Electrophysiological deterioration over time in patients with huntington's disease
Identifieur interne : 004C08 ( Main/Merge ); précédent : 004C07; suivant : 004C09Electrophysiological deterioration over time in patients with huntington's disease
Auteurs : Jean-Pascal Lefaucheur [France] ; Isabelle Menard-Lefaucheur [France] ; Patrick Maison [France] ; Sophie Baudic [France] ; Pierre Cesaro [France] ; Marc Peschanski [France] ; Anne-Catherine Bachoud-Levi [France]Source :
- Movement disorders [ 0885-3185 ] ; 2006.
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- Pascal (Inist)
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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.
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aut.</sZ></inist:fA14><country>France</country><placeName><settlement type="city">Créteil</settlement></placeName></affiliation><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>INSERM U421, IM3, Faculté de Médecine</s1><s2>Créteil</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ></inist:fA14><country>France</country><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="Menard-Lefaucheur">Isabelle Menard-Lefaucheur</name><affiliation wicri:level="1"><inist:fA14 i1="01"><s1>Service de Physiologie - Explorations Fonctionnelles, Hôpital Henri Mondor, AP-HP</s1><s2>Créteil</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>2 aut.</sZ></inist:fA14><country>France</country><placeName><settlement type="city">Créteil</settlement></placeName></affiliation></author><author><name sortKey="Maison, Patrick" sort="Maison, Patrick" uniqKey="Maison P" first="Patrick" last="Maison">Patrick Maison</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>INSERM U421, IM3, Faculté de Médecine</s1><s2>Créteil</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ></inist:fA14><country>France</country><placeName><settlement type="city">Créteil</settlement></placeName></affiliation><affiliation wicri:level="1"><inist:fA14 i1="03"><s1>Service de Pharmacologie Clinique, Hôpital Henri Mondor, AP-HP</s1><s2>Créteil</s2><s3>FRA</s3><sZ>3 aut.</sZ></inist:fA14><country>France</country><placeName><settlement type="city">Créteil</settlement></placeName></affiliation></author><author><name sortKey="Baudic, Sophie" sort="Baudic, Sophie" uniqKey="Baudic S" first="Sophie" last="Baudic">Sophie Baudic</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>INSERM U421, IM3, Faculté de Médecine</s1><s2>Créteil</s2><s3>FRA</s3><sZ>1 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sortKey="Peschanski, Marc" sort="Peschanski, Marc" uniqKey="Peschanski M" first="Marc" last="Peschanski">Marc Peschanski</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>INSERM U421, IM3, Faculté de Médecine</s1><s2>Créteil</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ></inist:fA14><country>France</country><placeName><settlement type="city">Créteil</settlement></placeName></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><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>INSERM U421, IM3, Faculté de Médecine</s1><s2>Créteil</s2><s3>FRA</s3><sZ>1 aut.</sZ><sZ>3 aut.</sZ><sZ>4 aut.</sZ><sZ>5 aut.</sZ><sZ>6 aut.</sZ><sZ>7 aut.</sZ></inist:fA14><country>France</country><placeName><settlement type="city">Créteil</settlement></placeName></affiliation><affiliation wicri:level="1"><inist:fA14 i1="04"><s1>Service de Neurologie, Hôpital Henri Mondor, AP-HP</s1><s2>Créteil</s2><s3>FRA</s3><sZ>5 aut.</sZ><sZ>7 aut.</sZ></inist:fA14><country>France</country><placeName><settlement type="city">Créteil</settlement></placeName></affiliation></author></analytic><series><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno><imprint><date when="2006">2006</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Deterioration</term><term>Human</term><term>Huntington disease</term><term>Nervous system diseases</term><term>Palpebral reflex</term><term>Program</term><term>Silent period</term><term>Somatosensory evoked potential</term><term>Transcranial magnetic stimulation</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Système nerveux pathologie</term><term>Chorée Huntington</term><term>Détérioration</term><term>Homme</term><term>Programme</term><term>Réflexe palpébral</term><term>Potentiel évoqué somatosensoriel</term><term>Période muette</term><term>Stimulation magnétique transcrânienne</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</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><affiliations><list><country><li>France</li></country><settlement><li>Créteil</li></settlement></list><tree><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><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><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><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="Cesaro, Pierre" sort="Cesaro, Pierre" uniqKey="Cesaro P" first="Pierre" last="Cesaro">Pierre Cesaro</name><name sortKey="Lefaucheur, Jean Pascal" sort="Lefaucheur, Jean Pascal" uniqKey="Lefaucheur J" first="Jean-Pascal" last="Lefaucheur">Jean-Pascal Lefaucheur</name><name sortKey="Maison, Patrick" sort="Maison, Patrick" uniqKey="Maison P" first="Patrick" last="Maison">Patrick Maison</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="Menard-Lefaucheur">Isabelle Menard-Lefaucheur</name><name sortKey="Peschanski, Marc" sort="Peschanski, Marc" uniqKey="Peschanski M" first="Marc" last="Peschanski">Marc Peschanski</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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