Papantoniou Michail, Panagou Georgia, Kanavouras Konstantinos
Neurology, General Hospital of Athens G. Gennimatas, Athens, GRC.
Cureus. 2024 Sep 30;16(9):e70546. doi: 10.7759/cureus.70546. eCollection 2024 Sep.
Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease refers to a clinical and radiological spectrum of demyelinating disorders of the Central Nervous System. We report the case of a female adult patient presenting to our department with an episode of seizures and cognitive dysfunction, compatible with Gerstmann syndrome. Brain MRI revealed a high T2 and DWI signal unilateral cortical lesion at the inferior left parietal lobe and leptomeningeal contrast enhancement. Lumbar puncture showed pleocytosis of the lymphocytic type and elevated protein. Upon suspicion of autoimmune encephalitis, extensive laboratory testing was performed, and the patient's serum and cerebrospinal fluid (CSF) tested positive for anti-MOG, while anti-glial fibrillary acidic protein (GFAP) antibodies were detected in her serum. A diagnosis of FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizures (FLAMES) was made, and the patient was treated with intravenous methylprednisolone for five days, leading to clinical remission within days. However, serum anti-MOG IgG titers were found to be higher on follow-up, and the patient experienced a relapse, thus treatment with azathioprine was initiated. We suggest that upon suspicion of autoimmune encephalitis, all patients should be tested for serum and CSF anti-MOG IgG antibodies. Furthermore, we consider that anti-MOG antibody titers and GFAP concentration could be used as possible biomarkers for the disease course and treatment strategy options.
髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病是指中枢神经系统脱髓鞘疾病的临床和影像学谱系。我们报告了一名成年女性患者的病例,该患者因癫痫发作和认知功能障碍前来我科就诊,符合格斯特曼综合征。脑部MRI显示左侧顶叶下部有一个高T2和DWI信号的单侧皮质病变,软脑膜有对比增强。腰椎穿刺显示淋巴细胞型细胞增多和蛋白升高。在怀疑自身免疫性脑炎后,进行了广泛的实验室检查,患者血清和脑脊液(CSF)抗MOG检测呈阳性,而其血清中检测到抗胶质纤维酸性蛋白(GFAP)抗体。诊断为伴有癫痫发作的抗MOG相关脑炎的FLAIR高信号病变(FLAMES),患者接受静脉注射甲基强的松龙治疗5天,数天内临床缓解。然而,随访时发现血清抗MOG IgG滴度更高,患者复发,因此开始使用硫唑嘌呤治疗。我们建议,在怀疑自身免疫性脑炎时,所有患者都应检测血清和脑脊液抗MOG IgG抗体。此外,我们认为抗MOG抗体滴度和GFAP浓度可作为疾病进程和治疗策略选择的可能生物标志物。