acute disseminated encephalomyelitis

Summary

Summary: An acute or subacute inflammatory process of the CENTRAL NERVOUS SYSTEM characterized histologically by multiple foci of perivascular demyelination. Symptom onset usually occurs several days after an acute viral infection or immunization, but it may coincide with the onset of infection or rarely no antecedent event can be identified. Clinical manifestations include CONFUSION, somnolence, FEVER, nuchal rigidity, and involuntary movements. The illness may progress to COMA and eventually be fatal. (Adams et al., Principles of Neurology, 6th ed, p921)

Top Publications

  1. Weng W, Peng S, Lee W, Fan P, Chien Y, Du J, et al. Acute disseminated encephalomyelitis in children: one medical center experience. Acta Paediatr Taiwan. 2006;47:67-71 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory demyelinating disease of the central nervous system. The experience in children is limited...
  2. Tenembaum S, Chitnis T, Ness J, Hahn J. Acute disseminated encephalomyelitis. Neurology. 2007;68:S23-36 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is an immune-mediated inflammatory disorder of the CNS characterized by a widespread demyelination that predominantly involves the white matter of the brain and spinal cord...
  3. Madan S, Aneja S, Tripathi R, Batra A, Seth A, Taluja V. Acute disseminated encephalomyelitis--a case series. Indian Pediatr. 2005;42:367-71 pubmed
    b>Acute disseminated encephalomyelitis(ADEM) is an uncommon inflammatory demyelinating disease involving central nervous system white matter. A series of seven cases seen over a period of one year have been described...
  4. Hollinger P, Sturzenegger M, Mathis J, Schroth G, Hess C. Acute disseminated encephalomyelitis in adults: a reappraisal of clinical, CSF, EEG, and MRI findings. J Neurol. 2002;249:320-9 pubmed
    To establish an adequate definition of acute disseminated encephalomyelitis (ADEM) in adults, based on our clinical observations of a case-series.
  5. Suppiej A, Manara R, De Palma L, De Grandis D, Citton V, Battistella P. Multiphasic acute disseminated encephalomyelitis or pediatric multiple sclerosis: report of an atypical case. J Child Neurol. 2009;24:241-6 pubmed publisher
    ..insight about clinical overlap between pediatric multiple sclerosis and all forms of relapsing acute disseminated encephalomyelitis may be gained from long-term follow-up...
  6. Refai D, Lee M, Goldenberg F, Frank J. Decompressive hemicraniectomy for acute disseminated encephalomyelitis: case report. Neurosurgery. 2005;56:E872; discussion E871 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) varies widely in symptoms and severity. Some cases are associated with massive life-threatening cerebral edema refractory to conventional medical management.
  7. Ramaswamy V, Sinclair D, Wheatley B, Richer L, Snyder T. Epilepsia partialis continua: acute disseminated encephalomyelitis or Rasmussen's encephalitis?. Pediatr Neurol. 2005;32:341-5 pubmed
    ..The relevant literature is reviewed with an emphasis on possible etiologies, including both acute disseminated encephalomyelitis and Rasmussen's encephalitis.
  8. Sunnerhagen K, Johansson K, Ekholm S. Rehabilitation problems after acute disseminated encephalomyelitis: four cases. J Rehabil Med. 2003;35:20-5 pubmed
    The aim of this study is to describe rehabilitation problems in patients with acute disseminated encephalomyelitis. The study examines retrospective clinical data...
  9. Voudris K, Vagiakou E, Skardoutsou A. Acute disseminated encephalomyelitis associated with parainfluenza virus infection of childhood. Brain Dev. 2002;24:112-4 pubmed
    b>Acute disseminated encephalomyelitis associated with the parainfluenza virus has rarely been reported in childhood. A 2...

More Information

Publications62

  1. Khong P, Ho H, Cheng P, Wong V, Goh W, Chan F. Childhood acute disseminated encephalomyelitis: the role of brain and spinal cord MRI. Pediatr Radiol. 2002;32:59-66 pubmed
    It is recognised that the clinical and radiological spectrum of childhood acute disseminated encephalomyelitis (ADEM) is wide.
  2. Leake J, Albani S, Kao A, Senac M, Billman G, Nespeca M, et al. Acute disseminated encephalomyelitis in childhood: epidemiologic, clinical and laboratory features. Pediatr Infect Dis J. 2004;23:756-64 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is a central nervous system demyelinating disease that usually follows an apparently benign infection in otherwise healthy young persons...
  3. Mikaeloff Y, Caridade G, Husson B, Suissa S, Tardieu M. Acute disseminated encephalomyelitis cohort study: prognostic factors for relapse. Eur J Paediatr Neurol. 2007;11:90-5 pubmed
    To date, there is no available epidemiological study about prognostic factors of acute disseminated encephalomyelitis (ADEM) in children, using a cohort of patients with homogenous inclusion criteria...
  4. Tardieu M, Mikaeloff Y. What is acute disseminated encephalomyelitis (ADEM)?. Eur J Paediatr Neurol. 2004;8:239-42 pubmed
    ..Macrophage activation syndrome and different types of angiitis may mimic ADEM, because they can occur in the absence of the usual non neurological symptoms. Both entities require specific treatments...
  5. Liptai Z, Ujhelyi E, Mihály I, Rudas G, Barsi P. [Acute disseminated encephalomyelitis in childhood]. Ideggyogy Sz. 2009;62:244-54 pubmed
    b>Acute disseminated encephalomyelitis is a rare inflammatory demyelinating disorder often preceded by infection or vaccination...
  6. Chowdhary J, Ashraf S, Khajuria K. Measles with acute disseminated encephalomyelitis (ADEM). Indian Pediatr. 2009;46:72-4 pubmed
    We report a seven year old male with measles associated acute disseminated encephalomyelitis (ADEM) despite having received measles vaccination in infancy. The diagnosis was based on serum antimeasles antibodies and MRI brain...
  7. Pohl D, Hennemuth I, Von Kries R, Hanefeld F. Paediatric multiple sclerosis and acute disseminated encephalomyelitis in Germany: results of a nationwide survey. Eur J Pediatr. 2007;166:405-12 pubmed
    The aim of this study was to evaluate the incidence of paediatric multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM) in Germany...
  8. Ishizu T, Minohara M, Ichiyama T, Kira R, Tanaka M, Osoegawa M, et al. CSF cytokine and chemokine profiles in acute disseminated encephalomyelitis. J Neuroimmunol. 2006;175:52-8 pubmed
    ..We simultaneously measured 16 cytokines/chemokines in cerebrospinal fluid (CSF) from 14 patients with acute disseminated encephalomyelitis (ADEM) and 20 controls using a fluorescent bead-based immunoassay...
  9. Dale R, Branson J. Acute disseminated encephalomyelitis or multiple sclerosis: can the initial presentation help in establishing a correct diagnosis?. Arch Dis Child. 2005;90:636-9 pubmed
    ..When the disease is monophasic, the term acute disseminated encephalomyelitis (ADEM) is used...
  10. Matsumoto N, Takahashi S, Toriumi N, Sarashina T, Makita Y, Tachibana Y, et al. Acute disseminated encephalomyelitis in an infant with incontinentia pigmenti. Brain Dev. 2009;31:625-8 pubmed publisher
    ..The patient developed repetitive seizures at the age of 7 months when she was diagnosed with acute disseminated encephalomyelitis (ADEM), an inflammatory demyelinating disease of the central nervous system that often occurs after ..
  11. Stonehouse M, Gupte G, Wassmer E, Whitehouse W. Acute disseminated encephalomyelitis: recognition in the hands of general paediatricians. Arch Dis Child. 2003;88:122-4 pubmed
    b>Acute disseminated encephalomyelitis will often present to the general paediatrician as an acute polysymptomatic encephalopathy, and initially the diagnosis may not be clear...
  12. Lin C, Jeng J, Yip P. Plasmapheresis in acute disseminated encephalomyelitis. J Clin Apher. 2004;19:154-9 pubmed
    High-dose corticosteroid administration has been regarded as the main therapy for acute disseminated encephalomyelitis (ADEM). However, some patients with ADEM do not respond well to this treatment...
  13. Ueda M, Kanamori A, Mihara T, Hara H, Mutoh T. [A case of acute disseminated encephalomyelitis (ADEM) following treatment for pneumococcal meningoencephalitis]. Rinsho Shinkeigaku. 2009;49:96-9 pubmed
    ..6 mm) with ring enhancements in the right frontal lobe. Acute disseminated encephalomyelitis (ADEM) was diagnosed based on MRI and CSF findings, and then additional corticosteroid pulse ..
  14. Matsuda M, Miki J, Tabata K, Ikeda S. Severe depression as an initial symptom in an elderly patient with acute disseminated encephalomyelitis. Intern Med. 2001;40:1149-53 pubmed
    We report a 63-year-old man with acute disseminated encephalomyelitis (ADEM), initially showing depression for one and a half months but subsequently meningoencephalitis followed by acute-onset myelopathy...
  15. Lu R, Keilson G. Combination regimen of methylprednisolone, IV immunoglobulin, and plasmapheresis early in the treatment of acute disseminated encephalomyelitis. J Clin Apher. 2006;21:260-5 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system that is associated with significant morbidity and mortality...
  16. Riedel K, Kempf V, Bechtold A, Klimmer M. Acute disseminated encephalomyelitis (ADEM) due to Mycoplasma pneumoniae infection in an adolescent. Infection. 2001;29:240-2 pubmed
    A 17-year-old boy presented with a severe form of an acute disseminated encephalomyelitis (ADEM) with hemiparesis and coma after initial symptoms of a flu-like febrile infection 1 week previously...
  17. Torisu H, Kira R, Ishizaki Y, Sanefuji M, Yamaguchi Y, Yasumoto S, et al. Clinical study of childhood acute disseminated encephalomyelitis, multiple sclerosis, and acute transverse myelitis in Fukuoka Prefecture, Japan. Brain Dev. 2010;32:454-62 pubmed publisher
    b>Acute disseminated encephalomyelitis (ADEM) has recently been studied in several countries owing to the development and wide spread use of imaging technology, but few epidemiological studies of childhood ADEM have been undertaken in Asian ..
  18. Donmez F, Aslan H, Coskun M. Evaluation of possible prognostic factors of fulminant acute disseminated encephalomyelitis (ADEM) on magnetic resonance imaging with fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging. Acta Radiol. 2009;50:334-9 pubmed publisher
    b>Acute disseminated encephalomyelitis (ADEM) may be a rapidly progressive disease with different clinical outcomes.
  19. Wingerchuk D, Lucchinetti C. Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis. Curr Opin Neurol. 2007;20:343-50 pubmed
    ..We review recent progress in differentiating and understanding the disease mechanisms of acute disseminated encephalomyelitis, neuromyelitis optica, and classical multiple sclerosis.
  20. Toivanen A, Valanne L, Tatlisumak T. Acute disseminated encephalomyelitis following nephropathia epidemica. Acta Neurol Scand. 2002;105:333-6 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is an acute monophasic inflammatory and demyelinating disease of the central nervous system (CNS) occurring days to weeks after a virus infection or vaccination...
  21. Hahn J, Lannin W, Sarwal M. Microangiopathy of brain, retina, and inner ear (Susac's syndrome) in an adolescent female presenting as acute disseminated encephalomyelitis. Pediatrics. 2004;114:276-81 pubmed
    ..a case of an adolescent female who presented at age 16 years with clinical and neuroimaging features of acute disseminated encephalomyelitis (ADEM). The full triad did not develop until 2.5 years after the initial neurologic presentation.
  22. Pradhan S, Gupta R, Shashank S, Pandey N. Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis. J Neurol Sci. 1999;165:56-61 pubmed
    We describe 4 patients with acute disseminated encephalomyelitis (ADEM) who were treated with intravenous immunoglobulins (IVIg) after getting no immediate response from a 3-5 day course of high dose intravenous methylprednisolone...
  23. Tanoue K, Yamashita S, Masuko K, Osaka J, Iai M, Yamada M. [Two cases of acute disseminated encephalomyelitis which occurred before the age of 24 months]. No To Hattatsu. 2006;38:363-7 pubmed
    We report two female cases of acute disseminated encephalomyelitis with onset of 19 and 15 months, respectively...
  24. Apak R, Kose G, Anlar B, Turanli G, Topaloglu H, Ozdirim E. Acute disseminated encephalomyelitis in childhood: report of 10 cases. J Child Neurol. 1999;14:198-201 pubmed
    We report 10 children with the diagnosis of acute disseminated encephalomyelitis. Diagnosis was based on clinical and radiologic findings, and after acute encephalitis was excluded by negative culture and antibody results...
  25. Saitoh A, Sawyer M, Leake J. Acute disseminated encephalomyelitis associated with enteroviral infection. Pediatr Infect Dis J. 2004;23:1174-5 pubmed
    A case of acute disseminated encephalomyelitis in 13-year-old boy associated with enterovirus is described. The patient had symptoms of severe headache and photophobia for 2 days...
  26. Chaudhuri A, Behan P. The clinical spectrum, diagnosis, pathogenesis and treatment of Hashimoto's encephalopathy (recurrent acute disseminated encephalomyelitis). Curr Med Chem. 2003;10:1945-53 pubmed
    ..data in these cases favour an immunopathological basis for this syndrome similar to relapsing acute disseminated encephalomyelitis. We suggest that Hashimoto's encephalomyelitis should be considered in the differential diagnosis ..
  27. Yamamoto Y, Takasaki T, Yamada K, Kimura M, Washizaki K, Yoshikawa K, et al. Acute disseminated encephalomyelitis following dengue fever. J Infect Chemother. 2002;8:175-7 pubmed
    A58-year-old man suffered from acute disseminated encephalomyelitis (ADEM) after dengue fever. ADEM has not been described as the cause of neurological complications in dengue fever...
  28. Wingerchuk D. The clinical course of acute disseminated encephalomyelitis. Neurol Res. 2006;28:341-7 pubmed
    ..review is to describe recent advances in understanding the clinical course of pediatric and adult acute disseminated encephalomyelitis (ADEM), especially the risk of relapse and conversion to multiple sclerosis (MS)...
  29. Masson C, Colombani J. [Primary acute disseminated encephalomyelitis in the adult. Study of 6 year follow-up of 2 patients]. Presse Med. 2002;31:1739-45 pubmed
    The relationship between acute disseminated encephalomyelitis (ADEM), a monophasic disease, and multiple sclerosis (MS), a potentially progressive disease with periods of recurrence or remission, remains debatable...
  30. Sahlas D, Miller S, Guerin M, Veilleux M, Francis G. Treatment of acute disseminated encephalomyelitis with intravenous immunoglobulin. Neurology. 2000;54:1370-2 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is a presumed immune-mediated, demyelinating disease of the CNS for which the standard treatment is high-dose steroids...
  31. Habek M, Brinar M, Brinar V, Poser C. Psychiatric manifestations of multiple sclerosis and acute disseminated encephalomyelitis. Clin Neurol Neurosurg. 2006;108:290-4 pubmed
    It is unusual for acute disseminated encephalomyelitis and multiple sclerosis to present as purely psychiatric disorders. We report five patients with such demyelinating diseases and symptoms of psychosis, depression or anxiety...
  32. Dale R. Acute disseminated encephalomyelitis. Semin Pediatr Infect Dis. 2003;14:90-5 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is a monophasic inflammatory disorder of the central nervous system (CNS). Unlike viral encephalitis, microorganisms do not invade the CNS...
  33. Anlar B, Basaran C, Kose G, Guven A, Haspolat S, Yakut A, et al. Acute disseminated encephalomyelitis in children: outcome and prognosis. Neuropediatrics. 2003;34:194-9 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is the most common demyelinating disorder of childhood. Its clinical features, prognosis and treatment vary in different reports.
  34. Yeh E, Collins A, Cohen M, Duffner P, Faden H. Detection of coronavirus in the central nervous system of a child with acute disseminated encephalomyelitis. Pediatrics. 2004;113:e73-6 pubmed
    ..case in which human coronavirus was detected in the cerebrospinal fluid of a child presumed to have acute disseminated encephalomyelitis. In murine models, coronavirus has been found to cause a chronic demyelinating condition that ..
  35. Bernard G, Riou E, Rosenblatt B, Dilenge M, Poulin C. Simultaneous Guillain-Barré syndrome and acute disseminated encephalomyelitis in the pediatric population. J Child Neurol. 2008;23:752-7 pubmed publisher
    ..Four patients diagnosed as having Guillain-Barré syndrome and acute disseminated encephalomyelitis during the same hospitalization are described herein...
  36. Menge T, Kieseier B, Nessler S, Hemmer B, Hartung H, Stuve O. Acute disseminated encephalomyelitis: an acute hit against the brain. Curr Opin Neurol. 2007;20:247-54 pubmed
    In this review, the possible etiology, clinical characteristics, diagnosis, and treatment of acute disseminated encephalomyelitis (ADEM) are discussed...
  37. Hynson J, Kornberg A, Coleman L, Shield L, Harvey A, Kean M. Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children. Neurology. 2001;56:1308-12 pubmed
    To identify the clinical and neuroradiologic features of acute disseminated encephalomyelitis (ADEM) in childhood.
  38. Politi M, Papanagiotou P, Grunwald I, Roth C, Reith W. [Acute disseminated encephalomyelitis]. Radiologe. 2008;48:553-9 pubmed publisher
    b>Acute disseminated encephalomyelitis (ADEM) is an acute widespread autoimmune demyelinating condition, which principally affects the white matter of the brain and spinal cord. It usually follows an infection or vaccination...
  39. Singhi P, Ray M, Singhi S, Kumar Khandelwal N. Acute disseminated encephalomyelitis in North Indian children: clinical profile and follow-up. J Child Neurol. 2006;21:851-7 pubmed
    b>Acute disseminated encephalomyelitis in children is not uncommon in developing countries, yet there is little systematic documentation of its clinical profile and follow-up...
  40. Bachmann S, Schroder J. Catatonic syndrome related to acute disseminated encephalomyelitis (ADEM). Schizophr Res. 2006;87:336-7 pubmed
  41. Garg R. Acute disseminated encephalomyelitis. Postgrad Med J. 2003;79:11-7 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system, and is characterised by multifocal white matter involvement...
  42. Young N, Weinshenker B, Parisi J, Scheithauer B, Giannini C, Roemer S, et al. Perivenous demyelination: association with clinically defined acute disseminated encephalomyelitis and comparison with pathologically confirmed multiple sclerosis. Brain. 2010;133:333-48 pubmed publisher
    Distinction between acute disseminated encephalomyelitis and acute multiple sclerosis is often clinically difficult...
  43. Tsuru T, Mizuguchi M, Ohkubo Y, Itonaga N, Momoi M. Acute disseminated encephalomyelitis after live rubella vaccination. Brain Dev. 2000;22:259-61 pubmed
    We report here a case involving a 14-year-old boy who developed acute disseminated encephalomyelitis following live rubella vaccination. The patient became febrile and began to experience nuchal pain 16 days after the immunization...
  44. Marchioni E, Marinou Aktipi K, Uggetti C, Bottanelli M, Pichiecchio A, Soragna D, et al. Effectiveness of intravenous immunoglobulin treatment in adult patients with steroid-resistant monophasic or recurrent acute disseminated encephalomyelitis. J Neurol. 2002;249:100-4 pubmed
    Randomized Controlled Trials have not let established the best pharmacological management of Acute Disseminated Encephalomyelitis (ADEM)...
  45. Schwarz S, Knauth M, Schwab S, Walter Sack I, Bonmann E, Storch Hagenlocher B. Acute disseminated encephalomyelitis after parenteral therapy with herbal extracts: a report of two cases. J Neurol Neurosurg Psychiatry. 2000;69:516-8 pubmed
    Two patients with acute disseminated encephalomyelitis after repeated injection of extracts from several different plants are described. There was no evidence of prior infection or vaccination...
  46. Murthy J. Acute disseminated encephalomyelitis. Neurol India. 2002;50:238-43 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is an uncommon inflammatory demyelinating disease of the central nervous system...
  47. Davis L, Booss J. Acute disseminated encephalomyelitis in children: a changing picture. Pediatr Infect Dis J. 2003;22:829-31 pubmed
  48. Brinar V, Habek M. Diagnostic imaging in acute disseminated encephalomyelitis. Expert Rev Neurother. 2010;10:459-67 pubmed publisher
    b>Acute disseminated encephalomyelitis is an idiopathic inflammatory demyelinating disease of the CNS that is particularly difficult to differentiate from the first episode of multiple sclerosis, so called clinically isolated syndrome...
  49. Aboagye Kumi M, Yango A, Fischer S, Donahue J, Morrissey P, Taylor N, et al. Acute disseminated encephalomyelitis in a renal transplant recipient: a case report. Transplant Proc. 2008;40:1751-3 pubmed publisher
    b>Acute disseminated encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system, mostly seen in children after viral or bacterial infection and vaccinations...
  50. Khurana D, Melvin J, Kothare S, Valencia I, Hardison H, Yum S, et al. Acute disseminated encephalomyelitis in children: discordant neurologic and neuroimaging abnormalities and response to plasmapheresis. Pediatrics. 2005;116:431-6 pubmed
    To describe our experience with acute disseminated encephalomyelitis (ADEM), focusing on (1) the relationship between clinical course and MRI findings and (2) the response to plasmapheresis in a subgroup of patients.
  51. Ning M, Smirnakis S, Furie K, Sheen V. Adult acute disseminated encephalomyelitis associated with poststreptococcal infection. J Clin Neurosci. 2005;12:298-300 pubmed
    b>Acute disseminated encephalomyelitis (ADEM) is a monophasic illness that is thought to develop from antigenic mimicry with antibodies having cross-reactivity to host epitopes in the nervous system...
  52. Kennedy P. Viral encephalitis: causes, differential diagnosis, and management. J Neurol Neurosurg Psychiatry. 2004;75 Suppl 1:i10-5 pubmed
  53. Apak R, Anlar B, Saatci I. A case of relapsing acute disseminated encephalomyelitis with high dose corticosteroid treatment. Brain Dev. 1999;21:279-82 pubmed
    We report a 16-month-old boy with acute disseminated encephalomyelitis (ADEM) who had an early relapse despite prompt treatment with high dose methylprednisolone. The second episode responded to intravenous immunoglobulin (IVIg)...