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In addition, several NMO variants have been discovered with antibodies other than those against AQP4, turning NMO into a heterogeneous disease. Six different patterns of damage have been reported in NMO, raising the possibility of six different types of auto-antibodies .
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.
Anti-MOG antibodies have been described in some patients with NMOSD [15] [16] who were negative for the aquaporin 4 (AQP-4) antibody. However, most NMOSD is an astrocytopathy, specifically an AQP4 antibody-associated disease, whereas MOG antibody-associated disease is an oligodendrocytopathy, suggesting that these are two separate pathologic entities. [2]
Neuromyelitis optica spectrum disorder is a rare autoimmune disorder in which immune system cells and autoantibodies attack and damage the optic nerves and spinal cord. [8] Neuromyelitis optica spectrum disorder can be associated with antibodies that bind to a protein called aquaporin-4 (AQP4).
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]
Antinuclear antibodies (ANA), B 12, folate, thyroid function tests, anti-aquaporin-4 antibodies (NMO-IgG), and glial fibrillary acidic protein (GFAP) can facilitate ruling out of other diseases. [3] Most patients are seronegative for NMO-IgG and GFAP, biomarkers for neuromyelitis optica. [3]
Blood tests are also often used to identify associated antibodies. "About 80% of patients with MG are positive" for the antibody behind the immune system dysfunction, says Weidmayer.
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.
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