A Man’s Love of Squirrel Meat Might Have Given Him a Horrifying, Fatal Brain Disease

A Rochester , New York , resident might have caught one of the most frightening brain diseases around in one of the unusual direction potential — eating squirrel meat contaminated with zombie - alike protein .

The man ’s bizarre story was include in a preliminary reportreleasedearlier this month at IDWeek , the annual conference of the Infectious Diseases Society of America . His typeface was one of several detailed by doctor from Rochester Regional Health , a major infirmary web in the city .

According to the paper , the 61 - year - erstwhile man had been admitted to a hospital at Rochester Regional with cognitive impairment , schizophrenia , and psychosis in 2015 ; he was also unable to walk under his own power . But there was nothing to be done . Five month after his symptom began , he die . Judging by his brain scans and other tests , the man had in all probability come down with variant Creutzfeldt - Jakob disease ( vCJD ) , a form of the rare and universally fatal neurodegenerative ailment .

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CJD is one of several diseases that are make by a kind of protein known as a prion . Prions exist by nature in the brain and are apparently harmless to us . But when these proteins set out to fold in ways they should n’t , they force other nearby prion to misfold too , make a grow army that , over years oreven tenner , in effect cannibalizes the brain .

Prion diseases can come about spontaneously or be extend down through family . But they can also be infective , and some are even open of spreading between different metal money of fauna . These diseases are also shout out ancestral spongiform encephalopathies , a reference to the hollowed out , quick study - alike appearance of the brain ( at least when canvas under a microscope ) in someone who has conk from it .

vCJD was first identified in 1996 , when relatively vernal UK residents start dying circumstantially from CJD . These victim had unluckily consume prion - infected beef from cows with their own form of prion disease , bovine spongiform encephalopathy ( or what most people in all likelihood know as mad moo-cow disease ) . And the cows had likely caught it from eat animal provender made with the infected mental capacity subject of other cow as well as yet another animate being vulnerable to prions , sheep .

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There have only been around 200 documented cases of vCJD worldwide , with the legal age occurring in the UK . Mad cow disease and its human discrepancy never made huge inroad into the U.S. , mostly due to food safety practice that had barred Farmer from feeding their cows food made from most other animate being ( the UKdidthe same in 1989 , while also massacre hundreds of thousands of cows as a precautionary measure ) . Not everyone who feed septic meat seems to derive down with vCJD either , likely meaning our genetics play a part there as well .

But as early as 1997 , scientist in the U.S. havebeen worriedabout the theory of regional delicacies such as squirrel and other hunted animals spreading prion disease . And in the man ’s case , Live Sciencereported , the house say his doctors that he was an avid Orion and connoisseur of squirrel nub .

The source of the written report , contribute by medical house physician Tara Chen , incidentally came across the causa , not having address the homo himself . They began digging through hospital records after seeing four suspected cases of CJD harvest up within the pair of six month last winter — an unusually gamey bit for arare diseasethat affect about 350 people in the entire land per year . Of the five case detailed in their report , however , two were finally confirmed not to be CJD after all .

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Though current tests can distinguish vCJD from the classical physique of the disease , Chen told Live Science that it ’s unknown whether the man was definitively diagnosed with CJD . With many fatal brain disease , such as Alzheimer ’s , doctors can only be certain of the diagnosis by examining the wit after death . If the man is confirmed to have had vCJD , he might very well be the only person ever known to have contracted it within the U.S. There have been four otherofficially recognizedcases of vCJD in the U.S. , and all are thought to have been give away to it before they entered the country .

harmonise to Live Science , Chen and her squad are currently attempt permission to obtain the Isle of Man ’s aesculapian book . to see if an autopsy diagnosis was made . By write their preliminary face report , they also hope to raise MD ’ awareness of CJD , noting that it took an average of two hebdomad to name or harness out the disease in their cases . And that wait could elevate the risk of cross - contagion within hospitals , thanks to contaminated aesculapian equipment that is n’t properly sterilized .

contribute to their zombie - like nature , prions can come through things that easily shoot down other pathogens , such as radiation and chemical like methanal .

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