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From: TSS ()
Subject: 3, POSSIBLY 4 die from disease similar to mad cow sCJD
Date: July 26, 2005 at 5:24 am PST

3 die from disease similar to mad cow


Rocky Barker

The Idaho Statesman | Edition Date: 07-26-2005
Three Idaho women died this year from Creutzfeldt-Jakob disease that was not connected to eating meat.

Officials are awaiting lab results from the autopsy of a fourth woman from Twin Falls County to determine whether her death was from the same disease or a variant linked to bovine spongiform encephalopathy, or mad cow disease.

CJD is a neurodegenerative disease similar to mad cow disease. But the version the three women had turns up sporadically and is not linked to eating animals stricken with mad cow, said Tom Shanahan, a spokesman for the Idaho Department of Health and Welfare.

The disease hits worldwide at a rate of one per 1 million people. Most victims are older than 50, according to the national Centers for Disease Control and Prevention.

Two of the women were from Twin Falls County and the third from Minidoka, Shanahan said.

Idaho has averaged 1.2 cases annually for the last 20 years and never had more than 3 cases reported in the same year.

The four Idaho women were older than 55.

A variant CJD is linked to eating meat from stricken cattle and can be distinguished in an autopsy. It usually strikes younger people. There has been no case of this variant in the United States, Shanahan said.

Several cases have been documented of people who picked up the disease overseas and moved to the United States.

All of these diseases are carried by prions, an abnormal form of protein. Prions cause folding of normal protein cells in the brain, leading to brain damage.

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

Creutzfeldt-Jakob disease

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;

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


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