The Family That Couldn't Sleep: A Medical Mystery
For two hundred years a noble Venetian family has suffered from an inherited disease that strikes their members in middle age, stealing their sleep, eating holes in their brains, and ending their lives in a matter of months. In Papua New Guinea, a primitive tribe is nearly obliterated by a sickness whose chief symptom is uncontrollable laughter. Across Europe, millions of...more
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I have a problem and it concerns books. If I see a title that sparks my curiosity, I must have it. I can normally keep this under control but then an enemy was unleashed in the form of my Kindle Paperwhite in February 2013. As a consequence, one click on Amazon a ...more
The Italian family in t ...more
At the center of this book is a Venetian family with a deadly leg ...more
Narrated by: Grover Gardner
Like a smart consumer in the market for electronics, appliances or cars, I research my purchases by looking up recommendations on Consumer Reports. When I am looking for a good book to read, I turn to my trusted source for reviews—Goodreads. Based on member recommendations, I know going in that the book I choose will more than likely receive a higher than average rating from me. You guys have never let me do ...more
Every time I donate blood (and I've donated well over ten gallons) I'm asked whether I've spent at least three months in the U.K. prior to 1996 (c.f.). This is because of what we all called "Mad Cow Disease" and what the medical folks now call Variant Creutzfeld-Jacob Disease (vCJD).
This book is poorly titled. Certainly, the horrible fate of an Italian family brings immediate human pathos to the story of prion diseases, but the more pressing story for many of us will be the atrocious practices i ...more
The book covers all aspects of prion diseases, the most famous of which is Mad Cow Disease (aka Bovine Spongiform Encepholopathy). I have a sort of superiority complex fascination/horror with BSE; as a vegetarian for the past 17 years I feel relatively safe from it. Prions are especially terrifying foodborne illne ...more
The book says that the gene encoding the "prion gene" involved in CJD/kuru/FFI has two alleles. One codes for a methionine at a particular site and the other codes for a valine in the same position. If you have two copies of the same allele, one maternal and one paternal, you are homozygous. If you have one of each kind, you are heter ...more
This is a true-medicine thriller that leads the reader through the investigation of prion diseases, with surprising revelations along the way. The mysteries include a wasting brain disease that plagues an Italian family for generations, an epidemic of a similar disease called "kuru" found in a remote tribe in New Guinea; and the origins of prion diseases in sheep and cattle.
The book also introduces us to the scientists who are resear ...more
The book is basically about protein diseases (almost all of which are brain-based, if not all?). It focuses on one where the prion protein ...more
1. It is poorly organized. The chapters alternate between telling the story of the "family that couldn't sleep"--an Italian family suffering from Fatal Familial Insomnia, or FFI--and covering the history of prion diseases & research. That would be fine on its own if there was still some kind of timeline holding everything together, but there isn't: one chapter will discuss prion research from 1970-2004 ...more
We learn the history of the Italian family whose members are at risk of dying a horrible death in middle age - long after they have already passed the genetic defect on to some of their children. It’s a little disturbing to ...more
Ya había estudiado los priones antes y por supuesto el Insomnio Familiar Fatal es algo que se estudia siempre en Psicofisiología cuando se tratan temas de sueño. Me habría gustado tener este libro entonces, porque de forma sencilla describe lo básico que hay que saber sobre el tema.
Habla de lo que son los priones, las historia de su estudio y las enfermedades que se pueden producir por ellos, tanto en animales como en personas. Todo llevado con el hilo conductor de la famosa familia italian ...more
A grim, satisfying look at a terrifying condition largely ignored, and largely of our own making.
This is an exquisitely researched and ultra-readable medical mystery thriller focused on prion diseases. They are awful diseases that cause stabbing pains, loss of muscular control, sweating, insomnia, madness and death. It’s an especially poignant read because the author has a degenerative disease as well, giving him a tragically appropriate vanta ...more
As others have pointed out, the book is more about the history of the discovery of prions and the researchers involved, with the titular family only taking up a portion of the text. You can't really blame Mr. Max for choosing a more interesting title than "Prions: A History" though.
I do hope that considerable resources are dedicated to solving the mysteries of protein misfolding. There are still so many questions.
One particular thing that struck me about the Italian family wit ...more