Acuted Disseminated Encephalomyelitis

  • Joy B. ParrishEmail author
  • E. Ann Yeh
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 724)


Acute disseminated encephalomyelitis (ADEM) is a disorder of the central nervous system (CNS) characterized by an acute event, typically with encephalopathy, in which diffuse CNS involvement occurs. It may follow an infectious event and occurs more commonly in young children. Pulse steroid treatment is frequently used to treat ADEM. Although ADEM is typically described as a benign condition, with children generally recovering motor function and resolution of lesions on magnetic resonance imaging (MRI), residual cognitive deficits may occur. This chapter aims to review the clinical features, typical presentation, differential diagnosis, treatment and prognosis of ADEM.


Multiple Sclerosis Neurodegenerative Disease Optic Neuritis Borrelia Burgdorferi Therapeutic Plasma Exchange 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Schwarz S, Mohr A, Knauth M et al. Acute disseminated encephalomyelitis: A follow-up study of 40 adult patients. Neurology 2001; 56:1313–1318.PubMedGoogle Scholar
  2. 2.
    Tenembaum S, Cahamoles N, Fejerman N. Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology 2002; 59(8):1224–1231.PubMedGoogle Scholar
  3. 3.
    Krupp LB, Banwell B, Tenembaum S. Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology 2007; 68(16 Suppl 2):S7–S12.PubMedCrossRefGoogle Scholar
  4. 4.
    McKeon A, Lennon VA, Lotze T et al. CNS aquaporin-4 autoimmunity in children. Neurology 2008; 71(2):93–100.PubMedCrossRefGoogle Scholar
  5. 5.
    Alper G, Heyman R, Wang L. Multiple sclerosis and acute disseminated encephalomyelitis diagnosed in children after long-term follow-up: Comparison of presenting features. Developmental Medicine and Child Neurology 2009; 51:480–486.PubMedCrossRefGoogle Scholar
  6. 6.
    Pohl D, Rostasy K, Reiber H, Hanefeld F. CSF characteristics in early-onset multiple sclerosis. Neurology 2004; 63(10):1966–1967.PubMedGoogle Scholar
  7. 7.
    Dale RC, de Sousa C, Chong WK et al. Acute disseminated encephalomylitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain 2000; 12:2407–2422.CrossRefGoogle Scholar
  8. 8.
    Anlar B, Basaran C, Kose G et al. Acute disseminated encephalomyelitis in children: outcome and prognosis. Neuropediatrics 2003; 34(4):194–199.PubMedCrossRefGoogle Scholar
  9. 9.
    Leake JA, Albani S, Kao AS et al. Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr Infect Dis J 2004; 23(8):756–764.PubMedCrossRefGoogle Scholar
  10. 10.
    Mikaeloff Y, Caridade G, Husson B et al. Acute disseminated encephalomyelitis cohort study: prognostic factors for relapse. Eur J Paediatr Neurol 2007; 11(2):90–95.PubMedCrossRefGoogle Scholar
  11. 11.
    Olgac Dundar N, Anlar B, Guven A et al. Relapsing Acute Disseminated Encephalomyelitis in Children: Further Evaluation of the Diagnosis. J Child Neurol 2010; [Epub ahead of print]Google Scholar
  12. 12.
    Banwell B, Kennedy J, Sadovnick D et al. Incidence of acquired demyelination of the CNS in Canadian children. Neurology 2009; 72(3):232–239.PubMedCrossRefGoogle Scholar
  13. 13.
    Tenembaum SN. Disseminated encephalomyelitis in children. Clin Neurol Neurosurg 2008; 110(9):928–938.PubMedCrossRefGoogle Scholar
  14. 14.
    Torisu H, Kira R, Ishizaki Y et al. Clinical study of childhood acute disseminated encephalomyelitis, multiple sclerosis and acute transverse myelitis in Fukuoka Prefecture, Japan. Brain and Development 2010; 32:454–462.PubMedCrossRefGoogle Scholar
  15. 15.
    Tenembaum S, Chitnis T, Ness J et al. Acute disseminated encephalomyelitis. Neurology 2007; 68(16 Suppl 2):S23–S36.PubMedCrossRefGoogle Scholar
  16. 16.
    Tenembaum S. Disseminated encephalomyelitis in children. Clinical Neurology and Neurosurgery 2008; 110:928–938.PubMedCrossRefGoogle Scholar
  17. 17.
    Atzori M, Battistella PA, Perini P et al. Clinical and diagnostic aspects of multiple sclerosis and acute monophasic encephalomyelitis in pediatric patients: A single centre prospective study. Mult Sclerosis 2009; 15:363–370.CrossRefGoogle Scholar
  18. 18.
    Murthy KSN, Faden HS, Cohen ME et al. Acute disseminated encephalomyelitis in children. Pediatrics 2002; 110(2).Google Scholar
  19. 19.
    Hynson JL, Kornberg AJ, Coleman LT et al. Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology 2001; 56(10):1308–1312.PubMedGoogle Scholar
  20. 20.
    Gupte G, Stonehouse M, Wassmer E et al. Acute disseminated encephalomyelitis: a review of 18 cases in childhood. J Paediatr Child Health 2003; 39(5):336–342.PubMedCrossRefGoogle Scholar
  21. 21.
    Mikaeloff Y, Suissa S, Vallee L et al. First episode of acute CNS inflammatory demyelination in childhood: prognostic factors for multiple sclerosis and disability. J Pediatr 2004; 144(2):246–252.PubMedCrossRefGoogle Scholar
  22. 22.
    Schwarz S, Mohr A, Knauth M et al. Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients. Neurology 2001; 56(10):1313–1318.PubMedGoogle Scholar
  23. 23.
    Marchioni E, Tavazzi E, Minoli L et al. Acute disseminated encephalomyelitis. Neurol Sci 2008; 29:S286–S288.PubMedCrossRefGoogle Scholar
  24. 24.
    Sonneville R, Demeret S, Klein I et al. Acute disseminated encephalomyelitisin the intensive care unit:clinical features and outcome of 20 adults. Intensive Care Med 2008; 34(3):528–532.PubMedCrossRefGoogle Scholar
  25. 25.
    Sonneville R, Klein I, de Broucker T et al. Post-infectious encephalitis in adults: diagnosis and management. J Infect 2009; 58(5):321–328.PubMedCrossRefGoogle Scholar
  26. 26.
    Axer H, Ragoschke-Schumm A, Bottcher J et al. Initial DWI and ADC imaging may predict outcome in acute disseminated encephalomyelitis: report of two cases of brain stem encephalitis. J Neurol Neurosurg Psychiatry 2005; 76(7):996–998.PubMedCrossRefGoogle Scholar
  27. 27.
    Menge T, Kiesseier BC, Nessler S et al. Acute disseminated encephalomyelitis: an acute hit against the brain. Curr Opin Neurol 2007; 20:247–254.PubMedCrossRefGoogle Scholar
  28. 28.
    Booss J, Davis LE. Smallpox and smallpox vaccination: neurological implications. Neurology 2003; 60(8):1241–1245.PubMedGoogle Scholar
  29. 29.
    Sejvar JJ, Labutta RJ, Chapman LE et al. Neurologic adverse events associated with smallpox vaccination in the United States, 2002-2004. JAMA 2005; 294(21):2744–2750.PubMedCrossRefGoogle Scholar
  30. 30.
    Bennetto L, Scolding N. Inflammatory/post-infectious encephalomyelitis. J Neurol Neurosurg Psychiatry 2004; (75 Suppl 1):i22–i28.Google Scholar
  31. 31.
    Fenichel GM. Neurological complications of immunization. Ann Neurol 1982; 12(2):119–128.PubMedCrossRefGoogle Scholar
  32. 32.
    Nalin DR. Mumps, measles and rubella vaccination and encephalitis. BMJ 1989; 299(6709):1219.PubMedCrossRefGoogle Scholar
  33. 33.
    Plesner AM, Arlien-Soborg P, Herning M. Neurological complications to vaccination against Japanese encephalitis. Eur J Neurol 1998; 5(5):479–485.PubMedCrossRefGoogle Scholar
  34. 34.
    Cheong JH, Bak KH, Kim CH et al. Acute disseminated encephalomyelitis associated with influenza vaccination. J Korean Neurosurg Soc 2004; 35:223–225.Google Scholar
  35. 35.
    Izurieta HS, Haber P, Wise RP et al. Adverse events reported following live, cold-adapted, intranasal influenza vaccine. JAMA 2005; 294(21):2720–2725.PubMedCrossRefGoogle Scholar
  36. 36.
    Dodick DW, Silber MH, Noseworthy JH et al. Acute disseminated encephalomyelitis after accidental injection of a hog vaccine: successful treatment with plasmapheresis. Mayo Clin Proc 1998; 73(12):1193–1195.PubMedCrossRefGoogle Scholar
  37. 37.
    Sonmez FM, Odemis E, Ahmetoglu A et al. Brainstem encephalitis and acute disseminated encephalomyelitis following mumps. Pediatr Neurol 2004; 30(2):132–134.PubMedCrossRefGoogle Scholar
  38. 38.
    Miller DH, Kay R, Schon F et al. Optic neuritis following chickenpox in adults. J Neurol 1986; 233(3):182–184.PubMedCrossRefGoogle Scholar
  39. 39.
    Fujimoto H, Asaoka K, Imaizumi T et al. Epstein-Barr virus infections of the central nervous system. Intern Med 2003; 42(1):33–40.PubMedCrossRefGoogle Scholar
  40. 40.
    Revel-Vilk S, Hurvitz H, Klar A et al. Recurrent acute disseminated encephalomyelitis associated with acute cytomegalovirus and Epstein-Barr virus infection. J Child Neurol 2000; 15(6):421–424.PubMedCrossRefGoogle Scholar
  41. 41.
    Kaji M, Kusuhara T, Ayabe M et al. Survey of herpes simplex virus infections of the central nervous system, including acute disseminated encephalomyelitis, in the Kyushu and Okinawa regions of Japan. Mult Scler 1996; 2(2):83–87.PubMedGoogle Scholar
  42. 42.
    Sacconi S, Salviati L, Merelli E. Acute disseminated encephalomyelitis associated with hepatitis C virus infection. Arch Neurol 2001; 58(10):1679–1681.PubMedCrossRefGoogle Scholar
  43. 43.
    Tan H, Kilicaslan B, Onbas O et al. Acute disseminated encephalomyelitis following hepatitis A virus infection. Pediatr Neurol 2004; 30(3):207–209.PubMedCrossRefGoogle Scholar
  44. 44.
    David P, Baleriaux D, Bank WO et al. MRI of acute disseminated encephalomyelitis after coxsackie B infection. J Neuroradiol 1993; 20(4):258–265.PubMedGoogle Scholar
  45. 45.
    Wang YH, Huang YC, Chang LY et al. Clinical characteristics of children with influenza A virus infection requiring hospitalization. J Microbiol Immunol Infect 2003; 36(2):111–116.PubMedGoogle Scholar
  46. 46.
    Rellosa N, Bloch KC, Shane AL et al. Neurologic Manifestations of Pediatric Novel H1n1 Influenza Infection. Pediatr Infect Dis J.Google Scholar
  47. 47.
    Silver B, McAvoy K, Mikesell S et al. Fulminating encephalopathy with perivenular demyelination and vacuolar myelopathy as the initial presentation of human immunodeficiency virus infection. Arch Neurol 1997; 54(5):647–650.PubMedCrossRefGoogle Scholar
  48. 48.
    Tachi N, Watanabe T, Wakai S et al. Acute disseminated encephalomyelitis following HTLV-I associated myelopathy. J Neurol Sci 1992; 110(1-2):234–235.PubMedCrossRefGoogle Scholar
  49. 49.
    Kamei A, Ichinohe S, Onuma R et al. Acute disseminated demyelination due to primary human herpesvirus-6 infection. Eur J Pediatr 1997; 156(9):709–712.PubMedCrossRefGoogle Scholar
  50. 50.
    Wei TY, Baumann RJ. Acute disseminated encephalomyelitis after Rocky Mountain spotted fever. Pediatr Neurol 1999; 21(1):503–505.PubMedCrossRefGoogle Scholar
  51. 51.
    Yeh EA, Collins A, Cohen ME et al. Detection of coronavirus in the central nervous system of a child with acute disseminated encephalomyelitis. Pediatrics 2004; 113(1 Pt 1):e73–e76.PubMedGoogle Scholar
  52. 52.
    Riedel K, Kempf VA, Bechtold A et al. Acute disseminated encephalomyelitis (ADEM) due to Mycoplasma pneumoniae infection in an adolescent. Infection 2001; 29(4):240–242.PubMedCrossRefGoogle Scholar
  53. 53.
    van Assen S, Bosma F, Staals LM et al. Acute disseminated encephalomyelitis associated with Borrelia burgdorferi. J Neurol 2004; 251(5):626–629.PubMedCrossRefGoogle Scholar
  54. 54.
    Nasralla CA, Pay N, Goodpasture HC et al. Postinfectious encephalopathy in a child following Campylobacter jejuni enteritis. AJNR Am J Neuroradiol 1993; 14(2):444–448.PubMedGoogle Scholar
  55. 55.
    Lelis SS, Fonseca LF, Xavier CC et al. Acute disseminated encephalomyelitis after leptospirosis. Pediatric Neurology 2009; 40(6):471–473.PubMedCrossRefGoogle Scholar
  56. 56.
    Chandra SR, Kalpana D, Anilkumar TV et al. Acute disseminated encephalomyelitis following leptospirosis. J Assoc Physicians India 2004; 52:327–329.PubMedGoogle Scholar
  57. 57.
    Heick A, Skriver E. Chlamydia pneumoniae-associated ADEM. Eur J Neurol 2000; 7(4):435–438.PubMedCrossRefGoogle Scholar
  58. 58.
    Spieker S, Petersen D, Rolfs A et al. Acute disseminated encephalomyelitis following Pontiac fever. Eur Neurol 1998; 40(3):169–172.PubMedCrossRefGoogle Scholar
  59. 59.
    Dale RC, Church AJ, Cardoso F et al. Poststreptococcal acute disseminated encephalomyelitis with basal ganglia involvement and auto-reactive antibasal ganglia antibodies. Ann Neurol 2001; 50(5):588–595.PubMedCrossRefGoogle Scholar
  60. 60.
    Huynh W, Cordato DJ, Kehdi E et al. Post-vaccination encephalomyelitis: Literature review and illustrative case. Journal of Clinical Neuroscience 2008; 15:1315–1322.PubMedCrossRefGoogle Scholar
  61. 61.
    Au WY, Lie AK, Cheung RT et al. Acute disseminated encephalomyelitis after para-influenza infection post bone marrow transplantation. Leuk Lymphoma 2002; 43(2):455–457.PubMedCrossRefGoogle Scholar
  62. 62.
    Horowitz MB, Comey C, Hirsch W et al. Acute disseminated encephalomyelitis (ADEM) or ADEM-like inflammatory changes in a heart-lung transplant recipient: a case report. Neuroradiology 1995; 37(6):434–437.PubMedCrossRefGoogle Scholar
  63. 63.
    Re A, Giachetti R. Acute disseminated encephalomyelitis (ADEM) after autologous peripheral blood stem cell transplant for non-Hodgkin’s lymphoma. Bone Marrow Transplant 1999; 24(12):1351–1354.PubMedCrossRefGoogle Scholar
  64. 64.
    Tomonari A, Tojo A, Adachi D et al. Acute disseminated encephalomyelitis (ADEM) after allogeneic bone marrow transplantation for acute myeloid leukemia. Ann Hematol 2003; 82(1):37–40.PubMedGoogle Scholar
  65. 65.
    Jaster JH, Niell HB, Dohan FC, Jr et al. Demyelination in the brain as a paraneoplastic disorder: candidates include some cases of leukemia and non-Hodgkin’s lymphoma. Ann Hematol 2003; 82(11):714–715.PubMedCrossRefGoogle Scholar
  66. 66.
    Mikaeloff Y, Caridade G, Suissa S et al. Hepatitis B vaccine and the risk of CNS inflammatory demyelination in childhood. Neurology 2008; 72(10):873–80PubMedCrossRefGoogle Scholar
  67. 67.
    Belman A, Chabas D, Chitnis T et al. Clinical Spectrum of Disorders Masquerading as Pediatric Multiple Sclerosis. Annals of Neurology 2007; Supplement: Abstracts CNS.Google Scholar
  68. 68.
    van der Knaap MS, Salomons GS, Li R et al. Unusual variants of Alexander’s disease. Ann Neurol 2005; 57(3):327–338.PubMedCrossRefGoogle Scholar
  69. 69.
    Harris MO, Walsh LE, Hattab EM et al. Is it ADEM, POLG, or both? Arch Neurol 67(4):493–496.Google Scholar
  70. 70.
    Gorman MP, Golomb MR, Walsh LE et al. Steroid-responsive neurologic relapses in a child with a proteolipid protein-1 mutation. Neurology 2007; 68(16):1305–1307.PubMedCrossRefGoogle Scholar
  71. 71.
    Weinstock A, Giglio P, Cohen ME et al. Diffuse magnetic resonance imaging white-matter changes in a 15-year-old boy with mitochondrial encephalomyopathy. J Child Neurol 2002; 17(1):47–49.PubMedCrossRefGoogle Scholar
  72. 72.
    van der Knaap MS, Valk J, de Neeling N et al. Pattern recognition in magnetic resonance imaging of white matter disorders in children and young adults. Neuroradiology 1991; 33(6):478–493.PubMedCrossRefGoogle Scholar
  73. 73.
    Lotze TE, Northrop JL, Hutton GJ et al. Spectrum of pediatric neuromyelitis optica. Pediatrics 2008; 122(5):e1039–e1047.PubMedCrossRefGoogle Scholar
  74. 74.
    Mikaeloff Y, Adamsbaum C, Husson B et al. MRI prognostic factors for relapse after acute CNS inflammatory demyelination in childhood. Brain 2004; 127(Pt 9):1942–1947.PubMedCrossRefGoogle Scholar
  75. 75.
    Parrish JB, Weinstock-Guttman B, Yeh EA. Cerebellar mutism in pediatric acute disseminated encephalomyelitis. Pediatr Neurol 2010; 42(4):259–266.PubMedCrossRefGoogle Scholar
  76. 76.
    Neuteboom RF, Boon M, Catsman Berrevoets CE et al. Prognostic factors after a first attack of inflammatory CNS demyelination in children. Neurology 2008; 71(13):967–973.PubMedCrossRefGoogle Scholar
  77. 77.
    Callen DJ, Shroff MM, Branson HM et al. Role of MRI in the differentiation of ADEM from MS in children. Neurology 2008.Google Scholar
  78. 78.
    Ketelslegers IA, Neuteboom RF, Boon M et al. A comparison of MRI criteria for diagnosing pediatric ADEM and MS. Neurology 2010; 74(18):1412–5PubMedCrossRefGoogle Scholar
  79. 79.
    Chabas D, Ness J, Belman A et al. Younger children with pediatric MS have a distinct CSF inflammatory profile at disease onset. Neurology 2010; 74(5):399–405.PubMedCrossRefGoogle Scholar
  80. 80.
    Selter RC, Brilot F, Grummel V et al. Antibody responses to EBV and native MOG in pediatric inflammatory demyelinating CNS diseases. Neurology 2010; 74(21):1711–1715.PubMedCrossRefGoogle Scholar
  81. 81.
    Banwell B, Tenembaum S, Lennon VA et al. Neuromyelitis optica-IgG in childhood inflammatory demyelinating CNS disorders. Neurology 2008; 70(5):344–352.PubMedCrossRefGoogle Scholar
  82. 82.
    Kotlus BS, Slavin ML, Guthrie DS et al. Ophthalmologic manifestations in pediatric patients with acute disseminated encephalomyelitis. J AAPOS 2005; 9(2):179–183.PubMedCrossRefGoogle Scholar
  83. 83.
    Simone IL, Carrara D, Tortorella C et al. Course and prognosis in early-onset MS: comparison with adult-onset forms. Neurology 2002; 59(12):1922–1928.PubMedGoogle Scholar
  84. 84.
    Pohl D, Rostasy K, Treiber-Held S et al. Pediatric multiple sclerosis: detection of clinically silent lesions by multimodal evoked potentials. J Pediatr 2006; 149(1):125–127.PubMedCrossRefGoogle Scholar
  85. 85.
    Frohman E, Costello F, Zivadinov R et al. Optical coherence tomography in multiple sclerosis. Lancet Neurol 2006; 5(10):853–863.PubMedCrossRefGoogle Scholar
  86. 86.
    Yeh E, Weinstock-Guttman B, Lincoff N et al. Retinal nerve fiber thickness in inflammatory demyelinating diseases of childhood onset. Mult Scler 2009; 15(7):802–810.PubMedCrossRefGoogle Scholar
  87. 87.
    Hollinger P, Sturzenegger M, Mathis J et al. Acute disseminated encephalomyelitis in adults: a reappraisal of clinical, CSF, EEG and MRI findings. J Neurol 2001; 249:320–329.Google Scholar
  88. 88.
    Shahar E, Andraus J, Savitzki D et al. Outcome of severe encephalomyelitis in children: effect of high-dose methylprednisolone and immunoglobulins. J Child Neurol 2002; 17(11):810–814.PubMedCrossRefGoogle Scholar
  89. 89.
    Khurana DS, Melvin JJ, Kothare SV et al. Acute disseminated encephalomyelitis in children: discordant neurologic and neuroimaging abnormalities and response to plasmapheresis. Pediatrics 2005; 116(2):431–436.PubMedCrossRefGoogle Scholar
  90. 90.
    Ravaglia S, Piccolo G, Ceroni M et al. Severe steroid-resistant post-infectious encephalomyelitis: general features and effects of IVIg. J Neurol 2007; 254(11):1518–1523.PubMedCrossRefGoogle Scholar
  91. 91.
    Straussberg R, Schonfeld T, Weitz R et al. Improvement of atypical acute disseminated encephalomyelitis with steroids and intravenous immunoglobulins. Pediatr Neurol 2001; 24(2):139–143.PubMedCrossRefGoogle Scholar
  92. 92.
    Kaynar L, Altuntas F, Aydogdu I et al. Therapeutic plasma exchange in patients with neurologic diseases: retrospective multicenter study. Transfus Apher Sci 2008; 38(2):109–115.PubMedCrossRefGoogle Scholar
  93. 93.
    Suppiej A, Vittorini R, Fontanin M et al. Acute disseminated encephalomyelitis in chidlren: Focus on Relapsing Patients. Pediatr Neurol 2008; 39:12–17.PubMedCrossRefGoogle Scholar
  94. 94.
    Tenembaum S, Chitnis T, Ness J et al. Acute disseminated encephalomyelitis. Neurology 2007; 68(Suppl 2):S23–S36.PubMedCrossRefGoogle Scholar
  95. 95.
    Jacobs RK, Anderson VA, Neale JL et al. Neuropsychological outcome after acute disseminated encephalomyelitis: impact of age at illness onset. Pediatr Neurol 2004; 31(3):191–197.PubMedCrossRefGoogle Scholar
  96. 96.
    Deery B, Anderson V, Jacobs R et al. Childhood MS and ADEM: Investigation and comparison of neurocognitive features in children. Developmental Neuropsychology 2010; 35(5):506–521.PubMedCrossRefGoogle Scholar
  97. 97.
    Hahn CD, Miles BS, MacGregor DL et al. Neurocognitive outcome after acute disseminated encephalomyelitis. Pediatr Neurol 2003; 29:117–123.PubMedCrossRefGoogle Scholar

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© Landes Bioscience and Springer Science+Business Media 2012

Authors and Affiliations

  1. 1.Jacobs Neurological Institute, Department of Neurology, School of Medicine and Biomedical SciencesUniversity at BuffaloBuffaloUSA

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