Some of the 1,848 patients who received cadaver hormone developed Creutzfeldt-Jakob disease (CJD), a rare but always fatal brain disorder caused by a type of prion known as PrP-amyloid plaques. Researchers eventually realized that some such patients had also developed shockingly early deposits of misfolded amyloid-beta plaques, which help fuel Alzheimer’s disease.
Five people—now middle-aged adults—appear to have contracted the disease from medical procedures in the late 1950s through mid-1980s. The growth-hormone deficient individuals, then children, received donor hormone from cadavers whose brains apparently contained infectious, misfolded proteins that lead to often-fatal neurodegenerative disease.
The new study, published this week in Nature Medicine, highlights a divisive discussion among doctors. Rare neurodegenerative diseases involving the initially identified type of misfolded protein, PrP-amyloid plaques—like the so-called “mad cow disease” in cows, “chronic wasting disease” in deer, and CJD in humans—are called prion diseases.
