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

John N. Caviness, Mathias Kurth

Research output: Contribution to journalArticle

20 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1046-1051
Number of pages6
JournalMovement Disorders
Volume12
Issue number6
DOIs
StatePublished - Dec 2 1997

Keywords

  • Electrophysiology
  • Fetal transplant surgery
  • Huntington's disease
  • Myoclonus
  • Somatosensory evoked potential

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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