Subject: DOH:MONTHLY CREUTZFELDT JAKOB DISEASE STATISTICS DEPARTMENT OF HEALTH 2004/0164 4 May 2004 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: Referral 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 4 1 18 89 1999 170 1999 62 6 2 0 15 85 2000 178 2000 50 1 2 1 28 82 2001 180 2001 58 5 3 2 20 88 2002 163 2002 72 0 4 1 17 94 2003 159 2003 70 5 3 2 18 98 2004* 33 2004* 12 0 0 1 2 15 Total 1839 Total 717 47 41 22 141 968 Deaths As at *5 April 2004 Summary of vCJD cases Deaths Deaths from definite vCJD (confirmed): 104 Deaths from probable vCJD (without neuropathological confirmation): 37 Deaths from probable vCJD (neuropathological confirmation pending): 0 Number of deaths from definite or probable vCJD (as above): 141 Alive Number of definite/probable vCJD cases still alive: 5 Total number of definite or probable vCJD (dead and alive): 146 The next table will be published on Monday 7th June 2004 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). For further information contact the Department of Health Media Centre on 020 7210 4860/5287. 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. -------------------------------------------------------------------- GNNREF: 94268 Issued by : DOH Press Office Contact : If you have any queries relating to this press release, please forward this e-mail to the Press Office e-mail address provided above, or alternatively contact the originating Press Office by telephone. (Media queries only) Please DO NOT attempt to reply to the sender of this e-mail. The sender is involved solely as a broadcasting agent and cannot process e-mailed queries. Any attached files with a .DOC extension should be read using a word processor capable of reading Word 97 files. If you believe that the originating body is sending you Word 2000 files that you are not yet capable of reading, ask them to save future files in an earlier version.