Biosense term paper, CJD

Biosense term paper, CJD - Bio-Sensory Systems Term Paper...

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Bio-Sensory Systems Term Paper Creutzfeldt-Jakob Disease (CJD) 4/21/08 By: Dann Moesta
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Creutzfeldt-Jakob disease (CJD) is one of a family of rare and completely fatal diseases known as Transmissible Spongiform Encephalopathy’s. TSE’s are generally characterized as an eventual manifestation of dementia and seem to be especially resistant to treatments based on their root cause, a mutant prion. Prions (which we will discuss in great detail later) are a hallmark of TSE’s, called such because of the tiny sponge-like holes that form over the course of the disease, CJD has been given a greater amount of attention over the past several years because of the related but different form found in many ruminants, most commonly, Bovine Spongiform Encephalopathy (BSE), known to the layperson as Mad Cow disease. The prion is a protein that mammals possess; while it is likely that all vertebrates and those animals with a central nervous system also possess prions in their neural tissue, research has focused on forms of CJD that have shown to pose the most harm to humanity, Mad Cow Disease seeming to be one of them. This variant of CJD, although having received the most press, is very uncommon, even for TSEs. Less than 1% of all reported cases are iatrogenic, meaning caused because of exposure to diseased tissue or fluid. BSE is a type of iatrogenic CJD (4). There have been reported cases of such an occurrence in indigenous tribes in Papua New Guinea because the tribe’s people observed ritual cannibalism in honor of their dead relatives, but this hasn’t been further studied since the mid- 80’s. In 5-10% of cases, the cause of the disease is hereditary. This means that the family of the infected has some history of dementia. Although not all dementia is a form of spongiform encephalopathy, there has been some correlation between greater likelihood of CJD diagnosis and family history with dementia (1). Even this marker for determination is somewhat flawed because in both familial and sporadic CJD, diagnosis usually occurs when the patient is in their mid-sixties. For this reason alone, many cases of CJD are misdiagnosed as dementia due to old age, a blanket diagnosis for many people who haven’t the resources to seek aggressive medicine
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designed to find less obtuse and more definitive diagnosis. Misdiagnosis due to lack of proper healthcare, or legitimate inaccessibility of the resources and technology needed to diagnose CJD. This system wide failure to have some control over any aspect of CJD stems from the nearly 90% of all CJD diagnosis being termed “sporadic”. Sporadic CJD (s-CJD) occurs when a neural tissue, the aforementioned prion, spontaneously morphs into a highly destructive mutant protein that not only voids the regular function of that protein, to fight the development of brain degradation, but has some affinitive trait that elicits the same response from surrounding proteins, causing a cascading event wherein prion mutation happens exponentially from the point
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Biosense term paper, CJD - Bio-Sensory Systems Term Paper...

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