New York Hunter Contracted Rare and Fatal Disease After Eating Squirrel Brains

Henrietta Strickland
October 18, 2018

A new report on the 2015 death in Rochester, New York, finds that he may have suffered from variant Creutzfeldt-Jakob disease (vCJD), a rare brain condition you've likely heard of as "mad cow disease".

The 61-year-old NY man was brought to hospital after he had difficulty thinking, was losing touch with reality and he couldn't walk by himself, according to a case report presented to an infectious diseases forum last week.

Only a few hundred cases of vCJD have ever been reported, a lot of them in the United Kingdom; just four cases have ever been confirmed in the US. Family members told doctors he was an avid hunter and had previously consumed the brains of squirrels, Live Science reported.

Tara Chen, a medical student who produced the report, said it wasn't entirely clear yet if the squirrel brains were the cause of the infection, and that researchers were trying to secure autopsy samples, according to LiveScience.

The report was presented on October 4 at IDWeek, a meeting of several organizations focused on infectious diseases.

Non-Variant Creutzfeldt-Jakob Disease - a similiar disease - is also rare and tends to affect older people.

A hunter may have died from an "ultra-rare mad cow-like disease" after he ate squirrel brains.

Infectious proteins called prions attack the brain tissue and leave spongey holes that can be viewed under a microscope.

There are three forms of CJD, and just one form (which includes vCJD) is caused by exposure to infected brain or nervous system tissue.

The sporadic type is the most common, responsible for 85 percent of cases, according to the NIH.

This high number of suspected CJD cases prompted Chen, Hanna and colleagues to conduct a review of suspected CJD cases occurring at their hospital from 2013 to 2018.

The case report urges doctors to consider the disease when making future diagnoses, as pinpointing it as a cause of symptoms is so often delayed to the point where there's no time to even consider treatment.

Squirrel-brain transmission of the disease is not a new concern.

Other reports by Click Lancashire

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