Subj: DoH News Release CJD figures

[Guest guest]

Date: 5/6/99 7:29:33 PM Eastern Daylight Time

From: jralphb@... (J Ralph Blanchfield)

Sender: BSE-L@... (Bovine Spongiform Encephalopathy)

Reply-to: BSE-L@... (Bovine Spongiform Encephalopathy)

To: BSE-L@...

Hello Everyone,

Here is the DoH News Release dated 4 May, giving CJD statistics to 31 March

1999.

Total definite and probable vCJD remains unchanged at 40.

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DEPARTMENT OF HEALTH

1999/0270 Tuesday 4th May 1999

MONTHLY CREUTZFELDT-JAKOB DISEASE FIGURES

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.

Deaths of definite and probable cases in the UK the UK

Year Referrals

Sporadic iatrogenic 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 58 6 4 1 10 79

1998 150 50 3 3 0 16 72

1999* 35 6 1 0 0 1 8

* To 31 March 1999. Total number of definite and probable cases of

nvCJD = 40.

1. The next table will be published on Monday 7 June 1999.

2. At its meeting on 18 March 1999 the Spongiform Encephalopathy

Advisory Committee (SEAC) agreed that 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 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 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 fulfil preliminary criteria for the

clinical diagnosis of vCJD. These criteria cannot yet be fully

validated because of the limited experience of vCJD.

[ENDS]

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Best wishes

Ralph

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J Ralph Blanchfield, MBE

Food Science, Food Technology & Food Law Consultant

Chair, IFST External Affairs

Web Editor, Institute of Food Science & Technology

IFST Web address http://www.easynet.co.uk/ifst/>

e-mail: jralphb@...> ICQ# 6254687.

ICQ Web page

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