Rasmussen脑炎临床病理研究  

Rasmussen＇s encephalitis：a cfinicopathologic study

作　　者：王丹丹[1] 桂秋萍[3] 郑重[2] 罗阳[4] 阮静[4] 胡京霞[2] 林久銮[4] 王世伦[2] 石岩芳[4] 田宏[4] 张光明[4] 周文静[4] 

机构地区：[1]首都医科大学宣武医院病理科,北京100053 [2]清华大学玉泉医院病理科 [3]解放军总医院病理科 [4]清华大学玉泉医院神经外科五病区

出　　处：《中华神经外科杂志》2013年第3期273-276,共4页Chinese Journal of Neurosurgery

基　　金：首都医学发展科研基金（2009-3048）

摘　　要：目的探讨Rasmussen脑炎的的发病机制、临床表现、神经影像学表现和病理特征。方法结合临床、影像、HE和免疫组化方法对6例大脑半球切除的Rasmussen脑炎患者的资料进行回顾分析（手术时年龄3．5～11．0岁）。结果患者均为难治性癫痫，患病时间1—4年，均有不同程度偏瘫。术后随访4个月-3年8个月，预后均良好，正规服用抗癫痫药物，均无癫痫发作。组织病理学上所有6例均可见局灶软脑膜下星形胶质细胞增生，脑实质淋巴细胞及小胶质细胞结节散在分布，5例脑实质血管周围慢性淋巴细胞浸润，淋巴套袖形成，所有淋巴细胞以T淋巴细胞为主（CD3＋，CD5＋，CD7＋），且有细胞毒性T淋巴细胞为主（CD8＋，GranzymeB＋，CD4-），B淋巴细胞罕见（CD79a-，CD20-）。未见病毒包涵体。结论Rasmussen脑炎中淋巴细胞为细胞毒性T细胞来源，患侧大脑半球切除是治疗Rasmussen脑炎、阻止疾病进展的有效的方法。Objective To discuss the clinicopathologic characteristics of surgically - removed tissues in patients with Rasmussen＇s encephalitis. Methods The elinicopathologic features and immunohistochemical profiles of 6 patients with Rasmussen encephalitis （age range, 3.5 - 11 years at surgery） were retrospectively reviewed. Results All patients had medically intractable seizures （ 1 to 4 years duration） ; all developed unilateral hemiparesis. Histologically, all cases were characterized by diffuse proliferation of microglial cells, and focal microglial nodule formations. All cases showed intraparencbymal lymphocytic infiltrate. 5 cases showed perivascular cuffing by lymphocytes. 3 cases showed lymphocytic meningitis. All the lymphocytes consist of primarily of T cell immunophenotype （ CD3 ＋ , CD5 ＋ , CD7 ＋ ）. A predominance of CD8 ＋ lymphoid cells was observed. All cases had rare B lymphocytes （ CD79a -, CD20 - ）. Viral inclusions were not noted. Conclusions Most lymphoid cells in Rasmussen＇s encephalitis have a T- -cytotoxic immunophenotype. Hemispherectomy is the only effective therapy.

关 键 词：RASMUSSEN脑炎 癫痫 大脑半球切除术 慢性脑炎 

分 类 号：R512.3[医药卫生—内科学]