eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Acute Disseminated Encephalomyelitis: Follow-up

Author: Robert Stanley Rust Jr, MD, MA, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia; Chair-Elect, Child Neurology Section, American Academy of Neurology
Contributor Information and Disclosures

Updated: Sep 3, 2009

Follow-up

Further Inpatient Care

  • After initial evaluation and initiation of therapy, further inpatient care is dictated by the evolution of disease and rate of recovery exhibited by the patient.
  • Physical and occupational therapy may be indicated in patients with paresis, ataxia, low vision, and other focal neurologic abnormalities that impair function.
  • Provision for feeding and for the treatment of abnormalities of bowel or bladder function may be indicated.
  • When seizures occur, they usually do so transiently at the onset of disease. In rare instances, additional management issues for seizures arise during the subsequent course of treatment.

Further Outpatient Care

  • Outpatient care indications depend on the course of illness and the extent of recovery at the time of discharge. It may include the involvement of physical, occupational, or speech therapists. Some patients require follow-up with urologists or gastroenterologists because of persistent bladder or bowel problems.
  • Patients who are placed on tapering doses of oral corticosteroids require follow-up to ascertain the rate and extent of improvement. Urgent return or consultation may be warranted by patients who display relapse during taper of corticosteroids. In such instances, the relapse is usually controlled by restoration of a higher medication dosage with slower ensuing taper. Some difficult cases require slow tapers.

Transfer

  • Some patients with more severe degrees of neurologic disability are transferred to rehabilitation facilities for some period of time before they are judged sufficiently recovered to be discharged home.

Complications

  • The most common inpatient complications include abnormalities of vision, motor function (ie, pyramidal, extrapyramidal, cerebellar), or bladder or bowel function.
  • Recurrence is the chief outpatient complication and is rare.

Prognosis

  • The outlook for recovery is generally excellent. Although some older series suggest up to a 10% mortality rate, only 1.5% of the authors' cases have resulted in mortality due to ADEM-related complications. Degree of recovery is unrelated to severity of illness. Complete recovery may be observed even in children who become blind, comatose, and quadriparetic. Recovery is poorest in children younger than 2 years, patients with myelitis, and those who have significant edema of the brain or spinal cord. Whether ultra–high-dose corticosteroid therapy and other treatments for edema might improve the outcome for these groups is not yet known, although limited experience suggests this possibility. In other cases of ADEM, modest visual or motor deficits may persist, as may sphincter abnormalities in patients with spinal cord disease. Disturbances of mood and personality may outlast motor deficits, but they may also wane over ensuing months.
  • The long-term (10-y follow-up) risk of patients with ADEM for development of MS is 25%. Risk for MS is highest in children whose ADEM onset was (1) afebrile, (2) without mental status change, (3) without prodromal viral illness or immunization, (4) without generalized EEG slowing, or (5) associated with an abnormal CSF immune profile (Rust, 1989).
  • Most patients who experience a bout of ADEM can look forward to complete recovery or the persistence of only mild deficits, such as modestly diminished visual acuity. This excellent outlook even applies to patients who experienced a global low state of function during the acute illness.
    • Whether any available form of treatment has a favorable effect on the time to maximal recovery or the risk for deficits is unknown. Exceptions to the excellent outlook are patients with transverse myelitis and infants younger than 2 years. Cord swelling may account for the high rate of residual paresis and the occasional death of patients with severe acute inflammatory myelitis. Prompt administration of high doses of corticosteroids can possibly improve the outlook for these patients, but no reliable data yet support this hypothesis. The risk of MS for prepubertal children who have a bout of ADEM is less than 10%.
    • The authors have observed children with a typical bout of ADEM in the first decade of life who manifest MS during the second decade, after a symptom-free hiatus of more than 10 years.
  • In the authors' experience, the risk for MS for children who have had a single prepubertal bout of ADEM is less than 6%. MS is defined, for these children, as persistence of an illness satisfying clinically definite multiple sclerosis (CD-MS) criteria into adolescence.
    • The risk for MS in children who have had 1-3 prepubertal relapses of ADEM, none of which was related to steroid withdrawal, ranges from 15-33%. Risk increases in proportion to the number of relapses that occur, especially relapses that occurred without exogenous provocation (eg, febrile prodrome, vaccination).
    • Children with more than 3 prepubertal clinical relapses are at significant risk of having rare vasculitic or inflammatory processes that must be diagnosed by biopsy of brain tissue or other organs (eg, CNS vasculitis, hypersensitivity vasculitides, sarcoidosis, histiocytic lymphogranulomatosis) and in some instances are found to be harboring neoplasm (the results of their imaging studies having been misinterpreted).
  • The hesitancy to apply a diagnosis of CD-MS has not prevented the authors from using immunomodulatory therapy (eg, IFN-beta1a) in the treatment of children with recurrences or persistent lesions that do not appear to respond to corticosteroid therapy or those who develop a pattern suggesting steroid dependence (steroid withdrawal–related recurrence); the sometimes gratifying response to IFN-beta1a has not been viewed as confirmation of MS.
    • These uncertainties have generated several additional worries, particularly whether too much delay is entailed in administration of immunomodulatory therapy to a prepubertal child for whom an MS diagnosis is withheld and whether at some point discontinuing immunomodulatory therapy is safe in a child who has experienced a good response to such therapy for a recurrent illness that may be MS despite the absence of characteristic changes in the CSF immune profile.
    • In this context, remember that as many as 8% of adults who are identified as having CD-MS do not have immune profile abnormalities even after the second or third clinical bout.

Patient Education

Miscellaneous

Medicolegal Pitfalls

  • Medicolegal issues that arise in association with ADEM include the following:
    • Delay in diagnosis and treatment of ADEM
    • Complications occurring in the course of ADEM either as the result of natural evolution of the disease or because of treatments
    • Allegations that immunizations are the cause of ADEM
 


More on Acute Disseminated Encephalomyelitis

Overview: Acute Disseminated Encephalomyelitis
Differential Diagnoses & Workup: Acute Disseminated Encephalomyelitis
Treatment & Medication: Acute Disseminated Encephalomyelitis
Follow-up: Acute Disseminated Encephalomyelitis
References
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References

  1. Ishizu T, Minohara M, Ichiyama T, et al. CSF cytokine and chemokine profiles in acute disseminated encephalomyelitis. J Neuroimmunol. Jun 2006;175(1-2):52-8. [Medline].

  2. Franciotta D, Zardini E, Ravaglia S, et al. Cytokines and chemokines in cerebrospinal fluid and serum of adult patients with acute disseminated encephalomyelitis. J Neurol Sci. Sep 25 2006;247(2):202-7. [Medline].

  3. Sacconi S, Salviati L, Merelli E. Acute disseminated encephalomyelitis associated with hepatitis C virus infection. Arch Neurol. Oct 2001;58(10):1679-81. [Medline].

  4. Chowdhary J, Ashraf SM, Khajuria K. Measles with acute disseminated encephalomyelitis (ADEM). Indian Pediatr. Jan 2009;46(1):72-4. [Medline].

  5. Alves-Leon SV, Veluttini-Pimentel ML, Gouveia ME, Malfetano FR, Gaspareto EL, Alvarenga MP, et al. Acute disseminated encephalomyelitis: clinical features, HLA DRB1*1501, HLA DRB1*1503, HLA DQA1*0102, HLA DQB1*0602, and HLA DPA1*0301 allelic association study. Arq Neuropsiquiatr. Sep 2009;67(3A):643-51. [Medline].

  6. Atalar MH. Acute disseminated encephalomyelitis in an adult patient. Magnetic resonance and diffusion-weighted imaging findings. Saudi Med J. Jan 2006;27(1):105-8. [Medline].

  7. Callen DJ, Shroff MM, Branson HM, Li DK, Lotze T, Stephens D, et al. Role of MRI in the differentiation of ADEM from MS in children. Neurology. Mar 17 2009;72(11):968-73. [Medline].

  8. Baum PA, Barkovich AJ, Koch TK, Berg BO. Deep gray matter involvement in children with acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. Aug 1994;15(7):1275-83. [Medline].

  9. Apak RA, Kose G, Anlar B, et al. Acute disseminated encephalomyelitis in childhood: report of 10 cases. J Child Neurol. Mar 1999;14(3):198-201. [Medline].

  10. Kesselring J, Miller DH, Robb SA, Kendall BE, Moseley IF, Kingsley D, et al. Acute disseminated encephalomyelitis. MRI findings and the distinction from multiple sclerosis. Brain. Apr 1990;113 ( Pt 2):291-302. [Medline].

  11. van der Meyden CH, de Villiers JF, Middlecote BD, Terblanchè J. Gadolinium ring enhancement and mass effect in acute disseminated encephalomyelitis. Neuroradiology. Apr 1994;36(3):221-3. [Medline].

  12. Honkaniemi J, Dastidar P, Kähärä V, Haapasalo H. Delayed MR imaging changes in acute disseminated encephalomyelitis. AJNR Am J Neuroradiol. Jun-Jul 2001;22(6):1117-24. [Medline].

  13. Trotter JL, Rust RS. Human cerebrospinal fluid immunology. In: Herndon RM, Brumback RA, eds. The Cerebrospinal Fluid. Boston, Mass:. Kluwer Academic Publishers;1989:179-226.

  14. Nishikawa M, Ichiyama T, Hayashi T, Ouchi K, Furukawa S. Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis. Pediatr Neurol. Aug 1999;21(2):583-6. [Medline].

  15. Stricker RB, Miller RG, Kiprov DD. Role of plasmapheresis in acute disseminated (postinfectious) encephalomyelitis. J Clin Apher. 1992;7(4):173-9. [Medline].

  16. Kanter DS, Horensky D, Sperling RA, Kaplan JD, Malachowski ME, Churchill WH Jr. Plasmapheresis in fulminant acute disseminated encephalomyelitis. Neurology. Apr 1995;45(4):824-7. [Medline].

  17. Sugita K, Suzuki N, Shimizu N, Takanashi J, Ishii M, Niimi N. Involvement of cytokines in N-methyl-N'-nitro-N-nitrosoguanidine-induced plasminogen activator activity in acute disseminated encephalomyelitis and multiple sclerosis lymphocytes. Eur Neurol. 1993;33(5):358-62. [Medline].

  18. Dale RC, Branson JA. Acute disseminated encephalomyelitis or multiple sclerosis: can the initial presentation help in establishing a correct diagnosis?. Arch Dis Child. Jun 2005;90(6):636-9. [Medline].

  19. Garg RK. Acute disseminated encephalomyelitis. Postgrad Med J. Jan 2003;79(927):11-7. [Medline].

  20. Hahn JS, Siegler DJ, Enzmann D. Intravenous gammaglobulin therapy in recurrent acute disseminated encephalomyelitis. Neurology. Apr 1996;46(4):1173-4. [Medline].

  21. Hartel C, Schilling S, Gottschalk S, Sperner J. Multiphasic disseminated encephalomyelitis associated with streptococcal infection. Eur J Paediatr Neurol. 2002;6(6):327-9. [Medline].

  22. Holtmannspotter M, Inglese M, Rovaris M, et al. A diffusion tensor MRI study of basal ganglia from patients with ADEM. J Neurol Sci. Jan 15 2003;206(1):27-30. [Medline].

  23. John L, Khaleeli AA, Larner AJ. Acute disseminated encephalomyelitis: a riddle wrapped in a mystery inside an enigma. Int J Clin Pract. Apr 2003;57(3):235-7. [Medline].

  24. Kadhim H, De Prez C, Gazagnes MD, Sebire G. In situ cytokine immune responses in acute disseminated encephalomyelitis: insights into pathophysiologic mechanisms. Hum Pathol. Mar 2003;34(3):293-7. [Medline].

  25. Mariotti P, Batocchi AP, Colosimo C,et al. Multiphasic demyelinating disease involving central and peripheral nervous system in a child. Neurology. Jan 28 2003;60(2):348-9. [Medline].

  26. Murthy JM. Acute disseminated encephalomyelitis. Neurol India. Sep 2002;50(3):238-43. [Medline].

  27. Oksuzler YF, Cakmakci H, Kurul S, et al. Diagnostic value of diffusion-weighted magnetic resonance imaging in pediatric cerebral diseases. Pediatr Neurol. May 2005;32(5):325-33. [Medline].

  28. Pena JA, Montiel-Nava C, Hernandez F, et al. [Disseminated acute encephalomyelitis in children]. Rev Neurol. Jan 16-31 2002;34(2):163-8. [Medline].

  29. Pradhan S, Gupta RP, Shashank S, Pandey N. Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis. J Neurol Sci. May 1 1999;165(1):56-61. [Medline].

  30. Rust RS. Multiple sclerosis, acute disseminated encephalomyelitis, and related conditions. Semin Pediatr Neurol. Jun 2000;7(2):66-90. [Medline].

  31. Rust RS, Dodson WE, Trotter JL. Cerebrospinal fluid IgG in childhood: the establishment of reference values. Ann Neurol. Apr 1988;23(4):406-10. [Medline].

  32. Sakakibara R, Yamanishi T, Uchiyama T, Hattori T. Acute urinary retention due to benign inflammatory nervous diseases. J Neurol. Aug 2006;253(8):1103-10. [Medline].

  33. Sunnerhagen KS, Johansson K, Ekholm S. Rehabilitation problems after acute disseminated encephalomyelitis: four cases. J Rehabil Med. Jan 2003;35(1):20-5. [Medline].

  34. Verbruggen SC, Catsman CE, Naghib S, et al. [Respiratory insufficiency caused by acute disseminated encephalomyelitis in a child]. Ned Tijdschr Geneeskd. May 20 2006;150(20):1134-8. [Medline].

  35. Weng WC, Peng SS, Lee WT, et al. Acute disseminated encephalomyelitis in children: one medical center experience. Acta Paediatr Taiwan. Mar-Apr 2006;47(2):67-71. [Medline].

  36. Wingerchuk DM. The clinical course of acute disseminated encephalomyelitis. Neurol Res. Apr 2006;28(3):341-7. [Medline].

  37. Yapici Z, Eraksoy M. Bilateral demyelinating tumefactive lesions in three children with hemiparesis. J Child Neurol. Sep 2002;17(9):655-60. [Medline].

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Further Reading

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Keywords

ADEM, acute disseminated perivenous encephalomyelitis, acute post-vaccinial encephalitis, demyelinating encephalomyelitis, acute disseminated vasculomyelinopathy, recurrent disseminated vasculomyelinopathy, drug-induced perivenular demyelination

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Contributor Information and Disclosures

Author

Robert Stanley Rust Jr, MD, MA, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia; Chair-Elect, Child Neurology Section, American Academy of Neurology
Robert Stanley Rust Jr, MD, MA is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Christopher Luzzio, MD, Clinical Assistant Professor, Department of Neurology, University of Wisconsin at Madison
Christopher Luzzio, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Glenn Lopate, MD, Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Chief of Neurology, St Louis ConnectCare, Consulting Staff, Barnes Jewish Hospital
Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

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