##################### Bovine Spongiform Encephalopathy #####################
Subject: Recent death may be Creutzfeldt-Jakob Disease Fort Collins Colorado
Date: January 28, 2006 at 7:53 am PST
Fort Collins Coloradoan
Article published Jan 27, 2006
Recent death may be Creutzfeldt-Jakob
Biopsy will tell if brain-wasting disease is to blame
By KEVIN DARST
[email protected]
The brain of a Fort Collins woman who died earlier this week will be tested
for Creutzfeldt-Jakob disease, a rare brain-wasting disease, a state
official confirmed.
Nancy Banks, 69, died Monday, just two months after suffering stroke-like
symptoms that her neurologist later determined to be CJD, according to her
obituary.
Colorado has had 39 cases of CJD since the state began monitoring the
disease in 1998 - an average of 4.9 cases a year, said John Pape, the state
epidemiologist. Banks is the second person to be tested this year for the
disease.
"We've had a couple listed as CJD on the death certificate but turn out not
to be (CJD) at the autopsy," Pape said.
An autopsy or brain biopsy is the only way to confirm a CJD diagnosis,
though not all the cases counted by the state had autopsies, Pape said.
Tests also can identify the type of CJD: sporadic, hereditary, acquired or
variant.
There is no treatment or cure for the disease, and about 90 percent of
people with the disease die within a year, according to the National
Institute of Health's National Institute of Neurological Disorders and
Stroke. CJD causes memory failures, behavioral changes, coordination and
vision problems, according to the institute. It can also cause mental
deterioration, involuntary movement, blindness, weakness and coma as it
advances.
The average person stricken with classic CJD is 68 years old and lives four
to five months after symptoms set in, according to the U.S. Centers for
Disease Control and Prevention.
CJD belongs to a group of diseases called transmissible spongiform
encephalopathies that includes chronic wasting disease in deer and elk,
scrapie in sheep and bovine spongiform encephalopathy, or mad cow disease,
in cattle. The diseases are caused by abnormal proteins called prions.
About 93 percent of the 1,133 CJD cases in the U.S. since 1997 have been
sporadic or hereditary, according to a Coloradoan analysis of CJD
surveillance by the National Prion Disease Pathology Surveillance Center at
Case Western Reserve University in Cleveland.
"About 90 percent of CJD cases are sporadic," said Dr. Patrick Bosque, a
neurologist and prion researcher at Denver Health Medical Center. "The one
concern in Colorado is, could it be related to deer?"
Just one person in the U.S. has died from vCJD, and that person likely
acquired the disease in the United Kingdom, according to the surveillance
center. Colorado never has had a verified vCJD case, Pape said.
Nearly 160 people worldwide, most from the United Kingdom, are known to have
contracted the variant type of the disease after they ate beef from cattle
with mad cow disease.
Banks, born in Denver, spent nearly two decades as head of the Library and
Media Center of Riffenburgh Elementary School before moving to the LINC
Library, a summer reading program of the Fort Collins Library and the Poudre
School District, according to the obituary. She also started a library at
First Christian Church.
Samples collected from Banks will be sent to the National Prion Disease
Pathology Surveillance Center. It could be several months before results are
available, Pape said.
Pape declined to say who was collecting the samples from Banks. He said it's
difficult to find coroners or physicians willing to do autopsies on
potential CJD victims because there's a small risk of catching the disease
if infected material comes in contact with an open cut or orifice.
"There's some concern with autopsies and contamination and exposure," Pape
said. "A lot of coroners and hospitals absolutely will not do them."
Though the disease is rare, Pape said he believes awareness is "heightened"
among the neurologists he's talked to in the state.
"There are some hallmarks that can clue in a neurologist," Pape said.
When contacted by the Coloradoan, Banks' daughter, Afton Rorvik, said it was
too difficult at this time to talk about her mother's illness and death.
http://www.coloradoan.com/apps/pbcs.dll/articleAID=/20060127/NEWS01/601270315/1002
Nancy Banks
Born in Denver, CO
Departed on Jan. 23, 2006 and resided in Fort Collins, CO.
Memorial Service: Saturday Jan. 28, 2006
Cemetery: Private
Please click on the links above for locations, times, maps, and directions.
Knitting. Nancy Banks knitted in the dark, at meetings, on the telephone, during movies. As she drove down the highway in her silver Honda civic, her thumbs and forefingers sometimes twitched up and down, as if she were impatient to get back to whatever important project she was creating. Yarn and cloth bags containing various knitting projects could be found all over her house.
Born in 1936, in post-depression Denver, Colorado, Nancy's mother taught her the rudiments of knitting at the age of five. Like most other things, Nancy approached knitting with energy, confidence, and a totally unique, self-taught approach. Sweaters for children, decorated blankets for married couples and grandchildren, baby booties and hats, crazy striped socks, scarves, and Christmas ornaments poured off her needles in a constant stream.
Nancy also understood and loved the written word. At her high school, the Kent school for girls, she edited the yearbook, and went on to Smith College, where she majored in German literature (after a brief flirtation with physics!) Her love of books led her to a Masters of Library Science from the University of Denver in 1973, and she headed the Library and Media Center of Riffenburgh Elementary School shortly after graduating.
In addition to repairing audio-visual equipment, and keeping the large collection of books, materials and computer equipment in order, Nancy understood her role as a caregiver, and a mentor. She loved making the library a safe, loving place for children. She brought her huge stuffed Gorilla named "George" to her story hour for them to hold, held programming classes, and helped a group of interested young students start a "Tolkien" group, exploring the world and mythologies of the author of "The Lord of the Rings."
After retiring from Riffenburgh almost 10 years ago, Nancy went to work on the LINC program, a summer reading program that meshed resources and personnel from both the Fort Collins Public Library and the Poudre R1 School District. Her years in the schools helped her create a program that enabled families to come to events hosted at their local school library over the summer, and encouraged them and their children to read. The LINC programs, offered by members of the Fort Collins community, often followed her own broad interests: opera, dog-sledding, physics, and of course knitting.
During the time she could spare from the LINC program, Nancy traveled widely. Her favorite international places included Germany (where she had spent a year studying in 1958), England, Ireland and Wales (where she vividly remembered hearing of the September 11 tragedy.) She also regularly visited her close friends and family all over the United States, especially prioritizing spending time with her six grandchildren. Equally at home in the car or the airport, she only became anxious when airport security once confiscated her knitting needles, apparently deciding that this tall, striking woman in her 60's needed disarming.
Nancy's faith was strong, and she worked hard to help others. She established a library at First Christian Church, and a cassette tape ministry that made sermons available to those who weren't physically able to attend services. She also loved singing in the choir, occasionally playing violin in the church orchestra. After moving her church home to First Presbyterian, she kept singing, adding her strong alto voice and determined energy to the choir. She also began visiting people on behalf of the church, in hospitals and in their homes. Her bible study, and her work there as a deacon were exceptionally important to her, and her strength and kindness helped her make lasting friendships wherever she went.
In late November of 2005, Nancy was admitted to Albany Medical Center, having shown stroke-like symptoms while visiting one of her sons for Thanksgiving. After some analysis, the neurologists determined that she had Cruetzfeldt-Jakob's disease, an extremely rare neurodegenerative disease which is fatal within months of the onset of symptoms.
In spite of her diagnosis, Nancy continued energetically adapting to the burdens of the disease, remaining open and in good spirits through the end. In the three months of her illness, she was constantly surrounded by her family and her extensive community of friends. As we all understand the depth of her love and involvement with so many people, it's clear that her largest knitting project is now complete – a beautiful tapestry of people connected with one another. She must be very pleased.
Nancy is preceded in death by her parents, Thompson George Marsh and Susan Raymond Marsh, and by her ex-husband Loy Banks.
She is survived by her three sisters, Alice Abbot, Lucy Marsh, and Mary Zulack, her three children, William Banks, Afton Rorvik, and Joseph Banks, and her 6 grandchildren, Ian Banks, Karl Rorvik, Stephen Banks, Robert Banks, Annalisa Rorvik, and Mark Banks.
http://obit.allnutt.com/obit_display.cgi?id=282112&listing=Current
GUESTBOOK
My heartfelt sympathy to the family of Nancy. Although I did not know her I especially feel your loss today because my 48 year old brother also passed from CJD 11 years ago. That was the 1st time I had heard of the disease. I too share a love of knitting. My prayers are with you all.
Jana Skradski Jan 27, 2006 Fort Collins, CO
Fort Collins, CO
City
Population 2000: 118,652
Metro area: Fort Collins-Loveland
Colorado — Population: 4,301,261 ; 24th, 12/00
According to http://www.50states.com/colorado.htm
question;
did she consume venison?
did she have surgery, where others that have consumed venison, that might have been exposed to CWD had surgery?
did she donate blood?
how many have been exposed via the surgical/medical arena by CWD in this area?
PAGE 22
IN CONFIDENCE
http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
Subject: Prions in Skeletal Muscles of Deer with Chronic Wasting Disease [SCIENCE FULL TEXT]
Date: January 26, 2006 at 12:23 pm PST
Prions in Skeletal Muscles of Deer with Chronic Wasting Disease
Rachel C. Angers,1* Shawn R. Browning,1*† Tanya S. Seward,2 Christina J. Sigurdson,4‡ Michael W. Miller,5 Edward A. Hoover,4 Glenn C. Telling1,2,3§
1Department of Microbiology, Immunology and Molecular Genetics, 2Sanders Brown Center on Aging, 3Department of Neurology, University of Kentucky, Lexington, KY 40536, USA. 4Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, CO 80523, USA. 5Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO 80526, USA.
*These authors contributed equally to this work.
†Present address: Department of Infectology, Scripps Research Institute, 5353 Parkside Drive, RF-2, Jupiter, Florida, 33458, USA.
‡Present address: Institute of Neuropathology, University of Zurich, Schmelzbergstrasse 12, 8091 Zurich, Switzerland.
§To whom correspondence should be addressed: E-mail:
[email protected]
Prions are transmissible proteinaceous agents of mammals that cause fatal neurodegenerative diseases of the central nervous system (CNS). The presence of infectivity in skeletal muscle of experimentally infected mice raised the possibility that dietary exposure to prions might occur through meat consumption (1). Chronic wasting disease (CWD), an enigmatic and contagious prion disease of North American cervids, is of particular concern. The emergence of CWD in an increasingly wide geographic area and the interspecies transmission of bovine spongiform encephalopathy (BSE) to humans as variant Creutzfeldt Jakob disease (vCJD) have raised concerns about zoonotic transmission of CWD.
To test whether skeletal muscle of diseased cervids contained prion infectivity, Tg(CerPrP)1536 mice (2) expressing cervid prion protein (CerPrP), were inoculated intracerebrally with extracts prepared from the semitendinosus/semimembranosus muscle group of CWD-affected mule deer or from CWD-negative deer. The availability of CNS materials also afforded direct comparisons of prion infectivity in skeletal muscle and brain. All skeletal muscle extracts from CWD-affected deer induced progressive neurological dysfunction in Tg(CerPrP)1536 mice with mean incubation times ranging between 360 and ~490 d, whereas the incubation times of prions from the CNS ranged from ~230 to 280 d (Table 1). For each inoculation group, the diagnosis of prion disease was confirmed by the presence of PrPSc in the brains of multiple infected Tg(CerPrP)1536 mice (see supporting online material for examples). In contrast, skeletal muscle and brain material from CWD-negative deer failed to induce disease in Tg(CerPrP)1536 mice (Table 1) and PrPSc was not detected in the brains of sacrificed asymptomatic mice as late as 523 d after inoculation (supporting online material).
Our results show that skeletal muscle as well as CNS tissue of deer with CWD contains infectious prions. Similar analyses of skeletal muscle BSE-affected cattle did not reveal high levels of prion infectivity (3). It will be important to assess the cellular location of PrPSc in muscle. Notably, while PrPSc has been detected in muscles of scrapie-affected sheep (4), previous studies failed to detect PrPSc by immunohistochemical analysis of skeletal muscle from deer with natural or experimental CWD (5, 6). Since the time of disease onset is inversely proportional to prion dose (7), the longer incubation times of prions from skeletal muscle extracts compared to matched brain samples indicated that prion titers were lower in muscle than in CNS where infectivity titers are known to reach high levels. Although possible effects of CWD strains or strain mixtures on these incubation times cannot be excluded, the variable 360 to ~490 d incubation times suggested a range of prion titers in skeletal muscles of CWD-affected deer. Muscle prion titers at the high end of the range produced the fastest incubation times that were ~30% longer than the incubation times of prions from the CNS of the same animal. Since all mice in each inoculation group developed disease, prion titers in muscle samples producing the longest incubation times were higher than the end point of the bioassay, defined as the infectious dose at which half the inoculated mice develop disease. Studies are in progress to accurately assess prion titers.
While the risk of exposure to CWD infectivity following consumption of prions in muscle is mitigated by relatively inefficient prion transmission via the oral route (8), these
results show that semitendinosus/semimembranosus muscle, which is likely to be consumed by humans, is a significant source of prion infectivity. Humans consuming or handling meat from CWD-infected deer are therefore at risk to prion exposure.
References and Notes
1. P. J. Bosque et al., Proc. Natl. Acad. Sci. U.S.A. 99, 3812 (2002).
2. S. R. Browning et al., J. Virol. 78, 13345 (2004).
3. A. Buschmann, M. H. Groschup, J. Infect. Dis. 192, 934 (2005).
4. O. Andreoletti et al., Nat. Med. 10, 591 (2004).
5. T. R. Spraker et al., Vet. Pathol. 39, 110 (2002).
6. A. N. Hamir, J. M. Miller, R. C. Cutlip, Vet. Pathol. 41, 78 (2004).
7. S. B. Prusiner et al., Biochemistry 21, 4883 (1980).
8. M. Prinz et al., Am. J. Pathol. 162, 1103 (2003).
9. This work was supported by grants from the U.S. Public Health Service 2RO1 NS040334-04 from the National Institute of Neurological Disorders and Stroke and N01-AI-25491 from the National Institute of Allergy and Infectious Diseases.
Supporting Online Material
www.sciencemag.org/
HUMAN TSE USA 2005
Animal Prion Diseases Relevant to Humans (unknown types?)
Thu Oct 27, 2005 12:05
71.248.128.109
About Human Prion Diseases /
Animal Prion Diseases Relevant to Humans
Bovine Spongiform Encephalopathy (BSE) is a prion disease of cattle. Since 1986, when BSE was recognized, over 180,000 cattle in the UK have developed the disease, and approximately one to three million are likely to have been infected with the BSE agent, most of which were slaughtered for human consumption before developing signs of the disease. The origin of the first case of BSE is unknown, but the epidemic was caused by the recycling of processed waste parts of cattle, some of which were infected with the BSE agent and given to other cattle in feed. Control measures have resulted in the consistent decline of the epidemic in the UK since 1992. Infected cattle and feed exported from the UK have resulted in smaller epidemics in other European countries, where control measures were applied later.
Compelling evidence indicates that BSE can be transmitted to humans through the consumption of prion contaminated meat. BSE-infected individuals eventually develop vCJD with an incubation time believed to be on average 10 years. As of November 2004, three cases of BSE have been reported in North America. One had been imported to Canada from the UK, one was grown in Canada, and one discovered in the USA but of Canadian origin. There has been only one case of vCJD reported in the USA, but the patient most likely acquired the disease in the United Kingdom. If current control measures intended to protect public and animal health are well enforced, the cattle epidemic should be largely under control and any remaining risk to humans through beef consumption should be very small. (For more details see Smith et al. British Medical Bulletin, 66: 185. 2003.)
Chronic Wasting Disease (CWD) is a prion disease of elk and deer, both free range and in captivity. CWD is endemic in areas of Colorado, Wyoming, and Nebraska, but new foci of this disease have been detected in Nebraska, South Dakota, New Mexico, Wisconsin, Mississippi Kansas, Oklahoma, Minnesota, Montana, and Canada. Since there are an estimated 22 million elk and deer in the USA and a large number of hunters who consume elk and deer meat, there is the possibility that CWD can be transmitted from elk and deer to humans. As of November 2004, the NPDPSC has examined 26 hunters with a suspected prion disease. However, all of them appeared to have either typical sporadic or familial forms of the disease. The NPDPSC coordinates with the Centers for Disease Control and state health departments to monitor cases from CWD-endemic areas. Furthermore, it is doing experimental research on CWD transmissibility using animal models. (For details see Sigurdson et al. British Medical Bulletin. 66: 199. 2003 and Belay et al. Emerging Infectious Diseases. 10(6): 977. 2004.)
http://www.cjdsurveillance.com/abouthpd-animal.html
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2004. SPORADIC CJD CASES TRIPLED, and that is with a human TSE surveillance system that is terrible flawed. in 1997 cases of the _reported_ cases of cjd were at 54, to 163 _reported_ cases in 2004. see stats here;
p.s. please note the 47 PENDING CASES to Sept. 2005
p.s. please note the 2005 Prion D. total 120(8) 8=includes 51 type pending, 1 TYPE UNKNOWN ???
p.s. please note sporadic CJD 2002(1) 1=3 TYPE UNKNOWN???
p.s. please note 2004 prion disease (6) 6=7 TYPE UNKNOWN???
http://www.cjdsurveillance.com/resources-casereport.html
CWD TO HUMANS = sCJD ???
AS implied in the Inset 25 we must not _ASSUME_ that
transmission of BSE to other species will invariably
present pathology typical of a scrapie-like disease.
snip...
http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
ATYPICAL TSEs in USA CATTLE AND SHEEP ?
http://www.bseinquiry.gov.uk/files/sc/seac17/tab03.pdf
WHAT WILL CWDTSE IN HUMAN LOOK LIKE cwdCJD???
TSS
#################### https://lists.aegee.org/bse-l.html ####################
