Abnormal conduction in corticospinal pathways in Wilson's disease: investigation of nine cases with magnetic brain stimulation.
Identifieur interne : 004E56 ( PubMed/Curation ); précédent : 004E55; suivant : 004E57Abnormal conduction in corticospinal pathways in Wilson's disease: investigation of nine cases with magnetic brain stimulation.
Auteurs : B U Meyer [Allemagne] ; T C Britton ; C. Bischoff ; J. Machetanz ; R. Benecke ; B. ConradSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 1991.
English descriptors
- KwdEn :
- Adult, Electromagnetic Fields, Electromyography, Follow-Up Studies, Hepatolenticular Degeneration (diagnosis), Hepatolenticular Degeneration (drug therapy), Hepatolenticular Degeneration (physiopathology), Humans, Motor Cortex (drug effects), Motor Cortex (physiopathology), Muscle Contraction (drug effects), Muscle Contraction (physiology), Muscles (innervation), Neurologic Examination, Penicillamine (therapeutic use), Pyramidal Tracts (drug effects), Pyramidal Tracts (physiopathology), Synaptic Transmission (drug effects), Synaptic Transmission (physiology).
- MESH :
- chemical , therapeutic use : Penicillamine.
- diagnosis : Hepatolenticular Degeneration.
- drug effects : Motor Cortex, Muscle Contraction, Pyramidal Tracts, Synaptic Transmission.
- drug therapy : Hepatolenticular Degeneration.
- innervation : Muscles.
- physiology : Muscle Contraction, Synaptic Transmission.
- physiopathology : Hepatolenticular Degeneration, Motor Cortex, Pyramidal Tracts.
- Adult, Electromagnetic Fields, Electromyography, Follow-Up Studies, Humans, Neurologic Examination.
Abstract
Electromyographic (EMG) responses evoked by transcranial magnetic brain stimulation were studied in nine patients with Wilson's disease (WD). Six of the nine patients had prolonged central motor latencies (CMLs), reduced amplitude, or absent responses in at least one of the examined muscles. In one patient, abnormal EMG responses normalized following treatment with penicillamine. Pathophysiologically abnormal EMG responses might result from a potentially reversible impairment of corticomotoneuronal pathways and/or a reduced excitability of motoneurons due to basal ganglia dysfunction. The possible pathophysiological mechanisms are discussed.
DOI: 10.1002/mds.870060409
PubMed: 1661845
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<author><name sortKey="Meyer, B U" sort="Meyer, B U" uniqKey="Meyer B" first="B U" last="Meyer">B U Meyer</name>
<affiliation wicri:level="1"><nlm:affiliation>Department of Neurology, Technical University, Munich, F.R.G.</nlm:affiliation>
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<author><name sortKey="Britton, T C" sort="Britton, T C" uniqKey="Britton T" first="T C" last="Britton">T C Britton</name>
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<author><name sortKey="Bischoff, C" sort="Bischoff, C" uniqKey="Bischoff C" first="C" last="Bischoff">C. Bischoff</name>
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<author><name sortKey="Machetanz, J" sort="Machetanz, J" uniqKey="Machetanz J" first="J" last="Machetanz">J. Machetanz</name>
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<author><name sortKey="Benecke, R" sort="Benecke, R" uniqKey="Benecke R" first="R" last="Benecke">R. Benecke</name>
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<author><name sortKey="Conrad, B" sort="Conrad, B" uniqKey="Conrad B" first="B" last="Conrad">B. Conrad</name>
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<sourceDesc><biblStruct><analytic><title xml:lang="en">Abnormal conduction in corticospinal pathways in Wilson's disease: investigation of nine cases with magnetic brain stimulation.</title>
<author><name sortKey="Meyer, B U" sort="Meyer, B U" uniqKey="Meyer B" first="B U" last="Meyer">B U Meyer</name>
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<term>Electromagnetic Fields</term>
<term>Electromyography</term>
<term>Follow-Up Studies</term>
<term>Hepatolenticular Degeneration (diagnosis)</term>
<term>Hepatolenticular Degeneration (drug therapy)</term>
<term>Hepatolenticular Degeneration (physiopathology)</term>
<term>Humans</term>
<term>Motor Cortex (drug effects)</term>
<term>Motor Cortex (physiopathology)</term>
<term>Muscle Contraction (drug effects)</term>
<term>Muscle Contraction (physiology)</term>
<term>Muscles (innervation)</term>
<term>Neurologic Examination</term>
<term>Penicillamine (therapeutic use)</term>
<term>Pyramidal Tracts (drug effects)</term>
<term>Pyramidal Tracts (physiopathology)</term>
<term>Synaptic Transmission (drug effects)</term>
<term>Synaptic Transmission (physiology)</term>
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<term>Synaptic Transmission</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Hepatolenticular Degeneration</term>
<term>Motor Cortex</term>
<term>Pyramidal Tracts</term>
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<term>Electromagnetic Fields</term>
<term>Electromyography</term>
<term>Follow-Up Studies</term>
<term>Humans</term>
<term>Neurologic Examination</term>
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<front><div type="abstract" xml:lang="en">Electromyographic (EMG) responses evoked by transcranial magnetic brain stimulation were studied in nine patients with Wilson's disease (WD). Six of the nine patients had prolonged central motor latencies (CMLs), reduced amplitude, or absent responses in at least one of the examined muscles. In one patient, abnormal EMG responses normalized following treatment with penicillamine. Pathophysiologically abnormal EMG responses might result from a potentially reversible impairment of corticomotoneuronal pathways and/or a reduced excitability of motoneurons due to basal ganglia dysfunction. The possible pathophysiological mechanisms are discussed.</div>
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<Abstract><AbstractText>Electromyographic (EMG) responses evoked by transcranial magnetic brain stimulation were studied in nine patients with Wilson's disease (WD). Six of the nine patients had prolonged central motor latencies (CMLs), reduced amplitude, or absent responses in at least one of the examined muscles. In one patient, abnormal EMG responses normalized following treatment with penicillamine. Pathophysiologically abnormal EMG responses might result from a potentially reversible impairment of corticomotoneuronal pathways and/or a reduced excitability of motoneurons due to basal ganglia dysfunction. The possible pathophysiological mechanisms are discussed.</AbstractText>
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