未成年人髓鞘少突胶质细胞糖蛋白抗体阳性急性播散性脑脊髓炎临床特点及治疗预后分析

Clinical features and prognosis of pediatric myelin oligodendrocyte glycoprotein antibody associated acute disseminated encephalomyelitis

目的探讨分析未成年人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)的临床特点、治疗效果及预后,为临床诊治提供参考依据。方法收集2014年1月1日至2019年2月1日在广州市妇女儿童医疗中心诊断为MOG抗体相关的ADEM患儿的临床资料,通过细胞间接免疫荧光法对患者血清MOG-IgG检测,同时收集并分析抗体阳性患者临床资料。结果起病年龄为(5.2±3.3)岁,男女比例为16∶14。50%患儿起病前有前驱感染或疫苗接种史,最常见的临床表现为脑病及抽搐,其次为运动障碍,部分病例可合并其他自身抗体阳性,头颅MRI表现为双侧皮质或皮质下广泛、不对称、边界不清晰的大片状病灶,脊髓以长节段脊髓炎多见,急性期激素联合静脉丙种球蛋白治疗效果较好,大部分预后较好,约有20%复发。结论未成年人MOG抗体相关的ADEM起病年龄在5岁左右,脑病和抽搐为最常见的临床表现,大部分患者激素联合IVIG治疗反应好,部分患儿可能复发。

Objective 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.Methods 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.Results 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%.Conclusions 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.