Pallidopyramidal disease: A misnomer?
Identifieur interne : 001437 ( Istex/Corpus ); précédent : 001436; suivant : 001438Pallidopyramidal disease: A misnomer?
Auteurs : Martin W. I. M. Horstink ; Marieke C. Dekker ; Pasquale Montagna ; Vincenzo Bonifati ; Bart P. Van De WarrenburgSource :
- Movement Disorders [ 0885-3185 ] ; 2010-07-15.
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Abstract
The combination of recessive early‐onset parkinsonism and pyramidal tract signs caused by pallidopyramidal degeneration is known as pallidopyramidal disease or syndrome (PPD/S). We investigated whether patients diagnosed as Davison's PPD/S showed any definite proof of pyramidal and pallidal involvement, without findings suggestive of other nosological entities. Since Davison's original description, 15 other PPD/S cases have been reported, yet all lack proof of pyramidal or pallidal degeneration. Because of the dopa‐responsiveness in all patients subsequent to Davison's report, we argue that these patients probably suffered from early‐onset nigral parkinsonism or dopa‐responsive dsystonia, rather than pallidal parkinsonism; in such cases, the presumed pyramidal Babinski could be a pseudobabinski (“striatal toe”). Secondary pallidopyramidal syndromes do occur, for example, in multiple system atrophy or Wilson's disease, but in these patients additional findings indicate diseases other than Davison's PPD/S. We conclude that the existence of PPD/S as a distinct clinico‐pathological nosological entity, as proposed by Davison, is doubtful. In cases reported as Davison's PPD/S, the description “pallidopyramidal” seems to be a misnomer. © 2010 Movement Disorder Society
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DOI: 10.1002/mds.23118
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Horstink</name><affiliation><mods:affiliation>Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands</mods:affiliation></affiliation></author><author><name sortKey="Dekker, Marieke C" sort="Dekker, Marieke C" uniqKey="Dekker M" first="Marieke C." last="Dekker">Marieke C. Dekker</name><affiliation><mods:affiliation>Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands</mods:affiliation></affiliation></author><author><name sortKey="Montagna, Pasquale" sort="Montagna, Pasquale" uniqKey="Montagna P" first="Pasquale" last="Montagna">Pasquale Montagna</name><affiliation><mods:affiliation>Department of Neurological Sciences, University of Bologna, Bologna, Italy</mods:affiliation></affiliation></author><author><name sortKey="Bonifati, Vincenzo" sort="Bonifati, Vincenzo" uniqKey="Bonifati V" first="Vincenzo" last="Bonifati">Vincenzo Bonifati</name><affiliation><mods:affiliation>Department of Clinical Genetics, Erasmus Medical Centre, Rotterdam, The Netherlands</mods:affiliation></affiliation></author><author><name sortKey="Van De Warrenburg, Bart P" sort="Van De Warrenburg, Bart P" uniqKey="Van De Warrenburg B" first="Bart P." last="Van De Warrenburg">Bart P. 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We investigated whether patients diagnosed as Davison's PPD/S showed any definite proof of pyramidal and pallidal involvement, without findings suggestive of other nosological entities. Since Davison's original description, 15 other PPD/S cases have been reported, yet all lack proof of pyramidal or pallidal degeneration. Because of the dopa‐responsiveness in all patients subsequent to Davison's report, we argue that these patients probably suffered from early‐onset nigral parkinsonism or dopa‐responsive dsystonia, rather than pallidal parkinsonism; in such cases, the presumed pyramidal Babinski could be a pseudobabinski (“striatal toe”). Secondary pallidopyramidal syndromes do occur, for example, in multiple system atrophy or Wilson's disease, but in these patients additional findings indicate diseases other than Davison's PPD/S. We conclude that the existence of PPD/S as a distinct clinico‐pathological nosological entity, as proposed by Davison, is doubtful. In cases reported as Davison's PPD/S, the description “pallidopyramidal” seems to be a misnomer. © 2010 Movement Disorder Society</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Martin W.I.M. Horstink MD, PhD</name><affiliations><json:string>Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands</json:string></affiliations></json:item><json:item><name>Marieke C. Dekker MD, PhD</name><affiliations><json:string>Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands</json:string></affiliations></json:item><json:item><name>Pasquale Montagna MD</name><affiliations><json:string>Department of Neurological Sciences, University of Bologna, Bologna, Italy</json:string></affiliations></json:item><json:item><name>Vincenzo Bonifati MD, PhD</name><affiliations><json:string>Department of Clinical Genetics, Erasmus Medical Centre, Rotterdam, The Netherlands</json:string></affiliations></json:item><json:item><name>Bart P. van De Warrenburg MD, PhD</name><affiliations><json:string>Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>pallidopyramidal</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>pseudobabinski</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>pyramidal syndrome</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>pallidal atrophy</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>juvenile parkinsonism</value></json:item></subject><language><json:string>eng</json:string></language><abstract>The combination of recessive early‐onset parkinsonism and pyramidal tract signs caused by pallidopyramidal degeneration is known as pallidopyramidal disease or syndrome (PPD/S). We investigated whether patients diagnosed as Davison's PPD/S showed any definite proof of pyramidal and pallidal involvement, without findings suggestive of other nosological entities. Since Davison's original description, 15 other PPD/S cases have been reported, yet all lack proof of pyramidal or pallidal degeneration. Because of the dopa‐responsiveness in all patients subsequent to Davison's report, we argue that these patients probably suffered from early‐onset nigral parkinsonism or dopa‐responsive dsystonia, rather than pallidal parkinsonism; in such cases, the presumed pyramidal Babinski could be a pseudobabinski (“striatal toe”). Secondary pallidopyramidal syndromes do occur, for example, in multiple system atrophy or Wilson's disease, but in these patients additional findings indicate diseases other than Davison's PPD/S. We conclude that the existence of PPD/S as a distinct clinico‐pathological nosological entity, as proposed by Davison, is doubtful. 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We investigated whether patients diagnosed as Davison's PPD/S showed any definite proof of pyramidal and pallidal involvement, without findings suggestive of other nosological entities. Since Davison's original description, 15 other PPD/S cases have been reported, yet all lack proof of pyramidal or pallidal degeneration. Because of the dopa‐responsiveness in all patients subsequent to Davison's report, we argue that these patients probably suffered from early‐onset nigral parkinsonism or dopa‐responsive dsystonia, rather than pallidal parkinsonism; in such cases, the presumed pyramidal Babinski could be a pseudobabinski (“striatal toe”). Secondary pallidopyramidal syndromes do occur, for example, in multiple system atrophy or Wilson's disease, but in these patients additional findings indicate diseases other than Davison's PPD/S. We conclude that the existence of PPD/S as a distinct clinico‐pathological nosological entity, as proposed by Davison, is doubtful. In cases reported as Davison's PPD/S, the description “pallidopyramidal” seems to be a misnomer. © 2010 Movement Disorder Society</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>pallidopyramidal</term></item><item><term>pseudobabinski</term></item><item><term>pyramidal syndrome</term></item><item><term>pallidal atrophy</term></item><item><term>juvenile parkinsonism</term></item></list></keywords></textClass><textClass><keywords scheme="Journal Subject"><list><head>Article category</head><item><term>Historical Review</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="2008-11-28">Received</change><change when="2010-03-02">Registration</change><change when="2010-07-15">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/A692173D6C2D061F18622D652E087FDF11FCEE30/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)1531-8257</doi><issn type="print">0885-3185</issn><issn type="electronic">1531-8257</issn><idGroup><id type="product" value="MDS"></id></idGroup><titleGroup><title type="main" xml:lang="en" sort="MOVEMENT DISORDERS">Movement Disorders</title><title type="short">Mov. Disord.</title></titleGroup></publicationMeta><publicationMeta level="part" position="90"><doi origin="wiley" registered="yes">10.1002/mds.v25:9</doi><numberingGroup><numbering type="journalVolume" number="25">25</numbering><numbering type="journalIssue">9</numbering></numberingGroup><coverDate startDate="2010-07-15">15 July 2010</coverDate></publicationMeta><publicationMeta level="unit" type="reviewArticle" position="10" status="forIssue"><doi origin="wiley" registered="yes">10.1002/mds.23118</doi><idGroup><id type="unit" value="MDS23118"></id></idGroup><countGroup><count type="pageTotal" number="7"></count></countGroup><titleGroup><title type="articleCategory">Historical Review</title><title type="tocHeading1">Historical Reviews</title></titleGroup><copyright ownership="thirdParty">Copyright © 2010 Movement Disorder Society</copyright><eventGroup><event type="manuscriptReceived" date="2008-11-28"></event><event type="manuscriptRevised" date="2009-06-10"></event><event type="manuscriptAccepted" date="2010-03-02"></event><event type="firstOnline" date="2010-05-14"></event><event type="publishedOnlineFinalForm" date="2010-07-19"></event><event type="publishedOnlineAcceptedOrEarlyUnpaginated" date="2010-05-14"></event><event type="xmlConverted" agent="Converter:JWSART34_TO_WML3G version:2.4.8 mode:FullText" date="2011-05-03"></event><event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:3.8.8" date="2014-02-02"></event><event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.1.7 mode:FullText,remove_FC" date="2014-10-31"></event></eventGroup><numberingGroup><numbering type="pageFirst">1109</numbering><numbering type="pageLast">1115</numbering></numberingGroup><correspondenceTo>Department of Neurology Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, PO Box 9101, Nijmegen 6500 HB, The Netherlands</correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:MDS.MDS23118.pdf"></link></linkGroup></publicationMeta><contentMeta><countGroup><count type="figureTotal" number="0"></count><count type="tableTotal" number="1"></count><count type="referenceTotal" number="68"></count><count type="wordTotal" number="5459"></count></countGroup><titleGroup><title type="main" xml:lang="en">Pallidopyramidal disease: A misnomer?<link href="#fn1"></link></title><title type="short" xml:lang="en">Pallidopyramidal Disease: A Misnomer?</title></titleGroup><creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1" noteRef="#fn2"><personName><givenNames>Martin W.I.M.</givenNames><familyName>Horstink</familyName><degrees>MD, PhD</degrees></personName></creator><creator xml:id="au2" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Marieke C.</givenNames><familyName>Dekker</familyName><degrees>MD, PhD</degrees></personName></creator><creator xml:id="au3" creatorRole="author" affiliationRef="#af2"><personName><givenNames>Pasquale</givenNames><familyName>Montagna</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au4" creatorRole="author" affiliationRef="#af3"><personName><givenNames>Vincenzo</givenNames><familyName>Bonifati</familyName><degrees>MD, PhD</degrees></personName></creator><creator xml:id="au5" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><givenNames>Bart P.</givenNames><familyName>van De Warrenburg</familyName><degrees>MD, PhD</degrees></personName><contactDetails><email>b.vandewarrenburg@neuro.umcn.nl</email></contactDetails></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="NL" type="organization"><unparsedAffiliation>Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="IT" type="organization"><unparsedAffiliation>Department of Neurological Sciences, University of Bologna, Bologna, Italy</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="NL" type="organization"><unparsedAffiliation>Department of Clinical Genetics, Erasmus Medical Centre, Rotterdam, The Netherlands</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">pallidopyramidal</keyword><keyword xml:id="kwd2">pseudobabinski</keyword><keyword xml:id="kwd3">pyramidal syndrome</keyword><keyword xml:id="kwd4">pallidal atrophy</keyword><keyword xml:id="kwd5">juvenile parkinsonism</keyword></keywordGroup><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>The combination of recessive early‐onset parkinsonism and pyramidal tract signs caused by pallidopyramidal degeneration is known as pallidopyramidal disease or syndrome (PPD/S). We investigated whether patients diagnosed as Davison's PPD/S showed any definite proof of pyramidal and pallidal involvement, without findings suggestive of other nosological entities. Since Davison's original description, 15 other PPD/S cases have been reported, yet all lack proof of pyramidal or pallidal degeneration. Because of the dopa‐responsiveness in all patients subsequent to Davison's report, we argue that these patients probably suffered from early‐onset nigral parkinsonism or dopa‐responsive dsystonia, rather than pallidal parkinsonism; in such cases, the presumed pyramidal Babinski could be a pseudobabinski (“striatal toe”). Secondary pallidopyramidal syndromes do occur, for example, in multiple system atrophy or Wilson's disease, but in these patients additional findings indicate diseases other than Davison's PPD/S. We conclude that the existence of PPD/S as a distinct clinico‐pathological nosological entity, as proposed by Davison, is doubtful. In cases reported as Davison's PPD/S, the description “pallidopyramidal” seems to be a misnomer. © 2010 Movement Disorder Society</p></abstract></abstractGroup></contentMeta><noteGroup><note xml:id="fn1"><p>Potential conflict of interest: None reported.</p></note><note xml:id="fn2"><p>Deceased</p></note></noteGroup></header></component></istex:document></istex:metadataXml><!--Version 0.6 générée le 4-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Pallidopyramidal disease: A misnomer?</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Pallidopyramidal Disease: A Misnomer?</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Pallidopyramidal disease: A misnomer?</title></titleInfo><name type="personal"><namePart type="given">Martin W.I.M.</namePart><namePart type="family">Horstink</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands</affiliation><description>Deceased</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Marieke C.</namePart><namePart type="family">Dekker</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Pasquale</namePart><namePart type="family">Montagna</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurological Sciences, University of Bologna, Bologna, Italy</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Vincenzo</namePart><namePart type="family">Bonifati</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Department of Clinical Genetics, Erasmus Medical Centre, Rotterdam, The Netherlands</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Bart P.</namePart><namePart type="family">van De Warrenburg</namePart><namePart type="termsOfAddress">MD, PhD</namePart><affiliation>Department of Neurology, Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands</affiliation><description>Correspondence: Department of Neurology Donders Centre for Brain, Cognition, and Behaviour, Radboud University Nijmegen Medical Centre, PO Box 9101, Nijmegen 6500 HB, The Netherlands</description><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre authority="originalCategForm">reviewArticle</genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">2010-07-15</dateIssued><dateCaptured encoding="w3cdtf">2008-11-28</dateCaptured><dateValid encoding="w3cdtf">2010-03-02</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="tables">1</extent><extent unit="references">68</extent><extent unit="words">5459</extent></physicalDescription><abstract lang="en">The combination of recessive early‐onset parkinsonism and pyramidal tract signs caused by pallidopyramidal degeneration is known as pallidopyramidal disease or syndrome (PPD/S). We investigated whether patients diagnosed as Davison's PPD/S showed any definite proof of pyramidal and pallidal involvement, without findings suggestive of other nosological entities. Since Davison's original description, 15 other PPD/S cases have been reported, yet all lack proof of pyramidal or pallidal degeneration. Because of the dopa‐responsiveness in all patients subsequent to Davison's report, we argue that these patients probably suffered from early‐onset nigral parkinsonism or dopa‐responsive dsystonia, rather than pallidal parkinsonism; in such cases, the presumed pyramidal Babinski could be a pseudobabinski (“striatal toe”). Secondary pallidopyramidal syndromes do occur, for example, in multiple system atrophy or Wilson's disease, but in these patients additional findings indicate diseases other than Davison's PPD/S. We conclude that the existence of PPD/S as a distinct clinico‐pathological nosological entity, as proposed by Davison, is doubtful. In cases reported as Davison's PPD/S, the description “pallidopyramidal” seems to be a misnomer. © 2010 Movement Disorder Society</abstract><note type="content">*Potential conflict of interest: None reported.</note><subject lang="en"><genre>Keywords</genre><topic>pallidopyramidal</topic><topic>pseudobabinski</topic><topic>pyramidal syndrome</topic><topic>pallidal atrophy</topic><topic>juvenile parkinsonism</topic></subject><relatedItem type="host"><titleInfo><title>Movement Disorders</title></titleInfo><titleInfo type="abbreviated"><title>Mov. 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