Acute Disseminated Encephalomyelitis and Acute Hemorrhagic Encephalomyelitis

  • Jacob Valk
  • Marjo S. van der Knaap

Abstract

Acute disseminated encephalomyelitis (ADEM) is an uncommon inflammatory disorder within the CNS, predominantly within the white matter of the brain and spinal cord. ADEM and acute hemorrhagic encephalomyelitis (AHEM) represent two clinical variants of a single pathological process. They occur following a viral infection or vaccination or, without recognized antecedent. Implicated viral infections are measles, chickenpox, rubella, smallpox, infectious mononucleosis, herpes simplex, herpes zoster, mumps, and influenza. They rarely occur after mycoplasma or bacterial infection. A nonspecific upper respiratory tract infection is the most common antecedent. The latent period varies from several days to several weeks, the mean being 4–6 days. Onset of the neurological symptoms is usually abrupt, with convulsions and progression to somnolence and coma. Illness may also commence subacutely with symptoms of headache, fever, irritability, drowsiness, and vomiting. Nuchal rigidity is often present. The neurological signs are quite polymorphic and consist of hemiplegia, paraplegia or quadriplegia, cerebellar ataxia, cranial nerve palsies, optic neuritis, nystagmus, sensory loss, and bladder paralysis. Subcortical blindness is rare. Extrapyramidal motor abnormalities such as chorea, athetosis, and ballismus may be found. The progression of the disease is variable. Patients with AHEM progress more rapidly into delirium and coma. The highest mortality is seen during the 1st week of the illness, and in fact most patients who survive the 1st week eventually recover, with varying degrees of disability. Prolonged disturbances in level of consciousness entail a poor prognosis both for morbidity and mortality. Neurological sequelae include epilepsy, spastic paresis, ataxia, decreased vision and, cognitive and psychiatric disturbances. Most of the neurological syndromes have a monophasic course lasting several weeks. Recurrent attacks of ADEM/AHEM have been described. The occurrence of the neurological abnormalities is apparently independent of the nature and severity of the antecedent infection or immunization.

Keywords

Permeability Toxicity Corticosteroid Influenza Vasculitis 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Adams RD, Cammermeijer J, Brown DD (1949) Acute necrotizing hemorrhagic encephalopathy. J Neuropathol Exp Neurol 8: 1–29PubMedCrossRefGoogle Scholar
  2. Alvord EC (1970) Acute disseminated encephalomyelitis and “allergic” neuro-encephalopathies. In: Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology, vol 9. Amsterdam: North Holland Publishing company: 500–571Google Scholar
  3. Atlas SW, Grossman RI, Goldberg HI, Hackney DB, et al. (1986) MR diagnosis of acute disseminated encephalomyelitis. J Comput Assist Tomogr 10: 798–801PubMedCrossRefGoogle Scholar
  4. Cohen IR (1986) Regulation of autoimmune disease physiological and therapeutic. Immunol Rev 94: 5–21PubMedCrossRefGoogle Scholar
  5. Grossman RI, Lisak RP, Macchi PJ, Joseph PM (1987) MR of acute experimental allergic encephalomyelitis. AJNR 8: 1045–1048PubMedGoogle Scholar
  6. Hart MN, Earle KM (1975) Haemorrhagic and perivenous encephalitis: a clinical-pathological review of 38 cases. J Neurol Neurosurg Psychiatry 38: 585–591PubMedCrossRefGoogle Scholar
  7. Johnson RT, Griffin DE, Hirsch RL, Wolinsky JS, et al. (1984) Measles encephalomyelitis–clinical and immunologic studies. N Engl J Med 310: 137–141PubMedCrossRefGoogle Scholar
  8. Kappelle LJ, Wokke JHJ, Huynen ChHJN, Gijn van J (1986) Acute disseminated encephalitis documented by magnetic resonance imaging and computed tomography. Clin Neurol Neurosurg 88: 197–202PubMedCrossRefGoogle Scholar
  9. Koening H, Rabinowitz SG, Day E, Miller V (1979) Post-infectious encephalomyelitis after successful treatment of herpes simplex encephalitis with adenine arabinoside. N Engl J Med 300: 1089–1093CrossRefGoogle Scholar
  10. Lebar R (1987) Démyélinisation et autoimmunité. Pathol Biol 35: 275–283PubMedGoogle Scholar
  11. Lisak RP, Behan PO, Zweiman B, Shetty T (1974) Cell-mediated immunity to myelin basic protein in acute disseminated encephalomyelitis. Neurology 24: 560–564PubMedCrossRefGoogle Scholar
  12. Lukes SA, Norman D (1983) Computed tomography in acute disseminated encephalomyelitis. Ann Neurol 13: 567–572PubMedCrossRefGoogle Scholar
  13. Lukes SA, Norman D, Mills C (1983) Acute disseminated encephalomyelitis: CT and NMR findings. J Comput Assist Tomogr 7: 182Google Scholar
  14. Okuno T, Fuseya Y, Ito M, Konishi Y, et al. (1981) Reversible multiple hypodense areas in white matter diagnosed as acute disseminated encephalomyelitis. J Comput Assist Tomogr 5: 119–121PubMedCrossRefGoogle Scholar
  15. Poser CM (1969) Disseminated vasculomyelinopathy. Acta Neurol 45: 7–44CrossRefGoogle Scholar
  16. Poser CM, Roman G, Emery III ES (1978) Recurrent dissem- inated vasculomyelinopathy. Arch Neurol 35: 166–170PubMedCrossRefGoogle Scholar
  17. Rabinowitz SG, Day ED, Paterson PY, Koening H (1983) Endogenous myelin basic protein-serum factors ( MBPSFS) and anti-MBP antibodies in a patient with post-herpes simplex virus acute disseminated encephalomyelitis. J Neurol Sci 60: 393–400Google Scholar
  18. Reich H, Lin SR, Goldblatt D (1979) Computerized tomography in acute hemorrhagic leukoencephalopathy: a case report. Neurology 29: 255–258PubMedCrossRefGoogle Scholar
  19. Reik L (1980) Disseminated vasculomyelinopathy: an immune complex disease. Ann Neurol 7: 291–296PubMedCrossRefGoogle Scholar
  20. Russell DS (1955) The nosological unity of acute haemorrhagic leucoencephalitis and acute disseminated encephalomyelitis. Brain 78: 369–376PubMedGoogle Scholar
  21. Saito H, Endo M, Takase S, Itahara K (1980) Acute disseminated encephalomyelitis after influenza vaccination. Arch Neurol 37: 564–566PubMedCrossRefGoogle Scholar
  22. Sriram S, Steinman L (1984) Postinfectious and postvaccinial encephalomyelitis. Neurol Clin 2: 341–353PubMedGoogle Scholar
  23. Ziegler DK (1966) Acute disseminated encephalitis. Arch Neurol 14: 476–488PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1989

Authors and Affiliations

  • Jacob Valk
    • 1
  • Marjo S. van der Knaap
    • 2
  1. 1.Department of Diagnostic Radiology and NeuroradiologyFree University HospitalAmsterdamThe Netherlands
  2. 2.Department of Child NeurologyAcademic HospitalUtrechtThe Netherlands

Personalised recommendations