Diagnostic criteria and treatment of neuromyelitis optica spectrum disorders (NMOSD)
https://doi.org/10.14412/2074-2711-2023-1S-71-75
Abstract
Neuromyelitis optic spectrum disorders (NMOSD) are rare, severely disabling diseases of the central nervous system. They are characterized by periodic, unpredictable exacerbations that lead to an accumulation of irreversible neurologic damage. Despite existing possibilities for diagnosing NMOSD, it can be difficult to make the correct diagnosis in clinical practice. Current treatment options for NMOSD include drugs that affect the pathogenesis of the disease, including complement C5 inhibitors, with which experience is still limited, but published data from clinical trials demonstrate high efficacy of these drugs with an acceptable safety profile. The article reviews the main current approaches to the diagnosis and treatment of NMOSD.
About the Authors
T. O. SimanivRussian Federation
27, Lomonosovsky Prosp., Build. 1, Moscow 119192
K. Z. Bakhtiyarova
Russian Federation
47, Zaki Validi St., Ufa 450008
A. N. Belova
Russian Federation
10/1, Minina and Pozharskogo Sq., Nizhny Novgorod 603005
Z. A. Goncharova
Russian Federation
119, Suvorova St., Rostov-on-Don 344022
S. A. Sivertseva
Russian Federation
112, Karla Marksa St., Kirov 610998
8/1, Yuriya Semovskih St., Tyumen 625000
N. N. Spirin
Russian Federation
5, Revolutsionnaya St., Yaroslavl 150000
N. A. Totolyan
Russian Federation
6-8, Lva Tolstogo St., St. Petersburg 197022
T. N. Trushnikova
Russian Federation
26, Petropavlovskaya St., Perm 614990
85, Pushkina St., Perm 614990
E. L. Turova
Russian Federation
185, Volgogradskaya St., Yekaterinburg 620102
T. I. Khaibullin
Russian Federation
36, Butlerova St., Kazan 420012
13, Vatutina St., Kazan 420021
M. A. Sherman
Russian Federation
112, Karla Marksa St., Kirov 610998
A. N. Boyko
Russian Federation
Alexey Nikolaevich Boyko
1, Ostrovityanova St., Moscow 117997
1, Ostrovityanova St., Build. 10, Moscow 117997
References
1. Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of neuromyelitis optica. Lancet Neurol. 2007 Sep;6(9):805-15. doi: 10.1016/S1474-4422(07)70216-8
2. Borisow N, Mori M, Kuwabara S, et al. Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis. Front Neurol. 2018 Oct 23;9:888. doi: 10.3389/fneur.2018.00888
3. Hor JY, Asgari N, Nakashima I, et al. Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide. Front Neurol. 2020 Jun 26;11:501. doi: 10.3389/fneur.2020.00501
4. Sherman MA, Boyko AN. Epidemiology of diseases of the spectrum of opticoneuromyelitis. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova. 2021;21(7-2):5-12. doi: 10.17116/jnevro20211210725 (In Russ.).
5. Simaniv TO, Vasiliev AV, Askarova LSh, Zakharova MN. Neuromyelitisoptica and neuromyelitisoptica spectrum disorders. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova. 2019;119(10-2):35-48. doi: 10/17116/jnevro20191191035 (In Russ.).
6. Starodubov VI, Zelenova OV, Abramov SI, et al. The first observational epidemiological study to determine the incidence and prevalence of diseases of the spectrum of opticoneuromyelitis (opticoneuromyelitis, Devik's disease) on the territory of the Russian Federation. Sovremennye problemy zdravookhraneniya i meditsinskoi statistiki = Current Problems of Health Care and Medical Statistics. 2021;(1):130-41. doi: 10.24411/2312-2935-2021-00007 (In Russ.).
7. Pandit L, Asgari N, Apiwattanakul M, et al.; GJCF International Clinical Consortium & Biorepository for Neuromyelitis Optica. Demographic and clinical features of neuromyelitis optica: A review. Mult Scler. 2015 Jun;21(7):845-53. doi: 10.1177/1352458515572406. Epub 2015 Apr 28.
8. Weinshenker BG, Wingerchuk DM. Neuromyelitis optica: clinical syndrome and the NMO-IgG autoantibody marker. Curr Top Microbiol Immunol. 2008;318:343-56. doi: 10.1007/978-3-540-73677-6_14
9. Lennon VA, Wingerchuk DM, Kryzer TJ, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004 Dec 11-17;364(9451):2106-12. doi: 10.1016/S0140-6736(04)17551-X
10. Jarius S, Ruprecht K, Wildemann B, et al. Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: A multicentre study of 175 patients. J Neuroinflammation. 2012 Jan 19;9:14. doi: 10.1186/1742-2094-9-14
11. Wingerchuk DM, Banwell B, Bennett J, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177-89. doi: 10.1212/WNL.0000000000001729
12. Kim W, Lee JE, Li XF, et al. Quantitative measurement of anti-aquaporin-4 antibodies by enzyme-linked immunosorbent assay using purified recombinant human aquaporin-4. Mult Scler. 2011;18(5):578-86. doi: 10.1177/1352458511424590
13. Whittam D, Wilson M, Hamid S, et al. What's new in neuromyelitis optica? A short review for clinical neurologist. J Neurol. 2017;264(11):2330-44. doi: 10.1007/s00415-017-8445-8
14. Waters P, Pittock SJ, Bennett JL, et al. Evaluation of aquaporine-4 antibody assays. Clin Exp Neuroimmunol. 2014;5(3):290-303. doi: 10.1111/cen3.12107
15. Banwell B, Bennett JL, Marignier R, et al. Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria. Lancet Neurol. 2023 Mar;22(3):268-82. doi: 10.1016/S1474-4422(22)00431-8
16. Tajfirouz DA, Bhatti MT, Chen JJ. Clinical Characteristics and Treatment of MOG-IgGAssociated Optic Neuritis. Curr Neurol Neurosci Rep. 2019;19:100. doi: 10.1007/s11910-019-1014-z
17. Kunchok A, Chen JJ, McKeon A, et al. Coexistence of Myelin Oligodendrocyte Glycoprotein and Aquaporin-4 Antibodies in Adult and Pediatric Patients. JAMA Neurol. 2020;77:257-9. doi: 10.1001/jamaneurol.2019.3656
18. Liu C, Shi M, Zhu M, et al. Comparisons of clinical phenotype, radiological and laboratory features, and therapy of neuromyelitis optica spectrum disorder by regions: update and challenges. Autoimmun Rev. 2022 Jan;21(1):102921. doi: 10.1016/j.autrev.2021.102921. Epub 2021 Aug 10.
19. Waters PJ, McKeon A, Leite MI, et al. Serologic diagnosis of NMO: a multicenter comparison of aquaporin-4-IgG assays. Neurology. 2012 Feb 28;78(9):665-71; discussion 669. doi: 10.1212/WNL.0b013e318248dec1. Epub 2012 Feb 1.
20. Krasnov VS, Totolyan NA, Nazarov VD, et al. Actual issues of serum aquaporin-4 autoantibodies evaluation in the diagnostics of neuromyelitis optica spectrum disorders. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova. 2020;120(7-2):24-31. doi: 10.17116/jnevro202012007224 (In Russ.).
21. Krotenkova MV, Bryukhov VV, Morozova SN, Krotenkova IA. Magnitno-rezonansnaya tomografiya v diagnostike i differentsial'noy diagnostike rasseyannogo skleroza. Rukovodstvo dlya vrachey Magnetic resonance imaging in the diagnosis and differential diagnosis of multiple sclerosis. Guide for doctors. Moscow: GEOTAR-Media; 2019. 160 p. (In Russ.).
22. Simaniv TO, Krasnov VS, Kasatkin DS. Optikonevromielit v fokuse. Prakticheskoe rukovodstvo v skhemakh i tablitsakh Opticoneuromyelitis in focus. Practical guidance in diagrams and tables. Moscow: Prakticheskaya meditsina; 2023. 176 p. (In Russ.).
23. Belov SE, Boyko AN. A symptom of the central vein in various diseases and protocols of MRI examination. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova. 2022;122(7-2):19-26. doi: 10.17116/jnevro202212207219 (In Russ.).
24. Kawachi I, Lassmann H. Neurodegeneration in multiple sclerosis and neuromyelitis optica. J Neurol Neurosurg Psychiatry. 2017 Feb;88(2):137-45. doi: 10.1136/jnnp-2016-313300. Epub 2016 Sep 26
25. Wingerchuk DM, Weinshenker BG. Neuromyelitis optica: clinical predictors of a relapsing course and survival. Neurology. 2003 Mar 11;60(5):848-53. doi: 10.1212/01.wnl.0000049912.02954.2c
26. Ma X, Kermode AG, Hu X, Qiu W. Risk of relapse in patients with neuromyelitis optica spectrum disorder: Recognition and preventive strategy. Mult Scler Relat Disord. 2020 Nov;46:102522. doi: 10.1016/j.msard.2020.102522. Epub 2020 Sep 19.
27. Palace J, Lin DY, Zeng D, et al. Outcome prediction models in AQP4-IgG positive neuromyelitis optica spectrum disorders. Brain. 2019 May 1;142(5):1310-23. doi: 10.1093/brain/awz054
28. Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology. 1983 Nov;33(11):1444-52. doi: 10.1212/wnl.33.11.1444
29. Ma X, Kermode AG, Hu X, Qiu W. NMOSD acute attack: Understanding, treatment and innovative treatment prospect. J Neuroimmunol. 2020 Nov 15;348:577387. doi: 10.1016/j.jneuroim.2020.577387. Epub 2020 Sep 8.
30. Schwartz CE, Stark RB, Stucky BD. Response-shift effects in neuromyelitis optica spectrum disorder: a secondary analysis of clinical trial data. Qual Life Res. 2021 May;30(5):1267-82. doi: 10.1007/s11136-020-02707-y. Epub 2020 Dec 2.
31. Pittock SJ, Fujihara K, Palace J, et al; PREVENT Study Group. Eculizumab monotherapy for NMOSD: Data from PREVENT and its openlabel extension. Mult Scler. 2022 Mar;28(3):480-6. doi: 10.1177/13524585211038291. Epub 2021 Sep 9.
32. Bonnan M, Valentino R, Debeugny S, et al. Short delay to initiate plasma exchange is the strongest predictor of outcome in severe attacks of NMO spectrum disorders. J Neurol Neurosurg Psychiatry. 2018 Apr;89(4):346-51. doi: 10.1136/jnnp-2017-316286. Epub 2017 Oct 13.
33. Mealy MA, Mossburg SE, Kim SH, et al. Long-term disability in neuromyelitis optica spectrum disorder with a history of myelitis is associated with age at onset, delay in diagnosis/preventive treatment, MRI lesion length and presence of symptomatic brain lesions. Mult Scler Relat Disord. 2019 Feb;28:64-8. doi: 10.1016/j.msard.2018.12.011. Epub 2018 Dec 9.
34. Carnero Contentti E, Rojas JI, Cristiano E, et al. Latin American consensus recommendations for management and treatment of neuromyelitis optica spectrum disorders in clinical practice Mult Scler Relat Disord. 2020 Oct;45:102428. Mult Scler Relat Disord. 2021 Jul;52:103026. doi: 10.1016/j.msard.2021.103026. Epub 2021 May 25. Erratum for: Mult Scler Relat Disord. 2020 Oct;45:102428.
35. Levy M, Fujihara K, Palace J. New therapies for neuromyelitis optica spectrum disorder. Lancet Neurol. 2021 Jan;20(1):60-7. doi: 10.1016/S1474-4422(20)30392-6. Epub 2020 Nov 10.
36. Ciron J, Audoin B, Bourre B, et al; NOMADMUS group, under the aegis of OFSEP, SFSEP. Recommendations for the use of Rituximab in neuromyelitis optica spectrum disorders. Rev Neurol (Paris). 2018 Apr;174(4):255-64. doi: 10.1016/j.neurol.2017.11.005. Epub 2018 Mar 30.
37. Gao F, Chai B, Gu C, et al. Effectiveness of rituximab in neuromyelitis optica: a meta-analysis. BMC Neurol. 2019 Mar 6;19(1):36. doi: 10.1186/s12883-019-1261-2
38. Nikoo Z, Badihian S, Shaygannejad V, et al. Comparison of the efficacy of azathioprine and rituximab in neuromyelitis optica spectrum disorder: a randomized clinical trial. J Neurol. 2017 Sep;264(9):2003-9. doi: 10.1007/s00415-017-8590-0. Epub 2017 Aug 22.
39. Songwisit S, Kosiyakul P, Jitprapaikulsan J, et al. Efficacy and safety of mycophenolate mofetil therapy in neuromyelitis optica spectrum disorders: a systematic review and meta-analysis. Sci Rep. 2020 Oct 7;10(1):16727. doi: 10.1038/s41598-020-73882-8
40. Ringelstein M, Ayzenberg I, Harmel J, et al. Long-term Therapy With Interleukin 6 Receptor Blockade in Highly Active Neuromyelitis Optica Spectrum Disorder. JAMA Neurol. 2015 Jul;72(7):756-63. doi: 10.1001/jamaneurol. 2015.0533
41. Yamamura T, Kleiter I, Fujihara K, et al. Trial of Satralizumab in Neuromyelitis Optica Spectrum Disorder. N Engl J Med. 2019 Nov 28;381(22):2114-24. doi: 10.1056/NEJMoa1901747
42. Traboulsee A, Greenberg BM, Bennett JL, et al. Safety and efficacy of satralizumab monotherapy in neuromyelitis optica spectrum disorder: a randomised, double-blind, multicentre, placebo-controlled phase 3 trial. Lancet Neurol. 2020 May;19(5):402-12. doi: 10.1016/S1474-4422(20)30078-8
43. Nytrova P, Potlukova E, Kemlink D, et al. Complement activation in patients with neuromyelitis optica. J Neuroimmunol. 2014 Sep 15;274(1-2):185-91. doi: 10.1016/j.jneuroim. 2014.07.001. Epub 2014 Jul 11.
44. Saadoun S, Papadopoulos MC. Role of membrane complement regulators in neuromyelitis optica. Mult Scler. 2015 Nov;21(13):1644-54. doi: 10.1177/1352458515571446. Epub 2015 Feb 19.
45. Saadoun S, Waters P, Bell BA, et al. Intracerebral injection of neuromyelitis optica immunoglobulin G and human complement produces neuromyelitis optica lesions in mice. Brain. 2010 Feb;133(Pt 2):349-61. doi: 10.1093/brain/awp309. Epub 2010 Jan 4.
46. Kuroda H, Fujihara K, Takano R, et al. Increase of complement fragment C5a in cerebrospinal fluid during exacerbation of neuromyelitis optica. J Neuroimmunol. 2013 Jan 15;254(1-2):178-82. doi: 10.1016/j.jneuroim.2012.09.002. Epub 2012 Sep 25.
47. Wang H, Wang K, Wang C, et al. Increased soluble C5b-9 in CSF of neuromyelitis optica. Scand J Immunol. 2014 Feb;79(2):127-30. doi: 10.1111/sji.12132
48. Pittock SJ, Berthele A, Fujihara K, et al. Eculizumab in Aquaporin-4-Positive Neuromyelitis Optica Spectrum Disorder. N Engl J Med. 2019 Aug 15;381(7):614-25. doi: 10.1056/NEJMoa1900866
49. Ultomiris approved in the EU for adults with neuromyelitis optica spectrum disorder (NMOSD). Published 10 May 2023. Available at: https://www.astrazeneca.com/media-centre/pressreleases/2023/ultomiris-approved-in-the-eu-foradults-with-neuromyelitis-optica-spectrum-disorder-nmosd.html (accessed 03.07.2023).
50. Ariceta G, Dixon BP, Kim SH, et al; 312 Study Group. The long-acting C5 inhibitor, ravulizumab, is effective and safe in pediatric patients with atypical hemolytic uremic syndrome naХve to complement inhibitor treatment. Kidney Int. 2021 Jul;100(1):225-37. doi: 10.1016/j.kint.2020.10.046. Epub 2020 Dec 8.
51. Pittock SJ, Barnett M, Bennett JL, et al. Ravulizumab in Aquaporin-4-Positive Neuromyelitis Optica Spectrum Disorder. Ann Neurol. 2023 Jun;93(6):1053-68. doi: 10.1002/ana.26626
Review
For citations:
Simaniv TO, Bakhtiyarova KZ, Belova AN, Goncharova ZA, Sivertseva SA, Spirin NN, Totolyan NA, Trushnikova TN, Turova EL, Khaibullin TI, Sherman MA, Boyko AN. Diagnostic criteria and treatment of neuromyelitis optica spectrum disorders (NMOSD). Nevrologiya, neiropsikhiatriya, psikhosomatika = Neurology, Neuropsychiatry, Psychosomatics. 2023;15:71-75. (In Russ.) https://doi.org/10.14412/2074-2711-2023-1S-71-75