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Myoclonus and tremor response to thalamic deep brain stimulation parameters in a patient with inherited myoclonus-dystonia syndrome.

Abstract
We present a 74-year-old woman with inherited myoclonus-dystonia, with predominant myoclonus and a novel mutation in the epsilon-sarcoglycan gene. The patient reports a life-long history of rapid, jerking movements, most severe in the upper extremities as well as a postural and action tremor. Bilateral deep brain stimulation (DBS) of the ventral intermediate nucleus of the thalamus was performed, and the patient demonstrated moderate clinical improvement in myoclonus. We studied the effects on myoclonus and tremor of varying DBS frequency and amplitude. The frequency tuning curve for myoclonus was similar to that of tremor, suggesting similar mechanisms by which DBS alleviates both disorders.
AuthorsAlexis M Kuncel, Dennis A Turner, Laurie J Ozelius, Paul E Greene, Warren M Grill, Mark A Stacy
JournalClinical neurology and neurosurgery (Clin Neurol Neurosurg) Vol. 111 Issue 3 Pg. 303-6 (Apr 2009) ISSN: 1872-6968 [Electronic] Netherlands
PMID19081669 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural)
Chemical References
  • Sarcoglycans
Topics
  • Aged
  • Deep Brain Stimulation (methods)
  • Dystonia (genetics, physiopathology, therapy)
  • Female
  • Humans
  • Mutation
  • Myoclonus (genetics, physiopathology, therapy)
  • Sarcoglycans (genetics)
  • Syndrome
  • Thalamus (physiopathology, surgery)
  • Treatment Outcome
  • Tremor (physiopathology, therapy)

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