Variant Creutzfeldt-Jakob disease with extremely low lymphoreticular deposition of prion protein.

Importance: Human transmission of bovine spongiform encephalopathy causes the fatal neurodegenerative condition variant Creutzfeldt-Jakob disease (vCJD) and, based on recent human prevalence studies, significant subclinical prion infection of the UK population. To date, all clinical cases have been fatal, totaling 228 mostly young adults residing in the United Kingdom.

Observations: Here we describe the investigation and case history of a patient recently diagnosed as having vCJD in the United Kingdom.