Electrophysiological deterioration over time in patients with Huntington's disease.
Identifieur interne : 001709 ( Ncbi/Merge ); précédent : 001708; suivant : 001710Electrophysiological deterioration over time in patients with Huntington's disease.
Auteurs : Jean-Pascal Lefaucheur [France] ; Isabelle Ménard-Lefaucheur ; Patrick Maison ; Sophie Baudic ; Pierre Cesaro ; Marc Peschanski ; Anne-Catherine Bachoud-LéviSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2006.
English descriptors
- KwdEn :
- Adult, Blinking (physiology), Cerebral Cortex (physiopathology), Disease Progression, Early Diagnosis, Electric Stimulation, Evoked Potentials, Somatosensory (physiology), Female, Galvanic Skin Response (physiology), Humans, Huntington Disease (diagnosis), Huntington Disease (genetics), Huntington Disease (physiopathology), Longitudinal Studies, Male, Median Nerve (physiopathology), Middle Aged, Neurologic Examination, Reaction Time (physiology), Reference Values, Sympathetic Nervous System (physiopathology), Transcranial Magnetic Stimulation.
- MESH :
- diagnosis : Huntington Disease.
- genetics : Huntington Disease.
- physiology : Blinking, Evoked Potentials, Somatosensory, Galvanic Skin Response, Reaction Time.
- physiopathology : Cerebral Cortex, Huntington Disease, Median Nerve, Sympathetic Nervous System.
- Adult, Disease Progression, Early Diagnosis, Electric Stimulation, Female, Humans, Longitudinal Studies, Male, Middle Aged, Neurologic Examination, Reference Values, Transcranial Magnetic Stimulation.
Abstract
In recent studies aimed at assessing the effects of original therapeutic strategies applied to patients with Huntington's disease (HD), we observed informative changes in electrophysiological results that recovered normal values in coherence with clinical improvement. However, longitudinal studies were lacking for determining whether electrophysiological test results evolve in parallel with clinical markers of the natural course of the disease and could consequently provide objective quantifiable markers of disease progression. For this purpose, electrophysiological testing was performed annually in a cohort of 20 patients with HD over a 2-year period (three examinations). The study included the recording of sympathetic skin responses and blink reflexes (BRs) to supraorbital nerve stimulation, long latency reflexes (LLRs) and somatosensory evoked potentials (SEPs) to median nerve stimulation, and cortical silent periods (CSPs) to transcranial magnetic stimulation. Clinical evaluation was based on the Total Functional Capacity scale (TFC) and the Motor part of the Unified Huntington's Disease Rating Scale (UHDRS). A significant deterioration with time was found for BR latency, LLR presence, various SEP parameters (parietal N20 peak amplitude and frontal N30 presence) and CSP duration. Attenuation of the N20 peak and CSP shortening correlated with functional decline, as assessed by the TFC score, whereas delayed BR and LLR abolition correlated with UHDRS Motor score deterioration. This study shows that several electrophysiological parameters are closely associated with dysfunction of various neural circuits in HD and could be useful markers of disease progression.
DOI: 10.1002/mds.20966
PubMed: 16721731
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Explorations Fonctionnelles, Hôpital Henri Mondor, AP-HP, 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, Hôpital Henri Mondor, AP-HP, Créteil</wicri:regionArea><placeName><settlement type="city">Créteil</settlement></placeName></affiliation></author><author><name sortKey="Menard Lefaucheur, Isabelle" sort="Menard Lefaucheur, Isabelle" uniqKey="Menard Lefaucheur I" first="Isabelle" last="Ménard-Lefaucheur">Isabelle Ménard-Lefaucheur</name></author><author><name sortKey="Maison, Patrick" sort="Maison, Patrick" uniqKey="Maison P" first="Patrick" last="Maison">Patrick Maison</name></author><author><name sortKey="Baudic, Sophie" sort="Baudic, Sophie" uniqKey="Baudic S" first="Sophie" last="Baudic">Sophie Baudic</name></author><author><name sortKey="Cesaro, Pierre" sort="Cesaro, Pierre" uniqKey="Cesaro P" first="Pierre" last="Cesaro">Pierre Cesaro</name></author><author><name sortKey="Peschanski, Marc" sort="Peschanski, Marc" uniqKey="Peschanski M" first="Marc" last="Peschanski">Marc Peschanski</name></author><author><name sortKey="Bachoud Levi, Anne Catherine" sort="Bachoud Levi, Anne Catherine" uniqKey="Bachoud Levi A" first="Anne-Catherine" last="Bachoud-Lévi">Anne-Catherine Bachoud-Lévi</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="2006" type="published">2006</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>Early Diagnosis</term><term>Electric Stimulation</term><term>Evoked Potentials, Somatosensory (physiology)</term><term>Female</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>Longitudinal Studies</term><term>Male</term><term>Median Nerve (physiopathology)</term><term>Middle Aged</term><term>Neurologic Examination</term><term>Reaction Time (physiology)</term><term>Reference Values</term><term>Sympathetic Nervous System (physiopathology)</term><term>Transcranial Magnetic Stimulation</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Huntington Disease</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></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Cerebral Cortex</term><term>Huntington Disease</term><term>Median Nerve</term><term>Sympathetic Nervous System</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Disease Progression</term><term>Early Diagnosis</term><term>Electric Stimulation</term><term>Female</term><term>Humans</term><term>Longitudinal Studies</term><term>Male</term><term>Middle Aged</term><term>Neurologic Examination</term><term>Reference Values</term><term>Transcranial Magnetic Stimulation</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">In recent studies aimed at assessing the effects of original therapeutic strategies applied to patients with Huntington's disease (HD), we observed informative changes in electrophysiological results that recovered normal values in coherence with clinical improvement. However, longitudinal studies were lacking for determining whether electrophysiological test results evolve in parallel with clinical markers of the natural course of the disease and could consequently provide objective quantifiable markers of disease progression. For this purpose, electrophysiological testing was performed annually in a cohort of 20 patients with HD over a 2-year period (three examinations). The study included the recording of sympathetic skin responses and blink reflexes (BRs) to supraorbital nerve stimulation, long latency reflexes (LLRs) and somatosensory evoked potentials (SEPs) to median nerve stimulation, and cortical silent periods (CSPs) to transcranial magnetic stimulation. Clinical evaluation was based on the Total Functional Capacity scale (TFC) and the Motor part of the Unified Huntington's Disease Rating Scale (UHDRS). A significant deterioration with time was found for BR latency, LLR presence, various SEP parameters (parietal N20 peak amplitude and frontal N30 presence) and CSP duration. Attenuation of the N20 peak and CSP shortening correlated with functional decline, as assessed by the TFC score, whereas delayed BR and LLR abolition correlated with UHDRS Motor score deterioration. This study shows that several electrophysiological parameters are closely associated with dysfunction of various neural circuits in HD and could be useful markers of disease progression.</div></front></TEI><pubmed><MedlineCitation Owner="NLM" Status="MEDLINE"><PMID Version="1">16721731</PMID><DateCreated><Year>2006</Year><Month>09</Month><Day>27</Day></DateCreated><DateCompleted><Year>2007</Year><Month>02</Month><Day>02</Day></DateCompleted><Article PubModel="Print"><Journal><ISSN IssnType="Print">0885-3185</ISSN><JournalIssue CitedMedium="Print"><Volume>21</Volume><Issue>9</Issue><PubDate><Year>2006</Year><Month>Sep</Month></PubDate></JournalIssue><Title>Movement disorders : official journal of the Movement Disorder Society</Title><ISOAbbreviation>Mov. Disord.</ISOAbbreviation></Journal><ArticleTitle>Electrophysiological deterioration over time in patients with Huntington's disease.</ArticleTitle><Pagination><MedlinePgn>1350-4</MedlinePgn></Pagination><Abstract><AbstractText>In recent studies aimed at assessing the effects of original therapeutic strategies applied to patients with Huntington's disease (HD), we observed informative changes in electrophysiological results that recovered normal values in coherence with clinical improvement. However, longitudinal studies were lacking for determining whether electrophysiological test results evolve in parallel with clinical markers of the natural course of the disease and could consequently provide objective quantifiable markers of disease progression. For this purpose, electrophysiological testing was performed annually in a cohort of 20 patients with HD over a 2-year period (three examinations). The study included the recording of sympathetic skin responses and blink reflexes (BRs) to supraorbital nerve stimulation, long latency reflexes (LLRs) and somatosensory evoked potentials (SEPs) to median nerve stimulation, and cortical silent periods (CSPs) to transcranial magnetic stimulation. Clinical evaluation was based on the Total Functional Capacity scale (TFC) and the Motor part of the Unified Huntington's Disease Rating Scale (UHDRS). A significant deterioration with time was found for BR latency, LLR presence, various SEP parameters (parietal N20 peak amplitude and frontal N30 presence) and CSP duration. Attenuation of the N20 peak and CSP shortening correlated with functional decline, as assessed by the TFC score, whereas delayed BR and LLR abolition correlated with UHDRS Motor score deterioration. This study shows that several electrophysiological parameters are closely associated with dysfunction of various neural circuits in HD and could be useful markers of disease progression.</AbstractText><CopyrightInformation>(c) 2006 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, Hôpital Henri Mondor, AP-HP, Créteil, France. jean-pascal.lefaucheur@hmn.ap-hop-paris.fr</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Ménard-Lefaucheur</LastName><ForeName>Isabelle</ForeName><Initials>I</Initials></Author><Author ValidYN="Y"><LastName>Maison</LastName><ForeName>Patrick</ForeName><Initials>P</Initials></Author><Author ValidYN="Y"><LastName>Baudic</LastName><ForeName>Sophie</ForeName><Initials>S</Initials></Author><Author ValidYN="Y"><LastName>Cesaro</LastName><ForeName>Pierre</ForeName><Initials>P</Initials></Author><Author ValidYN="Y"><LastName>Peschanski</LastName><ForeName>Marc</ForeName><Initials>M</Initials></Author><Author ValidYN="Y"><LastName>Bachoud-Lévi</LastName><ForeName>Anne-Catherine</ForeName><Initials>AC</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><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 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MajorTopicYN="N" UI="D011930">Reaction Time</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000502">physiology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D012016">Reference Values</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D013564">Sympathetic Nervous System</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D050781">Transcranial Magnetic Stimulation</DescriptorName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>2006</Year><Month>5</Month><Day>25</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2007</Year><Month>2</Month><Day>3</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2006</Year><Month>5</Month><Day>25</Day><Hour>9</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="doi">10.1002/mds.20966</ArticleId><ArticleId IdType="pubmed">16721731</ArticleId></ArticleIdList></PubmedData></pubmed><affiliations><list><country><li>France</li></country><settlement><li>Créteil</li></settlement></list><tree><noCountry><name sortKey="Bachoud Levi, Anne Catherine" sort="Bachoud Levi, Anne Catherine" uniqKey="Bachoud Levi A" first="Anne-Catherine" last="Bachoud-Lévi">Anne-Catherine Bachoud-Lévi</name><name sortKey="Baudic, Sophie" sort="Baudic, Sophie" uniqKey="Baudic S" first="Sophie" last="Baudic">Sophie Baudic</name><name sortKey="Cesaro, Pierre" sort="Cesaro, Pierre" uniqKey="Cesaro P" first="Pierre" last="Cesaro">Pierre Cesaro</name><name sortKey="Maison, Patrick" sort="Maison, Patrick" uniqKey="Maison P" first="Patrick" last="Maison">Patrick Maison</name><name sortKey="Menard Lefaucheur, Isabelle" sort="Menard Lefaucheur, Isabelle" uniqKey="Menard Lefaucheur I" first="Isabelle" last="Ménard-Lefaucheur">Isabelle Ménard-Lefaucheur</name><name sortKey="Peschanski, Marc" sort="Peschanski, Marc" uniqKey="Peschanski M" first="Marc" last="Peschanski">Marc Peschanski</name></noCountry><country name="France"><noRegion><name sortKey="Lefaucheur, Jean Pascal" sort="Lefaucheur, Jean Pascal" uniqKey="Lefaucheur J" first="Jean-Pascal" last="Lefaucheur">Jean-Pascal Lefaucheur</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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