Published Date: 2002-03-05 23:50:00
Subject: PRO/AH/EDR> CJD (new var.) - UK: update Mar 2002
Archive Number: 20020305.3693

CJD (NEW VAR.) - UK: UPDATE MAR 2002
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See Also

CJD (new var.) - UK: update Feb 2002 20020212.3549
CJD (new var.) - UK: update Jan 2002 20020111.3223
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
Date: Tue 5 Mar 2002
From: ProMED-mail <promed@promedmail.org>
Source: UK Department of Health, press release 2002/0057, Mon 4 Feb
2002 [edited
<http://tap.ccta.gov.uk/doh/intpress.nsf/page/2002-0108?OpenDocument>

Monthly Creutzfeldt-Jakob Disease Statistics; 4 Mar 2002
--------------------------------------------------------
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 as
(vCJD) or CJD (new var,) in ProMED-mail -- 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:
Year/Referrals/Sporadic/Iatrogenic/Familial/GSS/vCJD/Total deaths
1990/53/28/5/0/0/-/33
1991/75/32/1/3/0/-/36
1992/96/44/2/5/1/-/52
1993/78/38/4/2/2/-/46
1994/116/51/1/4/3/-/59
1995/87/35/4/2/3/3/47
1996/134/40/4/2/4/10/60
1997/161/59/6/4/1/10/80
1998/154/63/3/4/1/18/89
1999/169/61/6/2/0/15/84
2000/178/48/1/2/1/28/80
2001/172/48/3/2/2/20/75
2002*/23/3/0/1/0/5/9
*As of 4 Mar 2002
Deaths:
Definite vCJD (confirmed): 89
Probable vCJD (without neuropathological confirmation): 18
Probable vCJD (neuropathological confirmation pending): 2
Total: Definite or probable vCJD (as above): 109
Alive:
Number of probable vCJD cases still alive: 7
Total: Definite or probable vCJD (dead and alive): 116
Definitions:
Referrals: A simple count of all the cases that 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: 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 postmortem (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 postmortem 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 fulfill the 'probable' criteria
[see website and are either still alive, or have died and await
postmortem 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 percent of all cases.
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 percent of cases).
GSS: Gertsmann-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 unrecognized variant of CJD
discovered by the National CJD Surveillance Unit and reported in The
Lancet on 6 April 1996. This is characterized 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.
--
ProMED-mail
<promed@promedmail.org>
[Since the previous monthly statistics released by the Department of
Health on Mon 4 Feb 2002, the total number of definite or probable
vCJD cases has increased by 2. It is too early to predict the future
trend, but the figures do not suggest an imminent rise in the number
of vCJD cases. - Mod.CP
........................cp/pg/lm
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