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

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 f...

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Autores principales: Liu, Pei, Lin, Xuemei, Zong, Shenghua, Yan, Yan, Liu, Zhongzhong, Lu, Qingli, Chang, Qiaoqiao, Wu, Songdi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9856488/
https://www.ncbi.nlm.nih.gov/pubmed/36671998
http://dx.doi.org/10.3390/brainsci13010017
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author Liu, Pei
Lin, Xuemei
Zong, Shenghua
Yan, Yan
Liu, Zhongzhong
Lu, Qingli
Chang, Qiaoqiao
Wu, Songdi
author_facet Liu, Pei
Lin, Xuemei
Zong, Shenghua
Yan, Yan
Liu, Zhongzhong
Lu, Qingli
Chang, Qiaoqiao
Wu, Songdi
author_sort Liu, Pei
collection PubMed
description 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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spelling pubmed-98564882023-01-21 A Special Case of Relapsing–Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis Liu, Pei Lin, Xuemei Zong, Shenghua Yan, Yan Liu, Zhongzhong Lu, Qingli Chang, Qiaoqiao Wu, Songdi Brain Sci Case Report 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. MDPI 2022-12-22 /pmc/articles/PMC9856488/ /pubmed/36671998 http://dx.doi.org/10.3390/brainsci13010017 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Liu, Pei
Lin, Xuemei
Zong, Shenghua
Yan, Yan
Liu, Zhongzhong
Lu, Qingli
Chang, Qiaoqiao
Wu, Songdi
A Special Case of Relapsing–Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis
title A Special Case of Relapsing–Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis
title_full A Special Case of Relapsing–Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis
title_fullStr A Special Case of Relapsing–Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis
title_full_unstemmed A Special Case of Relapsing–Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis
title_short A Special Case of Relapsing–Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis
title_sort special case of relapsing–remitting bilateral encephalitis: without epilepsy, but responding to rituximab and with a brain biopsy coinciding with rasmussen encephalitis
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9856488/
https://www.ncbi.nlm.nih.gov/pubmed/36671998
http://dx.doi.org/10.3390/brainsci13010017
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