Successful deep brain stimulation surgery with intraoperative magnetic resonance imaging on a difficult neuroacanthocytosis case: Case report

Thien Thien Lim, Hubert H. Fernandez, Scott Cooper, Kathryn Mary K. Wilson, Andre G. Machado

Research output: Contribution to journalArticle

11 Scopus citations

Abstract

BACKGROUND AND IMPORTANCE:: 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. CLINICAL PRESENTATION:: 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. CONCLUSION:: 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.

Original languageEnglish (US)
JournalNeurosurgery
Volume73
Issue number1
DOIs
StatePublished - Jul 1 2013

Keywords

  • Chorea acanthocytosis
  • Deep brain stimulation
  • Neuroacanthocytosis

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