抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病的临床分析

邓兵梅; 刘卓; 项薇; 韩文杰; 周友田; 黎振声; 熊铁根; 康健捷

doi:10.16016/j.2097-0927.202309056

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抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病的临床分析

临床医学 | 更新时间：2026-01-09

- 抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病的临床分析
- Myelin oligodendrocyte glycoprotein antibody-associated disease: a clinical analysis of 14 cases
- 陆军军医大学学报 2024年46卷第12期页码：1434-1440
- 作者机构：
  
  510010 广州, 中国人民解放军南部战区总医院神经内科
- 作者简介：
  
  [ 康健捷，E-mail：26757551@qq.com KANG Jianjie, E-mail: 26757551@qq.com
- 基金信息：
  
  广州市科技计划项目(202102080514)
- DOI：10.16016/j.2097-0927.202309056
  中图分类号： R392.11;R445.2;R744.5
- 收稿：2023-09-11，
  
  修回：2023-10-22，
  
  纸质出版：2024-06-30
- 稿件说明：
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邓兵梅, 刘卓, 项薇, 等. 抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病的临床分析[J]. 陆军军医大学学报, 2024,46(12):1434-1440.

Bingmei DENG, Zhuo LIU, Wei XIANG, et al. Myelin oligodendrocyte glycoprotein antibody-associated disease: a clinical analysis of 14 cases[J]. Journal of Army Medical University, 2024, 46(12): 1434-1440.
邓兵梅, 刘卓, 项薇, 等. 抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病的临床分析[J]. 陆军军医大学学报, 2024,46(12):1434-1440. DOI： 10.16016/j.2097-0927.202309056.

Bingmei DENG, Zhuo LIU, Wei XIANG, et al. Myelin oligodendrocyte glycoprotein antibody-associated disease: a clinical analysis of 14 cases[J]. Journal of Army Medical University, 2024, 46(12): 1434-1440. DOI： 10.16016/j.2097-0927.202309056.

摘要

目的

探讨抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病(myelin oligodendrocyte glycoprotien antibody-associated disease，MOGAD)患者的临床及影像学特点。

方法

回顾性分析2016年6月至2022年6月就诊于我院14例MOGAD患者的临床表现、影像学特点、实验室检查和预后等资料，探讨其临床表现及影像学特点。

结果

男性10例，女性4例，男∶女=2.5∶1；首次发病年龄18岁以下3例，18~45岁8例，大于45岁3例。临床分型以视神经炎(10/14，71.43%)最为常见；其次为脑炎或脑膜脑炎(9/14，64.29%)、脑干脑炎(5/14，35.71%)和脊髓炎(5/14，35.71%)。临床症状以视力下降最多见(10/14，71.43%)；头痛8例(8/14，57.14%)、发热和头晕各6例(6/14，42.86%)、感觉异常5例(5/14，35.71%)；癫痫、肢体无力、括约肌功能障碍、共济失调、呕吐各4例(4/14，28.57%)。4例(4/14，28.57%)患者发病前有上呼吸道感染。10例患者完善了脑脊液(cerebrospinal fluid，CSF)检查，其中结果异常8例(8/10，80%)，CSF压力升高2例(2/10，20%)；WBC增高8例(8/10，80%)；脑脊液蛋白质增高5例(5/10，50%)。MRI可见多部位受累，大脑皮质及皮质下白质7例(7/14，50.00%)；脑干6例(6/14，42.86%)；视神经5例(5/14，35.71%)；脊髓4例(4/14，28.57%)；海马、丘脑、基底节区及脑室旁各3例(3/14，21.43%)；小脑、胼胝体各2例(2/14，14.29%)，T

WI、T

FLAIR表现为片状高信号影，可有点片状、结节状或线状强化。10例患者

行视觉诱发电位(visual evoked potential，VEP)检查，其中9例(9/10，90%)异常，8例(8/10，80%)提示双侧视觉通路异常。8例(8/14，57.14%)表现为复发缓解病程。急性期甲强龙冲击治疗和丙种球蛋白调节治疗均有效。5例表现为复发缓解的患者加用免疫抑制剂后复发均明显减少。

结论

MOGAD临床表现多样，以视力下降、头痛、发热、头晕最多见。病变常累及大脑皮质及皮质下白质、脑干、视神经等部位，T

WI、T

FLAIR表现为片状高信号影，部分病灶可强化。急性期激素冲击治疗和丙种球蛋白治疗有效，缓解期使用免疫抑制剂可减少复发。

Abstract

Objective

To investigate the clinical and imaging characteristics of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).

Methods

The clinical symptoms

MRI features

results of laboratory tests and clinical prognosis of 14 MOGAD patients who were hospitalized in our hospital from June 2016 to June 2022 were collected and retrospectively analyzed. Their clinical and imaging characteristics were summarized and discussed.

Results

Among the 14 enrolled patients

there were 10 males and 4 females

with a male to female ratio of 2.5∶1. Their age of first onset was

18 years in 3 cases

18~45 years in 8 cases

and

45 years in 3 cases. Optic neuritis (10/14

71.43%) was the most common clinical type

followed by encephalitis or meningoencephalitis (9/14

64.29%)

brainstem encephalitis (5/14

35.71%) and myelitis (5/14

35.71%). Visual impairment (10/14

71.43%) was the most common clinical symptom

followed by headache in 8 cases (8/14

57.14%)

fever in 6 cases (6/14

42.86%)

dizziness in 6 cases (6/14

42.86%)

parethesia in 5 cases (5/14

35.71%)

and seizures

limb paralysis

sphincter dysfunction

ataxia

and vomit were all in 4 cases (4/14

28.57%). Four patients (4/14

28.57%) had a history of upper respiratory tract infection before MOGAD onset. There were 10 patients undergoing cerebrospinal fluid (CSF) test

and 8 of them had abnormal results

including 2 patients (2/10

20%) of increased pressure

8 patients (8/10

80%) of larger WBC count in CSF

and 5 patients (5/10

50%) of elevated tot

al protein in CSF. MRI displayed multiple lesion involvement

and there were 7 cases (7/14

50.00%) in cortical/subcortical white matter

6 cases in brainstem (6/14

42.86%)

5 cases in optic nerve (5/14

35.71%)

4 cases in spinal cord (4/14

28.57%). The hippocampus

thalamus

basal ganglia

and paraventricular white matter were involved in 3 cases (3/14

21.43%)

respectively

and the cerebellum and corpus callosum were in 2 cases (2/14

14.29%)

respectively. MRI lesions demonstrated patchy hyperintensity on T

WI and T

FLAIR

with patchy

nodular and linear enhancement. Among the 10 patients undergoing visual evoked potential (VEP) test

abnormalities were detected in 9 cases (9/10

90%)

and 8 (8/10

80%) had bilateral visual pathway abnormalities. Eight patients (8/14

57.14%) experienced relapse and remission course. Both methylprednisolone pulse therapy and immunoglobulin modulation therapy were effective in the acute phase. Five patients with relapsed remission presented a significant reduction in recurrence after immunosuppressants.

Conclusion

MOGAD is manifested with various clinical features

with vision loss

headache

fever and dizziness more common. MRI lesions of MOGAD involve cerebral cortex

subcortical white matter

brainstem

and optic nerve

etc

. Patchy hyperintesive signals are observed on T

WI and T

FLAIR

and some lesions can be enhanced. Corticosteroid pulse therapy and immunoglobulin therapy show effective treatment in the acute phase

and immunosuppressants in the remission phase can reduce relapse.

关键词

Keywords

references

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