Erschienen in:
01.04.2012 | Original Article
Deep brain stimulation of the globus pallidus internal improves symptoms of chorea-acanthocytosis
verfasst von:
Peng Li, Rui Huang, Wei Song, Jie Ji, Jean-Marc Burgunder, Xing Wang, Qi Zhong, Alain Kaelin-Lang, Wei Wang, Hui-Fang Shang
Erschienen in:
Neurological Sciences
|
Ausgabe 2/2012
Einloggen, um Zugang zu erhalten
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.