7 died of CJD after eating beef at Garden State Race Track

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> Philadelphia Inquirer | 01/07/2004 | In N.J., a link to mad cow?Posted on> Wed, Jan. 07, 2004>> In N.J., a link to mad cow?> http://www.philly.com/mld/inquirer/news/local/7648379.htm>> Seven who apparently died of a similar illness are getting new attention.>> By Faye Flam> Inquirer Staff Writer>> 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 CherryHill.> 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.> 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.> 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.> Health experts are skeptical of the contentions of Skarbek. If a link were> established between the deaths and the victims' diets, it would be thefirst> time beef has been linked to sporadic CJD, they say.> 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.> 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.> 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.> Experts say the two diseases leave different patterns of destruction inthe> brain, which can be detected by microscopic examination at autopsy.> Skarbek said she first became interested when an acquaintance, CarrieMahan,> 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.> 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.> She said she contacted the CDC and the U.S. Agriculture Department aftershe> 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.> Tom Skinner, a spokesman for the CDC, said the federal agency and the New> Jersey State Health Department looked into the cluster. The cases, hesaid,> all had sporadic CJD, the brain disease that is not believed linked to> eating infected beef.> Skarbek said she did not hear back from CDC for several months, not untila> 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 numberof> questions. Schonberger could not be reached for comment yesterday.> Scientists who study sporadic CJD and mad cow disease say one recent study> in mice does suggest that mad cow disease might manifest itself assomething> that looks like sporadic CJD. John Collinge of Imperial College in London> used genetically modified mice that he infected with mad cow prions. Someof> the mice, he reported, developed a disease that looked like mad cowdisease,> others a malady that looked more like sporadic CJD.> "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.> "It's a tiny cluster - I don't know if it's real," Manuelides said, butshe> thought the case warranted a thorough investigation from the CDC.> Investigators should try to find out all they can about these sevenvictims,> their medical histories, their eating habits, and where meat served at the> racetrack came from, she said.> "Everything is possible unless they investigate and really go into it,"> Manuelides said.> Experts say that if mad cow disease was causing some cases of sporadicCJD,> 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.> "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 ifit> were indeed on the rise from consumption of infected cows.> He said there was a cluster of sporadic CJD cases in Allentown severalyears> 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 precludethe> chance that a few will show up in the same place.> 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 communityhas> 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.>>> Contact staff writer Faye Flam at 215-854-4977 or fflam.>