Follow-up study on Chinese children with relapsing MOG-IgG-associated central nervous system demyelination
Follow-up study on Chinese children with relapsing MOG-IgG-associated central nervous system demyelination
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DOI：
10.1016/j.msard.2018.12.001
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发表时间：
2019-02-01
影响因子：
4
通讯作者：
Wu, Ye
中科院分区：
文献类型：
--
作者：
Zhou, Ji;Lu, Xiaopeng;Wu, Ye
背景：一些研究报道了主要发生在高加索儿童的复发性MOG-Ig G相关性中枢神经系统脱髓鞘的临床特征。目前尚不清楚中国儿童是否具有相同的表型。目的：描述中国儿童复发性MOG-Ig G相关性脱髓鞘的临床特点。方法：对来自中国两家三级医院的23例复发性MOG-Ig G相关性脱髓鞘患儿进行随访研究。分析每次脱髓鞘发作的表型特征、神经影像特征、脑脊液/血清中的自身免疫抗体、对疾病改良剂的反应和病程中的功能缺陷。结果：发病年龄中位数为5.38(2.33~12.75)岁。男女比例为1：1.30。末次随访病程2.33年(1.00~8.92)年。(1)临床表型：ADEM是最常见的首发表现(12/23，52.17%)。在病程中的82次发作中，ADEM也是最常见的表型(30/82，36.59%)，其次是ON(24/82，29.27%)。(2)影像表现：57/70(81.43%)急性发作期MRI发现新的病变。脑部新发病灶最常见的部位为皮质旁白质(45/57，78.95%)。在46例幕上白质病变的MRI扫描中，ADEM样表现最常见(25/46，54.35%)，5/46(10.87%)表现为脑白质营养不良样表现。(3)实验室检查：2例(2/12，16.67%)脑脊液中检测到抗NMDAR受体抗体，其中1例出现抗-NMDAR脑炎相关症状。(4)疗效及结果：19例服用抗肿瘤药物(包括美罗华、霉酚酸酯、硫唑嘌呤等)6个月以上的患者，中位年复发率由治疗前的1.71降至治疗中的0.44(P<；0.05)，11例(57.89%)患者无复发。最后一次随访时EDSS评分中位数为1.0(0~3.5)。视觉功能障碍(12/23，52.17%)是最常见的神经系统后遗症，部分患者伴有认知功能障碍和癫痫。结论：复发性MOG-Ig G相关性CNS脱髓鞘患者的表型特征与高加索儿童相似。ADEM是所有脱髓鞘发作中最常见的表型，其次是On。脑部病变常见且广泛，表现为ADEM样甚至脑白质营养不良样改变。视觉功能障碍是最常见的神经后遗症。虽然一些疾病修饰药物可以降低ARR，但最佳治疗方法还需要进一步研究。
Background: Some studies have reported clinical features of relapsing MOG-IgG-associated CNS demyelination principally in Caucasians children. It is not clear whether Chinese children share the same phenotype.Objective: To delineate the clinical characteristics in Chinese children with relapsing MOG-IgG-associated demyelination.Methods: A follow-up study on 23 Children with relapsing MOG-IgG-associated demyelination from two Chinese tertiary hospitals was performed. Phenotypic features at each demyelinating attacks, neuroimaging characteristics, autoimmune antibodies in CSF/serum, response to disease modifying drugs and functional deficits during the disease course were analyzed.Results: The median age at disease onset was 5.38 (2.33-12.75) years. The male to female ratio was 1:1.30. The disease duration was 2.33(1.00-8.92) years at the last follow-up. (1) Clinical phenotypes: ADEM was the most common initial presentation (12/23, 52.17%). In 82 attacks during disease course, ADEM was also the most common phenotype (30/82, 36.59%), followed by ON (24/82, 29.27%). (2) Imaging findings: 57/70 (81.43%) brain MRI scans during acute attacks showed new lesions. The most common location of new lesions in brain was the juxtacortical white matter (45/57, 78.95%). In 46 brain MRI scans with supratentorial white matter lesions, ADEM-like patterns were most common (25/46, 54.35%), and 5/46 (10.87%) scans exhibited leukodystrophylike patterns. (3) Laboratory examinations: Anti-NMDA receptor IgG in CSF was detected in two patients (2/12, 16.67%), with one patient presented with anti-NMDAR encephalitis associated symptoms. (4) Therapeutic responses and outcomes: In 19 patients treated with disease-modifying drugs (including rituximab, mycophenolate mofetil, azathioprine and so on) longer than 6 months, median annualised relapse rates decreased from 1.71 before treatment to 0.44 during treatment (P < 0.05), with eleven patients (11/19, 57.89%) having no relapses. Median EDSS score at the last follow-up was 1.0(0-3.5). Visual dysfunction (12/23, 52.17%) was the most common neurological sequela, with cognitive dysfunction and epilepsy in some of patients.Conclusions: The phenotypic features of Chinese children with relapsing MOG-IgG-associated CNS demyelination were similar to that in Caucasian children. ADEM was the most common phenotype in all demyelinating attacks, followed by ON. Cerebral lesions were common and extensive, manifested as ADEM-like or even leukodystrophylike patterns. Visual dysfunction was the most common neurological sequela. Although some disease-modifying drugs could reduce ARR, optimal treatment needs future study.