Prion-Krankheiten

Zusammenfassung.

Transmissible spongiforme Enzephalopathien (TSE) sind bei Mensch und Tier bekannt. Wissenschaftlich wird eine selbst-propagierende Konformationsänderungen von infektiösen Proteinpartikel, den Prionen, als ursächlich für die TSE erachtet. Gesichert ist eine genetische Komponente bei Infektionsempfänglichkeit sowie die Übertragbarkeit über Artgrenzen hinweg. Große Aufmerksamkeit in der Öffentlichkeit erlangten die TSE durch die Erstbeschreibung der neuen Variante der Creutzfeld-Jakob-Krankheit im Zusammenhang mit einer möglichen Übertragung der Bovinen Spongiformen Enzephalitis (BSE) des Rindes auf den Menschen. Dies führt zu einer zunehmenden Unsicherheit von klinischem Personal im Umgang mit Patienten mit einer TSE. Diese Darstellung fasst in einer Übersicht alle bekannten TSE bei Mensch und Tier zusammen und gibt einen Überblick über jüngste Entwicklungen, sowohl was den Stand der Diagnostik und Therapie anbelangt als auch den Ausschluss von möglichen iatrogenen Übertragungswegen.

Prion Diseases.

Transmissible spongiform encephalopathies (TSEs) are clinical entities recognized both in humans and animals. Current evidence suggests that TSEs are caused by a self-propagating alteration of infectious protein particles known as prions. In addition, there is strong evidence for a genetic predisposition for the disease and documentation that transmission can cross species borders. The current public awareness of TSEs has resulted from the clinical symptoms observed in bovine spongiform encephalopathy (BSE) and the demonstration that a new variant of the Creutzfeld-Jacobs syndrome is probably the result of transmission from animals to humans. This has led to uncertainity amongst clinical personnel when dealing with patients presenting with TSEs. We review briefly the spectrum of TSEs known both in animals and humans, report on recent developments in the diagnostics and therapy, and discuss iatrogenic sources of infection.

Schlüsselwörter:

TSE - Prion - Creutzfeld-Jakob - BSE - Therapie

Key words:

- TSE - Prions - Creutzfeld-Jakob - BSE

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