Variant Creutzfeldt-Jakob disease

Abstract

Variant CJD (vCJD) was described first in the United Kingdom in 1996. It is a zoonotic form of human prion disease, originating from dietary contamination of human food with material from bovine spongiform encephalopathy (BSE)-affected cattle. It has important epidemiologic, clinical, and neuropathogic differences from other forms of human prion disease. Cases have occurred in several countries but the United Kingdom and France have been most affected. Following the decline in BSE in cattle and the dietary protective measures adopted, vCJD has become an extremely rare disease. However, important concerns remain about asymptomatic infection in human populations (especially the United Kingdom) and the possibility of human-to-human transmission via medical and surgical interventions. Definitive diagnosis depends on neuropathology, usually undertaken at autopsy, but sometimes on brain biopsy. Clinical diagnosis with a reasonable degree of likelihood is, however, possible based on the clinical features and the finding of the pulvinar sign on cerebral magnetic resonance. There are also emerging tests (including blood tests) that have promising sensitivity and specificity for vCJD. It is a progressive illness, inevitably fatal with no curative treatment.

Keywords: Creutzfeldt–Jakob disease; bovine spongiform encephalopthy; human prion disease; variant CJD.