been obtained for the great majority of patients. There has been a lower number of referrals since 2003 but analysis suggests that much, if not all, of the decline is due to changes in the number of referrals who turn out not to be CJD cases. Although the post mortem rate for patients with suspected CJD has declined, in line with general autopsy rates in the UK, it remains high (around 60%). The number of brain specimens examined in the neuropathology laboratory for sporadic CJD declined from 32 in 2006 to 23 in 2007. In 1990-2007 mortality rates from sporadic CJD in England, Wales, Scotland and Northern Ireland were, respectively, 0.90, 1.01, 0.97 and 0.57/million/year. The differences between these rates are not statistically significant (p>0.5). The mortality rates from sporadic CJD in the UK are comparable to those observed in most other European countries and elsewhere in the world, including countries that are free of BSE. The highest and lowest mortality rates from sporadic CJD were observed in the South West (SMR=132) and Northern Ireland (SMR=76) respectively. The variation in the observed mortality rates between the different regions within the UK is not statistically significant (p>0.1). Up to 31 December 2007, there were 163 deaths from definite or probable variant CJD (vCJD) in the UK. Of these, 115 were confirmed by neuropathology. A further 3 probable cases were alive on 31st December 2007. The clinical, neuropathological and epidemiological features of these cases of vCJD are remarkably uniform and consistent with our previous descriptions. Risk factors for the development of vCJD include age, residence in the UK and methionine homozygosity at codon 129 of the prion protein gene - all 146 clinically affected cases of vCJD with available genetic analysis have been methionine homozygotes. The incidence of vCJD is higher in the north of the UK than in the south. Section 1 T Sixteenth Annual Report 2007 4 Analysis of the incidence of vCJD onsets and deaths from January 1994 to December 2007 indicates that a peak has passed. While this is an encouraging finding, the incidence of vCJD may increase again, particularly if different genetic subgroups with longer incubation periods exist. The identification of disease-related PrP in the spleen of a clinically unaffected blood recipient of PRNP-129 MV genotype is not inconsistent with such an hypothesis. This case, along with the report of the prevalence of abnormal PrP in the large study of appendix and tonsil tissues, suggests the possibility of a greater number of preclinical or subclinical cases in the population than might be indicated by the present numbers of confirmed clinical cases. The only statistically significant geographic cluster of vCJD cases in the UK was in Leicestershire. All geographically associated cases of vCJD are considered for investigation according to a protocol which involves the NCJDSU, colleagues at the HPA, HPS and local public health physicians. The activities of the NCJDSU are strengthened by collaboration with other surveillance projects, including the Transfusion Medicine Epidemiology Review and the study of Progressive Intellectual and Neurological Deterioration in Children. The collaboration of our colleagues in these projects is greatly appreciated; the effectiveness of this collaboration allowed the identification in 2003 of a case of vCJD associated with blood transfusion and the identification in 2004 of PrPres in the spleen of a recipient of blood donated by someone incubating vCJD. In 2006 a further two cases of vCJD associated with blood transfusion were identified. The success of the National CJD Surveillance Unit continues to depend on the extraordinary level of cooperation from the neuroscience community and other medical and paramedical staff throughout the UK. Ongoing support is provided by the Infectious Diseases Epidemiology Unit, London School of Hygiene and Tropical Medicine. We are also particularly grateful to the relatives of patients for their collaboration.
snip...
Sporadic CJD
A recent publication describes the analysis of medical risk factors among 431 sCJD cases referred to the unit between 1998 and 2006 compared with 454 population controls. We also investigated possible geographical and temporal links between neurological and gynaecological operations in 857 sCJD cases referred to the unit between1990 and 2006 12. A reported history of ever having undergone surgery was associated with increased risk of sCJD (OR 2.0; 95% CIs 1.3, 2.1; p=0.003). Increased risk was not associated with surgical categories chosen a priori, but was confined to the residual category “other surgery”, covering a wide range of procedures from minor stitching of wounds to major cardiovascular procedures. Within the “other” category the increase in risk appeared most marked for 3 subcategories; skin stitches, nose/throat operations and removal of growths/cysts/moles. No convincing evidence was found of links (same hospital, within 2 years) between cases undergoing neurosurgery or gynaecological surgery. The conclusion of the paper was that it was unlikely that a high proportion of UK sCJD cases 10 National CJD Surveillance Unit 15th Annual Report, 2006. National CJD Surveillance Unit, Edinburgh, 2007. 11 Ward HJT et al. Risk factors for variant Creutzfeldt-Jakob disease: a case-control study. Ann Neurol 2006; 59: 111-120. 12 Ward HJT et al. Risk factors for sporadic Creutzfeldt-Jakob disease. Ann Neurol 2008; 63: 347- 354. Sixteenth Annual Report 2007 27 are the result of transmission during surgery, but we cannot exclude the possibility that such transmission occurs occasionally. To determine whether the increased risk associated with reported surgical history reflects a causal association or recall bias, a study based on accurate surgical histories obtained from medical records is required. As with variant CJD, we are in the process of obtaining medical risk factor data directly from primary care records (see below, section “2007 onwards” for more details).
2007 onwards
snip...
2.5 Transfusion Medicine Epidemiology Review
The Transfusion Medicine Epidemiology Review (TMER) is a collaborative project between the UK NCJDSU and UK Blood Services (UKBS). The main purpose is to investigate whether there is any evidence that CJD or vCJD may have been transmitted via the blood supply. The following report is based on vCJD cases who donated or received blood and does not include data from the ongoing study of sporadic CJD. Methods vCJD cases (definite and probables) are notified to the UKBS by NCJDSU; a search establishes whether any have acted as donors. Donation records are checked and all components traced through hospital records. Details of all identified recipients are forwarded to NCJDSU for subsequent checking. In the reverse procedure, patients with vCJD reported to have received blood transfusions are identified by NCJDSU and notified to UKBS. Details of transfusions are traced through hospital records and relevant blood donors identified. The identity of donors is notified to NCJDSU for subsequent checking. 2 Swerdlow AJ, Higgins CD, Adlard P, Jones ME, Preece MA. Creutzfeldt-Jakob disease in United Kingdom patients treated with human pituitary growth hormone. Neurology 2003; 61: 783-91. 3 Heath CA, Barker RA, Esmonde TFG, Harvey P, Trend P, Head MW, Smith C, Bell JE, Ironside JW, Will RG, Knight RSG. Dura mater-associated Creutzfeldt-Jakob disease: experience from surveillance in the UK. JNNP 2006; 77: 880-2. Sixteenth Annual Report 2007 23 Results Thirty-one vCJD cases were reported to have been blood donors. Four additional cases who were not reported to have been blood donors were found to be registered with UKBTS. One of these cases was found to have been a blood donor while the other three cases were registered as a donor but never made any donations. Twenty-four of the cases have been traced at blood centres, including the four additional cases mentioned above. Components derived from donations made by 18 of these individuals were actually issued to hospitals. It has been established that 66 components were transfused to identified recipients. Four instances of probable transfusion transmitted infection have been identified. The first recipient (Case 1) developed symptoms of vCJD 6½ years after receiving a transfusion of red cells donated 3½ years before the donor (Donor 1) developed symptoms of vCJD4. The second recipient (Case 2) died from a non-neurological disorder 5 years after receiving blood from a donor (Donor 2) who subsequently developed vCJD5; protease-resistant prion protein (PrPres) was detected in the spleen but not in the brain. This is the first recorded case in the UK of autopsy detection of presumed pre- or subclinical vCJD infection. The third recipient (Case 3) developed symptoms of vCJD 7 years, 10 months after receiving a transfusion of red cells donated about 21 months before the donor (Donor 3) developed symptoms of vCJD6. The fourth recipient (Case 4) who also received a transfusion from the same donor as Case 3, developed symptoms of vCJD 8 years, 4 months after receiving a transfusion of red cells donated about 17 months before this donor (Donor 3) developed symptoms of vCJD7. In the reverse study, 14 vCJD cases were reported to have received blood transfusions in the past. A further case received a blood transfusion after onset of illness and is excluded from further discussion. Checks revealed that of these 14 cases, one was not transfused, 4 had transfusions which pre-dated available records (pre-1980), and 9 had records of transfusion which could be traced. These 9 individuals had received 207 donor exposures (with one patient given 103 components), which have been traced to 190 named donors (two of whom had vCJD as described above). No additional links between donors and recipients have been identified by the reverse study.
Conclusion
The identification of 3 cases of vCJD in the small cohort of known recipients of blood from persons incubating vCJD, together with the fact that 2 of the cases were associated with a common blood donor, establishes beyond reasonable doubt that blood transfusion is a transmission route for vCJD.
(Collaborators on this project: Dr P.E. Hewitt, Dr C.A. Llewelyn, Ms M Malfroy). 4 Llewelyn CA, Hewitt PA, Knight RSG, Amar K, Cousens S, Mackenzie J, Will RG. Possible transmission of variant Creutzfeldt- Jakob disease by blood transfusion. Lancet 2004; 363: 417-421. 5 Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004 364: 527-529. 6 Wroe SJ, Pal S, Siddique D, Hyare H, Macfarlane R, Joiner S, Linehan JM, Brandner S, Wadsworth JD, Hewitt P, Collinge J. Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt-Jakob disease associated with blood transfusion: a case report. Lancet 2006; 368: 2061-2067. 7 Health Protection Agency. Fourth case of transfusion-associated variant-CJD. Health Protection Report 2007;1(3):
snip...
Dentistry
For public health purposes, it is important to assess whether dental treatment is a potential source of iatrogenic transmission of vCJD in the UK. A risk assessment by the Department of Health (2006) concluded that, given the existence of a carrier state, a self- sustaining epidemic of vCJD via dentistry was feasible. In addition, preliminary findings from a mouse model (HPA) has lead SEAC to conclude that the potential risk of transmission of vCJD via a range of dental procedures may be greater than previously anticipated. However, to date there has been no evidence in humans of transmission of CJD by dental treatment. This study aims to investigate dental treatment as a possible risk factor for vCJD by examining the records of cases of vCJD and general population controls. A previous study found no significant associations between dental treatment and vCJD13. However, the study was limited to data reported from relatives, which may be unreliable. Therefore, there is a need to examine dental records of cases (and controls) directly to obtain more accurate information. A pilot study (funded by the Department of Health) has demonstrated the feasibility of collating information from dental records. Dental records will be traced with the assistance of the Dental Practice Boards for vCJD cases (n=161) and general population controls (up to 700) resident in England, Wales and Scotland. Details of dental treatment will be collected onto a standardised data collection form by a dental professional. Partial or unavailable dental histories will be investigated through NHS Dental payment schemes. Data will be examined to determine if two or more cases had dental treatment at the same practice within a similar time-frame. Statistical analysis will also be undertaken to determine if there is evidence of an association between dental treatment and vCJD. We aim to have completed gathering dental records by early 2009 and to have analysed the data by spring/ summer 2009.
snip...
4.4 Molecular Genetics Familial CJD
Ninety-one cases of familial CJD (excluding cases of GSS) have been identified since 1970 by the NCJDSU (these data are incomplete as formal investigation of familial CJD in the UK is undertaken by the National Prion Clinic in London). Of the 91 cases, 82 were resident in England, 7 were resident in Wales and 2 were resident in Northern Ireland. Eighteen cases were still alive as at 31st December 2007. Forty-seven of the cases had insertions in the coding region of the PrP gene, 23 carried the mutation at codon 200 (Glu-Lys), 5 at codon 178 (Asp-Asn, with methionine at codon 129, ie FFI), 2 at codon 178 (MV), 2 at codon 210 (Val-Ile), one at codon D167G and one at codon V163STOP. The remaining 10 Sixteenth Annual Report 2007 33 were identified as familial on the basis of relatives known to have had CJD. The mean age at death was 55 years (range 31-77 years).
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Codon 129 distribution in sporadic CJD
The distribution of codon 129 genotypes in sporadic CJD has been analysed since the inception of the Unit in 1990. The overall distribution of codon 129 genotypes in sporadic CJD is 64% MM, 18% MV, 18% VV (see Table 9). There appears to be evidence (p=0.010) of a change in the codon 129 distribution in sporadic CJD between the periods 1990-1995 and 1996-2007. The explanation for this remains unclear and is being investigated further. It should be noted that not all cases are genotyped (data available on 64%) and, therefore, changes in codon 129 distribution may reflect changes in the way in which cases are selected for analysis. Table 9 Codon 129 genotypes of cases of sporadic CJD in the UK, 1990-2007 Deaths from sporadic CJD MM(%) MV(%) VV(%) Deaths from 1 May 1990 – 31 December 1995 97 (75) 15 (12) 17 (13) Deaths from 1 January 1996 – 31 December 2007 283 (61) 93 (20) 89 (19) Total 380 (64) 108 (18) 106 (18) Genotype distribution for the normal population Pooling data from five studies (39) (50) (11) Codon 129 distribution in vCJD All clinical cases for whom genetic data are available (n=146, 88%) were methionine homozygotes at codon 129 of the PrP gene. The genetic laboratory undertakes genetic analysis on a national and international basis. 4.5 CSF
snip...full text
http://www.cjd.ed.ac.uk/report16.pdf
Tuesday, November 11, 2008
SaBTO Summary of 1st Public Meeting - variant CJD and blood Tuesday 21st October 2008, 2pm-4pm
http://vcjdblood.blogspot.com/2008/11/sabto-summary-of-1st-public-meeting.html
Tuesday, November 11, 2008
Transmission of atypical bovine prions to mice transgenic for human prion protein
DOI: 10.3201/eid1412.080941
http://bse-atypical.blogspot.com/2008/11/transmission-of-atypical-bovine-prions.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
Wednesday, October 08, 2008
Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?
http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html
OIE Recognition of the BSE Status of Members RESOLUTION No. XXI (Adopted by the International Committee of the OIE on 27 May 2008)
snip...SEE FULL TEXT with facts and sources @ ;
http://usdavskorea.blogspot.com/2008/06/oie-recognition-of-bse-status-of.html
http://organicconsumers.org/forum/index.php?showtopic=1566
Friday, April 25, 2008
Substances Prohibited From Use in Animal Food or Feed [Docket No. 2002N-0273] (Formerly Docket No. 02N-0273) RIN 0910-AF46
http://madcowfeed.blogspot.com/2008/04/substances-prohibited-from-use-in.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 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
ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
Oct 23, 2008 at 9:00 AM
http://seac992007.blogspot.com/2008/10/one-hundred-and-first-meeting-of_23.html
http://flounder068.vox.com/library/post/one-hundred-and-first-meeting-of-the-spongiform-encephalopathy-advisory-committee.html
***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.
Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)
http://www.pnas.org/cgi/content/abstract/0502296102v1
Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA
http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html
NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 007
http://nor-98.blogspot.com/2008/04/seac-spongiform-encephalopathy-advisory.html
http://nor-98.blogspot.com/
SCRAPIE USA
http://scrapie-usa.blogspot.com/
Sunday, September 07, 2008
CWD LIVE TEST, and the political aspects or fallout of live testing for BSE in cattle in the USA
http://chronic-wasting-disease.blogspot.com/2008/09/cwd-live-test-and-political-aspects-or.html
Saturday, October 18, 2008 WYOMING STAR VALLEY MOOSE TESTS POSITIVE FOR CWD
http://chronic-wasting-disease.blogspot.com/2008/10/wyoming-star-valley-moose-tests.html
http://chronic-wasting-disease.blogspot.com/
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/
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/
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
TSS
Labels: ANNUAL REPORT, CJD, PRION, TSE
posted by Terry S. Singeltary Sr. at
10:14 AM
