Tillema J M, McKeon A
Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
J Child Neurol. 2012 Nov;27(11):1437-47. doi: 10.1177/0883073812451495. Epub 2012 Aug 1.
The evaluation of inflammatory central nervous system disorders in childhood with predominant involvement of the optic nerves and spinal cord has been greatly enhanced over the last decade with identification of a group of disorders unified by the detection of neuromyelitis optica (NMO)-IgG, an antibody targeting the central nervous system-predominant water channel aquaporin-4. Clinical syndromes are predominated by the relapsing form of NMO but also include encephalopathic variants that can mimic acute disseminated encephalomyelitis. Maintenance immunotherapy is used to prevent relapses in NMO-IgG-seropositive patients. In contrast, NMO-IgG-seronegative children with NMO more commonly have a monophasic course (simultaneous occurrence of optic neuritis and transverse myelitis) and do not require remission-maintaining immunotherapy, but close surveillance is advised. Current clinical, pathological, and pathogenetic knowledge is reviewed with a focus on clinical presentation, neuroimaging findings, serological investigations, and treatment of children with disorders within the spectrum of central nervous system aquaporin-4 autoimmunity.
在过去十年中，随着一组由视神经脊髓炎（NMO）-IgG检测所统一的疾病的发现，儿童期以视神经和脊髓受累为主的炎症性中枢神经系统疾病的评估有了很大进展。NMO-IgG是一种靶向中枢神经系统主要水通道水通道蛋白-4的抗体。临床综合征以复发型NMO为主，但也包括可模仿急性播散性脑脊髓炎的脑病变体。维持性免疫疗法用于预防NMO-IgG血清阳性患者的复发。相比之下，NMO-IgG血清阴性的NMO儿童更常见单相病程（视神经炎和横贯性脊髓炎同时发生），不需要缓解期维持免疫疗法，但建议密切监测。本文综述了当前的临床、病理和发病机制知识，重点关注中枢神经系统水通道蛋白-4自身免疫范围内疾病患儿的临床表现、神经影像学检查结果、血清学检查及治疗。
