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From: TSS ()
Subject: Monthly creutzfeldt jakob disease statistics 02/07/2007
Date: July 2, 2007 at 9:12 am PST

02/07/2007 13:20


Department of Health (National)

(DH) Monthly CJD stats



Monthly creutzfeldt jakob disease statistics

The Department of Health is today issuing the latest information about the numbers of known cases of Creutzfeldt Jakob disease. This includes cases of variant Creutzfeldt Jakob disease (vCJD) - the form of the disease thought to be linked to BSE. The position is as follows:

Definite and probable CJD cases in the UK:

As at 2 July 2007
Summary of vCJD cases

Deaths
Deaths from definite vCJD (confirmed): 114
Deaths from probable vCJD (without neuropathological confirmation): 47
Deaths from probable vCJD (neuropathological confirmation pending): 0
Number of deaths from definite or probable vCJD (as above): 161

Alive
Number of probable vCJD cases still alive: 4

Total number of definite or probable vCJD (dead and alive): 165

The next table will be published on Monday 6 August 2007

Referrals: a simple count of all the cases which have been referred to the Nationa CJD Surveillance Unit for further investigation in the year in question. CJD may be no more than suspected; about half the cases referred in the past have turned out not to be CJD. Cases are notified to the Unit from a variety of sources including neurologists, neuropathologists, neurophysiologists, general physicians, psychiatrists, electroencephalogram (EEG) departments etc. As a safety net, death certificates coded under the specific rubrics 046.1 and 331.9 in the 9th ICD Revisions are obtained from the Office for National Statistics in England and Wales, the General Register Office for Scotland and the General Register Office for Northern Ireland.

Deaths: All columns show the number of deaths that have occurred in definite and probable cases of all types of CJD and GSS in the year shown. The figures include both cases referred to the Unit for investigation while the patient was still alive and those where CJD was only discovered post mortem (including a few cases picked up by the Unit from death certificates). There is therefore no read across from these columns to the referrals column. The figures will be subject to retrospective adjustment as diagnoses are confirmed.

Definite cases: this refers to the diagnostic status of cases. In definite cases the diagnosis will have been pathologically confirmed, in most cases by post mortem examination of brain tissue (rarely it may be possible to establish a definite diagnosis by brain biopsy while the patient is still alive).

Probable vCJD cases: are those who fulfil the 'probable' criteria set out in the Annex and are either still alive, or have died and await post mortem pathological confirmation. Those still alive will always be shown within the current year's figures.

Sporadic: Classic CJD cases with typical EEG and brain pathology. Sporadic cases appear to occur spontaneously with no identifiable cause and account for 85% of all cases.

Probable sporadic: Cases with a history of rapidly progressive dementia, typical EEG and at least two of the following clinical features; myoclonus, visual or cerebellar signs, pyramidal/extrapyramidalsigns or akinetic mutism.

Iatrogenic: where infection with classic CJD has occurred accidentally as the result of a medical procedure. All UK cases have resulted from treatment with human derived pituitary growth hormones or from grafts using dura mater (a membrane lining the skull).

Familial: cases occurring in families associated with mutations in the PrP gene (10 - 15% of cases).

GSS: Gerstmann-Straussler-Scheinker syndrome - an exceedingly rare inherited autosomal dominant disease, typified by chronic progressive ataxia and terminal dementia. The clinical duration is from 2 to 10 years, much longer than for CJD.

vCJD: Variant CJD, the hitherto unrecognised variant of CJD discovered by the National CJD Surveillance Unit and reported in The Lancet on 6 April 1996. This is characterised clinically by a progressive neuropsychiatric disorder leading to ataxia, dementia and myoclonus (or chorea) without the typical EEG appearance of CJD. Neuropathology shows marked spongiform change and extensive florid plaques throughout the brain.

Definite vCJD cases still alive: These will be cases where the diagnosis has been pathologically confirmed (by brain biopsy).

CREUTZFELDT-JAKOB DISEASE IN THE UK
By Calendar Year


REF'S DEATHS OF
OF DEFINITE
SUSPECT AND
CJD PROBABLE
CJD
Year Ref's Year Sporadic Iatrogenic Familial GSS vCJD Total
Deaths
1990 [53] 1990 28 5 0 0 - 33
1991 75 1991 32 1 3 0 - 36
1992 96 1992 45 2 5 1 - 53
1993 78 1993 37 4 3 2 - 46
1994 118 1994 53 1 4 3 - 61
1995 87 1995 35 4 2 3 3 47
1996 133 1996 40 4 2 4 10 60
1997 162 1997 60 6 4 1 10 81
1998 154 1998 63 3 3 2 18 89
1999 170 1999 62 6 2 0 15 85
2000 178 2000 50 1 2 1 28 82
2001 179 2001 58 4 3 2 20 87
2002 163 2002 72 0 4 1 17 94
2003 162 2003 79 5 4 2 18 108
2004 114 2004 51 2 4 1 9 67
2005 123 2005 65 3 7 6 5 86
2006 110 2006 60 1 6 3 5 75
2007* 55 2007 14 2 0 1 3 20
Total 2210 Total 904 54 58 33 161 1210
Ref's Deaths

Ref's = Referrals

* As at 2nd July 2007

Summary of vCJD cases

Deaths
Deaths from definite vCJD (confirmed): 114
Deaths from probable vCJD (without neuropathological confirmation): 47
Deaths from probable vCJD (neuropathological confirmation pending): 0
Number of deaths from definite or probable vCJD (as above): 161

Alive
Number of definite/probable vCJD cases still alive: 4

Total number of definite or probable vCJD (dead and alive): 165

Notes to editor

ANNEX
DIAGNOSTIC CRITERIA FOR VARIANT CJD
I A) PROGRESSIVE NEUROPSYCHIATRIC DISORDER
B) DURATION OF ILLNESS > 6 MONTHS
C) ROUTINE INVESTIGATIONS DO NOT SUGGEST AN ALTERNATIVE
DIAGNOSIS
D) NO HISTORY OF POTENTIAL IATROGENIC EXPOSURE
II A) EARLY PSYCHIATRIC SYMPTOMS *
B) PERSISTENT PAINFUL SENSORY SYMPTOMS **
C) ATAXIA
D) MYOCLONUS OR CHOREA OR DYSTONIA
E) DEMENTIA
III A) EEG DOES NOT SHOW THE TYPICAL APPEARANCE OF SPORADIC CJD *** (OR NO EEG PERFORMED)
B) BILATERAL PULVINAR HIGH SIGNAL ON MRI SCAN
IV A) POSITIVE TONSIL BIOPSY
DEFINITE: IA (PROGRESSIVE NEUROPSYCHIATRIC DISORDER) and NEUROPATHOLOGICAL CONFIRMATION OF vCJD ****
PROBABLE: I and 4/5 OF II and III A and III B
or I and IV A
* depression, anxiety, apathy, withdrawal, delusions.
** this includes both frank pain and/ or unpleasant dysaesthesia
*** generalised triphasic periodic complexes at approximately one per second
****spongiform change and extensive PrP deposition with florid plaques, throughout the cerebrum and cerebellum.


[ENDS]

Client ref 2007/0184

GNN ref 148867P

http://www.wired-gov.net/wg/wg-news-1.nsf/lfi/148867

USA CJD STATISTICS SO HIGH THEY DONT SEEM TO REPORT IN 2007 YET ?

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...

http://www.cjdsurveillance.com/resources-casereport.html


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535


BRITISH MEDICAL JOURNAL

BMJ

http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406


BMJ

http://www.bmj.com/cgi/eletters/320/7226/8/b#6117


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


doi:10.1016/S1473-3099(03)00715-1
Copyright © 2003 Published by Elsevier Ltd.
Newsdesk

Tracking spongiform encephalopathies in North America

Xavier Bosch

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 my
mom to hvCJD (Heidenhain variant CJD)
and have been searching for answers ever since. What I have found is that we
have not been told the truth. CWD
in deer and elk is a small portion of a much bigger problem.”
............................


http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext

http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf


see history of cjd questionnaire

http://brain.hastypastry.net/forums/showthread.php?t=2408


Lancet 1996; 347: 921- 25


A new variant of Creutzfeldt-Jakob disease in the UK

R G Will, J W Ironside, M Zeidler, S N Cousens, K Estibeiro, A Alperovitch, S Poser, M Pocchiari, A Hofman, P G Smith


Summary


snip...

Discussion


The ten cases of CJD in this report are remarkable in that they have a specific neuropathological profile which, to our knowledge, has not been described previously[6,8] and which is so consistent that neuropathological samples from the cases are virtually indistinguishable. The cases are further characterised by having remarkably low ages at onset for CJD and other atypical features, including a generally protracted and unusual clinical course and absence of EEG changes typical of CJD. These findings raise the possibility that the cases represent a new clinicopathological variant of CJD.


Effect of age


It is possible that the unusual neuropathological profile of these cases is due to their young age. Review of published reports on previous young patients worldwide did not reveal any descriptions of neuropathology similar to these UK cases. In 14 cases of CJD aged less than 30 years previously reported outside the UK, plaques are described in only one, and in this report the possible diagnosis of Gerstmann- Straussler- Scheinker syndrome was raised. In four of these cases,[9-12] pathological reports have been reviewed and there was no evidence of PrP plaques (Paul Brown, personal communication). We did immunocytochemical staining on another of these cases of CJD aged 27 years from Poland (courtesy of Professor Kulczycki) and on a 16- year- old patient from the UK dying of CJD in 1980, and there was no evidence of plaque formation in either case. We also did immunocytochemical staining on 11 cases of CJD developing after administration of human growth hormone (mean age 27.5 years) and although PrP plaques were present predominantly in the cerebellum, the neuropathological features in these cases'3 were otherwise quite distinct from the young patients in this report. We emphasise that plaque distribution and spongiform change in these ten young cases were clearly apparent on routine light microscopy. Current evidence suggests, therefore, that the pathological profile in these cases is unlikely to be simply an age- related feature.


CJD has been described previously in young patients, but these are usually isolated case reports[9-12] and in systematic surveys the identification of CJD in patients aged less than 30 years old is exceptional. In the UK, only one such case was identified between 1970 and 1989. In France, between 1968 and 1982[14], only two patients aged less than 30 years old were identified; only one was identified in Japan between 1975 and 1977; and none at all in Israel between 1963 and 1987. Additional cases aged less than 40 years have been identified through the European surveillance project on CJD (1993- 95); two cases aged 22 and 34 years old were found in the Netherlands; two aged 31 and 33 years old in Germany; two aged 26 and 37 years old in France; and one aged 37 years old in Italy. Six of these cases are judged on clinical evidence not to be similar to the cases described in this report. Neuropathological information is available on two of these six cases, neither of which showed the characteristic changes. In the remaining case, full neuropathological information will be available shortly.


Case ascertainment


The overall incidence of CJD has risen in the UK in the 1990s[15], although this is due mainly to an increase in the incidence of CJD in those aged over 75 years (these cases have a typical clinicopathological profile). The most likely explanation for this is improved ascertainment of CJD in the elderly, with the possible implication that the identification of young cases of CJD may be due to similar improved case ascertainment due in part to the publicity surrounding the BSE epidemic. It is noteworthy that three of the ten cases in this report were notified to the CJD Surveillance Unit as suspect cases of CJD only after biopsy samples had been examined. In the absence of neuropathological examination, these cases might not have come to the attention of the CJD Surveillance Unit. It seems likely, however, that patients of this age dying of a progressive neurological condition would have undergone necropsy in the past. Two cases came to the attention of the CJD Surveillance Unit through unconventional means (through a newspaper report and after a clinical presentation of other cases) which led to their notification earlier than would otherwise have been the case. All of the ten cases were identified over 10 months and although there was extensive publicity surrounding two young cases in late 1995, there has been considerable publicity regarding CJD and BSE since 1990. Other European countries have undertaken systematic surveillance of CJD over a similar period and there has been no obvious increase in the incidence of CJD in young patients despite detailed investigation.


There is a possibility that the diagnosis of such atypical cases may previously have been previously missed. Three of the 14 cases discussed above were from Poland, aged 19, 23, and 27 years, and were identified in the course of a study of subacute sclerosing panencephalitis (SSPE)[16] A recent review of the clinical details of suspect but unconfirmed cases of SSPE held by the SSPE register in the UK has provided no evidence that cases of CJD were misdiagnosed as SSPE in the UK. Although improved ascertainment remains a potential explanation for the identification of the young patients we report, such information as is available does not support this interpretation.


Possible link with BSE


The first aim of the CJD Surveillance Unit has been to identify any changes in CJD that might be attributable to the transmission of BSE to the human population. Although the small number of cases in this report cannot be regarded as proof, the observation of a potentially new form of CJD in the UK is consistent with such a link. The common neuropathological picture may indicate infection by a common strain of the causative agent, as in sheep scrapie in which strains of the disease have been identified which can be distinguished on the basis of diseaseincubation period and distinctive neuropathological profile in mouse models[17]. Exposure of the human population to the BSE agent is likely to have been greatest in the 1980s, and especially towards the end of that decade, before the ban on the use of specified bovine offal was introduced. This would be consistent with an incubation period of between 5 and 10 years for these cases.


If the present cases are due to exposure to the BSE agent and this accounts for the distinctive neuropathological appearance, it is not clear why this previously unrecognised variant of the disease has been found only in persons under the age of 45 years. The absence of this variant in older persons could be due to age- related exposure to the agent; to reduced susceptibility among older persons; or to misdiagnosis of this variant of the disease in older age- groups, especially in those in which dementia is more common.


We were alerted earlier to a possible link between CJD and BSE by our finding of an apparent excess of CJD among cattle farmers[15]. Our interpretation of this was tempered by observations of high rates among cattle farmers in other European countries in which BSE was either very rare or had not been reported. None of the four farmers showed the neuropathological features described here, and all were consistent with previous experience of sporadic cases of CJD.


Conclusions


We believe that our observation of a previously unrecognised variant of CJD occurring, to date, only in persons under the age of 45 years is a cause for great concern. That it is due to exposure to the BSE agent is perhaps the most plausible interpretation of our findings. However, we emphasise that we do not have direct evidence of such a link and other explanations are possible. That these cases have been observed now because of improved ascertainment cannot be completely dismissed. It seems unlikely, however, that such a distinctive neuropathological pattern would have been missed previously, especially among persons dying at a young age. It is essential to obtain information on the clinical and neuropathological characteristics of young patients with CJD in Europe and elsewhere, and historically in the UK, but proof of an association between BSE and CJD may depend on animal transmission studies and continued epidemiological vigilance. If there is a causal link then, given the potentially long and widespread exposure to the BSE agent, further cases of this new variant of CJD are likely to arise.


We thank J Mackenzie for data management, P Brown for reviewing an early version of the manuscript, J Collinge for assistance with the molecular analysis, and W B Matthews who initiated CJD surveillance in the UK in the 1980 for advice. The CJD Surveillance Unit is funded by the Department of Health and the Scottish Home and Health Department and suported by BBSRC (grant no 15/BS204814). The Concerted Action on CJD Surveillance in Europe was funded through the EC Biomed I Programme. The epidemiological surveillance of CJD would not be possible without the collaboration of neurologists and neuropathologists throughout the UK and Europe.


References


snip.....


http://www.cjd.ed.ac.uk/lancet.htm


sporadic CJD, the big lie


Subject: Sporadic creutzfeldt-jakob disease in two adolescents (see sCJD,
the big lie)
Date: May 28, 2007 at 7:58 am PST

J Neurol Neurosurg Psychiatry. Published Online First: 23 May 2007.
doi:10.1136/jnnp.2006.104570
© 2007 by BMJ Publishing Group Ltd

Original articles

Sporadic creutzfeldt-jakob disease in two adolescents

K Murray 1, D L Ritchie 1, M Bruce 2, C A Young 3, M Doran 3, J W Ironside 4
and R G Will 4*
1 NationalCJD Surveillance Unit, United Kingdom
2 Neuropathogenesis Unit, United Kingdom
3 Walton Centre for Neurology and Neurosurgery, United Kingdom
4 National CJD Surveillance Unit, United Kingdom

* To whom correspondence should be addressed. E-mail: r.g.will@ed.ac.uk.

Accepted 15 April 2007


Abstract


Background: Sporadic Creutzfeldt-Jakob disease (CJD) is a condition
predominantly affecting older age groups, with cases aged less than 45 years
rare and an age at onset or death of less than 20 years exceptional.

Methods: Data from the systematic study of sporadic CJD in the UK are
available from 1970 onwards. Clinical and pathological data are reviewed in
order to identify atypical cases, including those at the extremes of the age
range of sporadic CJD. Detailed analysis of atypical cases is undertaken and
in selected cases laboratory transmission studies are carried out in order
to provide information on the characteristics of the infectious agent.

Results: In the UK two cases of sporadic CJD in adolescents have been
identified, dying aged 16 and 20 years. The first case predated the epidemic
of bovine spongiform encephalopathy and the characteristics of the second
case, including laboratory transmission studies, are consistent with a
diagnosis of sporadic rather than variant CJD.

Conclusion: The cases in this report indicate that sporadic CJD can develop
at a very young age, that variant CJD is not the only form of CJD occurring
in this age group and that neuropathological examination is essential to
accurate diagnosis of human prion disease.


http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1


Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE

Terry S. Singeltary Sr.


POLICY IN CONFIDENCE; CONFIDENTIAL; CJD IN FARMER WITH BSE COW ie
OCCUPATIONAL EXPOSURE


Subject: POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW


POLICY IN CONFIDENCE: CJD IN FARMER WITH BSE COW

LIKELY TO ATRACT MEDIA ATTENTION

snip...


DOES ANYONE BESIDES ME SEE A PATTERN YET ???


Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic
CJD, whatever the hell that is. and there have been 16 year old die from
sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly
are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the
ukbsenvcjd only myth.


snip...


see full text ;


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276


USA MAD COW STRAIN MORE VIRULENT TO HUMANS THAN UK STRAIN

18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7
December 2006 are now available.

snip...

64. A member noted that at the recent Neuroprion meeting, a study was
presented showing that in transgenic mice BSE passaged in sheep may be more
virulent and infectious to a wider range of species than bovine derived BSE.

Other work presented suggested that BSE and bovine amyloidotic spongiform
encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the
prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A
MUTATION FOUND IN CASES OF SPORADIC CJD.

snip...

http://www.seac.gov.uk/minutes/95.pdf

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western
Reserve
University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years.

***These results indicate that BASE is transmissible to humans and suggest
that BASE is more virulent than
classical BSE in humans.***

6:30 Close of Day One

http://www.healthtech.com/2007/tse/day1.asp

TSS




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