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Updated: May 12, 2026
Deep Brain Stimulation with Simultaneous fMRI in Rodents
Published on: February 15, 2014
Successful deep brain stimulation surgery with intraoperative magnetic resonance imaging on a difficult
Thien Thien Lim1, Hubert H Fernandez, Scott Cooper
1Center of Neurological Restoration, Neurological Institute, Cleveland Clinic, Ohio, USA. thienthienlim@gmail.com
Insights
Deep brain stimulation (DBS) can help manage chorea acanthocytosis hyperkinetic movements. Performing DBS under general anesthesia with intraoperative MRI guidance improves safety and efficacy for patients with this neurodegenerative disorder.
Area of Science:
- Neuroscience
- Genetics
- Neurosurgery
Background:
- Chorea acanthocytosis is a rare, progressive, hereditary neurodegenerative disorder.
- Characterized by hyperkinetic movements, seizures, and acanthocytosis.
- Current medical treatments offer limited efficacy.
Observation:
- A 32-year-old patient with chorea acanthocytosis experienced complications during awake DBS surgery.
- A subsequent DBS procedure under general anesthesia with intraoperative MRI guidance was performed.
- Significant improvements in dystonia, chorea, and quality of life were observed postoperatively.
Findings:
- Deep brain stimulation targeting the bilateral globus pallidus pars interna shows promise.
- Successful implantation was achieved using general anesthesia and intraoperative MRI guidance.
- This approach mitigates risks associated with seizures during surgery.
Implications:
- Deep brain stimulation may be a viable therapeutic option for hyperkinetic movements in neuroacanthocytosis.
- Intraoperative MRI guidance and general anesthesia enhance surgical safety and accuracy.
- Further research into DBS for neuroacanthocytosis is warranted.
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.
