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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.
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.
Inebilizumab, sold under the brand name Uplizna, is a medication for the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adults. [8] [9] [5] Inebilizumab is a humanized mAb that binds to and depletes CD19+ B cells including plasmablasts and plasma cells.
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.
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]
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 ...
Brain lesions associated with a clinically isolated syndrome may be indicative of several neurological diseases, like multiple sclerosis (MS) or neuromyelitis optica. In order for such a diagnosis , multiple sites in the central nervous system must present lesions, typically over multiple episodes, and for which no other diagnosis is likely.