Department of Neurosurgery, West China Hospital, Sichuan University, 37 Guoxue Alley, Chengdu, 610041, Sichuan Province, China.
Neurosurg Rev. 2023 Jan 25;46(1):40. doi: 10.1007/s10143-023-01950-4.
Given the good results of deep brain stimulation (DBS) in the treatment of movement disorders, DBS was initially tried to treat Lesch-Nyhan syndrome (LNS) with the aim to alleviate LNS-related dystonia. Some cases have reported clinical results of DBS in LNS thus far. This systematic review was conducted to comprehensively summarize cases of LNS treated with DBS and evaluate the efficacy and safety of DBS in LNS. Eight publications covering 12 LNS patients were included in this review. DBS improved dystonia of the LNS to varying degrees. All the included cases achieved partial or complete control of self-injurious behavior (SIB). Overall, DBS is a promising treatment for both motor and behavior disorders of LNS patients, but the results reported thus far have varied widely, especially for motor outcomes. The ultimate clinical benefits in LNS patients were still unpredictable. DBS-related complications were rather common, which raised questions about the safety of the procedure in LNS. More research is needed to further clarify the safety and effectiveness of this treatment.
鉴于深部脑刺激 (DBS) 在治疗运动障碍方面的良好效果，DBS 最初被尝试用于治疗 Lesch-Nyhan 综合征 (LNS)，旨在缓解 LNS 相关的肌张力障碍。迄今为止，已有一些病例报告了 DBS 在 LNS 中的临床效果。本系统评价旨在全面总结 DBS 治疗 LNS 的病例，并评估 DBS 在 LNS 中的疗效和安全性。本综述纳入了 8 篇文献，涵盖了 12 例 LNS 患者。DBS 改善了 LNS 的肌张力障碍，程度不一。所有纳入的病例均实现了对自伤行为 (SIB) 的部分或完全控制。总体而言，DBS 是一种有前途的治疗 LNS 患者运动和行为障碍的方法，但迄今为止报告的结果差异很大，尤其是在运动结果方面。LNS 患者的最终临床获益仍难以预测。DBS 相关并发症相当常见，这引发了对该程序在 LNS 中安全性的质疑。需要更多的研究来进一步阐明这种治疗的安全性和有效性。
