Department of Paediatric Neurology, Great Ormond Street Hospital for Children, London, UK.
Children's Neurosciences, Evelina London Children's Hospital @ Guy's and St. Thomas' NHS Foundation Trust, King's Health Partners Academic Health Science Centre, London, UK.
Mult Scler. 2020 Mar;26(3):333-342. doi: 10.1177/1352458518823486. Epub 2019 Feb 7.
To identify predictors of epilepsy and clinical relapses in children presenting with acute disseminated encephalomyelitis (ADEM).
Children presenting with ADEM between 2005 and 2017 and tested clinically for MOG-Ab were identified from three tertiary paediatric neurology centres in the United Kingdom. Patients were followed up for a median of 6 years (range, 1-16 years).
A total of 74 children were studied (38 females; median age at first presentation: 4.5 years (range, 1.4-16 years)). MOG-Ab was positive in 50/74 (67.6%) of cases, and 27 (54%) of MOG-Ab positive children presented with a neurological relapse over time. MOG-Ab was more frequently positive in the relapsing group than in the monophasic group (27/31 vs 23/43; odds ratio 5.9 (95% CI: 1.8-19.7); = 0.002). 16/74 (22%) children had seizures during the acute presentation with ADEM and 12/74 (16.2%) patients were diagnosed with post-ADEM epilepsy. The diagnosis of post-ADEM epilepsy was more frequently observed in children with relapsing disease than monophasic disease (10/31 vs 2/43; odds ratio 9.8 (95% confidence interval (CI): 2.0-48.7); = 0.003), in children who had positive intrathecal oligoclonal bands than those with negative bands (4/7 vs 4/30; odds ratio 8.7 (95% CI: 1.4-54.0); = 0.027) and in children who had positive MOG-Ab than negative MOG-Ab cases (11/12 vs 39/62; odds ratio 6.5 (95% CI:0.8-53.6); = 0.051).
A higher relapse rate and a greater risk of post-ADEM epilepsy in children with MOG-Ab-associated disease may indicate a chronic disease with immune-mediated seizures in these children.
确定急性播散性脑脊髓炎(ADEM)患儿发生癫痫和临床复发的预测因素。
从英国的三个三级儿科神经病学中心确定了 2005 年至 2017 年间出现 ADEM 且经临床检测存在髓鞘少突胶质细胞糖蛋白抗体(MOG-Ab)的患儿。对这些患者进行了中位 6 年(范围 1-16 年)的随访。
共研究了 74 例患儿(女性 38 例;首次发病时的中位年龄:4.5 岁(范围 1.4-16 岁))。MOG-Ab 阳性者 50/74(67.6%)例,MOG-Ab 阳性患儿中有 27 例(54%)随时间出现神经复发。复发组 MOG-Ab 阳性率高于单相组(27/31 比 23/43;比值比 5.9(95%CI:1.8-19.7); = 0.002)。74 例患儿中有 16 例(22%)在急性 ADEM 发作时出现癫痫发作,74 例患儿中有 12 例(16.2%)被诊断为 ADEM 后癫痫。与单相疾病相比,复发疾病患儿更易出现 ADEM 后癫痫(10/31 比 2/43;比值比 9.8(95%置信区间(CI):2.0-48.7); = 0.003),MOG-Ab 阳性患儿比 MOG-Ab 阴性患儿更易出现 ADEM 后癫痫(4/7 比 4/30;比值比 8.7(95%CI:1.4-54.0); = 0.027),MOG-Ab 阳性患儿比 MOG-Ab 阴性患儿更易出现 ADEM 后癫痫(11/12 比 39/62;比值比 6.5(95%CI:0.8-53.6); = 0.051)。
MOG-Ab 相关疾病患儿的复发率较高,且发生 ADEM 后癫痫的风险较高,这可能表明这些患儿存在免疫介导的癫痫发作的慢性疾病。