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) |
|---|---|
Division/Institute: |
04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology |
UniBE Contributor: |
Burgunder, Jean-Marc, Kaelin, Alain |
ISSN: |
1590-1874 |
Publisher: |
Springer |
Language: |
English |
Submitter: |
Factscience Import |
Date Deposited: |
04 Oct 2013 14:39 |
Last Modified: |
05 Dec 2022 14:12 |
Publisher DOI: |
10.1007/s10072-011-0741-y |
PubMed ID: |
21863267 |
Web of Science ID: |
000304242100005 |
BORIS DOI: |
10.48350/15973 |
URI: |
https://boris.unibe.ch/id/eprint/15973 (FactScience: 223496) |
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