CREUTZFELDT-JAKOB DISEASE (CJD) CJD is a rare and fatal neurodegenerative disease fi rst described in the 1920s. It has historically been classifi ed within the dementia syndromes because the symptoms that occur over the disease progression contain all of those that are experienced in other dementia syndromes, but at an accelerated pace. It is recognised as both a dementia syndrome and an acute neurological illness. CJD belongs to a group of diseases known as transmissible spongiform encephalopathies (TSE) that are known to occur in both humans and animals. Th e progress of multifocal encephalopathies is aggressive and brain damage that occurs is characterised by the spongy appearance of brain tissue seen under a microscope. It is also referred to as prion disease, an acronym for a proteinaceous infectious agent. Th e prion theory suggests that the infective agent of CJD is only composed of protein and does not contain nucleic acid, which would be necessary if the agent was a conventional virus. Th e transmittable agent is either genetic or an abnormal form of prion protein that causes aggregates of cellular protein to accumulate in the brain. Th e exact process that occurs that leads to brain damage from original inoculation/contamination is incompletely understood.1,2,3,4
Four types of CJD have been identifi ed: 1 iatrogenic 2 inherited 3 sporadic 4 variant CJD.