Notes on the history of the prion diseases. Part II

Clinical Neurology and Neurosurgery
Charles M Poser

Abstract

The protein-only theory of transmission of the prion diseases remains controversial. Other mechanisms such as the virus, virino, and viroid hypotheses are still under consideration. All these fit in the concept of 'slow' infections that had been proposed in 1954 by Bjorn Sigurdsson, an Icelandic pathologist. Regardless of the exact mode of infection, the presence of prions in the brain has served to unite Creutzfeldt-Jakob disease (CJD), the Gerstmann-Sträussler-Scheinker syndrome and fatal familial insomnia, as well as scrapie and a number of other animal diseases, into a single pathological entity, the transmissible spongiform encephalopathies. The appearance of bovine spongiform encephalopathy in the United Kingdom and its putative relationship to new variant CJD, have put a new and unpredictable light on these unusual and uncommon diseases.

References

Oct 31, 1987·The Veterinary Record·G A WellsR Bradley
Oct 16, 1986·The New England Journal of Medicine·E LugaresiP Gambetti
Sep 2, 1967·Nature·R A Gibbons, G D Hunter
Sep 2, 1967·Nature·J S Griffith
Apr 6, 1996·Lancet·R G WillP G Smith
Jun 27, 1996·Nature·C I LasmézasJ J Hauw
Jan 24, 1998·Science·B Chesebro
Nov 17, 1998·The Journal of Neuroscience Nursing : Journal of the American Association of Neuroscience Nurses·N Mocsny
Dec 19, 1998·BMJ : British Medical Journal·P Brown, R Bradley
Dec 29, 1998·The Journal of Biological Chemistry·C Weissmann
Aug 7, 1999·Lancet·A Aguzzi, S Brandner
Aug 10, 1999·Lancet·J Collinge

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Citations

Aug 8, 2008·Journal of Virology·James B StantonTimothy V Baszler
Mar 6, 2008·Neuroimaging Clinics of North America·Majda M Thurnher, Pia C Sundgren
Feb 15, 2019·Scientific Reports·Óscar López-PérezInmaculada Martín-Burriel
Oct 12, 2010·Biochimica Et Biophysica Acta·Edward Málaga-TrilloTheodoros Sklaviadis

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