Der lange Schatten des Rinderwahns

Die variante Creutzfeldt-Jakob-Krankheit (CJK) ist die in jedem Fall tödliche, menschliche Form der Bovinen Spongiformen Enzephalopathie (BSE), auch „Rinderwahnsinn“ genannt. Optimisten verkünden nun, dass das Auftreten der varianten CJK rückläufig sei. Angesichts des Leids und der allgemeinen Angst vor der varianten CJK ist die Nachricht von sinkenden Erkrankungszahlen natürlich höchst willkommen. Aber stimmt sie auch? 

Die CJK gehört zur Familie der so genannten Prionenkrankheiten, einer spezifischen Gruppe neurodegenerativer, übertragbarer Erkrankungen. Obwohl weiterhin unklar ist, wie diese Krankheit im Einzelnen übertragen wird, steht fest, dass ein Schlüsselereignis bei der Entstehung dieser Krankheit der vom Prionenprotein verursachte Umbau normaler Zellen zu abnormen Zellformen ist, die offenbar die wichtigste (wenn nicht gar die einzige) Komponente der Infektion darstellen.

Die variante CJK wurde 1996 nach intensiver Überwachungsarbeit am nationalen britischen Überwachungszentrum (NCJDSU) für CJK in Edinburgh erstmals beschrieben. Diese neue Form einer Prionenkrankheit zeigte markante klinische und pathologische Merkmale und trat bei jungen Patienten innerhalb einer genetischen Untergruppe auf.

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