Is there evidence for exogenous risk factors in the aetiology and spread of Creutzfeldt-Jakob disease?
From the Welsh Combined Centres for Public Health, University of Wales College of Medicine, Cardiff, UK Introduction The transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases of unknown aetiology, and affect both animals and man. As a group, they have several unique features that separate them from other neurodegenerative conditions, notably characteristic neuropathology and the ability to be transmitted to experimental laboratory animals by inoculation. Creutzfeldt-Jakob disease is a rapidly progressive dementia and is uniformly fatal. There appears to be a genetic basis in 10 15% cases, termed familial, and a small number of cases are associated with medical interventions and are termed iatrogenic. The majority of cases appear to arise spontaneously and are, thus, termed sporadic Creutzfeldt-Jakob disease (spCJD). Recently, a number of atypical cases of CJD have arisen, almost exclusively in young people in the UK. This new disease has been termed vari
