Neuromyelitis optica spectrum disorder
Neuromyelitis optica spectrum disorders (NMOSD) are a spectrum of autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis, ON) and the spinal cord (myelitis). Episodes of ON and myelitis can be simultaneous or successive. A relapsing disease course is common, especially in untreated patients.
- 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.
- Less common diseases with other manifestations are also part of the NMOSD spectrum.
| Neuromyelitis optica spectrum disorders | |
|---|---|
| Other names | Neuromyelitis optica (NMO), Devic's disease, Devic's syndrome |
| Specialty | Neurology, ophthalmology |
| Symptoms | Vision loss, sensory loss, weakness, bladder dysfunction |
| Usual onset | Median: age 40 for AQP4-IgG, age 31 for MOG-IgG |
| Types | AQP4-IgG-positive, MOG-IgG-positive (recurrent, monophasic) |
| Risk factors | Female sex, genetic factors |
| Diagnostic method | Symptoms, blood antibody titers, MRI |
| Differential diagnosis | Multiple sclerosis, various autoimmune disorders |
| Medication | Eculizumab, inebilizumab, satralizumab, rituximab, methylprednisolone, azathioprine, cellCept, mitoxantrone, methotrexate, intravenous immunoglobulin, cyclophosphamide |
| Frequency | Up to 1 in 10,000 |
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