Electrophysiological features of myoclonus‐dystonia
Identifieur interne : 002873 ( Main/Exploration ); précédent : 002872; suivant : 002874Electrophysiological features of myoclonus‐dystonia
Auteurs : Jie-Yuan Li [Canada, Taïwan] ; Danny I. Cunic [Canada] ; Guillermo Paradiso [Canada] ; Carolyn Gunraj [Canada] ; Pramod K. Pal [Canada] ; Anthony E. Lang [Canada] ; Robert Chen [Canada]Source :
- Movement Disorders [ 0885-3185 ] ; 2008-10-30.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
- Adult, Case-Control Studies, Dystonia, Dystonic Disorders (complications), Dystonic Disorders (genetics), Electric Stimulation, Electroencephalography (methods), Electromyography (methods), Electrophysiology (methods), Evoked Potentials, Somatosensory (physiology), Female, Humans, Male, Middle Aged, Molecular Chaperones (genetics), Myoclonus, Myoclonus (complications), Myoclonus (genetics), Nervous system diseases, Neural Conduction (physiology), Neural Inhibition (physiology), Physiology, Reaction Time (physiology), Reflex (physiology), Sarcoglycans (genetics), Transcranial Magnetic Stimulation (methods), Transcranial magnetic stimulation, cortical inhibition, dystonia, myoclonus, physiology, transcranial magnetic stimulation.
- MESH :
- chemical , genetics : Molecular Chaperones, Sarcoglycans.
- complications : Dystonic Disorders, Myoclonus.
- genetics : Dystonic Disorders, Myoclonus.
- methods : Electroencephalography, Electromyography, Electrophysiology, Transcranial Magnetic Stimulation.
- physiology : Evoked Potentials, Somatosensory, Neural Conduction, Neural Inhibition, Reaction Time, Reflex.
- Adult, Case-Control Studies, Electric Stimulation, Female, Humans, Male, Middle Aged.
Abstract
Inherited myoclonus‐dystonia (M‐D) is an autosomal dominant disorder characterized by myoclonus and dystonia that often improves with alcohol. To examine the electrophysiologic characteristics of M‐D, we studied 6 patients from 4 different families and 9 age‐matched healthy subjects. Neurophysiological studies performed include electromyography (EMG)‐electroencephalography (EEG) polygraphy, jerk‐locked back‐averaged EEG, somatosensory evoked potentials (SEP), long‐latency reflex (LLR) to median and digital nerve stimulation, and transcranial magnetic stimulation studies with short‐interval intracortical inhibition (SICI), intracortical facilitation (ICF), and long‐interval intracortical inhibition (LICI). All 6 patients showed myoclonus and dystonia on clinical examination and EMG testing. The EMG burst durations ranged from 30.4 to 750.6 milliseconds (mean, 101.5 milliseconds). Jerk‐locked back‐averaged EEG failed to reveal any preceding cortical correlates. Median nerve SEP revealed no giant potential. No patients had exaggerated LLR to median or digital nerve stimulation. There was no significant difference in SICI, ICF, and LICI between M‐D patients and normal subjects. Myoclonus in inherited M‐D is likely of subcortical origin. Normal intracortical inhibition and facilitation suggest that the GABAergic circuits in the motor cortex are largely intact and that the mechanisms of myoclonus and dystonia are different from those for cortical myoclonus and other dystonic disorders. © 2008 Movement Disorder Society
Url:
DOI: 10.1002/mds.22273
Affiliations:
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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="2008-10-30">2008-10-30</date><biblScope unit="vol">23</biblScope><biblScope unit="issue">14</biblScope><biblScope unit="page" from="2055">2055</biblScope><biblScope unit="page" to="2061">2061</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">6511E86CB7AC0819379EF8897FFD61B47E214AD7</idno><idno type="DOI">10.1002/mds.22273</idno><idno type="ArticleID">MDS22273</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Case-Control Studies</term><term>Dystonia</term><term>Dystonic Disorders (complications)</term><term>Dystonic Disorders (genetics)</term><term>Electric Stimulation</term><term>Electroencephalography (methods)</term><term>Electromyography (methods)</term><term>Electrophysiology (methods)</term><term>Evoked Potentials, Somatosensory (physiology)</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Molecular Chaperones (genetics)</term><term>Myoclonus</term><term>Myoclonus (complications)</term><term>Myoclonus (genetics)</term><term>Nervous system diseases</term><term>Neural Conduction (physiology)</term><term>Neural Inhibition (physiology)</term><term>Physiology</term><term>Reaction Time (physiology)</term><term>Reflex (physiology)</term><term>Sarcoglycans (genetics)</term><term>Transcranial Magnetic Stimulation (methods)</term><term>Transcranial magnetic stimulation</term><term>cortical inhibition</term><term>dystonia</term><term>myoclonus</term><term>physiology</term><term>transcranial magnetic stimulation</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Molecular Chaperones</term><term>Sarcoglycans</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Dystonic Disorders</term><term>Myoclonus</term></keywords><keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Dystonic Disorders</term><term>Myoclonus</term></keywords><keywords scheme="MESH" qualifier="methods" xml:lang="en"><term>Electroencephalography</term><term>Electromyography</term><term>Electrophysiology</term><term>Transcranial Magnetic Stimulation</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Evoked Potentials, Somatosensory</term><term>Neural Conduction</term><term>Neural Inhibition</term><term>Reaction Time</term><term>Reflex</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Case-Control Studies</term><term>Electric Stimulation</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Dystonie</term><term>Myoclonie</term><term>Pathologie du système nerveux</term><term>Physiologie</term><term>Stimulation magnétique transcrânienne</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Inherited myoclonus‐dystonia (M‐D) is an autosomal dominant disorder characterized by myoclonus and dystonia that often improves with alcohol. To examine the electrophysiologic characteristics of M‐D, we studied 6 patients from 4 different families and 9 age‐matched healthy subjects. Neurophysiological studies performed include electromyography (EMG)‐electroencephalography (EEG) polygraphy, jerk‐locked back‐averaged EEG, somatosensory evoked potentials (SEP), long‐latency reflex (LLR) to median and digital nerve stimulation, and transcranial magnetic stimulation studies with short‐interval intracortical inhibition (SICI), intracortical facilitation (ICF), and long‐interval intracortical inhibition (LICI). All 6 patients showed myoclonus and dystonia on clinical examination and EMG testing. The EMG burst durations ranged from 30.4 to 750.6 milliseconds (mean, 101.5 milliseconds). Jerk‐locked back‐averaged EEG failed to reveal any preceding cortical correlates. Median nerve SEP revealed no giant potential. No patients had exaggerated LLR to median or digital nerve stimulation. There was no significant difference in SICI, ICF, and LICI between M‐D patients and normal subjects. Myoclonus in inherited M‐D is likely of subcortical origin. Normal intracortical inhibition and facilitation suggest that the GABAergic circuits in the motor cortex are largely intact and that the mechanisms of myoclonus and dystonia are different from those for cortical myoclonus and other dystonic disorders. © 2008 Movement Disorder Society</div></front></TEI><affiliations><list><country><li>Canada</li><li>Taïwan</li></country><region><li>Ontario</li></region><settlement><li>Toronto</li></settlement><orgName><li>Université de Toronto</li></orgName></list><tree><country name="Canada"><region name="Ontario"><name sortKey="Li, Jie Uan" sort="Li, Jie Uan" uniqKey="Li J" first="Jie-Yuan" last="Li">Jie-Yuan Li</name></region><name sortKey="Chen, Robert" sort="Chen, Robert" uniqKey="Chen R" first="Robert" last="Chen">Robert Chen</name><name sortKey="Cunic, Danny I" sort="Cunic, Danny I" uniqKey="Cunic D" first="Danny I." last="Cunic">Danny I. Cunic</name><name sortKey="Gunraj, Carolyn" sort="Gunraj, Carolyn" uniqKey="Gunraj C" first="Carolyn" last="Gunraj">Carolyn Gunraj</name><name sortKey="Lang, Anthony E" sort="Lang, Anthony E" uniqKey="Lang A" first="Anthony E." last="Lang">Anthony E. Lang</name><name sortKey="Pal, Pramod K" sort="Pal, Pramod K" uniqKey="Pal P" first="Pramod K." last="Pal">Pramod K. Pal</name><name sortKey="Paradiso, Guillermo" sort="Paradiso, Guillermo" uniqKey="Paradiso G" first="Guillermo" last="Paradiso">Guillermo Paradiso</name></country><country name="Taïwan"><noRegion><name sortKey="Li, Jie Uan" sort="Li, Jie Uan" uniqKey="Li J" first="Jie-Yuan" last="Li">Jie-Yuan Li</name></noRegion><name sortKey="Li, Jie Uan" sort="Li, Jie Uan" uniqKey="Li J" first="Jie-Yuan" last="Li">Jie-Yuan Li</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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