Journal
CLINICAL NEUROLOGY AND NEUROSURGERY
Volume 111, Issue 3, Pages 303-306Publisher
ELSEVIER
DOI: 10.1016/j.clineuro.2008.10.015
Keywords
Stimulation frequency; Stimulation amplitude; Epsilon-sarcoglycan gene; Thalamus; Movement disorders
Categories
Funding
- NIH [R01-NS40894]
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We present a 74-year-old woman with inherited myoclonus-dystonia, with predominant myoclonus and a novel mutation in the E-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. (C) 2008 Elsevier B.V. All rights reserved.
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