OBJECTIVE The efficacy of globus pallidus internus-deep brain stimulation (GPi-DBS) for the treatment of Huntington's disease (HD) has not been validated in large-scale studies. We conducted an individual patient analysis to pool outcomes of all of the published HD-GPi-DBS studies. METHODS PubMed, Embase and the Cochrane Library were searched for relevant articles. The Unified Huntington's Disease Rating Scale (UHDRS)-motor and UHDRS-chorea improvements were analyzed during different follow-up periods. Secondary outcomes, including UHDRS-motor subitem scores and functional assessment results, were also analyzed. Correlation and regression analyses were conducted to find improvement predictors. This study was registered in PROSPERO (CRD42018105995). RESULTS Eighteen studies including 39 patients with 124 visits were analyzed. GPi-DBS significantly improved the UHDRS-motor score in < 3 months (p = 0.001), 3-9 months (p < 0.001), and 9-12 months (p < 0.001), but did not continue in later follow-ups. UHDRS-chorea was significantly improved even in the > 30-month follow-up (p = 0.003). Functional assessment was not improved 12 months postoperatively (p = 0.196). The Westphal variant of HD (W-HD) gained no motor benefits 6 months postoperatively (p = 0.178). The Westphal variant was the only risk factor for DBS efficacy (p = 0.044). The rate of stimulation-related adverse events was 87.2%. CONCLUSIONS GPi-DBS has a stable effect on chorea symptoms in HD patients. Chorea-dominant patients may be the best candidates for surgery, while attention should be paid to postoperative stimulation-related complications. Given that GPi-DBS has limited effects on other motor symptoms, W-HD patients are not surgical candidates.

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使用 GPi-DBS 治疗亨廷顿病的疗效的个体患者分析

目的 苍白球内部深部脑刺激 (GPi-DBS) 治疗亨廷顿病 (HD) 的疗效尚未在大规模研究中得到验证。我们进行了个体患者分析，以汇总所有已发表的 HD-GPi-DBS 研究的结果。方法在 PubMed、Embase 和 Cochrane 图书馆中搜索相关文章。统一亨廷顿舞蹈病评定量表 (UHDRS)-运动和 UHDRS-舞蹈病的改善在不同的随访期间进行了分析。次要结果，包括 UHDRS 运动分项评分和功能评估结果，也进行了分析。进行了相关性和回归分析以找到改进预测因子。本研究已在 PROSPERO (CRD42018105995) 中注册。结果 分析了 18 项研究，包括 39 名患者，共 124 次就诊。GPi-DBS 在 < 3 个月 (p = 0.001)、3-9 个月 (p < 0.001) 和 9-12 个月 (p < 0.001) 内显着改善了 UHDRS 运动评分，但在以后的随访中没有继续. 即使在 > 30 个月的随访中，UHDRS 舞蹈症也有显着改善（p = 0.003）。术后 12 个月功能评估没有改善（p = 0.196）。HD 的 Westphal 变体 (W-HD) 术后 6 个月没有获得运动方面的益处（p = 0.178）。Westphal 变体是 DBS 疗效的唯一危险因素（p = 0.044）。刺激相关不良事件的发生率为 87.2%。结论 GPi-DBS对HD患者舞蹈病症状有稳定的疗效。舞蹈病为主的患者可能是手术的最佳人选，同时应注意术后刺激相关的并发症。