US Worried CJD Screening
Misses Thousands Of Cases

From Patricia Doyle, PhD

Hello Jeff: The article attempts to differentiate cases of CJD i.e. v CJD, s CJD and CJD. The bottom line is that CJD whether we call is sporadic or variant is basically the same. A prion which is a protein simply changes shape or misfolds.
The fact of the matter is that many CJD cases are not diagnosed and that is the "meat" of the matter.
ProMED Mail
(The original version of this report frequently confuses variant CJD (associated with transmission of the BSE agent to humans), with the sporadic, iatrogenic, and genetic forms of CJD. I have edited the text to eliminate some of this confusion. There are precise diagnostic criteria for variant CJD, which can be viewed at the UK Department of Health website: -- ProMed)
US Concern CJD Screening May Miss Thousands Of Cases
By Steve Mitchell, United Press International (via COMTEX) 7-23-3
The UK Department of Health website maintains the only comprehensive source of comparative data on the incidence of the various forms of CJD in any population. The UK Department of Health monthly reports are reproduced in ProMED-mail close to the beginning of each month (see references below). - Mod.CP]
The federal government's monitoring system for cases of Creutzfeldt-Jakob disease (CJD), a fatal human brain illness, could be missing tens of thousands of victims, scientists and consumer advocates have told United Press International.
Variant CJD [abbreviated as CJD (new var.) or vCJD in ProMED-mail] can be [contracted] by eating beef [from cattle] with mad cow disease (bovine spongiform encephalopathy - abbreviated as BSE), but the critics assert that, without a better tracking system, it might be impossible to determine whether any [cases of sporadic CJD] are [cases of vCJD] or to obtain an accurate picture of the prevalence of the disorder in the United States.
Beginning in the late 1990s, more than 100 people contracted vCJD in the United Kingdom and several European countries after eating beef infected with BSE. [The mode of transmission of the BSE agent to humans has not been established conclusively, but is presumed on circumstantial grounds to be a consequence of consumption of contaminated meat. - Mod.CP]
No case of [BSE] has ever been detected in U.S. cattle, and the monitoring system of the Centers for Disease Control and Prevention (CDC) has never detected a case of vCJD . Nevertheless, critics say, the CDC's system [may] miss many cases of the disease, which currently is not treatable and is always fatal.
The first symptoms of CJD typically include memory loss and difficulty keeping balance and walking. As the disease destroys the brain, patients rapidly progress in a matter of months to difficulty with movement, an inability to talk and swallow and, finally, death. Spontaneously-occurring or sporadic CJD is a rare disorder. Only about 300 cases appear nationwide each year, but several studies have suggested that the disorder might be more common than thought and that as many as tens of thousands of cases might be going unrecognized. Clusters of [sporadic] CJD have been reported in various areas of the United States -- Pennsylvania in 1993, Florida in 1994, Oregon in 1996, New York in 1999-2000, and Texas in 1996. In addition, several people in New Jersey developed CJD in recent years, including a 56-year-old woman who died on 31 May 2003. Although in some instances, a [BSE] link was suspected, all of the cases ultimately were classified as sporadic CJD.
People who develop CJD [presumably as a result of] eating BSE-contaminated beef have been thought to develop the specific form of the disorder called variant CJD. But new research, released in December 2002 [see ProMED-mail post archived as: CJD (new var.) - UK: update 2003 (03) 20030204.0299], [suggests] that the [BSE] pathogen [may] cause both sporadic CJD and the variant form. "Now people are beginning to realize that because something looks like sporadic CJD they can't necessarily conclude that it is not linked to [BSE]," said Laura Manuelidis, section chief of surgery in the neuropathology department at Yale University, who conducted a 1989 study that found 13 percent of Alzheimer's patients actually had CJD.
Several studies, including the Manuelidis study, have found that autopsies reveal 3 to 13 percent of patients diagnosed with Alzheimer's or dementia actually suffered from CJD. Those numbers might sound low, but there are 4 million Alzheimer's cases and hundreds of thousands of dementia cases in the United States. A small percentage of those cases could add up to 120 000 or more CJD victims going undetected and not included in official statistics.
Experiences in [the UK] and Switzerland -- both countries that discovered mad cow disease in their cattle -- have heightened concerns about the possibility that some cases of sporadic CJD are due to consuming mad-cow-tainted beef. Both countries have reported increases in sporadic CJD since BSE was first detected in British herds in 1986. Switzerland discovered in 2002 that its CJD rate was twice that of any other country in the world. Switzerland had been seeing 8 to 11 cases per year from 1997 to 2000. Then the incidence more than doubled, to 19 cases in 2001 and 18 cases in 2002.
The CDC says the annual rate of CJD in the United States is one case per million people, but the above studies suggest the true prevalence of CJD is not known, Manuelidis told UPI. Diagnosing CJD or Alzheimer's is difficult because no test exists that can identify either disease in a living patient with certainty. So physicians must rely on the patient's symptoms to determine which illness might be present. Sometimes, however, the symptoms of one disease can appear similar to the other. The only way to determine the disease conclusively is to perform an autopsy on the brain after death. Unfortunately, although autopsies once were performed on approximately half of all corpses, the frequency has dropped to 15 percent or less in the United States. The National Center for Health Statistics (NCHS) -- a branch of the CDC -- stopped collecting autopsy data in 1995. "If we don't do autopsies and we don't look at people's brains ... we have no idea about the general prevalence of these kinds of infections and (whether) it is changing," Manuelidis said.
While autopsies have been declining, the number of deaths attributed to Alzheimer's has increased more than 50-fold since 1979, going from 857 deaths then to nearly 50 000 in 2000. Though it is unlikely the dramatic increase in Alzheimer's is due entirely to misdiagnosed CJD cases, it "could explain some of the increase we've seen," Manuelidis said.
"Neurodegenerative disease and Alzheimer's disease have become a waste-basket" for mental illness in the elderly that is difficult to diagnose conclusively, she said. "In other words, what people call Alzheimer's now is [broader] than what people used to call it, and that has the possibility of encompassing more diseases -- including CJD." The autopsy studies that found undiagnosed CJD cases raise the question of whether the United States "already has an undetected epidemic here," Jeff Nelson, director of, a vegetarian advocacy web-site, told UPI. "What's the source of that?" Nelson asked. "Could it be the same source of encephalopathy we saw in minks?"
Nelson referred to an outbreak of a [transmissible spongiform encephalopathy] in minks in Wisconsin in the 1980s. The origin was traced back to the animals' diet, which included parts of so-called downer cattle -- sick cows that are unable to stand, which often indicates a neurological disease, including mad cow. The mink disease raised concerns about whether U.S. cattle were carrying a mad-cow-like pathogen even prior to the U.K. epidemic that began in 1986.
Andrew Monjan, chief of the neuropsychology of aging program at the National Institute of Aging (part of the National Institutes of Health in Bethesda, Maryland), acknowledged there has been an increase in U.S. Alzheimer's cases. However, he told UPI, this probably is due to the aging of the population -- as people grow older, they develop a higher risk of developing Alzheimer's. "There's been no change in the number of CJD cases in the country, and there has been clearly a tracking of the unusual cases of CJD" that could be due to mad cow disease, Monjan said.
However, Terry Singletary, coordinator of CJD Watch -- an organization founded to track CJD cases -- says efforts to track the disease have been close to nonexistent. For example, only 12 states require such reports. Therefore, many cases might be going undetected, unreported, or misdiagnosed. If more states made CJD a reportable illness, there would be more clusters detected across the United States, said Singletary, who became involved with CJD advocacy after his mother died from a form of CJD known as Heidenhain variant.
In the 18-year period between 1979 and 1996, he noted, the country saw a jump from one case of sporadic CJD in people under the age of 30 -- a warning sign for a link to [BSE], because nearly all of the U.K. victims were 30 years of age or younger, to 5 cases in 5 years between 1997 and 2001. "That represents a substantial blip," he told UPI. Singletary also said there have been increases in sporadic CJD in France, Germany, and Italy, all of which have detected mad cow disease in their cattle.
So far, the CDC has refused to impose a national requirement that physicians and hospitals report cases of the disease. The agency has not chosen to make CJD a reportable disease because "making it reportable is not necessarily directly helpful in surveillance, because in some states where it's reportable you may not get the physician to report it," said Dr. Ermias Belay, CDC's medical epidemiologist working on CJD. Instead, the agency relies on other methods, including death certificates and urging physicians to send suspicious cases to the National Prion Disease Pathology Surveillance Center (NPDPSC) at Case Western Reserve University in Cleveland, which is funded by the CDC.
However, because autopsies generally are not done, if a CJD case is misdiagnosed as Alzheimer's or dementia, a correct diagnosis might never be made, and therefore the cause of death listed on a death certificate might be inaccurate. Belay told UPI he discounted this possibility. It is unlikely to happen, he said, because it is easy to distinguish CJD from Alzheimer's -- the 2 conditions display different symptoms.
Manuelidis disagreed. It can be quite difficult to determine accurately whether a patient has CJD, as evidenced by her study, in which respected and competent neurologists and psychiatrists at Yale originally diagnosed patients with Alzheimer's, yet were wrong at least 13 percent of the time. Another study conducted at the University of Pennsylvania, which found 6 percent of dementia patients actually were suffering from CJD, supports the difficulty in distinguishing the illnesses correctly. The U. Penn. researchers concluded that: "These results show that in patients with a clinical diagnosis of dementia, the etiology cannot be accurately predicted during life."
In addition, the NPDPSC sees less than half of all the CJD cases each year, so the CDC's investigational system not only is missing many of the misdiagnosed CJD cases, it also is not conducting autopsies on most of the detected cases. Belay said that the CDC follows up on all cases of CJD that occur in people under age 55, as these could be linked to variant (BSE-related) CJD. But so far, all have turned out to be sporadic forms of the disease. About 30 cases of the disorder occur each year in the United States in this age group, while the remaining 270 or so are older.
The case of a Philadelphia woman who developed a brain disorder that appeared to be CJD and died from it in 2000 at the age of 29 -- illustrates just how difficult it can be to diagnose the disease. Her physician, Dr. Peter Crinos of the University of Pennsylvania Medical Center, ruled out other disorders and felt certain the young woman had died of CJD, a concern that raised the possibility of a link to mad cow disease because of her young age. When neuropathologist Nicholas Gonatas, who had seen CJD before, examined the woman's brain after her death, he, likewise, was confident he detected the microscopic, sponge-like holes caused by the disease. But when he sent brain samples to the NPDPSC, the results came back negative. Gonatas, convinced the surveillance center's finding was erroneous, sent off 2 more samples, only to have them both come back negative. Subsequent research, however, has shown the test used by the surveillance center cannot rule out CJD, said Crinos, an assistant professor of neurology. "There's no question that the young woman had a spongiform encephalopathy," Crinos said, but added although it appeared to be CJD, it is difficult if not impossible to say whether it was due to mad cow disease.
Crinos told UPI until the CDC implements a better tracking system, a lot of questions will remain about CJD and cases like the young Philadelphia woman's. One central question: Why are cases of what is presumed to be a rare disease popping up in clusters in certain areas of the country? Crinos said the clustering suggests an environmental or food-borne cause, but so far, "No one knows the answer to that."
ProMED Mail
[The above article raises some significant issues related to CJD surveillance in the USA, and factors that may be contributing to a significant under-reporting of CJD cases. First and foremost, CJD is not a nationally reportable disease. Even with nationally reportable diseases, one sees significant under-reporting, probably related to the health care delivery system in the USA, which is heavily weighted towards private-sector providers. Unfortunately the private sector is less consistent with disease reporting to state and federal levels. In the absence of a national mandate for reporting of a disease, the incentive becomes negligible, so the traditional "tip of the iceberg" seen in disease reporting shrinks even further.
The study suggesting that up to 13 percent of cases diagnosed with Alzheimer's and other dementias may be due to CJD points out another weak link in CJD surveillance. The diagnosis does necessitate an autopsy, and autopsy rates have declined markedly in the USA, especially in the elderly. Hence the "Catch 22" -- what is needed for the diagnosis is an autopsy, but the autopsy isn't performed (as has been the case in many of the CJD "cluster" reports). The reverse of "seek and ye shall find" is "don't look and the problem isn't there." - Mod.MPP]
Patricia A. Doyle, PhD Please visit my "Emerging Diseases" message board at:
&Board=emergingdiseases Zhan le Devlesa tai sastimasa Go with God and in Good Health



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