Kuru, the first prion disease: a travel back in time from Papua New Guinea to Neanderthals extinction

• Authors: Paweł P. Liberski

Title variants

PL

Kuru – pierwsza choroba prionowa u człowieka: podróż w czasie z Papui-Nowej Gwinei do wyginięcia neandertalczyków

• Languages of publication: EN

Abstracts

EN

Kuru, the first human transmissible spongiform encephalopathy was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). In this review, I briefly summarize the history of this seminal discovery along its epidemiology, clinical picture, neuropathology and molecular genetics. The discovery of kuru opened new windows into the realms of human medicine and was instrumental in the later transmission of Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the XX century. “Kuru” in the Fore language of Papua New Guinea means to tremble from fever or cold. Kuru was restricted to natives of the Fore linguistic group in Papua New Guinea’s Eastern Highlands and neighbouring linguistic groups. Ritualistic endocannibalism (eating of relatives as part of a mourning ritual in contrast to eating enemies, i.e. exocannibalism) was practiced not only in the kuru area but in many surrounding Eastern Highland groups in which kuru never developed. The first who formally published the hypothesis that kuru spreads through cannibalism was Lindenbaum and Glasse.

PL

Kuru to pierwsza odkryta choroba z grupy pasażowalnych encefalopatii gąbczastych. Została przepasażowana na szympansy przez D. Carletona Gajduska (1923–2008). W niniejszym artykule pokrótce zostanie podsumowana historia tego wyjątkowego odkrycia, a także opisana epidemiologia, obraz kliniczny, neuropatologia i biologia molekularna kuru. Odkrycie kuru otworzyło nowe perspektywy medycyny człowieka i pomogło uzyskać późniejszy pasaż chorób Creutzfeldta-Jakoba i Gerstmanna-Sträusslera-Scheinkera na naczelne, a także przyczyniło się do odkrycia zagrożenia płynącego z pasażu encefalopatii gąbczastej bydła na człowieka. Odkrycie kuru i późniejszy pasaż tej choroby jest jednym z największych odkryć medycyny XX wieku. W języku Fore słowo kuru oznacza drżenie jak z zimna lub gorączki. Kuru występowało w grupie lingwistycznej Fore w Górach Wschodnich Papui-Nowej Gwinei i w przylegających grupach lingwistycznych. Rytualistyczny endokanibalizm (konsumpcja krewnych, a nie wrogów; to ostatnie zjawisko nosi nazwę egzokanibalizmu) praktykowano nie tylko w rejonie występowania kuru, lecz także w okolicach Gór Wschodnich, w których kuru nigdy nie stwierdzono. Hipotezę o kanibalizmie jako środka rozprzestrzeniania się kuru po raz pierwszy opublikowali Lindenbaum i Glasse.

Keywords

EN

D. Carleton Gajdusek; choroby wywoływane przez priony; kuru; neuropathology; neuropatologia; prion diseases; prions; priony

Discipline

• MEDICINE: MEDICINE

• Publisher: Medical Communications
• Journal: Aktualności Neurologiczne
• Year: 2013
• Volume: 13
• Issue: 3
• Pages: 217–229

Contributors

author

Paweł P. Liberski

• Zakład Patologii Molekularnej i Neuropatologii, Uniwersytet Medyczny w Łodzi,Instytut Nauk o Zdrowiu, Państwowa Wyższa Szkoła Zawodowa w Płocku

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