Notes on the history of the prion diseases. Part II

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 pat...

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Bibliographic Details
Published in:Clinical Neurology and Neurosurgery 2002-05, Vol.104 (2), p.77-86
Main Author: Poser, Charles M.
Format: Article
Language:English
Subjects:
Biological and medical sciences
Creutzfeldt–Jakob disease
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Disease Transmission, Infectious
Fatal familial insomnia
Gerstmann–Sträussler–Scheinker syndrome
History, 20th Century
Humans
Medical sciences
Neurology
Neurology - history
Prion Diseases - diagnosis
Prion Diseases - history
Prion Diseases - physiopathology
Prions
Public Health - history
Transmissible and bovine spongiform encephalopathy
United Kingdom
Variant CJD
Virino
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Summary: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.
ISSN:0303-8467
1872-6968
DOI:10.1016/S0303-8467(01)00200-1