Cross Shelley Ann
Department of Neurology, Mayo Clinic, Rochester, Minnesota 55905, USA.
J Neuroophthalmol. 2007 Mar;27(1):57-60. doi: 10.1097/WNO.0b013e3180334d27.
Neuromyelitis optica (NMO), or Devic disease, has been distinguished from multiple sclerosis (MS) by the presence of optic neuritis that is usually bilateral, simultaneous, and often severe, myelopathic findings accompanied by longitudinally extensive spinal cord imaging abnormalities, no brain imaging abnormalities typical of MS, and often rapid progression to debility and even death. Researchers at the Mayo Clinic have identified an immunoglobulin marker of NMO (the "NMO antibody") that binds selectively to the aquaphorin-4 water channel and may play a causative role. This marker has been found in Japanese patients with opticospinal MS, prompting the suggestion that NMO and Japanese opticospinal MS are the same disorder. The NMO antibody, which predicts frequent relapse of myelopathy and optic neuritis, is also found in patients with lupus erythematosus and Sjögren syndrome who also have severe optic neuritis and longitudinally extensive myelitis. Because this antibody is also found in patients with optic neuritis and myelitis who have brain signal abnormalities atypical of MS, the diagnosis of NMO has been revised to allow inclusion of these brain imaging abnormalities. Proper distinction of NMO from MS is important because the two disorders may respond differently to immune modulatory therapy.
视神经脊髓炎(NMO),即德维克病,已与多发性硬化症(MS)区分开来,其特征为通常双侧同时出现且往往较为严重的视神经炎、伴有纵向广泛脊髓成像异常的脊髓病变表现、无MS典型的脑成像异常,且病情往往迅速进展至虚弱甚至死亡。梅奥诊所的研究人员已确定一种NMO的免疫球蛋白标志物(“NMO抗体”),它能选择性地结合水通道蛋白4水通道,可能起致病作用。在日本视神经脊髓型MS患者中发现了这种标志物,这促使人们提出NMO与日本视神经脊髓型MS是同一种疾病的观点。预测脊髓病变和视神经炎频繁复发的NMO抗体,也存在于患有红斑狼疮和干燥综合征且同样患有严重视神经炎和纵向广泛脊髓炎的患者中。由于在患有非MS典型脑信号异常的视神经炎和脊髓炎患者中也发现了这种抗体,NMO的诊断已修订,以纳入这些脑成像异常情况。准确区分NMO和MS很重要,因为这两种疾病对免疫调节治疗的反应可能不同。