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.

Objective:To investigate the correlation between fluid-attenuated inversion recovery (FLAIR) vascular hyperintensities (FVHs) and outcomes after endovascular mechanical thrombectomy (EMT) in patients with anterior circulation large vessel occlusive stroke.Methods:Using " Nanjing First Hospital Stroke Database" , consecutive patients with anterior circulation large vessel occlusive stroke received EMT treatment from June 2015 to December 2018 were enrolled retrospectively. Before EMT treatment, the distal FVH grade and the American Society of Intervention and Therapeutic Neuroradiology/Society of Interventional Radiology (ASITN/SIR) collateral circulation grade were evaluated. The modified Rankin Scale was used to evaluate the functional outcome of patients at 3 months after onset, and 0-2 was defined as a good outcome. Spearman correlation analysis was used to analyze the correlation between the distal FVH grade and the ASITN/SIR collateral circulation grade. Multivariate logistic regression analysis was used to identify the independent predictors of the outcomes. Results:A total of 117 patients with acute anterior circulation large vessel occlusive stroke were enrolled, aged 70.74±12.50 years, 72 (61.5%) were male. The baseline National Institutes of Health Stroke Scale (NIHSS) score was 13.73±4.91. Seventy-four patients (63.2%) had a good outcome and 43 (36.8%) had a poor outcome. The distal FVH grade was grade 0 in 8 cases (6.84%), grade 1 in 34 cases (29.06%), and grade 2 in 75 cases (64.10%). Compared with the distal FVH low-grade group (grade 0-1), the high-grade group (grade 2) had a higher ASITN/SIR collateral circulation grade ( P<0.001) and lower baseline National Institutes of Health Stroke Scale (NIHSS) score ( P=0.026). Spearman correlation analysis showed that the distal FVH grade was significantly positively correlated with the ASITN/SIR collateral circulation grade ( r=0.620, P<0.001). Multivariate logistic regression analysis showed that the high distal FVH grade (odds ratio [ OR] 0.336, 95% confidence interval [ CI] 0.128-0.879; P=0.026) was independently associated with the good outcomes, while the higher baseline NIHSS score ( OR 1.036, 95% CI 0.988-1.229; P=0.048) and symptomatic cerebral hemorrhage ( OR 5.597, 95% CI 1.052-29.761; P=0.043) were independently associated with the poor outcomes. Conclusion:The distal FVHs can reflect the state of collateral circulation. The high grade of distal FVHs is associated with the good outcomes after EMT in patients with anterior circulation large vessel occlusive stroke.