title: Successful deep brain stimulation surgery with intraoperative magnetic resonance imaging on a difficult neuroacanthocytosis case: case report.
abstract: BACKGROUND
  Chorea acanthocytosis is a progressive hereditary neurodegenerative disorder characterized by hyperkinetic movements, seizures, and acanthocytosis in the absence of any lipid abnormality. Medical treatment is typically limited and disappointing.
  
  
  METHODS
  We report on a 32-year-old patient with chorea acanthocytosis with a failed attempt at awake deep brain stimulation (DBS) surgery due to intraoperative seizures and postoperative intracranial hematoma. He then underwent a second DBS operation, but under general anesthesia and with intraoperative magnetic resonance imaging guidance. Marked improvement in his dystonia, chorea, and overall quality of life was noted 2 and 8 months postoperatively.
  
  
  CONCLUSIONS
  DBS surgery of the bilateral globus pallidus pars interna may be useful in controlling the hyperkinetic movements in neuroacanthocytosis. Because of the high propensity for seizures in this disorder, DBS performed under general anesthesia, with intraoperative magnetic resonance imaging guidance, may allow successful implantation while maintaining accurate target localization.
authors.affiliation: Center of Neurological Restoration, Neurological Institute, Cleveland Clinic, Ohio, USA. thienthienlim@gmail.com
authors.forename: Thien Thien
authors.initials: TT
authors.lastname: Lim
authors.forename: Hubert H
authors.initials: HH
authors.lastname: Fernandez
authors.forename: Scott
authors.initials: S
authors.lastname: Cooper
authors.forename: Kathryn Mary K
authors.initials: KM
authors.lastname: Wilson
authors.forename: Andre G
authors.initials: AG
authors.lastname: Machado
mesh_terms: D000328:Adult; D046690:Deep Brain Stimulation / Q000295:instrumentation / Q000379:methods*; D004567:Electrodes, Implanted; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D054546:Neuroacanthocytosis / Q000175:diagnosis* / Q000628:therapy*; D019919:Prosthesis Implantation / Q000379:methods; D025321:Surgery, Computer-Assisted / Q000379:methods*; D016896:Treatment Outcome