Variant Creutzfeldt-Jakob Disease

**Definition:** A disease of the brain, that is suspected to be caused by a prion associated with Bovine Spongiform Encephalopathy. This disease is characterised by a long incubation period, psychiatric symptoms followed by neurological deficits, and is fatal. Transmission may be by ingestion of food (with a bovine origin) contaminated with infected brain or spinal cord from an infected cow, or blood transfusion. Confirmation is by pathological examination of the brain.

**Long definition:** Variant Creutzfeld-Jakob Disease (vCJD) was first described in a young patient in 1996 and has been reported mainly in the United Kingdom and other European countries. vCJD is thought to be caused by oral intake of food contaminated with materials from cattle with bovine spongiform encephalopathy (BSE). vCJD patients has steadily decreased in number in parallel with reduction of number of BSE cattle. There have been cases of vCJD due to blood transfusion from donors with vCJD.