American woman died of a mad-cow-like disease that lay dormant for 50 years: All you need to know

Sometimes life feels fine until the tragedy strikes and you realise that it was in the cards. An American woman recently died from an incurable prion disease that she had caught 50 years before.

In a paper published on Wednesday in the journal Emerging Infectious Diseases, doctors revealed that the 58-year-old woman had developed a form of Creutzfeldt-Jakob disease (CJD) that she likely contracted through contaminated human growth hormone (HGH) treatments she received as a child. While the globally fatal disease is popular, the death presented the longest latency period ever documented for it.

Creutzfeldt-Jakob disease


Creutzfeldt-Jakob disease is a rare, fatal and rapidly progressive neurodegenerative disorder caused by abnormal prion proteins. Prions are proteinaceous infectious particles, a rogue protein that triggers normal proteins to misfold and form a clump and disturb brain function. Over time, the accumulation of bad prions destroys the brain and leads to symptoms such as dementia and death.

What's concerning is that they are resistant to most standard methods of sterilisation that are used to kill other infectious agents like bacteria and viruses.

How are prions caused?
Prions can emerge sporadically with no clear reason. They can be caused by inherited mutations or can be transmitted through close contact with infected bodily fluids or brain matter. In the 1980s, doctors discovered that one could catch CJD through human growth hormone extracted from prion-infected cadavers. A decade later, it was learned that one could catch the disease via tainted beef from cows infected with their own version of the prion disease, which is nicknamed the mad cow disease.

What are the symptoms of CJD?


According to the National Institute of Neurological Disorders and Stroke, the main symptoms of CJD include cognitive troubles leading to dementia, involuntary muscle jerks, and lack of coordination of movements. While it can be diagnosed through a neurological exam, EEG, MRI and CSE tests, it has no cure as of now.

What happened to the woman?
According to the report, the woman initially visited doctors with tremors and trouble balancing. Once the symptoms of the disease started, her condition worsened, she was hospitalised, fell into a coma and died. An autopsy revealed her death's cause as CJD but ruled out genetic causes. Since she had received HGH treatment, it was considered to be the root cause of her illness by doctors.

HGH is used to treat severe growth-related conditions in children and has been used for many years. In this case, the woman received her first treatment 51.3 years before her symptoms appeared. Since the US switched the collection of HGH from cadavers in 1977, thus reducing the risk of prion contamination, the timeframe of the woman's infection is around 48.3 years earlier.

According to the authors, people who have a history of HGH treatment before 1977 must look out for similar delayed cases. “Although the US CJD outbreak has slowed substantially, the potential for new cases remains,” they wrote.