Validation of diagnostic criteria for variant Creutzfeldt–Jakob disease
Identifieur interne : 003187 ( Main/Corpus ); précédent : 003186; suivant : 003188Validation of diagnostic criteria for variant Creutzfeldt–Jakob disease
Auteurs : Craig A. Heath ; Sarah A. Cooper ; Katy Murray ; Andrea Lowman ; Colm Henry ; Margaret A. Macleod ; Gillian E. Stewart ; Martin Zeidler ; Jan M. Mackenzie ; James W. Ironside ; David M. Summers ; Richard S. G. Knight ; Robert G. WillSource :
- Annals of Neurology [ 0364-5134 ] ; 2010-06.
Abstract
Objective: Variant Creutzfeldt–Jakob disease (vCJD), a novel form of human prion disease, was recognized in 1996. The disease affected a younger cohort than sporadic CJD, and the early clinical course was dominated by psychiatric and sensory symptoms. In an attempt to aid diagnosis and establish standardization between surveillance networks, diagnostic criteria were established. These were devised from the features of a small number of cases and modified in 2000 as the clinical phenotype was established. Since then, only minor changes have been introduced; revalidation of the criteria in the current format is overdue. Methods: Included in this study are autopsy/cerebral biopsy‐proven cases of vCJD referred to the National CJD Surveillance Unit (NCJDSU) between 1995 and 2004 and suspect cases in which an alternative diagnosis was identified following autopsy/cerebral biopsy. Results: Over the 10‐year period, 106 definite cases of vCJD and 45 pathologically confirmed “noncases” were identified from the archives of the NCJDSU. The median age at onset of the cases was significantly younger than that of the noncases (27 years [range, 12–74 years] vs 43 years [range, 10–64 years]), and the median duration of illness was significantly shorter (14 months [range, 6–39 months] vs 22 months [range, 2–139 months]). The most commonly identified core clinical feature in cases was dementia; persistent painful sensory symptoms were the least frequent. Eighty‐eight of 106 (83%) vCJD cases were retrospectively classified as probable in life, 6 cases were classified as possible. Most cases were classified as probable on the basis of core clinical features and brain magnetic resonance imaging. To date, the diagnostic criteria remain 100% specific, with no autopsy/cerebral biopsy‐proven noncases classified as probable in life. Interpretation: This study confirms that the diagnostic criteria for vCJD are sensitive and specific and provide a useful standard framework for case classification in a surveillance setting. ANN NEUROL 2010
Url:
DOI: 10.1002/ana.21987
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Cooper</name><affiliation><mods:affiliation>Institute of Neurology, Southern General Hospital, Glasgow, UK</mods:affiliation></affiliation></author><author><name sortKey="Murray, Katy" sort="Murray, Katy" uniqKey="Murray K" first="Katy" last="Murray">Katy Murray</name><affiliation><mods:affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</mods:affiliation></affiliation></author><author><name sortKey="Lowman, Andrea" sort="Lowman, Andrea" uniqKey="Lowman A" first="Andrea" last="Lowman">Andrea Lowman</name><affiliation><mods:affiliation>Department of Neurology, University Hospital, Cardiff, UK</mods:affiliation></affiliation></author><author><name sortKey="Henry, Colm" sort="Henry, Colm" uniqKey="Henry C" first="Colm" last="Henry">Colm Henry</name><affiliation><mods:affiliation>Department of Medicine of the Elderly, Mercy University Hospital, Cork, Ireland</mods:affiliation></affiliation></author><author><name sortKey="Macleod, Margaret A" sort="Macleod, Margaret A" uniqKey="Macleod M" first="Margaret A." last="Macleod">Margaret A. 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Heath</name><affiliation><mods:affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</mods:affiliation></affiliation></author><author><name sortKey="Cooper, Sarah A" sort="Cooper, Sarah A" uniqKey="Cooper S" first="Sarah A." last="Cooper">Sarah A. 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Macleod</name><affiliation><mods:affiliation>Department of Neurology, Aberdeen Royal Infirmary, Aberdeen, UK</mods:affiliation></affiliation></author><author><name sortKey="Stewart, Gillian E" sort="Stewart, Gillian E" uniqKey="Stewart G" first="Gillian E." last="Stewart">Gillian E. Stewart</name><affiliation><mods:affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</mods:affiliation></affiliation></author><author><name sortKey="Zeidler, Martin" sort="Zeidler, Martin" uniqKey="Zeidler M" first="Martin" last="Zeidler">Martin Zeidler</name><affiliation><mods:affiliation>Department of Neurology, Victoria Hospital, Kirkcaldy, UK</mods:affiliation></affiliation></author><author><name sortKey="Mackenzie, Jan M" sort="Mackenzie, Jan M" uniqKey="Mackenzie J" first="Jan M." last="Mackenzie">Jan M. Mackenzie</name><affiliation><mods:affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</mods:affiliation></affiliation></author><author><name sortKey="Ironside, James W" sort="Ironside, James W" uniqKey="Ironside J" first="James W." last="Ironside">James W. Ironside</name><affiliation><mods:affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</mods:affiliation></affiliation></author><author><name sortKey="Summers, David M" sort="Summers, David M" uniqKey="Summers D" first="David M." last="Summers">David M. Summers</name><affiliation><mods:affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</mods:affiliation></affiliation></author><author><name sortKey="Knight, Richard S G" sort="Knight, Richard S G" uniqKey="Knight R" first="Richard S. G." last="Knight">Richard S. G. 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Will</name><affiliation><mods:affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Annals of Neurology</title><title level="j" type="abbrev">Ann Neurol.</title><idno type="ISSN">0364-5134</idno><idno type="eISSN">1531-8249</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2010-06">2010-06</date><biblScope unit="volume">67</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="761">761</biblScope><biblScope unit="page" to="770">770</biblScope></imprint><idno type="ISSN">0364-5134</idno></series><idno type="istex">E92195887B6E2ED5288C8E320329AB420842CF22</idno><idno type="DOI">10.1002/ana.21987</idno><idno type="ArticleID">ANA21987</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0364-5134</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Objective: Variant Creutzfeldt–Jakob disease (vCJD), a novel form of human prion disease, was recognized in 1996. The disease affected a younger cohort than sporadic CJD, and the early clinical course was dominated by psychiatric and sensory symptoms. In an attempt to aid diagnosis and establish standardization between surveillance networks, diagnostic criteria were established. These were devised from the features of a small number of cases and modified in 2000 as the clinical phenotype was established. Since then, only minor changes have been introduced; revalidation of the criteria in the current format is overdue. Methods: Included in this study are autopsy/cerebral biopsy‐proven cases of vCJD referred to the National CJD Surveillance Unit (NCJDSU) between 1995 and 2004 and suspect cases in which an alternative diagnosis was identified following autopsy/cerebral biopsy. Results: Over the 10‐year period, 106 definite cases of vCJD and 45 pathologically confirmed “noncases” were identified from the archives of the NCJDSU. The median age at onset of the cases was significantly younger than that of the noncases (27 years [range, 12–74 years] vs 43 years [range, 10–64 years]), and the median duration of illness was significantly shorter (14 months [range, 6–39 months] vs 22 months [range, 2–139 months]). The most commonly identified core clinical feature in cases was dementia; persistent painful sensory symptoms were the least frequent. Eighty‐eight of 106 (83%) vCJD cases were retrospectively classified as probable in life, 6 cases were classified as possible. Most cases were classified as probable on the basis of core clinical features and brain magnetic resonance imaging. To date, the diagnostic criteria remain 100% specific, with no autopsy/cerebral biopsy‐proven noncases classified as probable in life. Interpretation: This study confirms that the diagnostic criteria for vCJD are sensitive and specific and provide a useful standard framework for case classification in a surveillance setting. ANN NEUROL 2010</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Craig A. Heath MD</name><affiliations><json:string>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</json:string></affiliations></json:item><json:item><name>Sarah A. Cooper MD</name><affiliations><json:string>Institute of Neurology, Southern General Hospital, Glasgow, UK</json:string></affiliations></json:item><json:item><name>Katy Murray MD</name><affiliations><json:string>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</json:string></affiliations></json:item><json:item><name>Andrea Lowman MB ChB (Hons), MRCP</name><affiliations><json:string>Department of Neurology, University Hospital, Cardiff, UK</json:string></affiliations></json:item><json:item><name>Colm Henry MB MRCPI MRCGP</name><affiliations><json:string>Department of Medicine of the Elderly, Mercy University Hospital, Cork, Ireland</json:string></affiliations></json:item><json:item><name>Margaret A. MacLeod MD, MRCP</name><affiliations><json:string>Department of Neurology, Aberdeen Royal Infirmary, Aberdeen, UK</json:string></affiliations></json:item><json:item><name>Gillian E. Stewart MB ChB</name><affiliations><json:string>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</json:string></affiliations></json:item><json:item><name>Martin Zeidler FRCP</name><affiliations><json:string>Department of Neurology, Victoria Hospital, Kirkcaldy, UK</json:string></affiliations></json:item><json:item><name>Jan M. MacKenzie</name><affiliations><json:string>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</json:string></affiliations></json:item><json:item><name>James W. Ironside FRCPath</name><affiliations><json:string>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</json:string></affiliations></json:item><json:item><name>David M. Summers MD</name><affiliations><json:string>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</json:string></affiliations></json:item><json:item><name>Richard S. G. Knight FRCP</name><affiliations><json:string>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</json:string></affiliations></json:item><json:item><name>Robert G. Will FRCP</name><affiliations><json:string>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</json:string></affiliations></json:item></author><articleId><json:string>ANA21987</json:string></articleId><language><json:string>eng</json:string></language><abstract>Objective: Variant Creutzfeldt–Jakob disease (vCJD), a novel form of human prion disease, was recognized in 1996. The disease affected a younger cohort than sporadic CJD, and the early clinical course was dominated by psychiatric and sensory symptoms. In an attempt to aid diagnosis and establish standardization between surveillance networks, diagnostic criteria were established. These were devised from the features of a small number of cases and modified in 2000 as the clinical phenotype was established. Since then, only minor changes have been introduced; revalidation of the criteria in the current format is overdue. Methods: Included in this study are autopsy/cerebral biopsy‐proven cases of vCJD referred to the National CJD Surveillance Unit (NCJDSU) between 1995 and 2004 and suspect cases in which an alternative diagnosis was identified following autopsy/cerebral biopsy. Results: Over the 10‐year period, 106 definite cases of vCJD and 45 pathologically confirmed “noncases” were identified from the archives of the NCJDSU. The median age at onset of the cases was significantly younger than that of the noncases (27 years [range, 12–74 years] vs 43 years [range, 10–64 years]), and the median duration of illness was significantly shorter (14 months [range, 6–39 months] vs 22 months [range, 2–139 months]). The most commonly identified core clinical feature in cases was dementia; persistent painful sensory symptoms were the least frequent. Eighty‐eight of 106 (83%) vCJD cases were retrospectively classified as probable in life, 6 cases were classified as possible. Most cases were classified as probable on the basis of core clinical features and brain magnetic resonance imaging. To date, the diagnostic criteria remain 100% specific, with no autopsy/cerebral biopsy‐proven noncases classified as probable in life. Interpretation: This study confirms that the diagnostic criteria for vCJD are sensitive and specific and provide a useful standard framework for case classification in a surveillance setting. 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No. 121/5061;</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Validation of diagnostic criteria for variant Creutzfeldt–Jakob disease</title><author><persName><forename type="first">Craig A.</forename><surname>Heath</surname></persName><roleName type="degree">MD</roleName><note type="correspondence"><p>Correspondence: Specialist Registrar, Western General Hospital, Edinburgh, EH4 2XU UK</p></note><affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</affiliation></author><author><persName><forename type="first">Sarah A.</forename><surname>Cooper</surname></persName><roleName type="degree">MD</roleName><affiliation>Institute of Neurology, Southern General Hospital, Glasgow, UK</affiliation></author><author><persName><forename type="first">Katy</forename><surname>Murray</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</affiliation></author><author><persName><forename type="first">Andrea</forename><surname>Lowman</surname></persName><roleName type="degree">MB ChB (Hons), MRCP</roleName><affiliation>Department of Neurology, University Hospital, Cardiff, UK</affiliation></author><author><persName><forename type="first">Colm</forename><surname>Henry</surname></persName><roleName type="degree">MB MRCPI MRCGP</roleName><affiliation>Department of Medicine of the Elderly, Mercy University Hospital, Cork, Ireland</affiliation></author><author><persName><forename type="first">Margaret A.</forename><surname>MacLeod</surname></persName><roleName type="degree">MD, MRCP</roleName><affiliation>Department of Neurology, Aberdeen Royal Infirmary, Aberdeen, UK</affiliation></author><author><persName><forename type="first">Gillian E.</forename><surname>Stewart</surname></persName><roleName type="degree">MB ChB</roleName><affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</affiliation></author><author><persName><forename type="first">Martin</forename><surname>Zeidler</surname></persName><roleName type="degree">FRCP</roleName><affiliation>Department of Neurology, Victoria Hospital, Kirkcaldy, UK</affiliation></author><author><persName><forename type="first">Jan M.</forename><surname>MacKenzie</surname></persName><affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</affiliation></author><author><persName><forename type="first">James W.</forename><surname>Ironside</surname></persName><roleName type="degree">FRCPath</roleName><affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</affiliation></author><author><persName><forename type="first">David M.</forename><surname>Summers</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</affiliation></author><author><persName><forename type="first">Richard S. G.</forename><surname>Knight</surname></persName><roleName type="degree">FRCP</roleName><affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</affiliation></author><author><persName><forename type="first">Robert G.</forename><surname>Will</surname></persName><roleName type="degree">FRCP</roleName><affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</affiliation></author></analytic><monogr><title level="j">Annals of Neurology</title><title level="j" type="abbrev">Ann Neurol.</title><idno type="pISSN">0364-5134</idno><idno type="eISSN">1531-8249</idno><idno type="DOI">10.1002/(ISSN)1531-8249</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2010-06"></date><biblScope unit="volume">67</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="761">761</biblScope><biblScope unit="page" to="770">770</biblScope></imprint></monogr><idno type="istex">E92195887B6E2ED5288C8E320329AB420842CF22</idno><idno type="DOI">10.1002/ana.21987</idno><idno type="ArticleID">ANA21987</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>2010</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Objective: Variant Creutzfeldt–Jakob disease (vCJD), a novel form of human prion disease, was recognized in 1996. The disease affected a younger cohort than sporadic CJD, and the early clinical course was dominated by psychiatric and sensory symptoms. In an attempt to aid diagnosis and establish standardization between surveillance networks, diagnostic criteria were established. These were devised from the features of a small number of cases and modified in 2000 as the clinical phenotype was established. Since then, only minor changes have been introduced; revalidation of the criteria in the current format is overdue. Methods: Included in this study are autopsy/cerebral biopsy‐proven cases of vCJD referred to the National CJD Surveillance Unit (NCJDSU) between 1995 and 2004 and suspect cases in which an alternative diagnosis was identified following autopsy/cerebral biopsy. Results: Over the 10‐year period, 106 definite cases of vCJD and 45 pathologically confirmed “noncases” were identified from the archives of the NCJDSU. The median age at onset of the cases was significantly younger than that of the noncases (27 years [range, 12–74 years] vs 43 years [range, 10–64 years]), and the median duration of illness was significantly shorter (14 months [range, 6–39 months] vs 22 months [range, 2–139 months]). The most commonly identified core clinical feature in cases was dementia; persistent painful sensory symptoms were the least frequent. Eighty‐eight of 106 (83%) vCJD cases were retrospectively classified as probable in life, 6 cases were classified as possible. Most cases were classified as probable on the basis of core clinical features and brain magnetic resonance imaging. To date, the diagnostic criteria remain 100% specific, with no autopsy/cerebral biopsy‐proven noncases classified as probable in life. Interpretation: This study confirms that the diagnostic criteria for vCJD are sensitive and specific and provide a useful standard framework for case classification in a surveillance setting. 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number="10"></count></countGroup><titleGroup><title type="articleCategory">Original Article</title><title type="tocHeading1">Original Article</title></titleGroup><copyright ownership="thirdParty">Copyright © 2010 American Neurological Association</copyright><eventGroup><event type="manuscriptReceived" date="2009-10-27"></event><event type="manuscriptRevised" date="2010-01-12"></event><event type="manuscriptAccepted" date="2010-01-15"></event><event type="firstOnline" date="2010-01-27"></event><event type="publishedOnlineFinalForm" date="2010-05-25"></event><event type="publishedOnlineAcceptedOrEarlyUnpaginated" date="2010-01-27"></event><event type="xmlConverted" agent="Converter:JWSART34_TO_WML3G version:2.3.27 mode:FullText" date="2010-12-11"></event><event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:3.8.8" date="2014-01-03"></event><event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.1.7 mode:FullText,remove_FC" date="2014-10-14"></event></eventGroup><numberingGroup><numbering type="pageFirst">761</numbering><numbering type="pageLast">770</numbering></numberingGroup><correspondenceTo>Specialist Registrar, Western General Hospital, Edinburgh, EH4 2XU UK</correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:ANA.ANA21987.pdf"></link></linkGroup></publicationMeta><contentMeta><countGroup><count type="figureTotal" number="4"></count><count type="tableTotal" number="7"></count><count type="referenceTotal" number="16"></count><count type="wordTotal" number="5858"></count></countGroup><titleGroup><title type="main" xml:lang="en">Validation of diagnostic criteria for variant Creutzfeldt–Jakob disease<link href="#fn1"></link></title><title type="short" xml:lang="en">Diagnostic Criteria for vCJD</title></titleGroup><creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><givenNames>Craig A.</givenNames><familyName>Heath</familyName><degrees>MD</degrees></personName><contactDetails><email>craigheath@nhs.net</email></contactDetails></creator><creator xml:id="au2" creatorRole="author" affiliationRef="#af2"><personName><givenNames>Sarah A.</givenNames><familyName>Cooper</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au3" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Katy</givenNames><familyName>Murray</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au4" creatorRole="author" affiliationRef="#af3"><personName><givenNames>Andrea</givenNames><familyName>Lowman</familyName><degrees>MB ChB (Hons), MRCP</degrees></personName></creator><creator xml:id="au5" creatorRole="author" affiliationRef="#af4"><personName><givenNames>Colm</givenNames><familyName>Henry</familyName><degrees>MB MRCPI MRCGP</degrees></personName></creator><creator xml:id="au6" creatorRole="author" affiliationRef="#af5"><personName><givenNames>Margaret A.</givenNames><familyName>MacLeod</familyName><degrees>MD, MRCP</degrees></personName></creator><creator xml:id="au7" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Gillian E.</givenNames><familyName>Stewart</familyName><degrees>MB ChB</degrees></personName></creator><creator xml:id="au8" creatorRole="author" affiliationRef="#af6"><personName><givenNames>Martin</givenNames><familyName>Zeidler</familyName><degrees>FRCP</degrees></personName></creator><creator xml:id="au9" creatorRole="author" affiliationRef="#af7"><personName><givenNames>Jan M.</givenNames><familyName>MacKenzie</familyName></personName></creator><creator xml:id="au10" creatorRole="author" affiliationRef="#af7"><personName><givenNames>James W.</givenNames><familyName>Ironside</familyName><degrees>FRCPath</degrees></personName></creator><creator xml:id="au11" creatorRole="author" affiliationRef="#af1"><personName><givenNames>David M.</givenNames><familyName>Summers</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au12" creatorRole="author" affiliationRef="#af7"><personName><givenNames>Richard S. G.</givenNames><familyName>Knight</familyName><degrees>FRCP</degrees></personName></creator><creator xml:id="au13" creatorRole="author" affiliationRef="#af7"><personName><givenNames>Robert G.</givenNames><familyName>Will</familyName><degrees>FRCP</degrees></personName></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="GB" type="organization"><unparsedAffiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="GB" type="organization"><unparsedAffiliation>Institute of Neurology, Southern General Hospital, Glasgow, UK</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="GB" type="organization"><unparsedAffiliation>Department of Neurology, University Hospital, Cardiff, UK</unparsedAffiliation></affiliation><affiliation xml:id="af4" countryCode="IE" type="organization"><unparsedAffiliation>Department of Medicine of the Elderly, Mercy University Hospital, Cork, Ireland</unparsedAffiliation></affiliation><affiliation xml:id="af5" countryCode="GB" type="organization"><unparsedAffiliation>Department of Neurology, Aberdeen Royal Infirmary, Aberdeen, UK</unparsedAffiliation></affiliation><affiliation xml:id="af6" countryCode="GB" type="organization"><unparsedAffiliation>Department of Neurology, Victoria Hospital, Kirkcaldy, UK</unparsedAffiliation></affiliation><affiliation xml:id="af7" countryCode="GB" type="organization"><unparsedAffiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</unparsedAffiliation></affiliation></affiliationGroup><fundingInfo><fundingAgency>Department of Health and the Scottish Government</fundingAgency><fundingNumber>121/5061</fundingNumber></fundingInfo><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><section xml:id="abs1-1"><title type="main">Objective</title><p>Variant Creutzfeldt–Jakob disease (vCJD), a novel form of human prion disease, was recognized in 1996. The disease affected a younger cohort than sporadic CJD, and the early clinical course was dominated by psychiatric and sensory symptoms. In an attempt to aid diagnosis and establish standardization between surveillance networks, diagnostic criteria were established. These were devised from the features of a small number of cases and modified in 2000 as the clinical phenotype was established. Since then, only minor changes have been introduced; revalidation of the criteria in the current format is overdue.</p></section><section xml:id="abs1-2"><title type="main">Methods</title><p>Included in this study are autopsy/cerebral biopsy‐proven cases of vCJD referred to the National CJD Surveillance Unit (NCJDSU) between 1995 and 2004 and suspect cases in which an alternative diagnosis was identified following autopsy/cerebral biopsy.</p></section><section xml:id="abs1-3"><title type="main">Results</title><p>Over the 10‐year period, 106 definite cases of vCJD and 45 pathologically confirmed “noncases” were identified from the archives of the NCJDSU. The median age at onset of the cases was significantly younger than that of the noncases (27 years [range, 12–74 years] vs 43 years [range, 10–64 years]), and the median duration of illness was significantly shorter (14 months [range, 6–39 months] vs 22 months [range, 2–139 months]). The most commonly identified core clinical feature in cases was dementia; persistent painful sensory symptoms were the least frequent. Eighty‐eight of 106 (83%) vCJD cases were retrospectively classified as probable in life, 6 cases were classified as possible. Most cases were classified as probable on the basis of core clinical features and brain magnetic resonance imaging. To date, the diagnostic criteria remain 100% specific, with no autopsy/cerebral biopsy‐proven noncases classified as probable in life.</p></section><section xml:id="abs1-4"><title type="main">Interpretation</title><p>This study confirms that the diagnostic criteria for vCJD are sensitive and specific and provide a useful standard framework for case classification in a surveillance setting. ANN NEUROL 2010</p></section></abstract></abstractGroup></contentMeta><noteGroup><note xml:id="fn1"><p>The authors report no conflicts of interest.</p></note></noteGroup></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Validation of diagnostic criteria for variant Creutzfeldt–Jakob disease</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Diagnostic Criteria for vCJD</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Validation of diagnostic criteria for variant Creutzfeldt–Jakob disease</title></titleInfo><name type="personal"><namePart type="given">Craig A.</namePart><namePart type="family">Heath</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</affiliation><description>Correspondence: Specialist Registrar, Western General Hospital, Edinburgh, EH4 2XU UK</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Sarah A.</namePart><namePart type="family">Cooper</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Institute of Neurology, Southern General Hospital, Glasgow, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Katy</namePart><namePart type="family">Murray</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Andrea</namePart><namePart type="family">Lowman</namePart><namePart type="termsOfAddress">MB ChB (Hons), MRCP</namePart><affiliation>Department of Neurology, University Hospital, Cardiff, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Colm</namePart><namePart type="family">Henry</namePart><namePart type="termsOfAddress">MB MRCPI MRCGP</namePart><affiliation>Department of Medicine of the Elderly, Mercy University Hospital, Cork, Ireland</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Margaret A.</namePart><namePart type="family">MacLeod</namePart><namePart type="termsOfAddress">MD, MRCP</namePart><affiliation>Department of Neurology, Aberdeen Royal Infirmary, Aberdeen, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Gillian E.</namePart><namePart type="family">Stewart</namePart><namePart type="termsOfAddress">MB ChB</namePart><affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Martin</namePart><namePart type="family">Zeidler</namePart><namePart type="termsOfAddress">FRCP</namePart><affiliation>Department of Neurology, Victoria Hospital, Kirkcaldy, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Jan M.</namePart><namePart type="family">MacKenzie</namePart><affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">James W.</namePart><namePart type="family">Ironside</namePart><namePart type="termsOfAddress">FRCPath</namePart><affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">David M.</namePart><namePart type="family">Summers</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Richard S. G.</namePart><namePart type="family">Knight</namePart><namePart type="termsOfAddress">FRCP</namePart><affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Robert G.</namePart><namePart type="family">Will</namePart><namePart type="termsOfAddress">FRCP</namePart><affiliation>National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="article" displayLabel="article"></genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">2010-06</dateIssued><dateCaptured encoding="w3cdtf">2009-10-27</dateCaptured><dateValid encoding="w3cdtf">2010-01-15</dateValid><copyrightDate encoding="w3cdtf">2010</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType><extent unit="figures">4</extent><extent unit="tables">7</extent><extent unit="references">16</extent><extent unit="words">5858</extent></physicalDescription><abstract lang="en">Objective: Variant Creutzfeldt–Jakob disease (vCJD), a novel form of human prion disease, was recognized in 1996. The disease affected a younger cohort than sporadic CJD, and the early clinical course was dominated by psychiatric and sensory symptoms. In an attempt to aid diagnosis and establish standardization between surveillance networks, diagnostic criteria were established. These were devised from the features of a small number of cases and modified in 2000 as the clinical phenotype was established. Since then, only minor changes have been introduced; revalidation of the criteria in the current format is overdue. Methods: Included in this study are autopsy/cerebral biopsy‐proven cases of vCJD referred to the National CJD Surveillance Unit (NCJDSU) between 1995 and 2004 and suspect cases in which an alternative diagnosis was identified following autopsy/cerebral biopsy. Results: Over the 10‐year period, 106 definite cases of vCJD and 45 pathologically confirmed “noncases” were identified from the archives of the NCJDSU. The median age at onset of the cases was significantly younger than that of the noncases (27 years [range, 12–74 years] vs 43 years [range, 10–64 years]), and the median duration of illness was significantly shorter (14 months [range, 6–39 months] vs 22 months [range, 2–139 months]). The most commonly identified core clinical feature in cases was dementia; persistent painful sensory symptoms were the least frequent. Eighty‐eight of 106 (83%) vCJD cases were retrospectively classified as probable in life, 6 cases were classified as possible. Most cases were classified as probable on the basis of core clinical features and brain magnetic resonance imaging. To date, the diagnostic criteria remain 100% specific, with no autopsy/cerebral biopsy‐proven noncases classified as probable in life. Interpretation: This study confirms that the diagnostic criteria for vCJD are sensitive and specific and provide a useful standard framework for case classification in a surveillance setting. ANN NEUROL 2010</abstract><note type="content">*The authors report no conflicts of interest.</note><note type="funding">Department of Health and the Scottish Government - No. 121/5061; </note><relatedItem type="host"><titleInfo><title>Annals of Neurology</title></titleInfo><titleInfo type="abbreviated"><title>Ann Neurol.</title></titleInfo><genre type="Journal">journal</genre><subject><genre>article category</genre><topic>Original Article</topic></subject><identifier type="ISSN">0364-5134</identifier><identifier type="eISSN">1531-8249</identifier><identifier type="DOI">10.1002/(ISSN)1531-8249</identifier><identifier type="PublisherID">ANA</identifier><part><date>2010</date><detail type="volume"><caption>vol.</caption><number>67</number></detail><detail type="issue"><caption>no.</caption><number>6</number></detail><extent unit="pages"><start>761</start><end>770</end><total>10</total></extent></part></relatedItem><identifier type="istex">E92195887B6E2ED5288C8E320329AB420842CF22</identifier><identifier type="DOI">10.1002/ana.21987</identifier><identifier type="ArticleID">ANA21987</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2010 American Neurological Association</accessCondition><recordInfo><recordContentSource>WILEY</recordContentSource><recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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