Alzheimer's And CJD
Scientifically Linked
By Michael Greger

(First published 6-16-96)
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.
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).
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).
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).
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.
Altman, Lawrence K. "Four States Watching for Brain Disorder." New York Times, 9 April 1996a.
Altman, Lawrence K. "U.S. Officials Confident That Mad Cow Disease of Britain Has Not Occured Here." New York Times, 27 March 1996d: 12A.
"Apocalypse Cow: U.S. Denials Deepen Mad Cow Danger." PR Watch, 3.1 (1996): 1-8
Berger, Joseph R., et al. "Creutzfeldt-Jakob Disease: A Ten-Year Experience." Neurology, 44 (1994): A260.
Bleifuss, Joel. "Killer Beef." In These Times, 31 May 1993: 12-15.
Boller, F., O. L. Lopez, and J. Moossy. "Diagnosis of Dementia." Neurology, 38 (1989): 76-79.
Boule, Margie. "Despite Anectdotal Evidence, Docs Say No Mad Cow Disease Here." Oregonian, 16 April 1996: C01.
"Brain Disease May Be Commoner Than Thought - Expert." Reuter Information Service, 15 May 1996.
Brayne, C. "How Common are Cognitive Impairment and Dementia?" Dementia and Normal Aging, Canbridge: University Press, 1994.
Brown, Paul. "Central Nervous System Amyloidoses." Neurology, 39 (1989): 1103-1104.
Davanpour, Zoreth, et al. "Rate of Creutzfeldt-Jakob Disease in USA." Neurology, 43 (1993): A316.
Flannery, Mary. "Twelve - Fifteen 'Mad Cow' Victims a Year in Area." Philadelphia Daily News, 26 March 1996: 03.
Folstein, M. "The Cognitive Pattern of Familial Alzheimer's Disease." Biological Aspects of Alzheimer's Disease. Ed. R. Katzman. Cold Spring Harbor Laboratory, 1983.
Gruzen, Tara. "Sheep Parts Fail to Cause Mad Cow Disease in U. S. Test." Seattle Times, 29 March 1996: A11.
Hager, Mary and Mark Hosenball. "'Mad Cow Disease' in the U.S.?" Newsweek, 8 April 1996:58-59.
Harrison, Paul J., and Gareth W. Roberts. "'Life, Jim, But Not as We Know It'? Transmissible Dementias and the Prion Protein." British Journal of Psychiatry, 158 (1991): 457-70.
Holman, R. C., et al. "Edidemiology of Creutzfeldt-Jakob Disease in the United States, 1979-1990." Neuroepidemiology, 14 (1995): 174-181.
Hoyert, Donna L. "Vital and Health Statistics. Mortality Trends for Alzheimer's Disease, 1979-1991." Washington: U.S. Dept. of Health and Human Services, 1996.
Little, Brian W., et al. "The Epidemiology of Creutzfeldt-Jakob Disease in Eastern Pennsylvania." Neurology, 43 (1993): A316.
Mahendra, B. Dementia, Lancaster: MTP Press Limited, 1987: 174.
Manuelidis, Elias E. "Presidential Address." Journal of Neuropathology and Experimental Neurology, 44 (1985): 1-17.
Manuelidis, Elias E. and Laura Manuelidis. "Suggested Links between Different Types of Dementias: Creutzfeldt-Jakob Disease, Alzheimer Disease, and Retroviral CNS Infections." Alzheimer Disease and Associated Disorders, 2 (1989): 100-109.
McKhann, Guy., et al. "Clinical Diagnosis of Alzheimer's Disease." Neurology, 34 (1984): 939.
Prusiner, S. "Some Speculations about Prions, Amyloid, and Alzheimer's Disease." New England Journal of Medicine, 310 (1984): 661-663.
Perry, R.T., et al. "Human Prion Protein Gene: Two Different 24 BP Deletions in an Atypical Alzheimer's Disease Family." American Journal of Medical Genetics, 60 (1995): 12-18.
Scully, R. E., et al. "Case Records of the Massachusetts General Hospital." New England Journal of Medicine, 29 April 1993: 1259-1263.
Teixeira, F., et al. "Clinico-Pathological Correlation in Dementias." Journal of Psychiatry and Neuroscience, 20 (1995): 276-282.
United States Department of Commerce. Statistical Abstract of the United States, Washington: Bureau of the Census, 1995.
Van Duijn, C. M. "Epidemiology of the Dementia: Recent Developments and New Approaches." Neuroepidemiology, 60 (1996): 478-488.
Van Duijn, C. M. "Epidemiology of the Dementia: Recent Developments and New Approaches." Neuroepidemiology, 60 (1996): 478-488.
Wade, J. P. H., et al. "The Clinical Diagnosis of 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." Morning Call, 27 September 1990b: B04.
"World Health Organization Consultatoin on Public Health Issues Related to Bovine Spongiform Encephalopathy and the Emergence of a New Variant of Creutzfeldt-Jakob Disease.", Morbidity and Mortality Weekly Report, 12 April 1996: 295-303.


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