1Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto.
2University Health Network, Toronto.
J Neurosurg Pediatr. 2021 Jan 1;27(3):346-356. doi: 10.3171/2020.7.PEDS20322. Print 2021 Mar 1.
The objective of this study was to report the authors' experience with deep brain stimulation (DBS) of the internal globus pallidus (GPi) as a treatment for pediatric dystonia, and to elucidate substrates underlying clinical outcome using state-of-the-art neuroimaging techniques.
A retrospective analysis was conducted in 11 pediatric patients (6 girls and 5 boys, mean age 12 ± 4 years) with medically refractory dystonia who underwent GPi-DBS implantation between June 2009 and September 2017. Using pre- and postoperative MRI, volumes of tissue activated were modeled and weighted by clinical outcome to identify brain regions associated with clinical outcome. Functional and structural networks associated with clinical benefits were also determined using large-scale normative data sets.
A total of 21 implanted leads were analyzed in 11 patients. The average follow-up duration was 19 ± 20 months (median 5 months). Using a 7-point clinical rating scale, 10 patients showed response to treatment, as defined by scores < 3. The mean improvement in the Burke-Fahn-Marsden Dystonia Rating Scale motor score was 40% ± 23%. The probabilistic map of efficacy showed that the voxel cluster most associated with clinical improvement was located at the posterior aspect of the GPi, comparatively posterior and superior to the coordinates of the classic GPi target. Strong functional and structural connectivity was evident between the probabilistic map and areas such as the precentral and postcentral gyri, parietooccipital cortex, and brainstem.
This study reported on a series of pediatric patients with dystonia in whom GPi-DBS resulted in variable clinical benefit and described a clinically favorable stimulation site for this cohort, as well as its structural and functional connectivity. This information could be valuable for improving surgical planning, simplifying programming, and further informing disease pathophysiology.
本研究旨在报告作者使用深部脑刺激（DBS）治疗儿科肌张力障碍的经验，并利用最先进的神经影像学技术阐明与临床结果相关的基础。
回顾性分析了 2009 年 6 月至 2017 年 9 月期间接受 GPi-DBS 植入的 11 例药物难治性肌张力障碍儿童患者（6 名女性，5 名男性，平均年龄 12±4 岁）的资料。使用术前和术后 MRI，对组织激活体积进行建模，并根据临床结果进行加权，以确定与临床结果相关的脑区。还使用大规模的规范数据集确定与临床获益相关的功能和结构网络。
共分析了 11 例患者的 21 根植入导联。平均随访时间为 19±20 个月（中位数为 5 个月）。根据 7 分临床评分量表，10 例患者的治疗反应定义为评分<3 分。Burke-Fahn-Marsden 肌张力障碍评分运动评分的平均改善率为 40%±23%。疗效概率图显示，与临床改善最相关的体素簇位于 GPi 的后外侧，与经典 GPi 靶点的坐标相比，位置相对靠后和靠上。概率图与中央前回和中央后回、顶枕叶皮质和脑干等区域之间存在强烈的功能和结构连接。
本研究报告了一系列接受 GPi-DBS 治疗的肌张力障碍儿童患者，结果显示该治疗方法具有不同的临床获益，并描述了该队列中一个临床获益良好的刺激部位及其结构和功能连接。这些信息对于改善手术规划、简化编程以及进一步了解疾病的病理生理学可能具有重要价值。
