Monthly Creutzfeldt Jakob Disease Statistics, UK

The UK 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 30 September 2005


Summary of vCJD cases


Deaths

Deaths from definite vCJD (confirmed): 108

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): 151


Alive

Number of probable vCJD cases still alive: 6

Total number of definite or probable vCJD (dead and alive): 157


The next table will be published on Monday 7th November 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).


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.


UK Dept of Health