背景：视神经脊髓炎谱系障碍（NMOSD）是免疫介导的中枢神经系统（CNS）炎症性疾病，主要表现为视神经炎和急性脊髓炎。NMOSD可与水通道蛋白4抗体（AQP4IgG）的血清阳性相关，髓鞘少突胶质细胞糖蛋白抗体（MOGIgG），或者两者都可以是血清阴性的。在这项研究中,我们对血清阳性和血清阴性的儿科NMOSD患者进行了回顾性检查.
方法：数据来自全国所有参与中心。诊断为NMOSD的患者根据血清学分为三个亚组：AQP4IgGNMOSD，MOGIgGNMOSD，和双血清阴性（DN）NMOSD。对随访至少6个月的患者进行统计学比较。
结果：该研究包括45名患者，29名女性和16名男性（比例：1.8），平均年龄15.16±4.93（范围5.5-27）岁。发病年龄,临床表现，AQP4IgGNMOSD（n=17）和脑脊液检查结果相似，MOGIgGNMOSD（n=10），和DNNMOSD(n=18)组。与DNNMOSD相比，AQP4IgG和MOGIgGNMOSD组的多相过程更频繁（p=0.007）。组间的年复发率和残疾率相似。大多数常见的残疾类型与视路和脊髓受累有关。利妥昔单抗在AQP4IgGNMOSD中的作用MOGIgGNMOSD中的静脉免疫球蛋白，DNNMOSD中的硫唑嘌呤通常是维持治疗的首选。
结论：在我们的系列中，有相当数量的双重血清连贯剂，根据初次就诊时的临床和实验室检查结果,NMOSD的三个主要血清学组无法区分.他们的结果在残疾方面相似，但血清反应阳性的患者应该更密切地随访复发.
BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) are immune-mediated inflammatory disorders of the central nervous system (CNS) mostly presenting as optic neuritis and acute myelitis. NMOSD can be associated with seropositivity for aquaporin 4 antibody (AQP4 IgG), myelin oligodendrocyte glycoprotein antibody (MOG IgG), or can be seronegative for both. In this
study, we retrospectively examined our seropositive and seronegative pediatric NMOSD patients.
METHODS: Data were collected from all participating centres nationwide. Patients diagnosed with NMOSD were divided into three subgroups according to serology: AQP4 IgG NMOSD, MOG IgG NMOSD, and double seronegative (DN) NMOSD. Patients with at least six months of follow-up were compared statistically.
RESULTS: The
study included 45 patients, 29 female and 16 male (ratio:1.8), mean age 15.16 ± 4.93 (range 5.5-27) years. Age at onset, clinical manifestations, and cerebrospinal fluid findings were similar between AQP4 IgG NMOSD (n = 17), MOG IgG NMOSD (n = 10), and DN NMOSD (n = 18) groups. A polyphasic course was more frequent in the AQP4 IgG and MOG IgG NMOSD groups than DN NMOSD (p = 0.007). The annualized relapse rate and rate of disability were similar between groups. Most common types of disability were related to optic pathway and spinal cord involvement. Rituximab in AQP4 IgG NMOSD, intravenous immunoglobulin in MOG IgG NMOSD, and azathioprine in DN NMOSD were usually preferred for maintenance treatment.
CONCLUSIONS: In our series with a considerable number of double seronegatives, the three major serological groups of NMOSD were indistinguishable based on clinical and laboratory findings at initial presentation. Their outcome is similar in terms of disability, but seropositive patients should be more closely followed-up for relapses.
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