by Daniel Max (2006). Very readable account of the discovery of Fatal Familial Insomnia in an Italian family. It is an inherited prion disease and family members have a 50% of having the gene. Its onset is around 50 years and the patient dies within a year from lack of sleep (their thalamus is affected). A truly horrible way to die. Like all prion diseases, it is untreatable and is invariable fatal. Family records trace it back at least two centuries. Max also describes the other prion diseases, in particular kuru – the laughing disease that was endemic to the Fore tribe in Papua New Guinea (transmission occurred due to their cannibalistic of eating the brain of their dead relatives), Creutzfeldt-Jacob disease, bovine spongiform encephalopathy (BSE, also known as “mad cow disease”) and related diseases in other animals. The disease-causing agent is a protein, PrP. It has a normal form – of unknown function – and a lethal configuration. It is fascinating since this is a disease that involves stereo-chemistry. You take the normal protein, fold it up in a different configuration and many years later it expresses itself in amyloid form in the central nervous system, killing the organism in nasty ways. The book vividly describes all of this, including a colorful cast of characters, in particular Carleton Gajdusek and Stanley Prusiner (both of which won, independently, Nobel Prize; this is rare). The book has an insider twist as the author himself suffers from misfolded proteins, in his case a nonfatal and slowly progressing neuromuscular disease. I recommend this book.