Acute Disseminated Encephalomyelitis and Acute Hemorrhagic Encephalomyelitis

  • Jacob Valk
  • Marjo S. van der Knaap


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.


White Matter Herpes Zoster Myelin Basic Protein Optic Neuritis Cerebellar Ataxia 
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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

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