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BackgroundThe objective of this study was to describe the diagnostic panorama of human transmissible spongiform encephalopathies across 11 countries.MethodsFrom data collected for surveillance purposes, we describe annual proportions of deaths due to different human transmissible spongiform encephalopathies in eleven EUROCJD-consortium countries over the period 1993-2002, as well as variations in the use of diagnostic tests. Using logistic models we quantified international differences and changes across time.ResultsIn general, pre-mortem use of diagnostic investigations increased with time. International differences in pathological confirmation of sporadic Creutzfeldt-Jakob disease, stable over time, were evident. Compared to their counterparts, some countries displayed remarkable patterns, such as: 1) the high proportion, increasing with time, of variant Creutzfeldt-Jakob disease in the United Kingdom, (OR 607.99 95% CI 84.72-4363.40), and France (OR 18.35, 95% CI 2.20-152.83); 2) high, decreasing proportions of iatrogenic Creutzfeldt-Jakob disease in France, (OR 5.81 95% CI 4.09-8.24), and the United Kingdom, (OR 1.54 95% CI 1.03-2.30); and, 3) high and stable ratios of genetic forms in Slovakia (OR 21.82 95% CI 12.42-38.33) and Italy (OR 2.12 95% CI 1.69-2.68).ConclusionConsiderable international variation in aetiological subtypes of human transmissible spongiform encephalopathies was evident over the observation period. With the exception of variant Creutzfeldt-Jakob disease and iatrogenic Creutzfeldt-Jakob disease in France and the United Kingdom, these differences persisted across time.

Original publication




Journal article


BMC public health

Publication Date





Instituto de Salud Carlos III, Centro Nacional de Epidemiologia, Departamento de Epidemiologia Aplicada, Calle Sinesio Delgado 6, 28029, Madrid, Spain.


Humans, Creutzfeldt-Jakob Syndrome, Iatrogenic Disease, Prions, Magnetic Resonance Imaging, Electroencephalography, Population Surveillance, Registries, Genotype, Time, Cross-Cultural Comparison, Internationality, Canada, Australia, Europe, Slovakia, France, Italy, United Kingdom, Prion Proteins