• Florence Pache, Brigitte Wildemann, Friedemann Paul, and Sven Jarius.
• NeuroCure Clinical Research Center und Klinisches und Experimentelles Forschungszentrum für Multiple Sklerose, Klinik für Neurologie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland.
• Fortschr Neurol Psychiatr. 2017 Feb 1; 85 (2): 100-114.

AbstractNeuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system (CNS), that predominantly affects the spinal cord and optic nerves. The neuropathologic hallmarks comprise deposits of antibodies and complement as well as loss of astrocytes, secondary degeneration of oligodendrocytes and neurons, and necrotic lesions with infiltration of neutrophilic and eosinophilic granulocytes. Pathognomonic serum autoantibodies against aquaporin-4 (AQP4-IgG, also termed NMO-IgG) are detectable in around 80 % of NMO patients and help to distinguish this rare entity from multiple sclerosis. The target antigen of NMO-IgG, the water channel protein AQP4, is ubiquitously expressed within the CNS and, as a component of the blood-brain barrier, highly concentrated in the endfeet of astrocytes. New international consensus criteria for NMO spectrum disorders, published in 2015, allow earlier diagnosis. Besides the two index manifestations, optic neuritis and transverse myelitis, involvement of the brainstem and diencephalon is relatively common in NMO. Inflammatory lesions of the area postrema typically cause intractable nausea and vomiting and/or hiccups. NMO mostly follows a relapsing course, especially in AQP4-IgG-positive cases. The treatment of acute exacerbations comprises intravenous methylprednisolone pulses and/or plasma exchange, and prevention of attacks requires long-term therapy with immunosuppressants and/or B-cell-depleting monoclonal antibodies.© Georg Thieme Verlag KG Stuttgart · New York.

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