Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada
Identifieur interne : 00A544 ( Main/Exploration ); précédent : 00A543; suivant : 00A545Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada
Auteurs : A. Ladogana [Italie] ; M. Puopolo [Italie] ; E. A. Croes [Pays-Bas] ; H. Budka [Autriche] ; C. Jarius [Autriche] ; S. Collins [Australie] ; G. M. Klug [Australie] ; T. Sutcliffe [Canada] ; A. Giulivi [Canada] ; A. Alperovitch [France] ; N. Delasnerie-Laupretre [France] ; J.-P. Brandel [France] ; S. Poser [Allemagne] ; H. Kretzschmar [Allemagne] ; I. Rietveld [Pays-Bas] ; E. Mitrova [Slovaquie] ; J. De Pedro Cuesta [Espagne] ; P. Martinez-Martin [Espagne] ; M. Glatzel [Swaziland] ; A. Aguzzi [Swaziland] ; R. Knight [Royaume-Uni] ; H. Ward [Royaume-Uni] ; M. Pocchiari [Italie] ; C. M. Van Duijn [Pays-Bas] ; R. G. Will [Royaume-Uni] ; I. Zerr [Allemagne]Source :
- Neurology [ 0028-3878 ] ; 2005.
Descripteurs français
- Pascal (Inist)
- Wicri :
English descriptors
- KwdEn :
Abstract
Background: An international study of the epidemiologic characteristics of Creutzfeldt-Jakob disease (CJD) was established in 1993 and included national registries in France, Germany, Italy, the Netherlands, Slovakia, and the United Kingdom. In 1997, the study was extended to Australia, Austria, Canada, Spain, and Switzerland. Methods: Data were pooled from all participating countries for the years 1993 to 2002 and included deaths from definite or probable CJD of all etiologic subtypes. Results: Four thousand four hundred forty-one cases were available for analysis and included 3,720 cases of sporadic CJD, 455 genetic cases, 138 iatrogenic cases, and 128 variant cases. The overall annual mortality rate between 1999 and 2002 was 1.67 per million for all cases and 1.39 per million for sporadic CJD. Mortality rates were similar in all countries. There was heterogeneity in the distribution of cases by etiologic subtype with an excess of genetic cases in Italy and Slovakia, of iatrogenic cases in France and the UK, and of variant CJD in the UK. Conclusions: This study has established overall epidemiologic characteristics for Creutzfeldt-Jakob disease (CJD) of all types in a multinational population-based study. Intercountry comparisons did not suggest any relative change in the characteristics of sporadic CJD in the United Kingdom, and the evidence in this study does not suggest the occurrence of a novel form of human bovine spongiform encephalopathy infection other than variant CJD. However, this remains a possibility, and countries currently unaffected by variant CJD may yet have cases.
Affiliations:
- Allemagne, Australie, Autriche, Canada, Espagne, France, Italie, Pays-Bas, Royaume-Uni, Slovaquie, Swaziland
- Basse-Saxe, Bavière, Communauté de Madrid, District de Haute-Bavière, Hollande-Méridionale, Latium, Victoria (État), Vienne (Autriche), Écosse, Île-de-France
- Göttingen, Madrid, Melbourne, Munich, Paris, Rome, Rotterdam, Vienne (Autriche), Édimbourg
- Université Louis-et-Maximilien de Munich, Université de Göttingen, Université de Melbourne
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Collins</name><affiliation wicri:level="4"><inist:fA14 i1="04"><s1>Australian National CJD Registry, Department of Pathology, University of Melbourne</s1><s2>Victoria</s2><s3>AUS</s3><sZ>6 aut.</sZ><sZ>7 aut.</sZ></inist:fA14><country>Australie</country><placeName><settlement type="city">Melbourne</settlement><region type="état">Victoria (État)</region></placeName><orgName type="university">Université de Melbourne</orgName></affiliation></author><author><name sortKey="Klug, G M" sort="Klug, G M" uniqKey="Klug G" first="G. M." last="Klug">G. M. Klug</name><affiliation wicri:level="4"><inist:fA14 i1="04"><s1>Australian National CJD Registry, Department of Pathology, University of Melbourne</s1><s2>Victoria</s2><s3>AUS</s3><sZ>6 aut.</sZ><sZ>7 aut.</sZ></inist:fA14><country>Australie</country><placeName><settlement type="city">Melbourne</settlement><region type="état">Victoria (État)</region></placeName><orgName type="university">Université de Melbourne</orgName></affiliation></author><author><name sortKey="Sutcliffe, T" sort="Sutcliffe, T" uniqKey="Sutcliffe T" first="T." last="Sutcliffe">T. Sutcliffe</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Blood Safety Surveillance and Health Care Acquired Infections Division Centre for Infectious Disease Prevention and Control</s1><s2>Ottawa, Ontario</s2><s3>CAN</s3><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>Blood Safety Surveillance and Health Care Acquired Infections Division Centre for Infectious Disease Prevention and Control</wicri:noRegion></affiliation></author><author><name sortKey="Giulivi, A" sort="Giulivi, A" uniqKey="Giulivi A" first="A." last="Giulivi">A. Giulivi</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Blood Safety Surveillance and Health Care Acquired Infections Division Centre for Infectious Disease Prevention and Control</s1><s2>Ottawa, Ontario</s2><s3>CAN</s3><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>Blood Safety Surveillance and Health Care Acquired Infections Division Centre for Infectious Disease Prevention and Control</wicri:noRegion></affiliation></author><author><name sortKey="Alperovitch, A" sort="Alperovitch, A" uniqKey="Alperovitch A" first="A." last="Alperovitch">A. Alperovitch</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>U.360 INSERM, Hopital de la Salpetriere</s1><s2>Paris</s2><s3>FRA</s3><sZ>10 aut.</sZ><sZ>11 aut.</sZ><sZ>12 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Delasnerie Laupretre, N" sort="Delasnerie Laupretre, N" uniqKey="Delasnerie Laupretre N" first="N." last="Delasnerie-Laupretre">N. Delasnerie-Laupretre</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>U.360 INSERM, Hopital de la Salpetriere</s1><s2>Paris</s2><s3>FRA</s3><sZ>10 aut.</sZ><sZ>11 aut.</sZ><sZ>12 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Brandel, J P" sort="Brandel, J P" uniqKey="Brandel J" first="J.-P." last="Brandel">J.-P. Brandel</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>U.360 INSERM, Hopital de la Salpetriere</s1><s2>Paris</s2><s3>FRA</s3><sZ>10 aut.</sZ><sZ>11 aut.</sZ><sZ>12 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Poser, S" sort="Poser, S" uniqKey="Poser S" first="S." last="Poser">S. Poser</name><affiliation wicri:level="4"><inist:fA14 i1="07"><s1>Department of Neurology, Georg-August-Universität Göttingen</s1><s3>DEU</s3><sZ>13 aut.</sZ><sZ>26 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><settlement type="city">Göttingen</settlement><region type="land" nuts="1">Basse-Saxe</region></placeName><orgName type="university">Université de Göttingen</orgName></affiliation></author><author><name sortKey="Kretzschmar, H" sort="Kretzschmar, H" uniqKey="Kretzschmar H" first="H." last="Kretzschmar">H. Kretzschmar</name><affiliation wicri:level="4"><inist:fA14 i1="08"><s1>Institute of Neuropathology, University of Munich</s1><s3>DEU</s3><sZ>14 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><settlement type="city">Munich</settlement><region type="land" nuts="1">Bavière</region><region type="district" nuts="2">District de Haute-Bavière</region></placeName><orgName type="university">Université Louis-et-Maximilien de Munich</orgName></affiliation></author><author><name sortKey="Rietveld, I" sort="Rietveld, I" uniqKey="Rietveld I" first="I." last="Rietveld">I. Rietveld</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Department of Epidemiology and Biostatistics, Erasmus Medical College</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>3 aut.</sZ><sZ>15 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Mitrova, E" sort="Mitrova, E" uniqKey="Mitrova E" first="E." last="Mitrova">E. Mitrova</name><affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Institute of Preventative and Clinical Medicine, National Reference Centre of Slow Virus Neuroinfections</s1><s2>Bratislava</s2><s3>SVK</s3><sZ>16 aut.</sZ></inist:fA14><country>Slovaquie</country><wicri:noRegion>Bratislava</wicri:noRegion></affiliation></author><author><name sortKey="De Pedro Cuesta, J" sort="De Pedro Cuesta, J" uniqKey="De Pedro Cuesta J" first="J." last="De Pedro Cuesta">J. De Pedro Cuesta</name><affiliation wicri:level="3"><inist:fA14 i1="10"><s1>Instituto de Salud Carlos III, Departamento de Epidemiologia Aplicada, Centro Nacional de Epidemiologia</s1><s2>Madrid</s2><s3>ESP</s3><sZ>17 aut.</sZ><sZ>18 aut.</sZ></inist:fA14><country>Espagne</country><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author><author><name sortKey="Martinez Martin, P" sort="Martinez Martin, P" uniqKey="Martinez Martin P" first="P." last="Martinez-Martin">P. Martinez-Martin</name><affiliation wicri:level="3"><inist:fA14 i1="10"><s1>Instituto de Salud Carlos III, Departamento de Epidemiologia Aplicada, Centro Nacional de Epidemiologia</s1><s2>Madrid</s2><s3>ESP</s3><sZ>17 aut.</sZ><sZ>18 aut.</sZ></inist:fA14><country>Espagne</country><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author><author><name sortKey="Glatzel, M" sort="Glatzel, M" uniqKey="Glatzel M" first="M." last="Glatzel">M. Glatzel</name><affiliation wicri:level="1"><inist:fA14 i1="11"><s1>Swiss National Reference Centre for Prion Diseases, University Hospital of Zurich</s1><s3>SWZ</s3><sZ>19 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Swaziland</country><wicri:noRegion>Swiss National Reference Centre for Prion Diseases, University Hospital of Zurich</wicri:noRegion></affiliation></author><author><name sortKey="Aguzzi, A" sort="Aguzzi, A" uniqKey="Aguzzi A" first="A." last="Aguzzi">A. Aguzzi</name><affiliation wicri:level="1"><inist:fA14 i1="11"><s1>Swiss National Reference Centre for Prion Diseases, University Hospital of Zurich</s1><s3>SWZ</s3><sZ>19 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Swaziland</country><wicri:noRegion>Swiss National Reference Centre for Prion Diseases, University Hospital of Zurich</wicri:noRegion></affiliation></author><author><name sortKey="Knight, R" sort="Knight, R" uniqKey="Knight R" first="R." last="Knight">R. Knight</name><affiliation wicri:level="3"><inist:fA14 i1="12"><s1>National CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>21 aut.</sZ><sZ>22 aut.</sZ><sZ>25 aut.</sZ></inist:fA14><country>Royaume-Uni</country><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></affiliation></author><author><name sortKey="Ward, H" sort="Ward, H" uniqKey="Ward H" first="H." last="Ward">H. Ward</name><affiliation wicri:level="3"><inist:fA14 i1="12"><s1>National CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>21 aut.</sZ><sZ>22 aut.</sZ><sZ>25 aut.</sZ></inist:fA14><country>Royaume-Uni</country><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></affiliation></author><author><name sortKey="Pocchiari, M" sort="Pocchiari, M" uniqKey="Pocchiari M" first="M." last="Pocchiari">M. Pocchiari</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Istituto Superiors di Sanità, Department of Cell Biology and Neurosciences-ISS</s1><s2>Rome</s2><s3>ITA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Italie</country><placeName><settlement type="city">Rome</settlement><region nuts="2">Latium</region></placeName></affiliation></author><author><name sortKey="Van Duijn, C M" sort="Van Duijn, C M" uniqKey="Van Duijn C" first="C. M." last="Van Duijn">C. M. Van Duijn</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Department of Epidemiology and Biostatistics, Erasmus Medical College</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>3 aut.</sZ><sZ>15 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Will, R G" sort="Will, R G" uniqKey="Will R" first="R. G." last="Will">R. G. Will</name><affiliation wicri:level="3"><inist:fA14 i1="12"><s1>National CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>21 aut.</sZ><sZ>22 aut.</sZ><sZ>25 aut.</sZ></inist:fA14><country>Royaume-Uni</country><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></affiliation></author><author><name sortKey="Zerr, I" sort="Zerr, I" uniqKey="Zerr I" first="I." last="Zerr">I. Zerr</name><affiliation wicri:level="4"><inist:fA14 i1="07"><s1>Department of Neurology, Georg-August-Universität Göttingen</s1><s3>DEU</s3><sZ>13 aut.</sZ><sZ>26 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><settlement type="city">Göttingen</settlement><region type="land" nuts="1">Basse-Saxe</region></placeName><orgName type="university">Université de Göttingen</orgName></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">05-0242521</idno><date when="2005">2005</date><idno type="stanalyst">PASCAL 05-0242521 INIST</idno><idno type="RBID">Pascal:05-0242521</idno><idno type="wicri:Area/PascalFrancis/Corpus">004959</idno><idno type="wicri:Area/PascalFrancis/Curation">001744</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">004500</idno><idno type="wicri:explorRef" wicri:stream="PascalFrancis" wicri:step="Checkpoint">004500</idno><idno type="wicri:doubleKey">0028-3878:2005:Ladogana A:mortality:from:creutzfeldt</idno><idno type="wicri:Area/Main/Merge">00B150</idno><idno type="wicri:Area/Main/Curation">00A544</idno><idno type="wicri:Area/Main/Exploration">00A544</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Mortality from Creutzfeldt-Jakob disease and related disorders in Europe, Australia, and Canada</title><author><name sortKey="Ladogana, A" sort="Ladogana, A" uniqKey="Ladogana A" first="A." last="Ladogana">A. Ladogana</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Istituto Superiors di Sanità, Department of Cell Biology and Neurosciences-ISS</s1><s2>Rome</s2><s3>ITA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Italie</country><placeName><settlement type="city">Rome</settlement><region nuts="2">Latium</region></placeName></affiliation></author><author><name sortKey="Puopolo, M" sort="Puopolo, M" uniqKey="Puopolo M" first="M." last="Puopolo">M. Puopolo</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Istituto Superiors di Sanità, Department of Cell Biology and Neurosciences-ISS</s1><s2>Rome</s2><s3>ITA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Italie</country><placeName><settlement type="city">Rome</settlement><region nuts="2">Latium</region></placeName></affiliation></author><author><name sortKey="Croes, E A" sort="Croes, E A" uniqKey="Croes E" first="E. A." last="Croes">E. A. Croes</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Department of Epidemiology and Biostatistics, Erasmus Medical College</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>3 aut.</sZ><sZ>15 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Budka, H" sort="Budka, H" uniqKey="Budka H" first="H." last="Budka">H. Budka</name><affiliation wicri:level="3"><inist:fA14 i1="03"><s1>Austrian Reference Centre for Human Prion Diseases (OERPE) and Institute of Neurology</s1><s2>Vienna</s2><s3>AUT</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ></inist:fA14><country>Autriche</country><placeName><settlement type="city">Vienne (Autriche)</settlement><region nuts="2" type="province">Vienne (Autriche)</region></placeName></affiliation></author><author><name sortKey="Jarius, C" sort="Jarius, C" uniqKey="Jarius C" first="C." last="Jarius">C. Jarius</name><affiliation wicri:level="3"><inist:fA14 i1="03"><s1>Austrian Reference Centre for Human Prion Diseases (OERPE) and Institute of Neurology</s1><s2>Vienna</s2><s3>AUT</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ></inist:fA14><country>Autriche</country><placeName><settlement type="city">Vienne (Autriche)</settlement><region nuts="2" type="province">Vienne (Autriche)</region></placeName></affiliation></author><author><name sortKey="Collins, S" sort="Collins, S" uniqKey="Collins S" first="S." last="Collins">S. Collins</name><affiliation wicri:level="4"><inist:fA14 i1="04"><s1>Australian National CJD Registry, Department of Pathology, University of Melbourne</s1><s2>Victoria</s2><s3>AUS</s3><sZ>6 aut.</sZ><sZ>7 aut.</sZ></inist:fA14><country>Australie</country><placeName><settlement type="city">Melbourne</settlement><region type="état">Victoria (État)</region></placeName><orgName type="university">Université de Melbourne</orgName></affiliation></author><author><name sortKey="Klug, G M" sort="Klug, G M" uniqKey="Klug G" first="G. M." last="Klug">G. M. Klug</name><affiliation wicri:level="4"><inist:fA14 i1="04"><s1>Australian National CJD Registry, Department of Pathology, University of Melbourne</s1><s2>Victoria</s2><s3>AUS</s3><sZ>6 aut.</sZ><sZ>7 aut.</sZ></inist:fA14><country>Australie</country><placeName><settlement type="city">Melbourne</settlement><region type="état">Victoria (État)</region></placeName><orgName type="university">Université de Melbourne</orgName></affiliation></author><author><name sortKey="Sutcliffe, T" sort="Sutcliffe, T" uniqKey="Sutcliffe T" first="T." last="Sutcliffe">T. Sutcliffe</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Blood Safety Surveillance and Health Care Acquired Infections Division Centre for Infectious Disease Prevention and Control</s1><s2>Ottawa, Ontario</s2><s3>CAN</s3><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>Blood Safety Surveillance and Health Care Acquired Infections Division Centre for Infectious Disease Prevention and Control</wicri:noRegion></affiliation></author><author><name sortKey="Giulivi, A" sort="Giulivi, A" uniqKey="Giulivi A" first="A." last="Giulivi">A. Giulivi</name><affiliation wicri:level="1"><inist:fA14 i1="05"><s1>Blood Safety Surveillance and Health Care Acquired Infections Division Centre for Infectious Disease Prevention and Control</s1><s2>Ottawa, Ontario</s2><s3>CAN</s3><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>Blood Safety Surveillance and Health Care Acquired Infections Division Centre for Infectious Disease Prevention and Control</wicri:noRegion></affiliation></author><author><name sortKey="Alperovitch, A" sort="Alperovitch, A" uniqKey="Alperovitch A" first="A." last="Alperovitch">A. Alperovitch</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>U.360 INSERM, Hopital de la Salpetriere</s1><s2>Paris</s2><s3>FRA</s3><sZ>10 aut.</sZ><sZ>11 aut.</sZ><sZ>12 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Delasnerie Laupretre, N" sort="Delasnerie Laupretre, N" uniqKey="Delasnerie Laupretre N" first="N." last="Delasnerie-Laupretre">N. Delasnerie-Laupretre</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>U.360 INSERM, Hopital de la Salpetriere</s1><s2>Paris</s2><s3>FRA</s3><sZ>10 aut.</sZ><sZ>11 aut.</sZ><sZ>12 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Brandel, J P" sort="Brandel, J P" uniqKey="Brandel J" first="J.-P." last="Brandel">J.-P. Brandel</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>U.360 INSERM, Hopital de la Salpetriere</s1><s2>Paris</s2><s3>FRA</s3><sZ>10 aut.</sZ><sZ>11 aut.</sZ><sZ>12 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region">Île-de-France</region><region type="old region">Île-de-France</region><settlement type="city">Paris</settlement></placeName></affiliation></author><author><name sortKey="Poser, S" sort="Poser, S" uniqKey="Poser S" first="S." last="Poser">S. Poser</name><affiliation wicri:level="4"><inist:fA14 i1="07"><s1>Department of Neurology, Georg-August-Universität Göttingen</s1><s3>DEU</s3><sZ>13 aut.</sZ><sZ>26 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><settlement type="city">Göttingen</settlement><region type="land" nuts="1">Basse-Saxe</region></placeName><orgName type="university">Université de Göttingen</orgName></affiliation></author><author><name sortKey="Kretzschmar, H" sort="Kretzschmar, H" uniqKey="Kretzschmar H" first="H." last="Kretzschmar">H. Kretzschmar</name><affiliation wicri:level="4"><inist:fA14 i1="08"><s1>Institute of Neuropathology, University of Munich</s1><s3>DEU</s3><sZ>14 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><settlement type="city">Munich</settlement><region type="land" nuts="1">Bavière</region><region type="district" nuts="2">District de Haute-Bavière</region></placeName><orgName type="university">Université Louis-et-Maximilien de Munich</orgName></affiliation></author><author><name sortKey="Rietveld, I" sort="Rietveld, I" uniqKey="Rietveld I" first="I." last="Rietveld">I. Rietveld</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Department of Epidemiology and Biostatistics, Erasmus Medical College</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>3 aut.</sZ><sZ>15 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Mitrova, E" sort="Mitrova, E" uniqKey="Mitrova E" first="E." last="Mitrova">E. Mitrova</name><affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Institute of Preventative and Clinical Medicine, National Reference Centre of Slow Virus Neuroinfections</s1><s2>Bratislava</s2><s3>SVK</s3><sZ>16 aut.</sZ></inist:fA14><country>Slovaquie</country><wicri:noRegion>Bratislava</wicri:noRegion></affiliation></author><author><name sortKey="De Pedro Cuesta, J" sort="De Pedro Cuesta, J" uniqKey="De Pedro Cuesta J" first="J." last="De Pedro Cuesta">J. De Pedro Cuesta</name><affiliation wicri:level="3"><inist:fA14 i1="10"><s1>Instituto de Salud Carlos III, Departamento de Epidemiologia Aplicada, Centro Nacional de Epidemiologia</s1><s2>Madrid</s2><s3>ESP</s3><sZ>17 aut.</sZ><sZ>18 aut.</sZ></inist:fA14><country>Espagne</country><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author><author><name sortKey="Martinez Martin, P" sort="Martinez Martin, P" uniqKey="Martinez Martin P" first="P." last="Martinez-Martin">P. Martinez-Martin</name><affiliation wicri:level="3"><inist:fA14 i1="10"><s1>Instituto de Salud Carlos III, Departamento de Epidemiologia Aplicada, Centro Nacional de Epidemiologia</s1><s2>Madrid</s2><s3>ESP</s3><sZ>17 aut.</sZ><sZ>18 aut.</sZ></inist:fA14><country>Espagne</country><placeName><settlement type="city">Madrid</settlement><region nuts="2" type="region">Communauté de Madrid</region></placeName></affiliation></author><author><name sortKey="Glatzel, M" sort="Glatzel, M" uniqKey="Glatzel M" first="M." last="Glatzel">M. Glatzel</name><affiliation wicri:level="1"><inist:fA14 i1="11"><s1>Swiss National Reference Centre for Prion Diseases, University Hospital of Zurich</s1><s3>SWZ</s3><sZ>19 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Swaziland</country><wicri:noRegion>Swiss National Reference Centre for Prion Diseases, University Hospital of Zurich</wicri:noRegion></affiliation></author><author><name sortKey="Aguzzi, A" sort="Aguzzi, A" uniqKey="Aguzzi A" first="A." last="Aguzzi">A. Aguzzi</name><affiliation wicri:level="1"><inist:fA14 i1="11"><s1>Swiss National Reference Centre for Prion Diseases, University Hospital of Zurich</s1><s3>SWZ</s3><sZ>19 aut.</sZ><sZ>20 aut.</sZ></inist:fA14><country>Swaziland</country><wicri:noRegion>Swiss National Reference Centre for Prion Diseases, University Hospital of Zurich</wicri:noRegion></affiliation></author><author><name sortKey="Knight, R" sort="Knight, R" uniqKey="Knight R" first="R." last="Knight">R. Knight</name><affiliation wicri:level="3"><inist:fA14 i1="12"><s1>National CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>21 aut.</sZ><sZ>22 aut.</sZ><sZ>25 aut.</sZ></inist:fA14><country>Royaume-Uni</country><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></affiliation></author><author><name sortKey="Ward, H" sort="Ward, H" uniqKey="Ward H" first="H." last="Ward">H. Ward</name><affiliation wicri:level="3"><inist:fA14 i1="12"><s1>National CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>21 aut.</sZ><sZ>22 aut.</sZ><sZ>25 aut.</sZ></inist:fA14><country>Royaume-Uni</country><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></affiliation></author><author><name sortKey="Pocchiari, M" sort="Pocchiari, M" uniqKey="Pocchiari M" first="M." last="Pocchiari">M. Pocchiari</name><affiliation wicri:level="3"><inist:fA14 i1="01"><s1>Istituto Superiors di Sanità, Department of Cell Biology and Neurosciences-ISS</s1><s2>Rome</s2><s3>ITA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ><sZ>23 aut.</sZ></inist:fA14><country>Italie</country><placeName><settlement type="city">Rome</settlement><region nuts="2">Latium</region></placeName></affiliation></author><author><name sortKey="Van Duijn, C M" sort="Van Duijn, C M" uniqKey="Van Duijn C" first="C. M." last="Van Duijn">C. M. Van Duijn</name><affiliation wicri:level="3"><inist:fA14 i1="02"><s1>Department of Epidemiology and Biostatistics, Erasmus Medical College</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>3 aut.</sZ><sZ>15 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Pays-Bas</country><placeName><settlement type="city">Rotterdam</settlement><region nuts="2" type="province">Hollande-Méridionale</region></placeName></affiliation></author><author><name sortKey="Will, R G" sort="Will, R G" uniqKey="Will R" first="R. G." last="Will">R. G. Will</name><affiliation wicri:level="3"><inist:fA14 i1="12"><s1>National CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>21 aut.</sZ><sZ>22 aut.</sZ><sZ>25 aut.</sZ></inist:fA14><country>Royaume-Uni</country><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></affiliation></author><author><name sortKey="Zerr, I" sort="Zerr, I" uniqKey="Zerr I" first="I." last="Zerr">I. Zerr</name><affiliation wicri:level="4"><inist:fA14 i1="07"><s1>Department of Neurology, Georg-August-Universität Göttingen</s1><s3>DEU</s3><sZ>13 aut.</sZ><sZ>26 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><settlement type="city">Göttingen</settlement><region type="land" nuts="1">Basse-Saxe</region></placeName><orgName type="university">Université de Göttingen</orgName></affiliation></author></analytic><series><title level="j" type="main">Neurology</title><title level="j" type="abbreviated">Neurology</title><idno type="ISSN">0028-3878</idno><imprint><date when="2005">2005</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Neurology</title><title level="j" type="abbreviated">Neurology</title><idno type="ISSN">0028-3878</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Australia</term><term>Canada</term><term>Creutzfeldt Jakob disease</term><term>Europe</term><term>Mortality</term><term>Nervous system diseases</term><term>Prion</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Système nerveux pathologie</term><term>Mortalité</term><term>Encéphalopathie spongiforme Creutzfeldt Jakob</term><term>Europe</term><term>Australie</term><term>Canada</term><term>Prion</term></keywords><keywords scheme="Wicri" type="geographic" xml:lang="fr"><term>Australie</term><term>Canada</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Mortalité</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Background: An international study of the epidemiologic characteristics of Creutzfeldt-Jakob disease (CJD) was established in 1993 and included national registries in France, Germany, Italy, the Netherlands, Slovakia, and the United Kingdom. In 1997, the study was extended to Australia, Austria, Canada, Spain, and Switzerland. Methods: Data were pooled from all participating countries for the years 1993 to 2002 and included deaths from definite or probable CJD of all etiologic subtypes. Results: Four thousand four hundred forty-one cases were available for analysis and included 3,720 cases of sporadic CJD, 455 genetic cases, 138 iatrogenic cases, and 128 variant cases. The overall annual mortality rate between 1999 and 2002 was 1.67 per million for all cases and 1.39 per million for sporadic CJD. Mortality rates were similar in all countries. There was heterogeneity in the distribution of cases by etiologic subtype with an excess of genetic cases in Italy and Slovakia, of iatrogenic cases in France and the UK, and of variant CJD in the UK. Conclusions: This study has established overall epidemiologic characteristics for Creutzfeldt-Jakob disease (CJD) of all types in a multinational population-based study. Intercountry comparisons did not suggest any relative change in the characteristics of sporadic CJD in the United Kingdom, and the evidence in this study does not suggest the occurrence of a novel form of human bovine spongiform encephalopathy infection other than variant CJD. However, this remains a possibility, and countries currently unaffected by variant CJD may yet have cases.</div></front></TEI><affiliations><list><country><li>Allemagne</li><li>Australie</li><li>Autriche</li><li>Canada</li><li>Espagne</li><li>France</li><li>Italie</li><li>Pays-Bas</li><li>Royaume-Uni</li><li>Slovaquie</li><li>Swaziland</li></country><region><li>Basse-Saxe</li><li>Bavière</li><li>Communauté de Madrid</li><li>District de Haute-Bavière</li><li>Hollande-Méridionale</li><li>Latium</li><li>Victoria (État)</li><li>Vienne (Autriche)</li><li>Écosse</li><li>Île-de-France</li></region><settlement><li>Göttingen</li><li>Madrid</li><li>Melbourne</li><li>Munich</li><li>Paris</li><li>Rome</li><li>Rotterdam</li><li>Vienne (Autriche)</li><li>Édimbourg</li></settlement><orgName><li>Université Louis-et-Maximilien de Munich</li><li>Université de Göttingen</li><li>Université de Melbourne</li></orgName></list><tree><country name="Italie"><region name="Latium"><name sortKey="Ladogana, A" sort="Ladogana, A" uniqKey="Ladogana A" first="A." last="Ladogana">A. Ladogana</name></region><name sortKey="Pocchiari, M" sort="Pocchiari, M" uniqKey="Pocchiari M" first="M." last="Pocchiari">M. Pocchiari</name><name sortKey="Puopolo, M" sort="Puopolo, M" uniqKey="Puopolo M" first="M." last="Puopolo">M. Puopolo</name></country><country name="Pays-Bas"><region name="Hollande-Méridionale"><name sortKey="Croes, E A" sort="Croes, E A" uniqKey="Croes E" first="E. A." last="Croes">E. A. Croes</name></region><name sortKey="Rietveld, I" sort="Rietveld, I" uniqKey="Rietveld I" first="I." last="Rietveld">I. Rietveld</name><name sortKey="Van Duijn, C M" sort="Van Duijn, C M" uniqKey="Van Duijn C" first="C. M." last="Van Duijn">C. M. Van Duijn</name></country><country name="Autriche"><region name="Vienne (Autriche)"><name sortKey="Budka, H" sort="Budka, H" uniqKey="Budka H" first="H." last="Budka">H. Budka</name></region><name sortKey="Jarius, C" sort="Jarius, C" uniqKey="Jarius C" first="C." last="Jarius">C. Jarius</name></country><country name="Australie"><region name="Victoria (État)"><name sortKey="Collins, S" sort="Collins, S" uniqKey="Collins S" first="S." last="Collins">S. Collins</name></region><name sortKey="Klug, G M" sort="Klug, G M" uniqKey="Klug G" first="G. M." last="Klug">G. M. Klug</name></country><country name="Canada"><noRegion><name sortKey="Sutcliffe, T" sort="Sutcliffe, T" uniqKey="Sutcliffe T" first="T." last="Sutcliffe">T. Sutcliffe</name></noRegion><name sortKey="Giulivi, A" sort="Giulivi, A" uniqKey="Giulivi A" first="A." last="Giulivi">A. Giulivi</name></country><country name="France"><region name="Île-de-France"><name sortKey="Alperovitch, A" sort="Alperovitch, A" uniqKey="Alperovitch A" first="A." last="Alperovitch">A. Alperovitch</name></region><name sortKey="Brandel, J P" sort="Brandel, J P" uniqKey="Brandel J" first="J.-P." last="Brandel">J.-P. Brandel</name><name sortKey="Delasnerie Laupretre, N" sort="Delasnerie Laupretre, N" uniqKey="Delasnerie Laupretre N" first="N." last="Delasnerie-Laupretre">N. Delasnerie-Laupretre</name></country><country name="Allemagne"><region name="Basse-Saxe"><name sortKey="Poser, S" sort="Poser, S" uniqKey="Poser S" first="S." last="Poser">S. Poser</name></region><name sortKey="Kretzschmar, H" sort="Kretzschmar, H" uniqKey="Kretzschmar H" first="H." last="Kretzschmar">H. Kretzschmar</name><name sortKey="Zerr, I" sort="Zerr, I" uniqKey="Zerr I" first="I." last="Zerr">I. Zerr</name></country><country name="Slovaquie"><noRegion><name sortKey="Mitrova, E" sort="Mitrova, E" uniqKey="Mitrova E" first="E." last="Mitrova">E. Mitrova</name></noRegion></country><country name="Espagne"><region name="Communauté de Madrid"><name sortKey="De Pedro Cuesta, J" sort="De Pedro Cuesta, J" uniqKey="De Pedro Cuesta J" first="J." last="De Pedro Cuesta">J. De Pedro Cuesta</name></region><name sortKey="Martinez Martin, P" sort="Martinez Martin, P" uniqKey="Martinez Martin P" first="P." last="Martinez-Martin">P. Martinez-Martin</name></country><country name="Swaziland"><noRegion><name sortKey="Glatzel, M" sort="Glatzel, M" uniqKey="Glatzel M" first="M." last="Glatzel">M. Glatzel</name></noRegion><name sortKey="Aguzzi, A" sort="Aguzzi, A" uniqKey="Aguzzi A" first="A." last="Aguzzi">A. Aguzzi</name></country><country name="Royaume-Uni"><region name="Écosse"><name sortKey="Knight, R" sort="Knight, R" uniqKey="Knight R" first="R." last="Knight">R. Knight</name></region><name sortKey="Ward, H" sort="Ward, H" uniqKey="Ward H" first="H." last="Ward">H. Ward</name><name sortKey="Will, R G" sort="Will, R G" uniqKey="Will R" first="R. G." last="Will">R. G. Will</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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