56例MOG-IgG相关疾病首次发病临床特点回顾性分析
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南京医科大学附属脑科医院

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南京市医学科技发展项目(YKK19087 ); 南京医科大学科技发展基金一般项目,NMUB2019126


Clinical characteristics of 56 patients with myelin oligodendrocyte glycoprotein-IgG-associated disorders (MOGAD): a retrospective study
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Nanjing Medical Science and Technology Development Project (YKK19087);General Program of Science and Technology Development Fund of Nanjing Medical University, NMUB2019126

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    摘要:

    目的:探索抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病(anti-myelin oligodendrocyte glycoprotein-IgG associated disorders,MOGAD)首次发病的临床特点。方法:对56例临床确诊MOGAD患者首次发病临床资料回顾性分析总结,包括年龄、性别、症状、核磁共振(magnetic resonance imaging, MRI)、眼底、脑脊液(cerebrospinal fluid,CSF)、抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体(anti-myelin oligodendrocyte glycoprotein-IgG,MOG-IgG)滴度水平、扩展功能障碍量表 (expanded disability status scale, EDSS)评分、与第二次复发间隔时间等。结果:首次发病年龄10- 58岁,平均23(25)岁。男:女=1.8:1。主要临床表现依次是脑膜脑炎(32.14%)、视神经炎(25.00%)、脊髓炎(17.86%)、脑干脑炎(14.28%)及多症状(10.71%)。MRI表现无特异性,颅脑MRI病灶体积大、分布散,脑干病灶常伴随大脑半球病灶;脊髓受累以短节段损害为主。约一半患者脑脊液白细胞及蛋白轻中度升高。以视神经炎为主要表现患者眼底常见显著视乳头水肿。EDSS评分0.5-8.5分,平均3.55±0.25分。MOG-IgG抗体滴度以弱阳性多见,滴度水平与疾病严重程度及复发风险无显著性相关(p >0.05)。首次发病后96.43%患者(54/56)恢复良好;3.57%患者(2/56)有后遗症。75.00%患者(42/56)有复发,与首次发作间隔1月-60月,平均6(21)月。未接受免疫调节治疗患者的复发风险显著性高于免疫调节治疗患者(p<0.05)。第二次复发时的症状与首次症状无显著相关(p >0.05)。结论:MOGAD首次发病大部分在青少年及青年时期,40岁之前占75.00%,未见在老年期发病者。男性稍多于女性。临床表现多样。大部分患者首次发病后恢复良好。免疫调节治疗可显著降低复发风险。

    Abstract:

    Objective: To explore the clinical features of first-onset myelin oligodendrocyte glycoprotein-IgG-associated disorders. Methods: We undertook a retrospective analysis of the first attack in 56 MOGAD patients. Numerous clinical parameters were evaluated, including age, gender, symptoms, MRI, fundus, CSF, MOG-IgG titer level, EDSS score, and the interval between the first and second attack. Results: The study cohort included 56 patients: 36 males and 20 females (male: female ratio = 1.8:1), with a median age of 23 years (range, 10-58 years). The first-onset manifestations were meningoencephalitis (32.14%), optic neuritis (25.00%), myelitis (17.86%), brainstem encephalitis (14.28%), and multiple symptoms (10.71%). The brain MRI lesions have the characteristics of large volume and scattered distribution. Brain stem involvement is usually accompanied by hemispheric lesions. Short segmental damage is common in patients with spinal cord involvement. About half of the patients had mild to moderate elevations of leukocytes and proteins in the cerebrospinal fluid. In patients with optic neuritis, significant papilledema can be seen in the fundus. The titer of MOG-IgG antibody was mostly weak positive. The EDSS score was 0.50–8.50, with an average of 3.55±0.25. The titers of MOG-IgG antibody at first onset were mostly weakly positive, MOG-IgG titer does not reflect disease severity or recurrence risk (p>0.05). After the first onset, most of the patients (54/56, 96.43%) recovered well without sequelae. A small number of patients (2/56, or 3.57%) had sequelae. 75.00% of patients experienced disease recurrence. The second recurrence lasted from 1 month to 60 months, with an average of 6 (21) months. The recurrence risk of patients without immunomodulatory therapy was significantly higher than that of patients with immunomodulatory therapy. There was no significant correlation between the symptoms of the second relapse and those of the first. Conclusion: The age of the first onset of MOGAD is mostly in teenagers and young adults, 75.00% before the age of 40. There is no incidence of MOGAD in the elderly. It affects slightly more men than women. Clinical manifestations are varied. The vast majority of patients have a good prognosis after the first onset. Most patients do not relapse after immunomodulatory therapy.

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  • 收稿日期:2022-11-17
  • 最后修改日期:2023-02-24
  • 录用日期:2023-08-29
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