Alterman R L, Miravite J, Weisz D, Shils J L, Bressman S B, Tagliati M
Mount Sinai School of Medicine, New York, NY 10029, USA.
Neurology. 2007 Aug 14;69(7):681-8. doi: 10.1212/01.wnl.0000267430.95106.ff.
To evaluate the safety and efficacy of 60 Hz deep brain stimulation (DBS) of the globus pallidus internus (GPi) in 15 consecutive patients with primary dystonia.
We conducted a retrospective analysis of clinic charts relative to 15 consecutive patients with medically refractory primary dystonia who underwent stereotactic implantation of DBS leads within the GPi. Twelve had the DYT1 gene mutation. Frame-based MRI and intraoperative microelectrode recording were employed for targeting. All patients were treated exclusively with stimulation at 60 Hz from therapy outset. The Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) served as the primary measure of symptom severity at baseline and 1, 3, 6, and 12 months after treatment.
All patients tolerated DBS treatment well and showed a progressive median improvement of their BFMDRS motor subscores from 38% at 1 month to 89% at 1 year (p < 0.001, Wilcoxon rank sum test). The disability subscores were similarly improved. The clinical response to DBS allowed seven patients to completely discontinue their medications; six additional patients had reduced their medications by at least 50%. Surgical complications were limited to two superficial infections, which were treated successfully.
Stimulation of the internal globus pallidus at 60 Hz is safe and effective for treating medically refractory primary dystonia.
评估连续15例原发性肌张力障碍患者接受苍白球内侧部（GPi）60赫兹深部脑刺激（DBS）的安全性和有效性。
我们对连续15例药物难治性原发性肌张力障碍患者的临床病历进行了回顾性分析，这些患者接受了GPi内DBS电极的立体定向植入。其中12例有DYT1基因突变。采用基于框架的磁共振成像（MRI）和术中微电极记录进行靶点定位。所有患者从治疗开始就仅接受60赫兹的刺激治疗。伯克-法恩-马斯登肌张力障碍评定量表（BFMDRS）作为治疗前及治疗后1、3、6和12个月症状严重程度的主要衡量指标。
所有患者对DBS治疗耐受性良好，BFMDRS运动亚评分中位数从1个月时的38%逐步改善至1年时的89%（p<0.001，Wilcoxon秩和检验）。残疾亚评分也有类似改善。DBS的临床反应使7例患者完全停药；另外6例患者药物用量减少至少50%。手术并发症仅限于2例表浅感染，均成功治愈。
60赫兹刺激苍白球内侧部治疗药物难治性原发性肌张力障碍安全有效。
