Validation of diagnostic criteria for variant Creutzfeldt–Jakob disease
Identifieur interne : 000495 ( Main/Exploration ); précédent : 000494; suivant : 000496Validation of diagnostic criteria for variant Creutzfeldt–Jakob disease
Auteurs : Craig A. Heath [Royaume-Uni] ; Sarah A. Cooper [Royaume-Uni] ; Katy Murray [Royaume-Uni] ; Andrea Lowman [Royaume-Uni] ; Colm Henry [Irlande (pays)] ; Margaret A. Macleod [Royaume-Uni] ; Gillian E. Stewart [Royaume-Uni] ; Martin Zeidler [Royaume-Uni] ; Jan M. Mackenzie [Royaume-Uni] ; James W. Ironside [Royaume-Uni] ; David M. Summers [Royaume-Uni] ; Richard S. G. Knight [Royaume-Uni] ; Robert G. Will [Royaume-Uni]Source :
- 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
Affiliations:
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Macleod</name><affiliation wicri:level="1"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Neurology, Aberdeen Royal Infirmary, Aberdeen</wicri:regionArea><wicri:noRegion>Aberdeen</wicri:noRegion></affiliation></author><author><name sortKey="Stewart, Gillian E" sort="Stewart, Gillian E" uniqKey="Stewart G" first="Gillian E." last="Stewart">Gillian E. 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Mackenzie</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>National CJD Surveillance Unit, Western General Hospital, Edinburgh</wicri:regionArea><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></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 wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>National CJD Surveillance Unit, Western General Hospital, Edinburgh</wicri:regionArea><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></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 wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Clinical Neurosciences, Western General Hospital, Edinburgh</wicri:regionArea><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></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. Knight</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>National CJD Surveillance Unit, Western General Hospital, Edinburgh</wicri:regionArea><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></affiliation></author><author><name sortKey="Will, Robert G" sort="Will, Robert G" uniqKey="Will R" first="Robert G." last="Will">Robert G. Will</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>National CJD Surveillance Unit, Western General Hospital, Edinburgh</wicri:regionArea><placeName><settlement type="city">Édimbourg</settlement><region type="country">Écosse</region></placeName></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><affiliations><list><country><li>Irlande (pays)</li><li>Royaume-Uni</li></country><region><li>Écosse</li></region><settlement><li>Édimbourg</li></settlement></list><tree><country name="Royaume-Uni"><region name="Écosse"><name sortKey="Heath, Craig A" sort="Heath, Craig A" uniqKey="Heath C" first="Craig A." last="Heath">Craig A. Heath</name></region><name sortKey="Cooper, Sarah A" sort="Cooper, Sarah A" uniqKey="Cooper S" first="Sarah A." last="Cooper">Sarah A. Cooper</name><name sortKey="Ironside, James W" sort="Ironside, James W" uniqKey="Ironside J" first="James W." last="Ironside">James W. Ironside</name><name sortKey="Knight, Richard S G" sort="Knight, Richard S G" uniqKey="Knight R" first="Richard S. G." last="Knight">Richard S. G. Knight</name><name sortKey="Lowman, Andrea" sort="Lowman, Andrea" uniqKey="Lowman A" first="Andrea" last="Lowman">Andrea Lowman</name><name sortKey="Mackenzie, Jan M" sort="Mackenzie, Jan M" uniqKey="Mackenzie J" first="Jan M." last="Mackenzie">Jan M. Mackenzie</name><name sortKey="Macleod, Margaret A" sort="Macleod, Margaret A" uniqKey="Macleod M" first="Margaret A." last="Macleod">Margaret A. Macleod</name><name sortKey="Murray, Katy" sort="Murray, Katy" uniqKey="Murray K" first="Katy" last="Murray">Katy Murray</name><name sortKey="Stewart, Gillian E" sort="Stewart, Gillian E" uniqKey="Stewart G" first="Gillian E." last="Stewart">Gillian E. Stewart</name><name sortKey="Summers, David M" sort="Summers, David M" uniqKey="Summers D" first="David M." last="Summers">David M. Summers</name><name sortKey="Will, Robert G" sort="Will, Robert G" uniqKey="Will R" first="Robert G." last="Will">Robert G. Will</name><name sortKey="Zeidler, Martin" sort="Zeidler, Martin" uniqKey="Zeidler M" first="Martin" last="Zeidler">Martin Zeidler</name></country><country name="Irlande (pays)"><noRegion><name sortKey="Henry, Colm" sort="Henry, Colm" uniqKey="Henry C" first="Colm" last="Henry">Colm Henry</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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