Wingerchuk Dean M
Mayo Clinic College of Medicine, Scottsdale, AZ 85259, United States.
J Neurol Sci. 2009 Nov 15;286(1-2):18-23. doi: 10.1016/j.jns.2009.08.045. Epub 2009 Sep 8.
Neuromyelitis optica is a distinct CNS demyelinating disease associated with the autoantibody NMO-IgG, which targets the water channel aquaporin-4. Neuromyelitis optica may be distinguished from typical multiple sclerosis on the basis of key clinical and neuroimaging characteristics in addition to detection of NMO-IgG. The disease has an even stronger female predilection than does multiple sclerosis, with a reasonably consistent female to male gender ratio of >3:1 reported from samples of diverse racial and regional populations worldwide. The gender distribution appears to be similar in both childhood-onset and adult-onset cases. Female gender is associated with a relapsing course and familial disease, but the influence of gender on disease severity and treatment response are not clear. Population-based epidemiological and genetic studies, with case ascertainment aided by the high specificity of NMO-IgG and consistent disease definitions, are needed to better define and understand gender effects in neuromyelitis optica.
视神经脊髓炎是一种独特的中枢神经系统脱髓鞘疾病，与靶向水通道蛋白4的自身抗体NMO-IgG相关。除检测NMO-IgG外，视神经脊髓炎可根据关键的临床和神经影像学特征与典型的多发性硬化症相鉴别。该疾病比多发性硬化症更倾向于女性，全球不同种族和地区人群样本报告的女性与男性性别比相当一致，大于3:1。儿童期发病和成年期发病病例的性别分布似乎相似。女性与复发病程和家族性疾病相关，但性别对疾病严重程度和治疗反应的影响尚不清楚。需要开展基于人群的流行病学和遗传学研究，借助NMO-IgG的高特异性和一致的疾病定义来确定病例，以更好地界定和理解视神经脊髓炎中的性别效应。
