Tuesday, March 23


The death last week of Ronald Swartz of Denville, NJ is being examined as possibly the third case of CJD, the extremely rare ("one in a million") disease, in a little over a year in an area with a population of 789,000. Plus, the National Prion Disease Pathology Surveillance Center in Cleveland has "reopened the case of a Philadelphia woman who died in 2000 and is included among the cases in a possible cluster of CJD cases tied to southern New Jersey. Carrie Mahan, 29, died from a brain disorder that was never identified but physicians initially suspected of being the nation's first case of variant CJD."

This is showing up on the Washington Times site, which means we'll probably see a fuller Steve Mitchell-bylined story in a day or two - I'll keep you updated.

UPDATE 3/24: Yep, here's the long-form version, Five CJD deaths in north N.J. in 15 months. In reading through it, I'm inferring that the five number here is because that includes confirmed or probable deaths, while the Wash. Times version limited itself to confirmed. But there are some interesting additional tidbits... The case of the Philadelphia woman, Carrie Mahan, "has baffled neurologists, such as Dr. Pierluigi Gambetti, director of the Surveillance Center, because Mahan's condition was never identified conclusively. However, many experts, including Dr. Nicholas Gonatas -- the pathologist who performed the autopsy on Mahan -- thought it was CJD. Now Gambetti plans to determine if newer, more sensitive tests developed since 2000 can detect the presence of prions, the agents thought to be responsible for both CJD and vCJD, in Mahan's brain tissue." Steve Mitchell also notes that "Another factor driving the decision to re-examine Mahan's diagnosis could be the opinions of neurologists who observed the slides of her brain when Gambetti recently presented them anonymously at a neurology meeting. Allen said Gambetti told him most neurologists there had agreed the condition looked like CJD."

And on the other coast, the Seattle Times is noting the trend: "More people are wondering whether their elderly relative might have suffered from CJD -- a degenerative disease of the nervous system -- or its variant, the human form of mad cow disease. And with so little known about the deadly diseases, some are asking if any of those cases might be traced back to bad beef."

Another interesting piece in this one has to do with the meaning of "sporadic CJD." "Labeling traditional CJD 'sporadic' is a way of getting around the fact that no one knows what causes it, said Dr. Laura Manuelidis, a neuropathologist at Yale University. Parting with the prevailing theory, she believes the culprit is more likely some type of infectious agent, not misfolded proteins. 'Spontaneous CJD just means we don't know where the infection is coming from,' she said. There's also evidence that some cases of CJD could be mistakenly tagged Alzheimer's or some other type of dementia. Swiss officials found a CJD rate of three cases per million after improving their tracking system -- triple the previously accepted rate." Just sayin'.

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