髓鞘寡突胶质细胞糖蛋白抗体相关疾病的临床特点分析
作者:
作者单位:

中南大学湘雅医院神经内科,湖南 长沙 410008

作者简介:

李思灼(1996―),女,在读研究生,主要从事神经免疫疾病的研究。

通信作者:

周瑾瑕(1981―),女,主治医师,博士,主要从事神经免疫疾病及睡眠障碍疾病的诊疗研究,Email: 405782@csu.edu.cn。

基金项目:

国家自然科学基金青年项目(81401065);国家重点研发计划精准医学研究专项神经系统疾病专病队列研究(2017YFC0907700)。


Clinical features of myelin oligodendroglia glycoprotein antibody-associated disorders
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Department of Neurology, Xiangya Hospital Central South University, Changsha, Hunan 410008, China

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    摘要:

    目的 探讨髓鞘寡突胶质细胞糖蛋白(MOG)抗体相关疾病患者的临床特点、辅助检查、治疗及预后情况。方法 收集2019年1月至2021年1月在中南大学湘雅医院神经内科住院的24例MOG抗体相关疾病患者的临床资料,并进行回顾性分析。结果 24例患者中,男性13例,女性11例;平均年龄(31.4±14.9)岁。脑炎为最常见的表现形式(11/24),其中皮质脑炎10例;其次为急性播散性脑脊髓炎(6/24)、横贯性脊髓炎(3/24)、视神经炎(2/24)、视神经脊髓炎(2/24)。临床表现多样,头痛发热为皮质脑炎型患者最常见症状。24例患者中,9例脑脊液压力升高(190~380 mmH2O);9例白细胞计数升高(18~1 800/mm3),以单个核细胞为主;9例脑脊液蛋白升高(0.46~1.92 g/L)。所有患者血MOG抗体均为阳性,其中有6例患者脑脊液MOG抗体阳性,1例患者合并血清和脑脊液抗N-甲基-D-天冬氨酸受体(NMDAR)抗体阳性。所有患者进行了颅脑磁共振成像(MRI)检查,21例发现有颅内病变,部位累及皮质、基底节、丘脑、脑干、小脑、脑膜、视神经、颈髓和胸髓等,表现为长T1长T2异常信号,FLAIR明显高信号,DWI等信号或高信号,ADC等信号或低信号,增强后可见点线、斑片状强化或无强化。12例患者进行了脊髓MRI检查,其中有8例患者脊髓受累,病变主要位于颈髓和胸髓,常累及2个以上节段。24例患者中,除2例拒绝采用免疫治疗外,其余22例采用了激素和/或血浆置换、人免疫球蛋白治疗。除3例患者失访外,对其余21例患者进行了3~24个月的随访,15例患者预后良好,未遗留明显神经功能障碍;3例患者在激素减量过程中出现复发;3例患者遗留部分症状。结论 MOG抗体相关疾病的临床异质性大,应重视皮质脑炎型MOG抗体脑炎的诊断;脑脊液多为炎症改变,可见颅内压明显增高或白细胞数显著升高,注意与颅内感染性病变鉴别;血清MOG抗体阳性率高于脑脊液,偶可合并其他自身免疫性抗体阳性;大多数患者对免疫治疗敏感,预后良好。

    Abstract:

    Objective To investigate the clinical features, auxiliary examination results, treatment, and prognosis of patients with myelin oligodendroglia glycoprotein (MOG) antibody-associated disorders.Methods A retrospective analysis was performed for the clinical data of 24 patients with MOG antibody-associated disorders who were hospitalized in the Department of Neurology, Xiangya Hospital of Central South University, from January 2019 to January 2021.Results Among the 24 patients, there were 13 male patients and 11 female patients, with a mean age of (31.4±14.9) years. For all 24 patients, encephalitis was the most common manifestation observed in 11 patients (among whom 10 patients had cortical encephalitis), followed by acute disseminated encephalomyelitis in 6 patients, transverse myelitis in 3 patients, optic neuritis in 2 patients, and neuromyelitis optica in 2 patients. Patients tended to have diverse clinical manifestations, among which headache and fever were the most common symptoms in patients with cortical encephalitis. Among the 24 patients, 9 had elevated cerebrospinal fluid pressure (190-380 mmH2O); 9 had elevated leukocyte count (18-1800/mm3), mainly mononuclear cells; 9 had elevated protein in cerebrospinal fluid (0.46-1.92 g/L). All patients tested positive for serum MOG antibodies, among whom 6 patients had positive MOG antibodies in cerebrospinal fluid and 1 patient had positive N-methyl-D-aspartate receptor antibody in both serum and cerebrospinal fluid. All patients underwent cranial magnetic resonance imaging (MRI), among whom 21 were found to have intracranial lesions, involving the cortex, basal ganglia, thalamus, brainstem, cerebellum, meninges, optic nerve, cervical cord, and thoracic cord, which manifested as long T1 and long T2 abnormal signals, marked hyperintensity on FLAIR, isointensity or hyperintensity on DWI, and isointensity or hypointensity on ADC, with dotted enhancement, patchy enhancement, or no enhancement after contrast-enhanced scan. Spinal cord MRI was performed for 12 patients, among whom 8 had the involvement of the spinal cord, and the lesions were mainly located in the cervical cord and the thoracic cord and often involved more than two segments. Of all 24 patients, 2 refused to receive immunotherapy, and 22 were treated with hormone and/or plasma exchange and human immunoglobulin. Apart from 3 patients who were lost to follow-up, the other 21 patients were followed up for 3-24 months, among whom 15 had good prognosis with no obvious neurological dysfunction, 3 experienced recurrence during the reduction in hormone dose, and 3 still had some symptoms left.Conclusions MOG antibody-associated disorders have strong clinical heterogeneity, and the diagnosis of cortical encephalitis in MOG antibody-associated encephalitis should be taken seriously. Inflammatory changes are often observed in cerebrospinal fluid, with a significant increase in intracranial pressure or leukocyte count, which should be differentiated from intracranial infectious lesions. The positive rate of MOG antibodies in serum is higher than that in cerebrospinal fluid, with the presence of other autoimmune antibodies in occasional cases. Most patients are sensitive to immunotherapy and can achieve a good prognosis.

    图1 患者3颅脑MRI检查所见Fig.1
    图2 患者19颅脑及脊髓MRI检查所见Fig.2
    表 1 24例抗MOG抗体相关疾病患者的临床资料Table 1
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李思灼,姚天星,李静文,毕方方,肖波,周瑾瑕456.髓鞘寡突胶质细胞糖蛋白抗体相关疾病的临床特点分析[J].国际神经病学神经外科学杂志,2023,50(1):29-35111LI Sizhuo, YAO Tianxing, LI Jingwen, BI Fangfang, XIAO Bo, ZHOU Jinxia222. Clinical features of myelin oligodendroglia glycoprotein antibody-associated disorders[J]. Journal of International Neurology and Neurosurgery,2023,50(1):29-35

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  • 收稿日期:2022-09-19
  • 最后修改日期:2023-01-26
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  • 在线发布日期: 2023-04-12
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