Thought this might interest you prion-watchers. Via the ProMED list.
I had a bit of trouble understanding the table, with the loss of
formatting in ASCII text. I think it's the first vertical column
that's headered "Year," and the second "Referrals." Then the others
fall under the five subcategories of "Deaths of definite and probable
cases in the UK." Please note: these are ONLY nvCJD cases in the UK,
apparently. Please note that the source "M2 Communications Ltd." is
not identified by the person who posted this. And please note the
change in SEAC nomenclature from "new variant CJD" to "variant CJD."
Some folks have e-mailed me in the past to ask what "Iatrogenic"
means. It's a word that comes from the Greek /iatros/, meaning
physician (< /iasthai/, to heal), and -genic, origin. In other words,
illness caused by a physician. In a slightly broader sense, it's used
to mean any illness that people get from health care providers. For
example, exposure to hepatitis thru blood transfusions; infection
with "super viruses" (highly antibiotic-resistant viral strains)
while in hospital; or exposure to Creutzfeld-Jakob disease through
corneal transplants, dura mater (cerebral tissue) graft, growth
hormone administration, neurosurgery, etc.
I ran across a reference that might further interest some of you: a
joint workshop, apparently in June, 1998, on transmissible spongiform
encephalopathies sponsored by the University of MD and the FDA:
CJD (NEW VARIANT), CASE RATE - UK (03)
Date: Mon, 06 Sep 1999 14:37:31 -0400
From: Marjorie P. Pollack
Source: M2 Communications Ltd 09/06/1999 13:59 (edited)
The Department of Health is today issuing the latest monthly table, giving
the numbers of deaths of definite and probable cases of Creutzfeldt-Jakob
disease in the UK.
Year Referrals Deaths of definite and
probable cases in the UK
Sporadic latrogenic familial GSS vCJD Total
1985 - 26 1 1 0 - 28
1986 - 26 0 0 0 - 26
1987 - 23 0 0 1 - 24
1988 - 22 1 1 0 - 24
1989 - 28 2 2 0 - 32
1990 53 28 5 0 0 - 33
1991 75 32 1 3 0 - 36
1992 96 43 2 5 1 - 51
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 150 54 3 3 0 16 76
1999* 104 20 2 0 0 4 26
* To 31 July 1999. Total number of definite and probable cases of vCJD = 43.
1. The next table will be published on Monday 4 October 1999.
2. At its meeting on 18 March 1999 the Spongiform Encephalopathy Advisory
Committee (SEAC) agreed variant CJD (vCJD) should now be used in preference
to nvCJD in line with current practice in many scientific journals.
Referrals: This is a simple count of all the cases which 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: These columns show the number of deaths which have occurred in
definite and probable cases of all types of CJD and GSS in the year shown.
The figure includes both cases referred to the Unit for investigation while
the patient was still alive and those where CJD was only discovered post
mortem (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 and Probable: This refers to the diagnostic status of cases. In
definite cases the diagnosis will have been pathologically confirmed, in
most cases by post mortem examination of brain tissue (rarely it may be
possible to establish a definite diagnosis by brain biopsy while the
patient is still alive). Probable cases have not been confirmed
pathologically; some cases are never confirmed pathologically because a
post mortem examination does not take place (for instance where the
relatives of the patient refuse consent) and these cases remain permanently
in the probable category.
Sporadic: Classic CJD cases with typical EEG and brain pathology. Sporadic
cases appear to occur spontaneously with no identifiable cause and account
for 85% of all cases.
Probable sporadic: Cases with a history of rapidly progressive dementia,
typical EEG and at least two of the following clinical features; myoclonus,
visual or cerebellar signs, pyramidal/extrapyramidal signs or akinetic
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% 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 unrecognised variant of CJD discovered by
the National CJD Surveillance Unit and reported in The Lancet on 6 April
1996. This is characterised 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.
Definite vCJD cases still alive: These will be cases where the diagnosis
has been pathologically confirmed (by brain biopsy).
Probable vCJD: Cases in which post-mortem (or brain biopsy) has not been
carried out and which fulfill preliminary criteria for the clinical
diagnosis of vCJD. These criteria cannot yet be fully validated because of
the limited experience of vCJD.
Center for Integrated Ag Systems, UW-Madison
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