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Brain
Diagnosis
Pathology-Based Diagnoses
Infectious, Inflammatory, and Demyelinating Disease
Inflammatory and Demyelinating Disease
Neuromyelitis Optica Spectrum Disorders
Neuromyelitis Optica Spectrum Disorders
Miral D. Jhaveri, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Neuromyelitis optica spectrum disorders (NMOSDs)
              • Aquaporin-4 (AQP4)
              • Myelin oligodendrocyte glycoprotein (MOG)
              • MOG antibody-associated disease (MOG-AAD)

            • Synonyms

              • Devic syndrome

            • Definitions

              • Autoimmune inflammatory demyelinating disease of CNS with preferential involvement of optic nerves and spinal cord, AQP4 antibody (+/-)

            IMAGING

            • General Features

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Microscopic Features

                        CLINICAL ISSUES

                        • Presentation

                          • Demographics

                            • Natural History & Prognosis

                              • Treatment

                                DIAGNOSTIC CHECKLIST

                                • Consider

                                  Selected References

                                  1. Rosenthal JF et al: CNS inflammatory demyelinating disorders: MS, NMOSD and MOG antibody associated disease. J Investig Med. 68(2):321-30, 2020
                                  2. Salama S et al: MRI differences between MOG antibody disease and AQP4 NMOSD. Mult Scler. 1352458519893093, 2020
                                  3. Fujihara K: Neuromyelitis optica spectrum disorders: still evolving and broadening. Curr Opin Neurol. 32(3):385-94, 2019
                                  4. Huda S et al: Neuromyelitis optica spectrum disorders. Clin Med (Lond). 19(2):169-76, 2019
                                  5. Wynford-Thomas R et al: Neurological update: MOG antibody disease. J Neurol. 266(5):1280-6, 2019
                                  6. Dutra BG et al: Neuromyelitis optica spectrum disorders: spectrum of MR imaging findings and their differential diagnosis. Radiographics. 38(1):169-93, 2018
                                  7. Narayan R et al: MOG antibody disease: a review of MOG antibody seropositive neuromyelitis optica spectrum disorder. Mult Scler Relat Disord. 25:66-72, 2018
                                  8. Kim HJ et al: MRI characteristics of neuromyelitis optica spectrum disorder: an international update. Neurology. 84(11):1165-73, 2015
                                  9. Wingerchuk DM et al: International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 85(2):177-89, 2015
                                  10. Barnett Y et al: Conventional and advanced imaging in neuromyelitis optica. AJNR Am J Neuroradiol. 35(8):1458-66, 2014
                                  11. Kawachi I et al: [Characteristic features of radiological findings in multiple sclerosis and neuromyelitis optica.] Nihon Rinsho. 72(11):1976-82, 2014
                                  12. Uzawa A et al: Neuromyelitis optica: concept, immunology and treatment. J Clin Neurosci. 21(1):12-21, 2014
                                  13. Jacob A et al: Current concept of neuromyelitis optica (NMO) and NMO spectrum disorders. J Neurol Neurosurg Psychiatry. 84(8):922-30, 2013
                                  14. Sato DK et al: Clinical spectrum and treatment of neuromyelitis optica spectrum disorders: evolution and current status. Brain Pathol. 23(6):647-60, 2013
                                  15. Kim W et al: Brain abnormalities in neuromyelitis optica spectrum disorder. Mult Scler Int. 2012:735486, 2012
                                  16. Lana-Peixoto MA et al: The expanded spectrum of neuromyelitis optica: evidences for a new definition. Arq Neuropsiquiatr. 70(10):807-13, 2012
                                  17. Benavente E et al: Neuromyelitis optica-AQP4: an update. Curr Rheumatol Rep. 13(6):496-505, 2011
                                  18. Cree B: Neuromyelitis optica: diagnosis, pathogenesis, and treatment. Curr Neurol Neurosci Rep. 8(5):427-33, 2008
                                  19. Jacob A et al: Treatment of neuromyelitis optica with rituximab: retrospective analysis of 25 patients. Arch Neurol. 65(11):1443-8, 2008
                                  20. Li Y et al: Brain magnetic resonance imaging abnormalities in neuromyelitis optica. Acta Neurol Scand. 118(4):218-25, 2008
                                  21. Jarius S et al: NMO-IgG in the diagnosis of neuromyelitis optica. Neurology. 68(13):1076-7, 2007
                                  22. Matiello M et al: Neuromyelitis optica. Curr Opin Neurol. 20(3):255-60, 2007
                                  23. Benedetti B et al: Grading cervical cord damage in neuromyelitis optica and MS by diffusion tensor MRI. Neurology. 67(1):161-3, 2006
                                  24. Pittock SJ et al: Brain abnormalities in neuromyelitis optica. Arch Neurol. 63(3):390-6, 2006
                                  25. Wingerchuk DM et al: Revised diagnostic criteria for neuromyelitis optica. Neurology. 66(10):1485-9, 2006
                                  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

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Neuromyelitis optica spectrum disorders (NMOSDs)
                                              • Aquaporin-4 (AQP4)
                                              • Myelin oligodendrocyte glycoprotein (MOG)
                                              • MOG antibody-associated disease (MOG-AAD)

                                            • Synonyms

                                              • Devic syndrome

                                            • Definitions

                                              • Autoimmune inflammatory demyelinating disease of CNS with preferential involvement of optic nerves and spinal cord, AQP4 antibody (+/-)

                                            IMAGING

                                            • General Features

                                              • MR Findings

                                                • Imaging Recommendations

                                                  DIFFERENTIAL DIAGNOSIS

                                                    PATHOLOGY

                                                    • General Features

                                                      • Microscopic Features

                                                        CLINICAL ISSUES

                                                        • Presentation

                                                          • Demographics

                                                            • Natural History & Prognosis

                                                              • Treatment

                                                                DIAGNOSTIC CHECKLIST

                                                                • Consider

                                                                  Selected References

                                                                  1. Rosenthal JF et al: CNS inflammatory demyelinating disorders: MS, NMOSD and MOG antibody associated disease. J Investig Med. 68(2):321-30, 2020
                                                                  2. Salama S et al: MRI differences between MOG antibody disease and AQP4 NMOSD. Mult Scler. 1352458519893093, 2020
                                                                  3. Fujihara K: Neuromyelitis optica spectrum disorders: still evolving and broadening. Curr Opin Neurol. 32(3):385-94, 2019
                                                                  4. Huda S et al: Neuromyelitis optica spectrum disorders. Clin Med (Lond). 19(2):169-76, 2019
                                                                  5. Wynford-Thomas R et al: Neurological update: MOG antibody disease. J Neurol. 266(5):1280-6, 2019
                                                                  6. Dutra BG et al: Neuromyelitis optica spectrum disorders: spectrum of MR imaging findings and their differential diagnosis. Radiographics. 38(1):169-93, 2018
                                                                  7. Narayan R et al: MOG antibody disease: a review of MOG antibody seropositive neuromyelitis optica spectrum disorder. Mult Scler Relat Disord. 25:66-72, 2018
                                                                  8. Kim HJ et al: MRI characteristics of neuromyelitis optica spectrum disorder: an international update. Neurology. 84(11):1165-73, 2015
                                                                  9. Wingerchuk DM et al: International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 85(2):177-89, 2015
                                                                  10. Barnett Y et al: Conventional and advanced imaging in neuromyelitis optica. AJNR Am J Neuroradiol. 35(8):1458-66, 2014
                                                                  11. Kawachi I et al: [Characteristic features of radiological findings in multiple sclerosis and neuromyelitis optica.] Nihon Rinsho. 72(11):1976-82, 2014
                                                                  12. Uzawa A et al: Neuromyelitis optica: concept, immunology and treatment. J Clin Neurosci. 21(1):12-21, 2014
                                                                  13. Jacob A et al: Current concept of neuromyelitis optica (NMO) and NMO spectrum disorders. J Neurol Neurosurg Psychiatry. 84(8):922-30, 2013
                                                                  14. Sato DK et al: Clinical spectrum and treatment of neuromyelitis optica spectrum disorders: evolution and current status. Brain Pathol. 23(6):647-60, 2013
                                                                  15. Kim W et al: Brain abnormalities in neuromyelitis optica spectrum disorder. Mult Scler Int. 2012:735486, 2012
                                                                  16. Lana-Peixoto MA et al: The expanded spectrum of neuromyelitis optica: evidences for a new definition. Arq Neuropsiquiatr. 70(10):807-13, 2012
                                                                  17. Benavente E et al: Neuromyelitis optica-AQP4: an update. Curr Rheumatol Rep. 13(6):496-505, 2011
                                                                  18. Cree B: Neuromyelitis optica: diagnosis, pathogenesis, and treatment. Curr Neurol Neurosci Rep. 8(5):427-33, 2008
                                                                  19. Jacob A et al: Treatment of neuromyelitis optica with rituximab: retrospective analysis of 25 patients. Arch Neurol. 65(11):1443-8, 2008
                                                                  20. Li Y et al: Brain magnetic resonance imaging abnormalities in neuromyelitis optica. Acta Neurol Scand. 118(4):218-25, 2008
                                                                  21. Jarius S et al: NMO-IgG in the diagnosis of neuromyelitis optica. Neurology. 68(13):1076-7, 2007
                                                                  22. Matiello M et al: Neuromyelitis optica. Curr Opin Neurol. 20(3):255-60, 2007
                                                                  23. Benedetti B et al: Grading cervical cord damage in neuromyelitis optica and MS by diffusion tensor MRI. Neurology. 67(1):161-3, 2006
                                                                  24. Pittock SJ et al: Brain abnormalities in neuromyelitis optica. Arch Neurol. 63(3):390-6, 2006
                                                                  25. Wingerchuk DM et al: Revised diagnostic criteria for neuromyelitis optica. Neurology. 66(10):1485-9, 2006