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From: TSS ()
##################### Bovine Spongiform Encephalopathy ##################### CJD WATCH MESSAGE BOARD POSTED: 10:05 am EDT October 17, 2005 BOISE, Idaho -- From the moment Joan Kingsford first saw her husband stagger in his welding shop, she wanted two things: His recovery and to know what made him sick. She got neither. Alvin Kingsford, 72, died recently of suspected sporadic Creutzfeldt-Jakob disease, the fatal brain-wasting illness. The disease can only be conclusively diagnosed with an autopsy, which Kingsford declined to allow. State and federal health officials are trying to get to the bottom of nine reported cases of suspected sporadic CJD in Idaho this year. Sporadic, or naturally occurring, CJD differs from the permutation dubbed variant CJD, which is caused by eating mad-cow-tainted beef and has killed at least 180 people in the United Kingdom and continental Europe since the 1990s. "One thing is very clear in Idaho -- the number seems to be higher than the number reported in previous years," said Dr. Ermias Belay, a CJD expert with the federal Centers for Disease Control and Prevention. "So far, the investigations have not found any evidence of any exposure that might be common among the cases." Normally, sporadic CJD only strikes about one person in a million each year, with an average of just 300 cases per year in the United States, or just over one case a year in Idaho. Over the past two decades, the most cases reported in Idaho in a single year has been three. Until this year. Of the nine suspected cases reported so far in 2005, three tested positive for an infectious disease of the nervous system, though more tests are pending to determine if the fatal illness was in fact sporadic CJD. Four apparent victims were buried without autopsies. Two suspected cases tested negative. Still, federal and state health officials are stopping just short of calling the Idaho cases a "cluster," waiting for final test results from the victims who got autopsies. The best tool of investigators to pin down the diagnosis -- the autopsy -- is sometimes hard to get, said Tom Shanahan with the Idaho Department of Health and Welfare. Pathologists are often reluctant to perform the procedures, the cost of an autopsy can be high and some families are reluctant to give their consent, officials say. Joan Kingsford wanted an autopsy done on her husband, but no mortician in the area would agree to handle Alvin's body after his brain cavity had been opened. They feared they would catch the rare disease, Kingsford said. Ultimately, she opted to skip the autopsy and have a traditional funeral service. "A week before he passed away, the funeral homes said they wouldn't take the blood out" if an autopsy was done on him, she said. "They just put some embalming in him and told me I had to have a funeral in three days." CJD is transmitted through a malformed prion found primarily in the brain and spinal fluid of those infected, Belay said. Standard sterilization procedures don't eliminate the risk of infection; instead equipment must be soaked in a chemical solution for more than an hour and then heated, according to the World Health Organization. Mortuary procedures -- including embalming -- can be done safely on intact bodies of CJD victims as long as extra precautions are taken, but the World Health Organization does not recommend embalming patients who have had autopsies. Larry Whitaker, a Beaverton, Ore.-based regional salesman for the embalming chemical and equipment manufacturer Dodge Company, offers workshops to his clients on safe handling of CJD-infected bodies. "When the brain has been removed, it is an extraordinary risk," Whitaker said. "This is one time I think that cremation has to be more than mildly considered." A member of the Mormon Church, Joan Kingsford's church discourages cremation. She was thrown into making a decision about her husband's remains much sooner than she expected. "It was two and a half months before we knew what was wrong with him, and by that time he was in the hospital," she said. "I wish we could have done the autopsy, because I think people need to know about this." "We definitely have a problem in Idaho," she added. http://www.wftv.com/health/5106345/detail.html IN light of Asante/Collinge et al findings that BSE transmission to the -------- 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: Dear Terry, 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. 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. 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. 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) ____________________________________ snip... full text ; http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm Creutzfeldt-Jakob disease THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*, Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger ] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique Dormont*, and Jean-Philippe Deslys* et al, that The agent responsible http://www.pnas.org/cgi/content/full/041490898v1 Characterization of two distinct prion strains http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471 ALL human TSEs must be made reportable Nationally and Internationally, OF ALL AGES...TSS #################### https://lists.aegee.org/bse-l.html ####################
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