Pallidal Deep Brain Stimulation in Patients With Chorea-Acanthocytosis

Liu, Ziyuan; Liu, Yang; Wan, Xiaobo; Yang, Yiying; Wang, Lin; Dou, Wei; Guo, Jinzhu; Wang, Yu; Guo, Yi; Ma, Wenbin; Wang, Renzhi

doi:10.1111/ner.12763

Cited by 10 publications

(4 citation statements)

References 32 publications

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“…In the context of these overall positive findings, some limitations must be highlighted in the published case reports. First, the data come from case reports or case series studies and articles involving operations performed by different surgeons and evaluations performed by different doctors (Table 1 4,[10][11][12][13][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34] ), which may affect surgical efficacy evaluation. The majority of the reported cases did not use blind methods to evaluate symptoms, which could bias results.…”

Section: Discussionmentioning

confidence: 99%

“…Chorea-acanthocytosis (ChAc), an autosomal recessive neuroacanthocytosis associated with VSP13A mutations in the chorein gene, classically causes oromandibular chorea with lip and tongue biting, tongue protrusion or feeding dystonia, and paroxysmal head/neck and trunk movements. [1][2][3][4] ChAc is a chronic progres-sive disease. Currently, the management of ChAc is purely symptomatic and includes botulinum toxin for oral-oro-facio-lingual dystonia; phenytoin, lorazepam, and levetiracetam for seizure management; antidepressants or antipsychotics; and dopamine antagonists.…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pallidus Stimulation for Chorea-Acanthocytosis: A Systematic Review and Meta-Analysis of Individual Data

Dong

et al. 2022

JMD

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A significant proportion of patients with chorea-acanthocytosis (ChAc) fail to respond to standard therapies. Recent evidence suggests that globus pallidus internus (GPi) deep brain stimulation (DBS) is a promising treatment option; however, reports are few and limited by sample sizes. We conducted a systematic literature review to evaluate the clinical outcome of GPi-DBS for ChAc. PubMed, Embase, and Cochrane Library databases were searched for relevant articles published before August 2021. The improvement of multiple motor and nonmotor symptoms was qualitatively presented. Improvements in the Unified Huntington's Disease Rating Scale motor score (UHDRS-MS) were also analyzed during different follow-up periods. A multivariate linear regression analysis was conducted to identify potential predictors of clinical outcomes. Twenty articles, including 27 patients, were eligible. Ninety-six percent of patients with oromandibular dystonia reported significant improvement. GPi-DBS significantly improved the UHDRS-motor score at < 6 months (p < 0.001) and ≥ 6 months (p < 0.001). The UHDRS-motor score improvement rate was over 25% in 75% (15/20 cases) of patients at long-term follow-up (≥ 6 months). The multiple linear regression analysis showed that sex, age at onset, course of disease, and preoperative movement score had no linear relationship with motor improvement at long-term follow-up (p > 0.05). GPi-DBS is an effective and safe treatment in most patients with ChAc, but no reliable predictor of efficacy has been found. Oromandibular dystonia-dominant patients might be the best candidates for GPi-DBS.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pallidus Stimulation for Chorea-Acanthocytosis: A Systematic Review and Meta-Analysis of Individual Data

Dong

et al. 2022

JMD

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“…Oral medications and botulinum toxin injections have limited efficacy in patients with ChAc. Emerging evidence has supported the clinical benefit of deep brain stimulation (DBS) of the internal globus pallidus (GPi) for the treatment of patients with ChAc; however, sufficient beneficial results are usually observed only after several months [2]. Subthalamic nucleus (STN) DBS is an alternative to GPi-DBS for idiopathic isolated dystonia patients with the potential advantages of rapid-onset efficacy and less battery consumption [3].…”

mentioning

confidence: 99%