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Back To Vidyya News From The UK NHS:

Latest Creutzfeldt Jakob Disease (CJD & vCJD) Statistics


On 03 January 2001, the UK Department of Health issued the latest information about the numbers of known cases of Creutzfeldt Jakob disease. The position on variant Creutzfeldt Jakob disease (vCJD) - the form of
the disease thought to be linked to BSE - is as follows:

Definite and probable CJD cases in the UK by calender year (1 May
1990 - 28 December 2000):
Year Referrals Sporadic Iatrogenic Familial GSS vCJD - Still Alive vCJD - Probable Deaths - Awaiting Confirmation vCJD - Confirmed 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 54 63 3 4 1     -         -     18 89
1999 169 61 6 2 0     -         -     15 84
2000 172 38 0 2 0 5 2 25 72

To 28 December 2000. Total number of definite and probable cases of vCJD = 88.

* including 7 probable deaths from vCJD where neuropathological confirmation will never be possible.

The next table will be published on Monday 5 February 2001.

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: Apart from the 'still alive' column, all 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 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, 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 post mortem confirms diagnosis. After death, 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 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 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).

 


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 LATROGENIC 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.


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