Rasmussen脑炎免疫病理学研究进展  

Research advancement on immunopathology and characteristic double pathology of Rasmussen encephalitis

作　　者：乔娇 龚铭鲲 唐重阳 栾国明[1] QIAO Jiao;GONG Mingkun;TANG Chongyang;LUAN Guoming(Epilepsy Center,Sanbo Brain Hospital,Capital Medical University,Beijing 100093,China)

机构地区：[1]首都医科大学三博脑科医院癫痫中心,北京100093

出　　处：《癫痫杂志》2024年第1期78-82,共5页Journal of Epilepsy

基　　金：北京脑科学与类脑研究中心科研合作项目(2020-NKX-XM-02)。

摘　　要：Rasmussen脑炎(Rasmussen encephalitis,RE)是一种罕见的神经系统疾病(患病率为0.18/10万),主要影响儿童,平均发作年龄为6~7岁,临床表现为局灶性难治性癫痫、进行性的神经系统损害和认知功能下降。影像上主要是磁共振成像(Magnetic resonance imaging,MRI)体积和T2/液体衰减反转恢复信号的序列增加,之后进展为萎缩,正电子发射断层扫描(Positron emission tomography,PET)显示一侧半球代谢从高转为低。病理学主要表现为神经元丢失,血管淋巴袖套,小胶质细胞结节,其次10%的病例合并以皮质发育不良为主的双重病理。唯一的治疗方法是功能性半球切除术,但会导致永久性的运动和认知功能缺陷,免疫治疗只能暂且减缓,目前对于RE病因和发病机制尚不清楚,因此RE当前面临三大难题,一是如何在出现半球萎缩和神经认知障碍之前进行早期诊断;二是如何进行针对炎症的免疫治疗管理;三是术后的康复治疗如何进行能使神经系统功能最大程度地恢复。最近几年越来越多的证据表明RE脑中免疫微环境的改变是促进疾病进展的主要原因。本文针对RE的免疫病理学研究阐释了T淋巴细胞,小胶质细胞和星形胶质细胞在RE发生发展中的作用。Rasmussen’s encephalitis(RE)is a rare neurologic disorder,with an incidence of 0.18 per 100,000 population,primarily affecting children,with an average onset at 6-7 years of age.Clinical manifestations include focal refractory epilepsy,progressive neurological deterioration,and cognitive decline.In imaging,magnetic resonance imaging(MRI)typically shows an increase in volume and sequences with T2/fluid-attenuated inversion recovery(FLAIR)signal,followed by atrophy.Positron emission tomography(PET)demonstrates a decline in metabolism in one hemisphere.Pathologically,neuronal loss,perivascular lymphocytic cuffing,and small glial nodules are prominent,with 10%of cases exhibiting dual pathology,primarily cortical dysplasia.Functional hemispherectomy remains the only therapeutic option,albeit resulting in permanent motor and cognitive deficits.Immunomodulatory therapy provides only temporary relief.Currently,the etiology and pathogenesis of RE remain unclear,presenting three major challenges:early diagnosis before hemisphere atrophy and neurocognitive impairment,managing immune therapies targeting inflammation,and determining rehabilitation post-surgery to maximize neurological recovery.Emerging evidence suggests that alterations in the brain’s immune microenvironment play a pivotal role in disease progression.This article focuses on the immunopathological aspects of RE,elucidating the roles of T lymphocytes,small glial cells,and astrocytes in the development of RE.

关 键 词：免疫病理学 RASMUSSEN脑炎 癫痫 

分 类 号：R742.9[医药卫生—神经病学与精神病学]