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Demyelinating Diseases
Blaise V. Jones, MD
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KEY FACTS

  • Diagnostic Checklist

    TERMINOLOGY

    • Definitions

      • Acquired demyelinating processes characterized by inflammation
        • Multiple sclerosis (MS)
          • Demyelinating disease characterized by multiple lesions disseminated in time & space
        • Acute disseminated encephalomyelitis (ADEM)
          • Acute demyelinating disease with encephalopathy, without NMOSD or anti-MOG-associated antibodies
        • Neuromyelitis optica spectrum disorders (NMOSD)
          • Inflammatory CNS disease caused by antibodies to aquaporin-4 (AQP-4) on astrocytic end feet
          • Clinically characterized by optic neuritis & transverse myelitis
        • Antimyelin oligodendrocyte glycoprotein (MOG) syndromes
          • Acute demyelinating disease caused by antibodies to MOG
          • Extensive clinical overlap with ADEM & NMOSD
        • Lyme disease
          • CNS inflammation associated with Borrelia burgdorferi infection

    IMAGING

    • General Features

      • Imaging Recommendations

        DIFFERENTIAL DIAGNOSIS

          PATHOLOGY

          • General Features

            • Staging, Grading, & Classification

              • Gross Pathologic & Surgical Features

                • Microscopic Features

                  CLINICAL ISSUES

                  • Presentation

                    • Demographics

                      • Natural History & Prognosis

                        • Treatment

                          DIAGNOSTIC CHECKLIST

                          • Image Interpretation Pearls

                            Selected References

                            1. Chhabda S et al: Relapsing demyelinating syndromes in children: a practical review of neuroradiological mimics. Front Neurol. 11:627, 2020
                            2. Padilha IG et al: Pediatric multiple sclerosis: from clinical basis to imaging spectrum and differential diagnosis. Pediatr Radiol. 50(6):776-92, 2020
                            3. Bulut E et al: Brain MRI findings in pediatric-onset neuromyelitis optica spectrum disorder: challenges in differentiation from acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 40(4):726-31, 2019
                            4. Galardi MM et al: Differential diagnosis of pediatric multiple sclerosis. Children (Basel). 6(6), 2019
                            5. Lana-Peixoto MA et al: Neuromyelitis optica spectrum disorder and anti-MOG syndromes. Biomedicines. 7(2), 2019
                            6. Troxell RM et al: Atypical pediatric demyelinating diseases of the central nervous system. Curr Neurol Neurosci Rep. 19(12):95, 2019
                            7. Reich DS et al: Multiple sclerosis. N Engl J Med. 378(2):169-80, 2018
                            8. Berzero G et al: Diagnosis and therapy of acute disseminated encephalomyelitis and its variants. Expert Rev Neurother. 16(1):83-101, 2016
                            9. Faguy K: Multiple sclerosis: an update. Radiol Technol. 87(5):529-50, 2016
                            10. Filippi M et al: MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines. Lancet Neurol. 15(3):292-303, 2016
                            11. Borchers AT et al: Lyme disease: a rigorous review of diagnostic criteria and treatment. J Autoimmun. 57:82-115, 2015
                            12. Koelman DL et al: Acute disseminated encephalomyelitis: current controversies in diagnosis and outcome. J Neurol. 262(9):2013-24, 2015
                            13. Wingerchuk DM et al: International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 85(2):177-89, 2015
                            14. Flanagan EP et al: Neuromyelitis optica spectrum disorders. Curr Neurol Neurosci Rep. 14(9):483, 2014
                            15. Ketelslegers IA et al: A comparison of MRI criteria for diagnosing pediatric ADEM and MS. Neurology. 74(18):1412-5, 2010
                            16. VanLandingham M et al: An uncommon illness with a rare presentation: neurosurgical management of ADEM with tumefactive demyelination in children. Childs Nerv Syst. 26(5):655-61, 2010
                            17. Calabrese M et al: Cortical lesions in primary progressive multiple sclerosis: a 2-year longitudinal MR study. Neurology. 72(15):1330-6, 2009
                            18. Callen DJ et al: Role of MRI in the differentiation of ADEM from MS in children. Neurology. 72(11):968-73, 2009
                            19. Filippi M et al: Conventional MRI in multiple sclerosis. J Neuroimaging. 17 Suppl 1:3S-9S, 2007
                            20. Janardhan V et al: Multiple sclerosis: hyperintense lesions in the brain on nonenhanced T1-weighted MR images evidenced as areas of T1 shortening. Radiology. 244(3):823-31, 2007
                            21. Traboulsee AL et al: The role of MRI in the diagnosis of multiple sclerosis. Adv Neurol. 98:125-46, 2006
                            22. Polman CH et al: Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald Criteria". Ann Neurol. 58(6):840-6, 2005
                            Related Anatomy
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                            Related Differential Diagnoses
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                            References
                            Tables

                            Tables

                            KEY FACTS

                            • Diagnostic Checklist

                              TERMINOLOGY

                              • Definitions

                                • Acquired demyelinating processes characterized by inflammation
                                  • Multiple sclerosis (MS)
                                    • Demyelinating disease characterized by multiple lesions disseminated in time & space
                                  • Acute disseminated encephalomyelitis (ADEM)
                                    • Acute demyelinating disease with encephalopathy, without NMOSD or anti-MOG-associated antibodies
                                  • Neuromyelitis optica spectrum disorders (NMOSD)
                                    • Inflammatory CNS disease caused by antibodies to aquaporin-4 (AQP-4) on astrocytic end feet
                                    • Clinically characterized by optic neuritis & transverse myelitis
                                  • Antimyelin oligodendrocyte glycoprotein (MOG) syndromes
                                    • Acute demyelinating disease caused by antibodies to MOG
                                    • Extensive clinical overlap with ADEM & NMOSD
                                  • Lyme disease
                                    • CNS inflammation associated with Borrelia burgdorferi infection

                              IMAGING

                              • General Features

                                • Imaging Recommendations

                                  DIFFERENTIAL DIAGNOSIS

                                    PATHOLOGY

                                    • General Features

                                      • Staging, Grading, & Classification

                                        • Gross Pathologic & Surgical Features

                                          • Microscopic Features

                                            CLINICAL ISSUES

                                            • Presentation

                                              • Demographics

                                                • Natural History & Prognosis

                                                  • Treatment

                                                    DIAGNOSTIC CHECKLIST

                                                    • Image Interpretation Pearls

                                                      Selected References

                                                      1. Chhabda S et al: Relapsing demyelinating syndromes in children: a practical review of neuroradiological mimics. Front Neurol. 11:627, 2020
                                                      2. Padilha IG et al: Pediatric multiple sclerosis: from clinical basis to imaging spectrum and differential diagnosis. Pediatr Radiol. 50(6):776-92, 2020
                                                      3. Bulut E et al: Brain MRI findings in pediatric-onset neuromyelitis optica spectrum disorder: challenges in differentiation from acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 40(4):726-31, 2019
                                                      4. Galardi MM et al: Differential diagnosis of pediatric multiple sclerosis. Children (Basel). 6(6), 2019
                                                      5. Lana-Peixoto MA et al: Neuromyelitis optica spectrum disorder and anti-MOG syndromes. Biomedicines. 7(2), 2019
                                                      6. Troxell RM et al: Atypical pediatric demyelinating diseases of the central nervous system. Curr Neurol Neurosci Rep. 19(12):95, 2019
                                                      7. Reich DS et al: Multiple sclerosis. N Engl J Med. 378(2):169-80, 2018
                                                      8. Berzero G et al: Diagnosis and therapy of acute disseminated encephalomyelitis and its variants. Expert Rev Neurother. 16(1):83-101, 2016
                                                      9. Faguy K: Multiple sclerosis: an update. Radiol Technol. 87(5):529-50, 2016
                                                      10. Filippi M et al: MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines. Lancet Neurol. 15(3):292-303, 2016
                                                      11. Borchers AT et al: Lyme disease: a rigorous review of diagnostic criteria and treatment. J Autoimmun. 57:82-115, 2015
                                                      12. Koelman DL et al: Acute disseminated encephalomyelitis: current controversies in diagnosis and outcome. J Neurol. 262(9):2013-24, 2015
                                                      13. Wingerchuk DM et al: International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 85(2):177-89, 2015
                                                      14. Flanagan EP et al: Neuromyelitis optica spectrum disorders. Curr Neurol Neurosci Rep. 14(9):483, 2014
                                                      15. Ketelslegers IA et al: A comparison of MRI criteria for diagnosing pediatric ADEM and MS. Neurology. 74(18):1412-5, 2010
                                                      16. VanLandingham M et al: An uncommon illness with a rare presentation: neurosurgical management of ADEM with tumefactive demyelination in children. Childs Nerv Syst. 26(5):655-61, 2010
                                                      17. Calabrese M et al: Cortical lesions in primary progressive multiple sclerosis: a 2-year longitudinal MR study. Neurology. 72(15):1330-6, 2009
                                                      18. Callen DJ et al: Role of MRI in the differentiation of ADEM from MS in children. Neurology. 72(11):968-73, 2009
                                                      19. Filippi M et al: Conventional MRI in multiple sclerosis. J Neuroimaging. 17 Suppl 1:3S-9S, 2007
                                                      20. Janardhan V et al: Multiple sclerosis: hyperintense lesions in the brain on nonenhanced T1-weighted MR images evidenced as areas of T1 shortening. Radiology. 244(3):823-31, 2007
                                                      21. Traboulsee AL et al: The role of MRI in the diagnosis of multiple sclerosis. Adv Neurol. 98:125-46, 2006
                                                      22. Polman CH et al: Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald Criteria". Ann Neurol. 58(6):840-6, 2005