摘要目的 评价脑深部电刺激术(DBS)治疗原发性全身型肌张力障碍(PGD)的长期效果,并探讨疗效的影响因素.方法 回顾性分析空军军医大学唐都医院神经外科2004年12月至2013年4月行DBS治疗的21例PGD患者的临床资料.11例患者采用以丘脑底核(STN)为靶点的DBS(STN-DBS),10例患者行以苍白球内侧部(GPi)为靶点的DBS(GPi-DBS).应用Burke-Fahn-Marsden肌张力障碍评定量表(BFMDRS)评价患者肌张力障碍的严重程度.采用多元线性回归分析探讨影响临床疗效的因素.结果 随访时间为5 ～11年,中位时间为6.5年.21例PGD患者末次随访时的BFMDRS运动评分改善率为(65.7±13.9)％,残疾评分改善率[中位数(四分位间距)]为54.5％(17.0％).STN-DBS组运动评分和残疾评分的改善率分别为(64.4±15.8)％和57.1％(20.2％)[中位数(四分位间距)],GPi-DBS组分别为(67.1±12.2)％和52.8％(18.0％)[中位数(四分位间距)].STN-DBS和GPi-DBS组末次随访时的BFMDRS运动评分改善率、残疾评分改善率的差异均无统计学意义(均P＞0.05).多元线性回归分析结果显示,仅病程对临床疗效有显著影响,且与随访时BFMDRS运动评分改善率呈负相关(t=-7.082,P＜0.001),其余变量包括性别、发病年龄、手术年龄、DBS靶点以及术前BFMDRS运动评分对临床疗效均无影响(均P＞0.05).结论 DBS治疗PGD持久有效.GPi和STN均可作为可选择的治疗靶点,两者疗效相似.病程越短的患者可能获得更佳的临床疗效.

abstractsObjective To evaluate the long-term efficacy of deep brain stimulation (DBS) for the treatment of primary generalized dystonia (PGD) and to analyze the factors influencing the clinical outcome.Methods We retrospectively analyzed the data of 21 patients with PGD who underwent DBS from December 2004 to April 2013 at Department of Neurosurgery,Tangdu Hospital,Air Force Medical University.DBS targeting the subthalamic nucleus (STN-DBS) was applied in 11 patients,and DBS targeting the globus pallidus internus (GPi-DBS) was performed in 10 patients.The severity of dystonia was assessed using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS).Multiple linear regression analysis was used to explore the factors affecting clinical outcomes.Results The follow-up period ranged from 5 years to 11 years,and the median time was 6.5 years.The average improvement rate of BFMDRS movement scores was 65.7％ ± 13.9％ and the median improvement rate of disability scores was 54.5 ％ [IQR (interquartile range):17.0％] in the 21 patients with PGD at the last follow-up.The mean/median improvement rates of movement score and disability score in STN-DBS group were 64.4％ ± 15.8％ and 57.1％ (IQR:20.2％) respectively,and those in GPi-DBS group were 67.1％ ± 12.2％ and 52.8％ (IQR:18.0％) respectively.There was no significant difference in the average improvement rate of BFMDRS movement score or disability score between STN-DBS and GPi-DBS group (both P ＞ 0.05).Multiple linear regression analysis showed that only the course of disease had a significant effect on clinical outcome,which was negatively correlated with the results (t =-7.082,P ＜ 0.001).The remaining variables including gender,age of onset,age of surgery,DBS target and preoperative movement score had no effect on clinical efficacy (all P ＞ 0.05).Conclusions DBS treatment of PGD is effective.Both GPi and STN can be used as alternative therapeutic targets and their efficacies seem similar.Patients with shorter disease duration may achieve better clinical outcomes.