title: Bilateral subthalamic nucleus stimulation in the treatment of neurodegeneration with brain iron accumulation type 1.
abstract: BACKGROUND
  Neurodegeneration with brain iron accumulation type 1 (NBIA1), previously called Hallervorden-Spatz disease, is a rare neurodegenerative condition with abnormal brain iron accumulation. There have been some reports of deep brain stimulation (DBS) in the treatment of NBIA1. However, the target was usually the globus pallidus internus or thalamus.
  
  
  OBJECTIVE
  We present a case of NBIA1 in a 16-year-old male who was treated with bilateral subthalamic nucleus (STN)-DBS and explored its efficacy in the treatment of NBIA1. The patient presented with severe generalized dystonia and marked dysarthria and had previously had unsuccessful ablation surgery. MRI confirmed the diagnosis of NBIA1.
  
  
  RESULTS
  He had significant improvement postoperatively, and the benefit of surgery was maintained for 3 years during follow-up. Burke-Fahn-Marsden Dystonia Rating Scale score was 114/120 preoperatively, and dropped to 60/120 at 14 days, 35/120 at 1 month, 28/120 at 3 months, 14/120 at 1 year and 18/120 at 3 years postoperatively.
  
  
  CONCLUSIONS
  Our results suggest that bilateral STN-DBS might be considered as an effective treatment for selective NBIA1 patients.
authors.affiliation: Neurosurgical Department, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
authors.forename: Ming
authors.initials: M
authors.lastname: Ge
authors.forename: Kai
authors.initials: K
authors.lastname: Zhang
authors.forename: Yu
authors.initials: Y
authors.lastname: Ma
authors.forename: Fan-gang
authors.initials: FG
authors.lastname: Meng
authors.forename: Wen-han
authors.initials: WH
authors.lastname: Hu
authors.forename: An-chao
authors.initials: AC
authors.lastname: Yang
authors.forename: Jian-guo
authors.initials: JG
authors.lastname: Zhang
mesh_terms: D000293:Adolescent; D046690:Deep Brain Stimulation / Q000379:methods*; D006801:Humans; D019189:Iron Metabolism Disorders; D008297:Male; D019150:Neuroaxonal Dystrophies / Q000628:therapy*; D020531:Subthalamic Nucleus / Q000601:surgery*; D016896:Treatment Outcome