Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Pediatric Allergy and Immunology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Neuropediatrics. 2022 Oct;53(5):351-357. doi: 10.1055/a-1863-1862. Epub 2022 May 26.
This study aimed to determine the seropositivity of myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) and aquaporin-4 antibodies (AQP4-Ab) and outcomes in children with acquired demyelinating syndromes (ADSs). Children (6 months-15 years) with suspected ADS were enrolled and tested for MOG-Ab and AQP4-Ab prospectively over 18 months at a tertiary care hospital in North India. Children with proven nonimmune-mediated neurological disorders were enrolled as controls. Of 79 children with suspected ADS, 66 were enrolled. Among the enrolled children with ADS, acute demyelinating encephalomyelitis (ADEM) (25) was the most common first clinical event followed by optic neuritis (ON) (20) and transverse myelitis (TM) (19; one child had ON and TM simultaneously [neuromyelitis optica spectrum disorders [NMOSDs]]), while two children had clinically isolated syndrome (CIS) apart from ON and TM. Fourteen (21.2%, confidence interval [CI] 11.3-31.1) tested positive for one antibody (12 [18.1%; 95% CI 10.5-25.5%] for MOG-Ab and 2 [3%; 95% CI 0-7.2%] for AQP4-Ab). None of the 62 controls tested positive for any antibody. The final diagnosis in those with the monophasic ADS was ADEM (21), ON (13), TM (16), and other CIS (1) while that in children with recurrent events was multiphasic disseminated encephalomyelitis (MDEM) (2), NMOSD (3), ADEM-ON (4), recurrent ON (4), and MS (2). Among those with the first event, 4/51 (7.8%; 95% CI 0.5-15.2%) were MOG-Ab positive and 2 AQP4-Ab positive, whereas 8/15 (53.3% [95% CI 28.1-78.6%]) with recurrent events (MDEM [2], ADEM-ON [4], recurrent ON [1], and recurrent TM [1]) were MOG-Ab positive. Hence, MOG-Abs are the most common antibodies detected in one in five children with pediatric ADS, especially in relapsing disease. AQP4-Abs are rare in children with ADS.
本研究旨在确定髓鞘少突胶质细胞糖蛋白抗体 (MOG-Ab) 和水通道蛋白 4 抗体 (AQP4-Ab) 在获得性脱髓鞘综合征 (ADS) 患儿中的血清阳性率和结局。在印度北部的一家三级保健医院，前瞻性地对疑似 ADS 的患儿进行了为期 18 个月的 MOG-Ab 和 AQP4-Ab 检测。将确诊为非免疫介导的神经障碍患儿作为对照组纳入。在 79 例疑似 ADS 的患儿中，有 66 例患儿纳入。在患有 ADS 的患儿中，急性播散性脑脊髓炎 (ADEM) (25 例)是最常见的首发临床事件，其次是视神经炎 (ON) (20 例)和横贯性脊髓炎 (TM) (19 例；1 例患儿同时患有 ON 和 TM [视神经脊髓炎谱系疾病 (NMOSD)])，而另外 2 例患儿除了 ON 和 TM 外还患有临床孤立综合征 (CIS)。14 例 (21.2%，置信区间 [CI] 11.3-31.1) 一种抗体检测呈阳性 (12 例 [18.1%；95% CI 10.5-25.5%] 为 MOG-Ab，2 例 [3%；95% CI 0-7.2%] 为 AQP4-Ab)。62 例对照组患儿均未检测到任何抗体阳性。单相 ADS 患儿的最终诊断为 ADEM (21 例)、ON (13 例)、TM (16 例)和其他 CIS (1 例)，而复发性疾病患儿的最终诊断为复发性多灶性脑脊髓炎 (MDEM) (2 例)、NMOSD (3 例)、ADEM-ON (4 例)、复发性 ON (4 例)和多发性硬化症 (MS) (2 例)。在首发事件患儿中，51 例中有 4 例 (7.8%；95% CI 0.5-15.2%) 为 MOG-Ab 阳性，2 例为 AQP4-Ab 阳性，而 15 例复发性疾病患儿中有 8 例 (53.3% [95% CI 28.1-78.6%]) 为 MOG-Ab 阳性 (MDEM [2 例]、ADEM-ON [4 例]、复发性 ON [1 例]和复发性 TM [1 例])。因此，MOG-Abs 是五分之一患有儿科 ADS 患儿中最常见的抗体，尤其是在复发性疾病中。AQP4-Abs 在 ADS 患儿中罕见。
Zotero 插件