Unit of Functional Neurosurgery, Department of Neurosurgery, University of Athens, Evangelismos Hospital, Athens, Greece.
J Neurosurg. 2010 Jun;112(6):1279-82. doi: 10.3171/10.3171/2009.10.JNS09588.
Dystonia is a movement disorder notoriously difficult to treat. While primary dystonia is classically considered to respond well to deep brain stimulation (DBS), treatment of secondary dystonia yields variable results. Patient selection should be done on a case-by-case basis. Clearly, there is a need to accumulate additional information with regard to prognostic factors that may aid neurosurgeons in selecting those patients in whom the disorder is most likely to respond favorably to pallidal DBS. The authors report the case of a 29-year-old man with secondary dystonia due to perinatal hypoxia. The most prominent symptom was what we have termed ectatocormia-that is, severe, fixed truncal hyperextension and retrocollis, exacerbated by phasic, twisting movements of the trunk and head. This made it impossible for the patient to maintain a normal upright posture or to walk. The patient underwent bilateral DBS of the globus pallidus internus (GPi), and the authors observed impressive improvement in motor abilities and function. The patient's body adopted the normal upright posture and he became able to walk again, 4 months after the commencement of GPi stimulation. This report, along with others, emphasizes that the GPi as an ideal target for alleviating axial tonic symptoms. The presence of normal MR imaging findings, a phenotypical purity of predominantly dystonic symptoms, and a younger age seem to favor a positive outcome.
肌张力障碍是一种运动障碍疾病，治疗难度极大。虽然原发性肌张力障碍通常被认为对深部脑刺激（DBS）反应良好，但继发性肌张力障碍的治疗效果则各不相同。应根据具体情况进行患者选择。显然，需要积累更多与预后因素相关的信息，这可能有助于神经外科医生选择那些疾病最有可能对苍白球 DBS 产生良好反应的患者。作者报告了一例由围产期缺氧引起的继发性肌张力障碍的 29 岁男性患者。最突出的症状是我们称之为异位性扭转，即严重的、固定的躯干过度伸展和后屈，伴有躯干和头部的阶段性扭曲运动。这使得患者无法保持正常的直立姿势或行走。患者接受了双侧苍白球 internus（GPi）的 DBS 治疗，作者观察到运动能力和功能有了显著的改善。在 GPi 刺激开始后 4 个月，患者的身体恢复了正常的直立姿势，再次能够行走。这一报告与其他报告一起强调了 GPi 作为缓解轴向紧张症状的理想靶点。正常的磁共振成像结果、以肌张力障碍症状为主的表型纯度以及年龄较小似乎有利于获得积极的结果。
