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

Li, Peng; Huang, Rui; Song, Wei; Ji, Jie; Burgunder, Jean-Marc; Wang, Xing; Zhong, Qi; Kaelin-Lang, Alain; Wang, Wei; Shang, Hui-Fang (2012). Deep brain stimulation of the globus pallidus internal improves symptoms of chorea-acanthocytosis. Neurological sciences, 33(2), pp. 269-74. Milano: Springer 10.1007/s10072-011-0741-y

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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.

Item Type:

Journal Article (Further Contribution)


04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology

UniBE Contributor:

Burgunder, Jean-Marc and Kaelin, Alain








Factscience Import

Date Deposited:

04 Oct 2013 14:39

Last Modified:

17 Mar 2015 21:33

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URI: (FactScience: 223496)

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