* Chapter 1 A DEATH IN DEVIZES
An unusual death in the U.K. marks the arrival of a harrowing new brain disease.
-A Boundless Future
-Troubling Signs
-"You Don’t Die of Depression"
-No Answers

* Chapter 2 ONE IN A MILLION
A rare disease only gradually becomes recognized as the most common human spongiform encephalopathy.
-The Unlucky Few
-Diagnosing CJD
-Stephen’s Case: CJD?

* Chapter 3 THE CANNIBALS’ LAUGHING DEATH
On a South Pacific island, two pioneer researchers begin to unlock the mysterious epidemic of kuru.
-Epidemic in the Bush
-A Real-Life M*A*S*H Doctor
-A Lifelong Pursuit Begins
-Brain Clues

* Chapter 4 CONNECTING THE HOLES
Linking kuru to a disease of sheep enables researchers to experiment with a brain-destroying agent.
-An Uncanny Resemblance
-Studying Scrapie
-Trying Transmissions
-Georgette’s Sacrifice
-The Kuru–CJD Link
-An End to an Epidemic
-Nobel Worthy

* Chapter 5 THE BIRTH OF THE PRION
The unusual mode of attack and biochemical durability of the TSE agent leads to a heretical idea.
-A Tough Invader
-The Elusive Agent
-TSEs' New Player
-Prion Proposal
-Fatal Filaments
-The Normal and the Diabolical

* Chapter 6 FAMILY CURSES
Two rare inherited prion diseases both substantiate the prion theory—and challenge it, too.
-Coding for Disease
-The Family That Couldn’t Sleep
-One Codon, Two Diseases
-The Strains Puzzle
-Explaining Strains with Prions

(You can read a translation of a case report of by Josef Gerstmann, which describes an unusual reflex action in a familial prion disease.)

* Chapter 7 ON THE PRION PROVING GROUNDS
Research in yeast and other studies show how prions can possess hereditary information and change their shapes.
-Prions of Yeast
-From Helix to Sheet
-Cofactors or Cold Fusion?
-The Copper Connection
-Double Trouble

* Chapter 8 CONSUMING FEARS
Modern agriculture enables prions to adapt to a new host, creating the dread mad cow disease.
-Tracking the Source
-Forced Cannibalism
-Tackling an Epidemic
-Mad Max
-The Watcher
-Approaching the Watershed

* Chapter 9 MAD COW’S HUMAN TOLL
Figuring out how many people will succumb to variant Creutzfeld-Jakob disease isn’t easy—especially now that BSE had spread around the world.
-Calculating Mortality
-Mad Sheep Disease?
-Spreading the Madness

* Chapter 10 KEEPING THE MADNESS OUT
Several measures help ensure animal prion proteins do not contaminate the U.S. food supply—but there are no guarantees.
-Cows in the Crosshairs
-Bovine Barricades
-Breaks in the Firewall
-American Madness?
-In Case of Emergency . . .
-Pigs and Sheep

* Chapter 11 SCOURGE OF THE CERVIDS
Chronic wasting disease of deer and elk, once confined
to a patch in the Rockies, spreads across the nation.
-Out and About
-Venison and Beyond

A more up-to-date version of this chapter appears as a feature article in the June 2003 issue of Scientific American, entitled "Shoot This Deer."

* Chapter 12 MEDICAL MISHAPS
Prion diseases spread to humans through medical mishaps.
-Surgical Spread
-Deadly Eyes
-Hazardous Hormones
-Safe Blood?
-Risk versus Benefit
-Beyond Beef
-Mystery Pills

* Chapter 13 SEARCHING FOR CURES
Can the death sentence of prion diseases ever be lifted?
-New Uses for Old Drugs
-Taking the Rational Route
- Diagnosing Prions(sheep urine collection photos)

* Chapter 14 LAYING ODDS
Are prion diseases more prevalent than we thought?
-Revisiting Sporadic CJD
-A Case for Undercounting
-Maverick Mayhem
-Menu Choices
-Man-Made Madness

NOTES
GLOSSARY
BIBLIOGRAPHY
INDEX