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New Creutzfeldt-Jakob Disease cases raise questions

New Creutzfeldt-Jakob Disease cases raise questions

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Mad cow disease (BSE or bovine spongiform encephalopathy) and the related Creutzfeldt-Jakob Disease (CJD) found in humans are back in the news. In the southern Italian region of Puglia, two people died last month from confirmed cases of Creutzfeldt-Jacob Disease and beef contaminated with “mad cow” is considered a possible cause since they shopped at the same grocery outlets.

Meanwhile, two people in Tampa have been diagnosed with Creutzfeldt-Jakob Disease with the cause undetermined. 

Fears of  CJD and rumors that BSE is in the US food supply are a huge threat to domestic and export beef markets. When the first domestic US “mad cow” was discovered in late 2003, US export trade evaporated overnight.

Whether CJD is sporadic, genetic or variant, caused from infected meat, cannot be determined without a brain biopsy, usually after death. That is why when public health officials and meat industry representatives assure the public that a case of CJD is “not from eating meat” it is not comforting. 

In fact, the difficulty of getting a diagnosis of what kind of CJD a patient has is shown in the case of Patrick Hicks, who died of apparent CJD in California in 2004 at the age of 49. 

Ron Bailey, a neurologist at Riverside Medical Center in Riverside, California, who treated Hicks, said that “clinically, the case did look like it was variant CJD [from meat]—no question about that.” However, the National Prion Disease Pathology Surveillance Center (NPDPSC) in Cleveland ruled Hick’s condition sporadic CJD, not from meat, after receiving a brain sample.

When Dr. Bailey asked the Prion Center to perform a more accurate, genetic test he was told there was no frozen brain sample from Hicks, which violated the center’s own protocols that require a frozen sample. A different Prion Center employee told Bailey the entire brain had been “fixed in formalin” by someone other than the center, so genetic analysis was impossible.

Dr. Bailey was then told by an employee that the center had actually used 1-800-Autopsy (not a joke) of Los Angeles to collect the brain sample, reported United Press International. A spokesperson at 1-800-Autopsy confirmed to Bailey that the company had sent Hicks’s sample but said his and all samples are never frozen because “we don’t have the capability to freeze it.” The required refrigeration equipment was too expensive, said the employee.

The Prion Center was also confirmed to have known that 1-800-Autopsy did not have the ability to freeze brain tissue when it ordered the sample, said the employee. When Bailey asked the Prion Center why it would use a supplier unable to follow its own protocols, he got no answer.

The Prion Center was not the only entity that could not or would not confirm the type of CJD a patient had. The Riverside County coroner’s office refused to conduct an autopsy on Hicks even after the Prion Center phoned them, said Dr. Bailey, which is what initially led to the use of 1-800-Autopsy’s services  “If you have a condition that is this rare with the potential to be the first variant CJD case in the state, it raises questions in my mind why they would be so reluctant to do an autopsy on him,” observed Dr. Bailey. 

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