CJD SURVEILLANCE UK FIFTEENTH ANNUAL REPORT 2006
The national surveillance programme for Creutzfeldt-Jakob disease (CJD) in the UK wasinitiated in May 1990. In 1999, the National CJD Surveillance Unit (NCJDSU) became aWHO Collaborative Centre for Reference and Research on the surveillance and epidemiologyof human transmissible spongiform encephalopathies (TSEs). In September 2001 theNational Care Team was formed, which currently comprises a care coordinator and a secretary. Itis based within the NCJDSU and was formed in response to concerns regarding the care of CJDpatients.
The information provided in this fifteenth report continues to provide evidence of a good level ofcase ascertainment. There has been a lower number of referrals since 2003 but analysis suggeststhat much, if not all, of the decline is due to changes in the number of referrals who turn out notto be CJD cases. The number of sporadic cases remains relatively stable (the data for 2006 maystill be incomplete). Detailed clinical and epidemiological information has been obtained for thegreat majority of patients. Although the post mortem rate for patients with suspected CJD hasdeclined, in line with general autopsy rates in the UK, it remains high (around 60%). The numberof brain specimens examined in the neuropathology laboratory for sporadic CJD declined from 52in 2003 to 32 in 2004 but has remained stable (at 32) in both 2005 and 2006.
In 1990-2006 mortality rates from sporadic CJD in England, Wales, Scotland and NorthernIreland were, respectively, 0.89, 0.95, 0.95 and 0.57/million/year. The differences between theserates are not statistically significant (p>0.6). The mortality rates from sporadic CJD in the UK arecomparable 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 sporadicCJD were observed in the South West (SMR=132) and Northern Ireland (SMR=77). Thevariation in the observed mortality rates between the different regions within the UK is notstatistically significant (p>0.1).
Up to 31 December 2006, there were 158 deaths from definite or probable variant CJD (vCJD) inthe UK. Of these, 112 were confirmed by neuropathology. A further 7 probable cases were aliveon 31st December 2006. The clinical, neuropathological and epidemiological features of thesecases of vCJD are remarkably uniform and consistent with our previous descriptions. Risk factorsfor the development of vCJD include age, residence in the UK and methionine homozygosity atcodon 129 of the prion protein gene - all 145 clinically affected cases of vCJD with availablegenetic analysis have been methionine homozygotes. The incidence of vCJD is higher in thenorth of the UK than in the south. Analysis of the incidence of vCJD onsets and deaths fromJanuary 1994 to December 2006 indicates that a peak has been passed. While this is anencouraging finding, the incidence of vCJD may increase again, particularly if different geneticsubgroups with longer incubation periods exist. The identification of disease-related PrP in thespleen of a clinically unaffected blood recipient of PRNP-129 MV genotype is not inconsistent
Fifteenth Annual Report 2006 4
with such an hypothesis. This case, along with the report of the prevalence of abnormal PrP inthe large study of appendix and tonsil tissues, suggests the possibility of a greater number ofpreclinical or subclinical cases in the population than might be indicated by the present numbers ofconfirmed 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 aprotocol which involves the NCJDSU, colleagues at the HPA, HPS and local public healthphysicians.
The activities of the NCJDSU are strengthened by collaboration with other surveillance projects,including the Transfusion Medicine Epidemiology Review and the study of ProgressiveIntellectual and Neurological Deterioration in Children. The collaboration of our colleagues inthese projects is greatly appreciated; the effectiveness of this collaboration allowed theidentification in 2003 of a case of vCJD associated with blood transfusion and the identification in2004 of PrPres in the spleen of a recipient of blood donated by someone incubating vCJD. In2006 a further two cases of vCJD associated with blood transfusion were identified.The success of the National CJD Surveillance Project continues to depend on the extraordinarylevel of co-operation from the neuroscience community and other medical and paramedical staffthroughout the UK. We are particularly grateful to the relatives of patients for their help with thisstudy.
please see full text 42 pages at link below. ...tss
PRION DISEASE UPDATE 2007 (07)******************************A ProMED-mail post<http://www.promedmail.org>ProMED-mail is a program of theInternational Society for Infectious Diseases<http://www.isid.org>
[Note: With continuing decline of the number of cases of variantCreutzfeldt-Jacob disease (abbreviated previously as vCJD or CJD (newvar.) in ProMED-mail) in the human population, it has been decided tobroaden the scope of the occasional ProMED-mail reports to includeother prion-related diseases. These updates supersede the previousupdate thread. ...
 USA: National Prion Disease Pathology Surveillance CenterDate: June 2007Source: National Prion Disease Pathology Surveillance Center (USA) [edited]<http://www.cjdsurveillance.com/pdf/case-table.pdf>
CJD Cases examined----------------------Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD
1996 / 42 / 32 / 26 / 4 / 0 / 01997 / 115 / 68 / 57 / 9 / 0 / 01998 / 93 / 53 / 45 / 7 / 1 / 01999 / 114 / 69 / 61 / 8 / 0 / 02000 / 151 / 103 / 89 / 14 / 0 / 02001 / 208 / 116 / 106 / 9 / 0 / 02002 / 255 / 143 / 118 / 23 / 2 / 02003 / 272 / 174 / 132 / 41 / 0 / 02004 / 334 / 183 / 157 / 21 / 0 / 1*2005 / 352 / 195 / 152 / 37 / 1 / 02006 / 372 / 186 / 143 / 30 / 0 / 1**2007 / 120 / 68 / 35 / 7 / 0 / 0TOTAL / 2428*** / 1390**** / 1121 / 210 / 4 / 2
*Acquired in UK** Acquired in Saudi Arabia*** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007.**** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36type pending (2 from 2005, 8 from 2006, 26 from 2007).
-- Cases are listed based on the year of death when available. If theyear of death is not available, the year of sample receipt is used.
-- Referrals: Cases with possible or probable prion disease fromwhich brain tissue or blood in the case of familial disease were submitted.
-- Inconclusive: Cases in which the samples were not sufficient tomake a diagnosis.
-- Non-vCJD type unknown are cases in which the tissue submitted wasadequate to establish the presence but not the type; in all cases,vCJD could be excluded.
--Communicated by:Terry S. Singeltary Sr. <firstname.lastname@example.org>
[In submitting these data, Terry S. Singeltary Sr. draws attention tothe steady increase in the "type unknown" category, which, accordingto 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]
************************************************************Become a ProMED-mail Premium Subscriber at<http://www.isid.org/ProMEDMail_Premium.shtml>************************************************************Visit ProMED-mail's web site at <http://www.promedmail.org>.
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotypeof 'UNKNOWN' strain growing. ...
There is a growing number of human CJD cases, and they were presented lastweek in San Francisco by Luigi Gambatti(?) from his CJD surveillancecollection.
He estimates that it may be up to 14 or 15 persons which display selectivelySPRPSC and practically no detected RPRPSC proteins.
PLEASE NOTE IN USA CJD UPDATE AS AT JUNE 2007, please note steady increasein ''TYPE UNKNOWN''. ...TSS
1 Acquired in the United Kingdom; 2 Acquired in Saudi Arabia; 3 Includes 17inconclusive and 9 pending (1 from 2006, 8from 2007); 4 Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1from 2001, 1 from 2003, 4 from 2004, 3from 2005, 4 from 2006) and 36 type pending (2 from 2005, 8 from 2006,
*** 26 from 2007)
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
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  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?
doi:10.1016/S1473-3099(03)00715-1Copyright © 2003 Published by Elsevier Ltd.Newsdesk
Tracking spongiform encephalopathies in North America
Available online 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463
“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost mymom to hvCJD (Heidenhain variant CJD)and have been searching for answers ever since. What I have found is that wehave not been told the truth. CWDin deer and elk is a small portion of a much bigger problem.”............................
see history of cjd questionnaire
Sent: Monday May 28, 2007
Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE
Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and theUKBSEnvCJD only theoryDate: January 29, 2006 at 9:03 am PST
Comments sent via JAMA Feedback Page------------------------------------------------------------NAME: Terry S. Singeltary Sr.E-MAIL: email@example.comIP ADDRESS: xxxxxxxxHOSTNAME: xxxxxxxxPREVIOUS PAGE: http://jama.ama-assn.org/misc/authors.dtlBROWSER: Mozilla/5.0 (Windows; U; Win98; en-US; rv:1.0.2) Gecko/20030208Netscape/7.02PROMOTIONAL USE: (not answered)
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJDonly theory
TSEs have been rampant in the USA for decades in many species, and they allhave been rendered and fed back to animals for human/animal consumption.propose that the current diagnostic criteria for human TSEs only enhancesand helps the spreading of human TSE from the continued belief of theUKBSEnvCJD only theory in 2007. With all the science to date refuting it, tocontinue to validate this myth, will only spread this TSE agent through amultitude of potential routes and sources i.e. consumption, surgical, blood,medical, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey,Deslys, Dormont, Gibbs, Ironside, Manuelidis, Marsh, et al and many more,that the world of TSE Transmissible Spongiform Encephalopathy is far from anexact science, but there is enough provenscience to date that this myth should be put to rest once and for all, andthat we move forward with a new classification for human and animal TSE thatwould properly identify the infected species, the source species, and thenthe route.
This would further have to be broken down to strain of species and then theroute of transmission would further have to be broken down. Accumulation andTransmission are key to the threshold from sub-clinical to clinical disease,and key to all this, is to stop the amplification and transmission of thisagent, the spreading of, no matter what strain. In my opinion, to continuewith this myth that the U.K. strain of BSE (one strain TSE in cows), and thenv/v CJD (one strain TSE humans) and that all the rest of human TSE are justone single strain i.e. sporadic CJD (when to date there are 6 differentphenotypes of sCJD, and growing per Gambetti et al), and that no otheranimal TSE transmits to humans, to continue with this masquerade will onlycontinue to spread, expose, and kill, who knows how many more in the yearsand decades to come. ONE was enough for me, My Mom, hvCJD i.e. HeidenhainVariant CJD, DOD 12/14/97 confirmed, which is nothing more than another mansname added to CJD, like CJD itself, Jakob and Creutzfeldt, orGerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE,named after another human.
WE are only kidding ourselves with the current diagnostic criteria for humanand animal TSE, especially differentiating between the nvCJD vs the sporadicCJDstrains and then the GSS strains and also the FFI fatal familial insomniastrains or the ones that mimics one or the other of those TSE? Tissueinfectivity and strain typing of the many variants of the human and animalTSEs are paramount in all variants of all TSE. There must be a properclassification that will differentiate between all these human TSE in orderto do this. With the CDI and other more sensitive testing coming about, Ionly hope that my proposal will some day be taken seriously. ...
Terry S. Singeltary Sr.P.O. Box 42Bacliff, Texas USA firstname.lastname@example.org
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease inthe United States
i am reminded of a few things deep throat (high ranking official at usda)told me years ago;
The most frightening thing I have read all day is thereport of Gambetti's finding of a new strain ofsporadic cjd in young people.........Dear God,
BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA, A REVIEW OF SORTS
MADCOW USDA the untold story
MADCOW USDA the untold story continued
USA NOR-98 SCRAPIE UPDATE AUGUST 31, 2007 RISES TO 5 DOCUMENTED CASES
Government Accountability Project
Transmissible Mink Encephalopathy TME
TME hyper/drowsy, INTER-SPECIES TRANSMISSION CWD and strainproperties
USA NVCJD BLOOD RECALLS ONLY ;
vCJD case study highlights blood transfusion risk
CREUTZFELDT JAKOB DISEASE MAD COW BASE, CWD, SCRAPIE UPDATE OCT 2007
ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007
Date: Mon, 24 Sep 2007 21:31:55 -0500
I suggest that you all read the data out about h-BASE and sporadic CJD, GSS,blood, and some of the other abstracts from the PRION2007. ...
*** PLEASE READ AND UNDERSTAND THE RAMIFICATIONS OF THIS !!! THE PRICE OFPOKER INDEED GOES UP. ...TSS
USA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling ittoday), please note that both the ALABAMA COW, AND THE TEXAS COW, both were''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 200711:52 PM. ...TSS
From: "Terry S. Singeltary Sr."Subject: CWD UPDATE 88 AUGUST 31, 2007
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518