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From: TSS ()
Subject: MONTHLY CREUTZFELDT JAKOB DISEASE STATISTICS April 04, 2005
Date: April 4, 2005 at 10:05 am PST

-------- Original Message --------
Subject: MONTHLY CREUTZFELDT JAKOB DISEASE STATISTICS April 04, 2005
Date: Mon, 4 Apr 2005 09:10:30 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@KALIV.UNI-KARLSRUHE.DE


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

Date: April 04, 2005 Time: 13:45

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 1 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): 0
Number of deaths from definite or probable vCJD (as above): 149 Alive
Number of probable vCJD cases still alive: 6
Total number of definite or probable vCJD (dead and alive): 155 The next
table will be published on Monday 2nd May 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 available of cjd annual stats from the website (PDF, 145K)

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.wired-gov.net/WGLaunch.aspx?ARTCL=31013


SEE dramatic increase of sporadic CJD cases over a decade,
from 1993 to 2002-03 in BSE countries ;

http://www.eurocjd.ed.ac.uk/sporadic.htm


TOTAL CASES OF FAMILIAL/GENETIC CJD AND IATROGENIC CJD


DEATHS TO 31 DECEMBER 2004


http://www.eurocjd.ed.ac.uk/genetic.htm

NOTICE JUMP IN ANNUAL MORTALITY RATES PER MILLION
IN SOME BSE DOCUMENTED COUNTRIES FROM 1993-2002-03;

(France, Slovakia and Switzerland)

http://www.eurocjd.ed.ac.uk/allcjd.htm

TSS

######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html ##########






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