flatfish
01-03-2007, 12:00 PM
Text and figures of the latest annual report of the CJD unit (published 4th December 2006).
FOURTEENTH ANNUAL REPORT 2005
CREUTZFELDT-JAKOB DISEASE
SURVEILLANCE IN THE UK
SUMMARY
The national surveillance programme for Creutzfeldt-Jakob disease (CJD) in the UK was
initiated in May 1990. In 1999, the National CJD Surveillance Unit (NCJDSU) became a
WHO Collaborative Centre for Reference and Research on the surveillance and epidemiology
of human transmissible spongiform encephalopathies (TSEs). In September 2001 the
National Care Team was formed, which currently comprises a care coordinator and a secretary. It
is based within the NCJDSU and was formed in response to concerns regarding the care of CJD
patients.
The information provided in this fourteenth report continues to provide evidence of a high level
of case ascertainment. In last year's report, a decrease in the number of referrals was noted, along
with a drop in the number of recorded sporadic CJD deaths in 2004. This raised concerns about
the completeness of case ascertainment. Further data are now available. While the decline in the
number of referrals has been maintained in 2005, analysis suggests that much, if not all of the
decline is due to changes in the number of referrals who turn out not to be CJD cases. The
number of sporadic cases in 2005 was higher than in 2004 and the data for 2005 may still be
incomplete; thus the present data do not suggest a significant consistent decline in the number of
recorded sporadic CJD deaths (discussed in more detail in Section 2). Detailed clinical and
epidemiological information has been obtained for the great majority of patients. The case-control
study for risk factors of CJD has continued recruitment and initial analysis has been undertaken,
with the results with respect to vCJD published in early 2006. Although there is ongoing evidence
that the post mortem rate for patients with suspected CJD has declined, in line with general
autopsy rates in the UK, it remains high (around 60%). The decline is reflected in the reduced
number of brain specimens examined in the neuropathology laboratory; sporadic CJD numbers
being 52 in 2003, 32 in 2004 and 32 in 2005.
In 1990-2005 mortality rates from sporadic CJD in England, Wales, Scotland and Northern
Ireland were, respectively, 0.89, 1.01, 0.88 and 0.49/million/year. The differences between these
rates are not statistically significant (p>0.5). The variation in the observed mortality rates between
the different regions within the UK is not statistically significant (p>0.3). The highest and lowest
mortality rates from sporadic CJD were observed in the South West (SMR=131) and Northern
Ireland (SMR=67). The mortality rates from sporadic CJD in the UK are comparable to those
observed in most other European countries and elsewhere in the world, including countries that
are free of BSE.
Up to 31 December 2005, there were 153 deaths from definite or probable variant CJD (vCJD) in
the UK. Of these, 110 were confirmed by neuropathology. A further 6 probable cases were alive
as at 31st December 2005. The clinical, neuropathological and epidemiological features of these
cases of vCJD are remarkably uniform and consistent with our previous descriptions. Risk factors
for the development of vCJD include age, residence in the UK and methionine homozygosity at
Section 1
T
Fourteenth Annual Report 2005 4
codon 129 of the prion protein gene - all 139 cases (87%) of vCJD with available genetic analysis
have been methionine homozygotes. The incidence of vCJD is higher in the north of the UK
than in the south. Analysis of the incidence of vCJD onsets and deaths from January 1994 to
December 2005 indicates that a peak has been passed. While this is an encouraging finding, the
incidence of vCJD may increase again, particularly if different genetic subgroups are found but
with longer incubation periods. The identification of disease-related PrP in the spleen of a blood
recipient of PRNP-129 MVgenotype is not inconsistent with such an hypothesis. In addition, this
case along with the report of the prevalence of abnormal PrP in the large study of appendix and
tonsil tissues suggests a possibility of a greater number of preclinical or subclinical cases in the
population than might be indicated by the present numbers of confirmed clinical cases.
The only statistically significant geographic cluster of vCJD cases in the UK was in Leicestershire.
All geographically associated cases of vCJD are considered for investigation according to a
protocol which involves the NCJDSU, colleagues at the HPA, HPS and local public health
physicians.
The activities of the NCJDSU are strengthened by collaboration in other surveillance projects,
including the Transfusion Medicine Epidemiology Review and the study of Progressive
Intellectual and Neurological Deterioration in Children. The collaboration of our colleagues in
these projects is greatly appreciated; the effectiveness of this collaboration allowed the
identification in 2003 of a case of variant CJD associated with blood transfusion and the
identification in 2004 of PrPres in the spleen of a recipient of blood donated by someone
incubating vCJD. In early 2006 a further case of variant CJD associated with blood transfusion
was identified.
The success of the National CJD Surveillance Project continues to depend on the extraordinary
level of co-operation from the neuroscience community and other medical and paramedical staff
throughout the UK. We are particularly grateful to the relatives of patients for their help with this
study.
snip...full text some 40 pages or so ;
http://www.cjd.ed.ac.uk/report14.pdf
CONTINUED
FOURTEENTH ANNUAL REPORT 2005
CREUTZFELDT-JAKOB DISEASE
SURVEILLANCE IN THE UK
SUMMARY
The national surveillance programme for Creutzfeldt-Jakob disease (CJD) in the UK was
initiated in May 1990. In 1999, the National CJD Surveillance Unit (NCJDSU) became a
WHO Collaborative Centre for Reference and Research on the surveillance and epidemiology
of human transmissible spongiform encephalopathies (TSEs). In September 2001 the
National Care Team was formed, which currently comprises a care coordinator and a secretary. It
is based within the NCJDSU and was formed in response to concerns regarding the care of CJD
patients.
The information provided in this fourteenth report continues to provide evidence of a high level
of case ascertainment. In last year's report, a decrease in the number of referrals was noted, along
with a drop in the number of recorded sporadic CJD deaths in 2004. This raised concerns about
the completeness of case ascertainment. Further data are now available. While the decline in the
number of referrals has been maintained in 2005, analysis suggests that much, if not all of the
decline is due to changes in the number of referrals who turn out not to be CJD cases. The
number of sporadic cases in 2005 was higher than in 2004 and the data for 2005 may still be
incomplete; thus the present data do not suggest a significant consistent decline in the number of
recorded sporadic CJD deaths (discussed in more detail in Section 2). Detailed clinical and
epidemiological information has been obtained for the great majority of patients. The case-control
study for risk factors of CJD has continued recruitment and initial analysis has been undertaken,
with the results with respect to vCJD published in early 2006. Although there is ongoing evidence
that the post mortem rate for patients with suspected CJD has declined, in line with general
autopsy rates in the UK, it remains high (around 60%). The decline is reflected in the reduced
number of brain specimens examined in the neuropathology laboratory; sporadic CJD numbers
being 52 in 2003, 32 in 2004 and 32 in 2005.
In 1990-2005 mortality rates from sporadic CJD in England, Wales, Scotland and Northern
Ireland were, respectively, 0.89, 1.01, 0.88 and 0.49/million/year. The differences between these
rates are not statistically significant (p>0.5). The variation in the observed mortality rates between
the different regions within the UK is not statistically significant (p>0.3). The highest and lowest
mortality rates from sporadic CJD were observed in the South West (SMR=131) and Northern
Ireland (SMR=67). The mortality rates from sporadic CJD in the UK are comparable to those
observed in most other European countries and elsewhere in the world, including countries that
are free of BSE.
Up to 31 December 2005, there were 153 deaths from definite or probable variant CJD (vCJD) in
the UK. Of these, 110 were confirmed by neuropathology. A further 6 probable cases were alive
as at 31st December 2005. The clinical, neuropathological and epidemiological features of these
cases of vCJD are remarkably uniform and consistent with our previous descriptions. Risk factors
for the development of vCJD include age, residence in the UK and methionine homozygosity at
Section 1
T
Fourteenth Annual Report 2005 4
codon 129 of the prion protein gene - all 139 cases (87%) of vCJD with available genetic analysis
have been methionine homozygotes. The incidence of vCJD is higher in the north of the UK
than in the south. Analysis of the incidence of vCJD onsets and deaths from January 1994 to
December 2005 indicates that a peak has been passed. While this is an encouraging finding, the
incidence of vCJD may increase again, particularly if different genetic subgroups are found but
with longer incubation periods. The identification of disease-related PrP in the spleen of a blood
recipient of PRNP-129 MVgenotype is not inconsistent with such an hypothesis. In addition, this
case along with the report of the prevalence of abnormal PrP in the large study of appendix and
tonsil tissues suggests a possibility of a greater number of preclinical or subclinical cases in the
population than might be indicated by the present numbers of confirmed clinical cases.
The only statistically significant geographic cluster of vCJD cases in the UK was in Leicestershire.
All geographically associated cases of vCJD are considered for investigation according to a
protocol which involves the NCJDSU, colleagues at the HPA, HPS and local public health
physicians.
The activities of the NCJDSU are strengthened by collaboration in other surveillance projects,
including the Transfusion Medicine Epidemiology Review and the study of Progressive
Intellectual and Neurological Deterioration in Children. The collaboration of our colleagues in
these projects is greatly appreciated; the effectiveness of this collaboration allowed the
identification in 2003 of a case of variant CJD associated with blood transfusion and the
identification in 2004 of PrPres in the spleen of a recipient of blood donated by someone
incubating vCJD. In early 2006 a further case of variant CJD associated with blood transfusion
was identified.
The success of the National CJD Surveillance Project continues to depend on the extraordinary
level of co-operation from the neuroscience community and other medical and paramedical staff
throughout the UK. We are particularly grateful to the relatives of patients for their help with this
study.
snip...full text some 40 pages or so ;
http://www.cjd.ed.ac.uk/report14.pdf
CONTINUED