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Neuromyelitis optica (NMO) is a particular disease within the NMOSD spectrum. It is characterised by optic neuritis and longitudinally extensive myelitis. In more than 80% of NMO cases, the cause is immunoglobulin G autoantibodies to aquaporin 4 ( anti-AQP4 ), the most abundant water channel protein in the central nervous system.
Most patients are seronegative for NMO-IgG and GFAP, biomarkers for neuromyelitis optica. [3] ANA, indicative of autoimmune optic neuropathy, is also generally negative. [3] CSF can also be evaluated for oligoclonal bands typical of multiple sclerosis, which will not be present in CRION. [1]
Anti-AQP4 diseases, are a group of diseases characterized by auto-antibodies against aquaporin 4.. After the discovery of anti-AQP4 autoantibody in neuromyelitis optica, it was found that it was also present in some patients with other clinically defined diseases, including multiple sclerosis variants like optic-spinal MS.
MS patients show axonal loss in the retina and optic nerve, which can be measured by Optical coherence tomography [69] or by Scanning laser polarimetry. [70] This measure can be used to predict disease activity [71] and to establish a differential diagnosis from Neuromyelitis optica [72]
This last requirement makes MS an ill-defined entity, whose borders change every time that a new disease is set apart. Some cases previously considered MS are now considered distinct conditions, like Neuromyelitis optica or antiMOG associated encephalomyelitis. Because of the requirement of distributed lesions, a single lesion (RIS) is not ...
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After the discovery of anti-AQP4 auto-antibodies there are two kinds of Optic-Spinal MS (OSMS): Anti-AQP4 positive OSMS or Neuromyelitis optica; Anti-AQP4 negative OSMS, currently idiopathic, considered inside the Inflammatory demyelinating diseases of the central nervous system spectrum.
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