Epidemiology of Creutzfeldt-Jakob Disease: Incidence. risk factors and sulVival in European studies

CREUTZFELDT-JAKOB DISEASE (CJO) is a rare neurodegenerative disorder with a highly interesting aetiology and potentially important public health implications. l In aetiological terms, CJD is one of the human prion diseases, characterised by rapid neurodegeneration leading to a characteristic spongiform encephalopathy.' From a publlc health perspective, CJO is of interest because of the possible link between the bovine spongifonl1 encephalopathy (BSE) and CJO.3 In this thesis studies on the incidence, risk factors and prognosis of CJO are reported. It is based on a collaborative European study In which the incidence of CJO was investigated in various European countries on the basis of national registries. In addition a collaborative case-control study of determinants of CJO was performed in the European countries with a CJO register. And finally, the sUlvival of CJO patients was estimated on the basis of patients in the registers.

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