• Ji Hee Kim, Young Cheol Na, Won Hee Lee, Won Seok Chang, Hyun Ho Jung, and Jin Woo Chang.
• Department of Neurosurgery, Yonsei University College of Medicine, Seoul, Republic of Korea.
• Neuromodulation. 2014 Dec 1;17(8):724-8.

ObjectiveMyoclonus-dystonia is a movement disorder characterized by childhood onset of myoclonus and dystonia. We report a case of the epsilon-sarcoglycan mutation-negative myoclonus-dystonia patient who underwent bilateral globus pallidus interna deep-brain stimulation with subsequent improvement of both myoclonus and dystonia.Case ReportA 37-year-old woman with myoclonic jerks and dystonia affecting predominantly the lower limbs was treated with chronic bilateral globus pallidus interna deep-brain stimulation.ResultsThe movement subscore of the Burke-Fahns-Marsden Dystonia Rating Scale was 38 before surgery and improved to 7 after 3 years. The disability subscore of the Burke-Fahns-Marsden Dystonia Rating Scale improved from 7 to 2. The Unified Myoclonus Rating Scale also decreased significantly from 93 to 39. No hardware- or stimulation-related complications occurred during follow-up.ConclusionThis report suggests that patients with myoclonus-dystonia may significantly benefit from bilateral globus pallidus interna deep-brain stimulation. Larger studies of this patient population are needed to confirm the optimal target.© 2014 International Neuromodulation Society.

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