Abstract
Water channels are a recognized “novel” target for central nervous system (CNS) inflammatory autoimmune demyelinating diseases and represent an evolving spectrum of disorders termed neuromyelitis optica (NMO) spectrum disorders (SDs) that fall under the umbrella term “autoimmune astrocytopathies.” NMO is characterized by recurrent episodes of optic neuritis and transverse myelitis and may result in blindness or paraplegia. It is frequently misdiagnosed as multiple sclerosis (MS). NMO and its partial or inaugural forms (constituting a spectrum of NMO-related disorders termed NMOSDs) represent the first MS-like disease for which a specific antigen has been identified – the astrocytic water channel aquaporin-4 (AQP4). An autoantibody specific for AQP4 (AQP4-IgG, originally termed NMO-IgG) is a clinically validated serum biomarker that distinguishes relapsing NMO from MS. MS lacks a distinguishing biomarker and its therapies may exacerbate NMO.
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