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Key Features

  • Previously known as Devic disease and once regarded as a variant of multiple sclerosis

  • Associated with a specific antibody marker (NMO-IgG) targeting the water channel aquaporin-4 in 80% of cases, and with antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) in a third of NMO-IgG seronegative patients

Clinical Findings

  • Optic neuritis and acute myelitis with MRI changes that extend over at least three segments of the spinal cord

  • Isolated myelitis or optic neuritis may also occur

Diagnosis

  • MRI of the brain typically does not show widespread white matter involvement, but such changes do not exclude the diagnosis

Treatment

  • Long-term immunosuppression

  • Rituximab is first-line therapy

    • Two 1-g intravenous infusions spaced by 2 weeks, or four weekly infusions of 375 mg/m2

    • Re-dosing may occur every 6 months or when CD19/20-positive or CD27-positive lymphocytes become detectable

  • Alteratives include

    • Azathioprine, 2.5–3 mg/kg orally

    • Mycophenolate mofetil, 500–1500 mg orally twice daily, titrated until the absolute lymphocyte count falls below 1500/mcL)

  • Corticosteroids used for acute relapses

  • Plasma exchange given when severe relapses do not respond to corticosteroids

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