Subject: Creutzfeldt Jakob Disease statistics Monday 5 February 2007
Date: February 5, 2007 at 7:34 am PST
Monday 5 February 2007 10:57
Department of Health (National)
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 2 February 2007
Summary of vCJD cases
Deaths
Deaths from definite vCJD (confirmed): 112
Deaths from probable vCJD (without neuropathological confirmation): 46
Deaths from probable vCJD (neuropathological confirmation pending): 0
Number of deaths from definite or probable vCJD (as above): 158
Alive
Number of probable vCJD cases still alive: 7
Total number of definite or probable vCJD (dead and alive): 165
The next table will be published on Monday 5th March 2007
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)
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.
Client ref 2007/0025
GNN ref 143618P
http://www.gnn.gov.uk/environment/fullDetail.asp?ReleaseID=261721&NewsAreaID=2&NavigatedFromDepartment=False
http://www.eurocjd.ed.ac.uk/sporadic.htm
FRANCE CJD
http://212.234.146.165/publications/mcj/donnees_mcj.html
CANADA CJD
http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/stats_e.html#ref
Titre du document / Document title
La malattia di Creutzfeldt-Jakob e le altre forme umane di encefalopatie spongiformi trasmissibili in Italia : uno studio di mortalità condotto su fonti diverse (Creutzfeldt-jakob disease and related disorders in Italy : a mortality study carried out from different data sources)
Auteur(s) / Author(s)
CONTI Susanna (1) ; MASOCCO Maria (1) ; SOLIMINI Renata (1) ; TOCCACELI Virgilia (1) ; VICHI Monica (1) ; LADOGANA Anna (2) ; ALMONTI Susanna (2) ; PUOPOLO Maria (2) ; POCCHIARI Maurizio (2) ;
Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)
(1) Centra Nazionale di Epidemiologia, Sorveglianza e Promozione délia Salute, ITALIE
(2) Dipartimento di Biologia Cellulare e Neuroscienze, Istituto Superiore di Sanità, Roma, ITALIE
Résumé / Abstract
Creutzfeldt-Jakob Disease (CJD) is a rare pathology (about 1 case per million) but different data sources). - Creutzfeldt-Jakob Disease (CJD) is a rare pathology (about T case per inntion) but it has a great importance for Public Health; the Italian National CJD register has been established in the Istituto Superiore di Sanità (ISS) since 1993, and epidemiological studies on CJD have been carried out as well. This paper reports a mortality study carried out comparing and integrating data from the two available sources: the National CJD Register and the Italian Data Base on Mortality, processed by the ISS Statistics Unit, on the data collected by the Italian Census Bureau (ISTAT). The study allowed to estimate: the underreporting of CJD mortality to both sources, the misclassification of ISTAT data and the integrated mortality rates from CJD in Italy: 1.58 per million persons aged 25 or more, average rate during the period 1993-1999.
Revue / Journal Title
Annali dell' Istituto superiore di sanità (Ann. Ist. super. sanità) ISSN 0021-2571
Source / Source
2005, vol. 41, no1, pp. 103-111 [9 page(s) (article)] (26 ref.)
Langue / Language
Italien
Editeur / Publisher
Istituto superiore di sanita, Roma, ITALIE (1965- 2005) (Revue)
Mots-clés anglais / English Keywords
Nervous system diseases ; Central nervous system disease ; Degenerative disease ; Cerebral disorder ; Europe ; Infection ; Prion disease ; Medical data ; Prion ; Medicine ; Medical record ; Surveillance ; Public health ; Epidemiology ; Mortality ; Italy ; Creutzfeldt Jakob disease ;
Mots-clés français / French Keywords
Système nerveux pathologie ; Système nerveux central pathologie ; Maladie dégénérative ; Encéphale pathologie ; Europe ; Infection ; Prion maladie ; Donnée médicale ; 1993-1999 ; Prion ; Médecine ; Dossier médical ; Surveillance ; Santé publique ; Epidémiologie ; Mortalité ; Italie ; Encéphalopathie spongiforme Creutzfeldt Jakob ;
002b17g ;
Mots-clés espagnols / Spanish Keywords
Sistema nervioso patología ; Sistema nervosio central patología ; Enfermedad degenerativa ; Encéfalo patología ; Europa ; Infección ; Prion enfermedad ; Prion ; Medicina ; Historial clínico ; Vigilancia ; Salud pública ; Epidemiología ; Mortalidad ; Italia ; Encefalopatía espongiforme Creutzfeldt Jakob ;
Mots-clés d'auteur / Author Keywords
Creutzfeldt-Jakob disease ; mortality ; data sources ; surveillance ; record linkage ;
Localisation / Location
INIST-CNRS, Cote INIST : 6513, 35400013237541.0170
Copyright 2006 INIST-CNRS. All rights reserved
Toute reproduction ou diffusion même partielle, par quelque procédé ou sur tout support que ce soit, ne pourra être faite sans l'accord préalable écrit de l'INIST-CNRS.
No part of these records may be reproduced of distributed, in any form or by any means, without the prior written permission of INIST-CNRS.
Nº notice refdoc (ud4) : 17038684
http://cat.inist.fr/?aModele=afficheN&cpsidt=17038684
Neurology. 2005 May 10;64:1592-7 15883322
High incidence of genetic human transmissible spongiform encephalopathies in Italy.
[My paper] A Ladogana , M Puopolo , A Poleggi , S Almonti , V Mellina , M Equestre , M Pocchiari
OBJECTIVE: To assess the incidence and mortality rates of genetic transmissible spongiform encephalopathy (TSE) diseases in Italy. METHODS: The authors have sequenced the prion protein gene (PRNP) in 643 patients referred to the Italian Registry of Creutzfeldt-Jakob disease (CJD) and related disorders between 1993 and 2002. Crude age- and sex-specific incidence and mortality rates were calculated. Differences in morbidity from genetic TSE diseases in the 20 Italian regions were assessed by the standardized morbidity ratio (SMR). RESULTS: A total of 130 cases were classified as genetic TSE diseases with a mean yearly incidence rate of 0.28 cases per million people. Genetic TSE diseases represent 17.7% of all TSE diseases, including sporadic, iatrogenic, and variant CJD. The most frequent mutation was the V210I (n = 54), and the second most common the E200K (n = 42). Mortality rates for genetic TSE diseases did not increase in any of the age groups under examination over the 10 years of surveillance. The analysis of regional distribution of genetic cases by place of birth revealed that in Campania and Calabria regions the number of genetic TSE cases was higher than in other regions. CONCLUSIONS: In Italy the incidence of genetic transmissible spongiform encephalopathy (TSE) diseases is the second highest among European countries. Genetic analysis is important for a correct classification of patients with TSE.
Mesh-terms: Adult; Age Distribution; Aged; Cohort Studies; DNA Mutational Analysis; Female; Genetic Predisposition to Disease, genetics; Genetic Screening; Geography; Humans; Iatrogenic Disease, epidemiology; Incidence; Italy, epidemiology; Male; Middle Aged; Mortality; Mutation, genetics; Prion Diseases, genetics; Prion Diseases, mortality; Prions, genetics; Research Support, Non-U.S. Gov't; Sex Factors;
http://lib.bioinfo.pl/pmid:15883322
http://lib.bioinfo.pl/auth:Ladogana,A
USA CJD
3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit
Hall
3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse
Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve
University
Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years. ***These results indicate that BASE is
transmissible to humans and suggest that BASE is more virulent than
classical BSE in humans.
6:30 Close of Day One
http://www.healthtech.com/2007/tse/day1.asp
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...
http://www.cjdsurveillance.com/resources-casereport.html
There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.
He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7
December 2006 are now available.
snip...
64. A member noted that at the recent Neuroprion meeting, a study was
presented showing that in transgenic mice BSE passaged in sheep may be more
virulent and infectious to a wider range of species than bovine derived BSE.
Other work presented suggested that BSE and bovine amyloidotic spongiform
encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the
prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A
MUTATION FOUND IN CASES OF SPORADIC CJD.
snip...
http://www.seac.gov.uk/minutes/95.pdf
TSS