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.

abstractsObjective To discuss the clinicopathologic characteristics of surgically-removed tissues in patients with Rasmussen' s encephalitis.Methods The clinicopathologic features and immunohistochemical profiles of 6 patients with Rasmussen encephalitis (age range,3.5-11years 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 intraparenchymal 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.