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

Publication ,  Journal Article
Kuncel, AM; Turner, DA; Ozelius, LJ; Greene, PE; Grill, WM; Stacy, MA
Published in: Clin Neurol Neurosurg
April 2009

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.

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Published In

Clin Neurol Neurosurg

DOI

EISSN

1872-6968

Publication Date

April 2009

Volume

111

Issue

3

Start / End Page

303 / 306

Location

Netherlands

Related Subject Headings

  • Tremor
  • Treatment Outcome
  • Thalamus
  • Syndrome
  • Sarcoglycans
  • Neurology & Neurosurgery
  • Myoclonus
  • Mutation
  • Humans
  • Female
 

Citation

APA
Chicago
ICMJE
MLA
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Kuncel, A. M., Turner, D. A., Ozelius, L. J., Greene, P. E., Grill, W. M., & Stacy, M. A. (2009). Myoclonus and tremor response to thalamic deep brain stimulation parameters in a patient with inherited myoclonus-dystonia syndrome. Clin Neurol Neurosurg, 111(3), 303–306. https://doi.org/10.1016/j.clineuro.2008.10.015
Kuncel, Alexis M., Dennis A. Turner, Laurie J. Ozelius, Paul E. Greene, Warren M. Grill, and Mark A. Stacy. “Myoclonus and tremor response to thalamic deep brain stimulation parameters in a patient with inherited myoclonus-dystonia syndrome.Clin Neurol Neurosurg 111, no. 3 (April 2009): 303–6. https://doi.org/10.1016/j.clineuro.2008.10.015.
Kuncel AM, Turner DA, Ozelius LJ, Greene PE, Grill WM, Stacy MA. Myoclonus and tremor response to thalamic deep brain stimulation parameters in a patient with inherited myoclonus-dystonia syndrome. Clin Neurol Neurosurg. 2009 Apr;111(3):303–6.
Kuncel, Alexis M., et al. “Myoclonus and tremor response to thalamic deep brain stimulation parameters in a patient with inherited myoclonus-dystonia syndrome.Clin Neurol Neurosurg, vol. 111, no. 3, Apr. 2009, pp. 303–06. Pubmed, doi:10.1016/j.clineuro.2008.10.015.
Kuncel AM, Turner DA, Ozelius LJ, Greene PE, Grill WM, Stacy MA. Myoclonus and tremor response to thalamic deep brain stimulation parameters in a patient with inherited myoclonus-dystonia syndrome. Clin Neurol Neurosurg. 2009 Apr;111(3):303–306.
Journal cover image

Published In

Clin Neurol Neurosurg

DOI

EISSN

1872-6968

Publication Date

April 2009

Volume

111

Issue

3

Start / End Page

303 / 306

Location

Netherlands

Related Subject Headings

  • Tremor
  • Treatment Outcome
  • Thalamus
  • Syndrome
  • Sarcoglycans
  • Neurology & Neurosurgery
  • Myoclonus
  • Mutation
  • Humans
  • Female