髓鞘少突胶质细胞糖蛋白抗体相关疾病临床表现、脑脊液及影像学特征

Clinical manifestations, cerebrospinal fluid and imaging features of myelin oligodendrocyte glycoprotein antibody-related diseases

  • 摘要: 目的 总结髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)的临床表现、脑脊液及影像学特征。 方法 收集中国人民解放军空军军医大学唐都医院收治的11例MOGAD患者,总结其临床表现、脑脊液和影像学特点及治疗方法,对患者进行随访,并观察患者预后。 结果 11例MOGAD患者中,男性4例,女性7例,其中4例患者患病前有明确感染史。临床类型: 脑炎型3例,视神经炎型1例,脊髓炎型3例,视神经脊髓炎型2例,脑脊髓炎型1例,视神经脑炎型1例。2例患者颅内、脊髓以及视神经均无病灶,病灶强化形式各不相同。脑脊液白细胞计数为(0~280)×106/L, MOG抗体滴度为1∶10~1∶320。所有患者均给予激素治疗, 1例患者反复发作,其余患者恢复均较好。 结论 临床上此类患者应考虑为中枢神经系统脱髓鞘疾病,血清水通道蛋白(AQP4)抗体阴性,且不支持多发性硬化、急性播散性脑脊髓炎(ADEM)等诊断的患者,需警惕MOGAD的可能。

     

    Abstract: Objective To summarize the clinical manifestations, cerebrospinal fluid and imaging features of myelin oligodendrocyte glycoprotein antibody-related diseases(MOGAD). Methods A total of 11 patients with MOGAD admitted to Tangdu Hospital of Air Force Military Medical University of People's Liberation Army were collected, and their clinical manifestations, cerebrospinal fluid characteristics, imaging characteristics as well as treatment methods were summarized. These patients were followed up to observe their prognosis. Results Out of 11 MOGAD patients, there were 4 males and 7 females. Four patients had a definite history of infection before illness. Clinical types: 3 cases of encephalitis, 1 case of optic neuritis, 3 cases of myelitis, 2 cases of optic neuromyelitis, 1 case of encephalomyelitis, and 1 case of optic neuroencephalitis. There were no lesions in the brain, spinal cord, and optic nerves in two patients, and the form of lesion enhancement differed from each other. The white blood cell count in cerebrospinal fluid was(0~280)×106/L, and the MOG antibody titer ranged from 1∶10 to 1∶320. All patients were given hormone therapy, one patient had recurrent attacks, and the rest recovered well. Conclusion These patients are considered to be demyelinating disease of the central nervous system, and those who have negative serum aquaporin(AQP4)antibody and are not diagnosed as diseases such as multiple sclerosis and acute disseminated encephalomyelitis(ADEM), should be alerted the possibility of MOGAD.

     

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