|
||||||||||||||||||
 |
From: TSS ()
Monday 8 January 2007 10:16 Monthly Creutzfeldt Jakob Disease statistics Definite and probable CJD cases in the UK: As at 5 January 2007 Summary of vCJD cases Deaths Alive 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). * As at 5th January 2007 Total number of definite or probable vCJD (dead and alive): 165 Notes to editor ANNEX I II III IV Client ref 2007/0005 GNN ref 142643P https://www.gnn.gov.uk/Content/Detail.asp?ReleaseID=254733&NewsAreaID=2 Volume 12, Number 12–December 2006 On the Question of Sporadic or Atypical Bovine SpongiformEncephalopathy and Creutzfeldt-Jakob Disease Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§ http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm?s_cid=eid06_0965_e 3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain 6:30 Close of Day One There is a growing number of human CJD cases, and they were presented last He estimates that it may be up to 14 or 15 persons which display selectively MARCH 26, 2003 RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc? Singeltary, Sr et al. JAMA.2001; 285: 733-734. http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=dignosing+and+reporting+creutzfeldt+jakob+disease&searchid=1048865596978_1528&stored_search=&FIRSTINDEX=0&journalcode=jama Atypical features of dementia in a patient with Creutzfeldt-Jakob disease Andrzej Urbanik4ABDE, Grażyna Bierzyńska-Macyszyn5BDE, Paweł Właszczuk5BDE 1 Institute of Psychology, University of Gdańsk, Gdańsk, Poland 2 Department of Neurology, Ministerial Hospital, Ministry of Internal Affairs and Administration, Cracow, Poland 3 Eye Surgery Clinic, Specialized Ophthalmological Hospital no. 5, Katowice, Poland 4 Department of Radiology, Collegium Medicum, Jagiellonian University, Cracow, Poland 5 Histopathology Section, Department of Morphology, Medical University of Silesia, Katowice, Poland Summary Background: This article describes a Polish patient (female, right-handed, age 68 at onset) diagnosed with the Heidenhain variant of Creutzfeldt-Jakob Disease (HvCJD), characterized clinically by isolated visual disturbances with no ocular dysfunction prior to the development of myoclonus and other symptoms of CJD. Case Report: Nothing in the history pointed to iatrogenic or acquired CJD, and genetic testing ruled out familial CJD. The neuroradiological picture (MRI) showed non-specifi c features of cerebral atrophy (cortical and subcortical). An EEG revealed periodic triphasic sharp waves, particularly in the occipital lobes, and myoclonus occurring synchronically with generalized periodic epileptiform discharges. Comprehensive neuropsychological testing documented rapidly progressive dementia, with dysgraphia and aphasia deteriorating to organic mutism. Post-mortem neuropathological examination confi rmed spongiform encephalopathy, especially in occipital cortex, with amyloid plaques but without neurofi brillary tangles. Conclusions: Over the crucial 6-week period the patient went from “Mild Cognitive Impairment” to a status resembling the fi nal stages of Alzheimer’s disease, without any evidence of a CVA. The only aspect of this case that does not fi t the usual criteria for the Heidenhain variant is the fact that the patient survived over a year in a persistent vegetative state. Ophthalmologists and family physicians should be aware of the possibility of HvCJD in any patient over 60 presenting with otherwise inexplicable visual disturbances in the absence of signifi cant ocular pathology, even when other symptoms of dementia may not be immediately noticeable. snip... CONCLUSIONS In the case reported here, a clinical diagnosis of the Heidenhain variant of Creuzfeldt-Jakob Disease, initially suggested by the early and prominent presentation of visual symptoms (ranging from microopsia and macropsia to visual hallucinations), and backed by EEG and neuroadiological fi ndings, was con- fi rmed by neuropathological results. Neuropsychological testing documented the characteristically rapid course of the disease. The course of development of clinical symptoms and their relationship to the ophthalmological and neuroradiological picture are of considerable theoretical interest. Ophthalmologists and family physicians should be aware of the possibility of HvCJD in any patient over 60 presenting with otherwise inexplicable visual disturbances in the absence of signifi cant ocular pathology, even when other symptoms of dementia may not be immediately noticeable. ... http://www.medscimonit.com/pub/vol_13/no_1/9923.pdf 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 Reporting CJD cases to public health departments - Guidance Document. - (updated November 2006) http://www.cjd.ed.ac.uk/guidance.htm CURRENT DATA (DECEMBER 2006) http://www.cjd.ed.ac.uk/vcjdworld.htm http://www.cjd.ed.ac.uk/TREAT.htm http://www.eurocjd.ed.ac.uk/sporadic.htm http://www.eurocjd.ed.ac.uk/genetic.htm http://www.eurocjd.ed.ac.uk/nvcjd.html [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf THE SEVEN SCIENTIST REPORT *** http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000490-vol40.pdf SEROLOGICALS CORPORATION http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-c000383-01-vol35.pdf Embassy of Japan
|
