抗髓鞘少突胶质细胞糖蛋白IgG抗体相关疾病20例临床分析

Clinical analysis of 20 cases of anti-myelin oligodendrocyte glycoprotein-IgG antibody associated diseases

目的对抗髓鞘少突胶质细胞糖蛋白IgG抗体(MOG-IgG)相关疾病(MOGAD)病例进行分析,探讨其临床特点。方法收集2017年6月至2020年6月在郑州大学第一附属医院住院的20例MOGAD患者的临床资料并分析。结果患者中男性9例,女性11例,中位数发病年龄24.5岁(3~71岁)。脑部受累14例,视神经受累11例,脊髓受累8例。其中5例表现为水通道蛋白4-IgG阴性视神经脊髓炎谱系疾病,3例孤立性视神经炎,3例多发性硬化,1例急性播散性脑脊髓炎,2例横贯性脊髓炎,1例自身免疫性脑炎,5例脑病患者暂不能归类于上述疾病表型。3例视神经炎患者的视神经MRI示长节段病变,且呈纵向强化。8例脊髓炎患者MRI分别呈长、短节段病灶(2~17个脊髓节段);8例累及颈髓,5例累及胸髓,1例累及腰髓。14例脑病患者头颅MRI发现病灶多呈斑片状、点片状分布,部分可强化;可累及丘脑、脑干、桥臂、基底节、侧脑室旁白质、胼胝体、大脑脚、半卵圆中心、大脑皮质、小脑等部位。6例患者视觉诱发电位示P100潜伏期延迟或波幅下降。患者对大剂量糖皮质激素冲击或免疫球蛋白治疗敏感,大部分患者恢复较好,部分复发。结论MOGAD主要表现为视神经、脊髓、颅脑等部位的病变及功能障碍。急性期对大剂量糖皮质激素冲击及免疫球蛋白治疗效果显著,预后良好,部分患者可有复发。

Objective To investigate the clinical characteristics of anti-myelin oligodendrocyte glycoprotein-IgG antibody(MOG-IgG)associated diseases(MOGAD).Methods Clinical data of 20 MOGAD patients who were hospitalized in the First Affiliated Hospital of Zhengzhou University from June 2017 to June 2020 were collected and analyzed.Results Among the 20 patients,9 were male and 11 were female.The median age of onset was 24.5 years(3-71 years).There were 14 cases with brain lesions,11 cases with optic neuritis and 8 cases with myelitis.Among them,5 patients presented with aquaporin 4-IgG negative neuromyelitis optica spectrum disorders,3 with isolated optic neuritis,3 with multiple sclerosis,1 with acute disseminated encephalomyelitis,2 with transverse myelitis,1 with autoimmune encephalitis,and 5 cases with encephalopathy could not be classified into the above disease phenotype temporarily.MRI of the optic nerve in 3 patients with optic neuritis showed long segmental lesions with longitudinal enhancement.Spinal MRI showed long or short segments involvement(2-17 segments of spinal cord)in 8 patients.Cervical spinal cord was involved in 8 cases,thoracic spinal cord was involved in 5 cases and lumbar spinal cord was involved in 1 case.Brain MRI of 14 patients with encephalopathy found that most of the lesions were patchy and patchy,and some of them could be enhanced.It can involve thalamus,brainstem,pons,basal ganglia,lateral ventricle White matter,corpus callosum,cerebellum,center of semicooval,cerebral cortex,cerebellum etc.Visual evoked potential showed delayed latency or decreased amplitude of P100 in 6 patients.Patients were sensitive to high-dose intravenous methylprednisolone or intravenous immunoglobulin therapy.Most patients recovered well,and some patients relapsed.Conclusions The main manifestations of MOGAD are lesions and dysfunction of optic nerve,spinal cord,brain and other parts.It mainly causes the optic nerve,spinal cord,brain and other parts to be involved and cause functional dysfunction.In the acute phase,the effect of high-dose intravenous methylprednisolone or intravenous immunoglobulin is significant,and the prognosis is well,some patients may relapse.