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From: TSS ()
Subject: Suspected CJD numbers grow in Idaho, Eighth person dies after being diagnosed with CJD
Date: October 5, 2005 at 7:35 am PST


Suspected CJD numbers grow
Eighth person dies after being diagnosed with CJD
By Sandy Miller
Times-News writer

TWIN FALLS -- A man from southeastern Idaho has died after being diagnosed with Creutzfeldt-Jakob disease.

Alan Kingsford, 72, died Thursday at his home in Niter in Caribou County, according to an obituary in Saturday's Idaho State Journal. He owned a welding and repair business, the obituary said.

He is the eighth person to die since January in Idaho after being diagnosed with CJD.

"We're working with Southeastern District Health." said Tom Shanahan, spokesman for the Idaho Department of Health and Welfare. "It is a report that we're investigating."

However, the family has requested that no autopsy be performed, Shanahan said.

"As with all cases, we'd like an autopsy, but we respect the family's wishes," he said.

Shanahan said the state health department hopes to speak more with Kingsford's family and his neurologist to see if he might have had something in common with the other possible CJD victims.

CJD is a fatal brain-wasting disease carried by prions, an abnormal form of protein in the bloodstream. Prions cause folding of normal protein in the brain, leading to brain damage. Symptoms include dementia and other neurological signs. Its victims usually die within four or five months after onset of the disease, according to the Centers for Disease Control and Prevention.

Normally, there is only one case of CJD per million people. Between 1984 and 2004, Idaho averaged 1.2 cases a year, Shanahan said. He said there was one year during that period when Idaho had three cases.

Of the other seven cases, three of the victims were women from Twin Falls County, one woman was from Minidoka County, one victim was a man from Elmore County, one victim was a woman from Benewah County in northern Idaho and one victim was a woman from Bear Lake County located in the southern corner of Idaho on the Utah border.

Brain tissue of some of the victims has been sent to the National Prion Disease Pathology Surveillance Center at Cleveland's Case Western Reserve University. Shanahan said initial results on two of the women from Twin Falls County and the woman from Benewah County came back positive for a prion disease and state health officials are now waiting for the final results to determine what kind of prion disease it was. They're also waiting for initial results on the Elmore County man.

Autopsies were not done on three of the possible CJD victims, Shanahan said.

Because of their ages -- all of the victims except one were over the age of 60 -- health officials suspect they died of sporadic CJD, and not the variant of CJD that is caused by eating meat from a cow with bovine spongiform encephalopathy -- commonly known as mad cow disease. However, the only way to determine what kind of CJD it was is by testing brain tissue.

"Case Western said over 40 percent of the tissue samples they test come back negative," Shanahan said. "It's a difficult disease to diagnose."

Times-News writer Sandy Miller can be reached at 735-3264 or by e-mail at smiller@magicvalley.com.

Story published at magicvalley.com on Tuesday, October 04, 2005

http://www.magicvalley.com/articles/2005/10/04/news_localstate/news_local_state.2.txt


-------- 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:
"'flounder@wt.net'"

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


AND the new findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic

Creutzfeldt-Jakob disease


http://www.pnas.org/cgi/content/abstract/0305777101v1


Adaptation of the bovine spongiform encephalopathy agent to primates
and comparison with Creutzfeldt- Jakob disease: Implications for
human health

THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*,
Virginie Nouvel*,

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
for French iatrogenic growth hormone-linked CJD taken as a control is
very different from vCJD but is similar to that found in one case of
sporadic CJD and one sheep scrapie isolate;

http://www.pnas.org/cgi/content/full/041490898v1

Characterization of two distinct prion strains
derived from bovine spongiform encephalopathy
transmissions to inbred mice

http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471


ALL animals for human/animal consumption must be tested for TSE.

ALL human TSEs must be made reportable Nationally and Internationally of all ages...TSS




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