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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 , the most abundant water channel protein in the central nervous system. [5] [1] [4]
NMO-IgG seronegative; Contrast enhancement on imaging of acutely inflamed optic nerves; Response to immunosuppressive treatment and relapse on withdrawal or dose reduction. CRION has been included as a subtype in a 2022 international consensus classification of optic neuritis. [2]
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.
Tumefactive demyelination: Tumefactive lesions in NMO are not usual, but they have been reported to appear in several cases mistakenly treated with interferon beta. [9] Devic's disease is currently considered a syndrome more than a disease, presenting an overlapping with the wide spectrum of multiple sclerosis in the form of Optic-Spinal MS. [10]
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]
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.
She has also worked to determine the best treatment options for pediatric NMO-SD. 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 ...