Michigan brain-disease deaths 'unusual, disturbing'

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http://www.usatoday.com/usatonline/20020429/4066573s.htm

Michigan brain-disease deaths 'unusual, disturbing'

By Anita Manning

Two young men, ages 26 and 28, died last fall in the same Michigan hospital

of a rare brain disease that occurs mainly in elderly people. The incident,

which raised fears that the human form of mad cow disease, or something

similar, had emerged in the USA, prompted a swift investigation by federal

health officials, but doctors familiar with the cases say there is no

evidence to support that fear. They say autopsies and other tests indicate

the victims died from so-called ''classic'' forms of Creutzfeldt-Jakob

disease (CJD).

While the cases are ''highly unusual and disturbing,'' says University of

Michigan neurologist Norman , the data show that the forms of CJD

suffered by the young men are ones seen previously in older individuals.

CJD occurs at the rate of about 1 person per million per year, almost always

in people over age 60. What doctors feared is that a new form of CJD,

possibly similar to a variant that emerged in the mid-1990s in the United

Kingdom and linked to consumption of mad-cow-infected beef, had struck.

Unlike classic CJD, the new variant, vCJD, strikes mainly young adults. It

has killed more than 100 people. The only known case of vCJD in the USA was

diagnosed recently in a 22-year-old British woman living in Florida, who is

thought to have contracted the disease in England. Mad cow disease has not

been detected in cattle in the USA, but a similar disease in deer and elk is

spreading in the Midwest. Chronic wasting disease (CWD) is fatal to deer and

elk but is not known to cause illness in humans.Lawrence Schoenberger of the

Centers for Disease Control and Prevention in Atlanta says the agency sent

investigators to Michigan in late August, when the victims were still

alive.'

'The key thing here is the two were right together. We were worried that

there was maybe a common exposure, but our investigation revealed that was

not the case.'' The men lived in adjacent counties but did not know each

other, he says. In the rare cases when CJD strikes before age 30, it is

often caused by a hereditary form of the disease, says , and ''tests

are continuing to see if that may be a factor in these cases.''But extensive

family interviews determined that neither man had a family history of

dementia, nor had they eaten venison or elk meat or visited countries where

mad cow disease has been detected.''We feel as comfortable as anyone can

that this is not related to either CWD or (mad cow disease),'' says ,

who treated the patients at the University of Michigan Medical Center in Ann

Arbor. Not everyone is comfortable. ''I discount the statement that these

two young people, dying at the same time in the same hospital in southeast

Michigan, did not eat venison, after living their entire lives in that

state,'' says Stauber of the Center for Media & Democracy and co-author

of Mad Cow USA. He suspects a new American variant of CJD, perhaps related

to chronic wasting disease, may be emerging. ''Any attempt to portray these

CJD deaths as some sort of 'normal' occurrence that has simply, to date,

gone unobserved is absurd,'' Stauber says.

Current estimates of only five cases per billion of CJD in people 30 and

younger may be incorrect, says , who co-wrote a report on the cases

presented this month at a meeting of the American Academy of Neurology.'

'The fact that they both occurred at the same time in a relatively small

population suggests that (CJD in younger people) may be more common than

previously suspected,'' says. Doctors don't expect to see it in young

people, so misdiagnosis may occur. ''Any young individual with progressive

neurologic disease should be considered for CJD.' 'He says the cases also

underscore the need for a national system to seek out and report all cases

of CJD. ''There certainly is the possibility that other cases have been

seen and not diagnosed, or even if diagnosed, not reported.''