The Family That Couldn't Sleep: A Medical Mystery.THE FAMILY THAT COULDN'T SLEEP: A Medical Mystery DANIEL T. MAX
In 2001, Max attended a reunion for an Italian family that for 200 years had been afflicted with a mysterious and devastating disease. The symptoms are horrific: The afflicted, upon reaching middle age, suddenly find themselves unable to sleep, within months, the person dies of exhaustion. Scientists have discovered that the condition, called fatal familial insomnia Fatal familial insomnia (FFI) is a very rare autosomal dominant inherited disease of the brain. The dominant gene responsible has been found in just 40 families worldwide; if only one parent has the gene, the offspring have a 50% chance of inheriting it and developing the disease. , arises from a genetic mutation that creates prions, the same kind of misshapen mis·shape
tr.v. mis·shaped, mis·shaped or mis·shap·en , mis·shap·ing, mis·shapes
To shape badly; deform.
mis·shap proteins that cause scrapie scrapie: see prion. , mad cow disease mad cow disease: see prion.
mad cow disease
or bovine spongiform encephalopathy (BSE)
Fatal neurodegenerative disease of cattle. Symptoms include behavioral changes (e.g. , kuru kuru /ku·ru/ (koo´roo) an infectious form of prion disease with a long incubation period found only in New Guinea and thought to be associated with ritual cannibalism.
n. , and Creutzfeldt-Jakob disease. The author explains that scientists have been baffled by many aspects of these diseases because they're like infections and yet aren't caused by microbes. Max examines how prion diseases arise and considers the evidence that many of them are human-made. For example, mad cow disease emerged from the practice of feeding cow-flesh scraps to other cows. He also outlines how the similarity between symptoms of prion diseases and those of Alzheimer's disease and Parkinson's have led to increasing research into prions. Random House, 2006, 299 p., hardcover, $25.95.