Neurology Department of First Affiliated Hospital of Kunming Medical University, Kunming, China/Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, Oxford, UK.
Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, Oxford, UK/Neurology Department, Wexham Park hospital, Frimley Foundation Health Trust, Slough, UK.
Mult Scler. 2023 Oct;29(11-12):1353-1362. doi: 10.1177/13524585231199819. Epub 2023 Sep 23.
Most patients with neuromyelitis optica spectrum disorders (NMOSD) test positive for aquaporin-4 antibody (AQP4-IgG) or myelin oligodendrocyte glycoprotein antibodies (MOG-IgG). Those who are negative are termed double-negative (DN) NMOSD and may constitute a diagnostic and therapeutic challenge. DN NMOSD is a syndrome rather than a single disease, ranging from a (postinfectious) monophasic illness to a more chronic syndrome that can be indistinguishable from AQP4-IgG+ NMOSD or develop into other mimics such as multiple sclerosis. Thus, underlying disease mechanisms are likely to be heterogeneous. This topical review aims to (1) reappraise antibody-negative NMOSD definition as it has changed over time with the development of the AQP4 and MOG-IgG assays; (2) outline clinical characteristics and the pathophysiological nature of this rare entity by contrasting its differences and similarities with antibody-positive NMOSD; (3) summarize laboratory characteristics and magnetic resonance imaging findings of DN NMOSD; and (4) discuss the current treatment for DN NMOSD.
大多数视神经脊髓炎谱系疾病（NMOSD）患者的水通道蛋白 4 抗体（AQP4-IgG）或髓鞘少突胶质细胞糖蛋白抗体（MOG-IgG）检测呈阳性。那些检测结果为阴性的患者被称为双阴性（DN）NMOSD，可能构成诊断和治疗上的挑战。DN NMOSD 是一种综合征而不是单一疾病，从（感染后）单相疾病到更慢性的综合征不等，与 AQP4-IgG+ NMOSD 难以区分或发展为其他类似疾病，如多发性硬化症。因此，潜在的疾病机制可能是异质的。本专题综述旨在：（1）重新评估抗体阴性 NMOSD 的定义，因为随着 AQP4 和 MOG-IgG 检测的发展，其定义已经发生了变化；（2）通过对比抗体阳性 NMOSD，概述这种罕见实体的临床特征和病理生理性质；（3）总结 DN NMOSD 的实验室特征和磁共振成像结果；（4）讨论 DN NMOSD 的当前治疗方法。
