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Case Reports
. 2009 Apr;111(3):303-6.
doi: 10.1016/j.clineuro.2008.10.015. Epub 2008 Dec 10.

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

Alexis M Kuncel  1 , Dennis A Turner, Laurie J Ozelius, Paul E Greene, Warren M Grill, Mark A Stacy
Affiliations
Case Reports

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

Alexis M Kuncel et al. Clin Neurol Neurosurg. 2009 Apr.

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.

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Conflict of interest statement

Disclosure: The authors have reported no conflicts of interest.

Figures

Figure 1
Figure 1
Effect of DBS amplitude and frequency on myoclonic jerking and tremor. a–c) Raw accelerometry (left) and power spectrums (right) for a) DBS OFF, b) 130 Hz, 3.0 V, 60 μs stimulation, and c) 15 Hz, 1.5 V, 60 μs stimulation. Myoclonus (d–e) and tremor (f–g) as a function of stimulation frequency, averaged across stimulation amplitudes (1.5, 3.0, and 4.5 V). Standard deviations are shown. d, f) Myoclonus and tremor were quantified by summing the acceleration power over 0–3 Hz (black) and 3–6 Hz (gray), respectively. e, g) Myoclonus and tremor were rated by a clinician blinded to the stimulation condition. The myoclonus rating (0–16) was the product of the rating for myoclonus amplitude (0–4) and frequency (0–4) [17]. Tremor was rated on a 0–4 scale.
See this image and copyright information in PMC

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