Electrophysiological studies of early stage corticobasal degeneration
Identifieur interne : 003751 ( Istex/Checkpoint ); précédent : 003750; suivant : 003752Electrophysiological studies of early stage corticobasal degeneration
Auteurs : C. S. Lu [Taïwan] ; A. Ikeda [Japon] ; K. Terada [Japon] ; T. Mima [Japon] ; T. Nagamine [Japon] ; H. Fukuyama [Japon] ; N. Kohara [Japon] ; Y. Kojima [Japon] ; Y. Yonekura [Japon] ; R. S. Chen [Taïwan] ; C. H. Tsai [Taïwan] ; N. S. Chu [Taïwan] ; J. Kimura [Japon] ; Shibasaki [Japon]Source :
- Movement Disorders [ 0885-3185 ] ; 1998-01.
English descriptors
Abstract
We conducted electrophysiological studies in two Asian patients with probable corticobasal degeneration (CBD). The duration of illness from onset was 16 and 20 months, respectively. The clinical manifestations were markedly asymmetric and characterized by cortical sensory loss, apraxia, action myoclonus, action tremor, and akinetic‐rigid parkinsonism. Neither patient responded to levodopa therapy. Simple photon‐emission computed tomography (SPECT) study showed significantly decreased regional cerebral blood flow in the frontoparietal areas and thalamus opposite to the predominantly affected limb. A series of electrophysiological studies failed to identify giant somatosensory evoked potentials (SEPs), enhanced long latency electromyography (EMG) reflex, and cortical spikes preceding myoclonic jerk. However, the earliest cortical component of the median nerve SEP was exclusively enlarged in one patient and preserved with depression of the subsequent components in the other patient. Significantly shorter postmotor‐evoked potential (MEP) silent period was found after the transcranial magnetic stimulation of the motor cortex in both patients. CBD is a unique clinical entity characterized by action myoclonus probably the result of the pathologic hyperexcitability of the motor cortex, based on a loss of inhibitory input form the sensory cortex.
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DOI: 10.1002/mds.870130126
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Kohara</name></author><author><name sortKey="Kojima, Y" sort="Kojima, Y" uniqKey="Kojima Y" first="Y." last="Kojima">Y. Kojima</name></author><author><name sortKey="Yonekura, Y" sort="Yonekura, Y" uniqKey="Yonekura Y" first="Y." last="Yonekura">Y. Yonekura</name></author><author><name sortKey="Chen, R S" sort="Chen, R S" uniqKey="Chen R" first="R. S." last="Chen">R. S. Chen</name></author><author><name sortKey="Tsai, C H" sort="Tsai, C H" uniqKey="Tsai C" first="C. H." last="Tsai">C. H. Tsai</name></author><author><name sortKey="Chu, N S" sort="Chu, N S" uniqKey="Chu N" first="N. S." last="Chu">N. S. Chu</name></author><author><name sortKey="Kimura, J" sort="Kimura, J" uniqKey="Kimura J" first="J." last="Kimura">J. 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Lu</name><affiliation wicri:level="1"><country xml:lang="fr">Taïwan</country><wicri:regionArea>Department of Neurology, Chang Gung Medical College and Memorial Hospital, Taipei</wicri:regionArea><wicri:noRegion>Taipei</wicri:noRegion></affiliation></author><author><name sortKey="Ikeda, A" sort="Ikeda, A" uniqKey="Ikeda A" first="A." last="Ikeda">A. Ikeda</name><affiliation wicri:level="4"><country xml:lang="fr">Japon</country><wicri:regionArea>Departments of Brain Pathophysiology, Kyoto University, Faculty of Medicine, Shogoin, Sakyo‐ku, Kyoto</wicri:regionArea><orgName type="university">Université de Kyoto</orgName><placeName><settlement type="city">Kyoto</settlement><region type="prefecture">Région du Kansai</region></placeName></affiliation></author><author><name sortKey="Terada, K" sort="Terada, K" uniqKey="Terada K" first="K." last="Terada">K. Terada</name><affiliation wicri:level="4"><country xml:lang="fr">Japon</country><wicri:regionArea>Departments of Brain Pathophysiology, Kyoto University, Faculty of Medicine, Shogoin, Sakyo‐ku, Kyoto</wicri:regionArea><orgName type="university">Université de Kyoto</orgName><placeName><settlement type="city">Kyoto</settlement><region type="prefecture">Région du Kansai</region></placeName></affiliation></author><author><name sortKey="Mima, T" sort="Mima, T" uniqKey="Mima T" first="T." last="Mima">T. Mima</name><affiliation wicri:level="4"><country xml:lang="fr">Japon</country><wicri:regionArea>Departments of Brain Pathophysiology, Kyoto University, Faculty of Medicine, Shogoin, Sakyo‐ku, Kyoto</wicri:regionArea><orgName type="university">Université de Kyoto</orgName><placeName><settlement type="city">Kyoto</settlement><region type="prefecture">Région du Kansai</region></placeName></affiliation></author><author><name sortKey="Nagamine, T" sort="Nagamine, T" uniqKey="Nagamine T" first="T." last="Nagamine">T. Nagamine</name><affiliation wicri:level="4"><country xml:lang="fr">Japon</country><wicri:regionArea>Departments of Brain Pathophysiology, Kyoto University, Faculty of Medicine, Shogoin, Sakyo‐ku, Kyoto</wicri:regionArea><orgName type="university">Université de Kyoto</orgName><placeName><settlement type="city">Kyoto</settlement><region type="prefecture">Région du Kansai</region></placeName></affiliation></author><author><name sortKey="Fukuyama, H" sort="Fukuyama, H" uniqKey="Fukuyama H" first="H." last="Fukuyama">H. Fukuyama</name><affiliation wicri:level="4"><country xml:lang="fr">Japon</country><wicri:regionArea>Departments of Brain Pathophysiology, Kyoto University, Faculty of Medicine, Shogoin, Sakyo‐ku, Kyoto</wicri:regionArea><orgName type="university">Université de Kyoto</orgName><placeName><settlement type="city">Kyoto</settlement><region type="prefecture">Région du Kansai</region></placeName></affiliation></author><author><name sortKey="Kohara, N" sort="Kohara, N" uniqKey="Kohara N" first="N." last="Kohara">N. Kohara</name><affiliation wicri:level="4"><country xml:lang="fr">Japon</country><wicri:regionArea>Departments of Neurology, Kyoto University, Faculty of Medicine, Shogoin, Sakyo‐ku, Kyoto</wicri:regionArea><orgName type="university">Université de Kyoto</orgName><placeName><settlement type="city">Kyoto</settlement><region type="prefecture">Région du Kansai</region></placeName></affiliation></author><author><name sortKey="Kojima, Y" sort="Kojima, Y" uniqKey="Kojima Y" first="Y." last="Kojima">Y. Kojima</name><affiliation wicri:level="4"><country xml:lang="fr">Japon</country><wicri:regionArea>Departments of Neurology, Kyoto University, Faculty of Medicine, Shogoin, Sakyo‐ku, Kyoto</wicri:regionArea><orgName type="university">Université de Kyoto</orgName><placeName><settlement type="city">Kyoto</settlement><region type="prefecture">Région du Kansai</region></placeName></affiliation></author><author><name sortKey="Yonekura, Y" sort="Yonekura, Y" uniqKey="Yonekura Y" first="Y." last="Yonekura">Y. Yonekura</name><affiliation wicri:level="1"><country xml:lang="fr">Japon</country><wicri:regionArea>High Energy Medical Research Center, Fukui Medical School, Fukui</wicri:regionArea><wicri:noRegion>Fukui</wicri:noRegion></affiliation></author><author><name sortKey="Chen, R S" sort="Chen, R S" uniqKey="Chen R" first="R. S." last="Chen">R. S. Chen</name><affiliation wicri:level="1"><country xml:lang="fr">Taïwan</country><wicri:regionArea>Department of Neurology, Chang Gung Medical College and Memorial Hospital, Taipei</wicri:regionArea><wicri:noRegion>Taipei</wicri:noRegion></affiliation></author><author><name sortKey="Tsai, C H" sort="Tsai, C H" uniqKey="Tsai C" first="C. H." last="Tsai">C. H. Tsai</name><affiliation wicri:level="1"><country xml:lang="fr">Taïwan</country><wicri:regionArea>Department of Neurology, Chang Gung Medical College and Memorial Hospital, Taipei</wicri:regionArea><wicri:noRegion>Taipei</wicri:noRegion></affiliation></author><author><name sortKey="Chu, N S" sort="Chu, N S" uniqKey="Chu N" first="N. S." last="Chu">N. S. Chu</name><affiliation wicri:level="1"><country xml:lang="fr">Taïwan</country><wicri:regionArea>Department of Neurology, Chang Gung Medical College and Memorial Hospital, Taipei</wicri:regionArea><wicri:noRegion>Taipei</wicri:noRegion></affiliation></author><author><name sortKey="Kimura, J" sort="Kimura, J" uniqKey="Kimura J" first="J." last="Kimura">J. Kimura</name><affiliation wicri:level="4"><country xml:lang="fr">Japon</country><wicri:regionArea>Departments of Neurology, Kyoto University, Faculty of Medicine, Shogoin, Sakyo‐ku, Kyoto</wicri:regionArea><orgName type="university">Université de Kyoto</orgName><placeName><settlement type="city">Kyoto</settlement><region type="prefecture">Région du Kansai</region></placeName></affiliation></author><author><name sortKey="Shibasaki" sort="Shibasaki" uniqKey="Shibasaki" last="Shibasaki">Shibasaki</name><affiliation wicri:level="4"><country xml:lang="fr">Japon</country><wicri:regionArea>Departments of Brain Pathophysiology, Kyoto University, Faculty of Medicine, Shogoin, Sakyo‐ku, Kyoto</wicri:regionArea><orgName type="university">Université de Kyoto</orgName><placeName><settlement type="city">Kyoto</settlement><region type="prefecture">Région du Kansai</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>Hoboken</pubPlace><date type="published" when="1998-01">1998-01</date><biblScope unit="vol">13</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="140">140</biblScope><biblScope unit="page" to="146">146</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">50D8C2B0832A6AB1C96F96850090CDFCF8700710</idno><idno type="DOI">10.1002/mds.870130126</idno><idno type="ArticleID">MDS870130126</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Action myoclonus</term><term>Corticobasal degeneration</term><term>MEP</term><term>SPECT</term><term>electrophysiology</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">We conducted electrophysiological studies in two Asian patients with probable corticobasal degeneration (CBD). The duration of illness from onset was 16 and 20 months, respectively. The clinical manifestations were markedly asymmetric and characterized by cortical sensory loss, apraxia, action myoclonus, action tremor, and akinetic‐rigid parkinsonism. Neither patient responded to levodopa therapy. Simple photon‐emission computed tomography (SPECT) study showed significantly decreased regional cerebral blood flow in the frontoparietal areas and thalamus opposite to the predominantly affected limb. A series of electrophysiological studies failed to identify giant somatosensory evoked potentials (SEPs), enhanced long latency electromyography (EMG) reflex, and cortical spikes preceding myoclonic jerk. However, the earliest cortical component of the median nerve SEP was exclusively enlarged in one patient and preserved with depression of the subsequent components in the other patient. Significantly shorter postmotor‐evoked potential (MEP) silent period was found after the transcranial magnetic stimulation of the motor cortex in both patients. CBD is a unique clinical entity characterized by action myoclonus probably the result of the pathologic hyperexcitability of the motor cortex, based on a loss of inhibitory input form the sensory cortex.</div></front></TEI><affiliations><list><country><li>Japon</li><li>Taïwan</li></country><region><li>Région du Kansai</li></region><settlement><li>Kyoto</li></settlement><orgName><li>Université de Kyoto</li></orgName></list><tree><country name="Taïwan"><noRegion><name sortKey="Lu, C S" sort="Lu, C S" uniqKey="Lu C" first="C. S." last="Lu">C. S. Lu</name></noRegion><name sortKey="Chen, R S" sort="Chen, R S" uniqKey="Chen R" first="R. S." last="Chen">R. S. Chen</name><name sortKey="Chu, N S" sort="Chu, N S" uniqKey="Chu N" first="N. S." last="Chu">N. S. Chu</name><name sortKey="Tsai, C H" sort="Tsai, C H" uniqKey="Tsai C" first="C. H." last="Tsai">C. H. Tsai</name></country><country name="Japon"><region name="Région du Kansai"><name sortKey="Ikeda, A" sort="Ikeda, A" uniqKey="Ikeda A" first="A." last="Ikeda">A. Ikeda</name></region><name sortKey="Fukuyama, H" sort="Fukuyama, H" uniqKey="Fukuyama H" first="H." last="Fukuyama">H. Fukuyama</name><name sortKey="Kimura, J" sort="Kimura, J" uniqKey="Kimura J" first="J." last="Kimura">J. Kimura</name><name sortKey="Kohara, N" sort="Kohara, N" uniqKey="Kohara N" first="N." last="Kohara">N. Kohara</name><name sortKey="Kojima, Y" sort="Kojima, Y" uniqKey="Kojima Y" first="Y." last="Kojima">Y. Kojima</name><name sortKey="Mima, T" sort="Mima, T" uniqKey="Mima T" first="T." last="Mima">T. Mima</name><name sortKey="Nagamine, T" sort="Nagamine, T" uniqKey="Nagamine T" first="T." last="Nagamine">T. Nagamine</name><name sortKey="Shibasaki" sort="Shibasaki" uniqKey="Shibasaki" last="Shibasaki">Shibasaki</name><name sortKey="Terada, K" sort="Terada, K" uniqKey="Terada K" first="K." last="Terada">K. Terada</name><name sortKey="Yonekura, Y" sort="Yonekura, Y" uniqKey="Yonekura Y" first="Y." last="Yonekura">Y. Yonekura</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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