Department of Health and Social Care
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Monthly Creutzfeldt Jakob Disease statistics
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:
Definite and probable CJD cases in the UK:
As at 28 September 2007
Summary of vCJD cases
Deaths
Deaths from definite vCJD (confirmed): 114
Deaths from probable vCJD (without neuropathological confirmation): 47
Deaths from probable vCJD (neuropathological confirmation pending): 0
Number of deaths from definite or probable vCJD (as above): 161
Alive
Number of definite/probable vCJD cases still alive: 5
Total number of definite or probable vCJD (dead and alive):
166
Following this press notice the Department of Health will
stop issuing monthly CJD press notices because the same data is
also published by the National CJD Surveillance Unit in Edinburgh.
Up to date figures for the number of vCJD cases in the UK can be
found on the website of the National CJD Surveillance Unit at: http://www.cjd.ed.ac.uk/figures.htm.
CREUTZFELDT-JAKOB DISEASE IN THE UK
By Calendar Year
REF'S SUSPECT DEATHS OF DEFINITE AND PROB- ABLE CJD
OF CJD
Year Ref's Year Sporadic Iatrogenic Familial GSS vCJD Total
Deaths
1990 [53] 1990 28 5 0 0 - 33
1991 75 1991 32 1 3 0 - 36
1992 96 1992 45 2 5 1 - 53
1993 78 1993 37 4 3 2 - 46
1994 118 1994 53 1 4 3 - 61
1995 87 1995 35 4 2 3 3 47
1996 133 1996 40 4 2 4 10 60
1997 162 1997 60 6 4 1 10 81
1998 154 1998 63 3 3 2 18 89
1999 170 1999 62 6 2 0 15 85
2000 178 2000 50 1 2 1 28 82
2001 179 2001 58 4 3 2 20 87
2002 163 2002 72 0 4 1 17 94
2003 162 2003 79 5 4 2 18 108
2004 114 2004 51 2 4 1 9 67
2005 124 2005 66 4 7 6 5 88
2006 110 2006 65 1 6 3 5 80
2007* 82 2007 30 2 2 1 3 38
Total 2238 Total 926 55 60 33 161 1235
Ref's Deaths
* As at 28th September 2007
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).
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