Clinical relevance of electrophysiological tests in the assessment of patients with Huntington's disease.
Identifieur interne : 003908 ( PubMed/Curation ); précédent : 003907; suivant : 003909Clinical relevance of electrophysiological tests in the assessment of patients with Huntington's disease.
Auteurs : Jean-Pascal Lefaucheur [France] ; Anne-Catherine Bachoud-Levi ; Catherine Bourdet ; Thierry Grandmougin ; Philippe Hantraye ; Pierre Cesaro ; Jean-Denis Degos ; Marc Peschanski ; Fabrice LisovoskiSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2002.
English descriptors
- KwdEn :
- Adult, Blinking (physiology), Cerebral Cortex (physiopathology), Disease Progression, Electric Stimulation, Electrodiagnosis, Electromagnetic Fields, Electromyography, Evoked Potentials, Somatosensory (physiology), Female, Follow-Up Studies, Galvanic Skin Response (physiology), Humans, Huntington Disease (diagnosis), Huntington Disease (genetics), Huntington Disease (physiopathology), Hypokinesia (diagnosis), Hypokinesia (physiopathology), Male, Middle Aged, Reaction Time (physiology), Reflex, Stretch (physiology), Sensitivity and Specificity, Sympathetic Nervous System (physiopathology).
- MESH :
- diagnosis : Huntington Disease, Hypokinesia.
- genetics : Huntington Disease.
- physiology : Blinking, Evoked Potentials, Somatosensory, Galvanic Skin Response, Reaction Time, Reflex, Stretch.
- physiopathology : Cerebral Cortex, Huntington Disease, Hypokinesia, Sympathetic Nervous System.
- Adult, Disease Progression, Electric Stimulation, Electrodiagnosis, Electromagnetic Fields, Electromyography, Female, Follow-Up Studies, Humans, Male, Middle Aged, Sensitivity and Specificity.
Abstract
Assessment programs recently designed to follow-up patients with Huntington's disease (HD) in therapeutic trials have not included electrophysiological testing in the list of mandatory examinations. This omission is likely due to the current lack of data establishing a clear correlation between the electrophysiological results and those of clinical assessment. We address this issue in a cohort of 36 patients at relatively early stages of the disease (I and II). Electrophysiological studies comprised the recording of palmar sympathetic skin responses (SSRs), blink reflexes (BRs), thenar long latency reflexes (LLRs), cortical somatosensory evoked potentials (SEPs), and electromyographic silent periods evoked by transcranial magnetic stimulation (SPs). Results were analyzed with reference to disease duration and staging and to specific cognitive, psychiatric, and motor alteration. SEPs were the most and very sensitive markers, because they were abnormal in 94% of patients. Except for LLRs, alteration of electrophysiological results increased in parallel to the evolution of the disease. Except for LLRs and SSR latency, electrophysiological results correlated with those of specific clinical examinations. In particular, an increased BR latency or a reduced amplitude of the N20 component of SEPs correlated with the extent of bradykinesia, whereas a reduced amplitude of SSRs or of the N30 component of SEPs correlated with hyperkinesia. Overall, electrophysiological tests, in particular SEPs and BRs, appeared sensitive and interesting in the follow-up of HD patients and correlated with various clinical parameters, suggesting that these easy to perform and noninvasive repeatable examinations could be added fruitfully to the assessment programs for HD.
DOI: 10.1002/mds.10273
PubMed: 12465071
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Explorations Fonctionnelles (Neurophysiologie Clinique), Centre Hospitalier Universitaire Henri Mondor, Créteil, France. jean-pascal.lefaucheur@hmn.ap-hop-paris.fr</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Service de Physiologie - Explorations Fonctionnelles (Neurophysiologie Clinique), Centre Hospitalier Universitaire Henri Mondor, Créteil</wicri:regionArea></affiliation></author><author><name sortKey="Bachoud Levi, Anne Catherine" sort="Bachoud Levi, Anne Catherine" uniqKey="Bachoud Levi A" first="Anne-Catherine" last="Bachoud-Levi">Anne-Catherine Bachoud-Levi</name></author><author><name sortKey="Bourdet, Catherine" sort="Bourdet, Catherine" uniqKey="Bourdet C" first="Catherine" last="Bourdet">Catherine Bourdet</name></author><author><name sortKey="Grandmougin, Thierry" sort="Grandmougin, Thierry" uniqKey="Grandmougin T" first="Thierry" last="Grandmougin">Thierry Grandmougin</name></author><author><name sortKey="Hantraye, Philippe" sort="Hantraye, Philippe" uniqKey="Hantraye P" first="Philippe" last="Hantraye">Philippe Hantraye</name></author><author><name sortKey="Cesaro, Pierre" sort="Cesaro, Pierre" uniqKey="Cesaro P" first="Pierre" last="Cesaro">Pierre Cesaro</name></author><author><name sortKey="Degos, Jean Denis" sort="Degos, Jean Denis" uniqKey="Degos J" first="Jean-Denis" last="Degos">Jean-Denis Degos</name></author><author><name sortKey="Peschanski, Marc" sort="Peschanski, Marc" uniqKey="Peschanski M" first="Marc" last="Peschanski">Marc Peschanski</name></author><author><name sortKey="Lisovoski, Fabrice" sort="Lisovoski, Fabrice" uniqKey="Lisovoski F" first="Fabrice" last="Lisovoski">Fabrice Lisovoski</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="ISSN">0885-3185</idno><imprint><date when="2002" type="published">2002</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Blinking (physiology)</term><term>Cerebral Cortex (physiopathology)</term><term>Disease Progression</term><term>Electric Stimulation</term><term>Electrodiagnosis</term><term>Electromagnetic Fields</term><term>Electromyography</term><term>Evoked Potentials, Somatosensory (physiology)</term><term>Female</term><term>Follow-Up Studies</term><term>Galvanic Skin Response (physiology)</term><term>Humans</term><term>Huntington Disease (diagnosis)</term><term>Huntington Disease (genetics)</term><term>Huntington Disease (physiopathology)</term><term>Hypokinesia (diagnosis)</term><term>Hypokinesia (physiopathology)</term><term>Male</term><term>Middle Aged</term><term>Reaction Time (physiology)</term><term>Reflex, Stretch (physiology)</term><term>Sensitivity and Specificity</term><term>Sympathetic Nervous System (physiopathology)</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Huntington Disease</term><term>Hypokinesia</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Huntington Disease</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Blinking</term><term>Evoked Potentials, Somatosensory</term><term>Galvanic Skin Response</term><term>Reaction Time</term><term>Reflex, Stretch</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Cerebral Cortex</term><term>Huntington Disease</term><term>Hypokinesia</term><term>Sympathetic Nervous System</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Disease Progression</term><term>Electric Stimulation</term><term>Electrodiagnosis</term><term>Electromagnetic Fields</term><term>Electromyography</term><term>Female</term><term>Follow-Up Studies</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Sensitivity and Specificity</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Assessment programs recently designed to follow-up patients with Huntington's disease (HD) in therapeutic trials have not included electrophysiological testing in the list of mandatory examinations. This omission is likely due to the current lack of data establishing a clear correlation between the electrophysiological results and those of clinical assessment. We address this issue in a cohort of 36 patients at relatively early stages of the disease (I and II). Electrophysiological studies comprised the recording of palmar sympathetic skin responses (SSRs), blink reflexes (BRs), thenar long latency reflexes (LLRs), cortical somatosensory evoked potentials (SEPs), and electromyographic silent periods evoked by transcranial magnetic stimulation (SPs). Results were analyzed with reference to disease duration and staging and to specific cognitive, psychiatric, and motor alteration. SEPs were the most and very sensitive markers, because they were abnormal in 94% of patients. Except for LLRs, alteration of electrophysiological results increased in parallel to the evolution of the disease. Except for LLRs and SSR latency, electrophysiological results correlated with those of specific clinical examinations. In particular, an increased BR latency or a reduced amplitude of the N20 component of SEPs correlated with the extent of bradykinesia, whereas a reduced amplitude of SSRs or of the N30 component of SEPs correlated with hyperkinesia. Overall, electrophysiological tests, in particular SEPs and BRs, appeared sensitive and interesting in the follow-up of HD patients and correlated with various clinical parameters, suggesting that these easy to perform and noninvasive repeatable examinations could be added fruitfully to the assessment programs for HD.</div></front></TEI><pubmed><MedlineCitation Owner="NLM" Status="MEDLINE"><PMID Version="1">12465071</PMID><DateCreated><Year>2002</Year><Month>12</Month><Day>04</Day></DateCreated><DateCompleted><Year>2003</Year><Month>03</Month><Day>24</Day></DateCompleted><DateRevised><Year>2006</Year><Month>11</Month><Day>15</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0885-3185</ISSN><JournalIssue CitedMedium="Print"><Volume>17</Volume><Issue>6</Issue><PubDate><Year>2002</Year><Month>Nov</Month></PubDate></JournalIssue><Title>Movement disorders : official journal of the Movement Disorder Society</Title><ISOAbbreviation>Mov. Disord.</ISOAbbreviation></Journal><ArticleTitle>Clinical relevance of electrophysiological tests in the assessment of patients with Huntington's disease.</ArticleTitle><Pagination><MedlinePgn>1294-301</MedlinePgn></Pagination><Abstract><AbstractText>Assessment programs recently designed to follow-up patients with Huntington's disease (HD) in therapeutic trials have not included electrophysiological testing in the list of mandatory examinations. This omission is likely due to the current lack of data establishing a clear correlation between the electrophysiological results and those of clinical assessment. We address this issue in a cohort of 36 patients at relatively early stages of the disease (I and II). Electrophysiological studies comprised the recording of palmar sympathetic skin responses (SSRs), blink reflexes (BRs), thenar long latency reflexes (LLRs), cortical somatosensory evoked potentials (SEPs), and electromyographic silent periods evoked by transcranial magnetic stimulation (SPs). Results were analyzed with reference to disease duration and staging and to specific cognitive, psychiatric, and motor alteration. SEPs were the most and very sensitive markers, because they were abnormal in 94% of patients. Except for LLRs, alteration of electrophysiological results increased in parallel to the evolution of the disease. Except for LLRs and SSR latency, electrophysiological results correlated with those of specific clinical examinations. In particular, an increased BR latency or a reduced amplitude of the N20 component of SEPs correlated with the extent of bradykinesia, whereas a reduced amplitude of SSRs or of the N30 component of SEPs correlated with hyperkinesia. Overall, electrophysiological tests, in particular SEPs and BRs, appeared sensitive and interesting in the follow-up of HD patients and correlated with various clinical parameters, suggesting that these easy to perform and noninvasive repeatable examinations could be added fruitfully to the assessment programs for HD.</AbstractText><CopyrightInformation>Copyright 2002 Movement Disorder Society</CopyrightInformation></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Lefaucheur</LastName><ForeName>Jean-Pascal</ForeName><Initials>JP</Initials><AffiliationInfo><Affiliation>Service de Physiologie - Explorations Fonctionnelles (Neurophysiologie Clinique), Centre Hospitalier Universitaire Henri Mondor, Créteil, France. jean-pascal.lefaucheur@hmn.ap-hop-paris.fr</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Bachoud-Levi</LastName><ForeName>Anne-Catherine</ForeName><Initials>AC</Initials></Author><Author ValidYN="Y"><LastName>Bourdet</LastName><ForeName>Catherine</ForeName><Initials>C</Initials></Author><Author ValidYN="Y"><LastName>Grandmougin</LastName><ForeName>Thierry</ForeName><Initials>T</Initials></Author><Author ValidYN="Y"><LastName>Hantraye</LastName><ForeName>Philippe</ForeName><Initials>P</Initials></Author><Author ValidYN="Y"><LastName>Cesaro</LastName><ForeName>Pierre</ForeName><Initials>P</Initials></Author><Author ValidYN="Y"><LastName>Degos</LastName><ForeName>Jean-Denis</ForeName><Initials>JD</Initials></Author><Author ValidYN="Y"><LastName>Peschanski</LastName><ForeName>Marc</ForeName><Initials>M</Initials></Author><Author ValidYN="Y"><LastName>Lisovoski</LastName><ForeName>Fabrice</ForeName><Initials>F</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D003160">Comparative Study</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Mov Disord</MedlineTA><NlmUniqueID>8610688</NlmUniqueID><ISSNLinking>0885-3185</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName MajorTopicYN="N" UI="D000328">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D001767">Blinking</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D002540">Cerebral Cortex</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D018450">Disease Progression</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D004558">Electric Stimulation</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="Y" UI="D004568">Electrodiagnosis</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D004574">Electromagnetic Fields</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="Y" UI="D004576">Electromyography</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005073">Evoked Potentials, Somatosensory</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005260">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005500">Follow-Up Studies</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005712">Galvanic Skin Response</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006801">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006816">Huntington Disease</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000235">genetics</QualifierName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D018476">Hypokinesia</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008297">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008875">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D011930">Reaction Time</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D012026">Reflex, Stretch</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D012680">Sensitivity and Specificity</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D013564">Sympathetic Nervous System</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>2002</Year><Month>12</Month><Day>5</Day><Hour>4</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2003</Year><Month>3</Month><Day>26</Day><Hour>4</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2002</Year><Month>12</Month><Day>5</Day><Hour>4</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">12465071</ArticleId><ArticleId 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