A Special Case of Relapsing-Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis.
A Special Case of Relapsing-Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis.
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DOI：
10.3390/brainsci13010017
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发表时间：
2022-12-22
期刊：
影响因子：
3.3
通讯作者：
Wu, Songdi
中科院分区：
文献类型：
--
作者：
Liu, Pei;Lin, Xuemei;Zong, Shenghua;Yan, Yan;Liu, Zhongzhong;Lu, Qingli;Chang, Qiaoqiao;Wu, Songdi
关键词：
一名9岁男孩，表现为头痛，进行性轻度认知能力下降和偏瘫，但无临床癫痫发作（脑电图异常）。脑磁共振成像（MRI）显示双侧皮层病变主要在右半球，在旧病变周围的额叶、顶叶、枕叶和颞叶出现新病变，T1表现为带状或环状强化，病程5年。最初考虑怀疑诊断为中枢神经系统原发性脉管炎。在大剂量皮质类固醇、静脉注射免疫球蛋白和每月脉冲环磷酰胺治疗后，他的症状随着颅内病变的进展而恶化。发病后近5年行右额叶脑组织活检；皮层内可见明显的神经元丢失、小胶质结节、实质及血管周围淋巴细胞浸润，与RE病理改变一致。令人鼓舞的是，在一疗程的利妥昔单抗治疗后，脑部MRI检查显示病灶趋于稳定。显然，它是免疫介导的，但不严格符合任何已知的疾病实体，尽管它与RE相似。我们总结了这个独特的病例，包括临床特征、影像学和病理发现。我们还讨论了诊断和治疗，重点是与RE以及其他可能的神经系统疾病的比较。
A nine-year-old boy manifested with headache, progressive mild cognitive decline and hemiparesis, but without clinical epileptic seizures (with abnormal EEG waves). Brain magnetic resonance imaging (MRI) showed bilateral cortical lesions mainly on the right hemisphere, and new lesions developed in frontal, parietal, occipital and temporal lobes around the old lesions presenting as a lace-like or ring-like enhancement in T1 with contrast over a disease course of five years. A suspected diagnosis of primary angiitis of the central nervous system was initially considered. Treated with high-dose corticosteroids, intravenous immunoglobulins and monthly pulse cyclophosphamide, his symptoms worsened with the intracranial lesion progression. Brain biopsy of the right frontal lobe was performed nearly five years after onset; prominent neuronal loss, a microglial nodule, as well as parenchymal and perivascular lymphocytic infiltrate within the cortex were found, which coincided with RE pathology changes. Encouragingly, after a regimen of rituximab, lesions on the follow-up brain MRI tended to be stable. Apparently, it was immune-mediated, but did not strictly fit any known disease entity, although it was similar to RE. We summarize this unique case, including clinical characteristics, imaging and pathology findings. We also discuss the diagnosis and treatment, focusing on comparison to RE as well as other possible neurological diseases.
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