Neuromyelitis optica spectrum disorder

Neuromyelitis optica spectrum disorders
Other namesNeuromyelitis optica (NMO), Devic's disease, Devic's syndrome
SpecialtyNeurology, ophthalmology
SymptomsVision loss, sensory loss, weakness, bladder dysfunction
Usual onsetMedian: age 40 for AQP4-IgG, age 31 for MOG-IgG[1]
TypesAQP4-IgG-positive, MOG-IgG-positive (recurrent, monophasic)[1]
Risk factorsFemale sex, genetic factors[1]
Diagnostic methodSymptoms, blood antibody titers, MRI
Differential diagnosisMultiple sclerosis, various autoimmune disorders
MedicationEculizumab, inebilizumab, satralizumab, rituximab, methylprednisolone, azathioprine, cellCept, mitoxantrone, methotrexate, intravenous immunoglobulin, cyclophosphamide
FrequencyUp to 1 in 10,000[1]

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).[1][2][3] Episodes of ON and myelitis can be simultaneous or successive. A relapsing disease course is common, especially in untreated patients.[1][4]

  • 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.[5][1][4]
  • Less common diseases with other manifestations are also part of the NMOSD spectrum.[6][4]
  1. ^ a b c d e f g Jarius, Sven; Paul, Friedemann; Weinshenker, Brian G.; Levy, Michael; Kim, Ho Jin; Wildemann, Brigitte (2020-10-22). "Neuromyelitis optica". Nature Reviews. Disease Primers. 6 (1): 85. doi:10.1038/s41572-020-0214-9. ISSN 2056-676X. PMID 33093467. S2CID 224825516.
  2. ^ Banerjee S, Butcher R. Rituximab for the Treatment of Neuromyelitis Optica Spectrum Disorder [Internet]. Ottawa (ON): Canadian Agency for Drugs and Technologies in Health; 2021 Feb. Available from: https://www.ncbi.nlm.nih.gov/books/NBK571350/
  3. ^ "Neuromyelitis optica – Symptoms and causes". Mayo Clinic. Retrieved 2020-08-01.
  4. ^ a b c Lana-Peixoto, Marco A.; Talim, Natália (2019-06-12). "Neuromyelitis Optica Spectrum Disorder and Anti-MOG Syndromes". Biomedicines. 7 (2): 42. doi:10.3390/biomedicines7020042. ISSN 2227-9059. PMC 6631227. PMID 31212763.
  5. ^ A subset of anti-AQP4-negative cases is associated with antibodies against myelin oligodendrocyte glycoprotein (anti-MOG).
  6. ^ Gaillard, Frank. "Neuromyelitis optica spectrum disorder | Radiology Reference Article | Radiopaedia.org". Radiopaedia.