Abstract：
OBJECTIVE: Deep brain stimulation (DBS) of the subthalamic nucleus (STN) has demonstrated efficacy against multiple types of dystonia, but only a few case reports and small-sample studies have investigated the clinical utility of STN-DBS for Meige syndrome, a rare but distressing form of craniofacial dystonia. Furthermore, the effects of DBS on critical neuropsychological sequelae, such as depression and anxiety, are rarely examined. In this study, the authors investigated the therapeutic efficacy of STN-DBS for both motor and psychiatric symptoms of Meige syndrome.
METHODS: The authors retrospectively reviewed consecutive patients with Meige syndrome receiving bilateral STN-DBS at their institution from January 2016 to June 2023. Motor performance and nonmotor features including mood, cognitive function, and quality of life (QOL) were evaluated using standardized rating scales at baseline and at final postoperative follow-up. Clinical and demographic factors influencing postoperative motor outcome were evaluated by uni- and multivariable linear regression models.
RESULTS: Fifty-one patients were ultimately included, with a mean ± SD follow-up duration of 27.3 ± 18.0 months. The mean Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement score improved from 12.9 ± 5.2 before surgery to 5.3 ± 4.2 at the last follow-up (mean improvement 58.9%, p < 0.001) and the mean BFMDRS disability score improved from 5.6 ± 3.3 to 2.9 ± 2.9 (mean improvement 44.6%, p < 0.001). Hamilton Depression and Anxiety Rating Scale scores also improved by 35.3% and 34.2%, respectively, and the postoperative 36-item Short-Form Health Survey score indicated substantial QOL enhancement. Global cognition remained stable after treatment. Multiple linear regression analysis identified disease duration (β = -0.241, p = 0.027), preoperative anxiety severity (β = -0.386, p = 0.001), and volume of activated tissue within the dorsolateral (sensorimotor) STN (β = 0.483, p < 0.001) as independent predictors of motor outcome.
CONCLUSIONS: These findings support STN-DBS as an effective and promising therapy for both motor and nonmotor symptoms of Meige syndrome. Timely diagnosis, treatment of preoperative anxiety, and precise electrode placement within the dorsolateral STN are essential for optimal clinical outcome.
METHODS: The authors retrospectively reviewed consecutive patients with Meige syndrome receiving bilateral STN-DBS at their institution from January 2016 to June 2023. Motor performance and nonmotor features including mood, cognitive function, and quality of life (QOL) were evaluated using standardized rating scales at baseline and at final postoperative follow-up. Clinical and demographic factors influencing postoperative motor outcome were evaluated by uni- and multivariable linear regression models.
RESULTS: Fifty-one patients were ultimately included, with a mean ± SD follow-up duration of 27.3 ± 18.0 months. The mean Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) movement score improved from 12.9 ± 5.2 before surgery to 5.3 ± 4.2 at the last follow-up (mean improvement 58.9%, p < 0.001) and the mean BFMDRS disability score improved from 5.6 ± 3.3 to 2.9 ± 2.9 (mean improvement 44.6%, p < 0.001). Hamilton Depression and Anxiety Rating Scale scores also improved by 35.3% and 34.2%, respectively, and the postoperative 36-item Short-Form Health Survey score indicated substantial QOL enhancement. Global cognition remained stable after treatment. Multiple linear regression analysis identified disease duration (β = -0.241, p = 0.027), preoperative anxiety severity (β = -0.386, p = 0.001), and volume of activated tissue within the dorsolateral (sensorimotor) STN (β = 0.483, p < 0.001) as independent predictors of motor outcome.
CONCLUSIONS: These findings support STN-DBS as an effective and promising therapy for both motor and nonmotor symptoms of Meige syndrome. Timely diagnosis, treatment of preoperative anxiety, and precise electrode placement within the dorsolateral STN are essential for optimal clinical outcome.
摘要：
目的：丘脑下核（STN）的深部脑刺激（DBS）已证明对多种类型的肌张力障碍有效，但只有少数病例报告和小样本研究研究了STN-DBS对Meige综合征的临床应用，一种罕见但令人痛苦的颅面肌张力障碍。此外，DBS对严重神经心理后遗症的影响，比如抑郁和焦虑，很少检查。在这项研究中,作者研究了STN-DBS对Meige综合征运动和精神症状的疗效。
方法：作者回顾性回顾了2016年1月至2023年6月在其机构接受双侧STN-DBS治疗的连续Meige综合征患者。运动表现和非运动特征，包括情绪，认知功能,和生活质量(QOL)在基线和最后一次术后随访时使用标准化评定量表进行评估.通过单变量和多变量线性回归模型评估影响术后运动结局的临床和人口统计学因素。
结果：最终纳入51例患者，平均±SD随访时间为27.3±18.0个月。平均Burke-Fahn-Marsden肌张力障碍量表（BFMDRS）运动评分从手术前的12.9±5.2提高到末次随访时的5.3±4.2（平均改善58.9％，p<0.001），平均BFMDRS残疾评分从5.6±3.3提高到2.9±2.9（平均提高44.6%，p<0.001)。汉密尔顿抑郁和焦虑量表评分也分别提高了35.3%和34.2%,分别,术后36项简短健康调查评分显示QOL显著增强。治疗后总体认知保持稳定。多元线性回归分析确定了疾病持续时间(β=-0.241，p=0.027)，术前焦虑严重程度（β=-0.386，p=0.001），和背外侧（感觉运动）STN内激活组织的体积（β=0.483，p<0.001）作为运动结果的独立预测因子。
结论：这些发现支持STN-DBS作为Meige综合征的运动和非运动症状的有效且有前景的治疗方法。及时诊断,术前焦虑的治疗，和在背外侧STN内的精确电极放置对于最佳临床结果至关重要。
方法：作者回顾性回顾了2016年1月至2023年6月在其机构接受双侧STN-DBS治疗的连续Meige综合征患者。运动表现和非运动特征，包括情绪，认知功能,和生活质量(QOL)在基线和最后一次术后随访时使用标准化评定量表进行评估.通过单变量和多变量线性回归模型评估影响术后运动结局的临床和人口统计学因素。
结果：最终纳入51例患者，平均±SD随访时间为27.3±18.0个月。平均Burke-Fahn-Marsden肌张力障碍量表（BFMDRS）运动评分从手术前的12.9±5.2提高到末次随访时的5.3±4.2（平均改善58.9％，p<0.001），平均BFMDRS残疾评分从5.6±3.3提高到2.9±2.9（平均提高44.6%，p<0.001)。汉密尔顿抑郁和焦虑量表评分也分别提高了35.3%和34.2%,分别,术后36项简短健康调查评分显示QOL显著增强。治疗后总体认知保持稳定。多元线性回归分析确定了疾病持续时间(β=-0.241，p=0.027)，术前焦虑严重程度（β=-0.386，p=0.001），和背外侧（感觉运动）STN内激活组织的体积（β=0.483，p<0.001）作为运动结果的独立预测因子。
结论：这些发现支持STN-DBS作为Meige综合征的运动和非运动症状的有效且有前景的治疗方法。及时诊断,术前焦虑的治疗，和在背外侧STN内的精确电极放置对于最佳临床结果至关重要。
