Neuromyelitis Optica and Non–Organ-Specific Autoimmunity

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Abstract
Neuromyelitis optica (NMO) is characterized by optic neuritis and longitudinally extensive transverse myelitis (LETM, defined by a lesion extending 3 vertebral segments or longer on T2-weighted magnetic resonance images of the spine during an acute episode of myelitis). The course of NMO is relapsing in most cases.1 The serum autoantibody NMO-IgG is a sensitive and specific marker for NMO.2 The association between NMO and NMO-IgG has recently been confirmed by an independent European group.3 Its antigen is aquaporin-4 (AQP4), the predominant water channel protein in the central nervous system.4 The autoantibody NMO-IgG is detectable also in patients with recurrent optic neuritis without myelitis and in a high proportion of patients with a single episode or recurrence of LETM without optic neuritis.2 Detection of NMO-IgG in patients with a first LETM event predicts that LETM will relapse or that optic neuritis will develop in more than 50% of cases within 12 months.5 Brain lesions may also occur in patients with NMO, with or without symptoms, and in some cases may be hard to distinguish from those occurring in multiple sclerosis, although some hypothalamic and brainstem lesions are relatively specific for NMO.6

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