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From: TSS ()
TSS Health officials investigate unusual incidence of Creutzfeldt-Jakob Disease THE ASSOCIATED PRESS TWIN FALLS, Idaho -- Five southern Idaho women - four of whom have already died - have been diagnosed with Creutzfeldt-Jakob Disease since January, health officials say. None of the cases appeared to be the variant type of the fatal disease, which is usually linked to mad cow disease. Instead, health officials said, the women may have all developed the sporadic form of CJD, where the disease appears even though the person has no known risk factors. The women - four in Twin Falls County, one in nearby Minidoka County - were all between the ages of 60 and 83. "We do not think that there is any reason for the community to be alarmed about these cases," said Cheryle Becker, an epidemiologist at South Central District Health. "In our investigation so far, we do not see any common source of contagion between the women. We don't think there is any reason for people to change their eating, drinking or living habits. We will continue to investigate and inform the public of what we find." Still, the number of cases in one small region is unusually high. Normally the disease infects only one out of every million people worldwide. In Idaho, there are normally about three cases of Creutzfeldt-Jakob Disease a year, according to the district health office. In recent years the entire United States has reported fewer than 300 cases annually, according to the Centers for Disease Control and Prevention. The fatal, neurodegenerative disease causes normal brain proteins to fold, leading to brain damage. Symptoms include dementia and lack of coordination; most people die within one year of onset. Laboratory tests that investigators hope will provide more clues are pending on tissue from two of the women. "We need to find out if there's some situation that needs our attention," Becker said. "This may be five cases of sporadic CJD that just happened to occur in the same period of time." The average age of people with the sporadic form of the disease is 68. The variant form usually affects younger people, with an average age of 28, according to the CDC. In 2002, one case of variant CJD was diagnosed in a Florida resident who had lived in the United Kingdom. http://seattlepi.nwsource.com/local/aplocal_story.asp?category=6420&slug=ID%20Creutzfeldt%20Jakob%20Cluster http://www.kgw.com/sharedcontent/APStories/stories/D8BKTQC87.html -------- 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 kindest regards, http://members.aol.com/larmstr853/cjdvoice/cjdvoice.htm (where you can speak your mind and get some answers) REFUSE ALL OVER THE PHONE ONLY CJD QUESTIONNAIRES from anyone. DEMAND THE CJD QUESTIONNAIRE BE IN WRITING WITH COPY GOING TO FAMILY! nothing less is acceptable. WE cannot let the federal gov hide and conceal this data like they do data on BSE mad cow cases. IF we do, we will never get any answers to our questions about our loved ones...TSS
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