9例初诊腊斯默森脑炎患者的临床探讨

Treatment of newly diagnosed Rasmussen encephalitis

目的：通过初诊腊斯默森脑炎（Rasmussen encephalitis，RE）患者的临床处理过程，明确治疗方法的选择。方法：按照欧洲共识的诊断标准，初诊RE9例。分析患者的病史、病程、发作特点、脑电图、MRI和PET表现，动态观察患者的MRI和PET，其中6例患者建议大脑半球切除手术治疗，3例患者建议颅脑病理活检。结果：所有患者均表现为一侧起始的部分性发作，多种发作形式，6例患者出现部分性癫痫持续状态（epilepsia partialis continua，EPC）。就诊时7例患者有轻偏瘫。所有患者MRI均出现半球萎缩，PET示半球性或半球多灶性低代谢，脑电图示半球的慢活动。在临床处理上，4例行半球切除术，病理学检查支持RE诊断，其中2例合并局灶性皮质发育不良（FCD），为双重病理改变；另1例脑活检后排除RE。另外4例拒绝手术和脑活检。结论：对于初诊的RE患者，MRI及PET的动态观察是RE诊断的重要方法，脑手术组织病理学和脑活检亦可明确诊断。

Objective：To determine the treatment of Rasmussen encephalitis （RE） through clinical management of the disease. Methods：According to european diagnostic standard,9 patients with Rasmus- sen encephalitis（RE） were selected in our study. The patienfs medical history, duration, seizure charac- teristics, EEG and magnetic resonance imaging （MRI） and positron emission tomography （PET）. Dy- namic Observation of patients＇ MRI and PET were also conducted. Hemispherectomy were recommended for 6 patients and 3 patients recceived histopathological examination. Results： All patients showed uniheimisphere in partial seizures multiple forms, and 6 cases were presented with epilepsia partialis continua （EPC）. Hemiparesis occurred in 7 cases during clinical treament in our hospital. MRI suggested that all patients had unihemispheric atrophy; Unihemisphere or multiple focal regions hypometabolism were seen on PET, EEG revealed slow activities in unilateral hemisphere. 4 cases hemispherectomy and 4 cases have done it. Pathologic examination supported the diagnosis of RE, including 2 patients with focal cortical dysplasia （FCD）, bearing double pathological changes. 1 case was excluded from REI the other 14 cases refused surgery and biopsy. Conclusion： For newly diagnosed patients with RE, dynamic observation of MRI and PET are the keys to the diagnosis of RE; Cranial encephalopathy and biopsy are recommended for newly diagnosed patients.