- The Washington Times - Saturday, November 4, 2006

D.T. Max has written a book, The Family That Couldn’t Sleep: A Medical Mystery (Random House, $25.95, 336 pages), about a few diseases transmitted via prions, wayward protein molecules that disable their victims by distorting the shape of the normal protein molecules in their brains, multiplying rapidly as they do this.

Mr. Max’s compelling narrative describes the gruesome details of these diseases, explains the painstaking detective work of the researchers who identified them and elucidated the hitherto unknown way they damage humans and animals, and vividly portrays both victims and researchers.

The least known of these diseases, Fatal Familial Insomnia (FFI), is an extremely rare (found so far in just 28 families around the world) genetic malformation. Its symptoms, most notably total insomnia and, eventually, dementia, usually develop between ages 30 and 60. The disease runs its course over a period varying from a few months to three years and is invariably fatal.

Mr. Max shows how FFI has affected successive generations of an Italian family since at least the mid-1700s, when an upper-class Venetian doctor seems to have been the first recorded victim. He follows the family history through to the present, sketching the fates of victims in successive generations and different branches of the family.

Interspersed between the chapters dealing with FFI are discussions of other prion-caused diseases in humans and animals, and how researchers closed in on their common mechanism by noticing the similarity of their effects.

All these diseases wreak destruction on their victims’ brains, giving them spongy texture due to numerous microscopic holes. The first to be observed was scrapie, which makes sheep act in bizarre ways, including scraping themselves on available surfaces — hence the name, coined by Scottish shepherds in the 18th century. Fortunately, scrapie is not transmissible to humans.

The best known of the diseases is mad cow disease, formally termed bovine spongiform encephalopathy (BSE, for short). Mad cow affects cows in a way similar to scrapie in sheep. To date, says Mr. Max, it has infected some 800,000 British cows and 160 British citizens who were unfortunate enough to eat part of the brain or spine of an affected animal.

In humans, it is known as variant Creutzfeldt-Jakob disease (CJD) because of its similarity to another extremely rare brain disease identified in the first half of the 20th century. CJD affects a tiny number of people (about one in a million), but an outbreak of the variant version occurred among Englishmen who had eaten British beef coming from cows that had been fed with meal containing ground up parts of affected cows.

Mr. Max details how long it took to establish the nature and cause of the disease, partly because the British agricultural establishment tried to minimize knowledge of its prevalence and provenance.

Kuru, another prion disease, is limited to members of the Fore tribe in New Guinea. The Fores, a culturally flexible group, learned about cannibalism from other tribes comparatively recently, and took to it enthusiastically. They did not waste any body parts of their deceased friends and relatives, including their brains, some of which were diseased.

The propagation of kuru ceased half a century ago, after missionaries and the Australian colonial authorities put an end to cannibalism, although cases still crop up among Fore old enough to have indulged in the gory celebrations.

Mr. Max describes how Carleton Gajdusek, a young American medical researcher in search of the exotic, studied kurufor years and won the 1976 Nobel Prize in Medicine for establishing the existence of “slow viruses” that took decades to affect victims. However, Mr. Gajdusek missed the connection between kuru and cannibalism, which was established by a pair of Australian anthropologists.

Meanwhile, another dogged American researcher, Stanley Prusiner, set about investigating Mr. Gajdusek’s slow viruses and eventually won his own Nobel Prize in 1997 for showing that they were not viruses at all, but entities he dubbed “protinaceous infective particles” or “prions,” which better met his criteria for a short, catchy name. While Mr. Prusiner pronounces the word “Pree-on,” the British prefer “Pry-on,” partly to express their distaste for Mr. Prusiner’s pushy ways, says Mr. Max.

Neither Mr. Gajdusek nor Mr. Prusiner is a particularly appealing human being. The former exiled himself to Amsterdam in 1998 after serving a prison sentence for corrupting Micronesian youths who lived in his house. He describes himself as “a pedagogic pedophiliac pediatrician.” Mr. Prusiner is known for his fierce resistance to sharing credit with any other scientist. A fellow researcher who knows both men says “Carleton’s an egoist, Stan’s an egotist.”

Mr. Max aptly terms his engrossing tale of unraveling complex brain diseases “a medical mystery.” Bruce Rosenblum and Fred Kuttner’s The Quantum Enigma: Physics Encounters Consciousness (Oxford University Press, $29.95, 204 pages) deals with a much deeper mystery: the relationship between reality and the human mind. This is the source of what they call the quantum enigma.

Both authors, senior physics professors at the University of California at Santa Cruz, have reached the stage in life when they can afford to explore the philosophical underpinnings of their subject without worrying how it will affect their career prospects to say that those underpinnings are loose.

Assuming that their readers have no previous knowledge of quantum theory, they provide a short history of physics before discussing the shocking discovery that unfolded during the first quarter of the 20th century, that the behavior of matter defies not only common sense but the fundamental belief of scientists that nature can be understood. The theory of quantum mechanics provided a way of calculating the behavior of matter and energy with incredible precision, but anyone who tried to explain what was described by the mathematics ended up tied in knots.

Mr. Rosenblum and Mr. Kuttner describe in a simplified way the quantum mechanical paradox: Electrons and other sub-microscopic entities lead dual lives, behaving sometimes like localized particles and at other times like spread-out waves. The choice depends on whether and how an observer is watching.

The generally accepted explanation of this, known as the Copenhagen Interpretation in honor of the home town of Niels Bohr, its originator, is that the spread out probabilistic “wave function” that describes the behavior of the system, says that it exists simultaneously in many different states, which “collapse” to just one state when the system is observed.

The difficulty of accepting this interpretation has been pointed out many times, most famously in the hypothetical experiment involving “Schrodinger’s cat,” which is both dead and alive simultaneously. Another major problem, the reason why Albert Einstein, Bohr’s great friend and intellectual antagonist, never accepted quantum mechanics, is that knowledge appears to be transmitted instantaneously throughout the universe, reintroducing the long-discredited idea of “action at a distance,” which Einstein called “spooky.”

In their lively and thought-provoking presentation, Mr. Rosenblum and Mr. Kuttner present several proposed alternatives to the Copenhagen interpretation, but all raise their own questions. Notable among them is the bizarre but increasingly popular “many worlds” interpretation that says that quantum mechanics correctly describes the simultaneous existence of many different worlds, which are continuously being created and separating from each other.

They conclude with a discussion of the phenomenon of consciousness, which some suggest is a necessary and basic part of the universe. If true, that should boost the self-esteem of the human race.

Jeffrey Marsh has written widely on scientific topics and public issues ranging from nuclear strategy to social policy.



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