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Cortical myoclonus in huntington's disease associated with an enlarged somatosensory evoked potential

✍ Scribed by Dr. John N Caviness; Mathias Kurth

Publisher: John Wiley and Sons
Year: 1997
Tongue: English
Weight: 436 KB
Volume: 12
Category: Article
ISSN: 0885-3185
DOI: 10.1002/mds.870120633

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✦ Synopsis

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.

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