Rasmussen脑炎(附1例报告及文献复习)  

A Case Report of Rasmussen’s Encephalitis and Literature Review

作　　者：张桐 王媛媛 王明光 邓星强 刘晓鸣 ZHANG Tong;WANG Yuanyuan;WANG Ming-guang;DENG Xing-qiang;LIU Xiao-ming(Xuzhou Children’s Hospital,Xuzhou 221006,China)

机构地区：[1]徐州医科大学附属徐州儿童医院,徐州221006

出　　处：《中国临床神经科学》2024年第1期57-62,共6页Chinese Journal of Clinical Neurosciences

摘　　要：目的探讨Rasmussen脑炎的临床特点及诊断。方法回顾分析1例Rasmussen脑炎患儿的临床资料,并复习相关文献进行分析。结果女性,7岁。起病时表现为间断右侧眼睑抽动伴右侧肢体抖动,起病后有认知损害,予抗癫癎药及免疫抑制剂等治疗病情好转。病程约6个月时加重,出现累及右侧面部及右侧肢体的局灶性癫癎持续状态和语言功能障碍。患儿有定向障碍,记忆力减退,右侧肢体肌力4级,右侧巴氏征阳性。头颅MRI病初时仅有左侧额叶局部异常信号,一过性累及左侧颞叶,逐渐发展为左侧大脑半球萎缩。脑电图可见左侧半球弥漫性慢波,夹杂尖波、棘波发放。行左侧额、顶、岛叶癫癎灶切除术及大脑半球离断术后,患儿再无癫癎发作。能独走,右侧肢体肌力4级,精细动作差,能说简单句。结论临床表现为局灶性癫癎持续状态、进行性神经功能和认知损害,头颅影像学表现为大脑皮质进行性局部萎缩,脑电图表现为一侧大脑半球慢波可伴尖波、棘波,应考虑Rasmussen脑炎。Aim To explore the clinical features and diagnostic methods of Rasmussen’s encephalitis.Methods The clinical manifestations,electroencephalogram and cranial imaging results of one case of Rasmussen’s encephalitis were retrospectively analyzed,and the relevant literature was reviewed.Results The seven-year-old girl presented with right eyelid twitching and right limb tremor.Cognitive decline occurs after onset.The condition improved after the treatment of antiepileptic and immunosuppressive,and worsen after 6 months,with epileptic status on the right face and limbs,language dysfunction,disorientation on physical examination,impaired memory,calculation and reasoning ability,and muscle strength of the right limbs atⅣlevel,and positive pyramidal sign on the right side.Early cranial MRI showed abnormal signals in the left frontal lobe and transient involvement in the left temporal lobe,which gradually developed into atrophy of the left cerebral hemisphere.The electroencephalogram showed slow wave in the left hemisphere,mixed with sharp wave.After the surgery,the girl got seizure free and can walk alone,but the muscle strength of the right limbs isⅣlevel,and fine movement is poor and communicate simply with simple words.Conclusion Patient with the clinical manifestations such as refractory epilepsy,progressive neurological and cognitive dysfunction,especially epilepsia partialis continua and progressive local atrophy of the unilateral cerebral cortex,and electroencephalogram presenting slow and sharp waves mainly in unilateral hemisphere should be considered as Rasmussen’s encephalitis.

关 键 词：RASMUSSEN脑炎 偏瘫 难治性癫癎 局灶性癫癎持续状态 儿童 

分 类 号：R742[医药卫生—神经病学与精神病学]