Neuromyelitis Optica Brain Lesions Localized at Sites of High Aquaporin 4 Expression

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Abstract
Neuromyelitis optica (NMO) is a severe demyelinating disease recognized principally by its propensity to selectively affect optic nerves and the spinal cord, causing recurrent attacks of blindness and paralysis.1 The 1999 diagnostic criteria of Wingerchuk et al1 required fulfillment of 3 absolute criteria for a diagnosis of NMO: optic neuritis, acute myelitis, and no symptoms that implicated other central nervous system (CNS) structures. The criteria additionally required fulfillment of at least 1 of 3 major or 2 of 3 minor supportive criteria. Major supportive criteria were (1) brain magnetic resonance imaging (MRI) at onset of disease either normal or not fulfilling multiple sclerosis (MS) imaging criteria; (2) individual spinal cord MRI T2-weighted lesions that accompany myelitis and extend across 3 or more vertebral segments; and (3) cerebrospinal fluid leukocyte count that exceeds 50 white blood cells per microliter or 5 neutrophils per cubic millimeter, typically in the context of an acute attack. Minor supportive criteria were (1) bilateral optic neuritis, (2) severe residual visual loss, and (3) severe fixed postattack weakness.1