Double-Seronegative Neuromyelitis Optica Spectrum Disorder

Hyun, Jae-Won

doi:2026.17.1.1

J Mult Scler Neuroimmunol > Volume 17(1); 2026 > Article

Review Article

Journal of Multiple Sclerosis and Neuroimmunology 2026;17(1):1-6.
DOI: https://doi.org/10.59578/jmsni.2026.17.1.1-6 Published online June 30, 2026.

이중 항체 음성 시신경척수염 범주 질환

현재원

국립암센터 신경과

Double-Seronegative Neuromyelitis Optica Spectrum Disorder

Jae-Won Hyun

Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea

Correspondence: Jae-Won Hyun, Tel: +82-31-920-1660, Fax: +82-31-925-5524,
Email: 12141@ncc.re.kr

Received: 21 March 2026 • Revised: 9 June 2026 • Accepted: 15 June 2026

Abstract

Double-seronegative neuromyelitis optica spectrum disorder (DN-NMOSD) is characterized by the absence of both aquaporin-4 immunoglobulin-G (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG) immunoglobulin-G. Growing recognition of this entity has raised questions regarding its epidemiology, underlying pathophysiology, and optimal management. In this review, we summarize the current evidence regarding the epidemiology of DN-NMOSD and discuss proposed immunopathogenic mechanisms. We also describe the reported clinical and radiological characteristics in comparison with AQP4-IgG-positive NMOSD and MOG antibody-associated disease, and review current therapeutic approaches.

Key Words: Neuromyelitis optica, Aquaporin-4, Myelin oligodendrocyte glycoprotein