Published: Sat, October 20, 2018
Health Care | By Gwendolyn Kim

Doctors Suspect Man Died Of Extremely Rare Disease After Eating Squirrel Brains

Doctors Suspect Man Died Of Extremely Rare Disease After Eating Squirrel Brains

A man has died after he developed a rare and fatal infectious disease from eating squirrel brains - a deadly disorder likened to mad cow disease.

In 2015, the 61-year-old man was brought to a hospital in Rochester, New York, after experiencing a decline in his thinking abilities and losing touch with reality, the report said.

Judging by his brain scans and other tests, the man had likely come down with variant Creutzfeldt-Jakob disease (vCJD), a form of the rare and universally fatal neurodegenerative ailment.

Variant Creutzfeldt-Jakob disease is extremely rare, with only four people ever confirmed to have the disease in the USA, according to the Centers for Disease Control. His family told doctors he enjoyed hunting and had recently eaten squirrel brains - although it's unclear if he ate the whole brain or squirrel meat contaminated with parts of the brain.

Creutzfeldt-Jakob disease (CJD) is an extremely rare brain disease that affects about one in 1,000,000 people worldwide.

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A hunter may have died from an "ultra-rare mad cow-like disease" after he ate squirrel brains. Prions exist naturally in the brain and are seemingly being harmless to us.

Symptoms of CJD usually begin to appear around age 60 and include depression, anxiety, memory loss, personality changes, impaired thinking, difficulty swallowing and difficulty speaking.

Many sufferers lapse into coma and about 70 percent die within a year. There is no treatment or cure and no known way to prevent sporadic CJD.

The disease results from prion proteins that fold abnormally, leading to lesions in the brain. Most people develop the disease spontaneously, while a few inherit it.

A variant of Creutzfeldt-Jakob (vCJD) captured the public attention in the 1990s when people in the United Kingdom developed the disease from eating contaminated beef in an outbreak of mad cow disease. By publishing their preliminary case report, they also hope to raise doctors' awareness of CJD, noting that it took an average of two weeks to diagnose or rule out the disease in their cases. There are about 350 cases in the USA per year. It can only be confirmed after death, and doctors are waiting on medical records to see if an autopsy tested his brain tissue to confirm the diagnosis.

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