Neuromyelitis optica

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By Tiffani Stroup DO and Adil Javed MD PhD

Patients suspected of having neuromyelitis optica and NMOSD are treated immediately with immunosuppressive therapy. They continue to receive immunosuppressive treatments prophylactically for many years, perhaps indefinitely. No consensus on duration of therapy exists. For other autoimmune diseases such as systemic lupus erythematosus and myasthenia gravis, long-term immunosuppression with mycophenolate mofetil, azathioprine, or rituximab has been tolerated for years without safety concerns. Because neuromyelitis optica attacks are unpredictable, prolonged prophylactic therapy with immunosuppressive medications is often used for preventing disability. A syndrome of intractable hiccups and nausea can precede attacks of neuromyelitis optica and may rarely be a heralding sign, allowing preemptive treatment prior to a recurrence (Takahashi et al 2008). It is unknown whether a low Vitamin D level, possibly linked to multiple sclerosis, also plays a role in attacks of neuromyelitis optica.

In This Article

Historical note and nomenclature
Clinical manifestations
Clinical vignette
Pathogenesis and pathophysiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
References cited