Banwell B, Tenembaum S, Lennon V A, Ursell E, Kennedy J, Bar-Or A, Weinshenker B G, Lucchinetti C F, Pittock S J
Division of Pediatric Neurology and the Research Institute, The Hospital for Sick Children, University of Toronto, Canada.
Neurology. 2008 Jan 29;70(5):344-52. doi: 10.1212/01.wnl.0000284600.80782.d5. Epub 2007 Dec 19.
To determine seroprevalence of neuromyelitis optica (NMO)-IgG in childhood CNS inflammatory demyelinating disorders.
We analyzed demographic, clinical, and radiologic data in a blinded fashion and assessed serum NMO-IgG status for 87 children: 41 with relapsing-remitting multiple sclerosis (RRMS), 17 with NMO, 13 with monophasic/recurrent optic neuritis (ON), 13 with transverse myelitis, of whom 10 were longitudinally extensive on MRI spine (LETM), and another 3 with LETM in the context of acute disseminated encephalomyelitis (ADEM).
Ten of the 87 children (11%) were seropositive. Eight of 17 with NMO (47%) were seropositive (7 of 9 with relapsing NMO [78%], 1 of 8 with monophasic NMO [12.5%]). Two other children were seropositive: 1 of 5 with recurrent ON and one child with recurrent LETM. No seropositive case was identified among 41 with RRMS (14% of whom had LETM at some point in their clinical course), 8 with monophasic ON, 9 with monophasic LETM, or 3 with LETM in the context of ADEM.
The similar frequency of neuromyelitis optica (NMO)-IgG in both childhood and adult cases of NMO, and its rarity in relapsing-remitting multiple sclerosis, supports the concept that these diseases have a similar pathogenesis in childhood and adulthood. It is noteworthy that none of nine children with monophasic longitudinally extensive transverse myelitis (LETM) was NMO-IgG-seropositive. Furthermore, LETM does not appear to be as predictive of an NMO spectrum disorder in children as it is in adults. Longitudinal studies of larger pediatric LETM cohorts are required to ascertain whether the absence of NMO-IgG is a negative predictor for relapse in this childhood entity.
确定儿童中枢神经系统炎性脱髓鞘疾病中视神经脊髓炎（NMO）-IgG的血清阳性率。
我们以盲法分析了人口统计学、临床和放射学数据，并评估了87名儿童的血清NMO-IgG状态：41例复发缓解型多发性硬化症（RRMS）、17例NMO、13例单相/复发性视神经炎（ON）、13例横贯性脊髓炎，其中10例在脊柱MRI上为纵向广泛横贯性脊髓炎（LETM），另外3例在急性播散性脑脊髓炎（ADEM）背景下出现LETM。
87名儿童中有10名（11%）血清学阳性。17例NMO中有8例（47%）血清学阳性（9例复发型NMO中有7例[78%]，8例单相型NMO中有1例[12.5%]）。另外两名儿童血清学阳性：5例复发性ON中有1例，1例复发性LETM患儿。41例RRMS（其中14%在临床病程中的某个时间点出现LETM）、8例单相型ON、9例单相型LETM或3例ADEM背景下的LETM中均未发现血清学阳性病例。
儿童和成人NMO病例中视神经脊髓炎（NMO）-IgG的频率相似，且在复发缓解型多发性硬化症中罕见，这支持了这些疾病在儿童期和成年期具有相似发病机制的概念。值得注意的是，9例单相型纵向广泛横贯性脊髓炎（LETM）患儿中无一例NMO-IgG血清学阳性。此外，LETM在儿童中似乎不像在成人中那样能预测NMO谱系障碍。需要对更大的儿童LETM队列进行纵向研究，以确定NMO-IgG的缺失是否是该儿童疾病复发的阴性预测指标。
