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CJD (NEW VAR.), UPDATE 2005 (04)
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A ProMED-mail post
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ProMED-mail is a program of the
International Society for Infectious Diseases
<http://www.isid.org>
[The UK Department of Health web-site 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
<http://www.dh.gov.uk/assetRoot/04/10/53/47/04105347.pdf>.
The definition of the designations deaths, definite cases, probable vCJD
cases, and the case definitions can be found by accessing the Department of
Health web-site 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 Mon 1 Apr 2005
[2] Susceptibility of primates to BSE
[3[ & [4] French case of variant CJD in 1971?
[5] NIH brain collection
******
[1]
Date: Mon 4 Apr 2005
From: ProMED-mail <promed@promedmail.org>
Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease
Statistics, Press release no. 2005/0161, Mon 4 Apr 2005 [edited]
<http://www.dh.gov.uk/PublicationsAndStatistics/PressReleases/PressReleasesNotices/fs/en?CONTENT_ID=4107676&chk=q5G1uM>
Monthly Creutzfeldt Jakob Disease Statistics - As of 1 April 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): 0
Number of deaths from definite or probable vCJD (as above): 149
Summary of vCJD Cases - Alive
-----------------------------
Number of probable vCJD cases still alive: 6
Total
-----
Number of definite or probable vCJD (dead and alive): 155
(The next table will be published on Monday 2 May 2005)
[Since the previous monthly statistics were released on Mon 4 Mar 2005, the
number of deaths from definite vCJD has increased by one, but the total
number of deaths from definite or probable vCJD remains 149. The number of
probable vCJD cases still alive has increased from 5 to 6. Therefore the
overall total number of definite or probable vCJD cases (dead and alive)
has increased from 154 to 155.
As of 1 Apr 2005, so far in the UK for the year 2005 there have 25
referrals of suspected CJD; and there have been 9 deaths from sporadic CJC,
one each from GSS (Gerstmann-Straussler-Scheinker syndrome) and vCJD, and
none from familial CJD or iatrogenic CJD. - Mod.CP]
******
[2]
Date: Mon 14 Mar 2005
From: ProMED-mail <promed@promedmail.org>
Source: Lancet online, 27 Jan 2005; 365: 781-83, 2005 [edited]
<http://image.thelancet.com/extras/05let1056web.pdf>
Susceptibility of Primates to BSE
---------------------------------
[A paper entitled "Risk of oral infection with bovine spongiform
encephalopathy agent in primates," by Corinne Ida Lasmezas and 12
others,appeared in Lancet at the end of January 2005. The abstract is
reproduced below. ProMED-mail regrets the delay in posting this information
submitted by a correspondent- Mod.CP]
Abstract: "The uncertain extent of human exposure to bovine spongiform
encephalopathy (BSE) -- which can lead to variant Creutzfeldt-Jakob disease
(vCJD) -- is compounded by incomplete knowledge about the efficiency of
oral infection and the magnitude of any bovine-to-human biological barrier
to transmission. We therefore investigated oral transmission of BSE to
non-human primates. We gave 2 macaques a 5-g oral dose of brain homogenate
from a BSE-infected cow. One macaque developed vCJD-like neurological
disease 60 months after exposure, whereas the other remained free of
disease at 76 months. On the basis of these findings and data from other
studies, we made a preliminary estimate of the food exposure risk for man,
which provides additional assurance that existing public health measures
can prevent transmission of BSE to man."
[Reassuring or not? - Mod.CP]
*******
[3]
Date: Thu 24 Mar 2005
From: ProMED-mail <promed@promedmail.org>
Source: The Washington Times, United Press International, Thu 24 Mar 2005
[edited]
<http://www.washtimes.com/upi-breaking/20050323-061733-6847r.htm>
French Woman May Have Had Variant Creutzfeldt-Jakob Disease in 1971
--------------------------------------------------
The brain of a French woman who died in 1971 shows evidence consistent with
human mad cow disease, United Press International (UPI) has learned, a
finding that if confirmed would indicate the deadly disease began infecting
people more than 20 years earlier than previously thought.
A former National Institutes of Health scientist said he tested the woman's
brain in 2000 and it showed a pattern that looked like variant
Creutzfeldt-Jakob disease [abbreviated in ProMED-mail as CJD (new var.) or
vCJD] -- a fatal, brain-wasting illness humans can contract from eating
beef products infected with the pathogen that causes mad cow disease, also
known as bovine spongiform encephalopathy or BSE.
vCJD was unheard of in 1971. The 1st recognized case was detected in the
United Kingdom in 1995, so if the French woman did indeed suffer from vCJD,
the case would shift the origins of the disease back more than 2 decades
and possibly to a different country. The woman's brain is held at the
National Institutes of Health in Bethesda, Maryland.
"Variant CJD could've been around for donkey's years, who knows?" said
Bruce Johnson, a former researcher at the NIH's Laboratory for Central
Nervous System Studies (CNSS), who examined the woman's brain. The CNSS lab
received brains from CJD patients from all over the world and has samples
dating back to 1963. The woman's identity could not be revealed for
confidentiality reasons, but it is known she was French and approximately
40 to 50 years old when she died in 1971, Johnson said.
Johnson told UPI he tested the woman's brain using a technique called
Western blotting, which can detect prions -- the infectious agents thought
to play a role in causing vCJD and similar diseases. At the time of her
death, the woman was thought to be suffering from sporadic CJD, a condition
with no known cause that appears to arise spontaneously. However, Johnson
said, the prions he detected looked different from those associated with
[sporadic] CJD and instead were consistent with the prion strain associated
with vCJD. The pattern on the test "was more like BSE than CJD," Johnson
said, noting he never saw a pattern like that in the hundreds of other
brains from CJD patients he had tested.
A sample of the woman's brain had been injected into a chimpanzee sometime
around 1977, and when Johnson examined the chimpanzee's brain, it, too,
showed a pattern consistent with vCJD -- not sporadic CJD. "So she may have
been an early case of BSE in France before it ever got to England," he said.
Johnson said he never published his finding because he wanted to confirm
it, but he never had an opportunity to do so before he retired in 2003. The
CNSS lab was officially closed in April 2004. He said he hopes to conduct
further examinations of the woman's brain when he starts a new position
with the Food and Drug Administration. "If we've still got her brain, we
can look and see if it's BSE," he said. One possible way is to inject some
of the woman's brain into mice.
BSE first showed up in humans in the United Kingdom beginning around 1995.
In all, 154 [now 155] people in that country have been infected with the
human equivalent of mad cow disease. France runs a distant second in vCJD
cases with nine. A recent report published in the journal "Veterinary
Research" estimated that from 1980 to 2000 more than 300 000 cattle were
infected with BSE in France, yet went undetected. Stephen Dealler, a
microbiologist at Lancaster Royal infirmary, recently proposed a hypothesis
that some of the people who developed vCJD in the United Kingdom may have
been exposed to BSE in baby food beginning as early as 1970.
Johnson subscribes to the hypothesis put out by his NIH colleague Joe
Gibbs, who thought it was possible that all mammals, including cows,
spontaneously develop a mad cow-like disease at the rate of one per
million. If that is true, Johnson said, the French woman may have developed
her condition from being unfortunate enough to have eaten infected meat
from that one in a million animal.
Dr. Paul Brown, former medical director of the CNSS lab and an expert on
CJD and BSE, worked with Johnson. He told UPI he remembered Johnson
mentioning the French woman's brain, but the information did not sound
conclusive. He said more research would need to be done to determine if the
woman's disease was vCJD, including injecting it into laboratory animals
and having CJD experts examine the brain tissue.
Patient advocacy representatives had mixed reactions. "I would be looking
to get the opinion of more than one CJD neurologist before making any
further comment," Graham Steel, vice-chair of the patient advocacy group
Human BSE Foundation in the United Kingdom, told UPI. "It doesn't surprise
me at all that you can find a vCJD case in the NIH's brain collection,"
said Terry Singeltary, who is associated with several CJD patient groups
and closely monitors developments about these diseases. "It wouldn't
surprise me for it to go back that far," Singeltary, told UPI. "A lot of
scientists believe this BSE epidemic started way before 1984."
Johnson said it was possible there could be other vCJD cases in the NIH's
collection, which consists of brain samples from hundreds of patients
thought to have CJD. That may never be known, however. The brains have
never been screened for vCJD and the NIH may destroy part or all of the
collection.
[Byline: Steve Mitchell]
--
ProMED-mail
<promed@promedmail.org>
******
[4]
Date: Thu 31 Mar 2005
From: Terry S. Singeltary Sr. <flounder@wt.net>
Source: The Washington Times, United Press International, Thu 31 Mar 2005
[edited]
<http://www.washtimes.com/upi-breaking/20050331-095613-8807r.htm>
French re-testing 1971 case for vCJD
------------------------------------
French researchers are re-analyzing the brain of a woman who died in 1971
for possible variant Creutzfeldt Jakob disease (vCJD), the human version of
mad cow, United Press International (UPI) has learned. If the woman did
have vCJD it would suggest the deadly brain-wasting illness began infecting
people more than 20 years earlier than previously thought. The 1st
recognized case of vCJD, which humans can contract from eating beef
products contaminated with the mad-cow pathogen, was seen in 1995 in the
United Kingdom. As first reported by UPI last week, a former scientist for
the U.S. National Institutes of Health in Bethesda, Maryland, said he
conducted a test on the woman's brain in 2000 and saw patterns that
indicated possible vCJD, rather than sporadic CJD -- a similar condition
that has no known cause.
"We have been very recently informed of the statement of this scientist and
we have been able to identify the case," Dr. Annick Alperovitch, leader of
the French participation in The European and Allied Countries Collaborative
Study Group of CJD, wrote in an e-mail to UPI. "Available data and material
will be carefully re-analyzed, but this will take quite (some) time," added
Alperovitch, who also holds a position with Institut National de la Sante
et de la Recherche Medicale (INSERM) near Paris. She offered no further
details.
"This is potentially earth-shattering,"Neil Cashman, a vCJD expert at the
University of Toronto, told UPI. "It's like finding a case of autopsied
AIDS back in the 1700s." More than 160 cases of vCJD have occurred
worldwide, with nearly all appearing in the United Kingdom where there was
a massive outbreak of mad cow disease starting in the 1980s. 9 vCJD cases
have been detected in France, which also experienced an outbreak of mad cow.
Bruce Johnson, the former NIH scientist, said he used a technique called
western blotting to analyze the woman's brain for prions -- abnormal
proteins thought to play a role in causing CJD and vCJD. A western blot
test revealed a prion strain that looked more like it belonged to vCJD than
CJD, he said, and added he had obtained a similar pattern from a chimpanzee
that had been inoculated with the woman's brain. Further tests that could
be conducted on the French woman's brain to confirm which type of disease
she had contracted include inoculating a sample of the brain into mice or
non-human primates, Cashman said. The mice tests can take about one year,
however.
Patient-advocacy groups applauded the decision of French researchers to
reopen the case. "I'm glad they're going to look at it," said Terry
Singeltary, who lives in Bacliff, Texas, and is involved with several
groups composed of families of patients who developed CJD or vCJD.
Singeltary wanted more than one lab to examine the specimens to provide
confirmation and ensure accuracy of the testing. "I surely would like
someone else to look at the brain besides the French government," he told
UPI. Singeltary said he would like to see a re-examination of certain U.S.
patients who were diagnosed with CJD.
[Byline: Steve Mitchell]
******
[5]
Date: Fri 1 Apr 2005
From: Terry S. Singeltary Sr. <flounder@wt.net>
Source: The Washington Times, United Press International, Fri 1 apr 2005
[edited]
<http://washingtontimes.com/upi-breaking/20050401-033307-7296r.htm>
Groups Seek to Save NIH Brain Collection
----------------------------------------
Scientists, consumer groups, and patient-advocates have embarked upon
efforts -- including petitioning members of Congress and seeking storage
space at a Canadian university -- to prevent the National Institutes of
Health (NIH) from destroying an irreplaceable collection of human brains
from patients afflicted with a condition similar to mad cow disease.
As United Press International (UPI) reported last week, the NIH has begun
shopping for a new home for its collection of brains, spinal fluid and
other tissues from hundreds of patients around the world who died from
Creutzfeldt Jakob disease (CJD) -- an incurable, fatal, brain-wasting
illness. The collection dates back to 1963 and the consensus among
scientists in this field is it is invaluable for research and could provide
insights that might aid in developing diagnostic tests, treatments or cures
for CJD.
NIH officials, however, maintain the remaining samples in the collection --
stored in some 30 freezers by the National Institute for Neurological
Disorders and Stroke in Bethesda, Md. -- are of little value and may be
disposed of if researchers or institutions do not come forward to claim
them. Families of patients who died of CJD have reacted with outrage,
concerned that the effort mounted to collect the brains in the 1st place
has been all for naught. Several have contacted their respective members of
Congress and urged them to step in.
"The brains and brain tissue were sent to NIH in good faith for future
research and destroying them is an outrage," Terry Singeltary, a patient
advocate in Bacliff, Texas, wrote in a letter to Sen. Kay Bailey
Hutchinson, R-Texas, and several other members of the state's congressional
delegation.
CJD belongs to a group of diseases -- called transmissible spongiform
encephalopathies or TSEs -- that includes mad cow disease, chronic wasting
disease in deer and elk, scrapie in sheep and several types of CJD in
humans. There is no cure for CJD and it typically results in death within a
year after the onset of symptoms. Consumer groups also are concerned and
are considering taking steps to ensure the brain collection will be
preserved. "This is outrageous," Michael Hansen, a biologist and senior
research associate with Consumers Union in Yonkers, N.Y., told UPI. "Those
brains are a critical resource for CJD science and they must be at a
research facility.
How can we claim to be a scientific country if we're going to be throwing
away an irreplaceable repository of the 1st evidence of these diseases?"
asked Felicia Nestor, who serves as a consultant to Public Citizen.
There may be hope yet for the collection, however. Neil Cashman, an expert
on TSEs at the University of Toronto's Center for Research in
Neurodegenerative Diseases, told UPI he has been attempting to drum up
support for acquiring the collection with his colleagues at the University
of British Columbia in Vancouver -- where he plans to move this summer.
"The goal would be to make it a resource for the world and make the tissues
available to scientists who had a reasonable request," he added.
One brain in the collection, that of a French woman who died in 1971, may
help provide clues about the origins of variant CJD -- a condition similar
to CJD that humans can contract from eating beef products contaminated with
the mad-cow pathogen. The 1st recognized case of vCJD occurred in 1995 in
the United Kingdom, but an NIH scientist said he tested the French woman's
brain in 2000 and found signs consistent with vCJD -- not CJD. French
researchers currently are re-examining specimens from the case to determine
if the woman was indeed infected with vCJD. If she was, it would suggest
the disease began infecting people more than 20 years earlier than
previously thought. Cashman said the case underscores the value of the NIH
brain collection. "There is information locked up in these freezers that
will be lost forever if this collection is destroyed," he said.
[Byline: Steve Mitchell]
--
ProMED-mail
<promed@promedmail.org>
[see also:
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
2004
----
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
2003
----
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
2002
----
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
2001
----
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
1999
----
CJD (new var.), human - Ireland 19990715.1192]
.................as/mpp/cp/pg/dk
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