Pedro-Cuesta, Jesus deGlatzel, MarkusAlmazan-Isla, JavierStoeck, KatharinaMellina, VittorioPuopolo, MariaPocchiari, MaurizioZerr, IngaKretszchmar, Hans ABrandel, Jean-PhilippeDelasnerie-Lauprêtre, NicoleAlpérovitch, AnnickVan Duijn, CorneliaSánchez-Juan, PascualCollins, StevenLewis, VictoriaJansen, Gerard HCoulthart, Michael BGelpi, EllenBudka, HerbertMitrova, Eva2019-01-312019-01-312006-11-10BMC Public Health. 2006 Nov 10;6:278.1471-2458http://hdl.handle.net/20.500.12105/7046BACKGROUND: The objective of this study was to describe the diagnostic panorama of human transmissible spongiform encephalopathies across 11 countries. METHODS: From 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. RESULTS: In 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). CONCLUSION: Considerable 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.engVoRhttp://creativecommons.org/licenses/by/4.0/AustraliaCanadaCreutzfeldt-Jakob SyndromeCross-Cultural ComparisonElectroencephalographyEuropeFranceGenotypeHumansIatrogenic DiseaseInternationalityItalyMagnetic Resonance ImagingPopulation SurveillancePrion ProteinsPrionsRegistriesSlovakiaTimeUnited KingdomAtribución 4.0 Internacional170968296127810.1186/1471-2458-6-278BMC public healthopen access