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Two cases, one fatal, of suspected Creutzfeldt-Jakob disease (CJD) in Marin County, California - fatal case confirmed as sporadic CJD
Two Marin residents were recently diagnosed with Creutzfeldt-Jakob disease and one of them has died due to this exceedingly rare, fatal illness sometimes mistakenly referred to as mad cow disease.
I believe the first article has made a mistake. The second article indicates that the fatal case is ordinary CJD, not the variant CJD (vCJD) that is linked to mad cow disease.
Tests are likely still pending on the second case.
SAN RAFAEL, Calif. ?
Marin County resident recently died of a rare brain illness known as Creutzfeldt-Jakob disease, but it is not the form of the disease commonly called mad cow disease, a county health official said Thursday.
"In the last 24 hours, we received information from a lab analysis of tissue samples from the deceased that rules out mad cow disease," Dr. Craig Lindquist, Marin County's interim public health officer, said Thursday morning.
"It appears to be a rare, one-in-a-million form of Creutzfeldt-Jakob disease. It's not from eating beef. It's not contagious or spread by intimate contact or transmissible by common contact," Lindquist said.
A Marin County physician recently notified the California Department of Public Health of two suspected cases of Creutzfeldt-Jakob disease, Lindquist said. State public health officials then notified the Marin County Division of Public Health Services on Friday, Lindquist said.
The other Marin County resident with the suspected case is still alive, and a definitive diagnosis is not possible, Lindquist said.
"We have to examine the brain tissue post-mortem," Lindquist said
[snip]
It is not related to a variant form of Creutzfeldt-Jakob disease known as "mad cow disease", or bovine spongiform encephalopathy (BSE), a progressive neurological disorder of cattle that become infected with a transmissible prion in the meat and bone meal they are fed, according to the CDC. Humans, in turn, contract the disease by eating the meat of infected cattle.
BSE spread among cattle in Great Britain and peaked with almost 1,000 cases a week in 1993, according to the CDCP. Through the end of 2010, more than 145,500 cases were confirmed among more than 35,000 herds.
Lindquist said there is no link between the two Creutzfeldt-Jakob cases in Marin County, and it is not clear which form of the disease is afflicting the living victim.
Re: Two cases, one fatal, of suspected Creutzfeldt-Jakob disease (CJD) - fatal case confirmed as sporadic CJD
Apparently, here's the source of the confusion. The fatal case had sufficient exposure to be a suspected vCJD case having lived in London, but actual testing on the prion has apparently ruled it out:
On Monday, the Birmingham Mail in England reported that variant Creutzfeldt-Jakob disease had recently claimed the lives of eight people in the West Midlands, a metropolitan county in western central England. The newspaper reported concern that the disease may be incubating longer than expected and that there might be a second epidemic of variant Creutzfeldt-Jakob disease. According to the U.S. Centers for Disease Control and Prevention in Atlanta, the incubation period for variant Creutzfeldt-Jakob disease is unknown because it is a new disease; however, it is likely that the incubation period will be measured in terms of many years or decades.
No similar epidemic of variant Creutzfeldt-Jakob disease has occurred in the United States. Only three cases of variant Creutzfeldt-Jakob disease have ever been reported in the United States, and all three victims are believed to have been exposed to BSE while living outside the country.
McAllister said he and his wife lived in London from 1989 to 1992
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