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From: TSS ()
CJD WATCH MESSAGE BOARD 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 1 Setember 2006 Summary of vCJD cases Deaths Deaths from definite vCJD (confirmed): 112 Deaths from probable vCJD (without neuropathological confirmation): 44 Deaths from probable vCJD (neuropathological confirmation pending): 0 Number of deaths from definite or probable vCJD (as above): 156 Alive Number of probable vCJD cases still alive: 6 Total number of definite or probable vCJD (dead and alive): 162 The next table will be published on Monday 2nd October 2006 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, 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). 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. http://www.eurocjd.ed.ac.uk/sporadic.htm http://www.eurocjd.ed.ac.uk/genetic.htm Classification of sporadic Creutzfeldt–Jakob disease revisited Ignazio Cali1, Rudolph Castellani3, Jue Yuan1, Amer Al-Shekhlee2, Mark L. Cohen1, Xiangzhu Xiao1, Francisco J. Moleres1, Piero Parchi4, Wen-Quan Zou1 and Pierluigi Gambetti1 http://disc.server.com/discussion.cgi?disc=167318;article=3092;title=CJD%20WATCH Anna Krasnianski1, Walter J. Schulz-Schaeffer2, Kai Kallenberg3, Bettina Meissner1, Donald A. Collie5, Sigrun Roeber4, Mario Bartl1, Uta Heinemann1, Daniela Varges1, Hans A. Kretzschmar4 and Inga Zerr1 http://disc.server.com/discussion.cgi?disc=167318;article=3093;title=CJD%20WATCH S. J. Collins1,*, P. Sanchez-Juan2,*, C. L. Masters1, G. M. Klug1, C. van Duijn2, A. Poleggi3, M. Pocchiari3, S. Almonti3, N. Cuadrado-Corrales4, J. de Pedro-Cuesta4, H. Budka5, E. Gelpi5, M. Glatzel6,13, M. Tolnay6, E. Hewer6, I. Zerr7, U. Heinemann7, H. A. Kretszchmar8, G. H. Jansen9, E. Olsen9, E. Mitrova10, A. Alpérovitch11, J.-P. Brandel11, J. Mackenzie12, K. Murray12 and R. G. Will12 Inherited prion disease with six octapeptide repeat insertional mutation—molecular analysis of phenotypic heterogeneity Simon Mead, Mark Poulter, Jon Beck, Thomas E. F. Webb, Tracy A. Campbell, Jacqueline M. Linehan, Melanie Desbruslais, Susan Joiner, Jonathan D. F. Wadsworth, Andrew King, Peter Lantos and John Collinge http://disc.server.com/discussion.cgi?disc=167318;article=3087;title=CJD%20WATCH Re: 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? Diagnosis and Reporting of Creutzfeldt-Jakob Disease 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 -------- Original Message -------- Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43 -0000 From: "Asante, Emmanuel A" To: "'flounder@wt.net'" [if !supportEmptyParas]--> [endif]--> Dear Terry, [if !supportEmptyParas]--> [endif]--> I have been asked by Professor Collinge to respond to your request. I am a Senior Scientist in the MRC Prion Unit and the lead author on the paper. I have attached a pdf copy of the paper for your attention. Thank you for your interest in the paper. [if !supportEmptyParas]--> [endif]--> In respect of your first question, the simple answer is, yes. As you will find in the paper, we have managed to associate the alternate phenotype to type 2 PrPSc, the commonest sporadic CJD. It is too early to be able to claim any further sub-classification in respect of Heidenhain variant CJD or Vicky Rimmer's version. It will take further studies, which are on-going, to establish if there are sub-types to our initial finding which we are now reporting. The main point of the paper is that, as well as leading to the expected new variant CJD phenotype, BSE transmission to the 129-methionine genotype can lead to an alternate phenotype which is indistinguishable from type 2 PrPSc. [if !supportEmptyParas]--> [endif]--> I hope reading the paper will enlighten you more on the subject. If I can be of any further assistance please to not hesitate to ask. Best wishes. [if !supportEmptyParas]--> [endif]--> Emmanuel Asante <> ____________________________________ Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email: e.asante@ic.ac.uk (until 9/12/02) New e-mail: e.asante@prion.ucl.ac.uk (active from now) ____________________________________ http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
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