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中华实验和临床感染病杂志(电子版) ›› 2026, Vol. 20 ›› Issue (02) : 122 -128. doi: 10.3877/cma.j.issn.1674-1358.2026.02.008

病例报告

以假瘤样脱髓鞘病变为表现的髓鞘少突胶质细胞糖蛋白抗体相关性疾病一例
曹伊凡1, 胡良晨2, 徐冉冉2, 赵春霞1, 张梓钰1, 李若林2, 徐鹏2, 李相元2,()   
  1. 1 272067 济宁市,济宁医学院临床医学院
    2 272029 济宁市,济宁医学院附属医院神经内科
  • 收稿日期:2025-05-15 出版日期:2026-04-15
  • 通信作者: 李相元
  • 基金资助:
    国家自然科学基金青年项目(82301429); 济宁市重点研发项目(2024JNZC075)

A case of myelin oligodendrocyte glycoprotein antibody-associated disease presenting as tumefactive demyelinating lesions

Yifan Cao1, Liangchen Hu2, Ranran Xu2, Chunxia Zhao1, Ziyu Zhang1, Ruolin Li1, Peng Xu2, Xiangyuan Li2,()   

  1. 1 School of Clinical Medicine, Jining Medical University, Jining 272067, China
    2 Department of Neurology, the Affiliated Hospital of Jining Medical University, Jining 272029, China
  • Received:2025-05-15 Published:2026-04-15
  • Corresponding author: Xiangyuan Li
引用本文:

曹伊凡, 胡良晨, 徐冉冉, 赵春霞, 张梓钰, 李若林, 徐鹏, 李相元. 以假瘤样脱髓鞘病变为表现的髓鞘少突胶质细胞糖蛋白抗体相关性疾病一例[J/OL]. 中华实验和临床感染病杂志(电子版), 2026, 20(02): 122-128.

Yifan Cao, Liangchen Hu, Ranran Xu, Chunxia Zhao, Ziyu Zhang, Ruolin Li, Peng Xu, Xiangyuan Li. A case of myelin oligodendrocyte glycoprotein antibody-associated disease presenting as tumefactive demyelinating lesions[J/OL]. Chinese Journal of Experimental and Clinical Infectious Diseases(Electronic Edition), 2026, 20(02): 122-128.

目的

提高对以假瘤样脱髓鞘病变为表现的髓鞘少突胶质细胞糖蛋白抗体相关性疾病(MOGAD)相关特征、早期诊断和治疗的认识,避免误诊误治。

方法

回顾性分析2024年12月30日济宁医学院附属医院收治的1例MOGAD患者的临床资料和诊疗经过,并结合相关文献进行复习。

结果

患者女性,60岁,因头晕头痛20余天,步态不稳、不能言语6 d,临床表现为脑瘤样病变。该患者血清及脑脊液抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体(MOG-IgG)均为1∶10+。颅脑磁共振成像(MRI)示双侧颞叶及双侧额叶皮层下脑组织肿胀伴异常信号,呈肿瘤样改变,确诊为MOGAD。给予甲泼尼龙激素500 mg/d冲击治疗,逐渐减量至62.5 mg/d后联合免疫抑制剂吗替麦考酚酯胶囊治疗,明显缓解后出院3月余复查颅脑MRI,颅内异常信号较前缩小约70%,患者预后较好。

结论

MOGAD临床表现复杂,需与其他中枢神经系统疾病进行鉴别诊断,在临床实践中需高度重视MOG-IgG检测的重要性,避免误诊误治。

Objective

To raise the awareness of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), which presents as tumefactive demyelinating lesions, and to improve the early diagnosis and treatment, and to avoid the misdiagnosis and inappropriate management.

Methods

The clinical data and management of a patient with MOGAD admitted to the Affiliated Hospital of Jining Medical University on December 30th, 2024 were analyzed, retrospectively, and relevant literature were reviewed.

Results

A 60-year-old female patient presented with dizziness and headache lasting more than 20 days, as well as unsteady gait and aphasia for 6 days, whose clinical presentation was consistent with a brain tumefactive demyelinating lesions. The patient’s serum and cerebrospinal fluid samples were both positive for anti-myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) antibodies at a titre of 1∶10+. Cranial magnetic resonance imaging (MRI) revealed subcortical brain tissue swelling with abnormal signals in the bilateral temporal and frontal lobes, presenting as tumour-like changes. A diagnosis of MOG-associated demyelinating encephalopathy (MOGAD) was confirmed. The patient was treated with a corticosteroid pulse therapy of 500 mg/d of methylprednisolone, gradually tapered to 62.5 mg/d, followed by combination therapy with the immunosuppressant mycophenolate mofetil capsules, resulting in significant symptom relief. A follow-up MRI performed longer than three months after discharge showed that the abnormal intracranial signals had resolved by approximately 70% compared with the previous scan, with a favourable prognosis.

Conclusions

The clinical presentation of MOG is complex and requires differential diagnosis from other central nervous system disorders, and the importance of MOG-IgG testing must be given high priority to avoid misdiagnosis and inappropriate treatment.

表1 患者中枢神经系统脱髓鞘疾病自身抗体
图1 荧光显微镜下患者血清中枢神经系统脱髓鞘疾病自身抗体(× 200)
图2 荧光显微镜下患者脑脊液中枢神经系统脱髓鞘疾病自身抗体(× 200)
图3 2024年12月23日患者颅脑MRI(外院) 注:A示T1WI,右侧颞叶见斑片状低信号(长T1信号);B示T2-FLAIR,右侧颞叶病灶呈高信号(箭头所示);C示T2WI,右侧颞叶见斑片状高信号(长T2信号,箭头所示)
图4 2024年12月25日患者颅脑MRI(外院) 注:A示T2-FLAIR,右侧颞叶皮层及皮层下见斑片状高信号灶(箭头所示),邻近软脑膜呈线样高信号;B示增强T1WI,右侧颞叶病灶呈沿软脑膜分布的线样强化,邻近右侧颞叶软脑膜强化较对侧显著
图5 2024年12月27日患者颅脑MRV和MRS(外院) 注:A示MRV未见明显异常;B示T2WI,显示波谱定位,定位于右侧颞叶病变区域(十字交叉线所示);C示MRS,病变区N-乙酰天门冬氨酸(NAA)峰减低,胆碱(Cho)峰未见明显升高
图6 2025年1月12日患者颅脑MRI(本院) 注:A示T1WI,双侧颞叶皮层下脑组织肿胀,呈稍低信号;B示T2WI,双侧颞叶皮层下可见高信号影(箭头所示);C示T2-FLAIR,双侧颞叶病灶呈高信号影,边界相对清晰;D示T1WI,双侧额叶皮层下脑组织肿胀,呈稍低信;E示T2WI,双侧额叶皮层下及右放射冠区可见高信号影(箭头所示);F示T2-FLAIR,上述病灶T2水抑制成像后呈相对高信号影
图7 2025年2月4日患者脑部MRI(本院) 注:A示T1WI,右颞叶皮层下脑组织肿胀较前减轻,呈稍低信号;B示T2WI,右颞叶皮层下可见稍高信号影(白色箭头指示病灶区域);C示T2-FLAIR,右颞叶病灶呈稍高信号影
图8 2025年4月29日患者脑部MRI(本院) 注:A示TIWI,右颞叶皮层下脑组织肿胀进一步减轻;B示T2WI,右颞叶皮层下可见轻度稍高信号影(白色箭头指示残留病灶区域);C示T2-FLAIR,右颞叶病灶呈轻度稍高信号;D示T1WI,双额叶皮层下脑组织形态及信号基本恢复正常;E示T2WI,双额叶皮层下信号基本正常;F示T2-FLAIR,双额叶皮层下高信号范围较前进一步缩小(白色箭头指示残留病灶区域)
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