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A man in New York has developed an extremely rare and fatal brain disorder after eating the brain of a squirrel, according to a new report about him.
In 2015, the 61-year-old man was taken to a hospital in Rochester, New York, after seeing a decline in his thinking skills and lost touch with reality, the report said. The man had also lost the ability to walk alone.
An MRI of the human head revealed a striking finding: the brain scan resembled that seen in people with variant Creutzfeldt-Jakob Disease (vCJD), a deadly brain disease caused by an infectious protein called prion. Only a few hundred cases of vCJD were reported and most were related to the consumption of contaminated beef in the United Kingdom in the 1980s and 1990s. (In cows, vCJD is commonly referred to as "mad cow disease". ".)
But in this case, the man had another dietary habit that could have increased his risk of contracting vCJD: his family declared that he liked to hunt and that he would have eaten the brain. a squirrel, said Dr. Tara Chen, resident physician in Rochester. Regional Health and lead author of the report. It is not known if the man consumed all the squirrel brain or just squirrel meat that was contaminated with squirrel brain fragments, said Chen. [27 Oddest Medical Cases]
Chen did not treat the patient, but she discovered the case by writing a report about suspected cases of Creutzfeldt-Jakob disease seen at her hospital in the last five years.
The report was presented on October 4 at IDWeek, a meeting of several organizations focused on infectious diseases.
Creutzfeldt-Jakob Disease (CJD) is a progressive neurological disorder that affects approximately 1 in 1 million people each year worldwide, according to the National Institutes of Health (NIH). It is a "debilitating disease" that evolves rapidly and usually leads to death within one year of diagnosis, Chen said. There is no treatment or cure.
The disease results from prion proteins that fold abnormally, causing damage to the brain.
There are three forms of Creutzfeldt-Jakob Disease (CJD): a hereditary form, a form due to exposure to infected tissues of the brain or nervous system (this form includes v-CJD) and a "sporadic" form no seems to have a genetic or environmental cause.
The sporadic type is the most common, responsible for 85% of cases, according to the NIH.
Because of the scarcity of CJD, doctors at Rochester Regional Health were surprised when four suspected cases of the disease occurred in the hospital during a six-month period, from November 2017 to April 2018. This number is higher than expected based on the population of approximately 1 million people in the Rochester area, said study co-author Dr. John Hanna, also a medical resident in Rochester Regional Health.
This high number of suspected cases of CJD prompted Chen, Hanna and her colleagues to review suspected CJD cases at their hospital from 2013 to 2018. (Five cases were identified, but two of them were finally tested negative for CJD.)
That's when the doctors discovered the case of the squirrel brain. The tests indicated that it was a "probable" case of vCJD because of the discovery of MRI and a test showing specific proteins in the fluid cerebrospinal patient, which often indicate the disease.
However, CJD can only be confirmed by a brain tissue test at autopsy upon death. Although the patient died as a result of his diagnosis, Chen and his colleagues try to gain access to his medical file to find out if CJD was confirmed at autopsy. If so, such confirmation would be very unusual. According to the Center for Disease Control and Prevention, only four confirmed cases of vCJD have been reported in the United States.
The review of the five cases revealed a worrying finding: the diagnosis of the disease was often delayed; in one case, about two weeks elapsed before the doctors suspected a patient with CJD. In this case, the patient, a 65-year-old woman, underwent plasmapheresis, a blood filtration procedure, and gynecological surgery prior to diagnosis.
A rapid diagnosis of CJD is important because infectious prions could contaminate the material used on patients with the disease, which could transmit the disease to other people if the material is not properly cleaned. .
The diagnosis may be delayed, in part, because CJD is rare and is not "on the doctor's head" when assessing the patient, said Hanna. In addition, once doctors suspect CJD and order a weeks fluid test to get the test results.
The report highlights the need for physicians to keep in mind the diagnosis of CJD and for hospitals to have "infection control policies regarding CJD," said Hanna.
Originally published on .
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