Berenson-Allen Center for Noninvasive Brain Stimulation, Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts2Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston.
Division of Neurosurgery, Department of Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts.
JAMA Neurol. 2015 Jun;72(6):713-9. doi: 10.1001/jamaneurol.2015.51.
Dystonia is a heterogeneous neurologic disorder characterized by abnormal muscle contractions for which standard medical therapy is often inadequate. For such patients, therapeutic brain stimulation is becoming increasingly used.
To review the evidence and effect sizes for treating different types of dystonia with different types of brain stimulation and to discuss recent advances relevant to patient selection, surgical approach, programming, and mechanism of action.
PubMed was searched for publications on the clinical effect of brain stimulation in dystonia up through December 31, 2014. Recent meta-analyses, consensus statements, and evidence-based guidelines were incorporated. Emphasis was placed on deep brain stimulation (DBS) and randomized clinical trials; however, other stimulation modalities and trial designs were included. For each intervention the mean change in dystonia severity, number of patients studied, and evidence of efficacy based on American Academy of Neurology criteria were determined.
Strong (level B) evidence supports the use of DBS for the treatment of primary generalized or segmental dystonia, especially when due to mutation in the DYT1 gene, as well as for patients with cervical dystonia. Large effect sizes have also been reported for DBS treatment of tardive dystonia, writer's cramp, cranial dystonia, myoclonus dystonia, and off-state dystonia associated with Parkinson disease. Lesser benefit is generally seen in dystonia secondary to structural brain damage. Other brain stimulation techniques, including epidural cortical stimulation and noninvasive brain stimulation, have been investigated, but generally report smaller effect sizes in fewer patients.
Patients with dystonia that is not adequately controlled with standard medical therapy should be referred for consideration of DBS, especially patients with generalized, segmental, or cervical dystonia. Other less-invasive stimulation modalities require further research before being considered a therapeutic alternative.
肌张力障碍是一种异质性的神经系统疾病，其特征是肌肉异常收缩，标准的医学治疗往往效果不佳。对于这类患者，治疗性脑刺激越来越多地被应用。
综述不同类型脑刺激治疗不同类型肌张力障碍的证据和效应大小，并讨论与患者选择、手术方法、程控和作用机制相关的最新进展。
通过检索 PubMed 数据库，获取截至 2014 年 12 月 31 日关于脑刺激治疗肌张力障碍的临床效果的文献。纳入了最近的荟萃分析、共识声明和循证指南。重点是深部脑刺激（DBS）和随机临床试验，但也包括了其他刺激方式和试验设计。对于每种干预措施，确定了肌张力障碍严重程度的平均变化、研究的患者数量以及根据美国神经病学学会标准确定的疗效证据。
DBS 治疗原发性全身性或节段性肌张力障碍，特别是由 DYT1 基因突变引起的肌张力障碍，以及治疗颈性肌张力障碍，具有强有力（B 级）的证据支持。DBS 治疗迟发性肌张力障碍、书写痉挛、颅部肌张力障碍、肌阵挛性肌张力障碍和与帕金森病相关的开期肌张力障碍也报告了较大的效应大小。在由结构性脑损伤引起的肌张力障碍中，通常疗效较差。其他脑刺激技术，包括硬膜外皮质刺激和非侵入性脑刺激，也得到了研究，但一般报告的患者数量较少，效应大小较小。
对于标准医学治疗不能充分控制的肌张力障碍患者，应考虑进行 DBS 治疗，特别是全身性、节段性或颈性肌张力障碍患者。其他微创刺激方式在被考虑作为治疗选择之前，需要进一步的研究。
