title: 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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Neurosurgery, Yonsei University College of Medicine, Seoul, Republic of Korea.
authors.forename: Ji Hee
authors.initials: JH
authors.lastname: Kim
authors.forename: Young Cheol
authors.initials: YC
authors.lastname: Na
authors.forename: Won Hee
authors.initials: WH
authors.lastname: Lee
authors.forename: Won Seok
authors.initials: WS
authors.lastname: Chang
authors.forename: Hyun Ho
authors.initials: HH
authors.lastname: Jung
authors.forename: Jin Woo
authors.initials: JW
authors.lastname: Chang
mesh_terms: D000328:Adult; D046690:Deep Brain Stimulation / Q000379:methods*; D004185:Disability Evaluation; D020821:Dystonic Disorders / Q000628:therapy*; D005260:Female; D007839:Functional Laterality / Q000502:physiology*; D005917:Globus Pallidus / Q000502:physiology*; D006801:Humans
keywords: Deep brain stimulation; dystonia; globus pallidus interna; myoclonus; myoclonus-dystonia