Surgical Treatment of Patients with Rasmussen Encephalitis

Surgical Treatment of Patients with Rasmussen Encephalitis
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DOI:
10.1159/000355901
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发表时间:
2014-01-01
影响因子:
1.7
通讯作者:
Liu, Xingzhou
Liu, Xingzhou
中科院分区:
医学4区
文献类型:
--
作者:
Guan, Yuguang;Zhou, Jian;Liu, Xingzhou

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背景:拉斯穆森脑炎(Rasmussen encephalitis, RE)是一种罕见的进行性脑炎,可导致顽固性癫痫发作、认知能力下降和偏瘫。手术似乎是控制RE患者癫痫发作的唯一有效方法。目的:描述RE患者的临床、电生理、神经放射学和组织学表现,并评价其手术治疗的效果。方法:20例患者符合RE标准,其中左半球手术6例,右半球手术14例。手术方法包括解剖半球切除术、功能性肾盂切除术、半球切除术、病变切除术、多叶切除术、选择性切除术和功能皮质双极电凝。结果:平均随访时间5.45年(范围3 ~ 8年)。术后16例(80%)患者被评定为Engel i级。除1例患者为双侧RE外,其余患者术后认知能力均有所提高。术后大部分患者可独立行走,但手部精细运动功能丧失。1例患者在功能半球切除术后出现脑积水,本组无死亡病例。结论:半脑切除和半脑切除均可有效控制癫痫发作,提高RE患者的生活质量。(c) 2014 S. Karger AG,巴塞尔
Background: Rasmussen encephalitis (RE) is a rare progressive encephalitis that results in intractable seizures, cognitive decline and hemiparesis. Surgery seems to be the only effective way to control seizures in RE patients. Objective:To describe the clinical, electrophysiological, neuroradiological and histological findings of our patients with RE and to evaluate the outcome of their surgical treatment. Methods: A total of 20 patients were identified by the criteria of RE. Surgery was conducted in the left hemisphere in 6 patients and in the right hemisphere in 14. The surgical methods included anatomical hemispherectomy, functional hennispherectomy, hemispherotomy, lesion resection, multilobar resection, selective resection and bipolar electrocoagulation of functional cortexes. Results:The mean follow-up period was 5.45 years (range 3-8). After surgery, 16 patients (80%) were evaluated as being Engel class I. All of the patients had increases in cognitive abilities after surgery except 1 patient with bilateral RE. After surgery, most patients could walk independently, but the fine movement of the hands was lost. Postoperative hydrocephalus was observed in 1 patient after functional hemispherectomy, and there was no death in this series. Conclusion: Hemispherectomy and hemispherotonny were both confirmed as beneficial procedures in controlling seizures and improving quality of the life in RE cases. (c) 2014 S. Karger AG, Basel