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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.
MOG antibody disease (MOGAD) was first described in the early 2000s as a subset of cases of neuromyelitis optica with antibodies to myelin oligocyte glycoprotein (MOG). [24] Banwell and colleagues worked to characterize pediatric MOGAD to determine methods of diagnosis, [ 25 ] treatment options, [ 26 ] and prognosis.
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]
Satralizumab is indicated for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adults with a particular antibody – people who are anti-aquaporin-4 or AQP4 antibody-positive. [6] [14] NMOSD is a rare autoimmune disease of the central nervous system that mainly affects the optic nerves and spinal cord. [6]
Some cases of aquaporin-4-seronegative neuromyelitis optica: NMO derived from an antiMOG associated encephalomyelitis, [22] Some cases of acute disseminated encephalomyelitis, specially the recurrent ones (MDEM) [25] Some cases of McDonalds-positive multiple sclerosis [24] [26] [23] [27] isolated optic neuritis or transverse myelitis [24]
Multiple sclerosis, Neuromyelitis optica, MOG antibody disease: B Jean Alexandre Barré: 1880 - 1967 France Guillain–Barré syndrome, Barré–Liéou syndrome, Barré test: B Henry Charlton Bastian: 1837 - 1915 United Kingdom Bastian-Bruns sign: B Diana Beck: 1900 - 1956 United Kingdom B Peter Emil Becker: 1908 - 2000 Germany Becker's ...
Eculizumab, sold under the brand name Soliris among others, is a recombinant humanized monoclonal antibody used to treat paroxysmal nocturnal hemoglobinuria, atypical hemolytic uremic syndrome, generalized myasthenia gravis, and neuromyelitis optica.
In neuromyelitis optica higher AQP4 autoantibody levels are associated with the occurrence of optic neuritis. [16] Less common causes are: papilledema, brain tumor or abscess in the occipital region, cerebral trauma or hemorrhage, meningitis, arachnoidal adhesions, sinus thrombosis, liver dysfunction, or late stage kidney disease.
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