Wednesday, July 15, 2009

Best Post of May '09: D.T. Max writes the back story on prion disease

The next in our series of "Best Posts of the Month" is from May 7, 2009.

Most of us accept the party line that there are three basic forms of Creutzfeldt-Jakob disease (CJD): acquired, inherited, and sporadic. The general neurology and neuropathology textbooks do not acknowledge that the very existence of sporadic CJD is controversial. But D.T. Max (picture from, in his book The Family That Couldn't Sleep: A Medical Mystery (2006), does not skirt this controversy. Some patient advocacy groups claim that CJD cases now classified as sporadic are in fact infectious. "A surprising number of mainstream scientists also doubt the existence of sporadic CJD -- among them protein experts, epidemiologists, and neurologists," Max writes. "Their objection is that sporadic CJD is an unnecessary idea. If a disease is known to spread by infection, why assume that some people also get it by chance? Why not find the infectious source in their cases as well? They see theoretical gaps in the idea of sporadic CJD theory too. For one thing, it is strange, if sporadic CJD comes about as a result of the body's declining ability as it ages to manufacture proteins correctly, that the chance of getting sporadic CJD goes down at around seventy years of age." Among the sporadic CJD doubters is none other than D. Carleton Gajdusek, who in 1976 shared a Nobel prize for his work in tracking the cause of kuru (a disease later classified among the prion disorders) as resulting from the practice of funerary cannibalism among the Fore tribes in New Guinea. Of course, in 1976, the word prion had not yet been invented. It took Stanley B. Prusiner, who himself won the Nobel in 1997 for his discovery of prions as a new biologic principle of infection, to come up with that catchy term. But Gajdusek (who died this past December) never took to the term "prion", preferring to call the infectious proteins "nucleating amyloids" -- in large part because of his rabid animosity toward Prusiner. Gajdusek addresses Prusiner in this spicy journal entry made at the time of the announcement of Prusiner's award:

"I never heard a word of original thought from you nor read such ideas in anything you authored for which I did not recognize immediately its source, which you always went out of your way to obscure. You a heretic? You a martyr? You a defender of unacceptable ideas? Bullshit! You shrewdly jumped onto a bandwagon of creative ideas and experimental work and shrewdly got on to the winning cart, proclaiming outrageously in press and media it was yours! I respect you less and less as your despicable game succeeds and you bask in your coveted fame."

There is no doubt that the prejudice, jealousy, and ambition played a large role in both the development and elucidation of the prion diseases. D. T. Max has written a wonderful book about the fascinating history of this strange class of diseases -- including CJD, kuru, fatal familial insomnia, bovine spongiform encephalopathy, and scrapie. As the mysteries surrounding the prion diseases are further unraveled, the secrets behind the more common neurodegenerative diseases (which feature their own kinds of aberrant, aggregating proteins) may also begin to be revealed.

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