Chinese Journal of Contemporary Neurology and Neurosurgery ›› 2020, Vol. 20 ›› Issue (9): 821-826. doi: 10.3969/j.issn.1672-6731.2020.09.011

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Clinical and EEG characteristics of Rasmussen encephalitis in different age groups

YAN Zhao-fen1, WANG Jing1, LUAN Guo-ming2, ZHOU Jian2, GUANG Yu-guang2, ZHAI Feng2, WANG Meng-yang1   

  1. 1 Department of Neurology, Center of Epilepsy, Sanbo Brain Hospital, Capital Medical University, Beijing 100093, China;
    2 Department of Neurosurgery, Center of Epilepsy, Sanbo Brain Hospital, Capital Medical University, Beijing 100093, China
  • Received:2020-09-02 Online:2020-09-25 Published:2020-09-30
  • Supported by:

    This study was supported by the National Natural Sciences and Foundation of China (No. 81671285).

不同年龄阶段Rasmussen脑炎临床与脑电图特征

闫兆芬1, 王静1, 栾国明2, 周健2, 光宇光2, 翟峰2, 王梦阳1   

  1. 1 100093 北京, 首都医科大学三博脑科医院癫痫中心神经内科;
    2 100093 北京, 首都医科大学三博脑科医院癫痫中心神经外科
  • 通讯作者: 王梦阳,Email:niannujiao1@sina.com
  • 基金资助:

    国家自然科学基金资助项目(项目编号:81671285)

Abstract:

Objective To summarize the clinical and EEG features of patients with different ages of Rasmussen encephalitis to evaluate the efficacy of different surgical methods. Methods and Results According to the age of onset, the patients with Rasmussen encephalitis were divided into 2 groups, one group was under 6 years old (≤ 6 year group, n=18), the other group was over 6 years old (>6 year group, n=18). The medial duration of advancing to actue phase was 5 (2, 11) months in ≤ 6 year group and 12.00 (5.50, 24.00) months in >6 year group, respectively (P=0.014). Nine months after onset, 15 cases advanced to acute phase in <6 year group, and 6 cases advanced acute phase in >6 year group (P=0.006). EEG showed persistent/intermittent slow wave activity over the affected hemisphere (35 patients). Nine months after onset, 2 groups of patients with multi-region epileptiform discharges have advanced to acute phase from prodromal phase (10 cases in ≤ 6 year group and 2 in >6 year group; P=0.036). The prognosis of functional hemispherectomy was Engel Ⅰ in 21/27 cases (11 cases in ≤ 6 year group and 10 in >6 year group), anatomic hemispherectomy Engel Ⅰ in 8/8 cases (4 cases in both ≤ 6 year group and >6 year group, respectively). Conclusions The earlier the onset age of Rasmussen encephalitis patients, the faster the disease progresses. The EEG shows continuous slow wave and multi-epileptic discharge in the affected side hemisphere. Anatomic hemispherectomy and/or functional hemispherectomy still remains of be the only cure for the seizures caused by Rasmussen encephalitis.

Key words: Encephalitis, Epilepsy, Electroencephalography, Neurosurgical procedures

摘要:

目的 总结不同年龄阶段Rasmussen脑炎患者临床及脑电图特征,评价不同手术方式疗效。方法与结果 根据发病年龄将Rasmussen脑炎患儿分为≤ 6岁组(18例)和>6岁组(18例),两组脑电图主要表现为一侧半球持续性和(或)间歇性慢波(35例);≤ 6岁组患儿进入急性期中位病程为5(2,11)个月、>6岁组12.00(5.50,24.00)个月(P=0.014)。发病9个月时,≤ 6岁组进入急性期病例为15例、>6岁组6例(P=0.006),多脑区性样放电患儿均由前驱期进入急性期(≤ 6岁组10例、>6岁组2例,P=0.036)。功能性半球离断术预后达EngelⅠ级者21/27例(≤ 6岁组11例、>6岁组10例)、解剖性半球切除术达EngelⅠ级者8/8例(≤ 6岁组4例、>6岁组4例)。。结论 Rasmussen脑炎患者的脑电图主要表现为患侧半球持续性慢波或多脑区性样放电,发病年龄越早、病情进展越迅速。解剖性半球切除术和(或)功能性半球离断术是控制发作的唯一有效办法。

关键词: 脑炎, 癫, 脑电描记术, 神经外科手术