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

Ji Hee Kim, Young Cheol Na, Won Hee Lee, Won Seok Chang, Hyun Ho Jung, Jin Woo Chang

Research output: Contribution to journalArticle

8 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)724-728
Number of pages5
JournalNeuromodulation
Volume17
Issue number8
DOIs
Publication statusPublished - 2014 Dec 1

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology
  • Anesthesiology and Pain Medicine

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