Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India.
Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India; NeuroImmunology Laboratory, Department of Neurology, Amrita Vishwa Vidyapeetham, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala 682041, India.
Mult Scler Relat Disord. 2023 Jul;75:104736. doi: 10.1016/j.msard.2023.104736. Epub 2023 Apr 25.
Neuromyelitis Optica Spectrum Disorders (NMOSD) is an autoimmune syndrome that is frequently positive for Aquaporin 4 (AQP4) IgG or Myelin Oligodendrocyte Glycoproteins (MOG) IgG. However, dual positivity to both is rare.
To assess the prevalence of dual-positive NMOSD and outline its clinical phenotype.
DESIGN/METHODS: This is a retrospective cross-sectional study conducted at a tertiary healthcare center in South Asia between August 2018 and November 2021. The serum and/or CSF samples of suspected cases of NMOSD were tested for both AQP4-IgG and MOG-IgG using an Indirect immunofluorescence test on transfected cells.
During the study period, 1935 cases of NMOSD were tested for both antibodies- 65 patients (3.35%; 57 females and 8 males) tested positive for AQP4-IgG, 217 patients (11.21%; 122 females and 95 males) tested positive for MOG-IgG and 3 patients (0.15%; 2 females and 1 male) showed dual positivity. There was a strong female preponderance in all three groups (87.69%, 56.22%, and 66.66% respectively). This study identified 3 patients with dual positivity. The first patient (42 years, Male) presented with area postrema syndrome initially and subsequently relapsed by developing right-sided numbness of the temporal area and limbs during which he tested dual positive. The second patient (27 years, Female) presented with bilateral optic neuritis (left>right) initially and subsequently relapsed following an episode of a seizure with left-sided hemiplegia. The third patient (25 years, Female) initially presented with acute bilateral optic neuritis and later developed left-sided hemiplegia post-recovery at which point she tested dual positive. Management using methylprednisolone was ineffective for all three patients, however, plasmapheresis and/or periodic rituximab injections produced an excellent response.
Our study reports that the prevalence of dual-positive NMOSD is 0.15% and its clinical phenotype is more similar to NMO rather than MOG- associated disease.
视神经脊髓炎谱系疾病（NMOSD）是一种自身免疫综合征，常为水通道蛋白 4（AQP4）IgG 或髓鞘少突胶质细胞糖蛋白（MOG）IgG 阳性。然而，同时存在两种抗体的情况较为罕见。
评估双阳性 NMOSD 的患病率并概述其临床表型。
这是一项在南亚的一家三级医疗中心进行的回顾性横断面研究，时间为 2018 年 8 月至 2021 年 11 月。使用转染细胞的间接免疫荧光试验对疑似 NMOSD 病例的血清和/或 CSF 样本进行 AQP4-IgG 和 MOG-IgG 检测。
在研究期间，对 1935 例 NMOSD 患者进行了这两种抗体的检测-65 例患者（3.35%；57 名女性和 8 名男性）AQP4-IgG 检测阳性，217 例患者（11.21%；122 名女性和 95 名男性）MOG-IgG 检测阳性，3 例患者（0.15%；2 名女性和 1 名男性）表现为双阳性。在这三个组中均存在强烈的女性优势（分别为 87.69%、56.22%和 66.66%）。本研究共发现 3 例双阳性患者。第 1 例患者（42 岁，男性）最初表现为后极综合征，随后因右侧颞区和四肢麻木而复发，在此期间他的检测结果为双阳性。第 2 例患者（27 岁，女性）最初表现为双侧视神经炎（左侧>右侧），随后因左侧偏瘫发作伴癫痫复发。第 3 例患者（25 岁，女性）最初表现为急性双侧视神经炎，随后在恢复后出现左侧偏瘫，此时她的检测结果为双阳性。所有患者使用甲基强的松龙治疗均无效，但血浆置换和/或周期性利妥昔单抗注射产生了极好的反应。
我们的研究报告称，双阳性 NMOSD 的患病率为 0.15%，其临床表型更类似于 NMO，而不是 MOG 相关疾病。
