Children with multiphasic disseminated encephalomyelitis and antibodies to the myelin oligodendrocyte glycoprotein (MOG): Extending the spectrum of MOG antibody positive diseases
Children with multiphasic disseminated encephalomyelitis and antibodies to the myelin oligodendrocyte glycoprotein (MOG): Extending the spectrum of MOG antibody positive diseases
复制标题
DOI：
10.1177/1352458516631038
复制
发表时间：
2016-12-01
影响因子：
5.8
通讯作者：
Rostasy, Kevin
中科院分区：
文献类型：
--
作者：
Baumann, Matthias;Hennes, Eva-Maria;Rostasy, Kevin
背景：髓鞘少突胶质细胞糖蛋白(MOG)抗体在儿童急性播散性脑脊髓炎(ADEM)、反复发作性视神经炎、神经脊髓炎视神经谱系疾病以及最近的多相播散性脑脊髓炎(MDEM)儿童中已有报道。目的：描述具有MOG抗体的儿童MDEM的临床、脑脊液(CSF)和放射学特征。方法：回顾分析MDEM患儿的临床病程、血清抗体、脑脊液、磁共振成像(MRI)检查和转归。结果：295例急性脱髓鞘患儿中，8例ADEM发作2次或以上。所有儿童均有持续性MOG抗体(中位滴度：1：1280)。所有ADEM发作包括脑病、多灶性神经体征和典型的MRI。除ADEM发作外，三名儿童在没有脑病的情况下有进一步的临床发作。首次发病的中位年龄为3岁(1-7岁)，中位随诊年龄为4年(1-8岁)。新的ADEM发作与新的神经体征和新的MRI损害相关。临床结果从正常(8人中的4人)到轻度或中度损害(8人中的4人)不等。共4名儿童在病程中每月接受免疫球蛋白治疗。结论：MDEM和持续MOG抗体的儿童构成了复发性脱髓鞘事件的独特实体，并扩大了MOG抗体相关疾病的范围。
Background: Myelin oligodendrocyte glycoprotein (MOG) antibodies have been described in children with acute disseminated encephalomyelitis (ADEM), recurrent optic neuritis, neuromyelitis optica spectrum disorders and more recently in children with multiphasic disseminated encephalomyelitis (MDEM).Objective: To delineate the clinical, cerebrospinal fluid (CSF) and radiological features of paediatric MDEM with MOG antibodies.Methods: Clinical course, serum antibodies, CSF, magnetic resonance imaging (MRI) studies and outcome of paediatric MDEM patients were reviewed.Results: A total of 8 children with two or more episodes of ADEM were identified from a cohort of 295 children with acute demyelinating events. All children had persisting MOG antibodies (median titre: 1:1280). All ADEM episodes included encephalopathy, polyfocal neurological signs and a typical MRI. Apart from ADEM episodes, three children had further clinical attacks without encephalopathy. Median age at initial presentation was 3years (range: 1-7years) and median follow-up 4years (range: 1-8years). New ADEM episodes were associated with new neurological signs and new MRI lesions. Clinical outcome did range from normal (four of the eight) to mild or moderate impairment (four of the eight). A total of four children received monthly immunoglobulin treatment during the disease course.Conclusion: Children with MDEM and persisting MOG antibodies constitute a distinct entity of relapsing demyelinating events and extend the spectrum of MOG antibody-associated diseases.