Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point-of-care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors and editors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

Choose from the list below to learn more about subscriptions for a:


Subscribers log in here


Acute disseminated encephalomyelitis in children: Treatment and prognosis

INTRODUCTION

Acute disseminated encephalomyelitis (ADEM), also known as postinfectious encephalomyelitis, is a demyelinating disease of the central nervous system that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and disability.

This topic will review the prognosis and treatment of ADEM in children. Other aspects of ADEM are discussed separately. (See "Acute disseminated encephalomyelitis in children: Pathogenesis, clinical features, and diagnosis".)

TREATMENT

Children with ADEM typically present with fever, meningeal signs, acute encephalopathy, and evidence of inflammation in blood and cerebrospinal fluid. Thus, consideration should be given to treatment with broad-spectrum antibiotics and acyclovir until an infectious etiology is excluded. (See "Bacterial meningitis in children older than one month: Treatment and prognosis", section on 'Empiric therapy' and "Bacterial meningitis in the neonate: Treatment and outcome", section on 'Antimicrobial therapy' and "Viral meningitis: Management, prognosis, and prevention in children", section on 'Empiric therapy'.)

The mainstay of treatment for ADEM is high-dose intravenous glucocorticoids [1]. Glucocorticoids may be started at the time of the patient's presentation and can be used concurrently with antibiotics and acyclovir. Additional options include intravenous immune globulin and plasma exchange [2]. However, the effectiveness of these treatments (glucocorticoids, intravenous immune globulin, and plasma exchange) for ADEM has not been definitively confirmed, as there are no prospective clinical trial data to determine optimal treatment, including dose or duration.

Glucocorticoids — In several small observational studies, treatment of ADEM with intravenous methylprednisolone (10 to 30 mg/kg per day, maximum 1000 mg daily) or dexamethasone (1 mg/kg per day) for three to five days, followed by oral glucocorticoid taper over four to six weeks, was associated with full recovery in approximately 60 to 90 percent of patients [3-5].

      

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Aug 2014. | This topic last updated: Feb 2, 2013.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2014 UpToDate, Inc.
References
Top
  1. Alper G. Acute disseminated encephalomyelitis. J Child Neurol 2012; 27:1408.
  2. Tenembaum S, Chitnis T, Ness J, et al. Acute disseminated encephalomyelitis. Neurology 2007; 68:S23.
  3. Dale RC, de Sousa C, Chong WK, et al. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain 2000; 123 Pt 12:2407.
  4. Hynson JL, Kornberg AJ, Coleman LT, et al. Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology 2001; 56:1308.
  5. Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology 2002; 59:1224.
  6. Anlar B, Basaran C, Kose G, et al. Acute disseminated encephalomyelitis in children: outcome and prognosis. Neuropediatrics 2003; 34:194.
  7. Kleiman M, Brunquell P. Acute disseminated encephalomyelitis: response to intravenous immunoglobulin. J Child Neurol 1995; 10:481.
  8. Pradhan S, Gupta RP, Shashank S, Pandey N. Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis. J Neurol Sci 1999; 165:56.
  9. Sahlas DJ, Miller SP, Guerin M, et al. Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin. Neurology 2000; 54:1370.
  10. Nishikawa M, Ichiyama T, Hayashi T, et al. Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis. Pediatr Neurol 1999; 21:583.
  11. Stricker RB, Miller RG, Kiprov DD. Role of plasmapheresis in acute disseminated (postinfectious) encephalomyelitis. J Clin Apher 1992; 7:173.
  12. Balestri P, Grosso S, Acquaviva A, Bernini M. Plasmapheresis in a child affected by acute disseminated encephalomyelitis. Brain Dev 2000; 22:123.
  13. Miyazawa R, Hikima A, Takano Y, et al. Plasmapheresis in fulminant acute disseminated encephalomyelitis. Brain Dev 2001; 23:424.
  14. Khurana DS, Melvin JJ, Kothare SV, et al. Acute disseminated encephalomyelitis in children: discordant neurologic and neuroimaging abnormalities and response to plasmapheresis. Pediatrics 2005; 116:431.
  15. Greenberg BM, Thomas KP, Krishnan C, et al. Idiopathic transverse myelitis: corticosteroids, plasma exchange, or cyclophosphamide. Neurology 2007; 68:1614.
  16. Rosman NP, Gottlieb SM, Bernstein CA. Acute hemorrhagic leukoencephalitis: recovery and reversal of magnetic resonance imaging findings in a child. J Child Neurol 1997; 12:448.
  17. Klein CJ, Wijdicks EF, Earnest F 4th. Full recovery after acute hemorrhagic leukoencephalitis (Hurst's disease). J Neurol 2000; 247:977.
  18. Seales D, Greer M. Acute hemorrhagic leukoencephalitis. A successful recovery. Arch Neurol 1991; 48:1086.
  19. Kesselring J, Miller DH, Robb SA, et al. Acute disseminated encephalomyelitis. MRI findings and the distinction from multiple sclerosis. Brain 1990; 113 ( Pt 2):291.
  20. O'Riordan JI, Gomez-Anson B, Moseley IF, Miller DH. Long term MRI follow-up of patients with post infectious encephalomyelitis: evidence for a monophasic disease. J Neurol Sci 1999; 167:132.
  21. Murthy SN, Faden HS, Cohen ME, Bakshi R. Acute disseminated encephalomyelitis in children. Pediatrics 2002; 110:e21.
  22. Mar S, Lenox J, Benzinger T, et al. Long-term prognosis of pediatric patients with relapsing acute disseminated encephalomyelitis. J Child Neurol 2010; 25:681.
  23. Menge T, Hemmer B, Nessler S, et al. Acute disseminated encephalomyelitis: an update. Arch Neurol 2005; 62:1673.
  24. Khong PL, Ho HK, Cheng PW, et al. Childhood acute disseminated encephalomyelitis: the role of brain and spinal cord MRI. Pediatr Radiol 2002; 32:59.