Division of Neurosurgery, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada.
Mov Disord. 2013 Mar;28(3):282-7. doi: 10.1002/mds.25326. Epub 2013 Feb 7.
Myoclonus dystonia (M-D) syndrome is a heritable movement disorder characterized by myoclonic jerks and dystonia primarily of the upper extremities. M-D remains poorly responsive to pharmacological treatment. Emerging reports suggest good response to DBS of the internal globus pallidus (GPi) and ventral intermediate nucleus (VIM) of the thalamus. This study aimed to appraise the value of these two DBS targets by evaluating reports available in the literature. A systematic search of published case reports and case series was performed on Medline and Embase. Responses to DBS were evaluated. Myoclonus was assessed with the Unified Myoclonus Rating Scale (UMRS) and dystonia by the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). The primary outcome of interest was the relative improvements noted with GPi, compared to VIM stimulation. A total of 17 publications yielded 40 unique cases, with mean follow-up of 27.2 months. All patients demonstrated improvements in myoclonus scores, with 93.5% showing at least a 50% improvement in UMRS. The mean improvement in myoclonus scores was 72.6%. In contrast, dystonia scores were improved in 87.9% of patients, with 72.7% reporting at least a 50% improvement in BFMDRS. The mean improvement in dystonia scores was 52.6%. Improvements in myoclonus scores were similar for both GPi (75.7%) and VIM (70.4%; P = 0.27). However, the improvements in dystonia scores were greater with GPi (60.2%), compared to VIM (33.3%; P = 0.03). Although both targets achieve similar improvements in myoclonus, GPi stimulation may be a preferred target because it may achieve greater improvements in dystonia, compared to VIM stimulation.
肌阵挛性肌张力障碍（M-D）综合征是一种遗传性运动障碍，其特征为肌阵挛性抽搐和主要在上肢的肌张力障碍。M-D 对药物治疗反应不佳。新兴报告表明，对丘脑内苍白球（GPi）和腹侧中间核（VIM）的深部脑刺激（DBS）反应良好。本研究旨在通过评估文献中的报告来评估这两个 DBS 靶点的价值。在 Medline 和 Embase 上进行了已发表的病例报告和病例系列的系统搜索。评估了对 DBS 的反应。肌阵挛用统一肌阵挛评定量表（UMRS）进行评估，而肌张力障碍用 Burke-Fahn-Marsden 肌张力障碍评定量表（BFMDRS）进行评估。主要的研究结果是与 VIM 刺激相比，GPi 刺激的相对改善。共 17 篇文献产生了 40 个独特的病例，平均随访 27.2 个月。所有患者的肌阵挛评分均有改善，93.5%的患者 UMRS 评分至少改善 50%。肌阵挛评分的平均改善为 72.6%。相比之下，87.9%的患者的肌张力障碍评分得到改善，72.7%的患者 BFMDRS 评分至少改善 50%。肌张力障碍评分的平均改善为 52.6%。GPi（75.7%）和 VIM（70.4%；P = 0.27）的肌阵挛评分改善相似。然而，GPi 刺激（60.2%）改善的肌张力障碍评分明显大于 VIM 刺激（33.3%；P = 0.03）。虽然两个靶点在肌阵挛方面都有相似的改善，但与 VIM 刺激相比，GPi 刺激可能是一个更好的选择，因为它可能在改善肌张力障碍方面有更大的改善。
