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Risk factors associated with permanent disability in neuromyelitis optica spectrum disorders

Published:August 14, 2022DOI:https://doi.org/10.1016/j.msard.2022.104114

      Highlights

      • During their evolution, most NMOSD patients develop permanent disability
      • Diagnosis 12 months is the most important risk factor for permanent disability
      • EDSS 4.0 and age of onset 50 years old at first visit are also risk factors
      • Prompt recognition and treatment of NMOSD is paramount to prevent disability

      Abstract

      Background

      Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease of the central nervous system. In NMOSD, a relapse results in increased disability.

      Objective

      To assess risk factors associated with permanent disability (PD) in patients with neuromyelitis optica spectrum disorders (NMOSD).

      Methods

      We evaluated 34 cases (who developed permanent disability) and 33 controls. The assessment included the following variables: sociodemographic data and characteristics of the disease. Logistic regression analysis was performed to adjust variables associated with PD.

      Results

      fifty-one percent developed PD during follow-up; 15 (22%) developed permanent visual disability, 13 (19%) developed permanent motor disability and 6 (9%) were restricted to wheelchair. Factors associated with PD in the crude analysis were: age at onset 50 years (OR 3.95, 95% IC 1.12-13.94, p= 0.032), time from onset to diagnosis 12 months (OR 3.30, 95% IC 1.13-9.64, p= 0.029), time from onset to treatment 60 months (OR 4.16, 95% IC 1.03-16.85, p= 0.045), EDSS 4.0 at the first appointment (OR 3.21, 95% IC 1.18-8.76, p= 0.022) and severe relapses during disease evolution (OR 5.72, 95% IC 1.98-16.57, p= 0.001). Factors associated with PD in the adjusted analysis were: age at onset 50 years (OR 5.82, 95% IC 1.30-26.05, p= 0.021), time from onset to diagnosis 12 months (OR 5.43, 95% IC 1.47-20.08, p= 0.011) and severe relapses during disease evolution (OR 6.65, 95% IC 1.98-22.31, p= 0.002).

      Conclusion

      Half of patients with NMOSD may develop PD during disease evolution. Age of onset 50 years, delay to diagnosis 12 months and initial EDSS 4.0 constitute the strongest risk factors for PD.

      Keywords

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      References

        • Amato M.P.
        • Ponziani G.
        A prospective study on the prognosis of multiple sclerosis.
        Neurolog. Sci. 2000; 21: S831-S838
        • Amato M.P.
        • Fonderico M.
        • Portaccio E.
        • Pastò L.
        • Razzolini L.
        • Prestipino E.
        • Trojano M.
        Disease-modifying drugs can reduce disability progression in relapsing multiple sclerosis.
        Brain. 2020; 143: 3013-3024
        • Bender B.S.
        Infectious disease risk in the elderly.
        Immunol. Allergy Clin. 2003; 23: 57-64
        • Carnero Contentti E.
        • Daccach Marques V.
        • Soto de Castillo I.
        • Tkachuk V.
        • Ariel B.
        • Castillo M.C.
        • Rojas J.I
        Clinical features and prognosis of late-onset neuromyelitis optica spectrum disorders in a Latin American cohort.
        J. Neurol. 2020; 267: 1260-1268
        • Collongues N.
        • Marignier R.
        • Jacob A.
        • Leite M.I.
        • Siva A.
        • Paul F.
        • De Seze J.
        Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset.
        Multiple Sclerosis J. 2014; 20: 1086-1094
        • Fragoso Y.D.
        • Ruocco H.H.
        • Dias R.M.
        • Cabeça H.
        • Gonçalves R.
        • de Carvalho Sousa N.A.
        • Santos G.A.C.
        Late onset of neuromyelitis optica spectrum disorders.
        Neurology Therapy. 2019; 8: 477-482
        • Houzen H.
        • Kondo K.
        • Niino M.
        • Horiuchi K.
        • Takahashi T.
        • Nakashima I.
        • Tanaka K.
        Prevalence and clinical features of neuromyelitis optica spectrum disorders in northern Japan.
        Neurology. 2017; 89: 1995-2001
        • Jarius S.
        • Ruprecht K.
        • Wildemann B.
        • Kuempfel T.
        • Ringelstein M.
        • Geis C.
        • Paul F.
        Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: a multicentre study of 175 patients.
        J. Neuroinflammation. 2012; 9: 1-17
        • Jasiak-Zatonska M
        • Kalinowska-Lyszczarz A
        • Michalak S
        • Kozubski W.
        The immunology of neuromyelitis optica-current knowledge, clinical implications, controversies and future perspectives.
        Int. J. Mol. Sci. 2016 Mar 2; 17 (PMID: 26950113; PMCID: PMC4813137): 273https://doi.org/10.3390/ijms17030273
        • Jiao Y.
        • Fryer J.P.
        • Lennon V.A.
        • Jenkins S.M.
        • Quek A.M.
        • Smith C.Y.
        • Pittock S.J.
        Updated estimate of AQP4-IgG serostatus and disability outcome in neuromyelitis optica.
        Neurology. 2013; 81: 1197-1204
        • Kim S.H.
        • Hyun J.W.
        • Joung A.
        • Park E.Y.
        • Joo J.
        • Kim H.J.
        Predictors of response to first-line immunosuppressive therapy in neuromyelitis optica spectrum disorders.
        Multiple Sclerosis J. 2017; 23: 1902-1908
        • Kitley J.
        • Leite M.I.
        • Nakashima I.
        • Waters P.
        • McNeillis B.
        • Brown R.
        • Palace J.
        Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan.
        Brain. 2012; 135: 1834-1849
        • Ma X.
        • Kermode A.G.
        • Hu X.
        • Qiu W.
        Risk of relapse in patients with neuromyelitis optica spectrum disorder: recognition and preventive strategy.
        Multiple Sclerosis Related Disord. 2020; 46102522
        • Mealy M.A.
        • Wingerchuk D.M.
        • Greenberg B.M.
        • Levy M.
        Epidemiology of neuromyelitis optica in the United States: a multicenter analysis.
        Arch. Neurol. 2012; 69: 1176-1180
        • Mealy M.A.
        • Mossburg S.E.
        • Kim S.H.
        • Messina S.
        • Borisow N.
        • Lopez-Gonzalez R.
        • Ospina J.P.
        • Scheel M.
        • Yeshokumar A.K.
        • Awad A.
        • Leite M.I.
        • Arango J.J.
        • Paul F.
        • Palace J.
        • Kim H.J.
        • Levy M.
        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.
        Multiple Sclerosis Related Disord. 2019; 28: 64-68https://doi.org/10.1016/j.msard.2018.12.011
        • Nikolich-Žugich J.
        • Rudd B.D.
        Immune memory and aging: an infinite or finite resource?.
        Curr. Opin. Immunol. 2010; 22: 535-540
        • Papais-Alvarenga R.M.
        • Miranda-Santos C.M.
        • Puccioni-Sohler M.
        • De Almeida A.M.V.
        • Oliveira S.
        • De Oliveira C.B.
        • Poser C.M.
        Optic neuromyelitis syndrome in Brazilian patients.
        J. Neurol. Neurosur. Psychiatry. 2002; 73: 429-435
        • Papp V.
        • Magyari M.
        • Aktas O.
        • Berger T.
        • Broadley S.A.
        • Cabre P.
        • Illes Z.
        Worldwide incidence and prevalence of neuromyelitis optica: a systematic review.
        Neurology. 2021; 96: 59-77
        • Prasad S
        • Chen J.
        What you need to know about AQP4, MOG, and NMOSD.
        Semin. Neurol. 2019 Dec; 39 (Epub 2019 Dec 17. PMID: 31847043): 718-731https://doi.org/10.1055/s-0039-3399505
        • Rivera J.F.
        • Kurtzke J.F.
        • Booth V.J.A.
        • Corona T.
        Characteristics of Devic's disease (neuromyelitis optica) in Mexico.
        J. Neurol. 2008; 255: 710-715
        • Rivera V.M.
        • Hamuy F.
        • Rivas V.
        • Gracia F.
        • Rojas J.I.
        • Bichuetti D.B.
        • Abad-Herrera P.
        Status of the neuromyelitis optica spectrum disorder in Latin America.
        Multiple Sclerosis Related Disord. 2021; 53103083
        • Scalfari A.
        • Neuhaus A.
        • Daumer M.
        • DeLuca G.C.
        • Muraro P.A.
        • Ebers G.C.
        Early relapses, onset of progression, and late outcome in multiple sclerosis.
        JAMA Neurol. 2013; 70: 214-222
        • Seok J.M.
        • Cho H.J.
        • Ahn S.W.
        • Cho E.B.
        • Park M.S.
        • Joo I.S.
        • Min J.H.
        Clinical characteristics of late onset neuromyelitis optica spectrum disorder: a multicenter retrospective study in Korea.
        Multiple Sclerosis J. 2017; 23: 1748-1756
        • Sepulveda M.
        • Delgado-García G.
        • Blanco Y.
        • Sola-Valls N.
        • Martinez-Lapiscina E.H.
        • Armangué T.
        • Saiz A.
        Late-onset neuromyelitis optica spectrum disorder: the importance of autoantibody serostatus.
        Neurology-Neuroimmunology Neuroinflammation. 2019; 6
        • Tremlett H.
        • Paty D.
        • Devonshire V.
        Disability progression in multiple sclerosis is slower than previously reported.
        Neurology. 2006; 66: 172-177
        • Wingerchuk DM
        • Hogancamp WF
        • O'Brien PC
        • Weinshenker BG
        The clinical course of neuromyelitis optica (Devic's syndrome).
        Neurology. 1999; 53: 1107-1114
        • Wingerchuk DM
        • Lennon VA
        • Lucchinetti CF
        • Pittock SJ
        • Weinshenker BG.
        The spectrum of neuromyelitis optica.
        Lancet Neurol. 2007 Sep; 6 (PMID: 17706564): 805-815https://doi.org/10.1016/S1474-4422(07)70216-8
        • Wingerchuk DM.
        Neuromyelitis optica spectrum disorders.
        Continuum. 2010 Oct; 16 (Minneap Minn)Multiple SclerosisPMID: 22810601: 105-121https://doi.org/10.1212/01.CON.0000389937.69413.15
        • Wingerchuk D.M.
        • Banwell B.
        • Bennett J.L.
        • Cabre P.
        • Carroll W.
        • Chitnis T.
        • Weinshenker B.G.
        International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.
        Neurology. 2015; 85: 177-189
        • Zhang L.J.
        • Yang L.N.
        • Li T.
        • Wang J.
        • Qi Y.
        • Zhang D.Q.
        • Yang L.
        Distinctive characteristics of early-onset and late-onset neuromyelitis optica spectrum disorders.
        Int. J. Neurosci. 2017; 127: 334-338