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Clinical spectrum of neuromyelitis optica 2013

The spectrum of presentation of neuromyelitis optica (NMO) is now recognized as being more diverse than defined by current diagnostic criteria, and includes limited forms of optic neuritis and transverse myelitis, as well as other brain syndromes. Discovery of a specific biomarker, autoantibodies targeting aquaporin‐4, facilitated the recognition of this broader spectrum of disease. Brain magnetic resonance imaging lesions in NMO might be relatively specific or entirely non‐specific. The most specific lesions are lesions of the area postrema or hypothalamus, longitudinally extensive corpus callosum lesions, and long corticospinal tract lesions in the corona radiata and brainstem. Although the full spectrum of aquaporin‐4‐associated disease based on population‐based serological studies remains undefined, studies from service testing laboratories show that the vast majority of patients have had optic neuritis and myelitis or one of the now well‐accepted signature syndromes of NMO, such as intractable vomiting or hiccough, although ascertainment bias undoubtedly influences this conclusion. New diagnostic criteria based on deliberations of an international panel of experts are anticipated shortly. Almost certainly, new criteria will allow for diagnosis of NMO based on a single episode of a “signature” syndrome of NMO. Nevertheless, in the face of a syndrome that is less characteristic and less well associated with NMO, especially if associated with other disease entities, caution is necessary.