Electrophysiological deterioration over time in patients with Huntington's disease
Identifieur interne : 000367 ( France/Analysis ); précédent : 000366; suivant : 000368Electrophysiological deterioration over time in patients with Huntington's disease
Auteurs : Jean-Pascal Lefaucheur [France] ; Isabelle Ménard-Lefaucheur [France] ; Patrick Maison [France] ; Sophie Baudic [France] ; Pierre Cesaro [France] ; Marc Peschanski [France] ; Anne-Catherine Bachoud-Lévi [France]Source :
- Movement Disorders [ 0885-3185 ] ; 2006-09.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
- Adult, Blinking (physiology), Cerebral Cortex (physiopathology), Deterioration, Disease Progression, Early Diagnosis, Electric Stimulation, Evoked Potentials, Somatosensory (physiology), Female, Galvanic Skin Response (physiology), Human, Humans, Huntington Disease (diagnosis), Huntington Disease (genetics), Huntington Disease (physiopathology), Huntington disease, Huntington's disease, Longitudinal Studies, Male, Median Nerve (physiopathology), Middle Aged, Nervous system diseases, Neurologic Examination, Palpebral reflex, Program, Reaction Time (physiology), Reference Values, Silent period, Somatosensory evoked potential, Sympathetic Nervous System (physiopathology), Transcranial Magnetic Stimulation, Transcranial magnetic stimulation, assessment program, blink reflex, cortical silent period, longitudinal study, long‐latency reflex, somatosensory evoked potentials, 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. © 2006 Movement Disorder Society
Url:
DOI: 10.1002/mds.20966
Affiliations:
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sortKey="Menard Efaucheur, Isabelle" sort="Menard Efaucheur, Isabelle" uniqKey="Menard Efaucheur I" first="Isabelle" last="Ménard-Lefaucheur">Isabelle Ménard-Lefaucheur</name><affiliation wicri:level="1"><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="Maison, Patrick" sort="Maison, Patrick" uniqKey="Maison P" first="Patrick" last="Maison">Patrick Maison</name><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>INSERM U421, IM3, Faculté de Médecine, Créteil</wicri:regionArea><placeName><settlement type="city">Créteil</settlement></placeName></affiliation><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>Service de Pharmacologie Clinique, Hôpital Henri Mondor, AP‐HP, 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Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2006-09">2006-09</date><biblScope unit="vol">21</biblScope><biblScope unit="issue">9</biblScope><biblScope unit="page" from="1350">1350</biblScope><biblScope unit="page" to="1354">1354</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">CFD67A1CDDAC24507857ADBC4F0358996BEF6D29</idno><idno type="DOI">10.1002/mds.20966</idno><idno type="ArticleID">MDS20966</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Blinking (physiology)</term><term>Cerebral Cortex (physiopathology)</term><term>Deterioration</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>Human</term><term>Humans</term><term>Huntington Disease (diagnosis)</term><term>Huntington Disease (genetics)</term><term>Huntington Disease (physiopathology)</term><term>Huntington disease</term><term>Huntington's disease</term><term>Longitudinal Studies</term><term>Male</term><term>Median Nerve (physiopathology)</term><term>Middle Aged</term><term>Nervous system diseases</term><term>Neurologic Examination</term><term>Palpebral reflex</term><term>Program</term><term>Reaction Time (physiology)</term><term>Reference Values</term><term>Silent period</term><term>Somatosensory evoked potential</term><term>Sympathetic Nervous System (physiopathology)</term><term>Transcranial Magnetic Stimulation</term><term>Transcranial magnetic stimulation</term><term>assessment program</term><term>blink reflex</term><term>cortical silent period</term><term>longitudinal study</term><term>long‐latency reflex</term><term>somatosensory evoked potentials</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><keywords scheme="Pascal" xml:lang="fr"><term>Chorée Huntington</term><term>Détérioration</term><term>Homme</term><term>Potentiel évoqué somatosensoriel</term><term>Programme</term><term>Période muette</term><term>Réflexe palpébral</term><term>Stimulation magnétique transcrânienne</term><term>Système nerveux pathologie</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></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. © 2006 Movement Disorder Society</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 Ascal" sort="Lefaucheur, Jean Ascal" uniqKey="Lefaucheur J" first="Jean-Pascal" last="Lefaucheur">Jean-Pascal Lefaucheur</name></noRegion><name sortKey="Bachoud Evi, Anne Atherine" sort="Bachoud Evi, Anne Atherine" uniqKey="Bachoud Evi A" first="Anne-Catherine" last="Bachoud-Lévi">Anne-Catherine Bachoud-Lévi</name><name sortKey="Bachoud Evi, Anne Atherine" sort="Bachoud Evi, Anne Atherine" uniqKey="Bachoud Evi 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="Cesaro, Pierre" sort="Cesaro, Pierre" uniqKey="Cesaro P" first="Pierre" last="Cesaro">Pierre Cesaro</name><name sortKey="Lefaucheur, Jean Ascal" sort="Lefaucheur, Jean Ascal" 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 Efaucheur, Isabelle" sort="Menard Efaucheur, Isabelle" uniqKey="Menard Efaucheur 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></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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