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Prions and the like

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Michel Goedert
DOI: http://dx.doi.org/10.1093/brain/awt179 301-305 First published online: 23 July 2013

Fatal Flaws by Jay Ingram, the Canadian author, writer and broadcaster, is the latest popular science book on transmissible spongiform encephalopathies. Unlike previous books—such as Deadly feasts by Richard Rhodes, The pathological protein by Philip Yam, Comment les vaches sont devenues folles by Maxime Schwartz, The family that couldn’t sleep by Daniel Max and The collectors of lost souls by Warwick Anderson—Fatal Flaws is able to draw on the emerging mechanistic links between transmissible spongiform encephalopathies and common human neurodegenerative diseases, including Alzheimer’s disease, Parkinson’s disease, amyotrophic lateral sclerosis and chronic traumatic encephalopathy.

The diseases

Kuru came to the attention of western medicine in 1957, when Carleton Gajdusek and Vincent Zigas described a progressive cerebellar disorder with ataxia and tremor in a population of Fore natives of the Eastern Highlands of Papua New Guinea. The patients did not develop fever and there were no signs of inflammation or antibody production. The duration of disease was 7–9 months, ending in death. Zigas, who had been a medical officer in Papua New Guinea since 1949, brought kuru to the attention of the outside world. Gajdusek, an American national who was born in 1923 and died in 2008, was in search of an important scientific problem. At the time, he was a visiting scientist with the immunologist Frank Macfarlane Burnet at the Walter and Eliza Hall Institute of Medical Research in Melbourne, where he had worked since 1954. As mentioned by Ingram, in a draft letter of April 1957, Macfarlane Burnet described Gajdusek thus: ‘My own summing up was that he had an intelligence quotient up in the 180s and the emotional immaturity of a 15-year-old. He is quite maniacally energetic when his enthusiasm is roused, and can inspire enthusiasm in his technical assistants. He is completely self-centred, thick-skinned, and inconsiderate but equally won’t …