机构：[1]Neurology Department of First Affiliated Hospital of Kunming Medical University, Kunming, China/Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, Oxford, UK.内科科室外科科室神经内科泌尿外科昆明医科大学附属第一医院[2]Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, Oxford, UK/Neurology Department, Wexham Park hospital, Frimley Foundation Health Trust, Slough, UK.[3]Department of Neurology, Stroke and Neurological Rehabilitation, Wolski Hospital, Warsaw, Poland.[4]Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, Oxford, UK.[5]Department Clinical Neurology, John Radcliffe Hospital, Oxford OX3 9DU, UK.
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.
基金：
Yan Wu is supported by Yunnan Province Clinical Research Center for Neurological Diseases (grant no 202002AA100204) and Yunnan Fundamental Research Projects (grant nos 202201AT070291 and 202301AY070001-197).Maciej Juryńczyk receives funding from Polish National Agency For Academic Exchange (PPN/PPO/2020/1/00043/U/00001).
第一作者机构：[1]Neurology Department of First Affiliated Hospital of Kunming Medical University, Kunming, China/Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, Oxford, UK.
通讯作者：
通讯机构：[4]Nuffield Department of Clinical Neurosciences, Oxford University Hospitals, Oxford, UK.[5]Department Clinical Neurology, John Radcliffe Hospital, Oxford OX3 9DU, UK.
