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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]
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 ...
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.
Standard multiple sclerosis, the most known and extended variant. Devic's disease and neuromyelitis optica (NMO) (sometimes previously called optic-spinal MS) Acute disseminated encephalomyelitis or ADEM, a closely related disorder in which a known virus or vaccine triggers autoimmunity against myelin.
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]
With response to NMO-IgG:: NMO-IgG is the immunoglobulin that attacks Aquaporin-4 in Devic's disease. Multiple sclerosis patients do not have it in blood, but it has been shown that 13% of tested patients reacted with the epitope AQPaa252-275. It is not known if these antibodies define distinct MS subsets, or are simply markers of astrocytic damage
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