Chapter 24 : Prion Diseases

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Transmissible spongiform encephalopathies (TSEs), or prion diseases, are unlike any other malady. Prion diseases have long, largely asymptomatic incubation periods. Although the name would suggest all TSEs are acquired, in fact most prion diseases have no apparent cause and are called sporadic. TSEs are caused by a novel contagion once referred to as a slow virus and now called a prion. Scrapie is the oldest known TSE; it was first described in Europe at least 250 years ago. The presence of amyloid in the brains of kuru victims suggested a relationship with a much broader category of diseases. The relative resistance of a prion to proteinase K (PK) digestion is strain dependent. Worldwide, familial prion diseases account for 10 to 15% of all prion diseases. Kuru was eliminated when local authorities intervened to persuade members of the Fore tribe to stop ritual cannibalism. Diagnosing a prion disease is, by necessity, a diagnosis by elimination, since they are extremely rare. TSEs comprise a set of rare fatal neurological diseases found in many mammals and include such human diseases as Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker (GSS), fatal familial insomnia (FFI), and kuru.

Citation: Silva C. 2009. Prion Diseases, p 425-442. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch24
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Image of Figure 1.
Figure 1.

Version of mouse PrP (note that the sugar antennae are represented in their most complex form) ( ).

Citation: Silva C. 2009. Prion Diseases, p 425-442. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch24
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Image of Figure 2.
Figure 2.

Catalog of polymorphisms found in human PrP. Amino acid codes: A, alanine; D, aspartic acid; E, glutamic acid; F, phenylalanine; G, glycine; H, histidine; I, isoleucone), K, lysine; L, leucine; M, methionine; N, asparagine; P, proline; Q, glutamine; R, arginine; S, serine; T, threonine; V, valine; Y, tyrosine ( ).

Citation: Silva C. 2009. Prion Diseases, p 425-442. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch24
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Generic image for table
Table 1.

WHO case definition for CJD ( )

Citation: Silva C. 2009. Prion Diseases, p 425-442. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch24
Generic image for table
Table 2.

WHO case definition for vCJD ( )

Citation: Silva C. 2009. Prion Diseases, p 425-442. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch24
Generic image for table
Table 3.

WHO neuropathological criteria for CJD and other human TSE ( )

Citation: Silva C. 2009. Prion Diseases, p 425-442. In Fratamico P, Smith J, Brogden K (ed), Sequelae and Long-Term Consequences of Infectious Diseases. ASM Press, Washington, DC. doi: 10.1128/9781555815486.ch24

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