Monthly creutzfeldt jakob disease statistics 02/07/2007

[flatfish]

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

snip...

* 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.


continued

[flatfish]

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 sporadic CJD the big lie;


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


continued

[flatfish]

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