NMOSD and MOGAD: an evolving disease spectrum

Area of Science:

• Neuroimmunology
• Central Nervous System Inflammatory Disorders

Background:

• Neuromyelitis optica spectrum disorder (NMOSD) is a CNS inflammatory condition associated with aquaporin 4 autoantibodies (AQP4-IgGs).
• MOG antibody-associated disease (MOGAD) presents similarly but lacks AQP4-IgGs, featuring autoantibodies against myelin oligodendrocyte glycoprotein (MOG).
• The understanding of NMOSD is expanding to include MOGAD and double-seronegative NMOSD, forming a disease spectrum.

Purpose of the Study:

• To review and compare the epidemiology, clinical features, neuroimaging, pathology, and immunology of NMOSD and MOGAD.
• To discuss the evolving concept of NMOSD and its related conditions.
• To explore emerging therapeutic strategies for these neuroinflammatory diseases.

Main Methods:

• Comparative review of existing literature on NMOSD and MOGAD.
• Analysis of epidemiological data, clinical presentations, and diagnostic biomarkers.
• Discussion of current and potential future treatment modalities.

Main Results:

• NMOSD and MOGAD share optic neuritis and myelitis but differ in pathophysiology, clinical profiles, and neuroimaging.
• MOGAD can be monophasic or relapsing; NMOSD is typically relapsing.
• AQP4-IgG-seronegative NMOSD is a heterogeneous group requiring further investigation.

Conclusions:

• NMOSD is now understood as a spectrum including AQP4-IgG-seropositive NMOSD, MOGAD, and double-seronegative NMOSD.
• MOGAD has distinct characteristics from AQP4-IgG-seropositive NMOSD, necessitating tailored diagnostic and therapeutic approaches.
• Novel therapies targeting complement, B cells, or IL-6 receptors for NMOSD may offer future treatment options for MOGAD.