SEVENTEENTH ANNUAL REPORT 2008 CREUTZFELDT-JAKOB DISEASE SURVEILLANCE IN THE UK
The National CJD Surveillance Unit Western General Hospital, Edinburgh, EH4 2XU www.cjd.ed.ac.uk Infectious Disease Epidemiology Unit London School of Hygiene and Tropical Medicine Keppel Street, London, WC1E 7HT
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. The information provided in this Seventeenth Annual Report continues to indicate that the number of sporadic cases remains relatively stable (the data for 2008 may still be incomplete). Detailed clinical and epidemiological information has been obtained for the great majority of patients. Referrals, having been fewer between 2004 and 2007, increased in 2008, back towards pre-2004 levels. 2008 has seen the highest mortality rate from sporadic CJD in the UK (1.43 per million per year) since 1985; a rate which is comparable with other European countries. 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 for sporadic CJD in the neuropathology laboratory rose from 23 in 2007 to 28 in 2008 (32 in 2006). In 1990-2008 average annual mortality rates from sporadic CJD in England, Wales, Scotland and Northern Ireland were, respectively, 0.94, 1.08, 0.96 and 0.58/million/year. The differences between these rates are not statistically significant (p=0.4). 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=122) and Northern Ireland (SMR=74) 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 2008, there were 164 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 2008. The clinical, neuropathological and epidemiological features of the cases of vCJD are remarkably uniform and consistent with 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 147 clinically affected cases of vCJD with available genetic analysis have been methionine homozygotes. In 2008 the NCJDSU was referred the first case who met the clinical criteria in life for possible vCJD and was heterozygous (methionine/valine) at codon 129 of the PRNP gene (no postmortem was undertaken). The clinical picture was typical of vCJD seen to date, which is reassuring for surveillance purposes. For further information on this case, see Section 2.3, page 14. Although a single case with only a ‘possible’ classification, this may have implications for the presentation of further clinical cases in codon 129 heterozygotes in the future and for the estimation of prevalence of sub-clinical infection in the population. Section 1 T Seventeenth Annual Report 2008 4 Analysis of vCJD diagnoses and deaths from January 1994 to December 2008 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 an individual of the PRNP-129 MV genotype as a possible case of vCJD and, in a separate case, disease-related prion protein in the spleen of a clinically unaffected blood recipient (reported in 2004) is consistent with such a hypothesis. These cases, along with the report of the prevalence of abnormal prion protein in the large study of appendix and tonsil tissues, suggests the possibility of a greater number of prec inical or subclinical cases in the population than might be indicated by the present numbers of confirmed clinical cases. The incidence of vCJD is higher in the north of Britain than in the south and the only statistically significant geographic cluster of vCJD cases in the UK remains that seen in Leicestershire (5 cases occurring between 1996 and 1999. The NCJDSU continues to collaborate with the Health Protection Agency Centre for Infections and Health Protection Scotland, in relation to a range of activities, including testing of pathological specimens from the National Anonymous Tonsil Archive study through to input into the development and implementation of public health policy, for example, in relation to the follow up of those identified as at increased risk of CJD. This year, the neuropathology laboratory identified a UK adult haemophiliac patient with PrPres in a restricted distribution in the spleen. This patient had been entered into a joint study with the UK Haemophilia Centre Doctors’ Organisation and NCJDSU. The patient had no neurological signs or symptoms, and no neuropathological evidence of vCJD. This case raises the possibility of transmission of vCJD infectivity via plasma products, and is the subject of ongoing investigations. A case of protease-sensitive prionopathy was identified on neuropathological and biochemical grounds, the first case of this disorder identified in the UK since its description by Gambetti et al in the USA in 2008. 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 disease-related PrP in the spleen of a recipient of blood donated by someone incubating vCJD. In 2008, for the first time, the Unit 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 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 the 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 success of the National CJD Surveillance Unit continues to depend on the extraordinary level of cooperation from the neurology and neuropathology communities and other medical and paramedical staff throughout the UK. Ongoing support is provided by the Infectious Disease Epidemiology Unit, London School of Hygiene and Tropical Medicine. We are also particularly grateful to the relatives of patients for their collaboration.
see full text ;
Tuesday, August 11, 2009
Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants
Brian S. Appleby, MD; Kristin K. Appleby, MD; Barbara J. Crain, MD, PhD; Chiadi U. Onyike, MD, MHS; Mitchell T. Wallin, MD, MPH; Peter V. Rabins, MD, MPH
Background: The classic Creutzfeldt-Jakob disease (CJD), Heidenhain, and Oppenheimer-Brownell variants are sporadic CJD (sCJD) phenotypes frequently described in the literature, but many cases present with neuropsychiatric symptoms, suggesting that there may be additional sCJD phenotypes.
Objective: To characterize clinical, diagnostic, and molecular features of 5 sCJD variants.
Design: Retrospective analysis.
Setting: The Johns Hopkins and Veterans Administration health care systems.
Participants: Eighty-eight patients with definite or probable sCJD.
Main Outcome Measures: Differences in age at onset, illness progression, diagnostic test results, and molecular subtype.
Results: The age at onset differed among sCJD variants (P=.03); the affective variant had the youngest mean age at onset (59.7 years). Survival time (P.001) and the time to clinical presentation (P=.003) differed among groups. Patients with the classic CJD phenotype had the shortest median survival time from symptom onset (66 days) and those who met criteria for the affective sCJD variant had the longest (421 days) and presented to clinicians significantly later (median time from onset to presentation, 92 days; P=.004). Cerebrospinal fluid analyses were positive for 14-3-3 protein in all of the affective variants, regardless of illness duration. Periodic sharp-wave complexes were not detected on any of the electroencephalography tracings in the Oppenheimer-Brownell group; basal ganglia hyperintensity was not detected on brain magnetic resonance imaging in this group either. All of the Heidenhain variants were of the methionine/ methionine type 1 molecular subtype.
Conclusions: The classic CJD phenotype and the Heidenhain, Oppenheimer-Brownell, cognitive, and affective sCJD variants differ by age at disease onset, survival time, and diagnostic test results. Characteristics of these 5 phenotypes are provided to facilitate further clinicopathologic investigation that may lead to more reliable and timely diagnoses of sCJD.
Arch Neurol. 2009;66(2):208-215
snip...see full text ;
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009
August 10, 2009
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena’s. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. But, while sub-clinical, how many can one exposed human infect? Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.
The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub- clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? 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. ...
please see history, and the ever evolving TSE science to date ;
Saturday, June 13, 2009
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009
WHO WILL WATCH THE CHILDREN for CJD over the next 5 decades ?
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
Monday, October 19, 2009
Atypical BSE, BSE, and other human and animal TSE in North America Update October 19, 2009
Sunday, September 6, 2009
MAD COW USA 1997 SECRET VIDEO
U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ? see video at bottom
DAMNING TESTIMONY FROM STANLEY PRUSINER THE NOBEL PEACE PRIZE WINNER ON PRIONS SPEAKING ABOUT ANN VENEMAN see video
CVM Annual Report Fiscal Year 2008: October 1, 2007-September 30, 2008
PUTTING LIPSTICK ON A PIG AND TAKING HER TO A DANCE...TSS
BSE Feed Rule Enforcement: A Decade of Success OFF TO A FAST START
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006