- Although U.S. health officials say no one has gotten
mad cow disease from American beef, a Cinnaminson woman says seven people
died of a closely related disease after eating at the Garden State Race
Track in Cherry Hill.
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- The seven apparently died of sporadic Creutzfeldt-Jakob
disease, a fatal brain-wasting malady, according to a spokesman for the
Centers for Disease Control and Prevention.
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- Janet Skarbek, an accountant, discovered the cases after
an acquaintance died of the illness in 2000. She contacted the CDC. Now,
it is asking her questions.
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- The human version of mad cow disease is a very similar
condition known as new variant Creutzfeldt-Jakob disease. The differences
between the two can be definitively detected only by examining brain tissue
in an autopsy.
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- Health experts are skeptical of the contentions of Skarbek.
If a link were established between the deaths and the victims' diets, it
would be the first time beef has been linked to sporadic CJD, they say.
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- Several hundred people in the United States die from
sporadic CJD every year. That makes it rare, but not nearly as rare as
the human form of mad cow disease, which has been officially diagnosed
in only 153 people worldwide, including 143 in England, where the disease
spread through several hundred thousand cattle.
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- Both brain diseases cause mental degeneration and coordination
problems, progressing within months to paralysis and death. New variant
CJD - the human form of mad cow disease - tends to cause more psychiatric
problems initially - depression, anxiety or delusions, say experts. People
who get this disease tend to be much younger - teens, 20s and 30s - while
sporadic CJD usually occurs in those over 55.
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- Both diseases are caused by an unusual disease agent
known as a prion, which can apparently lie dormant for a decade or more
before rapidly growing in pockets and turning the brain into a spongelike
mass.
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- Experts say the two diseases leave different patterns
of destruction in the brain, which can be detected by microscopic examination
at autopsy.
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- Skarbek said she first became interested when an acquaintance,
Carrie Mahan, died of sporadic CJD at 29, an age when the disease is exceedingly
rare. The woman started feeling nauseated and within days was hospitalized
with delusions and tremors. Her doctors reported that she seemed to have
either sporadic CJD or the human form of mad cow disease.
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- Skarbek said she started to look for other cases of CJD
in the obituaries and found an 83-year-old man who had frequented the racetrack
where Mahan had worked between 1989 and 1995. Skarbek said she kept searching
for CJD cases and contacting families to see if they had any connection
to the racetrack. She found four more cases among patrons who had frequented
the racetrack restaurant and one 56-year-old woman who had worked at the
track and died from sporadic CJD in 2003. The track closed in 2001.
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- She said she contacted the CDC and the U.S. Agriculture
Department after she found the first four cases. She thought she might
have uncovered evidence that mad cow disease was infecting people but somehow
manifesting itself more like sporadic CJD.
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- Tom Skinner, a spokesman for the CDC, said the federal
agency and the New Jersey State Health Department looked into the cluster.
The cases, he said, all had sporadic CJD, the brain disease that is not
believed linked to eating infected beef.
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- Skarbek said she did not hear back from CDC for several
months, not until a case of mad cow disease surfaced in a Washington state
Holstein, the first such case confirmed in the United States. Then, she
said, she got a call Dec. 31 from epidemiologist Lawrence Schonberger,
who asked her a number of questions. Schonberger could not be reached for
comment yesterday.
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- Scientists who study sporadic CJD and mad cow disease
say one recent study in mice does suggest that mad cow disease might manifest
itself as something that looks like sporadic CJD. John Collinge of Imperial
College in London used genetically modified mice that he infected with
mad cow prions. Some of the mice, he reported, developed a disease that
looked like mad cow disease, others a malady that looked more like sporadic
CJD.
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- "It suggests that [mad cow disease] can sometimes
look like sporadic CJD," said Laura Manuelides, section chief of surgery
in the neuropathology department at Yale.
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- "It's a tiny cluster - I don't know if it's real,"
Manuelides said, but she thought the case warranted a thorough investigation
from the CDC. Investigators should try to find out all they can about these
seven victims, their medical histories, their eating habits, and where
meat served at the racetrack came from, she said.
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- "Everything is possible unless they investigate
and really go into it," Manuelides said.
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- Experts say that if mad cow disease was causing some
cases of sporadic CJD, epidemiologists should see a rise in its incidence.
In England, health officials realized the cow disease had jumped to humans
when they found a small increase in total cases and a significant increase
in the disease among younger people.
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- "In the last 10 years, we haven't seen an upsurge
of CJD cases," said John Trojanowski, a brain researcher at the University
of Pennsylvania. Some studies, including one at Penn, have shown that CJD
cases sometimes get misdiagnosed as Alzheimer's disease, but Trojanowski,
who coauthored that study, said these mistaken diagnoses would not mask
an increase in CJD if it were indeed on the rise from consumption of infected
cows.
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- He said there was a cluster of sporadic CJD cases in
Allentown several years ago but it was determined to be a chance occurrence.
About one person in a million gets sporadic CJD each year, he said, but
that doesn't preclude the chance that a few will show up in the same place.
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- He said he thought the USDA and meat industry should
be doing more testing to protect public health, but he does not believe
the medical community has missed an epidemic of people getting brain disease
from beef. "With 30 Alzheimer's centers around the country, people
pay close attention to demented people," he said.
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- Staff writer Faye Flam fflam@phillynews.com.
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