Li X J, Hou C, Qiu W, Chen L F, Zeng Y R, Zhu H X, Tian Y, Peng B W, Wu W L, Gan Y Y, Liang H C, Chen W X
Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou 510120, China.
Department of Neurology, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, China.
Zhonghua Yi Xue Za Zhi. 2020 Feb 11;100(5):339-344. doi: 10.3760/cma.j.issn.0376-2491.2020.05.005.
To analyze the clinical features, outcome and prognosis of pediatric myelin oligodendrocyte glycoprotein (MOG) antibody associated acute disseminated encephalomyelitis (ADEM), and provide evidence for improving the diagnosis and treatment of this disease. This study involved 30 MOG antibody-associated ADEM patients in the Department of Neurology, Guangzhou Women and Children's Medical Center. Patients' clinical information were analyzed. The mean onset age was (5.2±3.3) years old, the ration of male to female was 16∶14. Fifty percent of these patients had a history of precede infection or vaccination before onset. Encephalopathy and seizures were the most common clinical manifestations, followed by movement disorder. In addition, some patients had other positive autoantibodies. Brain Magnetic resonance imaging (MRI) showed extensive, asymmetrical, indefinite large patchy lesions in bilateral cortical and subcortical areas and the spinal cord was characterized by long segmental myelitis. In acute attack, the patients had a good response to corticosteroid combined immunoglobulin therapy. Most of these patients had a good prognosis and recurrence rate was about 20%. The onset age of MOG antibody-associated ADEM is around 5 years old. Encephalopathy and seizures were the most common clinical manifestations. Most patients have a good response to corticosteroid combined immunoglobulin therapy. Some patients may have a recurrent disease course.
分析儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体相关急性播散性脑脊髓炎(ADEM)的临床特征、转归及预后,为提高该病的诊治水平提供依据。本研究纳入广州妇女儿童医疗中心神经内科30例MOG抗体相关ADEM患者,分析患者的临床资料。平均发病年龄为(5.2±3.3)岁,男女比例为16∶14。50%的患者在发病前有前驱感染或疫苗接种史。脑病和癫痫是最常见的临床表现,其次是运动障碍。此外,部分患者有其他自身抗体阳性。头颅磁共振成像(MRI)显示双侧皮质及皮质下区域广泛、不对称、边界不清的大片状病灶,脊髓表现为长节段脊髓炎。急性发作期患者对糖皮质激素联合免疫球蛋白治疗反应良好。多数患者预后良好,复发率约为20%。MOG抗体相关ADEM发病年龄约5岁,脑病和癫痫是最常见的临床表现。多数患者对糖皮质激素联合免疫球蛋白治疗反应良好,部分患者可能有疾病复发过程。