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Neuromyelitis Optica Spectrum Disorder
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Neuromyelitis optica spectrum disorder (NMOSD)
              • Synonyms

                • Devic disease, Devic syndrome, Devic neuromyelitis optica (NMO), optic-spinal multiple sclerosis (MS)
              • Definitions

                • AQP4 antibody-seropositive NMOSD is autoimmune astrocytopathic disease of optic nerves and spinal cord with variable brain parenchymal involvement

              IMAGING

              • General Features

                • Imaging Recommendations

                  • MR Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Staging, Grading, & Classification

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. Dubey D et al: Clinical, radiologic, and prognostic features of myelitis associated with myelin oligodendrocyte glycoprotein autoantibody. JAMA Neurol. 76(3):301-9, 2019
                                        2. Borisow N et al: Diagnosis and treatment of NMO spectrum disorder and MOG-encephalomyelitis. Front Neurol. 9:888, 2018
                                        3. Weinshenker BG et al: Neuromyelitis spectrum disorders. Mayo Clin Proc. 92(4):663-79, 2017
                                        4. Kleiter I et al: Neuromyelitis optica: evaluation of 871 attacks and 1,153 treatment courses. Ann Neurol. 79(2):206-16, 2016
                                        5. Kim HJ et al: MRI characteristics of neuromyelitis optica spectrum disorder: an international update. Neurology. 84(11):1165-73, 2015
                                        6. Wingerchuk DM et al: International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 85(2):177-89, 2015
                                        7. Kitley J et al: Longitudinally extensive transverse myelitis with and without aquaporin 4 antibodies. JAMA Neurol. 70(11):1375-81, 2013
                                        8. Sato DK et al: Aquaporin-4 antibody-positive cases beyond current diagnostic criteria for NMO spectrum disorders. Neurology. 80(24):2210-6, 2013
                                        9. Morrow MJ et al: Neuromyelitis optica. J Neuroophthalmol. 32(2):154-66, 2012
                                        10. Chan KH et al: Brain involvement in neuromyelitis optica spectrum disorders. Arch Neurol. 68(11):1432-9, 2011
                                        11. de Seze J et al: Magnetic resonance spectroscopy evaluation in patients with neuromyelitis optica. J Neurol Neurosurg Psychiatry. 81(4):409-11, 2010
                                        12. Lu Z et al: Characteristic linear lesions and longitudinally extensive spinal cord lesions in Chinese patients with neuromyelitis optica. J Neurol Sci. 293(1-2):92-6, 2010
                                        13. Sellner J et al: The clinical spectrum and immunobiology of parainfectious neuromyelitis optica (Devic) syndromes. J Autoimmun. 34(4):371-9, 2010
                                        14. Bichuetti DB et al: Neuromyelitis optica in Brazil: a study on clinical and prognostic factors. Mult Scler. 15(5):613-9, 2009
                                        15. Heerlein K et al: Aquaporin-4 antibody positive longitudinally extensive transverse myelitis following varicella zoster infection. J Neurol Sci. 276(1-2):184-6, 2009
                                        16. Jacob A: Neuromyelitis optica - an update: 2007-2009. Ann Indian Acad Neurol. 12(4):231-7, 2009
                                        17. Zhang B et al: Correlation between serum thyroxine and complements in patients with multiple sclerosis and neuromyelitis optica. Neuro Endocrinol Lett. 29(2):256-60, 2008
                                        18. Krupp LB et al: Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology. 68(16 Suppl 2):S7-12, 2007
                                        19. Matiello M et al: Neuromyelitis optica. Curr Opin Neurol. 20(3):255-60, 2007
                                        20. Pittock SJ et al: The pathology of MS: new insights and potential clinical applications. Neurologist. 13(2):45-56, 2007
                                        21. Pohl D et al: Treatment of pediatric multiple sclerosis and variants. Neurology. 68(16 Suppl 2):S54-65, 2007
                                        22. Takahashi T et al: Anti-aquaporin-4 antibody is involved in the pathogenesis of NMO: a study on antibody titre. Brain. 130(Pt 5):1235-43, 2007
                                        23. Tanaka K et al: Anti-aquaporin 4 antibody in selected Japanese multiple sclerosis patients with long spinal cord lesions. Mult Scler. 2007
                                        24. Wingerchuk DM: Neuromyelitis optica: new findings on pathogenesis. Int Rev Neurobiol. 79:665-88, 2007
                                        25. Wingerchuk DM et al: Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis. Curr Opin Neurol. 20(3):343-50, 2007
                                        26. Wingerchuk DM et al: The spectrum of neuromyelitis optica. Lancet Neurol. 6(9):805-15, 2007
                                        27. Deshpande R et al: The natural history of multiple sclerosis. Adv Neurol. 98:1-15, 2006
                                        28. Mandler RN: Neuromyelitis optica - Devic's syndrome, update. Autoimmun Rev. 5(8):537-43, 2006
                                        29. Pittock SJ et al: Brain abnormalities in neuromyelitis optica. Arch Neurol. 63(3):390-6, 2006
                                        30. Weinstock-Guttman B et al: Study of mitoxantrone for the treatment of recurrent neuromyelitis optica (Devic disease). Arch Neurol. 63(7):957-63, 2006
                                        31. Wingerchuk DM et al: Revised diagnostic criteria for neuromyelitis optica. Neurology. 66(10):1485-9, 2006
                                        32. Lennon VA et al: IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med. 202(4):473-7, 2005
                                        33. Pearce JM: Neuromyelitis optica. Spinal Cord. 43(11):631-4, 2005
                                        34. Poppe AY et al: Neuromyelitis optica with hypothalamic involvement. Mult Scler. 11(5):617-21, 2005
                                        35. Ghezzi A et al: Clinical characteristics, course and prognosis of relapsing Devic's neuromyelitis Optica. J Neurol. 251(1):47-52, 2004
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Neuromyelitis optica spectrum disorder (NMOSD)
                                                    • Synonyms

                                                      • Devic disease, Devic syndrome, Devic neuromyelitis optica (NMO), optic-spinal multiple sclerosis (MS)
                                                    • Definitions

                                                      • AQP4 antibody-seropositive NMOSD is autoimmune astrocytopathic disease of optic nerves and spinal cord with variable brain parenchymal involvement

                                                    IMAGING

                                                    • General Features

                                                      • Imaging Recommendations

                                                        • MR Findings

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Staging, Grading, & Classification

                                                                • Gross Pathologic & Surgical Features

                                                                  • Microscopic Features

                                                                    CLINICAL ISSUES

                                                                    • Presentation

                                                                      • Demographics

                                                                        • Natural History & Prognosis

                                                                          • Treatment

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. Dubey D et al: Clinical, radiologic, and prognostic features of myelitis associated with myelin oligodendrocyte glycoprotein autoantibody. JAMA Neurol. 76(3):301-9, 2019
                                                                              2. Borisow N et al: Diagnosis and treatment of NMO spectrum disorder and MOG-encephalomyelitis. Front Neurol. 9:888, 2018
                                                                              3. Weinshenker BG et al: Neuromyelitis spectrum disorders. Mayo Clin Proc. 92(4):663-79, 2017
                                                                              4. Kleiter I et al: Neuromyelitis optica: evaluation of 871 attacks and 1,153 treatment courses. Ann Neurol. 79(2):206-16, 2016
                                                                              5. Kim HJ et al: MRI characteristics of neuromyelitis optica spectrum disorder: an international update. Neurology. 84(11):1165-73, 2015
                                                                              6. Wingerchuk DM et al: International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 85(2):177-89, 2015
                                                                              7. Kitley J et al: Longitudinally extensive transverse myelitis with and without aquaporin 4 antibodies. JAMA Neurol. 70(11):1375-81, 2013
                                                                              8. Sato DK et al: Aquaporin-4 antibody-positive cases beyond current diagnostic criteria for NMO spectrum disorders. Neurology. 80(24):2210-6, 2013
                                                                              9. Morrow MJ et al: Neuromyelitis optica. J Neuroophthalmol. 32(2):154-66, 2012
                                                                              10. Chan KH et al: Brain involvement in neuromyelitis optica spectrum disorders. Arch Neurol. 68(11):1432-9, 2011
                                                                              11. de Seze J et al: Magnetic resonance spectroscopy evaluation in patients with neuromyelitis optica. J Neurol Neurosurg Psychiatry. 81(4):409-11, 2010
                                                                              12. Lu Z et al: Characteristic linear lesions and longitudinally extensive spinal cord lesions in Chinese patients with neuromyelitis optica. J Neurol Sci. 293(1-2):92-6, 2010
                                                                              13. Sellner J et al: The clinical spectrum and immunobiology of parainfectious neuromyelitis optica (Devic) syndromes. J Autoimmun. 34(4):371-9, 2010
                                                                              14. Bichuetti DB et al: Neuromyelitis optica in Brazil: a study on clinical and prognostic factors. Mult Scler. 15(5):613-9, 2009
                                                                              15. Heerlein K et al: Aquaporin-4 antibody positive longitudinally extensive transverse myelitis following varicella zoster infection. J Neurol Sci. 276(1-2):184-6, 2009
                                                                              16. Jacob A: Neuromyelitis optica - an update: 2007-2009. Ann Indian Acad Neurol. 12(4):231-7, 2009
                                                                              17. Zhang B et al: Correlation between serum thyroxine and complements in patients with multiple sclerosis and neuromyelitis optica. Neuro Endocrinol Lett. 29(2):256-60, 2008
                                                                              18. Krupp LB et al: Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology. 68(16 Suppl 2):S7-12, 2007
                                                                              19. Matiello M et al: Neuromyelitis optica. Curr Opin Neurol. 20(3):255-60, 2007
                                                                              20. Pittock SJ et al: The pathology of MS: new insights and potential clinical applications. Neurologist. 13(2):45-56, 2007
                                                                              21. Pohl D et al: Treatment of pediatric multiple sclerosis and variants. Neurology. 68(16 Suppl 2):S54-65, 2007
                                                                              22. Takahashi T et al: Anti-aquaporin-4 antibody is involved in the pathogenesis of NMO: a study on antibody titre. Brain. 130(Pt 5):1235-43, 2007
                                                                              23. Tanaka K et al: Anti-aquaporin 4 antibody in selected Japanese multiple sclerosis patients with long spinal cord lesions. Mult Scler. 2007
                                                                              24. Wingerchuk DM: Neuromyelitis optica: new findings on pathogenesis. Int Rev Neurobiol. 79:665-88, 2007
                                                                              25. Wingerchuk DM et al: Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis. Curr Opin Neurol. 20(3):343-50, 2007
                                                                              26. Wingerchuk DM et al: The spectrum of neuromyelitis optica. Lancet Neurol. 6(9):805-15, 2007
                                                                              27. Deshpande R et al: The natural history of multiple sclerosis. Adv Neurol. 98:1-15, 2006
                                                                              28. Mandler RN: Neuromyelitis optica - Devic's syndrome, update. Autoimmun Rev. 5(8):537-43, 2006
                                                                              29. Pittock SJ et al: Brain abnormalities in neuromyelitis optica. Arch Neurol. 63(3):390-6, 2006
                                                                              30. Weinstock-Guttman B et al: Study of mitoxantrone for the treatment of recurrent neuromyelitis optica (Devic disease). Arch Neurol. 63(7):957-63, 2006
                                                                              31. Wingerchuk DM et al: Revised diagnostic criteria for neuromyelitis optica. Neurology. 66(10):1485-9, 2006
                                                                              32. Lennon VA et al: IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med. 202(4):473-7, 2005
                                                                              33. Pearce JM: Neuromyelitis optica. Spinal Cord. 43(11):631-4, 2005
                                                                              34. Poppe AY et al: Neuromyelitis optica with hypothalamic involvement. Mult Scler. 11(5):617-21, 2005
                                                                              35. Ghezzi A et al: Clinical characteristics, course and prognosis of relapsing Devic's neuromyelitis Optica. J Neurol. 251(1):47-52, 2004