儿童MOG抗体病与抗NMDAR脑炎重叠综合征临床观察

Clinical observation on the overlapping syndrome of myelin oligodendrocyte glycoprotein antibody and anti-N-methyl-D aspartate receptor in children

目的探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体病与抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎重叠综合征(MNOS)病例的临床特点、影像学表现及预后情况。方法回顾性总结2011年1月至2019年4月北京儿童医院神经内科11例MNOS病例的临床表现、血及脑脊液免疫学抗体和头颅影像学的特点以及治疗随访情况。结果11例患儿中男4例、女7例,首次发病年龄(10.4±2.3)岁。11例患儿共有29次发作事件。至末次随访,8例患儿呈复发缓解病程,复发间隔3~60个月。11例患儿首次发作以抽搐(10例)、嗜睡(6例)、精神行为异常(6例)常见;29次发作事件常见的表现为抽搐(16例次)、精神行为异常(13例次)及嗜睡(10例次)。根据抗NMDAR脑炎及MOG抗体病的诊断标准,29次发作临床分型抗NMDAR脑炎4例次,MOG抗体病10例次,重叠型15例次。头颅磁共振成像检测27例次,常见受累部位包括皮层(22例次),皮层下白质(7例次),脑干(9例次)。11例患儿对一线免疫治疗均敏感,8例患儿在激素减量过程中出现复发,6例复发患儿加用二线免疫抑制剂治疗,包括环磷酰胺1例,吗替麦考酚酯5例。随访5~99个月,末次随访时,所有患儿均处于缓解期,儿童脑功能评分1分10例,2分1例。结论MNOS以年长儿为主,急性发作期以抽搐、精神行为异常常见。头颅磁共振成像显示受累广泛,以皮层受累为主。复发率相对较高,对免疫治疗敏感,缓解期未发现明显神经系统功能障碍。

Objective To investigate the clinical features,imaging findings and prognosis of children with overlapping syndrome of myelin oligodendrocyte glycoprotein(MOG)antibody disease and anti-N-methyl-D aspartate receptor(NMDAR)encephalitis(MNOS).Methods The clinical manifestations,immunological antibodies in blood and cerebrospinal fluid,cranial image,treatment and follow-up of 11 patients diagnosed as MNOS in the Department of Neurology,Beijing Children′s Hospital from January 2011 to April 2019 were analyzed retrospectively.Results A total of 11 patients,including 4 males and 7 females were analyzed,the age of onset was(10.4±2.3)years.A total of 29 episodes occurred in 11 children.At the last follow-up,8 cases showed relapsed remission course,the interval of recurrence was 3 to 60 months.The onset symptoms of 11 patients included convulsions(10 cases),lethargy(6 cases),psychosis(6 cases).Among 29 episodes,the common symptoms were convulsions(16 episodes),psychosis(13 episodes),and lethargy(10 episodes).According to the diagnostic criteria of anti-NMDAR encephalitis and MOG-antibody disease,29 episodes were divided into three phenotypes,including anti-NMDAR encephalitis(4 episodes),MOG-antibody diseases(10 episodes)and overlapping types(15 episodes).Twenty-seven times of acute stage cranial magnetic resonance imaging(MRI)were available,common lesions included cortical focus(22 times),subcortical white matter(7 times),brainstem(9 times).All patients were sensitive to first-line immunotherapy.Eight patients had recurrence during glucocorticoid reduction,6 of them were treated with additional second-line immunosuppressive therapy,including cyclophosphamide(1 case)and mycophenolate mofetil(5 cases).The follow-up time of patients were 5-99 months.At the last follow-up,all patients were in remission,the pediatric cerebral performance category(PCPC)score was 1(10 cases)and 2(1 cases).Conclusions MNOS mainly affects older children.In the period of acute episodes,convulsions and psychosis are common.The cranial MRI showed extensive brain involvement and mainly in the cortex.The recurrence rates of MNOS are relatively high,patients are sensitive to first-line immunotherapy.No significant neurological dysfunction was left in the remission stage.