The Mysteries Surrounding Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob Disease is a killer. What we know is that it wastes no time. Creutzfeldt-Jakob Disease will kill a person within one year of contraction. It is yet unknown how Creutzfeldt-Jakob Disease chooses its victims, but it does seem to have accomplices, known as the spongiform encephalopathies, in the rest of the animal kingdom. It is possible that it may be known what means Creutzfeldt-Jakob Disease uses in order to annihilate its victims, but the theory surrounding that is still controversial. By examining the facts and observing the evidence we shall one day uncover the truth behind this malicious killer.
Creutzfeldt-Jakob Disease is a rare neuro-degenerative disease. Creutzfeldt-Jakob Disease is a rare neuro-degenerative disease that leads to the loss of coordination, dementia and death. Death usually occurs within a year of the onset of symptoms. Creutzfeldt-Jakob Disease is a human disease and can be dormant for as long as thirty years. The average age of onset is sixty years, but there are recorded instances of onset as easily as sixteen years and as old as 80 years. It is estimated that, world-wide, between 0.5 and 1 case per million population occurs annually. Increased incidence in some regions of the world has been attributed to the possibility of a genetic predisposition. In the United States the incidence has been reported as being 0.9 deaths per million population attributable to Creutzfeldt-Jakob Disease. This is an inexact figure however, due to the fact that Creutzfeldt-Jakob Disease is not a reportable disease in the United States (Holman) and the Center for Disease Control does not actively monitor the disease (Altman). To track the disease the Center for Disease Control has initiated a four-state study of death certificates (Altman), but since death certificates are not always accurate Davanpour) the survey may not provide an accurate assessment. This leaves the true prevalence in the United States and other countries remaining a mystery. Compounding the uncertainty, autopsies are rarely performed on atypical dementia patients (Harrison) because medical professionals fear infection (Altman). The officially reported rate of Creutzfeldt-Jakob Disease incidence is less than one case per million people per year (World). An informal survey of neuropathologists, however, registered a theoretical range of 2 - 21% of all dementia as actually Creutzfeldt-Jakob Disease (Harrison) and hundreds of thousands of Americans suffer from severe dementia every year (Brayne; United). Two other studies average about a 3% Creutzfeldt-Jakob Disease rate among dementia patients (Mahendra; Wade). A preliminary 1989 University of Pennsylvania study showed that 5% of patients diagnosed with dementia were actually dying from Creutzfeldt-Jakob Disease (Boller). It would seem that Creutzfeldt-Jakob Disease is seriously underdiagnosed.
The most common misdiagnosis of Creutzfeldt-Jakob Disease is Alzheimer's disease (Harrison). Creutzfeldt-Jakob Disease was even described as “Alzheimer’s in fast forward (Wlalzek).” The symptoms and pathology of both diseases overlap. There can be spongy changes in the brain (a classic effect of Creutzfeldt-Jakob Disease) in Alzheimer's disease, for example, and senile plaques deposited on the brain (a classic effect of Alzheimer's disease) in Creutzfeldt-Jakob Disease (Brown). 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 dementia than long-time vegetarians (Giem), although this result may be confounded by vascular factors (Van Duijn). Paul Brown, medical director for the U.S. Public Health Service (Gruzen), said that the brains of the young people who died from the new variant Creutzfeldt-Jakob disease in Britain even look like the brains of Alzheimer's patients (Hager). Stanley Prusiner, the scientist who coined the term prion, speculates that Alzheimer's may in fact turn out to be a prion disease as well (Prusiner). In younger victims the disease could look like multiple sclerosis or a severe viral infection, according to Alzheimer's expert Gareth Roberts (Brain). 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). At Yale, out of 46 patients clinically diagnosed with Alzheimer's, 6 were proven to have actually had Creutzfeldt-Jakob Disease at autopsy (Manuelidis). In another post-mortem study 3 out of 12 "Alzheimer" patients actually died from a spongiform encephalopathy which is the class of diseases that Creutzfeldt-Jakob Disease belongs to (Teixeira). These spongiform encephalopathies