脑深部电刺激治疗小儿全身性肌张力障碍的疗效分析

Q4 Medicine

中华神经外科杂志 Pub Date : 2019-12-28 DOI:10.3760/CMA.J.ISSN.1001-2346.2019.12.002

F. Ji, Dou Wanchen, W. Xinhua, Wang Lin, Yang Yingmai, Guo Yi, M. Wenbin, Wang Renzhi

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引用次数: 0

摘要

目的探讨深部脑刺激(DBS)治疗儿童全身性肌张力障碍的可行性及疗效。方法回顾性分析12例全身性肌张力障碍患儿的临床资料。所有患者均于2015年8月至2018年7月在中国医学科学院北京协和医院神经外科接受DBS治疗。遗传病因检测显示DYT-1型5例，DYT-6型1例，特发性全身性肌张力障碍6例。所有患儿术前均行头部敏感性加权成像(SWI)定位靶。采用Burke-Fahn-Marsden张力障碍评定量表(BFMDRS)评估患儿的临床疗效。此外，从我们的数据库中选择性别、基因型匹配的全身性肌张力障碍成人患者12例。我们比较和分析儿童和成人内苍白球(GPi)的解剖差异，以评估手术的可行性。结果所有患者均成功植入电极，无手术相关并发症。平均随访时间(19.8±9.6)个月(12 ~ 47个月)。术后3个月和1年BFMDRS评分中位数分别为15.5(10-50)和12.5(4-48)分，与术前评分49.0(28-103)分比较差异有统计学意义(8岁组P均为(75.5±4.7)mm (70.0-82.8 mm)，与成年组比较差异无统计学意义(P=0.190)。结论DBS治疗病因明确的小儿全身性肌张力障碍可改善临床症状，无严重并发症。0 ~ 8岁儿童GPi的解剖位置与成人无显著差异。但其远期预后及电极移位等并发症的发生率仍需进一步的随访研究。关键词:肌张力障碍;孩子;脑深部电刺激;广义

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Outcome analysis of deep brain stimulation for pediatric generalized dystonia

Objective To explore the feasibility and efficacy of deep brain stimulation (DBS) in the treatment of generalized dystonia in children. Methods A retrospective study was designed to investigate the clinical data of 12 children with generalized dystonia. All of them were treated with DBS at Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences from August 2015 to July 2018. Genetic etiology testing showed that 5 cases were type DYT-1, 1 case was type DYT-6, and 6 cases were idiopathic generalized dystonia. All children underwent head susceptibility-weighted imaging (SWI) to locate the target before surgery. The clinical efficacy of the children was assessed using the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). In addition, 12 adult patients with generalized dystonia with matched gender, genotype were selected from our database. We compared and analyzed the difference in anatomy of the internal globus pallidus (GPi) between children and adults to evaluate the feasibility of operation. Results All patients were successfully implanted with electrodes without surgery-related complications. The average duration of follow-up was (19.8±9.6) months (12-47 months). The median BFMDRS scores at 3 months and 1 year after surgery were 15.5 (10-50) and 12.5 (4-48) respectively, which, compared with the preoperative score of 49.0(28-103), were significantly different (both P 8 years old was (75.5±4.7)mm (70.0-82.8 mm), which was not statistically different from that in the adults group (P=0.190). Conclusions Preliminary findings suggest that DBS could improve clinical symptoms without serious complications for the treatment of pediatric generalized dystonia with clear etiological causes. The anatomical location of the GPi in children >8 years old is not significantly different from that in adults. However, the long-term prognosis and incidence of complications such as electrode displacement still require more follow-up studies. Key words: Dystonia; Child; Deep brain stimulation; Generalized

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来源期刊

中华神经外科杂志 Medicine-Surgery

CiteScore

0.10

自引率

0.00%

发文量

10706

期刊介绍： Chinese Journal of Neurosurgery is one of the series of journals organized by the Chinese Medical Association under the supervision of the China Association for Science and Technology. The journal is aimed at neurosurgeons and related researchers, and reports on the leading scientific research results and clinical experience in the field of neurosurgery, as well as the basic theoretical research closely related to neurosurgery.Chinese Journal of Neurosurgery has been included in many famous domestic search organizations, such as China Knowledge Resources Database, China Biomedical Journal Citation Database, Chinese Biomedical Journal Literature Database, China Science Citation Database, China Biomedical Literature Database, China Science and Technology Paper Citation Statistical Analysis Database, and China Science and Technology Journal Full Text Database, Wanfang Data Database of Medical Journals, etc.