CJD FOUNDATION UPDATE
I am sure this has probably already been sent through, considering the CJD Foundation members whom are on the cjdvoice list, but thought if they had not already sent it, i thought others on CJDVoice might like to read it. sorry if it's a repeat. ...tss
The CJD Foundation Newsletter
SEPTEMBER 2008 VOLUME 1, ISSUE 3
The CJD Foundation's largest ongoing project is our toll-free HelpLine (1-800-659-1991) for any family who needs support about a loved one's suspected CJD diagnosis, or any individual who has questions about prion diseases. Below you will find HelpLine statistics for January 1, 2008 - August 31, 2008. Please keep in mind that the CJD Foundation is not a reporting agency and families are not required to report their loved one's illness or death to us. These statistics are not intended to be scientific in nature, but instead to validate the work we do on a daily basis.
Note 1: Not all new cases and deaths reported are confirmed by autopsy.
Note 2: Total HelpLine contacts include phone calls and emails from families, medical professionals and others..
MONTH NEW CASES REPORTED DEATHS REPORTED TOTAL HL CONTACTS UNIQUE WEBSITE VISITORS
January 35 24 203 6,424
February 24 9 212 6,848
March 19 19 164 7,492
April 36 14 231 8,427
May 26 20 191 8,839
June 19 17 144 9,646
July 28 14 171 7,791
August 27 16 150 5,323
TOTALS = 214 133 1,466 60,790
P.O. Box 5312, Akron, Ohio 44334 ?? 330.665.5590 ?? HelpLine 1.800.659.1991 ?? firstname.lastname@example.org www.cjdfoundation.org
CJDF Questionnaire Update
With a generous grant from the Homer Family Foundation, we were recently able to hire an epidemiologist to review our questionnaire and data collection methods. Through a collaboration of efforts with Pierluigi Gambetti, MD, our Medical Director and Director of the National Prion Disease Pathology Surveillance Center, Lawrence Schonberger, MD, Assistant Director of Public Health, Centers for Disease Control and Prevention, our epidemiologist, Steven Korzeniewski, MSc, MA, and CJDF members Tracie Kedzierski, Marisa Boarman and Florence Kranitz, we were able to refine our questionnaire to better capture and track this valuable information. All of the information shared in the questionnaire is confidential. We use it to obtain an overview of case histories, look for possible trends or similarities in patient backgrounds and to offer each family who is willing to share their story a safe and meaningful way to do so. We never use names without the permission of the family. At the present time, we are the only repository for anecdotal patient information in the United States. Please help us by completing our questionnaire. You may find it helpful to fill it out with other family members and/or friends who were close to the patient in order to obtain the most accurate information possible. Also, having the patient's medical records on hand may assist you in answering the questions as accurately as possible. Although you may not be able to answer all of the questions, we truly appreciate your help. You may receive a follow-up call from a volunteer if we need clarification on any of your responses. If you are interested in completing a questionnaire, please contact us at email@example.com or 1-800-659-1991. We greatly appreciate your help with this important project!
CJD QUESTIONNAIRE HISTORY
Conference Video The following link will take you to the NeuroPrion website and the video presentations from CJD 2008 and the Sixth Annual CJD Foundation Family Conference: NeuroPrion Website
HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008
Tissue infectivity and strain typing of the many variants of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...
A New Prionopathy OR more of the same old BSe and sporadic CJD
Communicated by: Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]
There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.
He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.
sporadic Fatal Familial Insomnia
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
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  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?
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.
Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT
2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.
Creutzfeldt Jakob Disease
USA PRION UNIT BLOG
Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008
Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.
see full text ;
CJD TEXAS (cjd clusters)
USA WRITTEN CJD QUESTIONNAIRE ???
The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
Tuesday, August 19, 2008
Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate
Wednesday, August 20, 2008
Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?
August 20, 2008
Wednesday, October 08, 2008
Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
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