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MOG-IgG 相关疾病与自身免疫性 GFAP 星形胶质细胞病重叠综合征。

Overlapping syndrome of MOG-IgG-associated disease and autoimmune GFAP astrocytopathy.

机构信息

Department of Neurology, Tangdu Hospital, Air Force Military Medical University, Xi'an, 710038, China.

Department of Neurology, Xianyang Central Hospital, Xianyang, 712000, China.

出版信息

J Neurol. 2020 Sep;267(9):2589-2593. doi: 10.1007/s00415-020-09869-2. Epub 2020 May 7.

Abstract

Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been considered to be closely relevant to an inflammatory demyelinating disease of the central nervous system (CNS). Glial fibrillary acidic protein (GFAP) immunoglobulin G (IgG) has been identified as a biomarker for a novel autoimmune astrocytopathy. However, coexistence of MOG-IgG and GFAP-IgG is extremely unusual and only one patient has been described with simultaneous presence of MOG-IgG in serum and GFAP-IgG in cerebrospinal fluid (CSF). Herein, we reported the first case of overlapping syndrome of MOG-IgG-associated disease (MOG-AD) and autoimmune GFAP astrocytopathy in whom MOG-IgG and GFAP-IgG were detected both in serum and CSF. A 20-year-old male patient was referred to our department with the presentation of decreased vision, diplopia and weakness of right limb with unknown reasons. Magnetic resonance imaging (MRI) revealed multiple intracranial lesions presenting hypointensity on T1-weighted images, hyperintensity on T2-weighted and FLAIR images and patchy contrast enhancement. MOG-IgG and GFAP-IgG were detected both in serum and CSF, and the titers of both antibodies fluctuated with the severity of disease. Treatment strategy employing intravenous methylprednisolone pulse therapy followed by oral prednisone with slow tapering resulted in an improvement of his symptoms and a sustained remission. Coexistence of MOG-IgG and GFAP-IgG with distinct underlying pathogeneses necessitates the recommendations to screen all recognized pathogenic antibodies against CNS antigens when an autoimmune disease is suspected, since it shows great significance for definite diagnosis of disease and treatment strategy options.

摘要

抗髓鞘少突胶质细胞糖蛋白(MOG-IgG)抗体被认为与中枢神经系统(CNS)的炎症性脱髓鞘疾病密切相关。胶质纤维酸性蛋白(GFAP)免疫球蛋白 G(IgG)已被确定为一种新型自身免疫性星形胶质细胞病的生物标志物。然而,MOG-IgG 和 GFAP-IgG 的共存极为罕见,仅描述过一例血清中同时存在 MOG-IgG 和脑脊液(CSF)中存在 GFAP-IgG 的患者。在此,我们报告了首例 MOG-IgG 相关疾病(MOG-AD)和自身免疫性 GFAP 星形胶质细胞病重叠综合征的病例,该患者血清和 CSF 中均检测到 MOG-IgG 和 GFAP-IgG。一名 20 岁男性患者因视力下降、复视和右侧肢体无力就诊,原因不明。磁共振成像(MRI)显示多个颅内病变,T1 加权图像呈低信号,T2 加权和 FLAIR 图像呈高信号,呈斑片状对比增强。血清和 CSF 中均检测到 MOG-IgG 和 GFAP-IgG,两种抗体的滴度随疾病的严重程度而波动。采用静脉注射甲基强的松龙脉冲治疗后口服泼尼松并缓慢减量的治疗策略导致其症状改善和持续缓解。具有不同潜在发病机制的 MOG-IgG 和 GFAP-IgG 的共存需要建议在怀疑自身免疫性疾病时筛查所有针对 CNS 抗原的已识别致病性抗体,因为这对于明确诊断疾病和治疗策略选择具有重要意义。

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