A man developed an extremely fatal brain disorder after eating squirrel brains. The 61 year-old-man from New York was brought to the hospital, Rochester Regional Health, after he experienced a decline in his thinking abilities. He further felt like he was losing touch with reality, he even lost his ability to walk without support. The man’s MRI revealed a striking finding, the man’s brain scan looked similar to the scan of a brain with a fatal brain disorder, Creutzfeldt-Jakob disease (CJD). The disease is caused by an infectious protein called prions and only a few hundred cases have ever been reported. The outbreak of Creutzfeldt-Jakob disease was first seen in the 1980s and 1990s in United Kingdom because of consuming contaminated beef, for this reason, the disease is more commonly known as mad cow disease.

However, in this case, there was another dietary habit that raised this man’s risk of developing CJD. According to his family, he enjoyed hunting and that he had eaten squirrel brains. Although, it was unclear whether the man consumed just parts of a squirrel’s brain, an entire squirrel brain or just contaminated squirrel meat.

CJD affects 1 in 1 million people each year

CJD affects about 1 in 1 million people each year, worldwide. The progressive neurological disorder is a debilitating disease that progresses quickly and within a year of diagnosis, usually results in death. The disease is because of a prion protein that leads to lesions in the brain. CJD has three different variants, it is either inherited, caused because of an exposure to the infected tissue from the nervous system or brain or “sporadic” type which does not have an environmental or genetic cause. The most common type of CJD is the sporadic type and, according to the National Institute of Health, is responsible for 85 percent of the cases.

CJD is so rare that the doctors were surprised four different cases of the disease occurred in a time span of just 6 months. The number of cases brought in, based on the population of the Rochester area was higher than expected. This is when the doctors came across a case tied to squirrel brains.

The MRI finding and a test showed specific proteins in the cerebrospinal fluid of the patient, indicating the disease. However, the disease can only be confirmed by testing brain tissue on autopsy after death. Unfortunately, the patient passed away after being diagnosed of the disease. It is important to promptly diagnose CJD because any delay can cause the infectious prions to contaminate the equipment used on patients with the disease. This can cause a transmission of the condition to other if the equipment is not cleaned properly. CJD is rare, therefore, diagnosis may be delayed. Moreover, once the doctor suggests CJD, it takes around 2 weeks to get the test results. In addition to this, the case highlights an importance of a need for doctors to keep a differential diagnosis of CJD in mind, moreover, for hospitals to have policies concerning infection control when it comes to CJD.