In a follow-up to a report of a poster presented at IDWeek2018 concerning a relatively recent case of vCJD linked to the consumption of squirrel brains, an expert from National Prion Disease Pathology Surveillance Center at Case Western Reserve University School of Medicine said the link was “unjustifiably speculative”.
In a letter to ProMED mail, Brian Appleby, MD writes:
“This diagnosis was based on MRI results that reportedly appeared similar to those of patients with variant CJD (vCJD). However, all other clinical and demographic evidence (triphasic EEG pattern, patient age, disease duration, and, subsequently, prion protein codon 129 genotype) strongly suggested that this was a case of sporadic Creutzfeldt-Jakob disease (sCJD). Indeed, through national human prion disease surveillance, the patient was confirmed through autopsy as sCJD, not vCJD. The reporting of this case as probable vCJD — a disease linked to consumption of beef contaminated with bovine spongiform encephalopathy (BSE, also known as “mad cow disease”) — and suggesting that this case might be etiologically linked to squirrel consumption is inappropriate and may cause undue alarm.
“While prion diseases have been identified in several other types of mammals, they have never been identified in squirrels. Without additional experimental or epidemiological evidence, a link between consumption of squirrel brain and human prion disease is unjustifiably speculative.”
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