A recent victim of a rare, fatal neurogenerative condition called Creutzfeldt-Jakob disease (CJD) likely contracted the illness by eating infected squirrel brains, according to research by staff at the Rochester Regional Health hospital.
The 61-year-old man was one of five suspected and three confirmed CJD patients treated at the medical center between 2013 and 2018, prompting an investigation into how providers can better recognize the condition’s early signs, and therefore quarantine anyone who may be carrying the transmissible form. Their case analysis was presented earlier this month at IDWeek 2018, an international conference on infectious diseases.
CJD is one of a handful of disturbing, untreatable brain disorders within the category of prion diseases. Though much about the underlying mechanisms remain poorly understood, prion diseases are known to arise when a certain protein found on many types of cells begins to misfold in the brain, arranging itself into a non-functional clump. These abnormal proteins then induce normal versions of the prion protein to convert shape, setting off an as-of-yet irreversible feedback loop that results in toxic accumulations of the infectious proteins.
While acquired CJD is the least common form of an uncommon disease – representing fewer than 1 percent of the 7,000 or so worldwide cases per year – it is the most well-known thanks to its headline-grabbing subtype, variant CJD (vCJD).
In vCJD, humans "acquire" misfolded prions by ingesting brain tissue from animals infected with non-human prion disease, such as scrapie – which affects sheep and goats – or “mad cow disease”. According to Live Science, there have only been a few hundred reported cases of vCJD, and most of these were tied to several waves of contaminated ground beef produced and sold in the 1980s and 90s.