Abstract
Neuromyelitis optica (NMO), an autoimmune inflammatory disease of the central nervous system (CNS), is related with autoantibodies for aquaporin-4, which is the most abundant water channel in CNS. The clinical syndromes of NMO, such as longitudinally extensive transverse myelitis and optic neuritis, can occur in the context of systemic rheumatologic diseases, such as systemic lupus erythematosus and Sjögren syndrome. It is debatable as to whether NMO is a feature of genetic tendency toward humoral autoimmunity or a CNS complication of systemic rheumatologic diseases. Current evidence suggests that NMO coexists with systemic rheumatologic diseases rather than a complication from them. Early immunosuppressive therapies should be considered in these patients since just one or two attacks can cause severe neurological disability.
References
2. Devic E. Myelite subaigue compliquee de nevrite optique. Bull Med. 1894; 8:1033–4.
3. Wingerchuk DM, Hogancamp WF, O'Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic's syndrome). Neurology. 1999; 53:1107–14.
4. Lennon VA, Wingerchuk DM, Kryzer TJ, Pittock SJ, Lucchinetti CF, Fujihara K, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004; 364:2106–12.
5. Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med. 2005; 202:473–7.
6. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology. 2006; 66:1485–9.
7. Barnett MH, Sutton I. Neuromyelitis optica: not a multiple sclerosis variant. Curr Opin Neurol. 2012; 25:215–20.
8. Kim SH, Kim W, Li XF, Jung IJ, Kim HJ. Does interferon beta treatment exacerbate neuromyelitis optica spectrum disorder? Mult Scler. 2012; 18:1480–3.
9. Rash JE, Yasumura T, Hudson CS, Agre P, Nielsen S. Direct immunogold labeling of aquaporin-4 in square ar-rays of astrocyte and ependymocyte plasma membranes in rat brain and spinal cord. Proc Natl Acad Sci U S A. 1998; 95:11981–6.
10. Kim SH, Kim W, Li XF, Jung IJ, Kim HJ. Clinical spectrum of CNS aquaporin-4 autoimmunity. Neurology. 2012; 78:1179–85.
11. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007; 6:805–15.
12. O'Riordan JI, Gallagher HL, Thompson AJ, Howard RS, Kingsley DP, Thompson EJ, et al. Clinical, CSF, and MRI findings in Devic's neuromyelitis optica. J Neurol Neurosurg Psychiatry. 1996; 60:382–7.
13. Wingerchuk DM, Weinshenker BG. The emerging relationship between neuromyelitis optica and systemic rheumatologic autoimmune disease. Mult Scler. 2012; 18:5–10.
14. Pittock SJ, Lennon VA, de Seze J, Vermersch P, Homburger HA, Wingerchuk DM, et al. Neuromyelitis optica and non organ-specific autoimmunity. Arch Neurol. 2008; 65:78–83.
15. Wandinger KP, Stangel M, Witte T, Venables P, Charles P, Jarius S, et al. Autoantibodies against aquaporin-4 in patients with neuropsychiatric systemic lupus erythematosus and primary Sjögren's syndrome. Arthritis Rheum. 2010; 62:1198–200.
16. Jarius S, Jacobi C, de Seze J, Zephir H, Paul F, Franciotta D, et al. Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders. Mult Scler. 2011; 17:1067–73.
17. Kahlenberg JM. Neuromyelitis optica spectrum disorder as an initial presentation of primary Sjögren's syndrome. Semin Arthritis Rheum. 2011; 40:343–8.
18. Kim SM, Waters P, Vincent A, Kim SY, Kim HJ, Hong YH, et al. Sjogren's syndrome myelopathy: spinal cord involvement in Sjogren's syndrome might be a manifestation of neuromyelitis optica. Mult Scler. 2009; 15:1062–8.
19. Espinosa G, Mendizábal A, Mínguez S, Ramo-Tello C, Capellades J, Olivé A, et al. Transverse myelitis affecting more than 4 spinal segments associated with systemic lupus erythematosus: clinical, immunological, and radiological characteristics of 22 patients. Semin Arthritis Rheum. 2010; 39:246–56.
20. Rodrigues CE, de Carvalho JF. Clinical, radiologic, and therapeutic analysis of 14 patients with transverse myelitis associated with antiphospholipid syndrome: report of 4 cases and review of the literature. Semin Arthritis Rheum. 2011; 40:349–57.
21. Kister I, Gulati S, Boz C, Bergamaschi R, Piccolo G, Oger J, et al. Neuromyelitis optica in patients with myasthenia gravis who underwent thymectomy. Arch Neurol. 2006; 63:851–6.
22. McKeon A, Lennon VA, Jacob A, Matiello M, Lucchinetti CF, Kale N, et al. Coexistence of myasthenia gravis and serological markers of neurological autoimmunity in neuromyelitis optica. Muscle Nerve. 2009; 39:87–90.
23. Jacob S, Zarei M, Kenton A, Allroggen H. Gluten sensitivity and neuromyelitis optica: two case reports. J Neurol Neurosurg Psychiatry. 2005; 76:1028–30.
24. Jarius S, Jacob S, Waters P, Jacob A, Littleton E, Vincent A. Neuromyelitis optica in patients with gluten sensitivity associated with antibodies to aquaporin-4. J Neurol Neurosurg Psychiatry. 2008; 79:1084.
25. Cho JH, Gregersen PK. Genomics and the multifactorial nature of human autoimmune disease. N Engl J Med. 2011; 365:1612–23.
26. Johnson RT, Richardson EP. The neurological manifestations of systemic lupus erythematosus. Medicine (Baltimore). 1968; 47:337–69.
27. Hinson SR, Pittock SJ, Lucchinetti CF, Roemer SF, Fryer JP, Kryzer TJ, et al. Pathogenic potential of IgG binding to water channel extracellular domain in neuromyelitis optica. Neurology. 2007; 69:2221–31.
28. Bradl M, Misu T, Takahashi T, Watanabe M, Mader S, Reindl M, et al. Neuromyelitis optica: pathogenicity of patient immunoglobulin in vivo. Ann Neurol. 2009; 66:630–43.
29. Weinshenker BG, Wingerchuk DM, Vukusic S, Linbo L, Pittock SJ, Lucchinetti CF, et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol. 2006; 59:566–9.
30. Costanzi C, Matiello M, Lucchinetti CF, Weinshenker BG, Pittock SJ, Mandrekar J, et al. Azathioprine: toler-ability, efficacy, and predictors of benefit in neuromyelitis optica. Neurology. 2011; 77:659–66.
31. Kim SH, Huh SY, Lee SJ, Joung A, Kim HJ. A 5-year followup of rituximab treatment in patients with neuromyelitis optica spectrum disorder. JAMA Neurol. 2013; 70:1110–7.
32. Jacob A, Matiello M, Weinshenker BG, Wingerchuk DM, Lucchinetti C, Shuster E, et al. Treatment of neuromyelitis optica with mycophenolate mofetil: retrospective analysis of 24 patients. Arch Neurol. 2009; 66:1128–33.
33. Kitley J, Elsone L, George J, Waters P, Woodhall M, Vincent A, et al. Methotrexate is an alternative to azathio-prine in neuromyelitis optica spectrum disorders with aquaporin-4 antibodies. J Neurol Neurosurg Psychiatry. 2013; 84:918–21.
34. Bichuetti DB, Oliveira EM, Boulos Fde C, Gabbai AA. Lack of response to pulse cyclophosphamide in neuromyelitis optica: evaluation of 7 patients. Arch Neurol. 2012; 69:938–9.
35. Bosch X, Saiz A, Ramos-Casals M. BIOGEAS Study Group. Monoclonal antibody therapy-associated neurological disorders. Nat Rev Neurol. 2011; 7:165–72.
36. Ortiz de Zárate JC, Tamaroff L, Sica RE, Rodriguez JA. Neuromyelitis optica versus subacute necrotic myelitis. II. Anatomical study of two cases. J Neurol Neurosurg Psychiatry. 1968; 31:641–5.
37. Lucchinetti CF, Mandler RN, McGavern D, Bruck W, Gleich G, Ransohoff RM, et al. A role for humoral mechanisms in the pathogenesis of Devic's neuromyelitis optica. Brain. 2002; 125:1450–61.