- (First published 6-16-96)
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- If indeed a form of Bovine Spongiform Encephalopathy
(BSE) exists in the United States, one might expect to see a rise in the
number of cases of Creutzfeldt-Jakob disease (CJD). CJD, however, is not
a reportable illness in this country (Holman, 1995). Because the Centers
for Disease Control (CDC) does not actively monitor the disease (Altman,
1996d) a rise similar to the one in Britain could be missed (Altman, 1996d).
Already, a number of U.S. CJD clusters have been found. In the largest
known U.S. outbreak of sporadic cases to date(Flannery, 1996) a five-fold
expected rate was found to be associated with cheese consumption in Pennsylvania's
Lehigh Valley (Little, 1993) A striking increase in CJD was also reported
in Florida (Berger, 1994) and there is an anecdotal report of an cluster
in Oregon (Boule, 1996). An analysis of death certificates in a number
of states, though, showed an overall stable and typical CJD incidence rate
from 1979 to 1993 (World, 1996). To track the disease, the CDC has just
initiated a four-state study of death certificates (Altman, 1996a), but
since it is considered well known that death-certificate diagnoses are
not always accurate (Davanpour, 1993) the survey may not provide an accurate
assessment.
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- The true prevalence of prion diseases in this or any
other country remains a mystery (Harrison, 1991). Compounding the uncertainty,
autopsies are rarely performed on atypical dementias (Harrison, 1991),
because medical professionals fear infection (Altman, 1996a). The officially
reported rate in this country is less than 1 case in a million people per
year (World, 1996). An informal survey of neuropathologists, however, registered
a theoretical range of 2-12% of all dementias as actually CJD (Harrison,
1991). And hundreds of thousands of Americans suffer from severe dementias
every year (Brayne, 1994; United, 1995). Two other studies average about
a 3% CJD rate among dementia patients (Mahendra, 1987; Wade, 1987). A preliminary
1989 University of Pennsylvania study showed that 5% of patients diagnosed
with dementia were actually dying from Creutzfeldt-Jakob disease (Boller,
1989). It would seem CJD is seriously underdiagnosed at present (Harrison,
1991).
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- The most common misdiagnosis of CJD is Alzheimer's disease
(Harrison, 1991). CJD was even described by our government's top CJD researcher
(Wlazelek, 1990a) as "Alzheimer's in fast forward (Wlazelek, 1990b)."
The symptoms and pathology of both diseases overlap (Brown, 1989). There
can be spongy changes in Alzheimer's, for example, and senile plaques in
CJD (Brown, 1989). The causes may overlap as well; epidemiological evidence
suggests that people eating meat more than four times a week for a prolonged
period have a three times higher chance of suffering a dementia than long-time
vegetarians (Giem, 1993), although this result may be confounded by vascular
factors (Van Duijn, 1996).
-
- Paul Brown, medical director for the U.S. Public Health
Service (Gruzen, 1996), said that the brains of the young people who died
from the new CJD variant in Britain even look like Alzheimer's brains (Hager,
1996). Stanley Prusinger, the scientist who coined the term prion, speculates
Alzheimer's may in fact turn out to be a prion disease (Prusiner, 1984).
In younger victims the disease could look like multiple sclerosis or a
severe viral infection, according to Alzheimer's expert Gareth Roberts
(Brain, 1996).
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- An estimated two to three million Americans are afflicted
by Alzheimer's (Scully, 1993); it is the fourth leading cause of death
among the elderly in the U.S (Perry, 1995). Twenty percent or more of people
clinically diagnosed with Alzheimer's disease are found at autopsy to not
have had Alzheimer's at all (McKhann, 1984). At Yale, out of 46 patients
clinically diagnosed with Alzheimer's, 6 were proven to be CJD at autopsy
(Manuelidis, 1989). In another post-mortem study 3 out of 12 "Alzheimer"
patients actually died from a spongiform encephalopathy (Teixeira, 1995).
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- Carleton Gajdusek, who was awarded a Nobel Prize for
his work with prion diseases (Manuelidis, 1985), estimates that 1% of people
showing up in Alzheimer clinics actually have CJD (Folstein, 1983). That
means that hundreds of people (Hoyert, 1996; United, 1995) may already
be dying from mad cow disease each year in the United States.
-
-
- WORKS CITED
-
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-
- Altman, Lawrence K. "U.S. Officials Confident That
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- Berger, Joseph R., et al. "Creutzfeldt-Jakob Disease:
A Ten-Year Experience." Neurology, 44 (1994): A260.
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- Bleifuss, Joel. "Killer Beef." In These Times,
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-
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1259-1263.
-
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-
- United States Department of Commerce. Statistical Abstract
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-
- Van Duijn, C. M. "Epidemiology of the Dementia:
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478-488.
-
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Alzheimer's Disease." Archives of Neurology, 44 (1987): 24-29.
-
- Wlazelek, Ann. "Fatal Brain Disease Mystifies Experts."
Morning Call, 23 September 1990a: B01.
-
- Wlazelek, Ann. "Scientists Try to Track Fatal Disease;
International Expert Visits Area to Study Unusual Incedence Rate."
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-
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- http://www.cyber-dyne.com/~tom/Alzheimer_cjd.html#and%20C
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