After Silently Hiding For 48 Years, Prions Rapidly Take Over American Woman's Brain

After silently lurking for almost 50 years, a prion disease erupted in the brain of a woman in the US, triggering a rapid and severe neurological decline before taking her life. The source of the condition, it appears, was a since-discontinued hormone treatment she received decades earlier that unknowingly introduced a rogue protein into her body.

We make no apologies for being alarmist: prions are pretty terrifying. They are misfolded proteins that can trigger a domino effect by causing normal, neighboring proteins in the brain to misfold and become corrupted themselves. 

Unlike bacteria, viruses, or other infectious agents, prions contain no genetic material, yet they are nearly impossible to destroy and can lie dormant for decades before unleashing devastating effects. Once active, they carve out holes in the brain tissue, causing it to take on a sponge-like appearance. The result is rapid cognitive decline, behavioral changes, movement disorders, and ultimately death. 

There is no known cure or any treatment that can halt their progression. Fortunately, cases are relatively rare: around 300 cases are reported each year in the US.

One of the most infamous causes of prion diseases in humans is eating brain tissue of an animal that has itself been infected with prions, although there are several other unusual ways in which these abnormal proteins can enter the nervous system.

In a new medical case study, doctors in the US report the case of a 58-year-old woman who had the misfortune of falling sick with a human prion disease known as iatrogenic Creutzfeldt-Jakob disease (iCJD)

She first went to the doctor with tremors and difficulty walking, but things quickly took a sharp turn. Within weeks, she lost control of her bladder, her speech became slurred, and her movement issues worsened. As her condition spiraled, she developed rapid breathing, extreme startle reflexes, and rigid limbs. Soon after, she slipped into unconsciousness, required a ventilator to breathe, and began experiencing violent, uncontrollable muscle jerks – never regaining consciousness.

In line with her previously stated wishes, life support was gently withdrawn and she passed away peacefully.

After researching the case, the team concluded that the prion disease was linked to a hormone treatment she received as a child.

Starting in 1971, when she was 7 years old, the patient had received over nine years of cadaveric human growth hormone treatment. This treatment was designed to address growth hormone deficiency in children by regularly injecting them with human growth hormone that was extracted from the pituitary glands of people who had died. It was discontinued in 1985 after it was linked to cases of Creutzfeldt-Jakob disease.

The researchers explain that her case is just one in a wider outbreak of iCJD resulting from cadaveric human growth hormone. They noted that the number of cases has “slowed substantially” in recent years because the treatment was 40 years ago, halting further exposure. Most people who were at risk have either already died from the disease or are beyond the typical incubation period.

Nevertheless, they warn that doctors should remain vigilant as cases may still be out there, as this tragic case shows.

The new study is published in the CDC’s journal Emerging Infectious Diseases.