I first studied prion disease in a virology class during my last year of university. I must have enjoyed them because it was one of the only topics that kept my attention and prevented me from daydreaming like I usually did during that class. Because of this, I was familiar with the disease FFI (fatal familial insomnia). I find it so interesting that there are people who literally can't sleep. I know insomnia affects millions and decreases quality and quantity of sleep, but sufferers of FFI literally can't sleep because infectious proteins destroy their thalamus, a part of the brain that is responsible for circadian rhythms and other primal functions (body temperature, thirst, etc.).I liked how the author spoke to the reader as they already had some knowledge of virology and pathology - I didn't feel like the language had been dumbed down, but it was also easy enough to understand and follow.The molecular part of the prions is interesting, but I enjoyed the epidemiology parts the best - following the pedigree of the Italian family plagued with FFI, mapping the CJD and BSE outbreaks across Britain, and discovering the mystery behind Kuru.One of my favourite parts was reading about the contention between Gajdusek who studied Kuru on site in Papua New Guinea and Stanley Prusiner who studied prions back in the States. I actually found it quite funny that they hated each other and sabotaged each others' work. It brings more interest to the (sometimes) dry world of science work. The book gets 5/5 stars because it was incredibly interesting and enthralling, and taught me a lot more about prions. I wish I read it this time last year because maybe I would have paid more attention in virology class.

interesting read. its actually a misleading title: this is more about a history of prion diseases than specifically about that family suffering from an incurable insomnia. i love pop history books but i wish they wouldnt gloss over a lot of the awful racist imperialism in medical history. this book also downplays heavily the fact that one of the men critical to the discovery of prions was a pedophile and kidnapped (because thats what it was) preteen polynesian boys to rape in the US, where they were treated with the utmost contempt and hatred. but i guess he was a good guy because he did science stuff.things like that really anger me because i dont ask for perfection in doctors since theyre humans too, but its as if his victims, indigenous children, were more to blame than him. after all, what have natives, particularly native children, ever done for european society? and this book really tries to act like australias imperialist venture into papa new guinea was anything but an open land grab. they helped spread the racist myth of the cannibal native, something the book itself admits but is too cowardly to condemn.all in all, it was an interesting history that i could appreciate but the racist and rape apologia was a lot. how can we write about medical history if we dont also examine, criticize, learn from, and condemn the negative aspects of past medical professionals, INCLUDING the racism inherent in western scientific practices? guess im just being nitpicky!

Prion diseases are freaky! That little bits of proteins could mis-fold, and that topological change could decimate a brain is just bizarre. One of the facts I was most surprised by is that prion diseases have three methods of infection: genetic, direct contact (i.e. eating or touching infected tissue), and spontaneous (i.e. a protein accidentally misfolds in the body). No other disease vector can spread via all three methods like prions. They are freaky disease superstars!The Italian family in the title is beset with FFI, fatal familial insomnia, an inherited/genetic prion disease. It's sufferers tend to develop symptoms in middle-age (usually), and die fairly quickly. It's grim: their pupils turn into pin-pricks, they start sweating profusely, and they become unable to achieve any type of restful sleep. Eventually they lose all control, go into mad fits, finally fall into a coma and die. All the while their mind is intact.The family's biography is only a frame for the rest of the book. In order to explain FFI and how difficult it was to diagnose as a prion disease, you have to understand the history of prion diseases, and the history of the field. Max delves into scrapie, kuru, GSS, CJD, BSE, and other known prion diseases. In some ways, the story of the researchers trying to pin-down this new class of illness was more fascinating than the family that couldn't sleep. There are huge egos, government cover-ups, and other non-science dramas that affect the lives of many people.I really, really enjoyed this book - Max sets up the story in an extremely engaging way. It reads like a medical thriller - like something out of that TV show Mystery Diagnosis, but on steroids. There are twists and turns to the diagnosis, and a whole lotta shock factor.And yet I had to dock it a star. I thought there were two questions not just left unanswered, but totally unaddressed. 1) How does a genetic version of a prion disease, like FFI, not cause symptoms until middle-age? Is the disease building up slowly over time, or does something later set it off? 2) How does a simple mis-folding of a protein lead to a swiss-cheese brain? How do you connect the dots from misfolding to erosion of brain tissue and development of plaques.There's a good chance that there aren't satisfactory answers to either of those questions, but I was hoping Max would at least acknowledge or address them. He certainly didn't shy away from other more technical discussions.I had just finished the chapters on Mad Cow/BSE/CJD and Max goes into detail about the state of affairs today. Spoiler: it's not good at all, particularly the government's reluctance in the US. British beef is safer than US beef. Scary. My husband and I had already planned on eating beef for dinner - we had some leftover steaks that needed finishing. It certainly gave me pause. I don't eat a whole lot of beef as it is, but I might try to cut back a little more. Prions are just that freaky.