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Title of Journal: Neurol Sci

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Abbravation: Neurological Sciences

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Springer Milan

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DOI

10.1016/0022-3956(91)90024-5

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ISSN

1590-3478

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Deep brain stimulation of the globus pallidus inte

Authors: Peng Li Rui Huang Wei Song Jie Ji JeanMarc Burgunder Xing Wang Qi Zhong Alain KaelinLang Wei Wang HuiFang Shang
Publish Date: 2011/08/24
Volume: 33, Issue: 2, Pages: 269-274
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Abstract

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

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