Cortical myoclonus in huntington's disease associated with an enlarged somatosensory evoked potential
Identifieur interne : 005371 ( Main/Exploration ); précédent : 005370; suivant : 005372Cortical myoclonus in huntington's disease associated with an enlarged somatosensory evoked potential
Auteurs : Caviness [États-Unis] ; Mathias Kurth [États-Unis]Source :
- Movement Disorders [ 0885-3185 ] ; 1997-11.
English descriptors
- KwdEn :
- Adult, Brain Tissue Transplantation, Cerebral Cortex (pathology), Corpus Striatum (embryology), Corpus Striatum (transplantation), Electroencephalography, Electromyography, Electrophysiology, Evoked Potentials, Somatosensory, Fetal transplant surgery, Humans, Huntington Disease (complications), Huntington Disease (surgery), Huntington's disease, Male, Myoclonus, Myoclonus (complications), Myoclonus (pathology), Somatosensory evoked potential.
- MESH :
- complications : Huntington Disease, Myoclonus.
- embryology : Corpus Striatum.
- pathology : Cerebral Cortex, Myoclonus.
- surgery : Huntington Disease.
- transplantation : Corpus Striatum.
- Adult, Brain Tissue Transplantation, Electroencephalography, Electromyography, Evoked Potentials, Somatosensory, Humans, Male.
Abstract
We report the electrophysiologic findings of myoclonus in a patient with Huntington's disease (HD). This patient was studied postoperatively after a bilateral fetal cell transplant in his striatum. Incomplete transient improvement was seen in the myoclonus, followed by gradual deterioration. The myoclonus itself had a cortical correlate and was associated with an enlarged somatosensory evoked potential (SEP), consistent with the presence of cortical reflex myoclonus. An enlarged SEP has not been previously reported in myoclonus associated with HD. The postulated mechanisms for myoclonus, when it occurs in HD, have differed in the literature. The reason for the transient improvement of the myoclonus following transplantation is unclear, but this case raises the possibility that basal ganglia circuits may modulate cortical myoclonic activity.
Url:
DOI: 10.1002/mds.870120633
Affiliations:
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Le document en format XML
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<term>Corpus Striatum (transplantation)</term>
<term>Electroencephalography</term>
<term>Electromyography</term>
<term>Electrophysiology</term>
<term>Evoked Potentials, Somatosensory</term>
<term>Fetal transplant surgery</term>
<term>Humans</term>
<term>Huntington Disease (complications)</term>
<term>Huntington Disease (surgery)</term>
<term>Huntington's disease</term>
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<term>Myoclonus</term>
<term>Myoclonus (complications)</term>
<term>Myoclonus (pathology)</term>
<term>Somatosensory evoked potential</term>
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<term>Myoclonus</term>
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<front><div type="abstract" xml:lang="en">We report the electrophysiologic findings of myoclonus in a patient with Huntington's disease (HD). This patient was studied postoperatively after a bilateral fetal cell transplant in his striatum. Incomplete transient improvement was seen in the myoclonus, followed by gradual deterioration. The myoclonus itself had a cortical correlate and was associated with an enlarged somatosensory evoked potential (SEP), consistent with the presence of cortical reflex myoclonus. An enlarged SEP has not been previously reported in myoclonus associated with HD. The postulated mechanisms for myoclonus, when it occurs in HD, have differed in the literature. The reason for the transient improvement of the myoclonus following transplantation is unclear, but this case raises the possibility that basal ganglia circuits may modulate cortical myoclonic activity.</div>
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