Electrophysiological features of myoclonus‐dystonia
Identifieur interne : 001253 ( Istex/Corpus ); précédent : 001252; suivant : 001254Electrophysiological features of myoclonus‐dystonia
Auteurs : Jie-Yuan Li ; Danny I. Cunic ; Guillermo Paradiso ; Carolyn Gunraj ; Pramod K. Pal ; Anthony E. Lang ; Robert ChenSource :
- Movement Disorders [ 0885-3185 ] ; 2008-10-30.
English descriptors
Abstract
Inherited myoclonus‐dystonia (M‐D) is an autosomal dominant disorder characterized by myoclonus and dystonia that often improves with alcohol. To examine the electrophysiologic characteristics of M‐D, we studied 6 patients from 4 different families and 9 age‐matched healthy subjects. Neurophysiological studies performed include electromyography (EMG)‐electroencephalography (EEG) polygraphy, jerk‐locked back‐averaged EEG, somatosensory evoked potentials (SEP), long‐latency reflex (LLR) to median and digital nerve stimulation, and transcranial magnetic stimulation studies with short‐interval intracortical inhibition (SICI), intracortical facilitation (ICF), and long‐interval intracortical inhibition (LICI). All 6 patients showed myoclonus and dystonia on clinical examination and EMG testing. The EMG burst durations ranged from 30.4 to 750.6 milliseconds (mean, 101.5 milliseconds). Jerk‐locked back‐averaged EEG failed to reveal any preceding cortical correlates. Median nerve SEP revealed no giant potential. No patients had exaggerated LLR to median or digital nerve stimulation. There was no significant difference in SICI, ICF, and LICI between M‐D patients and normal subjects. Myoclonus in inherited M‐D is likely of subcortical origin. Normal intracortical inhibition and facilitation suggest that the GABAergic circuits in the motor cortex are largely intact and that the mechanisms of myoclonus and dystonia are different from those for cortical myoclonus and other dystonic disorders. © 2008 Movement Disorder Society
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DOI: 10.1002/mds.22273
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To examine the electrophysiologic characteristics of M‐D, we studied 6 patients from 4 different families and 9 age‐matched healthy subjects. Neurophysiological studies performed include electromyography (EMG)‐electroencephalography (EEG) polygraphy, jerk‐locked back‐averaged EEG, somatosensory evoked potentials (SEP), long‐latency reflex (LLR) to median and digital nerve stimulation, and transcranial magnetic stimulation studies with short‐interval intracortical inhibition (SICI), intracortical facilitation (ICF), and long‐interval intracortical inhibition (LICI). All 6 patients showed myoclonus and dystonia on clinical examination and EMG testing. The EMG burst durations ranged from 30.4 to 750.6 milliseconds (mean, 101.5 milliseconds). Jerk‐locked back‐averaged EEG failed to reveal any preceding cortical correlates. Median nerve SEP revealed no giant potential. No patients had exaggerated LLR to median or digital nerve stimulation. There was no significant difference in SICI, ICF, and LICI between M‐D patients and normal subjects. Myoclonus in inherited M‐D is likely of subcortical origin. Normal intracortical inhibition and facilitation suggest that the GABAergic circuits in the motor cortex are largely intact and that the mechanisms of myoclonus and dystonia are different from those for cortical myoclonus and other dystonic disorders. © 2008 Movement Disorder Society</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Jie‐Yuan Li MD</name><affiliations><json:string>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</json:string><json:string>Division of Neurology, Department of Internal Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan</json:string><json:string>Faculty of Medicine, School of Medicine, National Yang‐Ming University, Taipei, Taiwan</json:string></affiliations></json:item><json:item><name>Danny I. Cunic PhD</name><affiliations><json:string>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</json:string></affiliations></json:item><json:item><name>Guillermo Paradiso MD</name><affiliations><json:string>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</json:string></affiliations></json:item><json:item><name>Carolyn Gunraj MHSc</name><affiliations><json:string>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</json:string></affiliations></json:item><json:item><name>Pramod K. Pal MD</name><affiliations><json:string>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</json:string></affiliations></json:item><json:item><name>Anthony E. Lang MD, FRCPC</name><affiliations><json:string>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</json:string></affiliations></json:item><json:item><name>Robert Chen MBBChir, MSc, FRCPC</name><affiliations><json:string>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>myoclonus</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>dystonia</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>physiology</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>transcranial magnetic stimulation</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>cortical inhibition</value></json:item></subject><language><json:string>eng</json:string></language><abstract>Inherited myoclonus‐dystonia (M‐D) is an autosomal dominant disorder characterized by myoclonus and dystonia that often improves with alcohol. To examine the electrophysiologic characteristics of M‐D, we studied 6 patients from 4 different families and 9 age‐matched healthy subjects. Neurophysiological studies performed include electromyography (EMG)‐electroencephalography (EEG) polygraphy, jerk‐locked back‐averaged EEG, somatosensory evoked potentials (SEP), long‐latency reflex (LLR) to median and digital nerve stimulation, and transcranial magnetic stimulation studies with short‐interval intracortical inhibition (SICI), intracortical facilitation (ICF), and long‐interval intracortical inhibition (LICI). All 6 patients showed myoclonus and dystonia on clinical examination and EMG testing. The EMG burst durations ranged from 30.4 to 750.6 milliseconds (mean, 101.5 milliseconds). Jerk‐locked back‐averaged EEG failed to reveal any preceding cortical correlates. Median nerve SEP revealed no giant potential. No patients had exaggerated LLR to median or digital nerve stimulation. There was no significant difference in SICI, ICF, and LICI between M‐D patients and normal subjects. Myoclonus in inherited M‐D is likely of subcortical origin. 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No. MOP15128;</note><note>Elizabeth Barford Fellowship in Neurology</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Electrophysiological features of myoclonus‐dystonia</title><author><persName><forename type="first">Jie‐Yuan</forename><surname>Li</surname><roleName type="degree">MD</roleName></persName><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation><affiliation>Division of Neurology, Department of Internal Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan</affiliation><affiliation>Faculty of Medicine, School of Medicine, National Yang‐Ming University, Taipei, Taiwan</affiliation></author><author><persName><forename type="first">Danny I.</forename><surname>Cunic</surname><roleName type="degree">PhD</roleName></persName><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation></author><author><persName><forename type="first">Guillermo</forename><surname>Paradiso</surname><roleName type="degree">MD</roleName></persName><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation></author><author><persName><forename type="first">Carolyn</forename><surname>Gunraj</surname><roleName type="degree">MHSc</roleName></persName><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation></author><author><persName><forename type="first">Pramod K.</forename><surname>Pal</surname><roleName type="degree">MD</roleName></persName><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation></author><author><persName><forename type="first">Anthony E.</forename><surname>Lang</surname><roleName type="degree">MD, FRCPC</roleName></persName><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation></author><author><persName><forename type="first">Robert</forename><surname>Chen</surname><roleName type="degree">MBBChir, MSc, FRCPC</roleName></persName><note type="correspondence"><p>Correspondence: Toronto Western Hospital, 7MC411, 399 Bathurst Street, Toronto, Ontario, Canada M5T 2S8</p></note><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</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. 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To examine the electrophysiologic characteristics of M‐D, we studied 6 patients from 4 different families and 9 age‐matched healthy subjects. Neurophysiological studies performed include electromyography (EMG)‐electroencephalography (EEG) polygraphy, jerk‐locked back‐averaged EEG, somatosensory evoked potentials (SEP), long‐latency reflex (LLR) to median and digital nerve stimulation, and transcranial magnetic stimulation studies with short‐interval intracortical inhibition (SICI), intracortical facilitation (ICF), and long‐interval intracortical inhibition (LICI). All 6 patients showed myoclonus and dystonia on clinical examination and EMG testing. The EMG burst durations ranged from 30.4 to 750.6 milliseconds (mean, 101.5 milliseconds). Jerk‐locked back‐averaged EEG failed to reveal any preceding cortical correlates. Median nerve SEP revealed no giant potential. No patients had exaggerated LLR to median or digital nerve stimulation. There was no significant difference in SICI, ICF, and LICI between M‐D patients and normal subjects. Myoclonus in inherited M‐D is likely of subcortical origin. Normal intracortical inhibition and facilitation suggest that the GABAergic circuits in the motor cortex are largely intact and that the mechanisms of myoclonus and dystonia are different from those for cortical myoclonus and other dystonic disorders. © 2008 Movement Disorder Society</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>myoclonus</term></item><item><term>dystonia</term></item><item><term>physiology</term></item><item><term>transcranial magnetic stimulation</term></item><item><term>cortical inhibition</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="2007-12-07">Received</change><change when="2008-07-17">Registration</change><change when="2008-10-30">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/6511E86CB7AC0819379EF8897FFD61B47E214AD7/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="subtitle">Official Journal of the Movement Disorder Society</title><title type="short">Mov. 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affiliationRef="#af1"><personName><givenNames>Guillermo</givenNames><familyName>Paradiso</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au4" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Carolyn</givenNames><familyName>Gunraj</familyName><degrees>MHSc</degrees></personName></creator><creator xml:id="au5" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Pramod K.</givenNames><familyName>Pal</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au6" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Anthony E.</givenNames><familyName>Lang</familyName><degrees>MD, FRCPC</degrees></personName></creator><creator xml:id="au7" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><givenNames>Robert</givenNames><familyName>Chen</familyName><degrees>MBBChir, MSc, FRCPC</degrees></personName><contactDetails><email>robert.chen@uhn.on.ca</email></contactDetails></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="CA" type="organization"><unparsedAffiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="TW" type="organization"><unparsedAffiliation>Division of Neurology, Department of Internal Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="TW" type="organization"><unparsedAffiliation>Faculty of Medicine, School of Medicine, National Yang‐Ming University, Taipei, Taiwan</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">myoclonus</keyword><keyword xml:id="kwd2">dystonia</keyword><keyword xml:id="kwd3">physiology</keyword><keyword xml:id="kwd4">transcranial magnetic stimulation</keyword><keyword xml:id="kwd5">cortical inhibition</keyword></keywordGroup><fundingInfo><fundingAgency>Canadian Institutes of Health Research</fundingAgency><fundingNumber>MOP15128</fundingNumber></fundingInfo><fundingInfo><fundingAgency>Elizabeth Barford Fellowship in Neurology</fundingAgency></fundingInfo><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>Inherited myoclonus‐dystonia (M‐D) is an autosomal dominant disorder characterized by myoclonus and dystonia that often improves with alcohol. To examine the electrophysiologic characteristics of M‐D, we studied 6 patients from 4 different families and 9 age‐matched healthy subjects. Neurophysiological studies performed include electromyography (EMG)‐electroencephalography (EEG) polygraphy, jerk‐locked back‐averaged EEG, somatosensory evoked potentials (SEP), long‐latency reflex (LLR) to median and digital nerve stimulation, and transcranial magnetic stimulation studies with short‐interval intracortical inhibition (SICI), intracortical facilitation (ICF), and long‐interval intracortical inhibition (LICI). All 6 patients showed myoclonus and dystonia on clinical examination and EMG testing. The EMG burst durations ranged from 30.4 to 750.6 milliseconds (mean, 101.5 milliseconds). Jerk‐locked back‐averaged EEG failed to reveal any preceding cortical correlates. Median nerve SEP revealed no giant potential. No patients had exaggerated LLR to median or digital nerve stimulation. There was no significant difference in SICI, ICF, and LICI between M‐D patients and normal subjects. Myoclonus in inherited M‐D is likely of subcortical origin. Normal intracortical inhibition and facilitation suggest that the GABAergic circuits in the motor cortex are largely intact and that the mechanisms of myoclonus and dystonia are different from those for cortical myoclonus and other dystonic disorders. © 2008 Movement Disorder Society</p></abstract></abstractGroup></contentMeta><noteGroup><note xml:id="fn1"><p>Potential conflict of interest: None reported.</p></note></noteGroup></header></component></istex:document></istex:metadataXml><!--Version 0.6 générée le 3-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Electrophysiological features of myoclonus‐dystonia</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Electrophysiological Features of Inherited M‐D</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Electrophysiological features of myoclonus‐dystonia</title></titleInfo><name type="personal"><namePart type="given">Jie‐Yuan</namePart><namePart type="family">Li</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation><affiliation>Division of Neurology, Department of Internal Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan</affiliation><affiliation>Faculty of Medicine, School of Medicine, National Yang‐Ming University, Taipei, Taiwan</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Danny I.</namePart><namePart type="family">Cunic</namePart><namePart type="termsOfAddress">PhD</namePart><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Guillermo</namePart><namePart type="family">Paradiso</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Carolyn</namePart><namePart type="family">Gunraj</namePart><namePart type="termsOfAddress">MHSc</namePart><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Pramod K.</namePart><namePart type="family">Pal</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Anthony E.</namePart><namePart type="family">Lang</namePart><namePart type="termsOfAddress">MD, FRCPC</namePart><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Robert</namePart><namePart type="family">Chen</namePart><namePart type="termsOfAddress">MBBChir, MSc, FRCPC</namePart><affiliation>Division of Neurology, Department of Medicine and Toronto Western Research Institute, University of Toronto, Toronto, Ontario, Canada</affiliation><description>Correspondence: Toronto Western Hospital, 7MC411, 399 Bathurst Street, Toronto, Ontario, Canada M5T 2S8</description><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre authority="originalCategForm">article</genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">2008-10-30</dateIssued><dateCaptured encoding="w3cdtf">2007-12-07</dateCaptured><dateValid encoding="w3cdtf">2008-07-17</dateValid><copyrightDate encoding="w3cdtf">2008</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">4</extent><extent unit="tables">1</extent><extent unit="references">47</extent><extent unit="words">4469</extent></physicalDescription><abstract lang="en">Inherited myoclonus‐dystonia (M‐D) is an autosomal dominant disorder characterized by myoclonus and dystonia that often improves with alcohol. To examine the electrophysiologic characteristics of M‐D, we studied 6 patients from 4 different families and 9 age‐matched healthy subjects. Neurophysiological studies performed include electromyography (EMG)‐electroencephalography (EEG) polygraphy, jerk‐locked back‐averaged EEG, somatosensory evoked potentials (SEP), long‐latency reflex (LLR) to median and digital nerve stimulation, and transcranial magnetic stimulation studies with short‐interval intracortical inhibition (SICI), intracortical facilitation (ICF), and long‐interval intracortical inhibition (LICI). All 6 patients showed myoclonus and dystonia on clinical examination and EMG testing. The EMG burst durations ranged from 30.4 to 750.6 milliseconds (mean, 101.5 milliseconds). Jerk‐locked back‐averaged EEG failed to reveal any preceding cortical correlates. Median nerve SEP revealed no giant potential. No patients had exaggerated LLR to median or digital nerve stimulation. There was no significant difference in SICI, ICF, and LICI between M‐D patients and normal subjects. Myoclonus in inherited M‐D is likely of subcortical origin. Normal intracortical inhibition and facilitation suggest that the GABAergic circuits in the motor cortex are largely intact and that the mechanisms of myoclonus and dystonia are different from those for cortical myoclonus and other dystonic disorders. © 2008 Movement Disorder Society</abstract><note type="content">*Potential conflict of interest: None reported.</note><note type="funding">Canadian Institutes of Health Research - No. MOP15128; </note><note type="funding">Elizabeth Barford Fellowship in Neurology</note><subject lang="en"><genre>Keywords</genre><topic>myoclonus</topic><topic>dystonia</topic><topic>physiology</topic><topic>transcranial magnetic stimulation</topic><topic>cortical inhibition</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>Research Article</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>2008</date><detail type="volume"><caption>vol.</caption><number>23</number></detail><detail type="issue"><caption>no.</caption><number>14</number></detail><extent unit="pages"><start>2055</start><end>2061</end><total>7</total></extent></part></relatedItem><identifier type="istex">6511E86CB7AC0819379EF8897FFD61B47E214AD7</identifier><identifier type="DOI">10.1002/mds.22273</identifier><identifier type="ArticleID">MDS22273</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2008 Movement Disorder Society</accessCondition><recordInfo><recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin><recordContentSource>WILEY</recordContentSource></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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