Published: Sat, October 20, 2018
Health Care | By Belinda Paul

Creutzfeldt-Jakob disease linked to eating squirrel brains

Creutzfeldt-Jakob disease linked to eating squirrel brains

A case of a 61-year-old from Rochester, New York, is getting some attention lately as a stark reminder of that fact, as the man's consumption of squirrel brains ultimately resulted in his death.

The report identified a 61-year-old male who was diagnosed with the rare brain infection called variant Creutzfeldt-Jakob Disease after eating squirrel brains.

The fatal brain condition is usually tied to the mad cow disease, and only a few hundred cases of the have ever been reported, according to Live Science.

He was brought to the hospital after losing touch with reality and losing the ability to walk on his own, LiveScience reports.

The man was a hunter, and it was reported that he had eaten squirrel brains, though it's not clear whether he ate an entire brain or just squirrel meat contaminated with brains.

There are three forms of Creutzfeldt-Jakob disease (CJD): one that is inherited, one that comes from exposure to infected tissue from the brain or nervous system (this form includes vCJD), and one type that is "sporadic" and does not appear to have a genetic or environmental cause. Prions exist naturally in the brain and are seemingly being harmless to us.

Creutzfeldt-Jakob disease (CJD) is an extremely rare brain disease that affects about one in 1,000,000 people worldwide. There is no treatment or cure.

Most people who contract it only live around a year.

CJD is one of several diseases that are caused by a kind of protein known as a prion.

Because CJD is so rare, doctors at Rochester Regional Health were surprised when four suspected cases of the disease occurred at the hospital within a six-month period, from November of 2017 to April of 2018. Mad cow disease and its human variant never made huge inroads into the United States, largely due to food safety practices that had always barred farmers from feeding their cows food made from most other animals (the United Kingdom did the same in 1989, while also slaughtering hundreds of thousands of cows as a precautionary measure). There are about 350 cases in the US per year.

The authors note that CJD is only confirmed by testing the brain tissue during an autopsy.

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