[Transmissible spongiform encephalopathies--an unconventional contagious agent].
The clinical appearance, pathogenesis and epidemiology of the most important human and animal spongiform encephalopathies is reviewed. These comprise a heterogenous group of diseases with both transmissible and hereditary elements in their etiology. They are characterized by neurodegenerative changes in complete absence of inflammatory and immunological reaction. After a long incubation time there is a progressive development which usually leads to death. The most usual symptoms are motoric disturbances, coordination problems and mental retardation. The transmissible agent involved is unconventional, probably a modified host protein, called prion. Post-translational alterations of the prion protein seem to be of key importance in the development of pathogenic properties. The mechanism of replication and transmission of prions is not clear.
Kaptein W. Wilhelmsen og frues, Bakteriologiske Institutt, Rikshospitalet, Oslo.
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Degré
D000818:Animals; D006801:Humans; D017096:Prion Diseases / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology
Overførbare spongiforme encefalopatier--et ukonvensjonelt smittestoff.