Cortical myoclonus in huntington's disease
Identifieur interne : 001990 ( Istex/Corpus ); précédent : 001989; suivant : 001991Cortical myoclonus in huntington's disease
Auteurs : P. D. Thompson ; K. P. Bhatia ; P. Brown ; M. B. Davis ; M. Pires ; N. P. Quinn ; P. Luthert ; M. Honovar ; M. D. O'Brien ; C. D. Marsden ; HardingSource :
- Movement Disorders [ 0885-3185 ] ; 1994.
English descriptors
- KwdEn :
Abstract
We describe three patients with Huntington's disease, from two families, in whom myoclonus was the predominant clinical feature. The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. These patients all presented before the age of 30 years and were the offspring of affected fathers. Neurophysiological studies documented generalised and multifocal action myoclonus of cortical origin that was strikingly stimulus sensitive, without enlargement of the cortical somatosensory evoked potential. The myoclonus improved with piracetam therapy in one patient and a combination of sodium valproate and clonazepam in the other two. Cortical reflex myoclonus is a rare but disabling component of the complex movement disorder of Huntington's disease, which may lead to substantial diagnostic difficulties.
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DOI: 10.1002/mds.870090609
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ISTEX:82C825632CD0A6FB3924394F12E8570F844CB592Le document en format XML
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The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. These patients all presented before the age of 30 years and were the offspring of affected fathers. Neurophysiological studies documented generalised and multifocal action myoclonus of cortical origin that was strikingly stimulus sensitive, without enlargement of the cortical somatosensory evoked potential. The myoclonus improved with piracetam therapy in one patient and a combination of sodium valproate and clonazepam in the other two. Cortical reflex myoclonus is a rare but disabling component of the complex movement disorder of Huntington's disease, which may lead to substantial diagnostic difficulties.</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>P. D. 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Marsden</name><affiliations><json:string>University Department of Clinical Neurology, Guy's Hospital, London, England</json:string><json:string>MRC Human Movement and Balance Unit, Guy's Hospital, London, England</json:string></affiliations></json:item><json:item><name>Prof. Harding</name><affiliations><json:string>University Department of Clinical Neurology, Guy's Hospital, London, England</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>Cortical myoclonus</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Huntington's disease</value></json:item></subject><language><json:string>eng</json:string></language><abstract>We describe three patients with Huntington's disease, from two families, in whom myoclonus was the predominant clinical feature. The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. 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E.</givenNames><familyName>Harding</familyName></personName></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="GB" type="organization"><unparsedAffiliation>University Department of Clinical Neurology, Guy's Hospital, London, England</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="GB" type="organization"><unparsedAffiliation>MRC Human Movement and Balance Unit, Guy's Hospital, London, England</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="GB" type="organization"><unparsedAffiliation>Department of Neuropathology, Institute of Neurology, Guy's Hospital, London, England</unparsedAffiliation></affiliation><affiliation xml:id="af4" countryCode="GB" type="organization"><unparsedAffiliation>Department of Neuropathology, Institute of Psychiatry Guy's Hospital, London, England</unparsedAffiliation></affiliation><affiliation xml:id="af5" countryCode="GB" type="organization"><unparsedAffiliation>Department of Neurology, Guy's Hospital, London, England</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">Cortical myoclonus</keyword><keyword xml:id="kwd2">Huntington's disease</keyword></keywordGroup><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>We describe three patients with Huntington's disease, from two families, in whom myoclonus was the predominant clinical feature. The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. These patients all presented before the age of 30 years and were the offspring of affected fathers. Neurophysiological studies documented generalised and multifocal action myoclonus of cortical origin that was strikingly stimulus sensitive, without enlargement of the cortical somatosensory evoked potential. The myoclonus improved with piracetam therapy in one patient and a combination of sodium valproate and clonazepam in the other two. Cortical reflex myoclonus is a rare but disabling component of the complex movement disorder of Huntington's disease, which may lead to substantial diagnostic difficulties.</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>Cortical myoclonus in huntington's disease</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>CORTICAL MYOCLONUS IN HUNTINGTON'S DISEASE</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Cortical myoclonus in huntington's disease</title></titleInfo><name type="personal"><namePart type="given">P. D.</namePart><namePart type="family">Thompson</namePart><affiliation>University Department of Clinical Neurology, Guy's Hospital, London, England</affiliation><affiliation>MRC Human Movement and Balance Unit, Guy's Hospital, London, England</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">K. P.</namePart><namePart type="family">Bhatia</namePart><affiliation>University Department of Clinical Neurology, Guy's Hospital, London, England</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">P.</namePart><namePart type="family">Brown</namePart><affiliation>University Department of Clinical Neurology, Guy's Hospital, London, England</affiliation><affiliation>MRC Human Movement and Balance Unit, Guy's Hospital, London, England</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">M. B.</namePart><namePart type="family">Davis</namePart><affiliation>University Department of Clinical Neurology, Guy's Hospital, London, England</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">M.</namePart><namePart type="family">Pires</namePart><affiliation>Department of Neuropathology, Institute of Neurology, Guy's Hospital, London, England</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">N. P.</namePart><namePart type="family">Quinn</namePart><affiliation>University Department of Clinical Neurology, Guy's Hospital, London, England</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">P.</namePart><namePart type="family">Luthert</namePart><affiliation>Department of Neuropathology, Institute of Psychiatry Guy's Hospital, London, England</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">M.</namePart><namePart type="family">Honovar</namePart><affiliation>Department of Neuropathology, Institute of Psychiatry Guy's Hospital, London, England</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">M. D.</namePart><namePart type="family">O'Brien</namePart><affiliation>Department of Neurology, Guy's Hospital, London, England</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">C. D.</namePart><namePart type="family">Marsden</namePart><affiliation>University Department of Clinical Neurology, Guy's Hospital, London, England</affiliation><affiliation>MRC Human Movement and Balance Unit, Guy's Hospital, London, England</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="termsOfAddress">Prof.</namePart><namePart type="family">Harding</namePart><affiliation>University Department of Clinical Neurology, Guy's Hospital, London, England</affiliation><description>Correspondence: Harding at University Department of Clinical Neurology, Institute of Neurology, Queen Square, London WC1N 3BG, U.K.</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">1994</dateIssued><copyrightDate encoding="w3cdtf">1994</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">5</extent><extent unit="references">21</extent></physicalDescription><abstract lang="en">We describe three patients with Huntington's disease, from two families, in whom myoclonus was the predominant clinical feature. The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. These patients all presented before the age of 30 years and were the offspring of affected fathers. Neurophysiological studies documented generalised and multifocal action myoclonus of cortical origin that was strikingly stimulus sensitive, without enlargement of the cortical somatosensory evoked potential. The myoclonus improved with piracetam therapy in one patient and a combination of sodium valproate and clonazepam in the other two. Cortical reflex myoclonus is a rare but disabling component of the complex movement disorder of Huntington's disease, which may lead to substantial diagnostic difficulties.</abstract><subject lang="en"><genre>Keywords</genre><topic>Cortical myoclonus</topic><topic>Huntington's disease</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>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>1994</date><detail type="volume"><caption>vol.</caption><number>9</number></detail><detail type="issue"><caption>no.</caption><number>6</number></detail><extent unit="pages"><start>633</start><end>641</end><total>9</total></extent></part></relatedItem><identifier type="istex">82C825632CD0A6FB3924394F12E8570F844CB592</identifier><identifier type="DOI">10.1002/mds.870090609</identifier><identifier type="ArticleID">MDS870090609</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 1994 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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