Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach
Identifieur interne : 001457 ( Main/Corpus ); précédent : 001456; suivant : 001458Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach
Auteurs : Simone Spuler ; Henriette Krug ; Christine Klein ; Isabel Chaure Medialdea ; Wibke Jakob ; Georg Ebersbach ; Doreen Gruber ; Karl-Titus Hoffmann ; Thomas Trottenberg ; Andreas KupschSource :
- Movement Disorders [ 0885-3185 ] ; 2010-04-15.
English descriptors
Abstract
Extreme forward flexion of the spine, named camptocormia (CC), and head drop syndrome (HD) may be among the most disabling symptoms in Parkinson's disease (PD). This study aims to eludicate the etiology of PD‐associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13–14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the Parkin gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. Aberrant protein aggregation may link PD and CC. © 2009 Movement Disorder Society
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DOI: 10.1002/mds.22913
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ISTEX:9D421529BE0FE823F02CFE521929343BC7759FC6Le document en format XML
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This study aims to eludicate the etiology of PD‐associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13–14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the Parkin gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. Aberrant protein aggregation may link PD and CC. © 2009 Movement Disorder Society</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Simone Spuler MD</name><affiliations><json:string>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</json:string><json:string>Muscle Research Unit, Experimental and Clinical Research Center, Charité University Medicine Berlin and the Max‐Delbrück Centre of Molecular Medicine, Berlin, Germany</json:string><json:string>Simone Spuler, Muscle Research Unit, ECRC at the Charité and the Max‐Delbrück Center, Lindenberger Weg 80, Berlin 13125, GermanyAndreas Kupsch, Department of Neurology, Charité‐Campus virchow, 13353 Berlin, Germany</json:string></affiliations></json:item><json:item><name>Henriette Krug MD</name><affiliations><json:string>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</json:string></affiliations></json:item><json:item><name>Christine Klein MD</name><affiliations><json:string>Department of Neurology, University of Lübeck, Lübeck, Germany</json:string></affiliations></json:item><json:item><name>Isabel Chaure Medialdea</name><affiliations><json:string>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</json:string></affiliations></json:item><json:item><name>Wibke Jakob MD</name><affiliations><json:string>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</json:string></affiliations></json:item><json:item><name>Georg Ebersbach MD</name><affiliations><json:string>Movement Disorders Clinic, Beelitz Heilstätten, Germany</json:string></affiliations></json:item><json:item><name>Doreen Gruber MD</name><affiliations><json:string>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</json:string></affiliations></json:item><json:item><name>Karl‐Titus Hoffmann MD</name><affiliations><json:string>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</json:string></affiliations></json:item><json:item><name>Thomas Trottenberg MD</name><affiliations><json:string>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</json:string></affiliations></json:item><json:item><name>Andreas Kupsch MD</name><affiliations><json:string>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</json:string><json:string>Simone Spuler, Muscle Research Unit, ECRC at the Charité and the Max‐Delbrück Center, Lindenberger Weg 80, Berlin 13125, GermanyAndreas Kupsch, Department of Neurology, Charité‐Campus virchow, 13353 Berlin, Germany</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>camptocormia</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>head drop syndrome</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Parkinson's disease</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>myopathy</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>protein‐surplus myopathy</value></json:item></subject><articleId><json:string>MDS22913</json:string></articleId><language><json:string>eng</json:string></language><abstract>Extreme forward flexion of the spine, named camptocormia (CC), and head drop syndrome (HD) may be among the most disabling symptoms in Parkinson's disease (PD). This study aims to eludicate the etiology of PD‐associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13–14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the Parkin gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. 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No. Sp 1152/8–1;</note><note>Volkswagen Foundation</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach</title><author><persName><forename type="first">Simone</forename><surname>Spuler</surname></persName><roleName type="degree">MD</roleName><note type="biography">Simone Spuler and Henriette Krug contributed equally.</note><affiliation>Simone Spuler and Henriette Krug contributed equally.</affiliation><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation><affiliation>Muscle Research Unit, Experimental and Clinical Research Center, Charité University Medicine Berlin and the Max‐Delbrück Centre of Molecular Medicine, Berlin, Germany</affiliation><affiliation>Simone Spuler, Muscle Research Unit, ECRC at the Charité and the Max‐Delbrück Center, Lindenberger Weg 80, Berlin 13125, GermanyAndreas Kupsch, Department of Neurology, Charité‐Campus virchow, 13353 Berlin, Germany</affiliation></author><author><persName><forename type="first">Henriette</forename><surname>Krug</surname></persName><roleName type="degree">MD</roleName><note type="biography">Simone Spuler and Henriette Krug contributed equally.</note><affiliation>Simone Spuler and Henriette Krug contributed equally.</affiliation><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation></author><author><persName><forename type="first">Christine</forename><surname>Klein</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurology, University of Lübeck, Lübeck, Germany</affiliation></author><author><persName><forename type="first">Isabel Chaure</forename><surname>Medialdea</surname></persName><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation></author><author><persName><forename type="first">Wibke</forename><surname>Jakob</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation></author><author><persName><forename type="first">Georg</forename><surname>Ebersbach</surname></persName><roleName type="degree">MD</roleName><affiliation>Movement Disorders Clinic, Beelitz Heilstätten, Germany</affiliation></author><author><persName><forename type="first">Doreen</forename><surname>Gruber</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation></author><author><persName><forename type="first">Karl‐Titus</forename><surname>Hoffmann</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation></author><author><persName><forename type="first">Thomas</forename><surname>Trottenberg</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation></author><author><persName><forename type="first">Andreas</forename><surname>Kupsch</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation><affiliation>Simone Spuler, Muscle Research Unit, ECRC at the Charité and the Max‐Delbrück Center, Lindenberger Weg 80, Berlin 13125, GermanyAndreas Kupsch, Department of Neurology, Charité‐Campus virchow, 13353 Berlin, Germany</affiliation></author></analytic><monogr><title level="j">Movement Disorders</title><title level="j" type="abbrev">Mov. Disord.</title><idno type="pISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><idno type="DOI">10.1002/(ISSN)1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2010-04-15"></date><biblScope unit="volume">25</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="552">552</biblScope><biblScope unit="page" to="559">559</biblScope></imprint></monogr><idno type="istex">9D421529BE0FE823F02CFE521929343BC7759FC6</idno><idno type="DOI">10.1002/mds.22913</idno><idno type="ArticleID">MDS22913</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>2010</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Extreme forward flexion of the spine, named camptocormia (CC), and head drop syndrome (HD) may be among the most disabling symptoms in Parkinson's disease (PD). This study aims to eludicate the etiology of PD‐associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13–14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the Parkin gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. Aberrant protein aggregation may link PD and CC. © 2009 Movement Disorder Society</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>camptocormia</term></item><item><term>head drop syndrome</term></item><item><term>Parkinson's disease</term></item><item><term>myopathy</term></item><item><term>protein‐surplus myopathy</term></item></list></keywords></textClass><textClass><keywords scheme="Journal Subject"><list><head>article category</head><item><term>Research Article</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="2009-03-07">Received</change><change when="2009-10-30">Registration</change><change when="2010-04-15">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/9D421529BE0FE823F02CFE521929343BC7759FC6/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="50"><doi origin="wiley" registered="yes">10.1002/mds.v25:5</doi><numberingGroup><numbering type="journalVolume" number="25">25</numbering><numbering type="journalIssue">5</numbering></numberingGroup><coverDate startDate="2010-04-15">15 April 2010</coverDate></publicationMeta><publicationMeta level="unit" type="article" position="60" status="forIssue"><doi origin="wiley" registered="yes">10.1002/mds.22913</doi><idGroup><id type="unit" value="MDS22913"></id></idGroup><countGroup><count type="pageTotal" number="8"></count></countGroup><titleGroup><title type="articleCategory">Research Article</title><title type="tocHeading1">Research Articles</title></titleGroup><copyright ownership="thirdParty">Copyright © 2009 Movement Disorder Society</copyright><eventGroup><event type="manuscriptReceived" date="2009-03-07"></event><event type="manuscriptRevised" date="2009-08-22"></event><event type="manuscriptAccepted" date="2009-10-30"></event><event type="firstOnline" date="2009-12-11"></event><event type="publishedOnlineFinalForm" date="2010-04-21"></event><event type="publishedOnlineAcceptedOrEarlyUnpaginated" date="2009-12-11"></event><event type="xmlConverted" agent="Converter:JWSART34_TO_WML3G version:2.5.2 mode:FullText" date="2011-08-12"></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">552</numbering><numbering type="pageLast">559</numbering></numberingGroup><correspondenceTo><lineatedText><line>Simone Spuler, Muscle Research Unit, ECRC at the Charité and the Max‐Delbrück Center, Lindenberger Weg 80, Berlin 13125, Germany</line><line>Andreas Kupsch, Department of Neurology, Charité‐Campus virchow, 13353 Berlin, Germany</line></lineatedText></correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:MDS.MDS22913.pdf"></link></linkGroup></publicationMeta><contentMeta><countGroup><count type="figureTotal" number="3"></count><count type="tableTotal" number="2"></count><count type="referenceTotal" number="38"></count><count type="wordTotal" number="5566"></count></countGroup><titleGroup><title type="main" xml:lang="en">Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach<link href="#fn1"></link></title><title type="short" xml:lang="en">Camptocormia in PD</title></titleGroup><creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1 #af2" corresponding="yes" noteRef="#fn3"><personName><givenNames>Simone</givenNames><familyName>Spuler</familyName><degrees>MD</degrees></personName><contactDetails><email>simone.spuler@charite.de</email></contactDetails></creator><creator xml:id="au2" creatorRole="author" affiliationRef="#af1" noteRef="#fn3"><personName><givenNames>Henriette</givenNames><familyName>Krug</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au3" creatorRole="author" affiliationRef="#af3"><personName><givenNames>Christine</givenNames><familyName>Klein</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au4" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Isabel Chaure</givenNames><familyName>Medialdea</familyName></personName></creator><creator xml:id="au5" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Wibke</givenNames><familyName>Jakob</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au6" creatorRole="author" affiliationRef="#af4"><personName><givenNames>Georg</givenNames><familyName>Ebersbach</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au7" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Doreen</givenNames><familyName>Gruber</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au8" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Karl‐Titus</givenNames><familyName>Hoffmann</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au9" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Thomas</givenNames><familyName>Trottenberg</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au10" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><givenNames>Andreas</givenNames><familyName>Kupsch</familyName><degrees>MD</degrees></personName><contactDetails><email>andreas.kupsch@charite.de</email></contactDetails></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="DE" type="organization"><unparsedAffiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="DE" type="organization"><unparsedAffiliation>Muscle Research Unit, Experimental and Clinical Research Center, Charité University Medicine Berlin and the Max‐Delbrück Centre of Molecular Medicine, Berlin, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="DE" type="organization"><unparsedAffiliation>Department of Neurology, University of Lübeck, Lübeck, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af4" countryCode="DE" type="organization"><unparsedAffiliation>Movement Disorders Clinic, Beelitz Heilstätten, Germany</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">camptocormia</keyword><keyword xml:id="kwd2">head drop syndrome</keyword><keyword xml:id="kwd3">Parkinson's disease</keyword><keyword xml:id="kwd4">myopathy</keyword><keyword xml:id="kwd5">protein‐surplus myopathy</keyword></keywordGroup><fundingInfo><fundingAgency>German Research Foundation</fundingAgency><fundingNumber>KFO192</fundingNumber><fundingNumber>Sp 1152/8–1</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Volkswagen Foundation</fundingAgency></fundingInfo><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>Extreme forward flexion of the spine, named camptocormia (CC), and head drop syndrome (HD) may be among the most disabling symptoms in Parkinson's disease (PD). This study aims to eludicate the etiology of PD‐associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13–14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the <i>Parkin</i> gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. Aberrant protein aggregation may link PD and CC. © 2009 Movement Disorder Society</p></abstract></abstractGroup></contentMeta><noteGroup><note xml:id="fn1"><p>Potential conflict of interest: Nothing to report.</p></note><note xml:id="fn3"><p>Simone Spuler and Henriette Krug contributed equally.</p></note></noteGroup></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Camptocormia in PD</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Myopathy causing camptocormia in idiopathic Parkinson's disease: A multidisciplinary approach</title></titleInfo><name type="personal"><namePart type="given">Simone</namePart><namePart type="family">Spuler</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation><affiliation>Muscle Research Unit, Experimental and Clinical Research Center, Charité University Medicine Berlin and the Max‐Delbrück Centre of Molecular Medicine, Berlin, Germany</affiliation><description>Simone Spuler and Henriette Krug contributed equally.</description><affiliation>Simone Spuler, Muscle Research Unit, ECRC at the Charité and the Max‐Delbrück Center, Lindenberger Weg 80, Berlin 13125, GermanyAndreas Kupsch, Department of Neurology, Charité‐Campus virchow, 13353 Berlin, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Henriette</namePart><namePart type="family">Krug</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation><description>Simone Spuler and Henriette Krug contributed equally.</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Christine</namePart><namePart type="family">Klein</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, University of Lübeck, Lübeck, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Isabel Chaure</namePart><namePart type="family">Medialdea</namePart><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Wibke</namePart><namePart type="family">Jakob</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Georg</namePart><namePart type="family">Ebersbach</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Movement Disorders Clinic, Beelitz Heilstätten, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Doreen</namePart><namePart type="family">Gruber</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Karl‐Titus</namePart><namePart type="family">Hoffmann</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Thomas</namePart><namePart type="family">Trottenberg</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Andreas</namePart><namePart type="family">Kupsch</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Neurosurgery and Neuroradiology, Charité University Medicine Berlin, Berlin, Germany</affiliation><affiliation>Simone Spuler, Muscle Research Unit, ECRC at the Charité and the Max‐Delbrück Center, Lindenberger Weg 80, Berlin 13125, GermanyAndreas Kupsch, Department of Neurology, Charité‐Campus virchow, 13353 Berlin, Germany</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">2010-04-15</dateIssued><dateCaptured encoding="w3cdtf">2009-03-07</dateCaptured><dateValid encoding="w3cdtf">2009-10-30</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">3</extent><extent unit="tables">2</extent><extent unit="references">38</extent><extent unit="words">5566</extent></physicalDescription><abstract lang="en">Extreme forward flexion of the spine, named camptocormia (CC), and head drop syndrome (HD) may be among the most disabling symptoms in Parkinson's disease (PD). This study aims to eludicate the etiology of PD‐associated CC and HD via a multidisciplinary approach (clinical examination, electromyography, MRI, genetic analysis, muscle morphology) centering on the histology of the paraspinal muscles. We studied 17 patients with the clinical diagnosis of PD and CC or head drop syndrome and six controls. We performed muscle biopsies of paraspinal muscles and deep neck extensor muscles. Mean age at onset of postural abnormality was 66 years and mean latency between onset of parkinsonian symptoms to first signs of CC or head drop was 7 years. The electromyogram of paraspinal muscles was abnormal in 13–14 patients. Histopathology revealed chronic myopathic changes in 14 of 17 biopsies, consisting of abnormal variation in fiber size, increase in internal nuclei, and increase in connective tissue, myofibrillar disarray and similarities to protein surplus myopathies. Interestingly, heterozygous variants in the Parkin gene were found in 2 of 9 investigated patients. We conclude that CC and HD in PD are predominantly myopathic. Aberrant protein aggregation may link PD and CC. © 2009 Movement Disorder Society</abstract><note type="content">*Potential conflict of interest: Nothing to report.</note><note type="funding">German Research Foundation - No. KFO192; No. Sp 1152/8–1; </note><note type="funding">Volkswagen Foundation</note><subject lang="en"><genre>Keywords</genre><topic>camptocormia</topic><topic>head drop syndrome</topic><topic>Parkinson's disease</topic><topic>myopathy</topic><topic>protein‐surplus myopathy</topic></subject><relatedItem type="host"><titleInfo><title>Movement Disorders</title></titleInfo><titleInfo type="abbreviated"><title>Mov. 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