Electrophysiological studies of early stage corticobasal degeneration.
Identifieur interne : 004E81 ( Ncbi/Curation ); précédent : 004E80; suivant : 004E82Electrophysiological studies of early stage corticobasal degeneration.
Auteurs : C S Lu [Taïwan] ; A. Ikeda ; K. Terada ; T. Mima ; T. Nagamine ; H. Fukuyama ; N. Kohara ; Y. Kojima ; Y. Yonekura ; R S Chen ; C H Tsai ; N S Chu ; J. Kimura ; H. ShibasakiSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 1998.
English descriptors
- KwdEn :
- Aged, Basal Ganglia Diseases (physiopathology), Basal Ganglia Diseases (radionuclide imaging), Electromyography, Evoked Potentials, Motor, Evoked Potentials, Somatosensory, Female, Humans, Male, Middle Aged, Motor Cortex (physiopathology), Motor Cortex (radionuclide imaging), Movement Disorders (physiopathology), Movement Disorders (radionuclide imaging), Neurodegenerative Diseases (physiopathology), Neurodegenerative Diseases (radionuclide imaging), Tomography, Emission-Computed, Single-Photon.
- MESH :
- physiopathology : Basal Ganglia Diseases, Motor Cortex, Movement Disorders, Neurodegenerative Diseases.
- radionuclide imaging : Basal Ganglia Diseases, Motor Cortex, Movement Disorders, Neurodegenerative Diseases.
- Aged, Electromyography, Evoked Potentials, Motor, Evoked Potentials, Somatosensory, Female, Humans, Male, Middle Aged, Tomography, Emission-Computed, Single-Photon.
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 from the sensory cortex.
DOI: 10.1002/mds.870130126
PubMed: 9452339
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pubmed:9452339Le document en format XML
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<author><name sortKey="Terada, K" sort="Terada, K" uniqKey="Terada K" first="K" last="Terada">K. Terada</name>
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<author><name sortKey="Chen, R S" sort="Chen, R S" uniqKey="Chen R" first="R S" last="Chen">R S Chen</name>
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<author><name sortKey="Tsai, C H" sort="Tsai, C H" uniqKey="Tsai C" first="C H" last="Tsai">C H Tsai</name>
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<author><name sortKey="Chu, N S" sort="Chu, N S" uniqKey="Chu N" first="N S" last="Chu">N S Chu</name>
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<series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title>
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<term>Electromyography</term>
<term>Evoked Potentials, Motor</term>
<term>Evoked Potentials, Somatosensory</term>
<term>Female</term>
<term>Humans</term>
<term>Male</term>
<term>Middle Aged</term>
<term>Motor Cortex (physiopathology)</term>
<term>Motor Cortex (radionuclide imaging)</term>
<term>Movement Disorders (physiopathology)</term>
<term>Movement Disorders (radionuclide imaging)</term>
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<term>Neurodegenerative Diseases (radionuclide imaging)</term>
<term>Tomography, Emission-Computed, Single-Photon</term>
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<term>Movement Disorders</term>
<term>Neurodegenerative Diseases</term>
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<term>Female</term>
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<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 from the sensory cortex.</div>
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