Presentation, diagnosis and treatment of bilateral Rasmussen's encephalitis in a 12-year-old female
Presentation, diagnosis and treatment of bilateral Rasmussen's encephalitis in a 12-year-old female
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DOI：
10.1684/epd.2013.0594
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发表时间：
2013-09-01
影响因子：
2.3
通讯作者：
Greiner, Hansel M.
中科院分区：
文献类型：
--
作者：
Peariso, Katrina;Standridge, Shannon M.;Greiner, Hansel M.
瞄准描述1例12岁女性患者的临床病程和病理诊断，该患者表现为右半球癫痫和皮质功能障碍的急性综合征，脑MRI显示左侧大脑和右侧小脑半球萎缩。结果患者出现偶发部分性癫痫发作，包括左小腿感觉，随后为左腿阵挛性抽搐。初次脑部MRI显示左侧大脑和右侧小脑萎缩，左侧顶叶区域T2高信号。6个月后，癫痫发作频率增加，症状学演变为包括左侧面部、手臂和腿部的频繁阵挛性运动以及左臂和腿部的部分持续性癫痫（EPC）。左腿逐渐无力，左臂无力程度较轻，此时MRI显示右额叶T2高信号。对副肿瘤、线粒体和遗传性癫痫综合征的广泛评估未显示出结果。在活检评估，慢性T细胞介导的脑炎表现在双侧额叶。免疫调节治疗导致她的癫痫发作频率和运动功能有所改善。结论拉斯穆森脑炎可能是一个具有挑战性的诊断。患者的临床病史，包括EPC，双侧额叶活检证实T细胞介导的脑炎支持双侧拉斯穆森脑炎的诊断。本例突出了诊断的挑战和治疗的困境，出现在青少年提出双边炎症病变的拉斯穆森脑炎。
Aim. To describe the clinical course and pathological diagnosis of a 12-year-old female who presented with an acute syndrome of right hemispheric epilepsy and cortical dysfunction and brain MRI demonstrating atrophy of the left cerebral and right cerebellar hemispheres. Results. The patient presented with occasional partial seizures consisting of a left calf sensation followed by left leg clonic jerking. Initial brain MRI showed left cerebral and right cerebellar atrophy with T2 hyperintensity in the left parietal region. After six months, the seizure frequency increased and semiology evolved to include frequent clonic movements of the left side of the face, arm and leg and epilepsia partialis continua (EPC) of the left arm and leg. There was progressive weakness of the left leg and, to a lesser extent, her left arm. MRI at this time demonstrated an additional T2 hyperintensity in the right frontal lobe. An extensive evaluation for paraneoplastic, mitochondrial, and genetic epilepsy syndromes was unrevealing. On biopsy evaluation, chronic T-cell mediated encephalitis was demonstrated within bilateral frontal lobes. Treatment with immunomodulatory therapy resulted in some improvement in her seizure frequency and motor function. Conclusion. Rasmussen's encephalitis can be a challenging diagnosis. The patient's clinical history, including EPC, with bilateral frontal lobe biopsies confirming a T-cell mediated encephalitis supports a diagnosis of bilateral Rasmussen encephalitis. This case highlights the diagnostic challenges and treatment dilemmas that arise in an adolescent presenting with bilateral inflammatory lesions of Rasmussen's encephalitis.