J Korean Neurosurg Soc.  2015 Feb;57(2):143-146. 10.3340/jkns.2015.57.2.143.

Globus Pallidus Interna Deep Brain Stimulation for Chorea-Acanthocytosis

• Lee JH
• Cho WH
• Cha SH
• Kang DW

• KMID: 2191199
• DOI: http://doi.org/10.3340/jkns.2015.57.2.143

Abstract

Chorea-acanthocytosis (ChAc) is a rare hereditary disorder characterized by involuntary choreiform movements and erythrocytic acanthocytosis. Pharmacotherapy for control of involuntary movements has generally been of limited benefit. Deep brain stimulation (DBS) has recently been used for treatment of some refractory cases of ChAc. We report here on the effect of bilateral high-frequency DBS of globus pallidus interna in a patient with ChAc.

Keyword

Chorea-acanthocytosis; Deep brain stimulation; Chorea; Globus pallidus

MeSH Terms

Abetalipoproteinemia
Chorea
Deep Brain Stimulation*
Drug Therapy
Dyskinesias
Globus Pallidus*
Humans
Neuroacanthocytosis*

Figure

• 

  Fig. 1 T2-weighted MR imaging showed mild bilateral atrophy of the head of the caudate nucleus.

• 

  Fig. 2 A : Acanthocytosis (arrows) in a peripheral blood smear (wright stain, ×1000). B : Electron microscopic scanning.

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  Fig. 3 Plain skull X-ray and CT scan showing a deep brain stimulation electrode located on the globus pallidus bilaterally.

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