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White Matter Diseases pp 67–94Cite as

Neuromyelitis Optica Spectrum Disorders

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Abstract

Neuromyelitis optica spectrum disorders (NMOSD) are a heterogeneous group of inflammatory, demyelinating and antibody-mediated diseases of the central nervous system (CNS). The anti-aquaporin-4-IgG (AQP4-IgG) seems to be the pathogenetic agent, being present in the large majority of patients, although seronegative forms can occur. Clinical manifestations include core features, such as myelitis, optic neuritis, and area postrema syndrome, possibly associated with collateral symptoms like pain, dystonic movements, and pruritus. Diagnosis is based on both clinical presentation and anti-AQP4-IgG testing. MRI features provide supportive elements in seronegative patients. The most effective therapy in acute attacks is plasma exchange, whereas multiple drugs are used for chronic maintenance (azathioprine, mycophenolate mofetil, rituximab and eculizumab).

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Authors and Affiliations

  1. Neurology Unit, IRCCS San Raffaele Scientific Institute, Milano, Italy

    Massimo Filippi

  2. Neuroimaging Research Unit, IRCCS San Raffaele Scientific Institute, Milano, Italy

    Massimo Filippi & Maria A. Rocca

  3. Neurophysiology Unit, IRCCS San Raffaele Scientific Institute, Milano, Italy

    Massimo Filippi

  4. Vita-Salute San Raffaele University, Milano, Italy

    Massimo Filippi

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Filippi, M., Rocca, M.A. (2020). Neuromyelitis Optica Spectrum Disorders. In: White Matter Diseases . Springer, Cham. https://doi.org/10.1007/978-3-030-38621-4_3

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