Clinical relevance of electrophysiological tests in the assessment of patients with Huntington's disease
Identifieur interne : 000493 ( France/Analysis ); précédent : 000492; suivant : 000494Clinical relevance of electrophysiological tests in the assessment of patients with Huntington's disease
Auteurs : Jean-Pascal Lefaucheur [France] ; Anne-Catherine Bachoud-Levi [France] ; Catherine Bourdet [France] ; Thierry Grandmougin [France] ; Philippe Hantraye [France] ; Pierre Cesaro [France] ; Jean-Denis Degos [France] ; Marc Peschanski [France] ; Fabrice Lisovoski [France]Source :
- Movement Disorders [ 0885-3185 ] ; 2002-11.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
- Adult, Blinking (physiology), Cerebral Cortex (physiopathology), Diagnosis, Disease Progression, Electric Stimulation, Electrodiagnosis, Electromagnetic Fields, Electromyography, Electrophysiology, Evoked Potentials, Somatosensory (physiology), Female, Follow-Up Studies, Galvanic Skin Response (physiology), Human, Humans, Huntington Disease (diagnosis), Huntington Disease (genetics), Huntington Disease (physiopathology), Huntington disease, Huntington's disease, Hypokinesia (diagnosis), Hypokinesia (physiopathology), Male, Middle Aged, Performance evaluation, Reaction Time (physiology), Reflex, Stretch (physiology), Sensitivity and Specificity, Sympathetic Nervous System (physiopathology), clinical neurophysiology, movement disorders.
- 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. © 2002 Movement Disorder Society
Url:
DOI: 10.1002/mds.10273
Affiliations:
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uniqKey="Cesaro P" first="Pierre" last="Cesaro">Pierre Cesaro</name><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>Institut National de la Santé et de la Recherche Médicale U421/IM3, Faculté de Medecine, 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 Neurologie, Centre Hospitalier Universitaire Henri Mondor, Créteil</wicri:regionArea><placeName><settlement type="city">Créteil</settlement></placeName></affiliation></author><author><name sortKey="Degos, Jean Enis" sort="Degos, Jean Enis" uniqKey="Degos J" first="Jean-Denis" last="Degos">Jean-Denis Degos</name><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>Institut National de la Santé et de la Recherche Médicale U421/IM3, Faculté de Medecine, Créteil</wicri:regionArea><placeName><settlement 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xml:lang="fr">France</country><wicri:regionArea>Institut National de la Santé et de la Recherche Médicale U421/IM3, Faculté de Medecine, Créteil</wicri:regionArea><placeName><settlement type="city">Créteil</settlement></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="sub">Official Journal of the Movement Disorder Society</title><title level="j" type="abbrev">Mov. 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>New York</pubPlace><date type="published" when="2002-11">2002-11</date><biblScope unit="vol">17</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="1294">1294</biblScope><biblScope unit="page" to="1301">1301</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">A42C8C04233B0A65B9F795B814B0F3206C908C65</idno><idno type="DOI">10.1002/mds.10273</idno><idno type="ArticleID">MDS10273</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>Diagnosis</term><term>Disease Progression</term><term>Electric Stimulation</term><term>Electrodiagnosis</term><term>Electromagnetic Fields</term><term>Electromyography</term><term>Electrophysiology</term><term>Evoked Potentials, Somatosensory (physiology)</term><term>Female</term><term>Follow-Up Studies</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>Hypokinesia (diagnosis)</term><term>Hypokinesia (physiopathology)</term><term>Male</term><term>Middle Aged</term><term>Performance evaluation</term><term>Reaction Time (physiology)</term><term>Reflex, Stretch (physiology)</term><term>Sensitivity and Specificity</term><term>Sympathetic Nervous System (physiopathology)</term><term>clinical neurophysiology</term><term>movement disorders</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><keywords scheme="Pascal" xml:lang="fr"><term>Chorée Huntington</term><term>Diagnostic</term><term>Electrodiagnostic</term><term>Electrophysiologie</term><term>Evaluation performance</term><term>Homme</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">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. © 2002 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-Levi">Anne-Catherine Bachoud-Levi</name><name sortKey="Bachoud Evi, Anne Atherine" sort="Bachoud Evi, Anne Atherine" uniqKey="Bachoud Evi A" first="Anne-Catherine" last="Bachoud-Levi">Anne-Catherine Bachoud-Levi</name><name sortKey="Bourdet, Catherine" sort="Bourdet, Catherine" uniqKey="Bourdet C" first="Catherine" last="Bourdet">Catherine Bourdet</name><name sortKey="Bourdet, Catherine" sort="Bourdet, Catherine" uniqKey="Bourdet C" first="Catherine" last="Bourdet">Catherine Bourdet</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="Degos, Jean Enis" sort="Degos, Jean Enis" uniqKey="Degos J" first="Jean-Denis" last="Degos">Jean-Denis Degos</name><name sortKey="Degos, Jean Enis" sort="Degos, Jean Enis" uniqKey="Degos J" first="Jean-Denis" last="Degos">Jean-Denis Degos</name><name sortKey="Grandmougin, Thierry" sort="Grandmougin, Thierry" uniqKey="Grandmougin T" first="Thierry" last="Grandmougin">Thierry Grandmougin</name><name sortKey="Grandmougin, Thierry" sort="Grandmougin, Thierry" uniqKey="Grandmougin T" first="Thierry" last="Grandmougin">Thierry Grandmougin</name><name sortKey="Hantraye, Philippe" sort="Hantraye, Philippe" uniqKey="Hantraye P" first="Philippe" last="Hantraye">Philippe Hantraye</name><name sortKey="Lefaucheur, Jean Ascal" sort="Lefaucheur, Jean Ascal" uniqKey="Lefaucheur J" first="Jean-Pascal" last="Lefaucheur">Jean-Pascal Lefaucheur</name><name sortKey="Lisovoski, Fabrice" sort="Lisovoski, Fabrice" uniqKey="Lisovoski F" first="Fabrice" last="Lisovoski">Fabrice Lisovoski</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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