Neuromyelitis Optica Spectrum Disorder and Associated Infections

Neuromyelitis optica spectrum disorder (NMOSD) is a rare, relapsing inflammatory disease of the central nervous system characterised by immune-mediated damage predominantly to the optic nerves and spinal cord. The identification of pathogenic antibodies against aquaporin-4 water channels (AQP4-IgG) has transformed diagnosis and enabled targeted therapeutic strategies. Clinical manifestations range from acute bilateral optic neuritis and longitudinally extensive transverse myelitis to area postrema syndrome, brainstem syndromes and diencephalic disturbances. A growing body of evidence suggests that para-infectious immune triggers, including viral and bacterial pathogens, may precipitate first-onset attacks or relapses in predisposed individuals by promoting antigenic cross-reactivity or by altering blood–brain barrier integrity. Management requires rapid immunosuppression during acute attacks with high-dose corticosteroids, plasma exchange or immunoglobulin, followed by long-term maintenance with agents such as monoclonal antibodies or immunosuppressants to prevent further relapses. Early recognition of infection-associated presentations is essential to balance the risks of immunotherapy with the need to control underlying pathogens. Global efforts focus on refining biomarkers for disease activity and improving outcomes through personalised immunomodulation and vigilant infection surveillance.

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Neuromyelitis Optica Spectrum Disorder and Associated Infections publication trend

The graph below shows the total number of articles in neuromyelitis optica spectrum disorder and associated infections across all publications each year (not limited to Nature Index journals).

Technical terms

Aquaporin-4-IgG (AQP4-IgG): Autoantibody directed against the aquaporin-4 water channel on astrocytes, serving as a diagnostic biomarker for NMOSD.

Longitudinally Extensive Transverse Myelitis (LETM): Inflammation spanning three or more contiguous vertebral segments of the spinal cord, a hallmark of NMOSD.

Area Postrema Syndrome: Clinical syndrome characterised by intractable nausea, vomiting or hiccups due to lesions in the dorsal medullary area postrema region.

References

1. Therapy challenges for NMOSD in a patient with HIV. Multiple Sclerosis Journal (2023).
2. Neuromyelitis Optica Spectrum Disorder Attack Triggered by Herpes Zoster Infection. Multiple Sclerosis International (2020).
3. Herpes Zoster Radiculomyelitis With Aquaporin-4 Antibodies: A Case Report and Literature Review. Frontiers in Neurology (2020).
4. Neuromyelitis optica spectrum disorder and enterovirus-71 infection: A perplexing neurological constellations. Neuroimmunology Reports (2023).