rogue protein risk

Here’s another reason mad cow disease might keep you up at night. It turns out that there might be a small chance of contracting the human version, known as Creutzfeldt-Jakob disease (CJD), through routine surgery. Both mad cow and CJD are caused by prions, mutated, misshapen proteins that force healthy proteins to misfold in the brain, fatally clumping together. All prion diseases are characterized by loss of motor control, dementia, paralysis, and eventually death, due to massive destruction of brain tissue. Researchers had previously thought that the disease-producing proteins were confined to the brain and nervous system, but a recent article in the New England Journal of Medicine states otherwise, and also hints at the new — if extremely unlikely — route of transmission. Adriano Aguzzi of Switzerland’s University Hospital Zurich and his colleagues have also detected prions in the muscle tissue and spleens in approximately one-third of 36 tested CJD victims. The finding suggests that there is a chance the infectious proteins could be passed on to others by surgical equipment that had been previously used on CJD patients. Prions cannot be killed by normal hospital sterilization procedures.

This news brief appeared in the Random Data column of the Boston Globe’s Health/Science section on 11/18/2003.