关键词: 髓鞘少突胶质细胞糖蛋白抗体相关疾病, 临床特征, 脑脊液, 脑电图, 脑诱发电位, 头颅磁共振

Abstract:

Objective: To analyze the clinical manifestations, imaging features and prognosis of the patients with anti-myelin oligodendrocyte glycoprotein-IgG associated disorders (MOGAD). Methods: The data of eight MOGAD patients diagnosed in our hospital from January 2018 to December 2021 was analyzed, including the pathogenic factors, onset forms, clinical manifestations and auxiliary examinations. The therapeutic effect and prognosis of the patients were observed and discussed, and the relevant literatures were reviewed. Results: Among 8 patients with MOGAD, 6 were male and 2 were female, and the average age was 27.37 years. The most common clinical manifestations were limb weakness, limb twitching and blurred vision. The spinal puncture showed that the CSF pressure of the patients were 95-225 mmH₂O. It revealed that 3 cases had increased CSF pressure, 5 cases had increased CSF cell count and 4 cases had increased CSF protein, while the CSF sugar and chloride were all normal. Six patients had positive MOG antibody in blood and cerebrospinal fluid. Five cases were found to have abnormal brain evoked potentials, and 4 cases had abnormal electroencephalogram. The brain MRI showed that the wide area of brain was affected, including different degree of abnormal signals in optic nerve, pons arm, thalamus, paraventricular, corpus callosum, frontal lobe, parietal lobe, occipital lobe, subcortical, cerebellum and other parts. Seven patients showed intracranial lesions, while only one patient showed abnormal signals in the long segment of cervical and thoracic spinal cord. All 8 patients were treated with high-dose methylprednisolone pulse therapy, in which 3 cases were given methylprednisolone combined with gamma globulin, and 1 case was treated with methylprednisolone combined with cyclophosphamide. After regular treatment, 7 patients were followed up for 3 years. The symptoms were relieved in 7 patients, while 1 patient still had recurrent attacks. By searching CNKI, CSPD，and CSTJD and PubMed Databases, 161 patients with MOGAD were enrolled. After reviewing the relevant literatures, it revealed that the subacute and chronic onset of MOGAD was common, and its clinical manifestations were diverse. The imaging study showed that the whole brain and spinal cord could be affected, and the recurrence rate was 30.00%-43.39%. The MOGAD patients were sensitive to hormone and immune therapy. The clinical and imaging manifestations and treatment prognosis of 8 MOGAD cases were consistent with previous report. Conclusions: The subacute and chronic onset of MOGAD is common, and most of the patients are young, and the patients show diverse clinical manifestations. In MOGAD patients, the routine examination of cerebrospinal fluid is non-specific, while the imaging of the whole brain and spinal cord are abnormal, and the damage area could be widely. The patients with MOGAD are sensitive to hormone and immune therapy, which presents satisfied therapeutic effects and prognosis. For the suspected patients, MOG should be performed as early as possible, and the multiple tests can be performed to avoid missed diagnosis.

Key words: Anti-myelin oligodendrocyte glycoprotein-IgG associated disorders(MOGAD), Clinical features, Cerebrospinal fluid, Electroencephalography, Brain evoked potential, Brain magnetic resonance