Tosello B, Halbert C, Mancini J, Chabrol B, Boucraut J, Milh M
Service de neurologie pédiatrique, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue Saint-Pierre, 13005 Marseille, France.
Arch Pediatr. 2012 Aug;19(8):827-31. doi: 10.1016/j.arcped.2012.05.019. Epub 2012 Jul 11.
Devic neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system associating acute transverse myelitis and optic neuritis. This is a different disease from multiple sclerosis for which the presence of the NMO antibody directed against aquaporin 4 is a specific marker. Brain damage on MRI does not exclude the diagnosis; the location is superposable in the brain zones rich in aquaporin 4 channels. We report 2 cases of NMO with anti-aquaporin 4. One patient was not symptomatic of brain damage. In this patient, the affinity of anti-NMO for aquaporin 4, studied by flow cytometry, was particularly high. Both patients were treated with immunosuppressive agents (rituximab) due to the failure of or dependence on high-dose corticosteroids.
视神经脊髓炎（NMO）是一种中枢神经系统炎性脱髓鞘疾病，伴有急性横贯性脊髓炎和视神经炎。这是一种与多发性硬化不同的疾病，针对水通道蛋白4的NMO抗体的存在是其特异性标志物。MRI显示的脑损伤并不能排除该诊断；其损伤部位与富含水通道蛋白4通道的脑区重叠。我们报告2例抗水通道蛋白4的视神经脊髓炎病例。1例患者无脑损伤症状。在该患者中，通过流式细胞术研究发现，抗NMO对水通道蛋白4的亲和力特别高。由于高剂量皮质类固醇治疗无效或产生依赖，2例患者均接受了免疫抑制剂（利妥昔单抗）治疗。
