Date: 3 Jan 2001 12:26:02 -0500
From: Marjorie P. Pollack <pollackmp@mindspring.com>
Source: M2 Communications Ltd., Wed 3 Jan 2001 12:01 EST [edited]
UK Government: Monthly Creutzfeldt Jakob Disease Statistics
-----------------------------------------------------------
The Department of Health today issued the latest information about the
numbers of known cases of [classical and variant] Creutzfeldt Jakob
disease. The [figures for classic and] variant Creutzfeldt Jakob disease
(vCJD) - the form of the disease thought to be linked to BSE - are as
follows per calendar year for the period 1 Jan 1985 - 28 Dec 2000. [See
below for explanation].
Year / Referrals / Sporadic / Iatrogenic / Familial / GSS / vCJD* / vCJD**
/ 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 / 154 / 63 / 3 / 4 / 1 / - / - / 18 / 89
1999 / 169 / 61 / 6 / 2 / 0 / - / - / 15 / 84
2000 / 172 / 38 / 0 / 2 / 0 / 5 / 2 / 25 / 72
Key:
vCJD* = probable, still alive
vCJD** = probable, still awaiting postmortem results
vCJD*** = confirmed
Notes:
To 28 Dec 2000, the total number of definite and probable cases of vCJD is
88. The next table will be published on Mon 5 Feb 2001.
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: Apart from the 'still alive' column, all columns show number of
deaths which 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 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 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 set out
in the Annex 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, until they ultimately transfer across into the
'awaiting p.m.' or the 'vCJD confirmed' column. It follows therefore that
the figures in these columns will be subject to retrospective adjustment,
for example as and when postmortem confirms diagnosis. After death, some
cases are never confirmed pathologically because a postmortem examination
does not take place (for instance where the relatives of the patient refuse
consent) and these cases remain permanently in the probable category. There
are 7 probable deaths from vCJD in this category where neuropathological
confirmation will never be possible.
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 2 of the following clinical features; myoclonus,
visual or cerebellar signs, pyramidal/extrapyramidal signs or akinetic mutism.
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 unrecognized variant of CJD discovered by
the National CJD Surveillance Unit and reported in The Lancet on 6 Apr
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.
Definite vCJD cases still alive: These will be cases where the diagnosis
has been pathologically confirmed (by brain biopsy).
Diagnostic Criteria for Variant CJD
I.
A) progressive neuropsychiatric disorder
B) duration of illness 6 months
C) routine investigations do not suggest an alternative diagnosis
D) no history of potential iatrogenic exposure.
II.
A) early psychiatric symptoms *
B) persistent painful sensory symptoms **
C) ataxia
D) myoclonus or chorea or dystonia
E) dementia.
III.
A) eeg does not show the typical appearance of sporadic cjd *** (or no eeg
performed)
B) bilateral pulvinar high signal on mri scan.
IV.
A) positive tonsil biopsy.
D definite: IA (progressive neuropsychiatric disorder) and
neuropathological confirmation of vcjd ****.
Probable: I and 4/5 of II and III A and III B or I and IV A
* depression, anxiety, apathy, withdrawal, delusions.
** this includes both frank pain and/or unpleasant dysesthesia.
*** generalized triphasic periodic complexes at approximately one per second.
**** spongiform change and extensive PrP deposition with florid plaques,
throughout the cerebrum and cerebellum.
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