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

被引:47
作者
Kuncel, Alexis M. [1 ]
Turner, Dennis A. [2 ]
Ozelius, Laurie J. [3 ]
Greene, Paul E. [4 ]
Grill, Warren M. [1 ]
Stacy, Mark A. [5 ]
机构
[1] Duke Univ, Dept Biomed Engn, Durham, NC 27708 USA
[2] Duke Univ, Dept Neurobiol, Durham, NC USA
[3] Mt Sinai Sch Med, Dept Genet & Genom Sci, New York, NY USA
[4] Columbia Univ, Dept Neurol, New York, NY USA
[5] Duke Univ, Dept Neurol, Durham, NC USA
关键词
Stimulation frequency; Stimulation amplitude; Epsilon-sarcoglycan gene; Thalamus; Movement disorders; NUCLEUS; NEUROSTIMULATION; FREQUENCY; MUTATIONS;
D O I
10.1016/j.clineuro.2008.10.015
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:303 / 306
页数:4
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