脑深部电刺激术治疗原发性全身性肌张力障碍(附2例报告)  

Deep brain stimulation for primary generalized dystonia:2 cases reports

作　　者：马羽[1] 张凯[2] 葛明[2] 孟凡刚[1] 胡文瀚[1] 张建国[2] 

机构地区：[1]首都医科大学附属北京市神经外科研究所,北京100050 [2]首都医科大学附属北京天坛医院神经外科

出　　处：《中国神经精神疾病杂志》2009年第4期212-215,共4页Chinese Journal of Nervous and Mental Diseases

基　　金：北京市科技新星计划课题资助(编号:2008-B-43)

摘　　要：目的探讨关于脑深部电刺激术治疗原发性全身性肌张力障碍疗效的影响因素。方法评估病程、病情相似的2例原发性全身性肌张力障碍患者接受脑深部电刺激术治疗的术后疗效。病例1:8岁发病,BFMDRS评分100,运动评分80,功能障碍评分20,DYT1基因突变阳性。病例2:18岁发病,BFMDRS评分98,运动评分71,功能障碍评分27,DYT1基因突变阴性。结果2例患者术后症状均得到了改善,给予刺激治疗5个月时病例1的BFMDRS/运动评分/功能评分分别为38/32/6,病例2分别为66/44/22。病例1术后肌张力障碍症状改善的速度和程度均优于病例2,且病例2术后功能障碍改善较差。结论脑深部电刺激术治疗原发性全身性肌张力障碍的术后疗效主要与手术时机有很大关系,应早期手术。Objective To investigate the impact of surgical timing on the outcome of deep brain stimulation for primary generalized dystonia. Methods The outcomes following DBS for two primary generalized dystonic patients with similar disease duration and conditions were evaluated. Case 1 was DYT1 ＋ with onset on 8 years old, and his BFMDRS, movement and disability scores were 100, 80 and 20,repectively. Case 2 was DYT1 - with onset on 18 years old and his BFMDRS movement and disability scores were 98, 71, and 27, repectively. Results The dystonic symptoms of both patients had been improved by deep brain stimulation. Five months following DBS, the BFMDRS/movement/disability scores were 32/6/48 in case 1, and 66/44/22 in case 2, respectively. Case 1 achieved greater improvement than case 2 on disability scale. Conclusions Better outcome is associated with early DBS surgery in the course of primary generalized dystonia, suggesting DBS surgery should be considered early in the course of treatment of primary generalized dystonia.

关 键 词：脑深部电刺激术 原发性全身性肌张力障碍 术后疗效 

分 类 号：R746[医药卫生—神经病学与精神病学]