儿童髓鞘少突胶质细胞糖蛋白抗体相关疾病的临床特征及MRI特点分析

Analysis of Clinical Features and MRI Manifestations of Myelin Oligodendrocyte Glycoprotein Antibody-Related Disease(MOGAD) in Children

目的 探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病的临床特征和磁共振特点。方法 回顾性分析2019-01至2021-07郑州大学附属儿童医院神经内科收治的18例MOG抗体相关疾病患者的临床及影像学资料,总结其临床症状、MRI图像特点、预后及随访情况。结果 18例患者中,男女各9例,中位年龄8.3岁。临床首发症状表现多样,发热7例,头痛、呕吐、意识水平下降等脑病症状9例,抽搐7例,视力下降4例、颅神经症状2例,脊髓症状2例。头颅MRI检查为皮层下白质10例、基底节区6例、丘脑4例、脑干2例、小脑4例部位出现片状FLAIR高信号,大脑半球皮层不同程度受累10例,单纯皮层受累2例。视神经MRI信号异常6例,脊髓MRI信号异常6例。18例患儿MOG抗体滴度1:10~1:1000。所有患儿均接受糖皮质激素及免疫球蛋白治疗,预后情况良好,随访中有4例出现复发。结论 MOGAD患儿临床表现多样,头颅磁共振病变分布以大脑半球皮层下、深部白质和灰质核团为主,皮层可单独受累,视神经受累多为视交叉前长节段型,脊髓圆锥受累较多见。糖皮质激素治疗可显著缓解患儿的临床症状,预后良好,同时可预防疾病复发。

Objective To investigate the clinical and magnetic resonance characteristics of myelin oligodendrocyte glycoprotein(MOG) antibody related diseases in children. Methods Clinical and imaging data of 18 patients with MOG antibody related diseases admitted to the Department of Neurology, Children’s Hospital affiliated to Zhengzhou University from January 2019 to July 2021 were retrospectively analyzed, and their clinical symptoms, MRI features, prognosis and follow-up were summarized. Results There were 9 male and 9 female patients with a median age of 8.3 years. The first clinical symptoms were varied, including fever in 7 cases, headache, vomiting, decreased consciousness and other encephalopathy symptoms in 9 cases, convulsion in 7 cases, decreased vision in 4 cases, cranial nerve symptoms in 2 cases, spinal cord symptoms in 2 cases. Brain MRI showed fla-like FLAIR hypersignal in subcortical white matter in 10 cases, basal ganglia in 6 cases, thalamus in 4 cases, brainstem in 2 cases, cerebellum in 4 cases, cerebral cortex in 10 cases and pure cortex in 2 cases. There were 6 abnormal MRI signals of optic nerve and 6 abnormal MRI signals of spinal cord. The titer of MOG antibody was 1:10-1:1000 in 18 children. All of the children received glucocorticoid and immunoglobulin therapy, and the prognosis was good, and 4 cases recurred during follow-up. Conclusion There are many clinical manifestations in children with MOGAD, and the distribution of cerebral MAGNETIC resonance lesions is mainly subcortical and deep white and gray matter nuclei in the cerebral hemisphere, with or without spinal cord and optic neuropathy. Glucocorticoid treatment can significantly relieve the clinical symptoms of the children with a good prognosis and prevent the recurrence of the disease.