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From: TSS ()
Subject: Rare disease (cjd) hits close to home Douglas County resident
Date: August 11, 2005 at 11:24 am PST

Rare disease hits close to home
Douglas County resident has been diagnosed with deadly brain disorder

Thursday, August 11, 2005

Gale Armbrister, a longtime Douglas County resident, has been diagnosed with sporadic Creutzfeldt-Jakob disease, a deadly brain disorder that strikes about one person in a million and is often confused with mad cow disease.

“It has some symptoms that are similar to mad cow, but it’s very inaccurate to call it mad cow,” said Armbrister’s son, Matt.

“This has been going on for several weeks,” Armbrister said. “My father is declining.”

Gale Armbrister, 60, was moved to a Kansas City-area nursing home earlier this week.

“He’s still functional,” said Matt Armbrister, noting that Creutzfeldt-Jakob disease is terminal. There is no cure.

“We’ve been told it could be up to six months,” he said.

Gale Armbrister retired in February 1999 from Hallmark Cards in Lawrence, where he had been a supervisor for more than 25 years. His wife, Cheri, has been a nurse at Lawrence Memorial Hospital for more than 30 years.

Gale Armbrister has been involved in American Legion baseball and other youth activities for many years in Lawrence and Baldwin. He recently began substitute teaching in Baldwin.

Creutzfeldt-Jakob disease — best known by its acronym, CJD — is part of a group of rare brain disorders known as prion diseases that affect humans and animals.

“It’s not something you contract,” Matt Armbrister said. “It’s not a virus. It’s a malformed protein in the brain.”

According to the Ohio-based CJD Foundation, CJD strikes roughly one out of a million people.

“But that number is deceptive,” said CJD Foundation President Florence Kranitz, “because CJD is often underreported and underdiagnosed, and it doesn’t affect infants, children or young adults — so for adults over 55, we think the number is closer to one in 9,000.”

Matt Armbrister said his father’s condition was diagnosed early because his neurologist, Dr. Sanjeev Kumar, had encountered a case earlier in his practice.

“Many neurologists won’t see a case of CJD in their entire careers,” Matt Armbrister said. Kumar, he said, consulted neurologists at Kansas University Medical Center and at Mayo Clinic.

Armbrister asked well-wishers to respect the family’s privacy.

“This is a stressful time for us right now,” he said. “We appreciate everybody’s thoughts and prayers, but my family is not is a position to be taking a bunch of phone calls or having a bunch of people coming by.”

The family decided to share their story with the public because they wanted to raise awareness of the malady.

The couple have another son, Jay, 29. He lives in Baldwin.

Dale Armbrister’s mother, Ethel Armbrister, brother, Jackson Armbrister, and sister, Patty Johnston, live in Lawrence.

>>>said CJD Foundation President Florence Kranitz, “because CJD is often underreported and underdiagnosed, and it doesn’t affect infants, children or young adults — so for adults over 55, we think the number is closer to one in 9,000<<<

also looking on the cjd foundation site;

Sporadic CJD

Sporadic CJD is subdivided into five subtypes, some of which

have different ages at onset, duration and clinical presentation.

The typical subtype, which accounts for over 50% of all prion

diseases, generally presents at 60-65 years.The incidence of

sporadic CJD in the U.S is around 1 case per million population,

that is, around 300 new cases every year.

The incidence of sporadic CJD does not seem to be significantly

higher in countries where BSE and scrapie are common than it is in

countries free of these diseases.Therefore, based on the present

evidence, a link between animal prion diseases and

sporadic CJD is very unlikely.

well, i would dispute this. we have documented young in CJD in the USA, and the oldest documented to date

on the globe to die from nvCJD is 74 years old. so _any_ age limit on surveillance of any human TSE is not acceptable.

especially if not for only the sporadic cjds and the medical/surgical aspect of this nightmare.

scrapie, cwd rampant in USA, BSE in cattle, all of which transmits to primates, no transmission studies done on man, atypical TSE in cattle in the study done from US suffolk sheep to US cattle, and all the potential routes from all those products therof?

just from cjd watch unofficial data base we have very young dying in North America

from sporadic CJD;

20 year old died from sCJD in USA in 1980 and a 16 year
old in 1981. A 19 year old died from sCJD in
France in 1985. There is no evidence of an iatrogenic
cause for those cases....

Human health implications

14. Epidemiological data on possible CWD infection of humans are very limited.

The possibility that clinical symptoms of CWD in humans differ from those of Creutzfeldt-Jakob Disease (CJD)

cannot be excluded. There is no significant difference between the prevalence of CJD in CWD endemic areas and

other areas of the world. However, because CJD surveillance in the USA is relatively recent, not all CJD cases

may have been identified. Additionally, detection of a small increase in prevalence of such a rare disease is very

difficult. Investigation of six cases of prion disease in young people (< 30 years of age) in the USA found no definite

causal link with consumption of venison from known CWD endemic areas. The disease characteristics in these

cases were indistinguishable from sporadic CJD or Gerstmann-Sträussler-Scheinker syndrome. Likewise, in a

study of three hunters (> 54 years of age) diagnosed with sporadic CJD, no link with consumption of venison f

rom CWD endemic areas was found. No causal link was found in an investigation of three men with neurological

illnesses who were known to partake in “wild game feasts”. Only one of these subjects was found to have a prion

disease and this was also indistinguishable from sporadic CJD.


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.


NOT to forget James Alford whom is still alive, 26 years of age i believe with some strain of TSE, on PPS treatment.

I find it disturbing the real existance of sporadic CJD is still unknown in the USA. kinda like the rest of the surveillance

efforts of the USA for human and animal TSEs. In the latest update that I have from the USA National Prion Disease

Pathology Surveillance Center, the cases examined for sporadic CJD have increased from 54 in 1997 to 163 in 2004,

this increase documented with minimal input of cjd being made. CJD is in the process of being made reportable in only

34 states, with only 21 states agreeing to have cjd reportable. (2004 State Health Department Progress per NPDPSC)

sporadic CJD is also on the rise in other documented BSE countries; IN fact, many documented BSE countries have seen anincrease in sporadic CJD; Mouse model sheds new light on human prion disease snip... Professor John Collinge said "We are not saying that all or even most casesof sporadic CJD are as a result of BSE exposure, but some more recent casesmay be - the incidence of sporadic CJD has shown an upward trend in the UKover the last decade. While most of this apparent increase may be becausedoctors are now more aware of CJD and better at diagnosing it, seriousconsideration should be given to a proportion of this rise beingBSE-related. Switzerland, which has had a substantial BSE epidemic, hasnoted a sharp recent increase in sporadic CJD.snip...
ALSO, DRAMATIC INCREASE IN SPORADIC CJD IN SWITZERLANDMad Cow: Linked to thousands of CJD cases? By Steve MitchellUnited Press InternationalPublished 12/29/2003 9:50 AM snip... > Experiences in England and Switzerland -- two countries that> discovered mad cow disease in their cattle -- have heightened concerns> about the possibility some cases of sporadic CJD are due to consuming> mad-cow-tainted beef. Both countries have reported increases in> sporadic CJD since mad cow was first detected in British herds in 1986.>> Switzerland discovered last year its CJD rate was twice that of any> other country in the world. Switzerland had been seeing about eight to> 11 cases per year from 1997 to 2000. Then the incidence more than> doubled, to 19 cases in 2001 and 18 cases in 2002.>snip... > "There's been no change in the number of CJD cases in the country and> there has been clearly a tracking of the unusual cases of CJD" that> could be due to mad cow disease, Monjan said. However, Terry> Singletary, coordinator of CJD Watch -- an organization founded to> track CJD cases -- says efforts to track the disease have been close> to nonexistent. For example, only 12 states require such reports.> Therefore, many cases might be going undetected, unreported or> misdiagnosed.>> If more states made CJD a reportable illness, there would be more> clusters detected across the United States, said Singletary, who> became involved with CJD advocacy after his mother died from a form of> CJD known as Heidenhain variant. In the 18-year period between 1979> and 1996, he noted, the country saw a jump from one case of sporadic> CJD in people under the age of 30 -- a warning sign for a link to mad> cow because nearly all of the U.K. victims were 30 years of age or> younger -- to five cases in five years between 1997 and 2001. "That> represents a substantial blip," he told UPI.>> Singletary also said there have been increases in sporadic CJD in> France, Germany and Italy, all of which have detected mad cow disease> in their cattle.>
Is sporadic CJD increasing in the UK?In the UK, all cases of suspected CJD are reported to the National CJDSurveillance Unit in Edinburgh. The number of cases of sporadic CJD in theUK has increased since 1970, when figures first started being kept. In1970-71 there were 21 deaths from sporadic CJD and in 2002 there were 67deaths from sporadic CJD. Most of the increase has occurred since 1990 andin the over 70 age group.It is not clear whether this is due to greater awareness of CJD among themedical profession, or whether it represents a genuine increase in theincidence of the disease. from Creutzfeldt-Jakobdisease and related disorders in Europe,Australia, and CanadaA. Ladogana, MD; M. Puopolo, DStat; E.A. Croes, MD, PhD; H. Budka, MD; C.Jarius, MD;S. Collins, MD, FRACP; G.M. Klug, BSc(Hons); T. Sutcliffe, BA; A. Giulivi,MD, FRCP; A. Alperovitch, MD;N. Delasnerie-Laupretre, MD; J.-P. Brandel, MD; S. Poser, MD; H.Kretzschmar, MD, FRCPath;I. Rietveld, MD; E. Mitrova, MD; J. de Pedro Cuesta, MD, PhD; P.Martinez-Martin, MD, PhD;M. Glatzel, MD; A. Aguzzi, MD, PhD; R. Knight, FRCP(Ed); H. Ward, MRCP,FFPH; M. Pocchiari, MD;C.M. van Duijn, PhD; R.G. Will, MD, FRCP; and I. Zerr, MDAbstract-Background: An international study of the epidemiologiccharacteristics of Creutzfeldt-Jakob disease (CJD)was established in 1993 and included national registries in France, Germany,Italy, the Netherlands, Slovakia, and theUnited Kingdom. In 1997, the study was extended to Australia, Austria,Canada, Spain, and Switzerland. Methods: Datawere pooled from all participating countries for the years 1993 to 2002 andincluded deaths from definite or probable CJDof all etiologic subtypes. Results: Four thousand four hundred forty-onecases were available for analysis and included3,720 cases of sporadic CJD, 455 genetic cases, 138 iatrogenic cases, and128 variant cases. The overall annual mortalityrate between 1999 and 2002 was 1.67 per million for all cases and 1.39 permillion for sporadic CJD. Mortality rates weresimilar in all countries. There was heterogeneity in the distribution ofcases by etiologic subtype with an excess of geneticcases in Italy and Slovakia, of iatrogenic cases in France and the UK, andof variant CJD in the UK. Conclusions: Thisstudy has established overall epidemiologic characteristics forCreutzfeldt-Jakob disease (CJD) of all types in a multinationalpopulation-based study. Intercountry comparisons did not suggest anyrelative change in the characteristics ofsporadic CJD in the United Kingdom, and the evidence in this study does notsuggest the occurrence of a novel form ofhuman bovine spongiform encephalopathy infection other than variant CJD.However, this remains a possibility, andcountries currently unaffected by variant CJD may yet have cases.NEUROLOGY 2005;64:1586-1591snip...Overall, there has been an increase in mortalityfrom sCJD with time, and it is not possible to becertain whether this is related to improved efficiencyof the surveillance system with time or some othercause such as a new environmental source of infection.However, the increased mortality is relatedmainly to increasingly mortality rates in the elderly,rather than the younger age groups, and the changesin mortality have been found in all participatingcountries, including Australia and Canada, in whichindigenous BSE exposure is likely to have been negligible. ...May (1 of 2) 2005 NEUROLOGY 64 1591NO URL...TSSPOSITION STATEMENTMATERNAL TRANSMISSION OF vCJDsnip...3Conclusions12. In summary, there is currently no epidemiological evidence formaternal transmission of vCJD, including transmission via breastmilk. However, there is a hypothetical risk. Although availableevidence is limited and mostly indirect rather than direct, this risk, ifany, appears to be low. As a risk cannot be excluded, a watchingbrief should be maintained.SEACJanuary 20052 Tamai Y et al. Demonstration of the transmissible agent in tissue from a pregnant womanwith CJD. New Eng J Med 1992 327, 649.3 Wadsworth et al. Human prion protein with valine 129 prevents expression of variant CJDphenotype. Science. 2004 306, 1793-1796.4 Devereux G et al. Variations in neurodegenerative disease across the UK: findings from thenational study of Progressive Intellectual and Neurological Deterioration (PIND). Arch DisChild. 2004 89, 8-12. say never or has or will not with the present surveillance in the USA of human and animal TSE. ...TSS

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