苍白球内侧部脑深部电刺激治疗颅颈肌张力障碍的长期与短期随访疗效比较

目的探讨苍白球内侧部（GPi）脑深部电刺激（DBS）治疗颅颈肌张力障碍（CCD）患者的长期与短期疗效的差异情况。方法本研究回顾性分析2016年1月—2023年12月在首都医科大学宣武医院行GPi DBS的24例难治性颅颈肌张力障碍患者，使用Burke-Fahn-Marsden肌张力障碍评定量表（BFMDRS）评估肌张力障碍的严重程度和残疾情况。BFMDRS评分在术前、术后6个月和最近一次随访三个时间点分别进行评估。结果16例患者的初始症状为眼睑痉挛，7例患者表现为痉挛性斜颈，只有1例患者表现为口下颌肌张力障碍。平均随访时间为（37.5±23.5）个月（6~84个月）。3个时间点的BFMDRS-M总分平均分别为13.3±9.4、5.0±4.7（改善率为55.3%，P＜0.01）、4.5±3.6（改善率为56.6%，P＜0.01）。5例（20.8%）患者的手术效果评级为差（BFMDRS评分改善率＜30%）。结论GPi DBS治疗难治性CCD具有长期疗效，但临床结果并不稳定，整体改善较为有效。对于患有特定类型肌张力障碍的患者可能会考虑 GPi以外的靶点以获得更好的疗效。

Objective To investigate the difference between long-term and short-term clinical efficacy of globus pallidus internus(GPi) deep brain stimulation(DBS) in patients with craniocervical dystonia(CCD). Methods 24 patients with refractory craniocervical dystonia who underwent GPi DBS at Xuanwu Hospital, Capital Medical University from January 2016 to December 2023 were analyzed retrospectively. The study assessed the severity and disability of the dystonia with Burke-Fahn-Marsden dystonia rating scale(BFMDRS). The BFMDRS scores were collected before 6 months after and the most recent follow-up visit. Results The initial symptom was blepharospasm in 16 patients, 7 presented with cervical dystonia and only 1 started with oromandibular dystonia. The mean follow-up period was 37.5±23.5 months(range 6-84 months). The mean total BFMDRS-M scores at the 3 time points were 13.3±9.4, 5.0±4.7(55.3% improvement, P＜0.01), 4.5±3.6(56.6% improvement, P＜0.01) respectively. In 5 patients(20.8%), the results qualified as poor(＜30% improvement in BFMDRS). Conclusions The results indicate some long-term efficacy of GPi DBS in treatment of refractory CCD. The clinical outcome is not as stable as we expect, but the overall improvement is rather effective. Patients with specific type of dystonia might consider targets other than GPi for better response.