Creutzfeldt-Jakob disease (CJD) is a rare and ultimately fatal degenerative brain disease. It is one of a group of diseases called Transmissible Spongiform Encephalopathies (TSEs) that affect humans and animals. TSEs are thought to be caused by the build up in the brain of an abnormal form of the naturally occurring 'prion' protein.
CJD was initially described in its classical, or sporadic form, in 1920. A new variant known as variant CJD (vCJD) was first identified in 1996. Variant CJD is strongly linked to exposure, probably through food, to a TSE of cattle called Bovine Spongiform Encephalopathy (BSE). Most (85%) cases of CJD are sporadic, with no known cause, and occur worldwide at a rate of about 1 case per million population per year. There are also inherited forms of CJD (10-15%) and those which have been acquired from another source.
Acquired CJD includes iatrogenic CJD and vCJD. Iatrogenic CJD is very rare, and occurs when CJD is accidentally transmitted during medical or surgical procedures. Although there have been no reported cases of vCJD having been transmitted as a result of surgical procedures, the possibility cannot be ruled out. Precautionary measures have been taken to reduce such a risk by improving the standards of decontamination services for surgical instruments.