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继发于髓鞘少突胶质细胞糖蛋白抗体病的伴有广泛皮质脑磁共振成像改变的脑炎

Encephalitis with Extensive Cortical Brain Magnetic Resonance Imaging Changes Secondary to Myelin Oligodendrocyte Glycoprotein Antibody Disease.

作者信息

Satyasi Vivek, Mainali Gayatra, Chatterjee Jahnavi, Paudel Sita

机构信息

Department of Neurology, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA.

Department of Pediatric Neurology, Penn State Milton S. Hershey Medical Center, Hershey, PA, USA.

出版信息

Am J Case Rep. 2022 Aug 31;23:e936361. doi: 10.12659/AJCR.936361.

Abstract

BACKGROUND The relatively new autoimmune disorder, anti-myelin oligodendrocyte glycoprotein (MOG) disease is particularly interesting because of its broad range of presentations. This entity's appearance on magnetic resonance imaging (MRI) of the brain often makes identifying this disease a challenging process. Younger patients tend to present with an acute disseminated encephalomyelitis picture, with encephalopathy and multifocal neurological signs, while older patients are more likely to present with optic neuritis. We, however, report an atypical case of a patient who presented with encephalopathy, seizures, and significant cortical and subcortical gray matter involvement and was found to have anti-MOG positivity in serum. CASE REPORT A 17-month-old previously healthy boy presented to Emergency Department with fever, lethargy, vomiting, and left-sided weakness. Eventually, he required intubation due to a prolonged seizure. Continuous electroencephalogram captured several focal seizures, and MRI of the brain showed cortical and subcortical T2 hyperintensities. After extensive laboratory evaluation, he tested positive for anti-MOG antibody. He was empirically started on high-dose intravenous pulse methylprednisolone, followed by plasma exchange, given the poor response to the intravenous steroids. At the 5-month follow-up, the results of the neurological examination had dramatically improved, and MRI findings had largely resolved. CONCLUSIONS This case highlights the importance of suspecting anti-MOG antibody-mediated encephalitis, even while ruling out infectious etiologies, in children presenting with encephalopathy, seizures and MRI abnormalities. Prompt recognition would allow for less delay in treatment and hopefully improve prognosis.

摘要

背景 相对较新的自身免疫性疾病——抗髓鞘少突胶质细胞糖蛋白(MOG)病因其广泛的临床表现而格外引人关注。该疾病在脑部磁共振成像(MRI)上的表现常常使诊断过程颇具挑战性。年轻患者往往表现为急性播散性脑脊髓炎样症状,伴有脑病和多灶性神经体征,而老年患者更易出现视神经炎。然而,我们报告了一例非典型病例,该患者表现为脑病、癫痫发作,且大脑皮质和皮质下灰质有明显受累,血清抗MOG检测呈阳性。病例报告 一名17个月大、此前健康的男孩因发热、嗜睡、呕吐和左侧肢体无力被送往急诊科。最终,由于癫痫持续发作,他需要插管。连续脑电图监测捕捉到数次局灶性癫痫发作,脑部MRI显示皮质和皮质下T2高信号。经过全面的实验室检查,他的抗MOG抗体检测呈阳性。鉴于对静脉注射类固醇反应不佳,经验性地开始给予大剂量静脉脉冲甲基强的松龙治疗,随后进行血浆置换。在5个月的随访中,神经检查结果显著改善,MRI表现基本消失。结论 本病例强调,对于出现脑病、癫痫发作和MRI异常的儿童,即使在排除感染性病因时,也应怀疑抗MOG抗体介导的脑炎。及时识别可减少治疗延误,有望改善预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/384a/9444166/675d6443cf4f/amjcaserep-23-e936361-g001.jpg

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