title: Deep brain stimulation in DYT1 dystonia: a 10-year experience.
abstract: BACKGROUND
  Globus Pallidus Interna (GPi) deep brain stimulation (DBS) is an effective treatment for DYT1-associated dystonia, but long-term results are lacking.
  
  
  OBJECTIVE
  To evaluate the long-term effects of GPi DBS in patients with DYT1 dystonia.
  
  
  METHODS
  A retrospective chart review (cohort study) of 47 consecutive DYT1+ patients treated by a single surgical team over a 10-year period and followed for up to 96 months (mean, 46 months) was performed. Symptom severity was quantified with the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor (M) and disability (D) sub-scores.
  
  
  RESULTS
  As measured with the BFMDRS (M), symptom severity was reduced to less than 20% of baseline after 2 years of DBS therapy (P = .001). The disability scores were reduced to <30% of baseline (P = .001). Symptomatic improvement was durable throughout available follow-up. Sixty-one percent of patients had discontinued all dystonia-related medications at their last follow-up. Ninety-one percent had discontinued at least 1 class of medication. Infections requiring removal and later reimplantation of hardware occurred in 4 of 47 patients (8.5%). Hardware malfunction including lead fractures occurred in 4 of 47 cases (8.5%). Lead revision to address poor clinical response was performed in 2 of 92 implanted leads (2.2%).
  
  
  CONCLUSIONS
  GPi DBS is an effective therapy for DYT1-associated torsion dystonia. Statistically significant efficacy is maintained for up to 7 years. Neurologic complications are rare, but long-term hardware-related complications can be significant.
authors.affiliation: Department of Neurosurgery, Mount Sinai School of Medicine, New York, New York, USA.
authors.forename: Fedor
authors.initials: F
authors.lastname: Panov
authors.forename: Yakov
authors.initials: Y
authors.lastname: Gologorsky
authors.forename: Grayson
authors.initials: G
authors.lastname: Connors
authors.forename: Michele
authors.initials: M
authors.lastname: Tagliati
authors.forename: Joan
authors.initials: J
authors.lastname: Miravite
authors.forename: Ron L
authors.initials: RL
authors.lastname: Alterman
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D046690:Deep Brain Stimulation / Q000009:adverse effects* / Q000379:methods*; D004422:Dystonia Musculorum Deformans / Q000175:diagnosis* / Q000517:prevention & control*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000175:diagnosis* / Q000209:etiology*; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult