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Neuromyelitis Optica
Kevin R. Moore, MDJeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Neuromyelitis optica (NMO)
                • Longitudinally extensive (LTE)
              • Synonyms

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

                • Autoimmune inflammatory disorder involving myelin of neurons of optic nerves and spinal cord with limited brain parenchymal involvement

              IMAGING

              • General Features

                • Imaging Recommendations

                  • MR Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      Selected References

                                      1. Baghbanian SM et al: A comparison of pediatric and adult neuromyelitis optica spectrum disorders: A review of clinical manifestation, diagnosis, and treatment. J Neurol Sci. 388:222-31, 2018
                                      2. Baumann M et al: MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein. J Neurol. 265(4):845-55, 2018
                                      3. Gombolay GY et al: Pediatric neuromyelitis optica spectrum disorders. Curr Treat Options Neurol. 20(6):19, 2018
                                      4. Ikeda A et al: MRI findings in pediatric neuromyelitis optica spectrum disorder with MOG antibody: Four cases and review of the literature. Brain Dev. ePub, 2018
                                      5. McGee JC et al: Neuromyelitis optica spectrum disorder in the very young: An in depth review of the present data. J Neurol Sci. 388:232, 2018
                                      6. Akaishi T et al: Neuromyelitis optica spectrum disorders. Neuroimaging Clin N Am. 27(2):251-65, 2017
                                      7. Patterson SL et al: Neuromyelitis Optica. Rheum Dis Clin North Am. 43(4):579-91, 2017
                                      8. Chitnis T et al: Clinical features of neuromyelitis optica in children: US Network of Pediatric MS Centers report. Neurology. 86(3):245-52, 2016
                                      9. Tenembaum S et al: Neuromyelitis optica spectrum disorders in children and adolescents. Neurology. 87(9 Suppl 2):S59-66, 2016
                                      10. Kitley J et al: Longitudinally extensive transverse myelitis with and without aquaporin 4 antibodies. JAMA Neurol. 70(11):1375-81, 2013
                                      11. Mealy MA 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. 28:64-68, 2018
                                      12. Reindl M et al: Myelin oligodendrocyte glycoprotein antibodies in neurological disease. Nat Rev Neurol. ePub, 2018
                                      13. Shahmohammadi S et al: Autoimmune diseases associated with Neuromyelitis Optica Spectrum Disorders: A literature review. Mult Scler Relat Disord. 27:350-363, 2018
                                      14. Sato DK et al: Aquaporin-4 antibody-positive cases beyond current diagnostic criteria for NMO spectrum disorders. Neurology. 80(24):2210-6, 2013
                                      15. Morrow MJ et al: Neuromyelitis optica. J Neuroophthalmol. 32(2):154-66, 2012
                                      16. Wynford-Thomas R et al: Neurological update: MOG antibody disease. J Neurol. ePub, 2018
                                      17. Chan KH et al: Brain involvement in neuromyelitis optica spectrum disorders. Arch Neurol. 68(11):1432-9, 2011
                                      18. de Seze J et al: Magnetic resonance spectroscopy evaluation in patients with neuromyelitis optica. J Neurol Neurosurg Psychiatry. 81(4):409-11, 2010
                                      19. 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
                                      20. Sellner J et al: The clinical spectrum and immunobiology of parainfectious neuromyelitis optica (Devic) syndromes. J Autoimmun. 34(4):371-9, 2010
                                      21. Bichuetti DB et al: Neuromyelitis optica in Brazil: a study on clinical and prognostic factors. Mult Scler. 15(5):613-9, 2009
                                      22. 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
                                      23. Jacob A: Neuromyelitis optica - an update: 2007-2009. Ann Indian Acad Neurol. 12(4):231-7, 2009
                                      24. 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
                                      25. Krupp LB et al: Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology. 68(16 Suppl 2):S7-12, 2007
                                      26. Matiello M et al: Neuromyelitis optica. Curr Opin Neurol. 20(3):255-60, 2007
                                      27. Pittock SJ et al: The pathology of MS: new insights and potential clinical applications. Neurologist. 13(2):45-56, 2007
                                      28. Pohl D et al: Treatment of pediatric multiple sclerosis and variants. Neurology. 68(16 Suppl 2):S54-65, 2007
                                      29. 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
                                      30. Tanaka K et al: Anti-aquaporin 4 antibody in selected Japanese multiple sclerosis patients with long spinal cord lesions. Mult Scler. 2007
                                      31. Wingerchuk DM et al: The spectrum of neuromyelitis optica. Lancet Neurol. 6(9):805-15, 2007
                                      32. Wingerchuk DM et al: Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis. Curr Opin Neurol. 20(3):343-50, 2007
                                      33. Wingerchuk DM: Neuromyelitis optica: new findings on pathogenesis. Int Rev Neurobiol. 79:665-88, 2007
                                      34. Deshpande R et al: The natural history of multiple sclerosis. Adv Neurol. 98:1-15, 2006
                                      35. Mandler RN: Neuromyelitis optica - Devic's syndrome, update. Autoimmun Rev. 5(8):537-43, 2006
                                      36. Pittock SJ et al: Brain abnormalities in neuromyelitis optica. Arch Neurol. 63(3):390-6, 2006
                                      37. Weinstock-Guttman B et al: Study of mitoxantrone for the treatment of recurrent neuromyelitis optica (Devic disease). Arch Neurol. 63(7):957-63, 2006
                                      38. Wingerchuk DM et al: Revised diagnostic criteria for neuromyelitis optica. Neurology. 66(10):1485-9, 2006
                                      39. 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
                                      40. Pearce JM: Neuromyelitis optica. Spinal Cord. 43(11):631-4, 2005
                                      41. Poppe AY et al: Neuromyelitis optica with hypothalamic involvement. Mult Scler. 11(5):617-21, 2005
                                      42. 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 (NMO)
                                                    • Longitudinally extensive (LTE)
                                                  • Synonyms

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

                                                    • Autoimmune inflammatory disorder involving myelin of neurons of optic nerves and spinal cord with limited brain parenchymal involvement

                                                  IMAGING

                                                  • General Features

                                                    • Imaging Recommendations

                                                      • MR Findings

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Gross Pathologic & Surgical Features

                                                              • Microscopic Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Consider

                                                                          Selected References

                                                                          1. Baghbanian SM et al: A comparison of pediatric and adult neuromyelitis optica spectrum disorders: A review of clinical manifestation, diagnosis, and treatment. J Neurol Sci. 388:222-31, 2018
                                                                          2. Baumann M et al: MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein. J Neurol. 265(4):845-55, 2018
                                                                          3. Gombolay GY et al: Pediatric neuromyelitis optica spectrum disorders. Curr Treat Options Neurol. 20(6):19, 2018
                                                                          4. Ikeda A et al: MRI findings in pediatric neuromyelitis optica spectrum disorder with MOG antibody: Four cases and review of the literature. Brain Dev. ePub, 2018
                                                                          5. McGee JC et al: Neuromyelitis optica spectrum disorder in the very young: An in depth review of the present data. J Neurol Sci. 388:232, 2018
                                                                          6. Akaishi T et al: Neuromyelitis optica spectrum disorders. Neuroimaging Clin N Am. 27(2):251-65, 2017
                                                                          7. Patterson SL et al: Neuromyelitis Optica. Rheum Dis Clin North Am. 43(4):579-91, 2017
                                                                          8. Chitnis T et al: Clinical features of neuromyelitis optica in children: US Network of Pediatric MS Centers report. Neurology. 86(3):245-52, 2016
                                                                          9. Tenembaum S et al: Neuromyelitis optica spectrum disorders in children and adolescents. Neurology. 87(9 Suppl 2):S59-66, 2016
                                                                          10. Kitley J et al: Longitudinally extensive transverse myelitis with and without aquaporin 4 antibodies. JAMA Neurol. 70(11):1375-81, 2013
                                                                          11. Mealy MA 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. 28:64-68, 2018
                                                                          12. Reindl M et al: Myelin oligodendrocyte glycoprotein antibodies in neurological disease. Nat Rev Neurol. ePub, 2018
                                                                          13. Shahmohammadi S et al: Autoimmune diseases associated with Neuromyelitis Optica Spectrum Disorders: A literature review. Mult Scler Relat Disord. 27:350-363, 2018
                                                                          14. Sato DK et al: Aquaporin-4 antibody-positive cases beyond current diagnostic criteria for NMO spectrum disorders. Neurology. 80(24):2210-6, 2013
                                                                          15. Morrow MJ et al: Neuromyelitis optica. J Neuroophthalmol. 32(2):154-66, 2012
                                                                          16. Wynford-Thomas R et al: Neurological update: MOG antibody disease. J Neurol. ePub, 2018
                                                                          17. Chan KH et al: Brain involvement in neuromyelitis optica spectrum disorders. Arch Neurol. 68(11):1432-9, 2011
                                                                          18. de Seze J et al: Magnetic resonance spectroscopy evaluation in patients with neuromyelitis optica. J Neurol Neurosurg Psychiatry. 81(4):409-11, 2010
                                                                          19. 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
                                                                          20. Sellner J et al: The clinical spectrum and immunobiology of parainfectious neuromyelitis optica (Devic) syndromes. J Autoimmun. 34(4):371-9, 2010
                                                                          21. Bichuetti DB et al: Neuromyelitis optica in Brazil: a study on clinical and prognostic factors. Mult Scler. 15(5):613-9, 2009
                                                                          22. 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
                                                                          23. Jacob A: Neuromyelitis optica - an update: 2007-2009. Ann Indian Acad Neurol. 12(4):231-7, 2009
                                                                          24. 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
                                                                          25. Krupp LB et al: Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology. 68(16 Suppl 2):S7-12, 2007
                                                                          26. Matiello M et al: Neuromyelitis optica. Curr Opin Neurol. 20(3):255-60, 2007
                                                                          27. Pittock SJ et al: The pathology of MS: new insights and potential clinical applications. Neurologist. 13(2):45-56, 2007
                                                                          28. Pohl D et al: Treatment of pediatric multiple sclerosis and variants. Neurology. 68(16 Suppl 2):S54-65, 2007
                                                                          29. 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
                                                                          30. Tanaka K et al: Anti-aquaporin 4 antibody in selected Japanese multiple sclerosis patients with long spinal cord lesions. Mult Scler. 2007
                                                                          31. Wingerchuk DM et al: The spectrum of neuromyelitis optica. Lancet Neurol. 6(9):805-15, 2007
                                                                          32. Wingerchuk DM et al: Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis. Curr Opin Neurol. 20(3):343-50, 2007
                                                                          33. Wingerchuk DM: Neuromyelitis optica: new findings on pathogenesis. Int Rev Neurobiol. 79:665-88, 2007
                                                                          34. Deshpande R et al: The natural history of multiple sclerosis. Adv Neurol. 98:1-15, 2006
                                                                          35. Mandler RN: Neuromyelitis optica - Devic's syndrome, update. Autoimmun Rev. 5(8):537-43, 2006
                                                                          36. Pittock SJ et al: Brain abnormalities in neuromyelitis optica. Arch Neurol. 63(3):390-6, 2006
                                                                          37. Weinstock-Guttman B et al: Study of mitoxantrone for the treatment of recurrent neuromyelitis optica (Devic disease). Arch Neurol. 63(7):957-63, 2006
                                                                          38. Wingerchuk DM et al: Revised diagnostic criteria for neuromyelitis optica. Neurology. 66(10):1485-9, 2006
                                                                          39. 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
                                                                          40. Pearce JM: Neuromyelitis optica. Spinal Cord. 43(11):631-4, 2005
                                                                          41. Poppe AY et al: Neuromyelitis optica with hypothalamic involvement. Mult Scler. 11(5):617-21, 2005
                                                                          42. Ghezzi A et al: Clinical characteristics, course and prognosis of relapsing Devic's Neuromyelitis Optica. J Neurol. 251(1):47-52, 2004