Monday, June 30, 2008

The Family That Couldn't Sleep: A Medical Mystery

by William Grimes

For more than two centuries, a strange, incurable disease has haunted a family in the Veneto region of Italy. It signals its appearance when the victim, an otherwise healthy adult, begins holding his head stiffly and sweating profusely. His pupils contract to pinpoint size, his pulse races and his blood pressure shoots up. Sleep becomes an impossibility. After about 15 months, the victim slips into a comalike state and dies.

Until quite recently, doctors and medical researchers were simply baffled by this disorder, which affects about 40 families worldwide.

Today, fatal familial insomnia, as it was named in 1986, is believed by many researchers to belong to the same class of disorders as mad cow disease, Creutzfeldt-Jakob disease and chronic wasting disease, and its peculiarities may well shed light on neurodegenerative and neuromuscular diseases like Parkinson's and Alzheimer's.

"The Family That Couldn't Sleep," D.T. Max's gracefully written medical detective story, explores the mysteries of fatal familial insomnia, their eventual decoding and the strange history of one family...

http://www.iht.com/articles/2006/09/05/features/bookmer.php

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