Autoimmune Optic Neuropathy and Demyelinating Disorders

Autoimmune optic neuropathy encompasses a spectrum of immune-mediated disorders in which inflammatory processes target the optic nerve, resulting in demyelination, axonal injury and varying degrees of visual loss. Central to this group are conditions associated with autoantibodies against aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG), which define distinct clinical entities within neuromyelitis optica spectrum disorder (NMOSD) and MOG-antibody-associated disease (MOGAD). Patients typically present with acute or subacute vision decline, often accompanied by pain on eye movement, and may exhibit bilateral involvement or steroid-dependent relapsing courses. Imaging with magnetic resonance and optical coherence tomography (OCT) enables quantification of nerve-fibre loss and guides prognosis. While multiple sclerosis-related optic neuritis remains common, AQP4-ON and MOG-ON display unique immunopathological signatures, relapse patterns and long-term outcomes. Early recognition of seronegative and atypical forms, such as chronic relapsing inflammatory optic neuropathy (CRION), is crucial to prevent irreversible damage. Advances in immunotherapy, including targeted monoclonal antibodies and precision-medicine approaches, have demonstrated the potential to modify disease course and preserve vision. Globally, these disorders represent a major cause of visual morbidity in young adults, underscoring the need for rapid diagnostic pathways, standardised treatment protocols and ongoing research into neuroprotective strategies.

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Autoimmune Optic Neuropathy and Demyelinating Disorders publication trend

The graph below shows the total number of articles in autoimmune optic neuropathy and demyelinating disorders across all publications each year (not limited to Nature Index journals).

Technical terms

Optic neuritis: Inflammation of the optic nerve leading to acute vision loss and pain on eye movement.

Aquaporin-4 (AQP4): A water-channel protein expressed on astrocyte endfeet; autoantibodies against AQP4 characterise NMOSD.

Myelin oligodendrocyte glycoprotein (MOG): A myelin sheath antigen on oligodendrocytes; MOG-IgG is implicated in a distinct demyelinating syndrome.

Neuromyelitis optica spectrum disorder (NMOSD): A relapsing demyelinating condition of the central nervous system primarily affecting optic nerves and spinal cord, often AQP4-antibody positive.

Optical coherence tomography (OCT): A non-invasive imaging technique that quantifies retinal layer thickness to assess neuroaxonal damage.

Chronic relapsing inflammatory optic neuropathy (CRION): A steroid-dependent, recurrent form of optic neuritis not fulfilling MS or NMOSD criteria.

References

1. Different immunological mechanisms between AQP4 antibody-positive and MOG antibody-positive optic neuritis based on RNA sequencing analysis of whole blood. Frontiers in Immunology (2023).
2. Beyond Myelin Oligodendrocyte Glycoprotein and Aquaporin-4 Antibodies: Alternative Causes of Optic Neuritis. International Journal of Molecular Sciences (2023).
3. AQP4-IgG and MOG-IgG Related Optic Neuritis—Prevalence, Optical Coherence Tomography Findings, and Visual Outcomes: A Systematic Review and Meta-Analysis. Frontiers in Neurology (2020).
4. Chronic relapsing inflammatory optic neuropathy (CRION): a manifestation of myelin oligodendrocyte glycoprotein antibodies. Journal of Neuroinflammation (2018).
5. Age-dependent favorable visual recovery despite significant retinal atrophy in pediatric MOGAD: how much retina do you really need to see well?. Journal of Neuroinflammation (2021).