A ProMED-mail post
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International Society for Infectious Diseases
[With the continuing decline of the number of cases in the human
population of variant Creutzfeldt-Jakob disease -- abbreviated
previously as vCJD or CJD (new var.) in ProMED-mail -- it has been
decided to broaden the scope of the occasional ProMED-mail updates to
include other prion-related diseases. In addition to vCJD, data on
other forms of CJD: sporadic, iatrogenic, familial, and GSS
(Gerstmann-Straussler-Scheinker disease) are included also since they
may have some relevance to the incidence and etiology of vCJD. -
Mod.CP]
In this update:
[1] UK: National CJD Surveillance Unit - monthly statistics as of Mon
10 Jan 2011 - no new vCJD cases
[2] France: Institut de Veille Sanitaire - monthly statistics as of
Wed 4 Jan 2011 - no new vCJD cases
[3] USA: National Prion Disease Pathology Surveillance Center -
cumulative case numbers for 2010 up to 1 Nov 2010 - no vCJD cases
[4] Taiwan: suspected vCJD case, ex UK, DOH statement
[5] Taiwan: suspected vCJD case, 'ex UK'
[6] Italy (Livorno): 2nd vCJD case
******
[1]
UK: National CJD Surveillance Unit - monthly statistics as of Mon 10
Jan 2011 - no new vCJD cases
Date: Mon 10 Jan 2011
Source: UK National CJD Surveillance Unit, monthly statistics [edited]
The number of deaths due to definite or probable vCJD during 2010 was
170. A total of 4 definite/probable patients are still alive so the
total number of definite or probable vCJD recorded during 2010 was
174.
Although 3 new deaths due to vCJD were recorded in 2009 and 3 deaths
in 2010, the overall picture is still consistent with the view that
the vCJD outbreak in the UK is in decline, albeit now with a
pronounced tail. The 1st cases were observed in 1995, and the peak
number of deaths was 28 in the year 2000, followed by 20 in 2001, 17
in 2002, 18 in 2003, 9 in 2004, 5 in 2005, 5 in 2006, 5 in 2007, one
in 2008, 3 in 2009, and 3 in 2010.
Totals for all types of CJD cases in the UK in the year 2010
------------------------------------------------------------
During 2010, there were 146 referrals, 69 fatal cases of sporadic
CJD, 3 fatal cases of vCJD, 2 cases of iatrogenic CJD, 5 cases of
familial CJD, and one case of GSS.
Since records began in 1990 there have been 2729 referrals, 1199
fatal cases of sporadic CJD, 170 cases of vCJD, 64 cases of
iatrogenic CJD, 82 cases of familial CJD and 43 of GSS.
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[2] France: Institut de Veille Sanitaire - monthly statistics as of 4
Jan 2011 - no new vCJD cases
Date: Tue 4 Jan 2011
Source: IVS - Maladie de Creutzfeldt-Jakob et maladies apparentees
[in French, trans. & summ. Mod.CP, edited]
During the 12 months of 2010, there were 1610 referrals, 98 confirmed
cases of sporadic CJD, 6 cases of familial CJD, and no cases of
iatrogenic CJD or vCJD.
A total of 25 cases of confirmed or probable vCJD have been recorded
in France since records began in 1992. There was 1 case in 1996, 1 in
2000, 1 in 2001, 3 in 2002, 2 in 2004, 6 in 2005, 6 in 2006, 3 in
2007, 2 in 2009, and none in 2010.
The 25 confirmed cases comprise 13 females and 12 males. All 25 are
now deceased. Their median age is 37 (between 19 and 58). 7 were
resident in the Ile-de-France and 18 in the provinces. All the
identified cases have been Met-Met homozygotes. No risk factor has
been identified. One of the 25 had made frequent visits to the United
Kingdom, during about 10 years from 1987.
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[3] USA: National Prion Disease Pathology Surveillance Center -
cumulative case numbers for 2010 up to 1 Nov 2010 - no vCJD cases
Date: Mon 1 Nov 2010
Source: US National Prion Disease Pathology Surveillance Center [edited]
Cumulative data 1 Jan 2010 to 31 October 2010
---------------------------------------------
During the 10-month period 1 Jan 2010 to 31 Oct 2010, there were 333
referrals, 213 of whom were classified as prion disease cases,
comprising 158 cases of sporadic CJD, 33 of familial CJD, and none of
iatrogenic CJD or vCJD.
Overall of 3831 referrals examined since screening began in 1996 or
thereabouts, diagnosis is still pending in 18 cases, and another 18
cases have been considered inconclusive. [This is a measure of the
difficulty in achieving unequivocal diagnoses. - Mod.CP]
During the same period a total of 2265 prion disease cases have been
screened, a figure which includes 23 (22 during 2010) cases with type
determination pending, but in which a diagnosis of vCJD has been
excluded. 3 cases of vCJD recorded in the USA during the same period
have been attributed to infection in the United Kingdom in 2 cases,
and in Saudi Arabia in the other.
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[4] Taiwan: suspected vCJD case, ex UK, DOH statement
Date: Thu 9 Dec 2010
Source: Taiwan Today [edited]
A 36-year-old Taiwanese male is very likely to have died from
Creutzfeldt-Jakob disease, the Cabinet-level Department of Health
(DOH) said on [8 Dec 2010].
The patient, who died this May [2010], resided in the United Kingdom
between 1989 and 1997, when bovine spongiform encephalopathy (BSE) in
cattle was at its peak in the UK. It is unlikely that the patient
contracted his disease in Taiwan, the DOH said.
Scientists believe that many years after eating cattle infected with
BSE, patients can develop symptoms of CJD. In the case of the
36-year-old patient, he began to show CJD symptoms, including memory
loss and hypersomnia, in the 2nd half of 2008. Doctors reported to
health authorities in March 2009 that they suspected he had CJD.
After the patient died this year [2010], his family refused to give
doctors permission to perform [an autopsy], the only way the doctors
could determine the cause of death with certainty. His body has since
been cremated. "Though tests were not conducted, the man is an
extremely likely case of CJD, based on his symptoms, travel history,
and the results of magnetic resonance imaging and
electroencephalograms," the Centers for Disease Control under the DOH
said.
The DOH further specified that the patient probably died from variant
CJD [vCJD]. "The World Health Organization has listed the man as a
vCJD case as his brain waves and cerebrospinal fluid showed symptoms
of CJD and vCJD simultaneously," said Chen Shun-seng, convener of
DOH's CJD advisory committee and a neurologist at Kaohsiung Chang
Gung Memorial Hospital.
According to the World Health Organization, CJD is a fatal human
degenerative condition characterized by progressive brain dysfunction
and is categorized into 4 forms: sporadic, familial, iatrogenic, and
variant. "It is believed to be caused by self-replicating
host-encoded protein or prion protein," the WHO said.
According to the UK Department of Health, vCJD differs from CJD in
that the former is more likely to strike younger people. The average
age of death for vCJD victims is around 30 years old, it said.
As of October 2010, there have been 243 possible or extremely
possible cases of CJD reported in Taiwan, resulting in 102 deaths. Of
the deaths, 5 have been confirmed as resulting from CJD, the DOH said.
[byline: Grace Kuo]
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HealthMap Alerts via ProMED-mail
[This press report provides background to the case described in the
preceding update (ProMED-mail Prion disease update 2010 (11)
20101206.4364, part [4], which was an abstract reproduced from the
journal Psychiatry and Clinical Neurosciences
The press report above states that the World Health Organization has
listed the man as a vCJD case, but I have been unable to verify this
statement. - Mod.CP]
[The HealthMap/ProMED-mail interactive map of Taiwan is available at
******
[5] Taiwan: suspected vCJD case, ex UK
Date: Sat 18 Dec 2010
Source: Focus Taiwan, Central News Agency (CNA) report [edited]
The 1st probable human case of mad cow disease in Taiwan was listed
posthumously Saturday [18 Dec 2010], following the death in May
[2010] of a man who had symptoms of the fatal brain-wasting illness.
[The clinical investigation of this case was reported in the
preceding update: Prion disease update 2010 (11) 20101206.4364, part
[4] -- Yang CW, Fuh JL, Wang SJ, et al: Probable variant
Creutzfeldt-Jakob disease in Asia: A case report from Taiwan and
review of two prior cases. Psychiatry Clin Neurosci, 64(6): 652-8.
doi: 10.1111/j.1440-1819.2010.02151.x. published online 25 Nov 2010;
abstract available at
The listing was made after officials and experts on a Department of
Health (DOH) panel on Creutzfeldt-Jakob Disease (CJD) held a meeting
to determine whether the man had died from the disease as result of
eating infected beef.
"We believe it is very likely that the man who died in May this year
[2010] contracted the disease during his stay in the United Kingdom,"
the DOH said in a press statement. "We cannot rule out the
possibility that he ate infected beef during that time."
Scientists believe that many years after eating cattle infected with
bovine spongiform encephalopathy (BSE) , commonly known as mad cow
disease, humans can develop symptoms of new variant CJD (vCJD), also
known as human mad cow disease.
The 36-year-old man had lived in the UK between 1989 and 1997, when
mad cow disease was at its peak in that country. He began to show
vCJD symptoms, including memory loss and hypersomnia, in 2008.
Doctors reported the case to the DOH's Centers for Disease Control in
March 2009.
As his family refused to give doctors permission to perform an
autopsy, the DOH said, it could not list the patient as a confirmed
case of vCJD. His body has since been cremated.
Though the cause of death could not be determined by means of an
autopsy, it was an extremely likely case of vCJD, based on the man's
symptoms, travel history, and the results of magnetic resonance
imaging and electroencephalograms, the DOH said.
According to the DOH's CJD advisory panel, it was unlikely that the
patient contracted the disease in Taiwan.
"His death could be counted as an imported case of vCJD and unlikely
to pose any threat to Taiwan's disease control network," the DOH
statement said.
Citing its tracking records, the DOH said the man had donated blood
to 2 patients 10 years ago. "Neither of them has contracted the
disease," the statement said, adding that precautionary measures had
been taken to prevent any spread of the disease during the time the
man was hospitalized in Taiwan.
According to the World Health Organization, CJD is a fatal human
degenerative condition characterized by progressive brain dysfunction
and is categorized into four forms -- sporadic, familial, iatrogenic,
and variant.
The UK Department of Health has said vCJD differs from CJD in that
the former is more likely to strike younger people.
Since 1997 when the DOH set up a CJD reporting and monitoring system,
436 suspected CJD or vCJD have been reported in Taiwan, DOH figures
show. Of that number, 246 were listed as possible or extremely
possible cases of sporadic or familial forms of CJD, 5 were confirmed
as traditional forms of CJD and one was listed as probable vCJD,
according to the DOH tallies.
The others were ruled out as possible cases of CJD.
[byline: Chen ching-fang, Sofia Wu]
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[In view of the fact that the patient's body has been cremated, the
investigation cannot proceed further and the case must remain
suspected rather than confirmed vCJD. If the patient's disease was
vCJD, it is probable that it was contracted during the victim's 8
years residence in the UK. - Mod.CP]
*****
[6] Italy (Livorno): 2nd vCJD case
Date: Thu 6 Jan 2011
Source: Adnkronos Inernational (AKI) [edited]
The human variant of the brain-wasting 'mad cow' disease claimed a
2nd victim in Italy after a 44-year-old woman died in hospital in the
northwestern port city of Livorno, the local health authority
reported on Thursday [6 Jan 2011]. The health authority said the
44-year-old Livorno woman died on Wednesday 5 Jan 20011] and [had
been] admitted to a city hospice in July [2010] in the final stages
of her battle against variant Creutzfeldt-Jakob disease (vCJD),
commonly known as 'mad cow' disease. There is no known cure for vCJD.
Italy's 1st case, a Sicilian woman, died in 2002, a year after she
contracted the disease.
Variant Creutzfeldt-Jakob disease is the human form of the fatal
brain-wasting illness first identified in cattle -- BSE or bovine
spongiform encephalopathy. A total of 275 people have been infected
with the disease over the past decade, 170 in Britain, the
worst-affected country, 56 in Israel, and 25 in France. [As of 31 Dec
2010 the current totals of vCJD cases according to the European and
Allied Countries CJD Study Group
(
Eire - 4, Italy - 4, USA - 3*, Canada - 1, Saudi Arabia - 1*, Japan -
1*, Netherlands - 3, Portugal -2, Spain - 5, where * indicates
non-indigenous. - Mod.CP]
Nearly 200 000 cattle have died from BSE over the same period. The
human form of BSE is believed to be caused by eating meat infected
with the bovine disease. EU restrictions on the sale of T-bone beef
steaks and beef offal were lifted in 2006 when officials declared the
'mad cow' outbreak was over.
A massive cull in Britain prevented the spread of the disease but
isolated cases are still reported. Almost 190 000 cattle have died
from BSE over the past 10 years, the vast majority in the UK.
Italy's health ministry and various local health authorities have
always denied vCJD patients can infect their family members and
carers with the disease.
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[The HealthMap/ProMED-mail interactive map of Italy is available at
[see also:
2010
---
Prion disease update 2010 (11) 20101206.4364
Prion disease update 2010 (10) 20101105.4008
Prion disease update 2010 (09) 20101006.3622
Prion disease update 2010 (08) 20100911.3285
Prion disease update 2010 (07) 20100809.2720
Prion disease update 2010 (06) 20100706.2248
Prion disease update 2010 (05) 20100507.1488
Prion disease update 2010 (04) 20100405.1091
Prion disease update 2010 (03) 20100304.0709
Prion disease update 2010 (02) 20100205.0386
Prion disease update 2010 20100107.0076]
.................sb/cp/mj/lm
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