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Neuromyelitis optica(NMO)

MedGen UID:
45063
•Concept ID:
C0027873
•
Disease or Syndrome
Synonyms: Devic disease; Devic syndrome; Devic's neuromyelitis optica; NMO
SNOMED CT: Devic syndrome (25044007); Optic neuromyelitis (25044007); SMON - subacute myelo-optico-neuropathy (25044007); Neuromyelitis optica (25044007); Devic's disease (25044007)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Non-Mendelian inheritance (Orphanet)
 
Monarch Initiative: MONDO:0019100
Orphanet: ORPHA71211

Definition

A rare inflammatory disease of the central nervous system characterized mainly by attacks of uni- or bilateral optic neuritis (ON) and acute myelitis. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeuromyelitis optica
Follow this link to review classifications for Neuromyelitis optica in Orphanet.

Professional guidelines

PubMed

Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria.
Banwell B, Bennett JL, Marignier R, Kim HJ, Brilot F, Flanagan EP, Ramanathan S, Waters P, Tenembaum S, Graves JS, Chitnis T, Brandt AU, Hemingway C, Neuteboom R, Pandit L, Reindl M, Saiz A, Sato DK, Rostasy K, Paul F, Pittock SJ, Fujihara K, Palace J
Lancet Neurol 2023 Mar;22(3):268-282. Epub 2023 Jan 24 doi: 10.1016/S1474-4422(22)00431-8. PMID: 36706773
International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.
Wingerchuk DM, Banwell B, Bennett JL, Cabre P, Carroll W, Chitnis T, de Seze J, Fujihara K, Greenberg B, Jacob A, Jarius S, Lana-Peixoto M, Levy M, Simon JH, Tenembaum S, Traboulsee AL, Waters P, Wellik KE, Weinshenker BG; International Panel for NMO Diagnosis
Neurology 2015 Jul 14;85(2):177-89. Epub 2015 Jun 19 doi: 10.1212/WNL.0000000000001729. PMID: 26092914Free PMC Article
Classification, diagnosis, and differential diagnosis of multiple sclerosis.
Katz Sand I
Curr Opin Neurol 2015 Jun;28(3):193-205. doi: 10.1097/WCO.0000000000000206. PMID: 25887774

Recent clinical studies

Etiology

Update on the diagnosis and treatment of neuromyelits optica spectrum disorders (NMOSD) - revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part I: Diagnosis and differential diagnosis.
Jarius S, Aktas O, Ayzenberg I, Bellmann-Strobl J, Berthele A, Giglhuber K, Häußler V, Havla J, Hellwig K, Hümmert MW, Kleiter I, Klotz L, Krumbholz M, Kümpfel T, Paul F, Ringelstein M, Ruprecht K, Senel M, Stellmann JP, Bergh FT, Tumani H, Wildemann B, Trebst C; Neuromyelitis Optica Study Group (NEMOS)
J Neurol 2023 Jul;270(7):3341-3368. Epub 2023 Apr 6 doi: 10.1007/s00415-023-11634-0. PMID: 37022481Free PMC Article
Neuromyelitis Optica Spectrum Disorders.
Costello F
Continuum (Minneap Minn) 2022 Aug 1;28(4):1131-1170. doi: 10.1212/CON.0000000000001168. PMID: 35938660
Pregnancy Management in Multiple Sclerosis and Other Demyelinating Diseases.
Bove RM, Houtchens MK
Continuum (Minneap Minn) 2022 Feb 1;28(1):12-33. doi: 10.1212/CON.0000000000001108. PMID: 35133309
Treatment of Neuromyelitis Optica Spectrum Disorders.
Chan KH, Lee CY
Int J Mol Sci 2021 Aug 11;22(16) doi: 10.3390/ijms22168638. PMID: 34445343Free PMC Article
Interleukin-6 in neuromyelitis optica spectrum disorder pathophysiology.
Fujihara K, Bennett JL, de Seze J, Haramura M, Kleiter I, Weinshenker BG, Kang D, Mughal T, Yamamura T
Neurol Neuroimmunol Neuroinflamm 2020 Sep 3;7(5) Epub 2020 Aug 20 doi: 10.1212/NXI.0000000000000841. PMID: 32820020Free PMC Article

Diagnosis

Neuromyelitis Optica Spectrum Disorder.
Wingerchuk DM, Lucchinetti CF
N Engl J Med 2022 Aug 18;387(7):631-639. doi: 10.1056/NEJMra1904655. PMID: 36070711
Neuromyelitis Optica Spectrum Disorders.
Costello F
Continuum (Minneap Minn) 2022 Aug 1;28(4):1131-1170. doi: 10.1212/CON.0000000000001168. PMID: 35938660
Neuromyelitis optica.
Jarius S, Paul F, Weinshenker BG, Levy M, Kim HJ, Wildemann B
Nat Rev Dis Primers 2020 Oct 22;6(1):85. doi: 10.1038/s41572-020-0214-9. PMID: 33093467
Neuromyelitis optica spectrum disorders.
Huda S, Whittam D, Bhojak M, Chamberlain J, Noonan C, Jacob A
Clin Med (Lond) 2019 Mar;19(2):169-176. doi: 10.7861/clinmedicine.19-2-169. PMID: 30872305Free PMC Article
Neuromyelitis Optica Spectrum Disorders: Spectrum of MR Imaging Findings and Their Differential Diagnosis.
Dutra BG, da Rocha AJ, Nunes RH, Maia ACM Júnior
Radiographics 2018 Jan-Feb;38(1):169-193. doi: 10.1148/rg.2018170141. PMID: 29320331

Therapy

Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders (NMOSD) - revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part II: Attack therapy and long-term management.
Kümpfel T, Giglhuber K, Aktas O, Ayzenberg I, Bellmann-Strobl J, Häußler V, Havla J, Hellwig K, Hümmert MW, Jarius S, Kleiter I, Klotz L, Krumbholz M, Paul F, Ringelstein M, Ruprecht K, Senel M, Stellmann JP, Bergh FT, Trebst C, Tumani H, Warnke C, Wildemann B, Berthele A; Neuromyelitis Optica Study Group (NEMOS)
J Neurol 2024 Jan;271(1):141-176. Epub 2023 Sep 7 doi: 10.1007/s00415-023-11910-z. PMID: 37676297Free PMC Article
Adverse Events in NMOSD Therapy.
Giglhuber K, Berthele A
Int J Mol Sci 2022 Apr 9;23(8) doi: 10.3390/ijms23084154. PMID: 35456972Free PMC Article
Neuromyelitis optica spectrum disorders: from pathophysiology to therapeutic strategies.
Carnero Contentti E, Correale J
J Neuroinflammation 2021 Sep 16;18(1):208. doi: 10.1186/s12974-021-02249-1. PMID: 34530847Free PMC Article
Neuromyelitis optica.
Jarius S, Paul F, Weinshenker BG, Levy M, Kim HJ, Wildemann B
Nat Rev Dis Primers 2020 Oct 22;6(1):85. doi: 10.1038/s41572-020-0214-9. PMID: 33093467
Neuromyelitis optica spectrum disorders.
Huda S, Whittam D, Bhojak M, Chamberlain J, Noonan C, Jacob A
Clin Med (Lond) 2019 Mar;19(2):169-176. doi: 10.7861/clinmedicine.19-2-169. PMID: 30872305Free PMC Article

Prognosis

Worldwide Incidence and Prevalence of Neuromyelitis Optica: A Systematic Review.
Papp V, Magyari M, Aktas O, Berger T, Broadley SA, Cabre P, Jacob A, Kira JI, Leite MI, Marignier R, Miyamoto K, Palace J, Saiz A, Sepulveda M, Sveinsson O, Illes Z
Neurology 2021 Jan 12;96(2):59-77. Epub 2020 Dec 11 doi: 10.1212/WNL.0000000000011153. PMID: 33310876Free PMC Article
Neuromyelitis optica.
Jarius S, Paul F, Weinshenker BG, Levy M, Kim HJ, Wildemann B
Nat Rev Dis Primers 2020 Oct 22;6(1):85. doi: 10.1038/s41572-020-0214-9. PMID: 33093467
Neuromyelitis optica spectrum disorders.
Huda S, Whittam D, Bhojak M, Chamberlain J, Noonan C, Jacob A
Clin Med (Lond) 2019 Mar;19(2):169-176. doi: 10.7861/clinmedicine.19-2-169. PMID: 30872305Free PMC Article
Neurological update: MOG antibody disease.
Wynford-Thomas R, Jacob A, Tomassini V
J Neurol 2019 May;266(5):1280-1286. Epub 2018 Dec 19 doi: 10.1007/s00415-018-9122-2. PMID: 30569382Free PMC Article
The spectrum of neuromyelitis optica.
Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG
Lancet Neurol 2007 Sep;6(9):805-15. doi: 10.1016/S1474-4422(07)70216-8. PMID: 17706564

Clinical prediction guides

Inebilizumab: A Review in Neuromyelitis Optica Spectrum Disorder.
Nie T, Blair HA
CNS Drugs 2022 Oct;36(10):1133-1141. Epub 2022 Sep 7 doi: 10.1007/s40263-022-00949-7. PMID: 36070074Free PMC Article
Treatment of Neuromyelitis Optica Spectrum Disorders.
Chan KH, Lee CY
Int J Mol Sci 2021 Aug 11;22(16) doi: 10.3390/ijms22168638. PMID: 34445343Free PMC Article
Trial of Satralizumab in Neuromyelitis Optica Spectrum Disorder.
Yamamura T, Kleiter I, Fujihara K, Palace J, Greenberg B, Zakrzewska-Pniewska B, Patti F, Tsai CP, Saiz A, Yamazaki H, Kawata Y, Wright P, De Seze J
N Engl J Med 2019 Nov 28;381(22):2114-2124. doi: 10.1056/NEJMoa1901747. PMID: 31774956
Neurological update: MOG antibody disease.
Wynford-Thomas R, Jacob A, Tomassini V
J Neurol 2019 May;266(5):1280-1286. Epub 2018 Dec 19 doi: 10.1007/s00415-018-9122-2. PMID: 30569382Free PMC Article
The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review.
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923

Recent systematic reviews

Systematic Review of Safety and Efficacy of Second- and Third-Generation CD20-Targeting Biologics in Treating Immune-Mediated Disorders.
Kaegi C, Wuest B, Crowley C, Boyman O
Front Immunol 2021;12:788830. Epub 2022 Feb 2 doi: 10.3389/fimmu.2021.788830. PMID: 35185862Free PMC Article
Therapeutic Targeting of the Complement System: From Rare Diseases to Pandemics.
Garred P, Tenner AJ, Mollnes TE
Pharmacol Rev 2021 Apr;73(2):792-827. doi: 10.1124/pharmrev.120.000072. PMID: 33687995Free PMC Article
Worldwide Incidence and Prevalence of Neuromyelitis Optica: A Systematic Review.
Papp V, Magyari M, Aktas O, Berger T, Broadley SA, Cabre P, Jacob A, Kira JI, Leite MI, Marignier R, Miyamoto K, Palace J, Saiz A, Sepulveda M, Sveinsson O, Illes Z
Neurology 2021 Jan 12;96(2):59-77. Epub 2020 Dec 11 doi: 10.1212/WNL.0000000000011153. PMID: 33310876Free PMC Article
Systematic Review of Safety and Efficacy of Rituximab in Treating Immune-Mediated Disorders.
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article
Neuromyelitis optica.
Cree BA, Goodin DS, Hauser SL
Semin Neurol 2002 Jun;22(2):105-22. doi: 10.1055/s-2002-36534. PMID: 12524556

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