Neuromyelitis optica spectrum disorder secondary prevention

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Julinka Auta Fernandes

Overview

Secondary prevention of neuromyelitis optica spectrum disorder (NMOSD) aims to prevent further inflammatory attacks and limit cumulative attack-related disability. Long-term immunotherapy is the cornerstone of this approach.

Secondary Prevention

Secondary prevention strategies following neuromyelitis optica spectrum disorder include sustained immunotherapy, regular assessment of disease activity, and treatment safety monitoring.

Long-Term Immunotherapy

Neuromyelitis optica spectrum disorder patients with immunoglobulin G (IgG) antibodies against aquaporin-4 (AQP4), abbreviated AQP4-IgG, should be offered long-term immunotherapy after the first attack, according to the Neuromyelitis Optica Study Group (NEMOS) recommendations.

• Monoclonal antibodies: Initial options for AQP4-IgG-positive NMOSD include eculizumab, ravulizumab, inebilizumab, rituximab, or satralizumab, when available and accessible.
• Conventional immunosuppressants: Azathioprine or mycophenolate mofetil remain alternatives when biologic therapies are inaccessible.
• Other selected cases: Tocilizumab is another option considered in specialist practice, including refractory disease.

Treatment selection should consider efficacy, safety, disease severity, comorbidities, family planning, patient preferences, and access. Patients who remain relapse-free and tolerate their existing treatment do not automatically require a change to a newer agent.

For dosing, administration, and drug-specific precautions, see medical therapy.

AQP4-IgG-Negative Disease

Neuromyelitis optica spectrum disorder without detectable AQP4-IgG requires expert diagnostic reassessment, including exclusion of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and other mimics.

For double-seronegative NMOSD, with negative testing for both AQP4-IgG and myelin oligodendrocyte glycoprotein antibodies, the 2026 Malaysian consensus supports considering maintenance treatment in relapsing or severe disease with a high risk of further attacks or disability. Options include rituximab, azathioprine, or mycophenolate mofetil. Evidence is limited, and treatment should be individualized.

Treatment Continuity

AQP4-IgG-positive neuromyelitis optica spectrum disorder generally requires long-term maintenance treatment. The 2026 Canadian consensus recommends continued therapy because discontinuation carries a considerable risk of disabling relapses, including after prolonged disease stability.

A tailored oral corticosteroid taper may be used after an attack until maintenance treatment is optimized. Treatment reduction or withdrawal requires specialist review.

Monitoring and Breakthrough Attacks

Neuromyelitis optica spectrum disorder requires regular neurological review and drug-specific monitoring to assess treatment effectiveness and adverse effects. New or worsening neurological symptoms need prompt assessment to distinguish an inflammatory attack from infection-related worsening or other causes.

A confirmed relapse despite adequate treatment exposure warrants reassessment of the preventive regimen. Switching to a therapy with a different mechanism of action may be appropriate after treatment failure.

Pregnancy and the Postpartum Period

Neuromyelitis optica spectrum disorder relapse-prevention planning should begin before pregnancy because relapse risk increases during the postpartum period. Pregnancy should be planned during stable disease, with specialist selection of an appropriate immunotherapy strategy. Treatment should not be stopped solely because pregnancy is desired. Teratogenic agents, including mycophenolate mofetil, require a planned change before conception. If treatment is interrupted during pregnancy, early postpartum resumption should be arranged.

Vaccination and Treatment Safety

Neuromyelitis optica spectrum disorder patients receiving immunotherapy should have their vaccination status reviewed and updated, with timing coordinated around the selected treatment. The 2026 Canadian consensus specifically supports updating missing non-live vaccinations after an attack.

Some therapies used for multiple sclerosis, including interferon beta, fingolimod, and natalizumab, may worsen NMOSD and should not be used for NMOSD relapse prevention.