Ibis Soto de Castillo, Omaira Molina, Arnoldo Soto, Elizabeth Armas, Sandra Mendoza, Carlota Castillo Maria, Elizabeth Castro, Laura Vink, Rosalba Leon, Oscar Valbuena, Luisa Del Moral, Nahir Acosta, Hernandez Freda
Department of Neurology. Hospital Universitario de Maracaibo, Maracaibo, Venezuela.
Department of Neurology. Hospital Universitario de Maracaibo, Maracaibo, Venezuela.
Mult Scler Relat Disord. 2021 Jan;47:102652. doi: 10.1016/j.msard.2020.102652. Epub 2020 Nov 26.
Neuromyelitis optica spectrum disorders (NMOSD), is a rare autoimmune inflammatory disease of the central nervous system. Since the countries of Latin America (LATAM) show contrast in geographic, social, environmental factors, and genetic heterogeneity, the information about NMOSD epidemiology in the region allows a better understanding of the disease and its clinical outcome.
To determine the prevalence, relative frequency (RF), and clinical characteristics of NMOSD in a multiethnic Venezuelan cohort of patients with demyelinating disorders.
We conducted a retrospective descriptive multicenter study of hospital case records of individuals with an established diagnosis of MS and NMOSD in the National Program for Multiple sclerosis (MS) from 2011 to 2018. We selected those NMOSD cases based on the 2006 Wingerchuck and the 2015 International panel for the diagnosis of Neuromyelitis optica (IPND) criteria.
We identified 249 patients with NMOSD. The prevalence was 2.11 per100,000 individuals (95% confidence interval (CI)1.85 2.37), the RF was 23%, and the MS/NMOSD ratio was 3.2:1. The average disease onset occurred by the fourth decade of life (34±14.8 years of age); with a strong female predominance (female to male ratio: 4:1). Mestizos constituted 86,7% of this cohort. Most of the patients presented initially with simultaneous optic neuritis (ON) and acute transverse myelitis (ATM) and a recurrent course was registered in 82.3% of cases. The mean of the expanded disability status scale (EDSS) was 3.5 (IQR 2-7). Abnormal brain and spine MRI were present in 47.8% and 81.1% of patients, respectively. Antibodies against aquaporin-4 (AQP4) which were measured through a cell-based assay were positive in 55.3% of the individuals tested. The most used immunosuppressant agent was Azathioprine (57.4%).
NMOSD in Venezuela affects mainly young Mestizo women and shows one of the highest relative frequency in the region. Planning and developing healthcare programs for underserved populations as well as more comprehensive LATAM studies are required to identify the distribution and variations of its epidemiological picture.
视神经脊髓炎谱系障碍（NMOSD）是一种罕见的中枢神经系统自身免疫性炎症性疾病。由于拉丁美洲国家在地理、社会、环境因素和基因异质性方面存在差异，该地区有关NMOSD流行病学的信息有助于更好地了解该疾病及其临床结局。
确定委内瑞拉一个多民族脱髓鞘疾病患者队列中NMOSD的患病率、相对频率（RF）和临床特征。
我们对2011年至2018年国家多发性硬化症（MS）项目中已确诊为MS和NMOSD的个体的医院病例记录进行了一项回顾性描述性多中心研究。我们根据2006年Wingerchuck标准和2015年视神经脊髓炎国际诊断小组（IPND）标准选择了那些NMOSD病例。
我们确定了249例NMOSD患者。患病率为每10万人中2.11例（95%置信区间（CI）1.85 - 2.37），相对频率为23%，MS/NMOSD比率为3.2:1。平均发病年龄在生命的第四个十年（34±14.8岁）；女性占主导地位（女性与男性比例为4:1）。混血儿占该队列的86.7%。大多数患者最初同时出现视神经炎（ON）和急性横贯性脊髓炎（ATM），82.3%的病例有复发病程。扩展残疾状态量表（EDSS）的平均值为3.5（四分位间距2 - 7）。分别有47.8%和81.1%的患者脑部和脊柱MRI异常。通过基于细胞的检测方法检测的抗水通道蛋白4（AQP4）抗体在55.3%的检测个体中呈阳性。最常用的免疫抑制剂是硫唑嘌呤（57.4%）。
委内瑞拉的NMOSD主要影响年轻的混血女性，并且在该地区显示出最高的相对频率之一。需要为服务不足的人群规划和制定医疗保健项目，以及开展更全面的拉丁美洲研究，以确定其流行病学情况的分布和变化。
