全身性扭转痉挛的立体定向手术治疗报告

A report of generalized torsion dystonia treated with stereotactic neurosurgery

目的 评估脑立体定向手术对全身性扭转痉挛的临床治疗效果。方法 通过MRI定位手术靶点,微电极脑细胞电生理记录技术在术中对靶点行功能确认,对19例全身性扭转痉挛患者进行了立体定向手术治疗,其中单侧手术8例,同期双侧手术9例,分期双侧手术2例。毁损靶点包括苍白球腹后部、丘脑底核及丘脑腹外侧核。采用Burke的扭转痉挛运动功能评分法对6例患者进行手术疗效定量评估。结果 全部19例患者术后手术对侧的肢体肌张力基本恢复正常,运动功能得到不同程度的改善,术后无严重并发症发生。在行疗效评估的6例患者中,3例单侧手术患者术后1周、1个月、3个月时的改善率分别为（13.3±4.4）％、（27.2±5.6）％和（29.7±11.1）％,主要表现为手术对侧肢体症状明显改善,躯干有轻度改善;而3例同期双侧手术患者肢体和躯干症状均得到明显改善,术后1周、1个月、3个月时的改善率分别为（25.9±7.6）％、（58.1±1.7）％和（65.4±6.9）％。结论 应用立体定向神经外科手术治疗全身性扭转痉挛是一种安全有效的方法。

Objective To evaluate the clinical therapeutic effect of stereotactic neurosurgery for general torsion dystonia. Methods Nineteen patients underwent stereotactic neurosurgery by MRI and microelectrode electrophysiological recording for intraoperative target localization. Among them,8 underwent unilateral operation, 9 underwent simultaneous bilateral operations and the remaining 2 received bilateral operations respectively at different times. Burke-Fahn dystonia movement scale(BFDMS) was used in 6 patients for objective assessments before and after operation. Among them,3 underwent unilateral PVP, and the other 3 received simultaneous bilateral operations. Results The improvement in abnormal movements were found in all the 19 patients. In 3 patients treated by unilateral PVP, the mean BFDMS was improved by (13. 3±4.4)% , (27. 2 ±5. 6)% , and (29.7±11.1)% respectively 1 week, 1 month and 3 months after operation. Significant improvement was found in the contralateral limbs, and moderate improvement in the trunk, while bilateral operations significantly improved all dystonic symptoms. The mean BFDMS of 3 patients was improved by (25. 9±7. 6)% , (58. 1± 1.7)%, and (65. 4±6. 9)% respectively 1 week,l month and 3 months after operation. Conclusion It is suggested that PVP and STN lesioning is a safe and effective therapy for the patients with intractable general torsion dystonia.