Published Date: 2003-06-03 23:50:00
Subject: PRO/AH/EDR> CJD (new var.) - UK: update 2003 (07)
Archive Number: 20030603.1356

CJD (NEW VAR.) - UK: UPDATE 2003 (07)
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In this update:
[1] Revised mortality prediction
[2] Monthly UK Department of Health CJD statistics - June 2003
******
[1]
Date: Tue 20 May 2003
From: ProMED-mail <promed@promedmail.org>
Source: BBC News online, Tue 20 May 2003 [edited]
<http://news.bbc.co.uk/2/hi/health/3043227.stm>

Variant Creutzfeldt-Jakob Disease Cases Predicted to Decline
--------------------------------------------------
The worst of the variant Creutzfeldt-Jakob disease [abbreviated as
CJD (new var.) or vCJD in ProMED-mail] problem could be over,
researchers suggest. As few as 40 people in the UK could die from the
human form of BSE over the next 80 years, according to researchers at
Imperial College, London.
Statistics from the CJD Surveillance Unit show 129 [now 131, see
below] people have died from vCJD in the UK since it emerged in 1995.
Deaths are thought to have peaked in 2000, when 28 people died from
the condition. In 2001, 20 died, falling to 17 in 2002. So far this
year, 8 [now 10, see below] people have died from the disease.
Dr Azra Ghani, who carried out the work, said at worst, only another
540 cases would be reported in the UK by 2080. He said: "Our results
suggest that the vCJD epidemic will continue to decline, with a best
estimate of only 40 future cases." Dr Ghani's predictions are based
on data up to the 2002 figures, and do not include any cases arising
through secondary transmission, such as via surgical equipment.
But scientists are still reluctant to state categorically that the
incidence of vCJD is in permanent decline. vCJD tends to affect young
adults. So far, all cases have been seen in people with a specific
genetic characteristic that made them susceptible to the condition.
[A common human prion-protein-gene polymorphism (encoding either
methionine (M) or valine (V) at codon 129) is a strong susceptibility
factor for sporadic and acquired prion disease. All vCJD patients
tested thus far have been M/M homozygotes. - Mod.CP]. If it is seen
in other genetic groups, scientists say case numbers may then rise.
The Imperial College researchers used a statistical model based on
the number of vCJD cases and the level of exposure to cattle to
suggest the incubation period for the disease was likely to be around
12 years. Dr Ghani said: "The development of a diagnostic test that
is able to detect infection early in the incubation period and that
can be used to reduce the risk of secondary transmission remains a
high priority." The research was published in the online version of
BioMed Central Infectious Diseases magazine.
******
[2]
Date: Mon 2 Jun 2003
From: ProMED-mail <promed@promedmail.org>
Source: UK Department of Health, press release 2003/0217, CJD Monthly
Statistics, Mon 2 Jun 2003 [edited]
<http://www.info.doh.gov.uk/doh/intpress.nsf/page/2003-0217?OpenDocument>

Monthly Creutzfeldt-Jakob Disease Statistics; As of Mon 2 Jun 2003
-------------------------------------------------
The Department of Health issued on Mon 2 Jun 2003 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 (vCJD) or CJD (new var.)] -- the form
of the disease thought to be linked to bovine spongiform
encephalopathy (BSE). The position is as follows:
Definite and probable CJD cases in the UK:
Referrals of suspect CJD/ Deaths of definite and probable CJD
Year/ Referrals/ Sporadic/ Iatrogenic/ Familial/ GSS/ vCJD/ Total deaths
1990/ 53/ 28/ 5/ 0/ 0/ -/ 33
1991/ 75/ 32/ 1/ 3/ 0/ -/ 36
1992/ 96/ 45/ 2/ 5/ 1/ -/ 53
1993/ 78/ 37/ 4/ 3/ 2/ -/ 46
1994/ 118/ 53/ 1/ 4/ 3/ -/ 61
1995/ 87/ 35/ 4/ 2/ 3/ 3/ 47
1996/ 134/ 40/ 4/ 2/ 4/ 10/ 60
1997/ 161/ 60/ 6/ 4/ 1/ 10/ 81
1998/ 154/ 63/ 3/ 4/ 1/ 18/ 89
1999/ 170/ 62/ 6/ 2/ 0/ 15/ 85
2000/ 178/ 49/ 1/ 2/ 1/ 28/ 81
2001/ 179/ 56/ 3/ 2/ 2/ 20/ 83
2002/ 163/ 71/ 0/ 4/ 1/ 17/ 93
2003*/ 67/17/ 1/ 1/ 0/ 10/ 29
Total/1713/648/41/38/19/131/877
(*Provisional as of Mon 2 Jun 2003)
Summary of vCJD cases
Deaths:
Deaths from definite vCJD (confirmed): 97
Deaths from probable vCJD (without neuropathological confirmation): 30
Deaths from probable vCJD (neuropathological confirmation pending): 4
Number of deaths from definite or probable vCJD (as above): 131
Alive:
Number of probable vCJD cases still alive: 4
Total number of definite or probable vCJD (dead and alive): 135
The precise definitions of the terms: Referrals, Deaths, Definite
cases, Probable vCJD cases, Sporadic, Probable sporadic, Iatrogenic,
Familial, GSS, variant CJD, and the case definitions can be found by
accessing the Department of Health web-site or by reference to a
preceding ProMED-mail post in this thread [CJD (new var.) - UK:
update Mar 2002 20020305.3693].
The next monthly statistics will be published on Mon 7 Jul 2003.
--
ProMED-mail
<promed@promedmail.org>
[Since the previous monthly statistics released by the Department of
Health on Tue 6 May, the total number of definite or probable vCJD
cases (dead and alive) is unchanged, and the number of deaths has
increased by 2. The number of probable vCJD cases still alive has
decreased by 2. These data are consistent with the continued decline
in vCJD incidence reported during the preceding 6 months (see the
Eurosurveillance quarterly report above and part 1 above).
In view of the experimental evidence from transmission experiments in
mice reported by Asante et al. (MRC Prion Unit, University College,
London) in the EMBO Joural, Vol. 21, No. 23, 6358-6368, 2002, that:
"Some patients with a phenotype consistent with sporadic CJD may have
a disease arising from BSE exposure" (see: CJD, possible association
with BSE 20021129.5921), the figures for sporadic CJD cases now have
added significance in relation to the course of the vCJD outbreak.
<http://emboj.oupjournals.org/cgi/content/abstract/21/23/6358?etoc>
vCJD has been recognized to date only in individuals homozygous for
methionine at PRNP codon 129, and Asante et al. reported that
transgenic mice expressing human PrP methionine 129, inoculated with
either bovine spongiform encephalopathy (BSE) or variant CJD prions,
may develop the neuropathological and molecular phenotype of vCJD,
consistent with these diseases being caused by the same prion strain.
However, BSE transmission to these transgenic mice, in addition to
producing a vCJD-like phenotype, also resulted in a distinct
molecular phenotype that was indistinguishable from that of sporadic
CJD with PrP\Sc type 2. These data suggest that more than one
BSE-derived prion strain might infect humans; it is therefore
possible that some patients with a phenotype consistent with sporadic
CJD may have a disease arising from BSE exposure.
Since Tue 6 May 2003 the number of deaths attributed to sporadic CJD
has increased by 9. - Mod.CP]

See Also

CJD (new var.) - UK: update 2003 (01) 20030108.0057
CJD (new var.) - UK: update 2003 (02) 20030110.0082
CJD (new var.) - UK: update 2003 (03) 20030204.0299
CJD (new var.) - UK: update 2003 (04) 20030304.0544
CJD (new var.) - UK: update 2003 (05) 20030408.0854
CJD (new var.) - UK: update 2003 (06) 20030507.1136
2002
----
CJD, possible association with BSE 20021129.5921
CJD (new var.) - UK: update Dec 2002 20021207.5997
CJD (new var.) - UK: update Nov 2002 20021105.5721
CJD (new var.) - UK: update Oct 2002 20021007.5492
CJD (new var.) - UK: update Sep 2002 20020908.5258
CJD (new var.) - UK: update Aug 2002 20020806.4963
CJD (new var.) - UK: update Jul 2002 20020702.4652
CJD (new var.) - UK: update June 2002 20020606.4418
CJD & CJD (new var.) - UK: update May 2002 20020510.4157
CJD (new var.) - UK: update Apr 2002 20020404.3877
CJD (new var.) - UK: update Mar 2002 20020305.3693
CJD (new var.) - UK: update Feb 2002 20020212.3549
CJD (new var.) - UK: update Jan 2002 20020111.3223
CJD (new var), susp. case - Italy (Sicily) (04):conf 20020927.5418
CJD (new var.), suspected - USA (FL) ex UK 20020419.3989
CJD (new var.) - Canada (SK) ex UK (02) 20020809.5010
CJD (new var.) - China (Hong Kong): confirmed 20020222.3604
CJD (new var.) - France: sixth case 20020418.3983
2001
----
CJD (new var.), incidence & trends - UK 20011115.2816
CJD (new var.), incidence & trends - UK (02) 20011124.2875
CJD (new var.), second wave of cases possible - UK 20010515.0948
CJD (new var.) - UK: 9th Annual Report 20010706.1293
CJD (new var.) - UK: reassessment 20011029.2671
CJD (new var.) - UK: regional variation 20010628.1231
CJD (new var.) - UK: regional variation (02) 20010907.2145
CJD (new var.) - UK: update 3 Jan 2001 20010104.0025
CJD (new var.) - UK: update Aug 2001 20010808.1872
CJD (new var.) - UK: update June 2001 20010622.1188
CJD (new var.) - UK: update Oct 2001 20011005.2419
CJD (new var.) - UK: update Sep 2001 20010906.2134
1999
----
CJD (new var.), human - Ireland 19990715.1192
.................cp/pg/lm
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