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Monthly Creutzeldt Jakob Disease statistics
Published:
Monday 1 november 2004
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 (vCJD) - the form of the
disease thought to be linked to BSE. The position is as follows:
As at 1st November 2004
Summary of vCJD cases
Deaths
Deaths from definite vCJD (confirmed): 105
Deaths from probable vCJD (without neuropathological confirmation): 39
Deaths from probable vCJD (neuropathological confirmation pending): 2
Number of deaths from definite or probable vCJD (as above): 146
Alive
Number of probable vCJD cases still alive: 5
Total number of definite or probable vCJD (dead and alive): 151
The next table will be published on Monday 6th December 2004
Referrals: 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: All 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 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 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 vCJD cases: are those who fulfil the probable criteria set
out in the Annex and are either still alive, or have died and await post
mortem 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% 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/extrapyramidalsigns 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: Gerstmann-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).
For further information contact the Department of Health Media Centre on
020 7210 5707.
ANNEX
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
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 dysaesthesia
*** generalised triphasic periodic complexes at approximately one per second
****spongiform change and extensive PrP deposition with florid plaques,
throughout
the cerebrum and cerebellum.
[ENDS]
Definite and probable CJD cases in the UK (PDF, 121K
http://www.dh.gov.uk/
http://www.dh.gov.uk/assetRoot/04/09/27/80/04092780.pdf
TSS
