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Animal Disease and Human Health Risk

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How Many Humans Have Prion-related Illnesses?

Other than handfuls of cases in Europe and the relatively few individuals diagnosed with CJD worldwide, are there other cases with prion-related conditions? Is it possible that other individuals with dementia may have prion-induced illnesses? There are concerns in some quarters that some cases of "Alzheimer's" may actually be spongiform encephalopathy. Researchers at the University of Pittsburgh studied patients that were thought to have died from Alzheimer's Disease. On closer examination they found that some of them had actually died from the prion-related Creutzfeldt-Jakob disease.39 What was interesting about this report is that these individuals had a slower progression of disease than the typical CJD patient. Such slow progression is reminiscent of one of the differences in the variant CJD that has been diagnosed in Britain.

Neuropathologists with the Alzheimer's Disease Research Consortium occasionally do an autopsy of a patient who has clear-cut CJD but whose paperwork suggested only Alzheimer's.40 Despite carefully searching the medical literature, I have not come across any systematic study of the number of CJD patients misclassified as having Alzheimer's Disease. The experience of a couple of dementia research neuropathologists left me with a reasonable guess that anywhere between 1 in 50 to 1 in 200 patients who are clinically diagnosed with Alzheimer's really have unequivocal autopsy evidence of CJD. This may not sound like a very large percentage. However, when you consider the total number with Alzheimer's, this small percentage of cases accounts for a significant number of people. Currently, some four million Americans have Alzheimer's disease.41 According to a Harvard medical report, the estimates are that by 2050 the number will be over 10 million.42 If even one in 100 diagnosed Alzheimer's patients had CJD, this would translate into 40,000 cases currently and 100,000 by the middle of the next century.

So far, we have only been talking about clear-cut autopsy cases of CJD. There are concerns that not all prion-related conditions can be easily diagnosed even if an autopsy is done. For example, a recent medical report observed that in some cases, the distinction between Alzheimer's and CJD is "gray."43 Another report commented on the brain changes in a 32-year-old man who died with a dementia. The authors concluded: "This patient had features of both Creutzfeldt-Jakob disease and Alzheimer's disease, providing additional support for the existence of an overlap between these disorders."44

The human prion-related disease called Gerstmann-Straussler syndrome provides additional human evidence that these transmissible encephalopathies may strike and not leave their classic footprints. An individual who was demonstrated to have this prion-related condition did not show the sponge-like brain changes under the microscope.45 The researchers concluded that spongiform encephalopathy in humans "cannot always be excluded on neuropathologic grounds in an individual dying of a dementing condition, and the true prevalence of these diseases is likely to be underestimated." In other words, among people with dementias (conditions like Alzheimer's where there is severe loss of mental faculties), we are probably missing cases of prion-related diseases because not all such cases show the expected microscopic changes.

Perhaps an even more startling report found spongiform changes in the brains of 50 out of 66 patients with Alzheimer's disease. Smith and colleagues said that these changes were "virtually indistinguishable histologically [meaning, under the microscope] from the spongiform change characteristic of Creutzfeldt-Jakob disease (CJD)."46 Although the authors thought it unlikely that they were seeing evidence of CJD or another prion-related condition, they did at least raise that possibility. From my perspective, their work raises concerns that subtypes of Alzheimer's may exist. A vital question is: could some of these subtypes be related at least in part to prion exposure? The implications of such a possibility are far-reaching. The parallels between prion-related diseases and Alzheimer's are recorded in Figure 10: Parallels Between Prion-Related Diseases and Alzheimer's.

The sobering message from this collective body of research is: we need to seriously question our assumption that CJD is a rare disease. If just a small percentage of individuals who are diagnosed with Alzheimer's truly have CJD (in either typical or atypical forms), the numbers with this prion-related disease would be staggering.

39 Lopez OL, Larumbe MR, et al, Difficulties in differential diagnosis of long-term Creutzfeldt- Jakob disease. Neurologia 1995 Jan;10(1):37-40.

40 DJD personal communication with Dr. Ron Hamilton (412-647-6615) at the University of Pittsburgh (had 1 case in 60 who had CJD and had no suggestion on very sketchy clinical data of a prion disease being suspected) and Dr. Larry Hansen (619-534-6212) at UC San Diego who recalled 3 CJDs in some 500 Alzheimer's autopsies. From Hamilton, if a patient is suspected of CJD they are not supposed to go through the path eval that they are doing for Alzheimer's in their registry.

41 Taubes G. Misfolding the way to disease. Science 1996 Mar 15;271(5255):1493-1495.

42 Evans DA. Estimated prevalence of Alzheimer's disease in the United States. Milbank Q 1990;68(2):267-289.

43 Barcikowska M, Kwiecinski H, et al. Creutzfeldt-Jakob disease with Alzheimer-type A beta-reactive amyloid plaques. Histopathology 1995 May;26(5):445-50.

44 Liberski PP, Papierz W, Alwasiak J. Creutzfeldt-Jakob disease with plaques and paired helical filaments. Acta Neurol Scand 1987 Dec;76(6):428-432.

45 Collinge J, Owen F, et al. Prion dementia without characteristic pathology. Lancet 1990 Jul 7;336(8706):7-9.

46 Smith TW, Anwer U, et al. Vacuolar change in Alzheimer's disease. Arch Neurol 1987 Dec;44(12):1225-1228.

Notice of Credit
The article above is compliments of the Uchee Pines Institute, Seale, Alabama, a teaching and treatment facility devoted to natural remedies. For mor information, call 334-855-4781,e-mail:, or visit their Website:

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