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From: TSS ()
-------------------------------------------------------------------------------- Dave Wilkins Idaho health officials this week ruled out Creutzfeldt-Jakob Disease as the cause of death of one woman, but are still investigating four other possible cases. Since January, five women 60 years of age and older have died after being diagnosed with the rare neurodegenerative disorder. Autopsies were performed on three of the women and brain tissue samples submitted to a national laboratory. Prelminary results have come back for two of the three. One confirmed that the woman died of a prion disease, while the other was negative, health officials said this week. Four of the women lived in Twin Falls County, and one lived in nearby Minidoka County. A man in Elmore County has also been diagnosed with the disease and is still alive, health officials said this week. To have five suspected cases of CJD in one rural area in such a short time is unusual. On average, Idaho has had only about one CJD death per year. “The most we have ever had in the whole state is three in any year going back to 1984,” said Tom Shanahan, a spokesman for the Idaho Department of Health and Welfare. Authorities became concerned when the number of diagnosed cases in the Magic Valley began rising this year, Shanahan said. The state health department has launched an investigation, including interviews with doctors, examination of medical records and a survey of victims’ families. The survey includes questions about lifestyle and diet – whether the victims ate beef, chicken, pork or wild game – for instance. “We’ve also looked at their occupations and environmental factors,” Shanahan said. “We’re compiling all that in a database to see if there are any similarities.” So far, however, “nothing has jumped out,” he said. CJD progresses rapidly and is always fatal. An examination of brain tissue is the only way to confirm the presence of CJD. Officials are awaiting final test results from Case Western Reserve University in Cleveland. They won’t know until they get them back whether the deaths were indeed from CJD. For now, authorities believe the likely cause of death was a classic form of CJD that occurs sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions. Health officials said there’s little chance the deaths could be linked to variant CJD, which is related to BSE or “mad cow” disease. There has never been a confirmed case of variant CJD in the United States. In England, which has had several cases, the victims have been much younger – about 27 to 28 years of age – on average. “If it turns out to be CJD, we think it probably will be the sporadic form,” Shanahan said of the Idaho cases. Sporadic CJD strikes about one in every 1 million people. About 280 cases are reported in the United States every year. There have been other clusters of sporadic CJD cases elsewhere in the country without any definitive causes, Shanahan said. “They have never been able to point at something specific that has caused it,” he said. “I don’t know whether we’re going to find any answers.” Dave Wilkins is based in Twin Falls, Idaho. His email address is dwilkins@capitalpress.com. >>>>For now, authorities believe the likely cause of death was a classic form of CJD that occurs sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions<<<<< -------- 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
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