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伴有髓鞘碱性蛋白升高的 FLAMES 继以脊髓炎。

FLAMES with Elevated Myelin Basic Protein Followed by Myelitis.

机构信息

Department of Neurology, Graduate School of Medicine, Nippon Medical School, Japan.

Department of Neurology, Tohoku University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2022 Dec 1;61(23):3585-3588. doi: 10.2169/internalmedicine.9439-22. Epub 2022 May 7.

DOI:10.2169/internalmedicine.9439-22
PMID:35527028
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9790785/
Abstract

The pathophysiology of unilateral cortical fluid-attenuated inversion recovery (FLAIR)-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis with seizures (FLAMES) is unclear. A 26-year-old man was referred because of a seizure. FLAIR showed an increased signal intensity and swelling of the right frontal cortex. His symptoms and imaging abnormalities were improved after intravenous methylprednisolone therapy. MOG antibody was detected both in serum and cerebrospinal fluid (CSF). Therefore, the patient was diagnosed with FLAMES. Myelin basic protein (MBP) was elevated in CSF. The high MBP value in the CSF in the present case suggested that demyelination as well as inflammation can occur in some FLAMES patients.

摘要

抗髓鞘少突胶质细胞糖蛋白(MOG)相关脑炎伴发癫痫(FLAMES)患者单侧皮质液体衰减反转恢复(FLAIR)高信号病变的病理生理学机制尚不清楚。一名 26 岁男性因癫痫发作被转来我院。FLAIR 显示右侧额叶皮质信号强度增高和肿胀。他的症状和影像学异常在接受静脉注射甲基强的松龙治疗后得到改善。血清和脑脊液(CSF)中均检测到 MOG 抗体。因此,该患者被诊断为 FLAMES。CSF 中的髓鞘碱性蛋白(MBP)升高。本例中 CSF 中 MBP 值升高提示,一些 FLAMES 患者可能同时发生脱髓鞘和炎症。

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本文引用的文献

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Clinical Reasoning: An 11-year-old girl with focal seizures, fevers, and unilateral, enhancing cortical lesions.临床推理:一名11岁女孩,患有局灶性癫痫发作、发热,以及单侧强化性皮质病变。
Neurology. 2020 Dec 8;95(23):e3153-e3159. doi: 10.1212/WNL.0000000000010655. Epub 2020 Aug 14.
2
Unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES): characterization of a distinct clinico-radiographic syndrome.抗髓鞘少突胶质细胞糖蛋白抗体相关脑炎伴发癫痫的单侧皮质 FLAIR 高信号病变(FLAMES):一种独特的临床-影像学综合征的特征。
J Neurol. 2019 Oct;266(10):2481-2487. doi: 10.1007/s00415-019-09440-8. Epub 2019 Jun 26.
3
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Front Immunol. 2023 Jul 14;14:1203615. doi: 10.3389/fimmu.2023.1203615. eCollection 2023.
4
Meningo-cortical manifestations of myelin oligodendrocyte glycoprotein antibody-associated disease: Review of a novel clinico-radiographic spectrum.髓鞘少突胶质细胞糖蛋白抗体相关疾病的脑膜-皮质表现:一种新型临床-影像学谱的综述
Front Neurol. 2022 Oct 20;13:1044642. doi: 10.3389/fneur.2022.1044642. eCollection 2022.
Encephalitis is an important clinical component of myelin oligodendrocyte glycoprotein antibody associated demyelination: a single-center cohort study in Shanghai, China.
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J Neurol Neurosurg Psychiatry. 2018 Feb;89(2):127-137. doi: 10.1136/jnnp-2017-316880. Epub 2017 Nov 15.
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J Neurol Neurosurg Psychiatry. 2017 Jun;88(6):534-536. doi: 10.1136/jnnp-2016-315094. Epub 2017 Feb 16.
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