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Brain
Diagnosis
Pathology-Based Diagnoses
Infectious, Inflammatory, and Demyelinating Disease
Inflammatory and Demyelinating Disease
ADEM
ADEM
Kevin R. Moore, MD; Miral D. Jhaveri, MD, MBA; Surjith Vattoth, MD, FRCR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Acute disseminated encephalomyelitis (ADEM)

              • Definitions

                • Autoimmune-mediated white matter (WM) demyelination of brain &/or spinal cord, usually with remyelination

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      • Nuclear Medicine Findings

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Carvalho K et al: Acute disseminated encephalomyelitis (ADEM) associated with mosquito-borne diseases: Chikungunya virus X yellow fever immunization. Rev Soc Bras Med Trop. 53:e20190160, 2020
                                          2. Molero-Senosiain M et al: Neuro-ophthalmological manifestations as complication of an infection with Mycoplasma pneumoniae and subsequent development of disseminated acute encephalitis. Arch Soc Esp Oftalmol. 95(5(:254-8, 2020
                                          3. Stokes Brackett AC et al: Multiphasic acute disseminated encephalomyelitis and differential with early onset multiple sclerosis. Intractable Rare Dis Res. 9(1):61-3, 2020
                                          4. 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
                                          5. Aubert-Broche B et al: Monophasic demyelination reduces brain growth in children. Neurology. 88(18):1744-50, 2017
                                          6. Bester M et al: Neuroimaging of multiple sclerosis, acute disseminated encephalomyelitis, and other demyelinating diseases. Semin Roentgenol. 49(1):76-85, 2014
                                          7. Longoni G et al: White matter changes in paediatric multiple sclerosis and monophasic demyelinating disorders. Brain. 140(5):1300-15, 2017
                                          8. Koelman DLH et al: Acute disseminated encephalomyelitis: prognostic value of early follow-up brain MRI. J Neurol. 264(8):1754-62, 2017
                                          9. Marziali S et al: Acute disseminated encephalomyelitis following Campylobacter jejuni gastroenteritis: Case report and review of the literature. Neuroradiol J. 30(1):65-70, 2017
                                          10. Kanekar S et al: A pattern approach to focal white matter hyperintensities on magnetic resonance imaging. Radiol Clin North Am. 52(2):241-61, 2014
                                          11. Daida K et al: Cytomegalovirus-associated encephalomyelitis in an immunocompetent adult: a two-stage attack of direct viral and delayed immune-mediated invasions. case report. BMC Neurol. 16(1):223, 2016
                                          12. Pohl D et al: Acute disseminated encephalomyelitis: updates on an inflammatory CNS syndrome. Neurology. 87(9 Suppl 2):S38-45, 2016
                                          13. Yuan JL et al: Acute Disseminated Encephalomyelitis following Vaccination against Hepatitis B in a Child: A Case Report and Literature Review. Case Rep Neurol Med. 2016:2401809, 2016
                                          14. Baumann M et al: Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein. J Neurol Neurosurg Psychiatry. 86(3):265-72, 2015
                                          15. Karussis D: The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review. J Autoimmun. 48-49:134-42, 2014
                                          16. Mariotto S et al: Clinical spectrum and IgG subclass analysis of anti-myelin oligodendrocyte glycoprotein antibody-associated syndromes: a multicenter study. J Neurol. 264(12):2420-30, 2017
                                          17. Nakamura Y et al: Anti-MOG antibody-positive ADEM following infectious mononucleosis due to a primary EBV infection: a case report. BMC Neurol. 17(1):76, 2017
                                          18. Tenembaum SN: Acute disseminated encephalomyelitis. Handb Clin Neurol. 112:1253-62, 2013
                                          19. Wingerchuk DM et al: Acute disseminated encephalomyelitis, transverse myelitis, and neuromyelitis optica. Continuum (Minneap Minn). 19(4 Multiple Sclerosis):944-67, 2013
                                          20. Callen DJ et al: Role of MRI in the differentiation of ADEM from MS in children. Neurology. 72(11):968-73, 2009
                                          21. Noorbakhsh F et al: Acute disseminated encephalomyelitis: clinical and pathogenesis features. Neurol Clin. 26(3):759-80, ix, 2008
                                          22. Rossi A: Imaging of acute disseminated encephalomyelitis. Neuroimaging Clin N Am. 18(1):149-61; ix, 2008
                                          23. Tenembaum S et al: Acute disseminated encephalomyelitis. Neurology. 68(16 Suppl 2):S23-36, 2007
                                          24. Menge T et al: Acute disseminated encephalomyelitis: an update. Arch Neurol. 62(11):1673-80, 2005
                                          25. Yeh EA et al: Detection of coronavirus in the central nervous system of a child with acute disseminated encephalomyelitis. Pediatrics. 113(1 Pt 1):e73-6, 2004
                                          26. Dale RC: Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis. 14(2):90-5, 2003
                                          27. Garg RK: Acute disseminated encephalomyelitis. Postgrad Med J. 79(927):11-17, 2003
                                          28. Idrissova ZhR et al: Acute disseminated encephalomyelitis in children: clinical features and HLA-DR linkage. Eur J Neurol. 10(5):537-46, 2003
                                          29. Okamoto K et al: MR features of diseases involving bilateral middle cerebellar peduncles. AJNR Am J Neuroradiol. 24(10):1946-54, 2003
                                          30. Sener RN: Neuro-Behcet's disease: diffusion MR imaging and proton MR spectroscopy. AJNR Am J Neuroradiol. 24(8):1612-4, 2003
                                          31. Stonehouse M et al: Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians. Arch Dis Child. 88(2):122-4, 2003
                                          32. Inglese M et al: Magnetization transfer and diffusion tensor MR imaging of acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 23(2):267-72, 2002
                                          33. Murthy JM: Acute disseminated encephalomyelitis. Neurol India. 50(3):238-43, 2002
                                          34. Tenembaum S et al: Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology. 59(8):1224-31, 2002
                                          35. Bizzi A et al: Quantitative proton MR spectroscopic imaging in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 22(6):1125-30, 2001
                                          36. Honkaniemi J et al: Delayed MR imaging changes in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 22(6):1117-24, 2001
                                          37. Straussberg R et al: Improvement of atypical acute disseminated encephalomyelitis with steroids and intravenous immunoglobulins. Pediatr Neurol. 24(2):139-43, 2001
                                          38. Dale RC et al: Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain. 123 Pt 12:2407-22, 2000
                                          39. Rust RS: Multiple sclerosis, acute disseminated encephalomyelitis, and related conditions. Semin Pediatr Neurol. 7(2):66-90, 2000
                                          40. Schaefer PW et al: Diffusion-weighted MR imaging of the brain. Radiology. 217(2):331-45, 2000
                                          41. Kocer N et al: CNS involvement in neuro-Behcet syndrome: an MR study. AJNR Am J Neuroradiol. 20(6):1015-24, 1999
                                          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

                                                        • Acute disseminated encephalomyelitis (ADEM)

                                                      • Definitions

                                                        • Autoimmune-mediated white matter (WM) demyelination of brain &/or spinal cord, usually with remyelination

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR Findings

                                                            • Imaging Recommendations

                                                              • Nuclear Medicine Findings

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Carvalho K et al: Acute disseminated encephalomyelitis (ADEM) associated with mosquito-borne diseases: Chikungunya virus X yellow fever immunization. Rev Soc Bras Med Trop. 53:e20190160, 2020
                                                                                  2. Molero-Senosiain M et al: Neuro-ophthalmological manifestations as complication of an infection with Mycoplasma pneumoniae and subsequent development of disseminated acute encephalitis. Arch Soc Esp Oftalmol. 95(5(:254-8, 2020
                                                                                  3. Stokes Brackett AC et al: Multiphasic acute disseminated encephalomyelitis and differential with early onset multiple sclerosis. Intractable Rare Dis Res. 9(1):61-3, 2020
                                                                                  4. 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
                                                                                  5. Aubert-Broche B et al: Monophasic demyelination reduces brain growth in children. Neurology. 88(18):1744-50, 2017
                                                                                  6. Bester M et al: Neuroimaging of multiple sclerosis, acute disseminated encephalomyelitis, and other demyelinating diseases. Semin Roentgenol. 49(1):76-85, 2014
                                                                                  7. Longoni G et al: White matter changes in paediatric multiple sclerosis and monophasic demyelinating disorders. Brain. 140(5):1300-15, 2017
                                                                                  8. Koelman DLH et al: Acute disseminated encephalomyelitis: prognostic value of early follow-up brain MRI. J Neurol. 264(8):1754-62, 2017
                                                                                  9. Marziali S et al: Acute disseminated encephalomyelitis following Campylobacter jejuni gastroenteritis: Case report and review of the literature. Neuroradiol J. 30(1):65-70, 2017
                                                                                  10. Kanekar S et al: A pattern approach to focal white matter hyperintensities on magnetic resonance imaging. Radiol Clin North Am. 52(2):241-61, 2014
                                                                                  11. Daida K et al: Cytomegalovirus-associated encephalomyelitis in an immunocompetent adult: a two-stage attack of direct viral and delayed immune-mediated invasions. case report. BMC Neurol. 16(1):223, 2016
                                                                                  12. Pohl D et al: Acute disseminated encephalomyelitis: updates on an inflammatory CNS syndrome. Neurology. 87(9 Suppl 2):S38-45, 2016
                                                                                  13. Yuan JL et al: Acute Disseminated Encephalomyelitis following Vaccination against Hepatitis B in a Child: A Case Report and Literature Review. Case Rep Neurol Med. 2016:2401809, 2016
                                                                                  14. Baumann M et al: Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein. J Neurol Neurosurg Psychiatry. 86(3):265-72, 2015
                                                                                  15. Karussis D: The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review. J Autoimmun. 48-49:134-42, 2014
                                                                                  16. Mariotto S et al: Clinical spectrum and IgG subclass analysis of anti-myelin oligodendrocyte glycoprotein antibody-associated syndromes: a multicenter study. J Neurol. 264(12):2420-30, 2017
                                                                                  17. Nakamura Y et al: Anti-MOG antibody-positive ADEM following infectious mononucleosis due to a primary EBV infection: a case report. BMC Neurol. 17(1):76, 2017
                                                                                  18. Tenembaum SN: Acute disseminated encephalomyelitis. Handb Clin Neurol. 112:1253-62, 2013
                                                                                  19. Wingerchuk DM et al: Acute disseminated encephalomyelitis, transverse myelitis, and neuromyelitis optica. Continuum (Minneap Minn). 19(4 Multiple Sclerosis):944-67, 2013
                                                                                  20. Callen DJ et al: Role of MRI in the differentiation of ADEM from MS in children. Neurology. 72(11):968-73, 2009
                                                                                  21. Noorbakhsh F et al: Acute disseminated encephalomyelitis: clinical and pathogenesis features. Neurol Clin. 26(3):759-80, ix, 2008
                                                                                  22. Rossi A: Imaging of acute disseminated encephalomyelitis. Neuroimaging Clin N Am. 18(1):149-61; ix, 2008
                                                                                  23. Tenembaum S et al: Acute disseminated encephalomyelitis. Neurology. 68(16 Suppl 2):S23-36, 2007
                                                                                  24. Menge T et al: Acute disseminated encephalomyelitis: an update. Arch Neurol. 62(11):1673-80, 2005
                                                                                  25. Yeh EA et al: Detection of coronavirus in the central nervous system of a child with acute disseminated encephalomyelitis. Pediatrics. 113(1 Pt 1):e73-6, 2004
                                                                                  26. Dale RC: Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis. 14(2):90-5, 2003
                                                                                  27. Garg RK: Acute disseminated encephalomyelitis. Postgrad Med J. 79(927):11-17, 2003
                                                                                  28. Idrissova ZhR et al: Acute disseminated encephalomyelitis in children: clinical features and HLA-DR linkage. Eur J Neurol. 10(5):537-46, 2003
                                                                                  29. Okamoto K et al: MR features of diseases involving bilateral middle cerebellar peduncles. AJNR Am J Neuroradiol. 24(10):1946-54, 2003
                                                                                  30. Sener RN: Neuro-Behcet's disease: diffusion MR imaging and proton MR spectroscopy. AJNR Am J Neuroradiol. 24(8):1612-4, 2003
                                                                                  31. Stonehouse M et al: Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians. Arch Dis Child. 88(2):122-4, 2003
                                                                                  32. Inglese M et al: Magnetization transfer and diffusion tensor MR imaging of acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 23(2):267-72, 2002
                                                                                  33. Murthy JM: Acute disseminated encephalomyelitis. Neurol India. 50(3):238-43, 2002
                                                                                  34. Tenembaum S et al: Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology. 59(8):1224-31, 2002
                                                                                  35. Bizzi A et al: Quantitative proton MR spectroscopic imaging in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 22(6):1125-30, 2001
                                                                                  36. Honkaniemi J et al: Delayed MR imaging changes in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. 22(6):1117-24, 2001
                                                                                  37. Straussberg R et al: Improvement of atypical acute disseminated encephalomyelitis with steroids and intravenous immunoglobulins. Pediatr Neurol. 24(2):139-43, 2001
                                                                                  38. Dale RC et al: Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain. 123 Pt 12:2407-22, 2000
                                                                                  39. Rust RS: Multiple sclerosis, acute disseminated encephalomyelitis, and related conditions. Semin Pediatr Neurol. 7(2):66-90, 2000
                                                                                  40. Schaefer PW et al: Diffusion-weighted MR imaging of the brain. Radiology. 217(2):331-45, 2000
                                                                                  41. Kocer N et al: CNS involvement in neuro-Behcet syndrome: an MR study. AJNR Am J Neuroradiol. 20(6):1015-24, 1999