Neuromyelitis optica (NMO) is currently recognized as a broad spectrum of autoimmune disorders of the Central Nervous System (CNS), causing demyelinating and inflammatoryinjuries, primarily in the spinal cord and optic nerves, but also in other regions such as brainstem,diencephalon or specific brain areas. These disorders are grouped under the unifying term"NMO spectrum disorders" (NMOSD). For many years this pathological entity was thoughtlike a variant of the Multiple Sclerosis (MS). However, current evidence shows that there aredistinctive features of clinical presentation, pathophysiology, laboratory, neuroimaging andtherapy response that distinguish NMOSD from the latter. Most patients with NMOSD areseropositive for autoantibodies (AQP4-IgG) againstAQP4, the major water channel ofastrocytes.New advances in research have allowed recognize that AQP4-IgG is pathogenic in NMOSD,probably by a mechanism involving complement dependent cellular cytotoxicity. Due to theseverity of attacks in NMOSD and the high risk for neurological disability, treatment should beinitiated as soon as the diagnosis is confirmed. Acute attacks ofoptic neuritis or myelitis are treatedwith high-dose intravenous corticosteroid and plasmapheresis. Maintenance therapy to avoidfurther relapses is based on low-dose oral corticosteroid and non-specific immunosuppressantdrugs; nevertheless, to date there are no controlled randomized trials to confirm the safety andefficacy for the drugs currently used.
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