4.5 Article

A Special Case of Relapsing-Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis

期刊

BRAIN SCIENCES
卷 13, 期 1, 页码 -

出版社

MDPI
DOI: 10.3390/brainsci13010017

关键词

relapsing-remitting bilateral encephalitis; Rasmussen encephalitis; encephalomalacia; brain biopsy; rituximab

向作者/读者索取更多资源

A nine-year-old boy presented with headache, cognitive decline, and hemiparesis. Brain MRI revealed bilateral cortical lesions with new lesions developing over five years. Initial diagnosis of primary angiitis of the central nervous system was considered. Treatment with corticosteroids, immunoglobulins, and cyclophosphamide worsened symptoms. Brain biopsy after five years showed pathology changes consistent with RE. Treatment with rituximab resulted in stable lesions on follow-up MRI. This case highlights the uniqueness and treatment challenges of this immune-mediated condition.
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.

作者

我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。

评论

主要评分

4.5
评分不足

次要评分

新颖性
-
重要性
-
科学严谨性
-
评价这篇论文

推荐

暂无数据
暂无数据