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de Pedro-Cuesta, Jesus; Glatzel, Markus; Almazan, Javier; Stoeck, Katharina; Mellina, Vittorio; Puopolo, Maria; Pocchiari, Maurizio; Zerr, Inga; Kretszchmar, Hans A.; Brandel, Jean-Philippe; Delasnerie-Laupretre, Nicole; Alperovitch, Annick; Van Duijn, Cornelia; Sanchez-Juan, Pascual; Collins, Steven; Lewis, Victoria; Jansen, Gerard H.; Coulthart, Michael B.; Gelpi, Ellen; Budka, Herbert and Mitrova, Eva (2006): Human transmissible spongiform encephalopathies in eleven countries: diagnostic pattern across time, 1993-2002. In: BMC Public Health 6:278 [PDF, 348kB]


Background: 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.

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