Unusual clinical presentations of cortical‐basal ganglionic degeneration
Identifieur interne : 002544 ( Istex/Corpus ); précédent : 002543; suivant : 002545Unusual clinical presentations of cortical‐basal ganglionic degeneration
Auteurs : C. Bergeron ; M. S. Pollanen ; L. Weyer ; S. E. Black ; A. E. LangSource :
- Annals of Neurology [ 0364-5134 ] ; 1996-12.
Abstract
Cortical‐basal ganglionic degeneration classically presents predominantly as a motor disorder with a unique constellation of histological alterations characterized by the presence of neuronal loss and gliosis in a selective distribution, swollen achromasic neurons, and extensive deposition of abnormal tau in neurons and glia. We now report 3 patients with this distinctive pathology who presented with cognitive changes and only mild or delayed motor symptoms. In 2 patients with severe dementia, pathological changes were extensive in the anterior frontal lobe, amygdala, and hippocampus. In a third patient who had an isolated speech disturbance for 5 years before developing the more typical motor features of cortical‐basal ganglionic degeneration, the most severe changes were observed in the left motor cortex and adjacent Broca's area. It is therefore apparent that the histological changes of cortical‐basal ganglionic degeneration result in a variety of clinical presentations depending on the topography of the lesions. On this basis we conclude that cortical‐ basal ganglionic degeneration should be considered in the differential diagnosis of language disturbances and dementia, particularly when the latter is accompanied by frontal lobe symptomatology, early speech alterations, or parkinsonism.
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DOI: 10.1002/ana.410400611
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Bergeron</name><affiliation><mods:affiliation>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</mods:affiliation></affiliation><affiliation><mods:affiliation>Departments of Medicine (Neurology), University of Toronto, Toronto</mods:affiliation></affiliation><affiliation><mods:affiliation>Departments of pathology (Neuropathology), The Toronto Hospital, Toronto</mods:affiliation></affiliation><affiliation><mods:affiliation>Centre for Research in Neurodegenerative Diseases, University of Toronto, 6 Queen's Park West, Toronto, M5S 3H2 Ontario, Canada</mods:affiliation></affiliation></author><author><name sortKey="Pollanen, M S" sort="Pollanen, M S" uniqKey="Pollanen M" first="M. S." last="Pollanen">M. S. 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Pollanen</name><affiliation><mods:affiliation>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</mods:affiliation></affiliation></author><author><name sortKey="Weyer, L" sort="Weyer, L" uniqKey="Weyer L" first="L." last="Weyer">L. Weyer</name><affiliation><mods:affiliation>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</mods:affiliation></affiliation></author><author><name sortKey="Black, S E" sort="Black, S E" uniqKey="Black S" first="S. E." last="Black">S. E. Black</name><affiliation><mods:affiliation>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</mods:affiliation></affiliation><affiliation><mods:affiliation>Departments of Pathology (Neuropathology), University of Toronto, Toronto</mods:affiliation></affiliation><affiliation><mods:affiliation>The Department of Medicine (Neurology), Toronto, Ontario, Canada</mods:affiliation></affiliation><affiliation><mods:affiliation>Research Program in Aging, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada</mods:affiliation></affiliation></author><author><name sortKey="Lang, A E" sort="Lang, A E" uniqKey="Lang A" first="A. E." last="Lang">A. E. Lang</name><affiliation><mods:affiliation>Departments of Medicine (Neurology), University of Toronto, Toronto</mods:affiliation></affiliation><affiliation><mods:affiliation>Medicine (Neurology), The Toronto Hospital, Toronto</mods:affiliation></affiliation><affiliation><mods:affiliation>Morton and Gloria Shulman Movement Disorder Centre, The Toronto Hospital, Toronto</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Annals of Neurology</title><title level="j" type="sub">Official Journal of the American Neurological Association and the Child Neurology Society</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="1996-12">1996-12</date><biblScope unit="volume">40</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="893">893</biblScope><biblScope unit="page" to="900">900</biblScope></imprint><idno type="ISSN">0364-5134</idno></series><idno type="istex">4FC5057538C8A8E6F4777DAA9CCF08FB385F2111</idno><idno type="DOI">10.1002/ana.410400611</idno><idno type="ArticleID">ANA410400611</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">Cortical‐basal ganglionic degeneration classically presents predominantly as a motor disorder with a unique constellation of histological alterations characterized by the presence of neuronal loss and gliosis in a selective distribution, swollen achromasic neurons, and extensive deposition of abnormal tau in neurons and glia. We now report 3 patients with this distinctive pathology who presented with cognitive changes and only mild or delayed motor symptoms. In 2 patients with severe dementia, pathological changes were extensive in the anterior frontal lobe, amygdala, and hippocampus. In a third patient who had an isolated speech disturbance for 5 years before developing the more typical motor features of cortical‐basal ganglionic degeneration, the most severe changes were observed in the left motor cortex and adjacent Broca's area. It is therefore apparent that the histological changes of cortical‐basal ganglionic degeneration result in a variety of clinical presentations depending on the topography of the lesions. On this basis we conclude that cortical‐ basal ganglionic degeneration should be considered in the differential diagnosis of language disturbances and dementia, particularly when the latter is accompanied by frontal lobe symptomatology, early speech alterations, or parkinsonism.</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>C. Bergeron Dr., MD</name><affiliations><json:string>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</json:string><json:string>Departments of Medicine (Neurology), University of Toronto, Toronto</json:string><json:string>Departments of pathology (Neuropathology), The Toronto Hospital, Toronto</json:string><json:string>Centre for Research in Neurodegenerative Diseases, University of Toronto, 6 Queen's Park West, Toronto, M5S 3H2 Ontario, Canada</json:string></affiliations></json:item><json:item><name>M. S. 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Lang MD</name><affiliations><json:string>Departments of Medicine (Neurology), University of Toronto, Toronto</json:string><json:string>Medicine (Neurology), The Toronto Hospital, Toronto</json:string><json:string>Morton and Gloria Shulman Movement Disorder Centre, The Toronto Hospital, Toronto</json:string></affiliations></json:item></author><articleId><json:string>ANA410400611</json:string></articleId><language><json:string>eng</json:string></language><originalGenre><json:string>article</json:string></originalGenre><abstract>Cortical‐basal ganglionic degeneration classically presents predominantly as a motor disorder with a unique constellation of histological alterations characterized by the presence of neuronal loss and gliosis in a selective distribution, swollen achromasic neurons, and extensive deposition of abnormal tau in neurons and glia. We now report 3 patients with this distinctive pathology who presented with cognitive changes and only mild or delayed motor symptoms. In 2 patients with severe dementia, pathological changes were extensive in the anterior frontal lobe, amygdala, and hippocampus. In a third patient who had an isolated speech disturbance for 5 years before developing the more typical motor features of cortical‐basal ganglionic degeneration, the most severe changes were observed in the left motor cortex and adjacent Broca's area. It is therefore apparent that the histological changes of cortical‐basal ganglionic degeneration result in a variety of clinical presentations depending on the topography of the lesions. On this basis we conclude that cortical‐ basal ganglionic degeneration should be considered in the differential diagnosis of language disturbances and dementia, particularly when the latter is accompanied by frontal lobe symptomatology, early speech alterations, or parkinsonism.</abstract><qualityIndicators><score>6.466</score><pdfVersion>1.3</pdfVersion><pdfPageSize>576 x 792 pts</pdfPageSize><refBibsNative>true</refBibsNative><abstractCharCount>1311</abstractCharCount><pdfWordCount>4258</pdfWordCount><pdfCharCount>27883</pdfCharCount><pdfPageCount>8</pdfPageCount><abstractWordCount>184</abstractWordCount></qualityIndicators><title>Unusual clinical presentations of cortical‐basal ganglionic degeneration</title><genre><json:string>article</json:string></genre><host><title>Annals of Neurology</title><language><json:string>unknown</json:string></language><doi><json:string>10.1002/(ISSN)1531-8249</json:string></doi><issn><json:string>0364-5134</json:string></issn><eissn><json:string>1531-8249</json:string></eissn><publisherId><json:string>ANA</json:string></publisherId><volume>40</volume><issue>6</issue><pages><first>893</first><last>900</last><total>8</total></pages><genre><json:string>journal</json:string></genre><subject><json:item><value>Article</value></json:item></subject></host><categories><wos><json:string>science</json:string><json:string>neurosciences</json:string><json:string>clinical neurology</json:string></wos><scienceMetrix><json:string>health sciences</json:string><json:string>clinical medicine</json:string><json:string>neurology & neurosurgery</json:string></scienceMetrix><inist><json:string>sciences appliquees, technologies et medecines</json:string><json:string>sciences biologiques et medicales</json:string><json:string>sciences medicales</json:string></inist></categories><publicationDate>1996</publicationDate><copyrightDate>1996</copyrightDate><doi><json:string>10.1002/ana.410400611</json:string></doi><id>4FC5057538C8A8E6F4777DAA9CCF08FB385F2111</id><fulltext><json:item><extension>pdf</extension><original>true</original><mimetype>application/pdf</mimetype><uri>https://api-v5.istex.fr/document/4FC5057538C8A8E6F4777DAA9CCF08FB385F2111/fulltext/pdf</uri></json:item><json:item><extension>zip</extension><original>false</original><mimetype>application/zip</mimetype><uri>https://api-v5.istex.fr/document/4FC5057538C8A8E6F4777DAA9CCF08FB385F2111/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api-v5.istex.fr/document/4FC5057538C8A8E6F4777DAA9CCF08FB385F2111/fulltext/tei"><teiHeader><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Unusual clinical presentations of cortical‐basal ganglionic degeneration</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><availability><p>Copyright © 1996 American Neurological Association</p></availability><date>1996</date></publicationStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Unusual clinical presentations of cortical‐basal ganglionic degeneration</title><author xml:id="author-1"><persName><forename type="first">C.</forename><surname>Bergeron</surname></persName><roleName type="degree">Dr., MD</roleName><affiliation>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</affiliation><affiliation>Departments of Medicine (Neurology), University of Toronto, Toronto</affiliation><affiliation>Departments of pathology (Neuropathology), The Toronto Hospital, Toronto</affiliation><affiliation>Centre for Research in Neurodegenerative Diseases, University of Toronto, 6 Queen's Park West, Toronto, M5S 3H2 Ontario, Canada</affiliation></author><author xml:id="author-2"><persName><forename type="first">M. 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E.</forename><surname>Lang</surname></persName><roleName type="degree">MD</roleName><affiliation>Departments of Medicine (Neurology), University of Toronto, Toronto</affiliation><affiliation>Medicine (Neurology), The Toronto Hospital, Toronto</affiliation><affiliation>Morton and Gloria Shulman Movement Disorder Centre, The Toronto Hospital, Toronto</affiliation></author></analytic><monogr><title level="j">Annals of Neurology</title><title level="j" type="sub">Official Journal of the American Neurological Association and the Child Neurology Society</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="1996-12"></date><biblScope unit="volume">40</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="893">893</biblScope><biblScope unit="page" to="900">900</biblScope></imprint></monogr><idno type="istex">4FC5057538C8A8E6F4777DAA9CCF08FB385F2111</idno><idno type="DOI">10.1002/ana.410400611</idno><idno type="ArticleID">ANA410400611</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>1996</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Cortical‐basal ganglionic degeneration classically presents predominantly as a motor disorder with a unique constellation of histological alterations characterized by the presence of neuronal loss and gliosis in a selective distribution, swollen achromasic neurons, and extensive deposition of abnormal tau in neurons and glia. We now report 3 patients with this distinctive pathology who presented with cognitive changes and only mild or delayed motor symptoms. In 2 patients with severe dementia, pathological changes were extensive in the anterior frontal lobe, amygdala, and hippocampus. In a third patient who had an isolated speech disturbance for 5 years before developing the more typical motor features of cortical‐basal ganglionic degeneration, the most severe changes were observed in the left motor cortex and adjacent Broca's area. It is therefore apparent that the histological changes of cortical‐basal ganglionic degeneration result in a variety of clinical presentations depending on the topography of the lesions. On this basis we conclude that cortical‐ basal ganglionic degeneration should be considered in the differential diagnosis of language disturbances and dementia, particularly when the latter is accompanied by frontal lobe symptomatology, early speech alterations, or parkinsonism.</p></abstract><textClass><keywords scheme="Journal Subject"><list><head>article-category</head><item><term>Article</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="1996-01-19">Received</change><change when="1996-05-29">Registration</change><change when="1996-12">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><extension>txt</extension><original>false</original><mimetype>text/plain</mimetype><uri>https://api-v5.istex.fr/document/4FC5057538C8A8E6F4777DAA9CCF08FB385F2111/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration><istex:document><component version="2.0" type="serialArticle" xml:lang="en"><header><publicationMeta level="product"><publisherInfo><publisherName>Wiley Subscription Services, Inc., A Wiley Company</publisherName><publisherLoc>Hoboken</publisherLoc></publisherInfo><doi registered="yes">10.1002/(ISSN)1531-8249</doi><issn type="print">0364-5134</issn><issn type="electronic">1531-8249</issn><idGroup><id type="product" value="ANA"></id></idGroup><titleGroup><title type="main" xml:lang="en" sort="ANNALS OF NEUROLOGY">Annals of Neurology</title><title type="subtitle">Official Journal of the American Neurological Association and the Child Neurology Society</title><title type="short">Ann Neurol.</title></titleGroup></publicationMeta><publicationMeta level="part" position="60"><doi origin="wiley" registered="yes">10.1002/ana.v40:6</doi><numberingGroup><numbering type="journalVolume" number="40">40</numbering><numbering type="journalIssue">6</numbering></numberingGroup><coverDate startDate="1996-12">December 1996</coverDate></publicationMeta><publicationMeta level="unit" type="article" position="11" status="forIssue"><doi origin="wiley" registered="yes">10.1002/ana.410400611</doi><idGroup><id type="unit" value="ANA410400611"></id></idGroup><countGroup><count type="pageTotal" number="8"></count></countGroup><titleGroup><title type="articleCategory">Article</title><title type="tocHeading1">Articles</title></titleGroup><copyright ownership="thirdParty">Copyright © 1996 American Neurological Association</copyright><eventGroup><event type="manuscriptReceived" date="1996-01-19"></event><event type="manuscriptRevised" date="1996-05-28"></event><event type="manuscriptAccepted" date="1996-05-29"></event><event type="firstOnline" date="2004-10-16"></event><event type="publishedOnlineFinalForm" date="2004-10-16"></event><event type="xmlConverted" agent="Converter:JWSART34_TO_WML3G version:2.3.1 mode:FullText source:HeaderRef result:HeaderRef" date="2010-02-23"></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">893</numbering><numbering type="pageLast">900</numbering></numberingGroup><correspondenceTo>Centre for Research in Neurodegenerative Diseases, University of Toronto, 6 Queen's Park West, Toronto, M5S 3H2 Ontario, Canada</correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:ANA.ANA410400611.pdf"></link></linkGroup></publicationMeta><contentMeta><countGroup><count type="figureTotal" number="6"></count><count type="tableTotal" number="0"></count><count type="referenceTotal" number="29"></count></countGroup><titleGroup><title type="main" xml:lang="en">Unusual clinical presentations of cortical‐basal ganglionic degeneration</title><title type="short" xml:lang="en">Unusual Presentations CBGD</title></titleGroup><creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1 #af3 #af4" corresponding="yes"><personName><givenNames>C.</givenNames><familyName>Bergeron</familyName><degrees>Dr., MD</degrees></personName></creator><creator xml:id="au2" creatorRole="author" affiliationRef="#af1"><personName><givenNames>M. 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E.</givenNames><familyName>Lang</familyName><degrees>MD</degrees></personName></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="CA" type="organization"><unparsedAffiliation>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="CA" type="organization"><unparsedAffiliation>Departments of Pathology (Neuropathology), University of Toronto, Toronto</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="CA" type="organization"><unparsedAffiliation>Departments of Medicine (Neurology), University of Toronto, Toronto</unparsedAffiliation></affiliation><affiliation xml:id="af4" countryCode="CA" type="organization"><unparsedAffiliation>Departments of pathology (Neuropathology), The Toronto Hospital, Toronto</unparsedAffiliation></affiliation><affiliation xml:id="af5" countryCode="CA" type="organization"><unparsedAffiliation>Medicine (Neurology), The Toronto Hospital, Toronto</unparsedAffiliation></affiliation><affiliation xml:id="af6" countryCode="CA" type="organization"><unparsedAffiliation>Morton and Gloria Shulman Movement Disorder Centre, The Toronto Hospital, Toronto</unparsedAffiliation></affiliation><affiliation xml:id="af7" countryCode="CA" type="organization"><unparsedAffiliation>The Department of Medicine (Neurology), Toronto, Ontario, Canada</unparsedAffiliation></affiliation><affiliation xml:id="af8" countryCode="CA" type="organization"><unparsedAffiliation>Research Program in Aging, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada</unparsedAffiliation></affiliation></affiliationGroup><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>Cortical‐basal ganglionic degeneration classically presents predominantly as a motor disorder with a unique constellation of histological alterations characterized by the presence of neuronal loss and gliosis in a selective distribution, swollen achromasic neurons, and extensive deposition of abnormal tau in neurons and glia. We now report 3 patients with this distinctive pathology who presented with cognitive changes and only mild or delayed motor symptoms. In 2 patients with severe dementia, pathological changes were extensive in the anterior frontal lobe, amygdala, and hippocampus. In a third patient who had an isolated speech disturbance for 5 years before developing the more typical motor features of cortical‐basal ganglionic degeneration, the most severe changes were observed in the left motor cortex and adjacent Broca's area. It is therefore apparent that the histological changes of cortical‐basal ganglionic degeneration result in a variety of clinical presentations depending on the topography of the lesions. On this basis we conclude that cortical‐ basal ganglionic degeneration should be considered in the differential diagnosis of language disturbances and dementia, particularly when the latter is accompanied by frontal lobe symptomatology, early speech alterations, or parkinsonism.</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Unusual clinical presentations of cortical‐basal ganglionic degeneration</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Unusual Presentations CBGD</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Unusual clinical presentations of cortical‐basal ganglionic degeneration</title></titleInfo><name type="personal"><namePart type="given">C.</namePart><namePart type="family">Bergeron</namePart><namePart type="termsOfAddress">Dr., MD</namePart><affiliation>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</affiliation><affiliation>Departments of Medicine (Neurology), University of Toronto, Toronto</affiliation><affiliation>Departments of pathology (Neuropathology), The Toronto Hospital, Toronto</affiliation><affiliation>Centre for Research in Neurodegenerative Diseases, University of Toronto, 6 Queen's Park West, Toronto, M5S 3H2 Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">M. S.</namePart><namePart type="family">Pollanen</namePart><namePart type="termsOfAddress">PhD</namePart><affiliation>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">L.</namePart><namePart type="family">Weyer</namePart><namePart type="termsOfAddress">MLT</namePart><affiliation>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">S. E.</namePart><namePart type="family">Black</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto</affiliation><affiliation>Departments of Pathology (Neuropathology), University of Toronto, Toronto</affiliation><affiliation>The Department of Medicine (Neurology), Toronto, Ontario, Canada</affiliation><affiliation>Research Program in Aging, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">A. E.</namePart><namePart type="family">Lang</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Departments of Medicine (Neurology), University of Toronto, Toronto</affiliation><affiliation>Medicine (Neurology), The Toronto Hospital, Toronto</affiliation><affiliation>Morton and Gloria Shulman Movement Disorder Centre, The Toronto Hospital, Toronto</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">1996-12</dateIssued><dateCaptured encoding="w3cdtf">1996-01-19</dateCaptured><dateValid encoding="w3cdtf">1996-05-29</dateValid><copyrightDate encoding="w3cdtf">1996</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">6</extent><extent unit="references">29</extent></physicalDescription><abstract lang="en">Cortical‐basal ganglionic degeneration classically presents predominantly as a motor disorder with a unique constellation of histological alterations characterized by the presence of neuronal loss and gliosis in a selective distribution, swollen achromasic neurons, and extensive deposition of abnormal tau in neurons and glia. We now report 3 patients with this distinctive pathology who presented with cognitive changes and only mild or delayed motor symptoms. In 2 patients with severe dementia, pathological changes were extensive in the anterior frontal lobe, amygdala, and hippocampus. In a third patient who had an isolated speech disturbance for 5 years before developing the more typical motor features of cortical‐basal ganglionic degeneration, the most severe changes were observed in the left motor cortex and adjacent Broca's area. It is therefore apparent that the histological changes of cortical‐basal ganglionic degeneration result in a variety of clinical presentations depending on the topography of the lesions. On this basis we conclude that cortical‐ basal ganglionic degeneration should be considered in the differential diagnosis of language disturbances and dementia, particularly when the latter is accompanied by frontal lobe symptomatology, early speech alterations, or parkinsonism.</abstract><relatedItem type="host"><titleInfo><title>Annals of Neurology</title><subTitle>Official Journal of the American Neurological Association and the Child Neurology Society</subTitle></titleInfo><titleInfo type="abbreviated"><title>Ann Neurol.</title></titleInfo><genre type="journal">journal</genre><subject><genre>article-category</genre><topic>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>1996</date><detail type="volume"><caption>vol.</caption><number>40</number></detail><detail type="issue"><caption>no.</caption><number>6</number></detail><extent unit="pages"><start>893</start><end>900</end><total>8</total></extent></part></relatedItem><identifier type="istex">4FC5057538C8A8E6F4777DAA9CCF08FB385F2111</identifier><identifier type="DOI">10.1002/ana.410400611</identifier><identifier type="ArticleID">ANA410400611</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 1996 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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