Abstract: Neuromyelitis optica spectrum disorders (NMOSD) are rare autoimmune conditions characterized by recurrent attacks of optic neuritis, longitudinally extensive transverse myelitis, and other central nervous system manifestations. This article provides a comprehensive review of NMOSD, exploring its clinical manifestations, diagnostic approaches, therapeutic strategies, and emerging treatments. By enhancing our understanding of NMOSD, ophthalmologists can contribute to the multidisciplinary care of affected individuals.

Introduction: Neuromyelitis optica spectrum disorders represent a spectrum of autoimmune demyelinating disorders predominantly affecting the optic nerves and spinal cord. Historically mistaken for multiple sclerosis (MS), NMOSD is now recognized as a distinct entity with unique clinical, radiological, and serological features.

Clinical Features: The hallmark features of NMOSD include recurrent attacks of optic neuritis, leading to severe visual impairment or blindness, and longitudinally extensive transverse myelitis, resulting in varying degrees of motor and sensory deficits. Additionally, patients may experience other manifestations such as area postrema syndrome, brainstem syndrome, and symptomatic narcolepsy.

Diagnostic Approaches: Diagnosis of NMOSD relies on a combination of clinical presentation, neuroimaging findings, and serological testing for aquaporin-4 antibodies (AQP4-IgG) or, more recently, antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG). Advanced imaging techniques such as magnetic resonance imaging (MRI) and optical coherence tomography (OCT) play a crucial role in confirming the diagnosis and monitoring disease activity.

Management Strategies: Management of NMOSD aims to reduce the frequency and severity of relapses, preserve neurological function, and improve quality of life. Treatment typically involves immunosuppressive agents such as corticosteroids, azathioprine, mycophenolate mofetil, rituximab, and newer biologic agents like eculizumab. Acute attacks may require high-dose corticosteroids and plasma exchange.

Emerging Therapies: Recent advancements in NMOSD research have led to the development of novel targeted therapies, including monoclonal antibodies directed against interleukin-6 (IL-6) and IL-6 receptor, complement inhibitors, and B cell-targeted therapies. These treatments offer promising alternatives for patients refractory to conventional immunosuppressive agents.

Conclusion: Neuromyelitis optica spectrum disorders pose diagnostic and therapeutic challenges due to their heterogeneous presentation and potential for severe disability. Ophthalmologists, as key members of the healthcare team, play a vital role in recognizing ocular manifestations, facilitating early diagnosis, and implementing appropriate treatment strategies.

For further reading and reference:

• Guthy-Jackson Charitable Foundation – NMOSD Information: https://guthyjacksonfoundation.org/
• National Institute of Neurological Disorders and Stroke (NINDS) – Neuromyelitis Optica Information Page: https://www.ninds.nih.gov/
• Neurology – Emerging Therapies in Neuromyelitis Optica: https://n.neurology.org/