title: Successful Combination of Pallidal and Thalamic Stimulation for Intractable Involuntary Movements in Patients with Neuroacanthocytosis.
abstract: OBJECTIVE
  Neuroacanthocytosis (NA) is a rare neurodegenerative disease that involves severe involuntary movements including chorea, dystonia, and trunk spasms. Current treatments are not effective for these involuntary movements. Although there are a few reports on the use of deep brain stimulation to treat patients with NA, the optimal stimulation target is not yet definitive. Some authors have reported successful improvement of NA symptoms with stimulation of the globus pallidum interna, and others have reported a reduction in trunk spasm with stimulation of the ventralis oralis complex of the thalamus. We investigated whether the optimal target is well defined for NA.
  
  
  METHODS
  We describe the effect of combination stimulation of the globus pallidum interna and the ventralis oralis complex of the thalamus in 2 patients with NA who presented with severe intractable involuntary movements.
  
  
  RESULTS
  Gpi stimulation alone was an insufficient effect for trunk spasm and/or chorea. Vo complex stimulation given without Gpi stimulation resulted in improvement of trunk spasm after 2 weeks and might also have had an incomplete effect on involuntary movement including a chorea. The combination of Gpi and Vo complex stimulation reduced the trunk spasms and chorea. This improvement was maintained at 3 months after surgery. The Unified Huntington's Disease Rating Scale score at 1 year after surgery was lower than that before surgery.
  
  
  CONCLUSIONS
  Gpi stimulation appears to be insufficient to control violent involuntary movements; therefore, combined GPi and Vo complex stimulation provided some moderate advantage over Gpi stimulation alone.
authors.affiliation: Department of Neurosurgery, Kinki University, School of Medicine, Osaka, Japan. Electronic address: nnakano@med.kindai.ac.jp.
authors.forename: Naoki
authors.initials: N
authors.lastname: Nakano
authors.affiliation: Department of Neurosurgery, Kinki University, School of Medicine, Osaka, Japan.
authors.forename: Masaharu
authors.initials: M
authors.lastname: Miyauchi
authors.affiliation: Department of Neurosurgery, Kinki University, School of Medicine, Osaka, Japan.
authors.forename: Kinya
authors.initials: K
authors.lastname: Nakanishi
authors.affiliation: Department of Neurology, Kinki University, School of Medicine, Osaka, Japan.
authors.forename: Kazumasa
authors.initials: K
authors.lastname: Saigoh
authors.affiliation: Department of Neurology, Kinki University, School of Medicine, Osaka, Japan.
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Mitsui
authors.affiliation: Department of Neurosurgery, Kinki University, School of Medicine, Osaka, Japan.
authors.forename: Amami
authors.initials: A
authors.lastname: Kato
mesh_terms: D000328:Adult; D002819:Chorea / Q000209:etiology / Q000628:therapy; D046690:Deep Brain Stimulation / Q000379:methods*; D005917:Globus Pallidus / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009069:Movement Disorders / Q000209:etiology* / Q000628:therapy*; D054546:Neuroacanthocytosis / Q000150:complications*; D013035:Spasm / Q000209:etiology / Q000628:therapy; D013788:Thalamus / Q000601:surgery*; D016896:Treatment Outcome
keywords: Combination; Deep brain stimulation; Globus pallidum interna; Involuntary movement; Neuroacanthocytosis; Ventralis oralis complex