Progressive supranuclear palsy and corticobasal degeneration: Lumping versus splitting
Identifieur interne : 001210 ( Main/Corpus ); précédent : 001209; suivant : 001211Progressive supranuclear palsy and corticobasal degeneration: Lumping versus splitting
Auteurs : Tomaso Scaravilli ; Eduardo Tolosa ; Isidre FerrerSource :
- Movement Disorders [ 0885-3185 ] ; 2005-08.
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Abstract
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are both sporadic disorders with tau pathology. Criteria have been defined that in most instances allow for adequate diagnosis of the two disorders both clinically and neuropathologically; however, overlap is not uncommon. For example, patients with PSP may present with severe unilateral apraxia and supranuclear gaze palsy can occur in CBD. Pathological overlap also occurs and pathologically “mixed” cases are encountered. Common to both these two tauopathies is that isoforms of four‐repeat tau due to splicing of exon 10 define the tau filamentous aggregates. This is in contrast to other tau disorders such as Pick's with three‐repeat tau aggregates. Additional evidence for a causal link between PSP and CBD is the finding that both disorders are homozygous for the H1 tau haplotype. Furthermore, in some families with parkinsonism linked to defined mutations of the tau gene (FTDP‐17), involved relatives have presented with PSP whereas others with the CBD phenotype. Although PSP and CBD frequently can be clearly separated clinically and pathologically, the degree of clinicopathological and genetic overlap is important and suggests that they represent different phenotypes of the same disorder, with differences occurring perhaps in relation to different genetic background. That PSP and CBD are distinct nosological entities occurring in patients with similar genetic predisposition cannot be ruled out. © 2005 Movement Disorder Society
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DOI: 10.1002/mds.20536
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Although PSP and CBD frequently can be clearly separated clinically and pathologically, the degree of clinicopathological and genetic overlap is important and suggests that they represent different phenotypes of the same disorder, with differences occurring perhaps in relation to different genetic background. That PSP and CBD are distinct nosological entities occurring in patients with similar genetic predisposition cannot be ruled out. © 2005 Movement Disorder Society</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Tomaso Scaravilli MD</name><affiliations><json:string>Department of Neuroscience, University of Padua, Padua, Italy</json:string></affiliations></json:item><json:item><name>Eduardo Tolosa MD</name><affiliations><json:string>Parkinson's Disease and Movement Disorders Unit, Neurology Service, Institut Clínic de Malalties del Sistema Nerviós. 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Although PSP and CBD frequently can be clearly separated clinically and pathologically, the degree of clinicopathological and genetic overlap is important and suggests that they represent different phenotypes of the same disorder, with differences occurring perhaps in relation to different genetic background. 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Although PSP and CBD frequently can be clearly separated clinically and pathologically, the degree of clinicopathological and genetic overlap is important and suggests that they represent different phenotypes of the same disorder, with differences occurring perhaps in relation to different genetic background. That PSP and CBD are distinct nosological entities occurring in patients with similar genetic predisposition cannot be ruled out. © 2005 Movement Disorder Society</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Progressive supranuclear palsy and corticobasal degeneration: Lumping versus splitting</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>PSP and CBD: Lumping Versus Splitting</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Progressive supranuclear palsy and corticobasal degeneration: Lumping versus splitting</title></titleInfo><name type="personal"><namePart type="given">Tomaso</namePart><namePart type="family">Scaravilli</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neuroscience, University of Padua, Padua, Italy</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Eduardo</namePart><namePart type="family">Tolosa</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Parkinson's Disease and Movement Disorders Unit, Neurology Service, Institut Clínic de Malalties del Sistema Nerviós. Hospital Clínic de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain</affiliation><description>Correspondence: Neurology Service, Hospital Clinic, University of Barcelona, Barcelona 0836, Spain</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Isidre</namePart><namePart type="family">Ferrer</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Institut de Neuropatologia, Servei Anatomia Patològica, Hospital de Bellvitge, Hospitalet de Llobregat, Spain</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="review-article" displayLabel="reviewArticle"></genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">2005-08</dateIssued><copyrightDate encoding="w3cdtf">2005</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="references">75</extent><extent unit="words">6271</extent></physicalDescription><abstract lang="en">Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are both sporadic disorders with tau pathology. Criteria have been defined that in most instances allow for adequate diagnosis of the two disorders both clinically and neuropathologically; however, overlap is not uncommon. For example, patients with PSP may present with severe unilateral apraxia and supranuclear gaze palsy can occur in CBD. Pathological overlap also occurs and pathologically “mixed” cases are encountered. Common to both these two tauopathies is that isoforms of four‐repeat tau due to splicing of exon 10 define the tau filamentous aggregates. This is in contrast to other tau disorders such as Pick's with three‐repeat tau aggregates. Additional evidence for a causal link between PSP and CBD is the finding that both disorders are homozygous for the H1 tau haplotype. Furthermore, in some families with parkinsonism linked to defined mutations of the tau gene (FTDP‐17), involved relatives have presented with PSP whereas others with the CBD phenotype. Although PSP and CBD frequently can be clearly separated clinically and pathologically, the degree of clinicopathological and genetic overlap is important and suggests that they represent different phenotypes of the same disorder, with differences occurring perhaps in relation to different genetic background. That PSP and CBD are distinct nosological entities occurring in patients with similar genetic predisposition cannot be ruled out. © 2005 Movement Disorder Society</abstract><note type="funding">Red CIEN IDIBAPS‐ISCIII RTIC - No. C03/06; </note><note type="funding">FIS - No. C03/006; </note><subject lang="en"><genre>Keywords</genre><topic>PSP</topic><topic>CBD</topic><topic>tau disorders</topic><topic>neurodegen‐eration</topic></subject><relatedItem type="host"><titleInfo><title>Movement Disorders</title><subTitle>Official Journal of the Movement Disorder Society</subTitle></titleInfo><titleInfo type="abbreviated"><title>Mov. Disord.</title></titleInfo><name type="personal"><namePart type="given">Werner</namePart><namePart type="family">Poewe</namePart><namePart type="termsOfAddress">MD</namePart></name><name type="personal"><namePart type="given">Gregor</namePart><namePart type="family">Wenning</namePart><namePart type="termsOfAddress">MD, PhD</namePart></name><genre type="Journal">journal</genre><subject><genre>article category</genre><topic>Research Review</topic></subject><identifier type="ISSN">0885-3185</identifier><identifier type="eISSN">1531-8257</identifier><identifier type="DOI">10.1002/(ISSN)1531-8257</identifier><identifier type="PublisherID">MDS</identifier><part><date>2005</date><detail type="volume"><caption>vol.</caption><number>20</number></detail><detail type="issue"><caption>no.</caption><number>S12</number></detail><detail type="supplement"><caption>Suppl. no.</caption><number>S12</number></detail><extent unit="pages"><start>S21</start><end>S28</end><total>8</total></extent></part></relatedItem><identifier type="istex">3BAD8FFCD7D37414D82D222CF372403E83C140C3</identifier><identifier type="DOI">10.1002/mds.20536</identifier><identifier type="ArticleID">MDS20536</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2005 Movement Disorder Society</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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