Involvement of the medial pallidum in focal myoclonic dystonia: A clinical and neurophysiological case study
Identifieur interne : 004417 ( Main/Exploration ); précédent : 004416; suivant : 004418Involvement of the medial pallidum in focal myoclonic dystonia: A clinical and neurophysiological case study
Auteurs : Xuguang Liu [Royaume-Uni] ; Ivan C. Griffin [Royaume-Uni] ; Simon G. Parkin [Royaume-Uni] ; R. Christopher Miall [Royaume-Uni] ; Jeremy G. Rowe [Royaume-Uni] ; Ralph P. Gregory [Royaume-Uni] ; Richard B. Scott [Royaume-Uni] ; Tipu Z. Aziz [Royaume-Uni] ; John F. Stein [Royaume-Uni]Source :
- Movement Disorders [ 0885-3185 ] ; 2002-03.
English descriptors
- KwdEn :
- Adult, Dominance, Cerebral (physiology), Dystonic Disorders (diagnosis), Dystonic Disorders (genetics), Dystonic Disorders (physiopathology), Dystonic Disorders (therapy), Electric Stimulation Therapy, Electrodes, Implanted, Electroencephalography, Electromyography, Globus Pallidus (physiopathology), Humans, Male, Neck Muscles (innervation), basal ganglia, focal myoclonic dystonia, medial pallidum.
- MESH :
- diagnosis : Dystonic Disorders.
- genetics : Dystonic Disorders.
- innervation : Neck Muscles.
- physiology : Dominance, Cerebral.
- physiopathology : Dystonic Disorders, Globus Pallidus.
- therapy : Dystonic Disorders.
- Adult, Electric Stimulation Therapy, Electrodes, Implanted, Electroencephalography, Electromyography, Humans, Male.
Abstract
We successfully treated a patient with familial myoclonic dystonia (FMD), which primarily affected his neck muscles, with bilateral deep brain stimulation (DBS) to the medial pallidum, and investigated the role of the medial pallidum in FMD. A patient with FMD underwent bilateral implantation of DBS electrodes during which field potentials (FPs) in the medial pallidum and electromyograms (EMGs) from the affected neck muscles were recorded. The effects of high‐frequency DBS to the medial pallidum on the FMD were also assessed by recording EMGs during and immediately after implantation, as well as 6 days and 8 weeks postoperatively. During spontaneous myoclonic episodes, increased FPs oscillating at 4 and 8 Hz were recorded from the medial pallidum; these correlated strongly with phasic EMG activity at the same frequencies in the contralateral affected muscles. The EMG activity was suppressed by stimulating the contralateral medial pallidum at 100 Hz during the operation and continuous bilateral DBS from an implanted stimulator abolished myoclonic activity even more effectively postoperatively. The phasic pallidal activity correlated with and led the myoclonic muscle activity, and the myoclonus was suppressed by bilateral pallidal DBS, suggesting that the medial pallidum was involved in the generation of the myoclonic activity. High‐frequency DBS may suppress the myoclonus by desynchronising abnormal pallidal oscillations. This case study has significant clinical implications, because at present, there is no effective treatment for focal myoclonic dystonia. © 2002 Movement Disorder Society.
Url:
DOI: 10.1002/mds.10038
Affiliations:
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Griffin</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University Laboratory of Physiology, Parks Road, Oxford</wicri:regionArea><placeName><settlement type="city">Oxford</settlement><region type="country">Angleterre</region><region type="comté" nuts="2">Oxfordshire</region></placeName></affiliation></author><author><name sortKey="Parkin, Simon G" sort="Parkin, Simon G" uniqKey="Parkin S" first="Simon G." last="Parkin">Simon G. Parkin</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Neurosurgery, Radcliffe Infirmary, Oxford</wicri:regionArea><placeName><settlement type="city">Oxford</settlement><region type="country">Angleterre</region><region type="comté" nuts="2">Oxfordshire</region></placeName></affiliation></author><author><name sortKey="Miall, R Christopher" sort="Miall, R Christopher" uniqKey="Miall R" first="R. Christopher" last="Miall">R. Christopher Miall</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University Laboratory of Physiology, Parks Road, Oxford</wicri:regionArea><placeName><settlement type="city">Oxford</settlement><region type="country">Angleterre</region><region type="comté" nuts="2">Oxfordshire</region></placeName></affiliation></author><author><name sortKey="Rowe, Jeremy G" sort="Rowe, Jeremy G" uniqKey="Rowe J" first="Jeremy G." last="Rowe">Jeremy G. Rowe</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Neurosurgery, Radcliffe Infirmary, Oxford</wicri:regionArea><placeName><settlement type="city">Oxford</settlement><region type="country">Angleterre</region><region type="comté" nuts="2">Oxfordshire</region></placeName></affiliation></author><author><name sortKey="Gregory, Ralph P" sort="Gregory, Ralph P" uniqKey="Gregory R" first="Ralph P." last="Gregory">Ralph P. 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Aziz</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University Laboratory of Physiology, Parks Road, Oxford</wicri:regionArea><placeName><settlement type="city">Oxford</settlement><region type="country">Angleterre</region><region type="comté" nuts="2">Oxfordshire</region></placeName></affiliation><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Department of Neurosurgery, Radcliffe Infirmary, Oxford</wicri:regionArea><placeName><settlement type="city">Oxford</settlement><region type="country">Angleterre</region><region type="comté" nuts="2">Oxfordshire</region></placeName></affiliation></author><author><name sortKey="Stein, John F" sort="Stein, John F" uniqKey="Stein J" first="John F." last="Stein">John F. Stein</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>University Laboratory of Physiology, Parks Road, Oxford</wicri:regionArea><placeName><settlement type="city">Oxford</settlement><region type="country">Angleterre</region><region type="comté" nuts="2">Oxfordshire</region></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="2002-03">2002-03</date><biblScope unit="vol">17</biblScope><biblScope unit="issue">2</biblScope><biblScope unit="page" from="346">346</biblScope><biblScope unit="page" to="353">353</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">6B1A2066ACADCFA0A5C85419E91D2E206C01AE9C</idno><idno type="DOI">10.1002/mds.10038</idno><idno type="ArticleID">MDS10038</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Dominance, Cerebral (physiology)</term><term>Dystonic Disorders (diagnosis)</term><term>Dystonic Disorders (genetics)</term><term>Dystonic Disorders (physiopathology)</term><term>Dystonic Disorders (therapy)</term><term>Electric Stimulation Therapy</term><term>Electrodes, Implanted</term><term>Electroencephalography</term><term>Electromyography</term><term>Globus Pallidus (physiopathology)</term><term>Humans</term><term>Male</term><term>Neck Muscles (innervation)</term><term>basal ganglia</term><term>focal myoclonic dystonia</term><term>medial pallidum</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Dystonic Disorders</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Dystonic Disorders</term></keywords><keywords scheme="MESH" qualifier="innervation" xml:lang="en"><term>Neck Muscles</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Dominance, Cerebral</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Dystonic Disorders</term><term>Globus Pallidus</term></keywords><keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Dystonic Disorders</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Electric Stimulation Therapy</term><term>Electrodes, Implanted</term><term>Electroencephalography</term><term>Electromyography</term><term>Humans</term><term>Male</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">We successfully treated a patient with familial myoclonic dystonia (FMD), which primarily affected his neck muscles, with bilateral deep brain stimulation (DBS) to the medial pallidum, and investigated the role of the medial pallidum in FMD. A patient with FMD underwent bilateral implantation of DBS electrodes during which field potentials (FPs) in the medial pallidum and electromyograms (EMGs) from the affected neck muscles were recorded. The effects of high‐frequency DBS to the medial pallidum on the FMD were also assessed by recording EMGs during and immediately after implantation, as well as 6 days and 8 weeks postoperatively. During spontaneous myoclonic episodes, increased FPs oscillating at 4 and 8 Hz were recorded from the medial pallidum; these correlated strongly with phasic EMG activity at the same frequencies in the contralateral affected muscles. The EMG activity was suppressed by stimulating the contralateral medial pallidum at 100 Hz during the operation and continuous bilateral DBS from an implanted stimulator abolished myoclonic activity even more effectively postoperatively. The phasic pallidal activity correlated with and led the myoclonic muscle activity, and the myoclonus was suppressed by bilateral pallidal DBS, suggesting that the medial pallidum was involved in the generation of the myoclonic activity. High‐frequency DBS may suppress the myoclonus by desynchronising abnormal pallidal oscillations. This case study has significant clinical implications, because at present, there is no effective treatment for focal myoclonic dystonia. © 2002 Movement Disorder Society.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country><region><li>Angleterre</li><li>Oxfordshire</li></region><settlement><li>Oxford</li></settlement></list><tree><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Liu, Xuguang" sort="Liu, Xuguang" uniqKey="Liu X" first="Xuguang" last="Liu">Xuguang Liu</name></region><name sortKey="Aziz, Tipu Z" sort="Aziz, Tipu Z" uniqKey="Aziz T" first="Tipu Z." last="Aziz">Tipu Z. Aziz</name><name sortKey="Aziz, Tipu Z" sort="Aziz, Tipu Z" uniqKey="Aziz T" first="Tipu Z." last="Aziz">Tipu Z. Aziz</name><name sortKey="Gregory, Ralph P" sort="Gregory, Ralph P" uniqKey="Gregory R" first="Ralph P." last="Gregory">Ralph P. Gregory</name><name sortKey="Griffin, Ivan C" sort="Griffin, Ivan C" uniqKey="Griffin I" first="Ivan C." last="Griffin">Ivan C. Griffin</name><name sortKey="Miall, R Christopher" sort="Miall, R Christopher" uniqKey="Miall R" first="R. Christopher" last="Miall">R. Christopher Miall</name><name sortKey="Parkin, Simon G" sort="Parkin, Simon G" uniqKey="Parkin S" first="Simon G." last="Parkin">Simon G. Parkin</name><name sortKey="Rowe, Jeremy G" sort="Rowe, Jeremy G" uniqKey="Rowe J" first="Jeremy G." last="Rowe">Jeremy G. Rowe</name><name sortKey="Scott, Richard B" sort="Scott, Richard B" uniqKey="Scott R" first="Richard B." last="Scott">Richard B. Scott</name><name sortKey="Stein, John F" sort="Stein, John F" uniqKey="Stein J" first="John F." last="Stein">John F. Stein</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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