Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease
Identifieur interne : 007F91 ( Main/Exploration ); précédent : 007F90; suivant : 007F92Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease
Auteurs : I. Zerr [Allemagne] ; K. Kallenberg [Allemagne] ; D. M. Summers [Royaume-Uni] ; C. Romero [Argentine] ; A. Taratuto [Argentine] ; U. Heinemann [Allemagne] ; M. Breithaupt [Allemagne] ; D. Varges [Allemagne] ; B. Meissner [Allemagne] ; A. Ladogana [Italie] ; M. Schuur [Pays-Bas] ; S. Haik [France] ; S. J. Collins [Australie] ; Gerard H. Jansen [Canada] ; G. B. Stokin [Slovénie] ; J. Pimentel [Portugal] ; E. Hewer [Suisse] ; D. Collie [Royaume-Uni] ; P. Smith [Australie] ; H. Roberts [Australie] ; J. P. Brandel [France] ; C. Van Duijn [Pays-Bas] ; M. Pocchiari [Italie] ; C. Begue [Argentine] ; P. Cras [Belgique] ; R. G. Will [Royaume-Uni] ; P. Sanchez-Juan [Espagne]Source :
- Brain [ 0006-8950 ] ; 2009.
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- Pascal (Inist)
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Abstract
Several molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt-Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt-Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Patients with sporadic Creutzfeldt-Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as 'suspected sporadic Creutzfeldt-Jakob disease' but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt-Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt-Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic resonance imaging was positive in 83% of cases. In all definite cases, the amended criteria would cover the vast majority of suspected cases, being positive in 98%. Cerebral cortical signal increase and high signal in caudate nucleus and putamen on fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging are useful in the diagnosis of sporadic Creutzfeldt-Jakob disease. We propose an amendment to the clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease to include findings from magnetic resonance imaging scans.
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Affiliations:
- Allemagne, Argentine, Australie, Belgique, Canada, Espagne, France, Italie, Pays-Bas, Portugal, Royaume-Uni, Slovénie, Suisse
- Hollande-Méridionale, Latium, Province d'Anvers, Région flamande, Victoria (État), Écosse, Île-de-France
- Anvers, Melbourne, Rome, Rotterdam, Édimbourg
- Université d'Anvers, Université de Melbourne
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Taratuto</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Departamento de Neuropatologia, Centro de Referencia de Encefalopatias Espongiformes Transmisibles, Instituto de Investigaciones Neurologicas/FLENI Montañeses 2325</s1><s2>C1428AQK, Buenos Aires</s2><s3>ARG</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Argentine</country><wicri:noRegion>C1428AQK, Buenos Aires</wicri:noRegion></affiliation></author><author><name sortKey="Heinemann, U" sort="Heinemann, U" uniqKey="Heinemann U" first="U." last="Heinemann">U. Heinemann</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>National TSE Reference Center, Department of Neurology, Georg-August University Goettingen</s1><s2>Goettingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Goettingen</wicri:noRegion><wicri:noRegion>Georg-August University Goettingen</wicri:noRegion><wicri:noRegion>Goettingen</wicri:noRegion></affiliation></author><author><name sortKey="Breithaupt, M" sort="Breithaupt, M" uniqKey="Breithaupt M" first="M." last="Breithaupt">M. Breithaupt</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>National TSE Reference Center, Department of Neurology, Georg-August University Goettingen</s1><s2>Goettingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Goettingen</wicri:noRegion><wicri:noRegion>Georg-August University Goettingen</wicri:noRegion><wicri:noRegion>Goettingen</wicri:noRegion></affiliation></author><author><name sortKey="Varges, D" sort="Varges, D" uniqKey="Varges D" first="D." last="Varges">D. Varges</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>National TSE Reference Center, Department of Neurology, Georg-August University Goettingen</s1><s2>Goettingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Goettingen</wicri:noRegion><wicri:noRegion>Georg-August University Goettingen</wicri:noRegion><wicri:noRegion>Goettingen</wicri:noRegion></affiliation></author><author><name sortKey="Meissner, B" sort="Meissner, B" uniqKey="Meissner B" first="B." last="Meissner">B. Meissner</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>National TSE Reference Center, Department of Neurology, Georg-August University Goettingen</s1><s2>Goettingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Goettingen</wicri:noRegion><wicri:noRegion>Georg-August University Goettingen</wicri:noRegion><wicri:noRegion>Goettingen</wicri:noRegion></affiliation></author><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="05"><s1>Istituto Superiore di Sanità, Department of Cell Biology and Neurosciences</s1><s2>Roma</s2><s3>ITA</s3><sZ>10 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="Schuur, M" sort="Schuur, M" uniqKey="Schuur M" first="M." last="Schuur">M. Schuur</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Genetic Epidemiology Unit, Department of Epidemiology, Biostatistics and Clinical Genetics, Erasmus Medical Center</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>11 aut.</sZ><sZ>22 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="Haik, S" sort="Haik, S" uniqKey="Haik S" first="S." last="Haik">S. Haik</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>INSERM UMRS 975, Hôpital de la Salpêtrière Cellule Nationale de référence des maladies de Creutzfeldt-Jakob</s1><s2>Paris, 75013</s2><s3>FRA</s3><sZ>12 aut.</sZ><sZ>21 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region" nuts="2">Île-de-France</region></placeName></affiliation></author><author><name sortKey="Collins, S J" sort="Collins, S J" uniqKey="Collins S" first="S. J." last="Collins">S. J. Collins</name><affiliation wicri:level="4"><inist:fA14 i1="08"><s1>Australian National Creutzfeldt-Jakob Disease Registry, Department of Pathology, the University of Melbourne</s1><s2>Parkville, 3010</s2><s3>AUS</s3><sZ>13 aut.</sZ><sZ>20 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="Jansen, Gerard H" sort="Jansen, Gerard H" uniqKey="Jansen G" first="Gerard H." last="Jansen">Gerard H. Jansen</name><affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Creutzfeldt-Jakob Disease Surveillance System, Prion Diseases Program, Public Health Agency of Canada</s1><s2>Ottawa</s2><s3>CAN</s3><sZ>14 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>Ottawa</wicri:noRegion></affiliation></author><author><name sortKey="Stokin, G B" sort="Stokin, G B" uniqKey="Stokin G" first="G. B." last="Stokin">G. B. Stokin</name><affiliation wicri:level="1"><inist:fA14 i1="10"><s1>Division of Neurology, University Medical Center and Gerontopsychiatric Unit, University Psychiatric Hospital</s1><s2>1000 Ljubljana</s2><s3>SVN</s3><sZ>15 aut.</sZ></inist:fA14><country>Slovénie</country><wicri:noRegion>1000 Ljubljana</wicri:noRegion></affiliation></author><author><name sortKey="Pimentel, J" sort="Pimentel, J" uniqKey="Pimentel J" first="J." last="Pimentel">J. Pimentel</name><affiliation wicri:level="1"><inist:fA14 i1="11"><s1>Department of Neurology, Laboratory of Neuropathology, Institute of Molecular Medicine, Hospital de Santa Maria, Lisbon Faculty of Medicine</s1><s2>Lisbon</s2><s3>PRT</s3><sZ>16 aut.</sZ></inist:fA14><country>Portugal</country><wicri:noRegion>Lisbon</wicri:noRegion></affiliation></author><author><name sortKey="Hewer, E" sort="Hewer, E" uniqKey="Hewer E" first="E." last="Hewer">E. Hewer</name><affiliation wicri:level="1"><inist:fA14 i1="12"><s1>Institute of Neuropathology, University Hospital Zurich</s1><s2>Zurich</s2><s3>CHE</s3><sZ>17 aut.</sZ></inist:fA14><country>Suisse</country><wicri:noRegion>Zurich</wicri:noRegion></affiliation></author><author><name sortKey="Collie, D" sort="Collie, D" uniqKey="Collie D" first="D." last="Collie">D. Collie</name><affiliation wicri:level="3"><inist:fA14 i1="03"><s1>CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>3 aut.</sZ><sZ>18 aut.</sZ><sZ>26 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="Smith, P" sort="Smith, P" uniqKey="Smith P" first="P." last="Smith">P. Smith</name><affiliation wicri:level="1"><inist:fA14 i1="13"><s1>Clinic Director, Mercy Private Radiology, Grey st</s1><s2>East Melbourne, 3002</s2><s3>AUS</s3><sZ>19 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>East Melbourne, 3002</wicri:noRegion></affiliation></author><author><name sortKey="Roberts, H" sort="Roberts, H" uniqKey="Roberts H" first="H." last="Roberts">H. Roberts</name><affiliation wicri:level="4"><inist:fA14 i1="08"><s1>Australian National Creutzfeldt-Jakob Disease Registry, Department of Pathology, the University of Melbourne</s1><s2>Parkville, 3010</s2><s3>AUS</s3><sZ>13 aut.</sZ><sZ>20 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="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="07"><s1>INSERM UMRS 975, Hôpital de la Salpêtrière Cellule Nationale de référence des maladies de Creutzfeldt-Jakob</s1><s2>Paris, 75013</s2><s3>FRA</s3><sZ>12 aut.</sZ><sZ>21 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region" nuts="2">Île-de-France</region></placeName></affiliation></author><author><name sortKey="Van Duijn, C" sort="Van Duijn, C" uniqKey="Van Duijn C" first="C." last="Van Duijn">C. Van Duijn</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Genetic Epidemiology Unit, Department of Epidemiology, Biostatistics and Clinical Genetics, Erasmus Medical Center</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>11 aut.</sZ><sZ>22 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="Pocchiari, M" sort="Pocchiari, M" uniqKey="Pocchiari M" first="M." last="Pocchiari">M. Pocchiari</name><affiliation wicri:level="3"><inist:fA14 i1="05"><s1>Istituto Superiore di Sanità, Department of Cell Biology and Neurosciences</s1><s2>Roma</s2><s3>ITA</s3><sZ>10 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="Begue, C" sort="Begue, C" uniqKey="Begue C" first="C." last="Begue">C. Begue</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Departamento de Neuropatologia, Centro de Referencia de Encefalopatias Espongiformes Transmisibles, Instituto de Investigaciones Neurologicas/FLENI Montañeses 2325</s1><s2>C1428AQK, Buenos Aires</s2><s3>ARG</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Argentine</country><wicri:noRegion>C1428AQK, Buenos Aires</wicri:noRegion></affiliation></author><author><name sortKey="Cras, P" sort="Cras, P" uniqKey="Cras P" first="P." last="Cras">P. Cras</name><affiliation wicri:level="4"><inist:fA14 i1="14"><s1>Laboratory of Neurobiology, Department of Neurology, Born Bunge Institute, University of Antwerp</s1><s3>BEL</s3><sZ>25 aut.</sZ></inist:fA14><country>Belgique</country><placeName><settlement type="city">Anvers</settlement><region>Région flamande</region><region type="district" nuts="2">Province d'Anvers</region></placeName><orgName type="university">Université d'Anvers</orgName></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="03"><s1>CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>3 aut.</sZ><sZ>18 aut.</sZ><sZ>26 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="Sanchez Juan, P" sort="Sanchez Juan, P" uniqKey="Sanchez Juan P" first="P." last="Sanchez-Juan">P. Sanchez-Juan</name><affiliation wicri:level="1"><inist:fA14 i1="15"><s1>Fundación 'Marqués de Vaidecilla' IFIMAV and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED)</s1><s2>Santander</s2><s3>ESP</s3><sZ>27 aut.</sZ></inist:fA14><country>Espagne</country><wicri:noRegion>Fundación 'Marqués de Vaidecilla' IFIMAV and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED)</wicri:noRegion></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">09-0437741</idno><date when="2009">2009</date><idno type="stanalyst">PASCAL 09-0437741 INIST</idno><idno type="RBID">Pascal:09-0437741</idno><idno type="wicri:Area/PascalFrancis/Corpus">002B14</idno><idno type="wicri:Area/PascalFrancis/Curation">003510</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">002722</idno><idno type="wicri:explorRef" wicri:stream="PascalFrancis" wicri:step="Checkpoint">002722</idno><idno type="wicri:doubleKey">0006-8950:2009:Zerr I:updated:clinical:diagnostic</idno><idno type="wicri:Area/Main/Merge">008691</idno><idno type="wicri:source">PMC</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2759336</idno><idno type="RBID">PMC:2759336</idno><idno type="wicri:Area/Pmc/Corpus">002355</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">002355</idno><idno type="wicri:Area/Pmc/Curation">002205</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">002205</idno><idno type="wicri:Area/Pmc/Checkpoint">002696</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">002696</idno><idno type="wicri:Area/Ncbi/Merge">000646</idno><idno type="wicri:Area/Ncbi/Curation">000646</idno><idno type="wicri:Area/Ncbi/Checkpoint">000646</idno><idno type="wicri:doubleKey">0006-8950:2009:Zerr I:updated:clinical:diagnostic</idno><idno type="wicri:Area/Main/Merge">008188</idno><idno type="wicri:Area/Main/Curation">007F91</idno><idno type="wicri:Area/Main/Exploration">007F91</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease</title><author><name sortKey="Zerr, I" sort="Zerr, I" uniqKey="Zerr I" first="I." last="Zerr">I. Zerr</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>National TSE Reference Center, Department of Neurology, Georg-August University Goettingen</s1><s2>Goettingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Goettingen</wicri:noRegion><wicri:noRegion>Georg-August University Goettingen</wicri:noRegion><wicri:noRegion>Goettingen</wicri:noRegion></affiliation></author><author><name sortKey="Kallenberg, K" sort="Kallenberg, K" uniqKey="Kallenberg K" first="K." last="Kallenberg">K. Kallenberg</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Neuroradiology, Georg-August University Goettingen</s1><s2>Goettingen</s2><s3>DEU</s3><sZ>2 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Goettingen</wicri:noRegion><wicri:noRegion>Georg-August University Goettingen</wicri:noRegion><wicri:noRegion>Goettingen</wicri:noRegion></affiliation></author><author><name sortKey="Summers, D M" sort="Summers, D M" uniqKey="Summers D" first="D. M." last="Summers">D. M. Summers</name><affiliation wicri:level="3"><inist:fA14 i1="03"><s1>CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>3 aut.</sZ><sZ>18 aut.</sZ><sZ>26 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="Romero, C" sort="Romero, C" uniqKey="Romero C" first="C." last="Romero">C. Romero</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Departamento de Neuropatologia, Centro de Referencia de Encefalopatias Espongiformes Transmisibles, Instituto de Investigaciones Neurologicas/FLENI Montañeses 2325</s1><s2>C1428AQK, Buenos Aires</s2><s3>ARG</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Argentine</country><wicri:noRegion>C1428AQK, Buenos Aires</wicri:noRegion></affiliation></author><author><name sortKey="Taratuto, A" sort="Taratuto, A" uniqKey="Taratuto A" first="A." last="Taratuto">A. Taratuto</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Departamento de Neuropatologia, Centro de Referencia de Encefalopatias Espongiformes Transmisibles, Instituto de Investigaciones Neurologicas/FLENI Montañeses 2325</s1><s2>C1428AQK, Buenos Aires</s2><s3>ARG</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Argentine</country><wicri:noRegion>C1428AQK, Buenos Aires</wicri:noRegion></affiliation></author><author><name sortKey="Heinemann, U" sort="Heinemann, U" uniqKey="Heinemann U" first="U." last="Heinemann">U. Heinemann</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>National TSE Reference Center, Department of Neurology, Georg-August University Goettingen</s1><s2>Goettingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Goettingen</wicri:noRegion><wicri:noRegion>Georg-August University Goettingen</wicri:noRegion><wicri:noRegion>Goettingen</wicri:noRegion></affiliation></author><author><name sortKey="Breithaupt, M" sort="Breithaupt, M" uniqKey="Breithaupt M" first="M." last="Breithaupt">M. Breithaupt</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>National TSE Reference Center, Department of Neurology, Georg-August University Goettingen</s1><s2>Goettingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Goettingen</wicri:noRegion><wicri:noRegion>Georg-August University Goettingen</wicri:noRegion><wicri:noRegion>Goettingen</wicri:noRegion></affiliation></author><author><name sortKey="Varges, D" sort="Varges, D" uniqKey="Varges D" first="D." last="Varges">D. Varges</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>National TSE Reference Center, Department of Neurology, Georg-August University Goettingen</s1><s2>Goettingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Goettingen</wicri:noRegion><wicri:noRegion>Georg-August University Goettingen</wicri:noRegion><wicri:noRegion>Goettingen</wicri:noRegion></affiliation></author><author><name sortKey="Meissner, B" sort="Meissner, B" uniqKey="Meissner B" first="B." last="Meissner">B. Meissner</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>National TSE Reference Center, Department of Neurology, Georg-August University Goettingen</s1><s2>Goettingen</s2><s3>DEU</s3><sZ>1 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ><sZ>8 aut.</sZ><sZ>9 aut.</sZ></inist:fA14><country>Allemagne</country><wicri:noRegion>Goettingen</wicri:noRegion><wicri:noRegion>Georg-August University Goettingen</wicri:noRegion><wicri:noRegion>Goettingen</wicri:noRegion></affiliation></author><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="05"><s1>Istituto Superiore di Sanità, Department of Cell Biology and Neurosciences</s1><s2>Roma</s2><s3>ITA</s3><sZ>10 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="Schuur, M" sort="Schuur, M" uniqKey="Schuur M" first="M." last="Schuur">M. Schuur</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Genetic Epidemiology Unit, Department of Epidemiology, Biostatistics and Clinical Genetics, Erasmus Medical Center</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>11 aut.</sZ><sZ>22 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="Haik, S" sort="Haik, S" uniqKey="Haik S" first="S." last="Haik">S. Haik</name><affiliation wicri:level="3"><inist:fA14 i1="07"><s1>INSERM UMRS 975, Hôpital de la Salpêtrière Cellule Nationale de référence des maladies de Creutzfeldt-Jakob</s1><s2>Paris, 75013</s2><s3>FRA</s3><sZ>12 aut.</sZ><sZ>21 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region" nuts="2">Île-de-France</region></placeName></affiliation></author><author><name sortKey="Collins, S J" sort="Collins, S J" uniqKey="Collins S" first="S. J." last="Collins">S. J. Collins</name><affiliation wicri:level="4"><inist:fA14 i1="08"><s1>Australian National Creutzfeldt-Jakob Disease Registry, Department of Pathology, the University of Melbourne</s1><s2>Parkville, 3010</s2><s3>AUS</s3><sZ>13 aut.</sZ><sZ>20 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="Jansen, Gerard H" sort="Jansen, Gerard H" uniqKey="Jansen G" first="Gerard H." last="Jansen">Gerard H. Jansen</name><affiliation wicri:level="1"><inist:fA14 i1="09"><s1>Creutzfeldt-Jakob Disease Surveillance System, Prion Diseases Program, Public Health Agency of Canada</s1><s2>Ottawa</s2><s3>CAN</s3><sZ>14 aut.</sZ></inist:fA14><country>Canada</country><wicri:noRegion>Ottawa</wicri:noRegion></affiliation></author><author><name sortKey="Stokin, G B" sort="Stokin, G B" uniqKey="Stokin G" first="G. B." last="Stokin">G. B. Stokin</name><affiliation wicri:level="1"><inist:fA14 i1="10"><s1>Division of Neurology, University Medical Center and Gerontopsychiatric Unit, University Psychiatric Hospital</s1><s2>1000 Ljubljana</s2><s3>SVN</s3><sZ>15 aut.</sZ></inist:fA14><country>Slovénie</country><wicri:noRegion>1000 Ljubljana</wicri:noRegion></affiliation></author><author><name sortKey="Pimentel, J" sort="Pimentel, J" uniqKey="Pimentel J" first="J." last="Pimentel">J. Pimentel</name><affiliation wicri:level="1"><inist:fA14 i1="11"><s1>Department of Neurology, Laboratory of Neuropathology, Institute of Molecular Medicine, Hospital de Santa Maria, Lisbon Faculty of Medicine</s1><s2>Lisbon</s2><s3>PRT</s3><sZ>16 aut.</sZ></inist:fA14><country>Portugal</country><wicri:noRegion>Lisbon</wicri:noRegion></affiliation></author><author><name sortKey="Hewer, E" sort="Hewer, E" uniqKey="Hewer E" first="E." last="Hewer">E. Hewer</name><affiliation wicri:level="1"><inist:fA14 i1="12"><s1>Institute of Neuropathology, University Hospital Zurich</s1><s2>Zurich</s2><s3>CHE</s3><sZ>17 aut.</sZ></inist:fA14><country>Suisse</country><wicri:noRegion>Zurich</wicri:noRegion></affiliation></author><author><name sortKey="Collie, D" sort="Collie, D" uniqKey="Collie D" first="D." last="Collie">D. Collie</name><affiliation wicri:level="3"><inist:fA14 i1="03"><s1>CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>3 aut.</sZ><sZ>18 aut.</sZ><sZ>26 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="Smith, P" sort="Smith, P" uniqKey="Smith P" first="P." last="Smith">P. Smith</name><affiliation wicri:level="1"><inist:fA14 i1="13"><s1>Clinic Director, Mercy Private Radiology, Grey st</s1><s2>East Melbourne, 3002</s2><s3>AUS</s3><sZ>19 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>East Melbourne, 3002</wicri:noRegion></affiliation></author><author><name sortKey="Roberts, H" sort="Roberts, H" uniqKey="Roberts H" first="H." last="Roberts">H. Roberts</name><affiliation wicri:level="4"><inist:fA14 i1="08"><s1>Australian National Creutzfeldt-Jakob Disease Registry, Department of Pathology, the University of Melbourne</s1><s2>Parkville, 3010</s2><s3>AUS</s3><sZ>13 aut.</sZ><sZ>20 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="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="07"><s1>INSERM UMRS 975, Hôpital de la Salpêtrière Cellule Nationale de référence des maladies de Creutzfeldt-Jakob</s1><s2>Paris, 75013</s2><s3>FRA</s3><sZ>12 aut.</sZ><sZ>21 aut.</sZ></inist:fA14><country>France</country><placeName><region type="region" nuts="2">Île-de-France</region></placeName></affiliation></author><author><name sortKey="Van Duijn, C" sort="Van Duijn, C" uniqKey="Van Duijn C" first="C." last="Van Duijn">C. Van Duijn</name><affiliation wicri:level="3"><inist:fA14 i1="06"><s1>Genetic Epidemiology Unit, Department of Epidemiology, Biostatistics and Clinical Genetics, Erasmus Medical Center</s1><s2>Rotterdam</s2><s3>NLD</s3><sZ>11 aut.</sZ><sZ>22 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="Pocchiari, M" sort="Pocchiari, M" uniqKey="Pocchiari M" first="M." last="Pocchiari">M. Pocchiari</name><affiliation wicri:level="3"><inist:fA14 i1="05"><s1>Istituto Superiore di Sanità, Department of Cell Biology and Neurosciences</s1><s2>Roma</s2><s3>ITA</s3><sZ>10 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="Begue, C" sort="Begue, C" uniqKey="Begue C" first="C." last="Begue">C. Begue</name><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Departamento de Neuropatologia, Centro de Referencia de Encefalopatias Espongiformes Transmisibles, Instituto de Investigaciones Neurologicas/FLENI Montañeses 2325</s1><s2>C1428AQK, Buenos Aires</s2><s3>ARG</s3><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>24 aut.</sZ></inist:fA14><country>Argentine</country><wicri:noRegion>C1428AQK, Buenos Aires</wicri:noRegion></affiliation></author><author><name sortKey="Cras, P" sort="Cras, P" uniqKey="Cras P" first="P." last="Cras">P. Cras</name><affiliation wicri:level="4"><inist:fA14 i1="14"><s1>Laboratory of Neurobiology, Department of Neurology, Born Bunge Institute, University of Antwerp</s1><s3>BEL</s3><sZ>25 aut.</sZ></inist:fA14><country>Belgique</country><placeName><settlement type="city">Anvers</settlement><region>Région flamande</region><region type="district" nuts="2">Province d'Anvers</region></placeName><orgName type="university">Université d'Anvers</orgName></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="03"><s1>CJD Surveillance Unit, Western General Hospital</s1><s2>Edinburgh</s2><s3>GBR</s3><sZ>3 aut.</sZ><sZ>18 aut.</sZ><sZ>26 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="Sanchez Juan, P" sort="Sanchez Juan, P" uniqKey="Sanchez Juan P" first="P." last="Sanchez-Juan">P. Sanchez-Juan</name><affiliation wicri:level="1"><inist:fA14 i1="15"><s1>Fundación 'Marqués de Vaidecilla' IFIMAV and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED)</s1><s2>Santander</s2><s3>ESP</s3><sZ>27 aut.</sZ></inist:fA14><country>Espagne</country><wicri:noRegion>Fundación 'Marqués de Vaidecilla' IFIMAV and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED)</wicri:noRegion></affiliation></author></analytic><series><title level="j" type="main">Brain</title><title level="j" type="abbreviated">Brain</title><idno type="ISSN">0006-8950</idno><imprint><date when="2009">2009</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Brain</title><title level="j" type="abbreviated">Brain</title><idno type="ISSN">0006-8950</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Creutzfeldt-Jakob disease</term><term>Dementia</term><term>Diagnosis</term><term>Nervous system diseases</term><term>Nuclear magnetic resonance imaging</term><term>Prion</term><term>Sporadic</term><term>Subtype</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Démence</term><term>Pathologie du système nerveux</term><term>Diagnostic</term><term>Sporadique</term><term>Encéphalopathie spongiforme de Creutzfeldt-Jakob</term><term>Imagerie RMN</term><term>Soustype</term><term>Prion</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Several molecular subtypes of sporadic Creutzfeldt-Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but with variable utility according to subtype. In recent years, a series of publications have demonstrated a potentially important role for magnetic resonance imaging in the pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease. Magnetic resonance imaging signal alterations correlate with distinct sporadic Creutzfeldt-Jakob disease molecular subtypes and thus might contribute to the earlier identification of the whole spectrum of sporadic Creutzfeldt-Jakob disease cases. This multi-centre international study aimed to provide a rationale for the amendment of the clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Patients with sporadic Creutzfeldt-Jakob disease and fluid attenuated inversion recovery or diffusion-weight imaging were recruited from 12 countries. Patients referred as 'suspected sporadic Creutzfeldt-Jakob disease' but with an alternative diagnosis after thorough follow up, were analysed as controls. All magnetic resonance imaging scans were assessed for signal changes according to a standard protocol encompassing seven cortical regions, basal ganglia, thalamus and cerebellum. Magnetic resonance imaging scans were evaluated in 436 sporadic Creutzfeldt-Jakob disease patients and 141 controls. The pattern of high signal intensity with the best sensitivity and specificity in the differential diagnosis of sporadic Creutzfeldt-Jakob disease was identified. The optimum diagnostic accuracy in the differential diagnosis of rapid progressive dementia was obtained when either at least two cortical regions (temporal, parietal or occipital) or both caudate nucleus and putamen displayed a high signal in fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging. Based on our analyses, magnetic resonance imaging was positive in 83% of cases. In all definite cases, the amended criteria would cover the vast majority of suspected cases, being positive in 98%. Cerebral cortical signal increase and high signal in caudate nucleus and putamen on fluid attenuated inversion recovery or diffusion-weight imaging magnetic resonance imaging are useful in the diagnosis of sporadic Creutzfeldt-Jakob disease. We propose an amendment to the clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease to include findings from magnetic resonance imaging scans.</div></front></TEI><affiliations><list><country><li>Allemagne</li><li>Argentine</li><li>Australie</li><li>Belgique</li><li>Canada</li><li>Espagne</li><li>France</li><li>Italie</li><li>Pays-Bas</li><li>Portugal</li><li>Royaume-Uni</li><li>Slovénie</li><li>Suisse</li></country><region><li>Hollande-Méridionale</li><li>Latium</li><li>Province d'Anvers</li><li>Région flamande</li><li>Victoria (État)</li><li>Écosse</li><li>Île-de-France</li></region><settlement><li>Anvers</li><li>Melbourne</li><li>Rome</li><li>Rotterdam</li><li>Édimbourg</li></settlement><orgName><li>Université d'Anvers</li><li>Université de Melbourne</li></orgName></list><tree><country name="Allemagne"><noRegion><name sortKey="Zerr, I" sort="Zerr, I" uniqKey="Zerr I" first="I." last="Zerr">I. Zerr</name></noRegion><name sortKey="Breithaupt, M" sort="Breithaupt, M" uniqKey="Breithaupt M" first="M." last="Breithaupt">M. Breithaupt</name><name sortKey="Heinemann, U" sort="Heinemann, U" uniqKey="Heinemann U" first="U." last="Heinemann">U. Heinemann</name><name sortKey="Kallenberg, K" sort="Kallenberg, K" uniqKey="Kallenberg K" first="K." last="Kallenberg">K. Kallenberg</name><name sortKey="Meissner, B" sort="Meissner, B" uniqKey="Meissner B" first="B." last="Meissner">B. Meissner</name><name sortKey="Varges, D" sort="Varges, D" uniqKey="Varges D" first="D." last="Varges">D. Varges</name></country><country name="Royaume-Uni"><region name="Écosse"><name sortKey="Summers, D M" sort="Summers, D M" uniqKey="Summers D" first="D. M." last="Summers">D. M. Summers</name></region><name sortKey="Collie, D" sort="Collie, D" uniqKey="Collie D" first="D." last="Collie">D. Collie</name><name sortKey="Will, R G" sort="Will, R G" uniqKey="Will R" first="R. G." last="Will">R. G. Will</name></country><country name="Argentine"><noRegion><name sortKey="Romero, C" sort="Romero, C" uniqKey="Romero C" first="C." last="Romero">C. Romero</name></noRegion><name sortKey="Begue, C" sort="Begue, C" uniqKey="Begue C" first="C." last="Begue">C. Begue</name><name sortKey="Taratuto, A" sort="Taratuto, A" uniqKey="Taratuto A" first="A." last="Taratuto">A. Taratuto</name></country><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></country><country name="Pays-Bas"><region name="Hollande-Méridionale"><name sortKey="Schuur, M" sort="Schuur, M" uniqKey="Schuur M" first="M." last="Schuur">M. Schuur</name></region><name sortKey="Van Duijn, C" sort="Van Duijn, C" uniqKey="Van Duijn C" first="C." last="Van Duijn">C. Van Duijn</name></country><country name="France"><region name="Île-de-France"><name sortKey="Haik, S" sort="Haik, S" uniqKey="Haik S" first="S." last="Haik">S. Haik</name></region><name sortKey="Brandel, J P" sort="Brandel, J P" uniqKey="Brandel J" first="J. P." last="Brandel">J. P. Brandel</name></country><country name="Australie"><region name="Victoria (État)"><name sortKey="Collins, S J" sort="Collins, S J" uniqKey="Collins S" first="S. J." last="Collins">S. J. Collins</name></region><name sortKey="Roberts, H" sort="Roberts, H" uniqKey="Roberts H" first="H." last="Roberts">H. Roberts</name><name sortKey="Smith, P" sort="Smith, P" uniqKey="Smith P" first="P." last="Smith">P. Smith</name></country><country name="Canada"><noRegion><name sortKey="Jansen, Gerard H" sort="Jansen, Gerard H" uniqKey="Jansen G" first="Gerard H." last="Jansen">Gerard H. Jansen</name></noRegion></country><country name="Slovénie"><noRegion><name sortKey="Stokin, G B" sort="Stokin, G B" uniqKey="Stokin G" first="G. B." last="Stokin">G. B. Stokin</name></noRegion></country><country name="Portugal"><noRegion><name sortKey="Pimentel, J" sort="Pimentel, J" uniqKey="Pimentel J" first="J." last="Pimentel">J. Pimentel</name></noRegion></country><country name="Suisse"><noRegion><name sortKey="Hewer, E" sort="Hewer, E" uniqKey="Hewer E" first="E." last="Hewer">E. Hewer</name></noRegion></country><country name="Belgique"><region name="Région flamande"><name sortKey="Cras, P" sort="Cras, P" uniqKey="Cras P" first="P." last="Cras">P. Cras</name></region></country><country name="Espagne"><noRegion><name sortKey="Sanchez Juan, P" sort="Sanchez Juan, P" uniqKey="Sanchez Juan P" first="P." last="Sanchez-Juan">P. Sanchez-Juan</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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