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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.
Neuromyelitis optica (Devic's disease)/NMOSD: Optic nerves and spinal cord AQP4-IgG (NMO-IgG) Confirmed 0.5 - 4 per 100,000 [66] Restless legs syndrome: Central nervous system (thought to involve dopaminergic pathways) Unknown Uncertain 5-15% (more common in older adults) [67] Stiff-person syndrome: Central nervous system Anti-GAD, anti-amphiphysin
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]
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]
NMO may refer to: Neuromyelitis optica, also known as Devic's disease or Devic's syndrome; N-Methylmorpholine N-oxide, an organic compound; Nitronate monooxygenase, an enzyme; Normal Move Out, in reflection seismology; National Measurement Office, a government agency in the United Kingdom; New Motorola, a mobile antenna connector
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.
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.
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]