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From: TSS ()
Subject: Monthly Creutzfeldt Jakob Disease statistics As at 2 March 2007
Date: March 5, 2007 at 7:34 am PST

05/03/2007 10:23


Department of Health (National)

(DH) 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 March 2007

Summary of vCJD cases

Deaths

Deaths from definite vCJD (confirmed): 112

Deaths from probable vCJD (without neuropathological confirmation): 46

Deaths from probable vCJD (neuropathological confirmation pending): 0

Number of deaths from definite or probable vCJD (as above): 158

Alive

Number of probable vCJD cases still alive: 7

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

The next table will be published on Monday 2nd April 2007

Referrals: a simple count of all the cases which have been referred to the National 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).

Related links

Download cjd annual stats (PDF, 145K)

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.

Client ref 2007/0051

GNN ref 144667P

https://www.gnn.gov.uk/Content/Detail.asp?ReleaseID=268589&NewsAreaID=2

sporadic CJD


http://www.eurocjd.ed.ac.uk/sporadic.htm


FRANCE CJD

http://212.234.146.165/publications/mcj/donnees_mcj.html


CANADA CJD

http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats_e.html#ref

USA CJD

3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit
Hall


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


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

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:


flounder9@verizon.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

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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

http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=dignosing+and+reporting+creutzfeldt+jakob+disease&searchid=1048865596978_1528&stored_search=&FIRSTINDEX=0&journalcode=jama

BRITISH MEDICAL JOURNAL


BMJ


http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2


BMJ


http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1


Tracking spongiform encephalopathies in North America

THE LANCET Infectious Diseases Vol 3 August 2003 http://infection.thelancet.com


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

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


----- Original Message -----
From: XXXXXXXX XXXXXXXXXXXX(SEAC)
To: 'flounder9@verizon.net'
Sent: Friday, February 23, 2007 8:13 AM
Subject: RE: SEAC ANNUAL REPORT 2006


Dear Mr Singeltary

Thank you for your email about a possible relationship between BASE and
sCJD.

SEAC is continually informed about and considers the emerging science on
both animal and human TSEs. The committee regularly receives updates from
the UK's National CJD Surveillance Centre Unit on the epidemiology of vCJD
and sCJD and is also aware of emerging research on BASE. It is envisaged
that SEAC will conduct a detailed consideration of the available science and
the possible animal and human health implications of BASE at its meeting on
10th May 2007.

Yours sincerely

XXXXXXXXXXXXXX

----------------------------------------------------------------------------
----
From: Terry S. Singeltary Sr. [mailto:flounder9@verizon.net]
Sent: 02 February 2007 16:44
To: Bovine Spongiform Encephalopathy
Cc: SEACsecretariat (AHEG); cjdvoice@yahoogroups.com;
BLOODCJD@YAHOOGROUPS.COM; madcow@lists.iatp.org; Sustainable Agriculture
Network Discussion Group
Subject: SEAC ANNUAL REPORT 2006


Date: February 2, 2007 at 8:25 am PST
SEAC ANNUAL REPORT 2006 (ignoring the obvious)


http://www.seac.gov.uk/publicats/annualreport2006.pdf


i guess the sporadic CJD cases have all be resolved, and the route and cause
classified.
to this day, i cannot figure this out. if BSE farmers died from sporadic
CJD, and now another study seems to point
that BSE and BASE may be the same thing, and that BASE does not produce vCJD
like pathology, but pathology resembling that of sporadic CJD, then why does
SEAC still seem to put sporadic CJD on the back burners ??? IN fact, SEAC
could not even speak about it in there 2006 report. would it not seem
prudent to mention these findings in this 2006 SEAC report ;


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


***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

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.


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

Asante/Collinge et al, 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;


http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

I STILL think to ignore these findings below is not only rediculous, but
indeed very suspicious ;


CJD FARMERS WIFE 1989


http://www.bseinquiry.gov.uk/files/yb/1989/10/13007001.pdf


http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf


cover-up of 4th farm worker ???


http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf


http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf


CONFIRMATION OF CJD IN FOURTH FARMER


http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf


now story changes from;

SEAC concluded that, if the fourth case were confirmed, it would be
worrying, especially as all four farmers with CJD would have had BSE
cases on their farms.

to;

This is not unexpected...

was another farmer expected?

http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf


4th farmer, and 1st teenager

http://www.bseinquiry.gov.uk/files/yb/1996/02/27003001.pdf


2. snip...


Over a 5 year period, which is the time period on which the advice
from Professor Smith and Dr. Gore was based, and assuming a
population of 120,000 dairy farm workers, and an annual incidence
of 1 per million cases of CJD in the general population, a
DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN
an individual in the general population to develop CJD. Using the
actual current annual incidence of CJD in the UK of 0.7 per
million, this figure becomes 7.5 TIMES.

3. You will recall that the advice provided by Professor Smith in
1993 and by Dr. Gore this month used the sub-population of dairy
farm workers who had had a case of BSE on their farms -
63,000, which is approximately half the number of dairy farm
workers - as a denominator. If the above sums are repeated using
this denominator population, taking an annual incidence in the general
population of 1 per million the observed rate in this sub-population
is 10 TIMES, and taking an annual incidence of 0.7 per million,
IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than
that in the general population...


http://www.bseinquiry.gov.uk/files/yb/1995/01/31004001.pdf

CJD YOUNG PEOPLE

in the USA, a 16 year old in 1978;

ALSO IN USA;

(20 year old died from sCJD in USA in 1980 and a 16 year
old in 1981. see second url below)

in France, a 19 year old in 1982;

in Canada, a 14 year old of UK origin in 1988;

in Poland, cases in people aged 19, 23, and 27 were identified in
a retrospective study (published 1991), having been originally
misdiagnosed with a viral encephalitis;

Creutzfeldt's first patient in 1923 was aged 23.

http://www.bseinquiry.gov.uk/files/yb/1995/10/27013001.pdf


20 year old died from sCJD in USA in 1980 and a 16 year
old in 1981. A 19 year old died from sCJD in
France in 1985. There is no evidence of an iatrogenic
cause for those cases....

http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf


FOR SEAC TO continue to flounder with this ukbsenvcjd only theory, when we
know that sporadic CJD's were proven long ago to transmit via the medical
and surgical arena, will only continue to spread this agent around the globe
via a multitude of proven routes and sources.

IN my opinion, for SEAC to continue to go down this same road, year after
year, and continue to ignore sporadic CJD's, and continue to say that "BSE
in the UK sheep population is most likely to be zero, or very low if present
at all." "Consequently, any impact of the schemes on human health from
removing BSE from sheep is likely to be negligible." when in fact they do
not know. and in fact science is 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." IN my opinion this continued neglect of
other TSEs in human and animals, the continued belief in this ukbsenvcjd
only theory, and the risk thereof, is reckless, dangerous, and in my mind,
criminal. SEAC has failed the public terribly in this regard. ...TSS


----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Friday, February 02, 2007 10:43 AM
Subject: SEAC ANNUAL REPORT 2006


Date: February 2, 2007 at 8:25 am PST
SEAC ANNUAL REPORT 2006 (ignoring the obvious)


http://www.seac.gov.uk/publicats/annualreport2006.pdf


i guess the sporadic CJD cases have all be resolved, and the route and cause
classified.
to this day, i cannot figure this out. if BSE farmers died from sporadic
CJD, and now another study seems to point
that BSE and BASE may be the same thing, and that BASE does not produce vCJD
like pathology, but pathology resembling that of sporadic CJD, then why does
SEAC still seem to put sporadic CJD on the back burners ??? IN fact, SEAC
could not even speak about it in there 2006 report. would it not seem
prudent to mention these findings in this 2006 SEAC report ;
........SNIP.........END............TSS




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