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From: TSS ()
-------- Original Message -------- March 2, 2005, 12:58AM ANGLETON - When Jones Creek housewife LaNeita Means started doing ADVERTISEMENT Her final diagnosis was much more sinister. She had Creutzfeldt-Jakob For more than a year and a half, her family watched helplessly as she "We couldn't do anything," said her daughter, Sherry Means, 26. "Nobody It's a bad disease that earned an even more evil reputation during the Although humans can get a variant type of Creutzfeldt-Jakob disease from "Nobody knows how she got it," Sherry Means said. Indeed, researchers don't know why or how 85 percent of the sufferers of Only about one person in a million contracts Creutzfeldt-Jakob disease, An outbreak of mad cow disease in cattle in the United Kingdom in the That case was in 2002, and the victim was a 22-year-old woman in Florida No cases in humans or cattle have been reported in Texas, Taylor said. Creutzfeldt-Jakob, named for two German researchers who first diagnosed It isn't spread through close contact with infected individuals, said It can take years for the symptoms to show up, Kranitz said. Most of the LaNeita Means hung on longer than most. So did her family. "I hate to say this, but I even thought of divorcing her," said her She started acting so strange in the fall of 2003 that she no longer She became fearful and accused her family of hurting her and taking and "I am so glad I didn't divorce her," he said softly. "Really, I thought They went to many local doctors. Nobody could find anything wrong. When no doctors could find anything physically wrong with her, her But the doctors there said she didn't respond to any of the normal Finally, a spinal tap indicated she had a prion disease — most likely "I didn't know what that was," Sherry Means said. "The doctor told me it "He said, 'I have no good news for you. There is no cure.' " The diagnosis couldn't be certain. Only a brain biopsy can show the The Means family moved her to a nursing home in Lake Jackson to await There were some good times when LaNeita Means recognized her family. She She also told her family that she was comforted by someone she called "When she talked about Emily, she would look at a particular spot in the Even when she couldn't see, her eyes would stare at a spot for long By then, she probably felt no pain, Kranitz said. Researchers think Her brain was sent to the National Prion Disease Pathology Surveillance Doctors there confirmed the diagnosis, but Sherry Means said a final "We have some very good research going on," Kranitz said. "But no, Meanwhile, her organization tries to inform and comfort families like Kranitz became involved in the foundation after her husband, Aaron Her group can be reached at www.cjdfoundation.org richard.stewart@chron.com http://www.chron.com/cs/CDA/ssistory.mpl/metropolitan/3064119 > For more than a year and a half, her family watched helplessly as she Cristina Casalone *{dagger} , Gianluigi Zanusso {dagger} {ddagger} , *Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto Edited by Stanley B. Prusiner, University of California, San Francisco, Transmissible spongiform encephalopathies (TSEs), or prion diseases, are ------------------------------------------------------------------------ {dagger} C.C. and G.Z. contributed equally to this work. ||To whom correspondence should be addressed. E-mail: salvatore.monaco@mail.univr.it http://www.pnas.org/cgi/content/abstract/0305777101v1 Sarah E. Lloyd, Jacqueline M. Linehan, Melanie Desbruslais, Susan MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Correspondence Distinct prion strains can be distinguished by differences in incubation http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471 Corinne Ida Lasmézas*,dagger * Commissariat à l'Energie Atomique, Service de Neurovirologie, Edited by D. Carleton Gajdusek, Centre National de la Recherche Top Materials and Methods Results There is substantial scientific evidence to support the notion that snip... Conclusions Materials and Methods Results From BSE and vCJD transmissions in nonhuman primates, a number of The risk assessment of biological products of human origin, notably snip... full text; http://www.pnas.org/cgi/content/full/041490898v1 Under strict embargo until 19.00 British Time Thursday 11 November 2004 GENETIC MAKE-UP MAY DETERMINE WHAT TYPE OF CJD OCCURS WHEN HUMANS ARE INFECTED WITH BSE New research published today (19.00 hours Thursday 11th November) by a team from the Medical Research Council (MRC) Prion Unit offers an explanation about why only people with a particular genetic make-up have so far developed vCJD. It also provides evidence that other types of BSE-derived prion infection with a different pattern of symptoms might occur in humans. The findings are published in the journal Science. Variant CJD (vCJD) is the human disease thought to be caused by eating food contaminated with the infectious agent, known as a prion, responsible for the epidemic of BSE or mad cow disease in cattle. So far, everyone known to have developed vCJD has been of a particular genetic type known as MM. Until now it has been a mystery why everyone that has developed vCJD is of the MM type and one possibility is that they are simply the first to develop the disease when infected with BSE, and that people with the other genetic types1 (known as VV and MV) infected with BSE prions will also develop vCJD, but some years later. In a series of experiments spanning more than ten years, the MRC team has been studying mice genetically modified so that they make human prion proteins which are used to model human susceptibility to BSE. The team has now shown that mice with the human VV genetic type do become infected when given BSE or vCJD prions, but manifest a different form of the disease which looks quite different to vCJD and has a novel prion strain type. Remarkably, when these novel prions were used to infect mice of the MM genetic type, the mice either developed a disease very like vCJD, or else a pattern of disease that looks like so-called sporadic CJD the classical form of CJD. This form has been known about for many years, is seen all over the world and has not hitherto been associated with BSE. However, the new strain identified in the mice, being called type 5, has not been seen yet in people and we do not know what pattern of disease it would cause. It could look like one of the forms of classical or sporadic CJD or perhaps be yet another different variant form. The work from the MRC team suggests that type 4 prions, the type associated with vCJD, can only propagate themselves in people that make the M form of the protein. It seems the V form of the protein just cannot adopt the particular molecular shape that characterises type 4. The studies in mice also suggest that if these prions were to pass from person to person (for example by blood transfusion) then, depending on the genetic type of the person becoming infected, at least three different patterns of disease might result: type 2 (which is seen in sporadic CJD); type 4 (which causes vCJD) or type 5 (which may cause a new pattern of disease). Professor John Collinge, Director of the MRC Prion Unit, which is based at University College London, said: These mouse studies give us vital clues about the behaviour of prions and how they appear to modify and adapt depending on the genetic makeup of the individual they are infecting. We always have to be cautious about making direct comparison to the human condition, but our work strongly suggests that we can not assume only those with one genetic profile are vulnerable to BSE infection. At this stage it is not possible to say how this should alter estimates of those likely to become ill, but our findings do suggest we should be taking steps to draw up a more sophisticated system of categorizing the disease so that we dont mistake BSE related infection for a version of sporadic CJD. ENDS For more information call the MRC press office on 020 7 637 6011 Notes to Editors 1The human prion protein comes in two common forms, known as M and V. Because everyone has two copies of this gene, there are three possible genetic types: MM, MV and VV. Paper - Human Prion protein v129 prevent expression of vCJD phenotype Science On line 11.11.04 Prions are rogue forms of one of the bodys own proteins known as the prion protein which are misshapen. There are several different rogue or misshapen forms that can infect humans, and these different types of prions are known as strains . This is analogous to different strains of other germs such flu virus causing influenza or strains of salmonella causing different forms of food poisoning for example. The strain of prion causing vCJD is known as type 4, types 1-3 cause the different forms of sporadic or classical CJD. Each strain causes a different pattern or type of disease. It is known that prion strains can change or mutate when they pass between different animals. The Medical Research Council (MRC) is a national organisation funded by the UK tax-payer. Its business is medical research aimed at improving human health; everyone stands to benefit from the outputs. The research it supports and the scientists it trains meet the needs of the health services, the pharmaceutical and other health-related industries and the academic world. MRC has funded work which has led to some of the most significant discoveries and achievements in medicine in the UK. About half of the MRCs expenditure of £430 million is invested in its 40 Institutes, Units and Centres. The remaining half goes in the form of grant support and training awards to individuals and teams in universities and medical schools. Web site at: http://www.mrc.ac.uk . Send Post-Publication Peer Review to journal: Re: RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States Email Terry S. Singeltary: flounder@wt.net 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? http://www.neurology.org/cgi/eletters/60/2/176#535 Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION] http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt I SAY AGAIN, until the CJD Foundation acknowledges the https://web01.aphis.usda.gov/regpublic.nsf/0/eff9eff1f7c5cf2b87256ecf000df08d?OpenDocument Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt Docket Management Docket: 02N-0273 - Substances Prohibited From Use in Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed Comment Number: EC -10 Accepted - Volume 2 PART 2 PDF]Freas, William TSS SUBMISSION File Format: PDF/Adobe Acrobat - Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary Sr. [flounder@wt.net] Monday, January 08,200l 3:03 PM freas ... http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf Asante/Collinge et al, 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; http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt # Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of Docket Management Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food Facilities, Section 305 http://www.fda.gov/ohrms/dockets/dockets/02n0276/02N-0276-EC-254.htm OTC External Analgesic Drug Products, ... EMC 7, Terry S. Singeltary Sr. www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm DOCKETS ENTERED on 2/5/03. ... EMC 4 Terry S. Singeltary Sr. Vol#: 2. 03N-0009 Federal Preemption of State & Local Medical Device Requireme. ... Docket: 02N-0370 - Neurological Devices; Classification of Human Dura Mater Comment Number: EC -1 Accepted - Volume 1 ... 00D-1662 Use of Xenotransplantation Products in Humans. http://www.fda.gov/ohrms/dockets/dailys/03/Jun03/060903/060903.htm 2003D-0186 01N-0423 Substances Prohibited from use in animal food/Feed Ruminant APE 5 National Renderers Association, Inc. Vol#: 2 APE 6 Animal Protein Producers Industry Vol#: 2 APE 7 Darling International Inc. Vol#: 2 EMC 1 Terry S. Singeltary Sr. Vol#: 3 http://www.fda.gov/ohrms/dockets/dailys/01/Oct01/101501/101501.htm Send Post-Publication Peer Review to journal: 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? LANCET INFECTIOUS DISEASE JOURNAL My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem. largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation. occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally , he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source. region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle. transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive. is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticised for not having performed enough prionic tests in farm deer and elk. issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE. teeth , Singeltary argues. You get it when they want you to have it, and only what they want you to have. University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings ; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure . The USA should develop a system modelled on that established in the UK, he points out. Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunterstwo of whom were friendswho died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans ; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further . Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison. the [Samii] cases because there is no evidence that the men ate CWD-infected meat . He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited . Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source. <<< actually, that quote was from a more recent article in the Journal of Neurology (see below), not the JAMA article... Full Text 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 BRITISH MEDICAL JOURNAL SOMETHING TO CHEW ON BMJ http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2 BMJ http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1 THE PATHOLOGICAL PROTEIN BY Philip Yam Yam Philip Yam News Editor Scientific American www.sciam.com 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 ALL animals for human/animal consumption must be tested for TSE. ALL human TSEs must be made reportable Nationally and Internationally...TSS Terry S. Singeltary Sr. P.O. Box 42 Bacliff, TEXAS USA 77518 CJD VOICE http://members.aol.com/larmstr853/cjdvoice/cjdvoice.htm CJD VOICE MESSAGE BOARD http://disc.server.com/Indices/7498.html ######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html ##########
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