Pathophysiology of chorea and bradykinesia in Huntington's disease
Identifieur interne : 000743 ( Istex/Corpus ); précédent : 000742; suivant : 000744Pathophysiology of chorea and bradykinesia in Huntington's disease
Auteurs : Alfredo Berardelli ; Johannes Noth ; Philip D. Thompson ; Eduard L. E. M. Bollen ; Antonio Currà ; Gunther Deuschl ; J. Gert Van Dijk ; Rudolf Töpper ; Michael Schwarz ; Raymund A. C. RoosSource :
- Movement Disorders [ 0885-3185 ] ; 1999-05.
English descriptors
- KwdEn :
Abstract
This article reviews the neurophysiological abnormalities described in Huntington's disease. Among the typical features of choreic movements are variable and random patterns of electromyographic (EMG) activity, including cocontraction of agonist and antagonist muscles. Studies of premotor potentials show that choreic movements are not preceded by a Bereitschaftspotential, therefore demonstrating that choreic movement is involuntary. Early cortical median‐nerve somatosensory‐evoked potentials have reduced amplitudes and the reduction correlates with reduced glucose consumption in the caudate nucleus. Long‐latency stretch reflexes evoked in the small hand muscles are depressed. These findings may reflect failed thalamocortical relay of sensory information. In Huntington's disease, the R2 response of the blink reflex has prolonged latencies, diminished amplitudes, and greater habituation than normal. These abnormalities correlate with the severity of chorea in the face. Patients with Huntington's disease perform simple voluntary movements more slowly than normal subjects and with an abnormal triphasic EMG pattern. Bradykinesia is also present during their performance of simultaneous and sequential movements. Eye movements show abnormalities similar to those seen in arm movements. In Huntington's disease, arm movement execution is associated with reduced PET activation of cortical frontal areas. Studies using transcranial magnetic stimulation show that patients with Huntington's disease have normal corticospinal conduction but some patients have a prolonged cortical silent period. Bradykinesia results from degeneration of the basal ganglia output to the supplementary motor areas concerned with the initiation and maintenance of sequential movements. The coexisting hyperkinetic and hypokinetic movement disorders in patients with Huntington's disease probably reflect the involvement of direct and indirect pathways in the basal ganglia–thalamus–cortical motor circuit.
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DOI: 10.1002/1531-8257(199905)14:3<398::AID-MDS1003>3.0.CO;2-F
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Bollen</name><affiliation><mods:affiliation>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</mods:affiliation></affiliation></author><author><name sortKey="Curra, Antonio" sort="Curra, Antonio" uniqKey="Curra A" first="Antonio" last="Currà">Antonio Currà</name><affiliation><mods:affiliation>Dipartimento di Scienze Neurologiche Università di Roma “La Sapienza,” v.le Università, Rome, Italy, and Istituto Neurologico Mediterraneo “Neuromed,” Pozzilli, Italy</mods:affiliation></affiliation></author><author><name sortKey="Deuschl, Gunther" sort="Deuschl, Gunther" uniqKey="Deuschl G" first="Gunther" last="Deuschl">Gunther Deuschl</name><affiliation><mods:affiliation>Klinik fúr Neurologie, Christian‐Albrechts‐Universität zu Kiel, Kiel, Germany</mods:affiliation></affiliation></author><author><name sortKey="Gert Van Dijk, J" sort="Gert Van Dijk, J" uniqKey="Gert Van Dijk J" first="J." last="Gert Van Dijk">J. Gert Van Dijk</name><affiliation><mods:affiliation>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</mods:affiliation></affiliation></author><author><name sortKey="Topper, Rudolf" sort="Topper, Rudolf" uniqKey="Topper R" first="Rudolf" last="Töpper">Rudolf Töpper</name><affiliation><mods:affiliation>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</mods:affiliation></affiliation></author><author><name sortKey="Schwarz, Michael" sort="Schwarz, Michael" uniqKey="Schwarz M" first="Michael" last="Schwarz">Michael Schwarz</name><affiliation><mods:affiliation>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</mods:affiliation></affiliation></author><author><name sortKey="Roos, Raymund A C" sort="Roos, Raymund A C" uniqKey="Roos R" first="Raymund A. C." last="Roos">Raymund A. C. Roos</name><affiliation><mods:affiliation>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="sub">Official Journal of the Movement Disorder Society</title><title level="j" type="abbrev">Mov. 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Among the typical features of choreic movements are variable and random patterns of electromyographic (EMG) activity, including cocontraction of agonist and antagonist muscles. Studies of premotor potentials show that choreic movements are not preceded by a Bereitschaftspotential, therefore demonstrating that choreic movement is involuntary. Early cortical median‐nerve somatosensory‐evoked potentials have reduced amplitudes and the reduction correlates with reduced glucose consumption in the caudate nucleus. Long‐latency stretch reflexes evoked in the small hand muscles are depressed. These findings may reflect failed thalamocortical relay of sensory information. In Huntington's disease, the R2 response of the blink reflex has prolonged latencies, diminished amplitudes, and greater habituation than normal. These abnormalities correlate with the severity of chorea in the face. Patients with Huntington's disease perform simple voluntary movements more slowly than normal subjects and with an abnormal triphasic EMG pattern. Bradykinesia is also present during their performance of simultaneous and sequential movements. Eye movements show abnormalities similar to those seen in arm movements. In Huntington's disease, arm movement execution is associated with reduced PET activation of cortical frontal areas. Studies using transcranial magnetic stimulation show that patients with Huntington's disease have normal corticospinal conduction but some patients have a prolonged cortical silent period. Bradykinesia results from degeneration of the basal ganglia output to the supplementary motor areas concerned with the initiation and maintenance of sequential movements. The coexisting hyperkinetic and hypokinetic movement disorders in patients with Huntington's disease probably reflect the involvement of direct and indirect pathways in the basal ganglia–thalamus–cortical motor circuit.</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Alfredo Berardelli MD</name><affiliations><json:string>Dipartimento di Scienze Neurologiche Università di Roma “La Sapienza,” v.le Università, Rome, Italy, and Istituto Neurologico Mediterraneo “Neuromed,” Pozzilli, Italy</json:string></affiliations></json:item><json:item><name>Johannes Noth MD</name><affiliations><json:string>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</json:string></affiliations></json:item><json:item><name>Philip D. Thompson MD</name><affiliations><json:string>Department of Medicine, The Royal Adelaide Hospital, Adelaide, South Australia</json:string></affiliations></json:item><json:item><name>Eduard L. E. M. Bollen MD</name><affiliations><json:string>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</json:string></affiliations></json:item><json:item><name>Antonio Currà MD</name><affiliations><json:string>Dipartimento di Scienze Neurologiche Università di Roma “La Sapienza,” v.le Università, Rome, Italy, and Istituto Neurologico Mediterraneo “Neuromed,” Pozzilli, Italy</json:string></affiliations></json:item><json:item><name>Gunther Deuschl MD</name><affiliations><json:string>Klinik fúr Neurologie, Christian‐Albrechts‐Universität zu Kiel, Kiel, Germany</json:string></affiliations></json:item><json:item><name>J. Gert van Dijk MD</name><affiliations><json:string>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</json:string></affiliations></json:item><json:item><name>Rudolf Töpper MD</name><affiliations><json:string>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</json:string></affiliations></json:item><json:item><name>Michael Schwarz MD</name><affiliations><json:string>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</json:string></affiliations></json:item><json:item><name>Raymund A. C. Roos MD</name><affiliations><json:string>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>Huntington's disease</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Neurophysiology</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Motor system</value></json:item></subject><language><json:string>eng</json:string></language><abstract>This article reviews the neurophysiological abnormalities described in Huntington's disease. Among the typical features of choreic movements are variable and random patterns of electromyographic (EMG) activity, including cocontraction of agonist and antagonist muscles. Studies of premotor potentials show that choreic movements are not preceded by a Bereitschaftspotential, therefore demonstrating that choreic movement is involuntary. Early cortical median‐nerve somatosensory‐evoked potentials have reduced amplitudes and the reduction correlates with reduced glucose consumption in the caudate nucleus. Long‐latency stretch reflexes evoked in the small hand muscles are depressed. These findings may reflect failed thalamocortical relay of sensory information. In Huntington's disease, the R2 response of the blink reflex has prolonged latencies, diminished amplitudes, and greater habituation than normal. These abnormalities correlate with the severity of chorea in the face. Patients with Huntington's disease perform simple voluntary movements more slowly than normal subjects and with an abnormal triphasic EMG pattern. Bradykinesia is also present during their performance of simultaneous and sequential movements. Eye movements show abnormalities similar to those seen in arm movements. In Huntington's disease, arm movement execution is associated with reduced PET activation of cortical frontal areas. Studies using transcranial magnetic stimulation show that patients with Huntington's disease have normal corticospinal conduction but some patients have a prolonged cortical silent period. Bradykinesia results from degeneration of the basal ganglia output to the supplementary motor areas concerned with the initiation and maintenance of sequential movements. The coexisting hyperkinetic and hypokinetic movement disorders in patients with Huntington's disease probably reflect the involvement of direct and indirect pathways in the basal ganglia–thalamus–cortical motor circuit.</abstract><qualityIndicators><score>7.311</score><pdfVersion>1.3</pdfVersion><pdfPageSize>612 x 792 pts (letter)</pdfPageSize><refBibsNative>true</refBibsNative><abstractCharCount>1994</abstractCharCount><pdfWordCount>4311</pdfWordCount><pdfCharCount>28920</pdfCharCount><pdfPageCount>6</pdfPageCount><abstractWordCount>259</abstractWordCount></qualityIndicators><title>Pathophysiology of chorea and bradykinesia in Huntington's disease</title><genre><json:string>Serial article</json:string></genre><host><volume>14</volume><pages><total>6</total><last>403</last><first>398</first></pages><issn><json:string>0885-3185</json:string></issn><issue>3</issue><subject><json:item><value>Review</value></json:item></subject><genre></genre><language><json:string>unknown</json:string></language><title>Movement Disorders</title><doi><json:string>10.1002/(ISSN)1531-8257</json:string></doi></host><publicationDate>1999</publicationDate><copyrightDate>1999</copyrightDate><doi><json:string>10.1002/1531-8257(199905)14:3>398::AID-MDS1003>3.0.CO;2-F</json:string></doi><id>B090E3887BDA361200F04109E6801C05EE8A85E5</id><fulltext><json:item><original>true</original><mimetype>application/pdf</mimetype><extension>pdf</extension><uri>https://api.istex.fr/document/B090E3887BDA361200F04109E6801C05EE8A85E5/fulltext/pdf</uri></json:item><json:item><original>false</original><mimetype>application/zip</mimetype><extension>zip</extension><uri>https://api.istex.fr/document/B090E3887BDA361200F04109E6801C05EE8A85E5/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/B090E3887BDA361200F04109E6801C05EE8A85E5/fulltext/tei"><teiHeader type="text"><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Pathophysiology of chorea and bradykinesia in Huntington's disease</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>John Wiley & Sons, Inc.</publisher><pubPlace>New York</pubPlace><availability><p>John Wiley & Sons, Inc.</p></availability><date>1999</date></publicationStmt><notesStmt><note>EC (Nest‐HD) and the association “Ricerca in Movement” - No. (BMH1‐CT93‐1621);</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Pathophysiology of chorea and bradykinesia in Huntington's disease</title><author><persName><forename type="first">Alfredo</forename><surname>Berardelli</surname><roleName type="degree">MD</roleName></persName><note type="correspondence"><p>Correspondence: Dipartimento Scienze Neurologiche, Università “La Sapienza,” Viale Università 30‐00185, Rome, Italy</p></note><affiliation>Dipartimento di Scienze Neurologiche Università di Roma “La Sapienza,” v.le Università, Rome, Italy, and Istituto Neurologico Mediterraneo “Neuromed,” Pozzilli, Italy</affiliation></author><author><persName><forename type="first">Johannes</forename><surname>Noth</surname><roleName type="degree">MD</roleName></persName><affiliation>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</affiliation></author><author><persName><forename type="first">Philip D.</forename><surname>Thompson</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Medicine, The Royal Adelaide Hospital, Adelaide, South Australia</affiliation></author><author><persName><forename type="first">Eduard L. 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M.</forename><surname>Bollen</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</affiliation></author><author><persName><forename type="first">Antonio</forename><surname>Currà</surname><roleName type="degree">MD</roleName></persName><affiliation>Dipartimento di Scienze Neurologiche Università di Roma “La Sapienza,” v.le Università, Rome, Italy, and Istituto Neurologico Mediterraneo “Neuromed,” Pozzilli, Italy</affiliation></author><author><persName><forename type="first">Gunther</forename><surname>Deuschl</surname><roleName type="degree">MD</roleName></persName><affiliation>Klinik fúr Neurologie, Christian‐Albrechts‐Universität zu Kiel, Kiel, Germany</affiliation></author><author><persName><forename type="first">J.</forename><surname>Gert van Dijk</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</affiliation></author><author><persName><forename type="first">Rudolf</forename><surname>Töpper</surname><roleName type="degree">MD</roleName></persName><affiliation>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</affiliation></author><author><persName><forename type="first">Michael</forename><surname>Schwarz</surname><roleName type="degree">MD</roleName></persName><affiliation>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</affiliation></author><author><persName><forename type="first">Raymund A. C.</forename><surname>Roos</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</affiliation></author></analytic><monogr><title level="j">Movement Disorders</title><title level="j" type="sub">Official Journal of the Movement Disorder Society</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>John Wiley & Sons, Inc.</publisher><pubPlace>New York</pubPlace><date type="published" when="1999-05"></date><biblScope unit="vol">14</biblScope><biblScope unit="issue">3</biblScope><biblScope unit="page" from="398">398</biblScope><biblScope unit="page" to="403">403</biblScope></imprint></monogr><idno type="istex">B090E3887BDA361200F04109E6801C05EE8A85E5</idno><idno type="DOI">10.1002/1531-8257(199905)14:3<398::AID-MDS1003>3.0.CO;2-F</idno><idno type="ArticleID">MDS1003</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>1999</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>This article reviews the neurophysiological abnormalities described in Huntington's disease. Among the typical features of choreic movements are variable and random patterns of electromyographic (EMG) activity, including cocontraction of agonist and antagonist muscles. Studies of premotor potentials show that choreic movements are not preceded by a Bereitschaftspotential, therefore demonstrating that choreic movement is involuntary. Early cortical median‐nerve somatosensory‐evoked potentials have reduced amplitudes and the reduction correlates with reduced glucose consumption in the caudate nucleus. Long‐latency stretch reflexes evoked in the small hand muscles are depressed. These findings may reflect failed thalamocortical relay of sensory information. In Huntington's disease, the R2 response of the blink reflex has prolonged latencies, diminished amplitudes, and greater habituation than normal. These abnormalities correlate with the severity of chorea in the face. Patients with Huntington's disease perform simple voluntary movements more slowly than normal subjects and with an abnormal triphasic EMG pattern. Bradykinesia is also present during their performance of simultaneous and sequential movements. Eye movements show abnormalities similar to those seen in arm movements. In Huntington's disease, arm movement execution is associated with reduced PET activation of cortical frontal areas. Studies using transcranial magnetic stimulation show that patients with Huntington's disease have normal corticospinal conduction but some patients have a prolonged cortical silent period. Bradykinesia results from degeneration of the basal ganglia output to the supplementary motor areas concerned with the initiation and maintenance of sequential movements. The coexisting hyperkinetic and hypokinetic movement disorders in patients with Huntington's disease probably reflect the involvement of direct and indirect pathways in the basal ganglia–thalamus–cortical motor circuit.</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>Huntington's disease</term></item><item><term>Neurophysiology</term></item><item><term>Motor system</term></item></list></keywords></textClass><textClass><keywords scheme="Journal Subject"><list><head>Article category</head><item><term>Review</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="1998-02-04">Received</change><change when="1998-09-30">Registration</change><change when="1999-05">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/B090E3887BDA361200F04109E6801C05EE8A85E5/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>John Wiley & Sons, Inc.</publisherName><publisherLoc>New York</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="subtitle">Official Journal of the Movement Disorder Society</title><title type="short">Mov. Disord.</title></titleGroup></publicationMeta><publicationMeta level="part" position="30"><doi origin="wiley" registered="yes">10.1002/1531-8257(199905)14:3<>1.0.CO;2-I</doi><numberingGroup><numbering type="journalVolume" number="14">14</numbering><numbering type="journalIssue">3</numbering></numberingGroup><coverDate startDate="1999-05">May 1999</coverDate></publicationMeta><publicationMeta level="unit" type="reviewArticle" position="3" status="forIssue"><doi origin="wiley" registered="yes">10.1002/1531-8257(199905)14:3<398::AID-MDS1003>3.0.CO;2-F</doi><idGroup><id type="unit" value="MDS1003"></id></idGroup><countGroup><count type="pageTotal" number="6"></count></countGroup><titleGroup><title type="articleCategory">Review</title><title type="tocHeading1">Review</title></titleGroup><copyright ownership="thirdParty">Copyright © 1999 Movement Disorder Society</copyright><eventGroup><event type="manuscriptReceived" date="1998-02-04"></event><event type="manuscriptRevised" date="1998-07-16"></event><event type="manuscriptAccepted" date="1998-09-30"></event><event type="firstOnline" date="2001-01-23"></event><event type="publishedOnlineFinalForm" date="2001-01-23"></event><event type="xmlConverted" agent="Converter:JWSART34_TO_WML3G version:2.3.2 mode:FullText source:HeaderRef result:HeaderRef" date="2010-03-09"></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">398</numbering><numbering type="pageLast">403</numbering></numberingGroup><correspondenceTo>Dipartimento Scienze Neurologiche, Università “La Sapienza,” Viale Università 30‐00185, Rome, Italy</correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:MDS.MDS1003.pdf"></link></linkGroup></publicationMeta><contentMeta><countGroup><count type="figureTotal" number="0"></count><count type="tableTotal" number="0"></count><count type="referenceTotal" number="70"></count><count type="wordTotal" number="4181"></count></countGroup><titleGroup><title type="main" xml:lang="en">Pathophysiology of chorea and bradykinesia in Huntington's disease</title><title type="short" xml:lang="en">Pathophysiology of HD</title></titleGroup><creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><givenNames>Alfredo</givenNames><familyName>Berardelli</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au2" creatorRole="author" affiliationRef="#af2"><personName><givenNames>Johannes</givenNames><familyName>Noth</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au3" creatorRole="author" affiliationRef="#af4"><personName><givenNames>Philip D.</givenNames><familyName>Thompson</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au4" creatorRole="author" affiliationRef="#af5"><personName><givenNames>Eduard L. E. M.</givenNames><familyName>Bollen</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au5" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Antonio</givenNames><familyName>Currà</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au6" creatorRole="author" affiliationRef="#af3"><personName><givenNames>Gunther</givenNames><familyName>Deuschl</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au7" creatorRole="author" affiliationRef="#af5"><personName><givenNames>J.</givenNames><familyName>Gert van Dijk</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au8" creatorRole="author" affiliationRef="#af2"><personName><givenNames>Rudolf</givenNames><familyName>Töpper</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au9" creatorRole="author" affiliationRef="#af2"><personName><givenNames>Michael</givenNames><familyName>Schwarz</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au10" creatorRole="author" affiliationRef="#af5"><personName><givenNames>Raymund A. C.</givenNames><familyName>Roos</familyName><degrees>MD</degrees></personName></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="IT" type="organization"><unparsedAffiliation>Dipartimento di Scienze Neurologiche Università di Roma “La Sapienza,” v.le Università, Rome, Italy, and Istituto Neurologico Mediterraneo “Neuromed,” Pozzilli, Italy</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="DE" type="organization"><unparsedAffiliation>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="DE" type="organization"><unparsedAffiliation>Klinik fúr Neurologie, Christian‐Albrechts‐Universität zu Kiel, Kiel, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af4" countryCode="AU" type="organization"><unparsedAffiliation>Department of Medicine, The Royal Adelaide Hospital, Adelaide, South Australia</unparsedAffiliation></affiliation><affiliation xml:id="af5" countryCode="NL" type="organization"><unparsedAffiliation>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">Huntington's disease</keyword><keyword xml:id="kwd2">Neurophysiology</keyword><keyword xml:id="kwd3">Motor system</keyword></keywordGroup><fundingInfo><fundingAgency>EC (Nest‐HD) and the association “Ricerca in Movement”</fundingAgency><fundingNumber>(BMH1‐CT93‐1621)</fundingNumber></fundingInfo><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>This article reviews the neurophysiological abnormalities described in Huntington's disease. Among the typical features of choreic movements are variable and random patterns of electromyographic (EMG) activity, including cocontraction of agonist and antagonist muscles. Studies of premotor potentials show that choreic movements are not preceded by a Bereitschaftspotential, therefore demonstrating that choreic movement is involuntary. Early cortical median‐nerve somatosensory‐evoked potentials have reduced amplitudes and the reduction correlates with reduced glucose consumption in the caudate nucleus. Long‐latency stretch reflexes evoked in the small hand muscles are depressed. These findings may reflect failed thalamocortical relay of sensory information. In Huntington's disease, the R2 response of the blink reflex has prolonged latencies, diminished amplitudes, and greater habituation than normal. These abnormalities correlate with the severity of chorea in the face. Patients with Huntington's disease perform simple voluntary movements more slowly than normal subjects and with an abnormal triphasic EMG pattern. Bradykinesia is also present during their performance of simultaneous and sequential movements. Eye movements show abnormalities similar to those seen in arm movements. In Huntington's disease, arm movement execution is associated with reduced PET activation of cortical frontal areas. Studies using transcranial magnetic stimulation show that patients with Huntington's disease have normal corticospinal conduction but some patients have a prolonged cortical silent period. Bradykinesia results from degeneration of the basal ganglia output to the supplementary motor areas concerned with the initiation and maintenance of sequential movements. The coexisting hyperkinetic and hypokinetic movement disorders in patients with Huntington's disease probably reflect the involvement of direct and indirect pathways in the basal ganglia–thalamus–cortical motor circuit.</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><!--Version 0.6 générée le 4-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Pathophysiology of chorea and bradykinesia in Huntington's disease</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Pathophysiology of HD</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Pathophysiology of chorea and bradykinesia in Huntington's disease</title></titleInfo><name type="personal"><namePart type="given">Alfredo</namePart><namePart type="family">Berardelli</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Dipartimento di Scienze Neurologiche Università di Roma “La Sapienza,” v.le Università, Rome, Italy, and Istituto Neurologico Mediterraneo “Neuromed,” Pozzilli, Italy</affiliation><description>Correspondence: Dipartimento Scienze Neurologiche, Università “La Sapienza,” Viale Università 30‐00185, Rome, Italy</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Johannes</namePart><namePart type="family">Noth</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Philip D.</namePart><namePart type="family">Thompson</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Medicine, The Royal Adelaide Hospital, Adelaide, South Australia</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Eduard L. E. M.</namePart><namePart type="family">Bollen</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Antonio</namePart><namePart type="family">Currà</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Dipartimento di Scienze Neurologiche Università di Roma “La Sapienza,” v.le Università, Rome, Italy, and Istituto Neurologico Mediterraneo “Neuromed,” Pozzilli, Italy</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Gunther</namePart><namePart type="family">Deuschl</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Klinik fúr Neurologie, Christian‐Albrechts‐Universität zu Kiel, Kiel, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">J.</namePart><namePart type="family">Gert van Dijk</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Rudolf</namePart><namePart type="family">Töpper</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Michael</namePart><namePart type="family">Schwarz</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Neurologische Klinik, RWTH, Aachen Technical Univeersity, Aachen</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Raymund A. C.</namePart><namePart type="family">Roos</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology and Clinical Neurophysiology, Leiden University Hospital, Leiden, The Netherlands</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre authority="originalCategForm">reviewArticle</genre><originInfo><publisher>John Wiley & Sons, Inc.</publisher><place><placeTerm type="text">New York</placeTerm></place><dateIssued encoding="w3cdtf">1999-05</dateIssued><dateCaptured encoding="w3cdtf">1998-02-04</dateCaptured><dateValid encoding="w3cdtf">1998-09-30</dateValid><copyrightDate encoding="w3cdtf">1999</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">70</extent><extent unit="words">4181</extent></physicalDescription><abstract lang="en">This article reviews the neurophysiological abnormalities described in Huntington's disease. Among the typical features of choreic movements are variable and random patterns of electromyographic (EMG) activity, including cocontraction of agonist and antagonist muscles. Studies of premotor potentials show that choreic movements are not preceded by a Bereitschaftspotential, therefore demonstrating that choreic movement is involuntary. Early cortical median‐nerve somatosensory‐evoked potentials have reduced amplitudes and the reduction correlates with reduced glucose consumption in the caudate nucleus. Long‐latency stretch reflexes evoked in the small hand muscles are depressed. These findings may reflect failed thalamocortical relay of sensory information. In Huntington's disease, the R2 response of the blink reflex has prolonged latencies, diminished amplitudes, and greater habituation than normal. These abnormalities correlate with the severity of chorea in the face. Patients with Huntington's disease perform simple voluntary movements more slowly than normal subjects and with an abnormal triphasic EMG pattern. Bradykinesia is also present during their performance of simultaneous and sequential movements. Eye movements show abnormalities similar to those seen in arm movements. In Huntington's disease, arm movement execution is associated with reduced PET activation of cortical frontal areas. Studies using transcranial magnetic stimulation show that patients with Huntington's disease have normal corticospinal conduction but some patients have a prolonged cortical silent period. Bradykinesia results from degeneration of the basal ganglia output to the supplementary motor areas concerned with the initiation and maintenance of sequential movements. The coexisting hyperkinetic and hypokinetic movement disorders in patients with Huntington's disease probably reflect the involvement of direct and indirect pathways in the basal ganglia–thalamus–cortical motor circuit.</abstract><note type="funding">EC (Nest‐HD) and the association “Ricerca in Movement” - No. (BMH1‐CT93‐1621); </note><subject lang="en"><genre>Keywords</genre><topic>Huntington's disease</topic><topic>Neurophysiology</topic><topic>Motor system</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><subject><genre>article category</genre><topic>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>1999</date><detail type="volume"><caption>vol.</caption><number>14</number></detail><detail type="issue"><caption>no.</caption><number>3</number></detail><extent unit="pages"><start>398</start><end>403</end><total>6</total></extent></part></relatedItem><identifier type="istex">B090E3887BDA361200F04109E6801C05EE8A85E5</identifier><identifier type="DOI">10.1002/1531-8257(199905)14:3<398::AID-MDS1003>3.0.CO;2-F</identifier><identifier type="ArticleID">MDS1003</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 1999 Movement Disorder Society</accessCondition><recordInfo><recordOrigin>John Wiley & Sons, Inc.</recordOrigin><recordContentSource>WILEY</recordContentSource></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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