Neuromyelitis Optica Spectrum Disorder (NMOSD) is a demyelinating disease with a high relapse rate and risk of disability accrual. The condition is an astrocytopathy, with antibodies to the aquaporin-4 (AQP4) water channel being detected in AQP4-IgG seropositive disease. Presentation is uncommon in the pediatric age range, accounting for about 3%-5% of cases. NMOSD is more prevalent in populations of Black or East Asian ancestry. Core clinical syndromes include optic neuritis, acute myelitis, area postrema syndrome, acute brainstem syndrome, acute diencephalic syndrome, and symptomatic cerebral syndrome. First-line treatment options in pediatrics include rituximab, azathioprine, and mycophenolate mofetil. Over half of children with AQP4-IgG seropositive NMOSD develop permanent disability, particularly in visual and motor domains. Novel therapeutic targets in the adult population have been developed and are changing the treatment landscape for this disorder.

视神经脊髓炎谱系障碍 (NMOSD) 是一种脱髓鞘疾病，具有高复发率和致残风险。这种情况是一种星形细胞病，在 AQP4-IgG 血清阳性疾病中检测到水通道蛋白 4 (AQP4) 水通道抗体。表现在儿科年龄范围内并不常见，约占病例的 3%-5%。NMOSD 在黑人或东亚血统人群中更为普遍。核心临床综合征包括视神经炎、急性脊髓炎、后区综合征、急性脑干综合征、急性间脑综合征和症状性脑综合征。儿科的一线治疗选择包括利妥昔单抗、硫唑嘌呤和吗替麦考酚酯。超过一半的 AQP4-IgG 血清阳性 NMOSD 儿童会出现永久性残疾，特别是在视觉和运动领域。