MOG antibody-related disorders: common features and uncommon presentations
MOG antibody-related disorders: common features and uncommon presentations
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DOI：
10.1007/s00415-017-8583-z
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发表时间：
2017-09-01
影响因子：
6
通讯作者：
Marignier, Romain
中科院分区：
文献类型：
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作者：
Cobo-Calvo, Alvaro;Ruiz, Anne;Marignier, Romain
髓磷脂少突胶质细胞糖蛋白抗体（MOG-AB）已在急性脱髓鞘性脑脊髓炎（ADEM），视神经炎（ON）和神经瘤性疾病中的敏锐脱髓鞘性疾病中，成人和谱系神经肌炎谱谱疾病（NMOSD）中的成人和疾病。我们的目的是描述儿童和成人中MOG-AB相关疾病的共同特征，并报告罕见的演讲。包括27个连续的儿科和成年患者，对MOG-AB测试阳性，最小随访为6个月。回顾性地分析了全面的流行病学，临床，放射学和实验室数据。此外，我们比较了ADEM MOG-AB阳性患者与一组ADEM MOG-AB阴性患者之间的放射学特征，该患者在同一时期招募。在整个队列中，有13（48.1％）为儿科，女性有14（51.9％）。 MOG-AB相关疾病包括8个ADEM，八个，五个分离的脊髓炎，四名NMOSD和两名患有多发性硬化症的患者，最后随访。经过17.8个月的中位随访后，11名（40.7％）患者复发。发作时最常见的临床表型是成人儿科（53.9％）和骨髓炎的脑病（50％）（p = 0.013）。两组之间没有其他差异。在比较ADEM MOG-AB阳性和阴性患者时，双侧丘脑病变在正组中更常见（P = 0.010）。在三名患者中鉴定出异常的表现：斑点脊髓增强病变，相关的畸胎瘤和一名癫痫持续状态。 MOG-AB相关疾病具有共同的临床和预后特征，但范围比最近报道的频谱宽。
Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) have been reported in acute demyelinating encephalomyelitis (ADEM), optic neuritis (ON), and neuromyelitis optica spectrum disorders (NMOSD) in adults and pediatrics. We aimed to delineate the common features of MOG-Ab-related disorders in children and adults, and report uncommon presentations. Twenty-seven consecutive pediatric and adult patients testing positive for MOG-Ab, with a minimum follow-up of 6 months, were included. Comprehensive epidemiological, clinical, radiological, and laboratory data were retrospectively analyzed. Additionally, we compared radiological features between ADEM MOG-Ab-positive patients, and a group of ADEM MOG-Ab-negative ones, recruited during the same period. Among the whole cohort, 13 (48.1%) were pediatric, and 14 (51.9%) were female. MOG-Ab-related disorders comprised eight ADEM, eight ON, five isolated myelitis, four with NMOSD and two patients with multiple sclerosis, at last follow-up. After a median follow-up of 17.8 months, 11 (40.7%) patients presented a relapse. The most frequent clinical phenotype at onset was encephalopathy in pediatrics (53.9%) and myelitis in adults (50%) (p = 0.013). There were no other differences between both groups. When comparing ADEM MOG-Ab positive and negative patients, bilateral thalamic lesions were more often found in the positive group (p = 0.010). Unusual presentations were identified in three patients: patchy spinal cord gadolinium-enhancing lesions, an associated teratoma, and one presented with status epilepticus. MOG-Ab-related disorders shared common clinical and prognostic features, but encompass a spectrum wider than recently reported.