10/19/18 National Prion Disease Pathology Surveillance Center Response to ProMed’s recent article suggesting that there is a connection between human prion disease and squirrel brain: “The article reproduced recently in ProMed suggested that a US patient developed variant Creutzfeldt-Jakob disease (vCJD). 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.
