'The Family That Couldn't Sleep: A Medical Mystery'

Chapter One: The Doctor's Dilemma, Venice, 1765

In November 1765 a respected doctor from a good Venetian family died in the Campo Santi Apostoli, near the Jewish ghetto in Venice. The cause of his death was "an organic defect of the heart's sack" -- or so contemporary parish records state. In truth no one would have known for sure what he suffered from and we can't know either. But priests ordinarily only wrote detailed descriptions of diseases when what they saw was noteworthy, and it is intriguing that the description of the doctor's illness in the parish book is one of the longest of the year.

The entry records that the deceased suffered for more than a year from "intermittent difficulty in breathing" and adds that he was bedridden and "totally paralyzed for two months" before his death.

Many of the doctor's descendants would experience similar symptoms in the course of dying from fatal familial insomnia, suggesting that the Venetian doctor may have been the earliest recorded case of a disease that has gone on to torment his relatives for more than two centuries.

Mid-eighteenth-century Venice was a place of gaiety and vice. The city always had a fairy-tale aspect, but until the seventeenth century its unreality was checked by an appetite for business. Venice stood at the crossroads of east and west, Asia and Europe, and it avidly cashed in on its location. But with the colonization of the Americas, trade turned the other way -- across the Atlantic -- and Venice began spending down its inheritance. Goethe, visiting the city in 1786, noted that the Venetians' lagoon was silting up, their trade "declining, their political power dwindling ...Venice, like everything else that has a phenomenal existence, is subject to Time." The end was near, and everyone there knew it.

Venice's fall, though, was the time of its greatest opulence. This was the era of Casanova's wanton memoirs and the splendid Venetian regattas and processions painted by Canaletto and Francesco Guardi. One story may help to convey the moment: in 1709, there was a ball at the home of a Venetian noble in honor of the king of Denmark, Frederick IV. As the king danced with one of the guests, a newly married noblewoman named Caterina Quirini, his buckle caught a string of pearls that adorned the belt of her dress, scattering them on the poor. The lady paid them no mind. The king was about to bend down to retrieve the pearls, whereupon her husband stood up, walked across the dance floor, and crushed them under his feet, while his wife danced on.

Venice was a hereditary oligarchy. Its ruling class -- its doges, procurators, and ambassadors -- were drawn from two hundred families whose names, like that of the Quirini, had been inscribed in a "book of nobility" in the early fourteenth century. The Venetian doctor descended from one of these patrician families, great merchants and secretaries of state, whose surname adorned one of the central squares of the city. Though he was not himself entitled to wear the red toga that indicated patrician status, he enjoyed many of the other privileges of high birth in the republic.

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