Bilateral globus pallidus interna deep-brain stimulation in a patient with myoclonus-dystonia: a case report.

Abstract

OBJECTIVE: Myoclonus-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 REPORT: A 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. RESULTS: The 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. CONCLUSION: This 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.