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From: TSS ()
Subject: PRO/AH/EDR> CJD (new var.) update 2005 (05)
Date: May 6, 2005 at 9:22 am PST

-------- Original Message --------
Subject: PRO/AH/EDR> CJD (new var.) update 2005 (05)
Date: Thu, 5 May 2005 22:47:26 -0400 (EDT)
From: ProMED-mail

CJD (NEW VAR.) UPDATE 2005 (05)
A ProMED-mail post

ProMED-mail is a program of the
International Society for Infectious Diseases

[The UK Department of Health website has been revised, and the monthly new
variant Creutzfeldt-Jakob disease statistics are now appended to the
table "Creutzfeldt-Jakob disease in the UK by Calendar Year (since 1990)"
which can be accessed at
The definition of the designations deaths, definite cases, probable vCJD
cases, and the case definitions can be found by accessing the Department of
Health website, or, by reference to a previous ProMED-mail post in this
thread (for example, CJD (new var.) - UK: update Mar 2002 20020305.3693)

The incidence of variant Creutzfeldt-Jakob disease, abbreviated CJD (new
var.) or vCJD in ProMED-mail, in the UK appears to have plateaued, or
perhaps to be in decline. Therefore, since many of the reports appearing in
the update are only peripherally related to the situation in the UK, the
opportunity is being taken to drop the designation UK from the title of this
thread. - Mod.CP]

In this update:

[1] UK: DH variant CJD monthly statistics - as of Tue 3 May 2005
[2] The Netherlands: Death of 1st Dutch case
[3] France: 2 new cases (10th & 11th)
[4] USA: Fate of CJD Brain Collection Still in Doubt

[1] UK: DH variant CJD monthly statistics - as of Tue 3 May 2005
Date: Thu 5 May 2005
From: ProMED-mail
Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease
Statistics, Press release no. 2005/0161, Tue 3 May 2005 [edited]

Monthly Creutzfeldt Jakob Disease Statistics - As of 3 May 2005
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 [abbreviated in ProMED-mail as CJD (new
var.) or 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

Summary of vCJD Cases - Deaths
Deaths from definite vCJD (confirmed): 107
Deaths from probable vCJD (without neuropathological confirmation): 42
Deaths from probable vCJD (neuropathological confirmation pending): 1

Number of deaths from definite or probable vCJD (as above): 150

Summary of vCJD Cases - Alive
Number of probable vCJD cases still alive: 5

Number of definite or probable vCJD (dead and alive): 155

(The next table will be published on Mon 6 Jun 2005)

[Since the previous monthly statistics were released on Mon 4 Apr 2005, the
number of deaths from probable vCJD has increased by one, and the total
number of deaths from definite or probable vCJD is now 150 (an increase of
one). The number of probable vCJD cases still alive has decreased from 6 to
5. Therefore the overall total number of definite or probable vCJD cases
(dead and alive) remains 155. - Mod.CP]

Total Creutzfeldt-Jakob Cases in the UK for 2005
As of 29 Apr 2005, so far in the UK for the year 2005 there have 31
referrals of suspected CJD; and there have been 10 deaths from sporadic CJD,
one from GSS (Gerstmann-Straussler-Scheinker syndrome), 2 from vCJD, and
none from familial CJD or iatrogenic CJD. Data for years since 1990 can be
accessed at

- Mod.CP]


[2] The Netherlands: Death of 1st Dutch case
Date: Tue 3 May 2005
From: Mary Marshall
Source: Reuters Foundation Alert, Tue 3 May 2005 [edited]

The Netherlands: First Dutch "Mad Cow" Disease Patient Dies
A 26-year-old woman who had recently been diagnosed with the human variant
of "mad cow" disease died on Tue 3 May 2005, the 1st Dutch victim of the
brain wasting illness, her hospital said. The Mesos hospital in the city of
Utrecht declined to give further details at the request of the woman's
family. The hospital had made a diagnosis of probable variant Creutzfeldt-
Jakob disease (vCJD), the human form of bovine spongiform encephalopathy
(BSE), on 15 Apr 2005. Specialists at the Erasmus Medical Centre in
Rotterdam confirmed the diagnosis on 18 Apr 2005.

[More than] 150 cases of vCJD have been reported around the world, mostly in
Britain, but also in France, Ireland, Italy, Japan, Canada and the United
States. The disease is fatal and incurable. It is thought to be caused by
eating food tainted with material from cattle with BSE, a progressive
neurological disorder. The Dutch health ministry has said the woman had
neither travelled to England nor received a blood transfusion, so that her
illness was probably caused by past consumption of tainted meat.

There have been some 77 BSE cases in animals in the Netherlands since 1997
with a peak in 2002, but the government says Dutch beef is safe because all
cattle are tested for BSE, and brain and spinal material is kept apart and
destroyed. The Netherlands is one of the world's biggest exporters of meat
and dairy products, and its livestock sector has undergone major
intensification in the past few years, with most animals raised on
specialised farms.

The country has suffered a series of animal disease crises in the past
decade, including swine fever, foot-and-mouth and avian influenza, leading
to the culling of millions of animals. The Netherlands announced strict new
restrictions in 2004 on blood donation over concerns about the transmission
of vCJD. Mad cow disease first emerged in Britain in the 1980s and forced
the destruction of millions of cattle.

Mary Marshall

[The Dutch daily NRC Handelsblad stated on Tue 3 May 2005 that Dutch experts
were surprised to have their 1st vCJD case that early. This, in view of the
fact that in the UK there was one human case per 1300 cows with BSE, while
in Holland there have been, so far, only 77 BSE cows. They raise the
possibility that the victim consumed (imported) British beef at an earlier
date. - Mod.AS]

[3] France: 2 new cases (10th & 11th)
Date: Tue 5 Apr 2005
From: ProMED-mail
Source: Science Daily, UPI report, Tue 5 Apr 2005 [edited]

France: 2 More Cases of Varian Creutzfeldt-Jakob Disease
2 new cases of human mad cow disease were detected in France, raising the
total number of cases in that country to 11, health officials said. The
Institut de Veille Sanitaire said in a statement the 2 patients were not
known to be blood donors. Human mad cow disease, known formally as variant
Creutzfeldt Jakob disease (vCJD) can be transmitted via blood.

Humans can contract the incurable, fatal disease by consuming beef products
infected with the mad cow pathogen. The agency did not offer further details
about the status or identity of the 2 new vCJD cases. Of the 11 cases
reported in France, 8 have died and 3 are still alive.

More than 160 instances of vCJD have been identified around the world, with
the bulk [now 155 - Mod.CP]) in the United Kingdom, where a massive outbreak
of mad cow disease began in the 1980s. A recent report in the
journal "Veterinary Research" estimated more than 300 000 cows infected with
bovine spongiform encephalopathy (mad cow disease) went undetected in France
between 1980 and 2000.


[4] USA: Fate of CJD Brain Collection Still in Doubt
Date: Sat 9 Apr 2005
From: Terry S. Singeltary Sr.
Source: Washington Times, UPI report, 8 Apr 2005 [edited]

USA: NIH Sends Mixed Signals on CJD Brains
A National Institutes of Health (NIH) official who told United Press
International (UPI) the agency might destroy its collection of brains from
human patients afflicted with a condition similar to mad cow disease
reportedly has told the head of a patient-advocate group the collection
would be preserved.

The official, Eugene Major, Acting Director of the Basic Neuroscience
Program at the NIH, has not responded to e-mail or a phone call from UPI
seeking clarification of his remarks, and the official status of the
collection remains unknown. As reported by UPI on 24 Mar 2005, the
collection is stored in freezers by the NIH's National Institute for
Neurological Disorders and Stroke in Bethesda, Maryland. It contains brains
and other tissue samples from hundreds of people who died from the brain-
wasting illness Creutzfeldt-Jakob disease (CJD), as well as tissues from an
untold number of experimental animals. The consensus of scientists in this
field is that the collection, which dates back to 1963, is invaluable for
research and could even provide insight into treatments for the fatal

Currently, there is no cure for CJD and patients typically die within a year
after symptoms begin. Florence Kranitz, president of the non-profit advocacy
group CJD Foundation, told UPI she had "a very long conversation" with
Major, in which he told her the remaining tissues in the collection would
not be destroyed. "He reassured me in no uncertain terms," Kranitz said,
noting constituents of the foundation and other CJD advocacy groups had been
expressing concerns to her the tissues would be destroyed. Kranitz said she
plans to meet with Major at the end of April 2005 to discuss the issue

CJD belongs to a group of diseases collectively known as transmissible
spongiform encephalopathies, or TSEs, that includes mad cow disease in cows,
chronic wasting disease in deer and elk, and scrapie in sheep. All TSEs are
incurable and fatal. Major previously told UPI some samples already have
been destroyed and others have been given to researchers at the Food and
Drug Administration and the National Prion Disease Pathology Surveillance
Center in Cleveland. Major said the remaining collection "has very little
remaining value" and could be destroyed if another entity does not claim

Bruce Johnson, a former NIH scientist who retired at the end of 2003, said
he had been told the collection would be destroyed in 2 years if no one took
the samples from the NIH. In response to hearing that Major had failed to
confirm to UPI the brain collection would not be destroyed, Patricia
Ewanitz, who lives in Port Jefferson Station, N.Y., and is founder of the
advocacy group CJD Voice, said, "The brain tissue might not be indispensable
to the National Institutes of Health, but it is absolutely necessary to the
families who thought enough of science to donate the brains, brain tissue
and blood in hopes of someday finding an answer to why their loved one died."

The revelation the NIH might destroy part or all of the collection sparked
an outcry from patient advocates, consumer groups and scientists. Advocates
have been contacting their members of Congress, urging them to investigate
and prevent the NIH from destroying the brains. Consumer groups also have
gotten involved, and scientists have taken steps to obtain the collection or
have urged Major not to destroy the samples. Felicia Nestor, who serves as a
consultant to Public Citizen, told UPI she had contacted certain legislators
and at least one was considering looking into the situation. Kranitz said
Major also told her he plans "to advertise in professional neurological
journals and by whatever means necessary to make it known" to researchers in
the field the tissues are available.

Major previously said, however, that efforts to inform researchers of the
availability of the collection were already underway and included informing
NIH grantees. He added he had personally notified researchers at scientific
meetings, but no TSE researcher contacted by UPI was aware of this. "I was
never informed," said Laura Manuelidis, an expert on these diseases and
section chief of surgery in the neuropathology department at Yale
University. She said the first she had heard of the situation was in UPI's
24 Mar 2005 report.

Manuelidis also said she contacted Major, expressing interest in the
specimens, but so far has not received a response. Neil Cashman, a TSE
expert at the University of Toronto, who said he was not aware the samples
might be destroyed, has lobbied colleagues at the University of British
Columbia -- where Cashman is scheduled to move this summer -- to help draft
a letter requesting the collection. The Memorial Institute for
Neurodegenerative Diseases Inc., a non-profit organization consisting of
more than 40 university and institute researchers from the United States,
Canada, the United Kingdom and France, requested the collection in January,
2004. So far, the institute has not been informed of a decision by the NIH.

[Byline: Steve Mitchel]

Terry S. Singeltary Sr

[see also:
CJD (new var.) - Netherlands (02): 1st case 20050423.1135
CJD (new var.) - Netherlands: 1st case 20050422.1118
CJD (new var.) update 2005 (04) 20050405.0982
CJD (new var.) update 2005 (03) 20050308.0687
CJD (new var.) update 2005 (02) 20050211.0467
CJD (new var.) - UK: update 2005 (01) 20050111.0095
CJD, genetic susceptibility 20041112.3064
CJD (new var.) - UK: update 2004 (14) 20041206.3242
CJD (new var.) - UK: update 2004 (13) 20041103.2977
CJD (new var.) - UK: update 2004 (12) 20041023.2871
CJD (new var.) - UK: update 2004 (11) 20041008.2758
CJD (new var.) - UK: update 2004 (10) 20040909.2518
CJD (new var.) - UK: update 2004 (09) 20040809.2199
CJD (new var.) - UK: update 2004 (08) 20040806.2150
CJD (new var.) - UK: update 2004 (07) 20040706.1807
CJD (new var.) - UK: update 2004 (06) 20040608.1535
CJD (new var.) - UK: update 2004 (05) 20040510.1262
CJD (new var.) - UK: update 2004 (04) 20040406.0937
CJD (new var.) - UK: update 2004 (03) 20040314.0713
CJD (new var.) - UK: update 2004 (02) 20040202.0400
CJD (new var.) - UK: update 2004 (01) 20040106.0064
CJD (new var.) - France: 8th case 20041022.2864
CJD (new var.) - France: 9th case 20041123.3138
CJD (new var.), blood supply - UK 20040318.0758
CJD (new var.), carrier frequency study - UK 20040521.1365
CJD (new var.) - UK: update 2003 (13) 20031216.3072
CJD (new var.) - New Zealand: suspected (04) 20030817.2057
CJD (new var.) - Italy (05): death 20030809.1969
CJD (new var.) - New Zealand: suspected 20030807.1941
CJD (new var.) - Czech Republic: suspected 20030711.1707
CJD (new var.) - Spain (Madrid): suspected 20030208.0333
CJD (new var.) - UK: update 2003 (01) 20030108.0057
CJD (new var.) - UK: update Dec 2002 20021207.5997
CJD, possible association with BSE 20021129.5921
CJD (new var), susp. case - Italy (Sicily) (04):conf 20020927.5418
CJD (new var.) - Canada (SK) ex UK (02) 20020809.5010
CJD (new var.), suspected - USA (FL) ex UK 20020419.3989
CJD (new var.) - France: sixth case 20020418.3983
CJD (new var.) - China (Hong Kong): confirmed 20020222.3604
CJD (new var.) - UK: update Jan 2002 20020111.3223
CJD (new var.), incidence & trends - UK (02) 20011124.2875
CJD (new var.), incidence & trends - UK 20011115.2816
CJD (new var.) - UK: reassessment 20011029.2671
CJD (new var.) - UK: update Oct 2001 20011005.2419
CJD (new var.) - UK: regional variation (02) 20010907.2145
CJD (new var.) - UK: update Sep 2001 20010906.2134
CJD (new var.) - UK: update Aug 2001 20010808.1872
CJD (new var.) - UK: 9th Annual Report 20010628.1231
CJD (new var.) - UK: update June 2001 20010622.1188
CJD (new var.) - UK: update 3 Jan 2001 20010104.0025
CJD (new var.), human - Ireland 19990715.1192]
ProMED-mail makes every effort to verify the reports that
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