A woman who died from an incurable prion disease, Creutzfeldt-Jakob disease (CJD), was caught by its victim’s death roughly 50 years after receiving the diagnosis. The 58-year-old developed a form of CJD likely contracted through contaminated human growth hormone (HGH) treatments she received as a child.
Doctors described the unusual case in a paper published this week, detailing how the disease-causing prions accumulated over time, leading to symptoms like dementia and death. The patient’s first treatment was 51.3 years prior to her symptoms appearing, while her last treatment was 42.1 years earlier.
According to researchers, the woman possessed a mutation in the gene that codes for the prion protein linked to longer latency periods. This highlights the potential for delayed cases of HGH-related CJD.
The discovery comes as a warning to doctors treating neurological illnesses, who should be on the lookout for these delayed cases, especially if their patients have a history of HGH treatment before 1977.
Source: https://gizmodo.com/u-s-woman-dies-from-mad-cow-like-brain-disease-that-lay-dormant-for-50-years-2000603359