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髓鞘少突胶质细胞糖蛋白抗体相关的大脑皮质脑炎:一例报告,突出诊断挑战及治疗意义

Myelin oligodendrocyte glycoprotein antibody-associated cerebral cortical encephalitis: a case report highlighting diagnostic challenges and therapeutic implications.

作者信息

Liu MengChan, Li DaWei

机构信息

Department of Neurology, The Fourth People's Hospital of Shenzhen (Shenzhen Sami Medical Center), Shenzhen, China.

出版信息

Front Immunol. 2025 Jun 18;16:1619807. doi: 10.3389/fimmu.2025.1619807. eCollection 2025.

DOI:10.3389/fimmu.2025.1619807
PMID:40607435
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12213655/
Abstract

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) encompasses a spectrum of inflammatory demyelinating disorders of the central nervous system (CNS). Recognized clinical phenotypes include optic neuritis (ON), transverse myelitis (TM), acute disseminated encephalomyelitis (ADEM), brainstem encephalitis, aseptic meningitis, cortical encephalitis, demyelinating pseudotumor, and cranial nerve involvement. MOG antibody-associated cerebral cortical encephalitis (MOG-CCE) represents a rare but clinically significant subtype, often misdiagnosed due to heterogeneous clinical and neuroimaging features overlapping with other CNS disorders. We present a case of 36-year-old man with new-onset acute seizures and persistent headache. Initial brain magnetic resonance imaging (MRI) revealed no obvious signal abnormalities; however, subtle cortical swelling with sulcal effacement was identified in the left frontoparietal region, suggestive of focal cortical inflammation. Diagnostic workup revealed elevated MOG-IgG antibody titers in both serum and cerebrospinal fluid (CSF) using live cell-based assay. The patient demonstrated remarkable clinical response to combination immunotherapy with intravenous methylprednisolone and immunoglobulin. This case highlights the inclusion of MOG-CCE in the differential diagnosis of unexplained seizures and headaches accompanied by cortical swelling on neuroimaging, even in the absence of overt demyelinating lesions, and further underscores the critical role of early MOG-IgG testing and prompt immunotherapy to mitigate disease progression and improve neurological outcomes.

摘要

髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病(MOGAD)包括一系列中枢神经系统(CNS)炎性脱髓鞘疾病。公认的临床表型包括视神经炎(ON)、横贯性脊髓炎(TM)、急性播散性脑脊髓炎(ADEM)、脑干脑炎、无菌性脑膜炎、皮质脑炎、脱髓鞘假瘤和脑神经受累。MOG抗体相关的大脑皮质脑炎(MOG-CCE)是一种罕见但具有临床意义的亚型,由于其临床和神经影像学特征与其他中枢神经系统疾病重叠,常被误诊。我们报告一例36岁男性,新发急性癫痫发作和持续性头痛。最初的脑部磁共振成像(MRI)未发现明显信号异常;然而,在左额顶叶区域发现了伴有脑沟变浅的轻微皮质肿胀,提示局灶性皮质炎症。诊断检查显示,使用基于活细胞的检测方法,血清和脑脊液(CSF)中的MOG-IgG抗体滴度均升高。该患者对静脉注射甲泼尼龙和免疫球蛋白的联合免疫治疗表现出显著的临床反应。该病例强调,即使在没有明显脱髓鞘病变的情况下,在对伴有神经影像学皮质肿胀的不明原因癫痫发作和头痛进行鉴别诊断时,也应考虑MOG-CCE,进一步强调了早期MOG-IgG检测和及时免疫治疗对减轻疾病进展和改善神经学结局的关键作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2088/12213655/6ca3cd660880/fimmu-16-1619807-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2088/12213655/6ca3cd660880/fimmu-16-1619807-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2088/12213655/6ca3cd660880/fimmu-16-1619807-g001.jpg

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Epstein-Barr virus infection in patients with MOGAD.视神经脊髓炎谱系疾病(MOGAD)患者的爱泼斯坦-巴尔病毒感染
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Myelin Oligodendrocyte Glycoprotein-Antibody Associated Disease: An Updated Review of the Clinical Spectrum, Pathogenetic Mechanisms and Therapeutic Management.髓鞘少突胶质细胞糖蛋白抗体相关疾病:临床谱、发病机制及治疗管理的最新综述
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