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Deep brain stimulation of the globus pallidus internal improves symptoms of chorea-acanthocytosis

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Abstract

Chorea-acanthocytosis is a rare autosomal recessive disorder. To date, treatment is only symptomatic and supportive. Results from the few reports of chorea-acanthocytosis patients treated with deep brain stimulation (DBS) have been inconsistent. We present case reports for two patients with chorea-acanthocytosis who received DBS treatment and compare the outcomes with results from the literature. Both patients showed the typical clinical features of chorea-acanthocytosis with motor symptoms resistant to medical treatment. Chorea was significantly improved following low-frequency DBS treatment in both patients. However, dystonia was only mildly improved. Four chorea-acanthocytosis patients treated with DBS treatment have been reported in the literature. One patient had improvement with low-frequency DBS stimulation, while another two had improvement with higher-frequency DBS. One patient, however, did not improve with either low-frequency or high-frequency DBS. Bilateral DBS to the GPi can improve chorea and dystonia in some patients with intractable chorea-acanthocytosis. However, selection criteria for the most promising candidates must be defined, and the long-term benefits evaluated in clinical studies.

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There is no actual or potential financial and other conflict of interest related to the submitted manuscript.

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Authors and Affiliations

  1. Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, 610041, SiChuan, China

    Peng Li, Xing Wang, Qi Zhong & Wei Wang

  2. Department of Neurology, West China Hospital, Sichuan University, Chengdu, 610041, SiChuan, China

    Rui Huang, Wei Song, Jean-Marc Burgunder & Hui-Fang Shang

  3. Department of Hematology, West China Hospital, Sichuan University, Chengdu, 610041, SiChuan, China

    Jie Ji

  4. Department of Neurology, Inselsptial, Bern University, 3010, Bern, Switzerland

    Jean-Marc Burgunder & Alain Kaelin-Lang

Authors
  1. Peng Li
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  2. Rui Huang
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  3. Wei Song
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  4. Jie Ji
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  5. Jean-Marc Burgunder
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  6. Xing Wang
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  7. Qi Zhong
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  8. Alain Kaelin-Lang
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  9. Wei Wang
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  10. Hui-Fang Shang
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Corresponding authors

Correspondence to Wei Wang or Hui-Fang Shang.

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Li, P., Huang, R., Song, W. et al. Deep brain stimulation of the globus pallidus internal improves symptoms of chorea-acanthocytosis. Neurol Sci 33, 269–274 (2012). https://doi.org/10.1007/s10072-011-0741-y

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  • DOI: https://doi.org/10.1007/s10072-011-0741-y

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