丘脑脑深部电刺激术治疗肌阵挛肌张力障碍综合征的临床效果

Clinical effect of deep thalamic stimulation on myoclonus dystonia syndrome

目的探讨丘脑脑深部电刺激(DBS)术治疗肌阵挛肌张力障碍综合征(MDS)的临床效果。方法回顾性分析2011年5月至2017年4月于首都医科大学宣武医院功能神经外科接受丘脑腹外侧中间核DBS术治疗的4例男性MDS患者的临床资料,手术均分二期完成,一期在局部麻醉下植入颅内电极,观察到症状减轻后行二期手术植入延长线和脉冲发生器。患者入院后应用约翰霍普金斯跌倒风险评估量表进行跌倒评分,于术前、一期测试1周后、出院时采用Barthel指数量表进行日常生活能力评定,DBS术前和出院时采用统一性肌阵挛评定量表(UMRS)评估肌阵挛严重程度,应用BFMDRS运动量表和功能障碍量表进行肌张力障碍的运动障碍评分和功能障碍评分。结果 4例患者术前Barthel指数评分为40～70分,平均55分,属于中度生活依赖者,日常生活需要很大的帮助;跌倒评分为8～30分,平均16分,属于跌倒高风险患者。一期测试期间4例患者症状均减轻,行二期手术植入延长线和脉冲发生器,术后无严重并发症发生。出院时4例患者肌阵挛评分由平均36分降为平均1分,运动障碍评分和功能障碍评分分别由平均24分降为平均4分、平均13分降为平均2分,日常生活能力评分平均92分,顺利出院。结论丘脑DBS术对MDS患者有显著的治疗效果。在围术期做好风险评估与应对措施,仔细观察判断症状和刺激效果,是保障患者顺利完成手术的重要条件。

Objective To explore the clinical effect of thalamic deep brain stimulation（DBS） on myoclonus dystonia syndrome（MDS）. Methods From January 2011 to April 2017, 4 male patients with MDS who underwent Vim-DBS surgery at Xuanwu Hospital, Capital Medical University were retrospectively analyzed. The operation included two phases; the first phase was intracranial electrode implantation; the second phase was pulse generator implantation. Fall risk was assessed by Johns Hopkins Fall Risk Assessment Tool（JHFRAT）. Activity of daily living was assessed by Barthel index. Myoclonia severity was assessed by Unified Myoclonus Rating Scale（UMRS）. Movement disorder and functional disorder of dystonia were assessed by Burke-Fahn-Marsden Dystonia Rating Scale（BFMDRS）. Results Among the 4 patients, preoperative Barthel index was 40-70; the mean score was 55 with a moderate life dependence. Preoperative JHFRAT score was 8-30; the mean score was 16, indicating a high fall risk. The symptoms were alleviated after the first phase operation; no severe complication was observed after the second phase operation. At discharge, the myoclonia score, the movement and functional disorder scores were reduced; the mean score of Barthel index was 92. Conclusion Thalamic DBS has a good therapeutic effect on MDS.