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Chapter 92 : Prion Diagnostics
The natural forms of prion disease are shown in this chapter. Diagnostics for transmissible spongiform encephalopathies (TSEs) have become important following the realization that several of them may infect humans (or should be assumed to do so) and that, because of modern agricultural and medicinal techniques, large numbers of people may be put at risk. Also, prophylactic agents and progress in potential methods of treatment for Creutzfeldt-Jakob disease (CJD) patients suggest that diagnostics may be necessary to permit the therapeutics to be used early in a symptomatic phase. Several methods are claimed to show tubulofilamentous particles present in brain that are specific to TSEs and start to appear in the tissues at around half the incubation period of the disease. Standard staining methods can be used for the demonstration of specific histopathological changes in the brain, e.g., hematoxylin and eosin stains. The growth of academic groups in the field of prion research has led to a tendency for each of them to be large enough to declare that their own methods are adequate. The development of many diagnostic systems and methods has meant that many groups have difficulty knowing which would be of most value for individual patients despite the appearance of national and international guidelines. Currently over 20 million cattle are being tested annually for bovine spongiform encephalopathy (BSE) in Europe, and blood transfusion risks may demand that a similar number of blood donations also be tested for the foreseeable future.