link
Bookmarks
Pediatrics
Diagnosis
Brain
Metabolic, Infectious, and Inflammatory Disorders
Demyelinating Diseases
Demyelinating Diseases
Blaise V. Jones, MD
To access 4,300 diagnoses written by the world's leading experts in radiology.Try it free - 15 days
0
2
3
0

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
                            Loading...
                            Related Differential Diagnoses
                            Loading...
                            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