• Thien Thien Lim, Hubert H Fernandez, Scott Cooper, Kathryn Mary K Wilson, and Andre G Machado.
• Center of Neurological Restoration, Neurological Institute, Cleveland Clinic, Ohio, USA. thienthienlim@gmail.com
• Neurosurgery. 2013 Jul 1;73(1):E184-7; discussion E188.

Background And ImportanceChorea 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.Clinical PresentationWe 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.ConclusionDBS 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.

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