Clinical Focus ›› 2025, Vol. 40 ›› Issue (1): 70-75.doi: 10.3969/j.issn.1004-583X.2025.01.011
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Received:
2024-05-30
Online:
2025-01-20
Published:
2025-01-17
Contact:
Meng Fenglei,Email:13810775741@163.comCLC Number:
Zhong Yuan, Meng Fenglei. A case report of adult MOGAD and literature review[J]. Clinical Focus, 2025, 40(1): 70-75.
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URL: https://huicui.hebmu.edu.cn/EN/10.3969/j.issn.1004-583X.2025.01.011
Fig. 1 Head MRI a. In acute phase, Flair showed high signal changes; b. The enhanced MRI showed partial enhancement, but no enhancement of the optic nerve. c. Flair lesions were significantly reduced after 4 months
Fig. 3 Spinal cord MRI in acute phase of MOGAD left arrows showed T2-lesion extending over at least 3 continuous vertebral segments down to the conus, and H-shaped hyperintensity in sagittal. Right arrows showed strengthened performance
诊断标准 | 具体表现 | ||
---|---|---|---|
1.临床表现 | 视神经炎 | ||
脊髓炎 | |||
急性播散性脑脊髓炎 | |||
大脑、脑干或小脑病变 | |||
大脑皮质脑炎 | |||
2.MOG-IgG检测阳性 | 血清CBA检测 | 强阳性:抗体滴度≥1:100 | 不需要临床及影像学支持 |
弱阳性:1:10≤抗体滴度<1:100 | 需满足A和B: | ||
抗体阴性 | A:AQP4-IgG阴性 | ||
血清抗体阴性但是脑脊液抗体阳性 | B:一种或多种临床或影像学表现支持 | ||
3.排除其他诊断,包括MS |
Tab.1 Proposed MOGAD diagnostic criteria by an international panel of experts in 2023
诊断标准 | 具体表现 | ||
---|---|---|---|
1.临床表现 | 视神经炎 | ||
脊髓炎 | |||
急性播散性脑脊髓炎 | |||
大脑、脑干或小脑病变 | |||
大脑皮质脑炎 | |||
2.MOG-IgG检测阳性 | 血清CBA检测 | 强阳性:抗体滴度≥1:100 | 不需要临床及影像学支持 |
弱阳性:1:10≤抗体滴度<1:100 | 需满足A和B: | ||
抗体阴性 | A:AQP4-IgG阴性 | ||
血清抗体阴性但是脑脊液抗体阳性 | B:一种或多种临床或影像学表现支持 | ||
3.排除其他诊断,包括MS |
药物 | 治疗方案 |
---|---|
激素 | 静脉注射1 000 mg/d(对于视神经炎可改为口服激素1 250 mg/d),连续5d。后阶梯口服激素:20~40 mg,或1 mg/kg/d,数周到数月逐渐减停。 |
血浆置换 | 5~7个循环,每隔1 d |
静脉注射免疫球蛋白 | 1 g/kg给药2 d或0.4 g/kg给药5 d[ |
Tab.2 Acute treatment of MOGAD
药物 | 治疗方案 |
---|---|
激素 | 静脉注射1 000 mg/d(对于视神经炎可改为口服激素1 250 mg/d),连续5d。后阶梯口服激素:20~40 mg,或1 mg/kg/d,数周到数月逐渐减停。 |
血浆置换 | 5~7个循环,每隔1 d |
静脉注射免疫球蛋白 | 1 g/kg给药2 d或0.4 g/kg给药5 d[ |
药物 | 治疗方案 |
---|---|
糖皮质激素(a) | 甲泼尼龙1 000 mg,静脉滴注3 d;然后改为500 mg,静脉滴注3 d。而后甲泼尼龙减量为40~80 mg/d,静脉滴注2周;或改为口服醋酸泼尼松1 mg/kg/d,2周;之后每2周减5 mg |
静脉注射免疫球蛋白(b) | 每一疗程:总量2 g/kg,分3~5 d静脉滴注 |
强化一线免疫治疗:可每2~4周重复应用 | |
血浆置换(c) | 每一疗程:在7~10 d内进行5~7次1~2个血浆当量置换 |
Tab.3 Chinese expert consensus in the treatment of autoimmune encephalitis
药物 | 治疗方案 |
---|---|
糖皮质激素(a) | 甲泼尼龙1 000 mg,静脉滴注3 d;然后改为500 mg,静脉滴注3 d。而后甲泼尼龙减量为40~80 mg/d,静脉滴注2周;或改为口服醋酸泼尼松1 mg/kg/d,2周;之后每2周减5 mg |
静脉注射免疫球蛋白(b) | 每一疗程:总量2 g/kg,分3~5 d静脉滴注 |
强化一线免疫治疗:可每2~4周重复应用 | |
血浆置换(c) | 每一疗程:在7~10 d内进行5~7次1~2个血浆当量置换 |
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