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From: TSS ()
Subject: CJD Figures up to 29th April 2005 UK - USA STILL NOT LOOKING
Date: May 4, 2005 at 8:30 am PST

-------- Original Message --------
Subject: CJD Figures up to 29th April 2005
Date: Wed, 4 May 2005 10:24:42 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@aegee.org


##################### Bovine Spongiform Encephalopathy #####################

Monthly Creutzfeldt Jakob Disease statistics

Published:
Tuesday 3 May 2005
Reference number:
2005/0161

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 29 April 2005

Summary of vCJD cases

Deaths

Deaths from definite vCJD (confirmed): 107

Deaths from probable vCJD (without neuropathological confirmation): 42

Deaths from probable vCJD (neuropathological confirmation pending): 1

Number of deaths from definite or probable vCJD (as above): 150

Alive

Number of probable vCJD cases still alive: 5

Total number of definite or probable vCJD (dead and alive): 155

The next table will be published on Monday 6th June 2005

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).

Related links

Download cjd annual stats (PDF, 145K)


Related links

Download CJD Table Stats May (PDF, 76K)

Notes to editor

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.

http://www.dh.gov.uk/PublicationsAndStatistics/PressReleases/PressReleasesNotices/fs/en?CONTENT_ID=4109443&chk=RClc%2BW


CJD Statistics

------------------------------------------------------------------------


CJD Figures

These figures show the number of suspect cases referred to the CJD
surveillance unit in Edinburgh, and the number of deaths of definite and
probable cases in the UK, up to 29th April 2005.

REFERRALS OF SUSPECT CJD

DEATHS OF DEFINITE AND PROBABLE CJD

Year

Referrals

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

134

1996

40

4

2

4

10

60

1997

161

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

77

5

4

2

18

106

2004

112

2004

49

2

3

1

9

64

2005* 31 2005 10 0 0 1 2 13

Total Referrals

1951

Total Deaths

771

48

44

24

150

1037

*As at 29th April 2005

Summary of vCJD cases

Deaths

Deaths from definite vCJD (confirmed):

Deaths from probable vCJD (without neuropathological confirmation):

Deaths from probable vCJD (neuropathological confirmation pending):

Number of deaths from definite or probable vCJD:

107

42

1

150

Alive

Number of definite/probable vCJD cases still alive:

5

Total number of definite or probable vCJD cases (dead and alive):

155

(Table updated 3 May 2005)

http://www.dh.gov.uk/assetRoot/04/10/94/42/04109442.pdf

TSS

############ https://www.lists.uni-karlsruhe.de/warc/bse-l.html ############






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