Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein
Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein
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DOI：
10.1136/jnnp-2014-308346
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发表时间：
2015-03-01
影响因子：
11
通讯作者：
Rostasy, K.
中科院分区：
文献类型：
--
作者：
Baumann, M.;Sahin, K.;Rostasy, K.
背景近年来，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）中有报道，但缺乏对该亚群的临床和神经影像学特征的研究。目的比较MOG抗体阳性和阴性的ADEM患儿的临床和神经影像学特征。结果19例ADEM患儿血清MOG抗体阳性率为1：160-1：20480，中位数为1：2560。大多数儿童显示血清MOG-IgG滴度随时间下降。与无MOG抗体的儿童相比，MOG抗体的儿童在就诊时的年龄、性别比、寡克隆带的存在、临床症状或初始严重程度方面没有差异，除了CSF细胞计数较高（p=0.038）。此外，仅在MOG抗体儿童中观察到与MOG抗体相关的进一步复发性脱髓鞘发作。所有19例MOG抗体患儿的MRI表现均一致，其特征为大、模糊和双侧病变，无非典型MRI特征（例如，主要为小病变、边界清晰的病变），与无MOG抗体患儿相比有显著差异（分别为p=0.003和p=0.032）。此外，MOG抗体阳性的儿童有更多的解剖区域受累（p=0.035）包括以纵向广泛横肌萎缩为特征的髓髓（p=0.003），更常见的是病变完全消退（p=0.036）和更好的结局结论在我们的队列中，MOG抗体阳性的ADEM患者具有一致的MRI特征，双侧和广泛的病变，纵向广泛横向肌萎缩的频率增加，与缺乏MOG抗体的儿童相比，临床结果良好。
Background Myelin oligodendrocyte glycoprotein (MOG) antibodies have been recently described in children with acute disseminating encephalomyelitis (ADEM), but the clinical and neuroradiological characterisation of this subgroup is lacking.Objective To compare the clinical and neuroradiological features of paediatric ADEM with and without MOG antibodies.Methods Clinical course, cerebrospinal fluid (CSF)-, MRI studies, outcome and MOG status of 33 paediatric ADEM prospectively studied were reviewed.Results MOG antibodies (median 1: 2560; range 1: 160-1: 20 480) were detected in 19 children with ADEM. The majority of children showed a decline of serum MOG-IgG titres over time. Children with MOG antibodies did not differ in their age at presentation, sex ratio, the presence of oligoclonal bands, clinical symptoms or initial severity, apart from a higher CSF cell count (p=0.038), compared with children without MOG antibodies. In addition, further relapsing demyelinating episodes associated with MOG antibodies were observed only in children with MOG antibodies. All 19 children with MOG antibodies had a uniform MRI pattern, characterised by large, hazy and bilateral lesions and the absence of atypical MRI features (eg, mainly small lesions, well-defined lesions), which was significantly different compared to that of children without MOG antibodies (p=0.003; and p=0.032, respectively). In addition, children with MOG antibodies had involvement of more anatomical areas (p=0.035) including the myelon characterised by a longitudinally extensive transverse myelitis (p=0.003), more often a complete resolution of lesions (p=0.036) and a better outcome (p=0.038).Conclusions Patients with ADEM with MOG antibodies in our cohort had a uniform MRI characterised by large, bilateral and widespread lesions with an increased frequency of longitudinal extensive transverse myelitis and a favourable clinical outcome in contrast to children lacking MOG antibodies.