脑深部电刺激术治疗Hallervorden-Spatz病一例及文献复习

目的 观察双侧丘脑底核脑深部电刺激术治疗1例Hallervorden-Spatz病的手术效果.方法 将脑深部刺激电极植入双侧丘脑底核,分别于术前、开机后1个月、开机后3个月,12个月时对其进行BFM及BFMDMS评分,评价治疗效果.结果 术前、开机后1个月、开机后3个月、12个月的BFMDMS评分分别为114、35、28、14分,症状改善率刺激1个月时为69%、3个月为75%、12个月为88%.持续刺激未引起任何不良反应,患者不再服用相关药物.结论 双侧丘脑底核脑深部电刺激术能够有效的改善严重的全身性肌张力障碍Hallervorden-Spatz病症状,是一种可供选择的安全有效的治疗方法.

Bilateral subthalamic nucleus deep brain stimulation for Hallervorden-Spatz disease and literature review

Objective To observe the efficacy of deep brain stimulation on subthalamic nucleus (STN) on the treatment of one case of Hallervorden-Spatz Disease (HSD). Methods Two stimulating electrodes were implanted into bilateral subthalamic nuclei. BFM and BFMDMS were used before operation, one month, three months and twelve months after stimulator turned on postoperatively to evaluate the efficacy. Results BFMDMS scale was 114, 35, 28, 14 before operation, one month, three months and twelve months after stimulator-on. The improvements were 69%, 75%, 88% respectively. There were no adverse effects attributable to stimulation at the optimal setting. Preoperative medication was tapered off totally after programming. Conclusions This suggests that neurostimulation of the STN may be an effective treatment for severe generalized dystonia Hallervorden-Spatz Disease, which maybe a new selection of treatments for refractory focal dystonia.