Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
권호 표지
DOI QR코드

DOI QR Code

Acute disseminated encephalomyelitis in children: differential diagnosis from multiple sclerosis on the basis of clinical course

  • Lee, Yun-Jin (Department of Pediatrics, Pusan National University Children’s Hospital, Pusan National University School of Medicine)
  • Received : 2011.04.18
  • Accepted : 2011.05.11
  • Published : 2011.06.15
Download PDF
⟨ Previous Next ⟩

Abstract

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalopathy. ADEM is considered an autoimmune disorder that is triggered by an environmental stimulus in genetically susceptible individuals. The diagnosis of ADEM is based on clinical and radiological features. Most children with ADEM initially present with fever, meningeal signs, and acute encephalopathy. The level of consciousness ranges from lethargy to frank coma. Deep and subcortical white-matter lesions and gray-matter lesions such as thalami and basal ganglia on magnetic resonance imaging (MRI) are associated with ADEM. In a child who presents with signs of encephalitis, bacterial and viral meningitis or encephalitis must be ruled out. Sequential MRI is required to confirm the diagnosis of ADEM, as relapses with the appearance of new lesions on MRI may suggest either multiphasic ADEM or multiple sclerosis (MS). Pediatric MS, defined as onset of MS before the age of 16, is being increasingly recognized. MS is characterized by recurrent episodes of demyelination in the CNS separated in space and time. The McDonald criteria for diagnosis of MS include evidence from MRI and allow the clinician to make a diagnosis of clinically definite MS on the basis of the interval preceding the development of new white matter lesions, even in the absence of new clinical findings. The most important alternative diagnosis to MS is ADEM. At the initial presentation, the 2 disorders cannot be distinguished with certainty. Therefore, prolonged follow-up is needed to establish a diagnosis.

Keywords

References

  1. Dale RC, de Sousa C, Chong WK, Cox TC, Harding B, Neville BG. Acute disseminated encephalomyelitis, multiphasic disseminated encephalomyelitis and multiple sclerosis in children. Brain 2000;123 Pt 12:2407-22. https://doi.org/10.1093/brain/123.12.2407
  2. Hynson JL, Kornberg AJ, Coleman LT, Shield L, Harvey AS, Kean MJ. Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology 2001;56:1308-12. https://doi.org/10.1212/WNL.56.10.1308
  3. Tenembaum S, Chamoles N, Fejerman N. Acute disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology 2002;59:1224-31. https://doi.org/10.1212/WNL.59.8.1224
  4. Tenembaum S, Chitnis T, Ness J, Hahn JS; International Pediatric MS Study Group. Acute disseminated encephalomyelitis. Neurology 2007;68(16 Suppl 2):S23-36. https://doi.org/10.1212/01.wnl.0000259404.51352.7f
  5. Pohl-Koppe A, Burchett SK, Thiele EA, Hafler DA. Myelin basic protein reactive Th2 T cells are found in acute disseminated encephalomyelitis. J Neuroimmunol 1998;91:19-27. https://doi.org/10.1016/S0165-5728(98)00125-8
  6. Stüve O, Zamvil SS. Pathogenesis, diagnosis, and treatment of acute disseminated encephalomyelitis. Curr Opin Neurol 1999;12:395-401. https://doi.org/10.1097/00019052-199908000-00005
  7. Caldemeyer KS, Smith RR, Harris TM, Edwards MK. MRI in acute disseminated encephalomyelitis. Neuroradiology 1994;36:216-20. https://doi.org/10.1007/BF00588134
  8. Kim SC, Jang HJ, Han DJ. Acute disseminated encephalomyelitis after renal transplantation in patients with positive Epstein-Barr virus antibody. Transplant Proc 1998;30:3139. https://doi.org/10.1016/S0041-1345(98)00967-1
  9. Apak RA, Köse G, Anlar B, Turanli G, Topaloğlu H, Ozdirim E. Acute disseminated encephalomyelitis in childhood: report of 10 cases. J Child Neurol 1999;14:198-201. https://doi.org/10.1177/088307389901400312
  10. Huber S, Kappos L, Fuhr P, Wetzel S, Steck AJ. Combined acute disseminated encephalomyelitis and acute motor axonal neuropathy after vaccination for hepatitis A and infection with Campylobacter jejuni. J Neurol 1999;246:1204-6. https://doi.org/10.1007/s004150050546
  11. Murthy JM. MRI in acute disseminated encephalomyelitis following Semple antirabies vaccine. Neuroradiology 1998;40:420-3. https://doi.org/10.1007/s002340050615
  12. Stonehouse M, Gupte G, Wassmer E, Whitehouse WP. Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians. Arch Dis Child 2003;88:122-4. https://doi.org/10.1136/adc.88.2.122
  13. Fenichel GM. Neurological complications of immunization. Ann Neurol 1982;12:119-28. https://doi.org/10.1002/ana.410120202
  14. Boiko A, Vorobeychik G, Paty D, Devonshire V, Sadovnick D; University of British Columbia MS Clinic Neurologists. Early onset multiple sclerosis: a longitudinal study. Neurology 2002;59:1006-10. https://doi.org/10.1212/WNL.59.7.1006
  15. Duquette P, Murray TJ, Pleines J, Ebers GC, Sadovnick D, Weldon P, et al. Multiple sclerosis in childhood: clinical profile in 125 patients. J Pediatr 1987;111:359-63. https://doi.org/10.1016/S0022-3476(87)80454-7
  16. Gadoth N. Multiple sclerosis in children. Brain Dev 2003;25:229-32. https://doi.org/10.1016/S0387-7604(03)00035-4
  17. Renoux C, Vukusic S, Mikaeloff Y, Edan G, Clanet M, Dubois B, et al. Natural history of multiple sclerosis with childhood onset. N Engl J Med 2007;356:2603-13. https://doi.org/10.1056/NEJMoa067597
  18. Brass SD, Caramanos Z, Santos C, Dilenge ME, Lapierre Y, Rosenblatt B. Multiple sclerosis vs acute disseminated encephalomyelitis in childhood. Pediatr Neurol 2003;29:227-31. https://doi.org/10.1016/S0887-8994(03)00235-2
  19. Davis LE, Booss J. Acute disseminated encephalomyelitis in children: a changing picture. Pediatr Infect Dis J 2003;22:829-31. https://doi.org/10.1097/01.inf.0000087847.37363.78
  20. Paty D, Studney D, Redekop K, Lublin F. MS COSTAR: a computerized patient record adapted for clinical research purposes. Ann Neurol 1994;36 Suppl:S134-5. https://doi.org/10.1002/ana.410360732
  21. Neuteboom RF, Boon M, Catsman Berrevoets CE, Vles JS, Gooskens RH, Stroink H, et al. Prognostic factors after a first attack of inflammatory CNS demyelination in children. Neurology 2008;71:967-73. https://doi.org/10.1212/01.wnl.0000316193.89691.e1
  22. Baum PA, Barkovich AJ, Koch TK, Berg BO. Deep gray matter involvement in children with acute disseminated encephalomyelitis. AJNR Am J Neuroradiol 1994;15:1275-83.
  23. Krupp LB, Banwell B, Tenembaum S; International Pediatric MS Study Group. Consensus definitions proposed for pediatric multiple sclerosis and related disorders. Neurology 2007;68(16 Suppl 2):S7-12. https://doi.org/10.1212/01.wnl.0000259422.44235.a8
  24. Poser CM, Paty DW, Scheinberg L, McDonald WI, Davis FA, Ebers GC, et al. New diagnostic criteria for multiple sclerosis: guidelines for research protocols. Ann Neurol 1983;13:227-31. https://doi.org/10.1002/ana.410130302
  25. McDonald WI, Compston A, Edan G, Goodkin D, Hartung HP, Lublin FD, et al. Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001;50:121-7. https://doi.org/10.1002/ana.1032
  26. Polman CH, Reingold SC, Edan G, Filippi M, Hartung HP, Kappos L, et al. Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald Criteria". Ann Neurol 2005;58:840-6. https://doi.org/10.1002/ana.20703
  27. Waubant E, Chabas D, Okuda DT, Glenn O, Mowry E, Henry RG, et al. Difference in disease burden and activity in pediatric patients on brain magnetic resonance imaging at time of multiple sclerosis onset vs adults. Arch Neurol 2009;66:967-71. https://doi.org/10.1001/archneurol.2009.135

Cited by

  1. Acute disseminated encephalomyelitis progressing to multiple sclerosis: Are infectious triggers involved? vol.60, pp.1, 2011, https://doi.org/10.1007/s12026-014-8499-y
  2. Multiphasic acute disseminated encephalomyelitis associated with atypical rubella virus infection vol.21, pp.8, 2015, https://doi.org/10.1177/1352458514567729
  3. A spectrum of inflammation and demyelination in acute disseminated encephalomyelitis (ADEM) of children vol.14, pp.10, 2011, https://doi.org/10.1016/j.autrev.2015.06.002
  4. Imaging in viral infections of the central nervous system: can images speak for an acutely ill brain? vol.24, pp.3, 2011, https://doi.org/10.1007/s10140-016-1463-5
  5. Acute Intermittent Porphyria: A Rare Cause of Acute Disseminated Encephalomyelitis vol.10, pp.7, 2011, https://doi.org/10.7759/cureus.2989
  6. Diagnostic Approach to Intrinsic Abnormality of Spinal Cord Signal Intensity vol.39, pp.6, 2011, https://doi.org/10.1148/rg.2019190021
  7. Acute Disseminated Encephalomyelitis (ADEM) versus Multiple Sclerosis (MS)- A Diagnostic Challenge in an Adult vol.9, pp.21, 2011, https://doi.org/10.14260/jemds/2020/367
  8. MIS-C is a Clinically Different Entity from Acute COVID-19 in Adults vol.25, pp.8, 2021, https://doi.org/10.5005/jp-journals-10071-23927
  9. Genetic disorders with central nervous system white matter abnormalities: An update vol.99, pp.1, 2011, https://doi.org/10.1111/cge.13863
  10. The Potential Role of COVID-19 in the Pathogenesis of Multiple Sclerosis-A Preliminary Report vol.13, pp.10, 2011, https://doi.org/10.3390/v13102091
  11. To be, or not to be… Guillain-Barré Syndrome vol.20, pp.12, 2021, https://doi.org/10.1016/j.autrev.2021.102983