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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.
Optic neuritis typically affects young adults ranging 18–45 years of age, with a mean age of 30–35 years. There is a strong female predominance. The annual incidence is approximately 5/100,000, with a prevalence estimated to be 115/100,000 (0.12%). [29]
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]
the neuromyelitis optica (NMO) spectrum disorder; multiple sclerosis associated optic neuritis (MSON) unclassified optic neuritis (UCON) forms. [4] Medical examination of the optic nerve with an ophthalmoscope may reveal a swollen optic nerve, but the nerve may also appear normal.
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]
Encephalomyelitis is inflammation of the brain and spinal cord.Various types of encephalomyelitis include: Acute disseminated encephalomyelitis or postinfectious encephalomyelitis, a demyelinating disease of the brain and spinal cord, possibly triggered by viral infection.
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
Multiple sclerosis and Neuromyelitis optica are autoimmune diseases which both frequently present with optic neuritis, an inflammatory demyelinating neuropathy of the optic nerve. [3] Multiple sclerosis is a disease of unknown etiology which is characterized by neurological lesions "disseminated in time and space". [24]
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