Human transmissible spongiform encephalopathies in eleven countries: diagnostic pattern across time, 1993-2002.
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).


CONCLUSIONS
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.
de Pedro-Cuesta
Jesús
J
Instituto de Salud Carlos III, Centro Nacional de Epidemiologia, Departamento de Epidemiologia Aplicada, Calle Sinesio Delgado 6, 28029, Madrid, Spain. jpedro@isciii.es
Glatzel
Markus
M
Almazán
Javier
J
Stoeck
Katharina
K
Mellina
Vittorio
V
Puopolo
Maria
M
Pocchiari
Maurizio
M
Zerr
Inga
I
Kretszchmar
Hans A
HA
Brandel
Jean-Philippe
JP
Delasnerie-Lauprêtre
Nicole
N
Alpérovitch
Annick
A
Van Duijn
Cornelia
C
Sanchez-Juan
Pascual
P
Collins
Steven
S
Lewis
Victoria
V
Jansen
Gerard H
GH
Coulthart
Michael B
MB
Gelpi
Ellen
E
Budka
Herbert
H
Mitrova
Eva
E
D001315:Australia / Q000453:epidemiology; D002170:Canada / Q000453:epidemiology; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000401:mortality* / Q000635:transmission; D003431:Cross-Cultural Comparison; D004569:Electroencephalography; D005060:Europe; D005602:France / Q000453:epidemiology; D005838:Genotype; D006801:Humans; D007049:Iatrogenic Disease / Q000453:epidemiology; D038622:Internationality; D007558:Italy / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D011159:Population Surveillance / Q000379:methods*; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics* / Q000472:pathogenicity; D012042:Registries; D018154:Slovakia / Q000453:epidemiology; D013995:Time; D006113:United Kingdom / Q000453:epidemiology
C512656:PRNP protein, human; D000072002:Prion Proteins; D011328:Prions