Acute
Disseminated Encephalomyelopathy
(ADEM, Perivenous Encephalomyelitis)
Epidemiology:
Common sites:
Gross features:
- grayish
discoloration around white matter vessels
Histologic features:
- myelin loss with
relative preservation of axons
- diffuse perivenular distribution of demyelination
- lipid-laden
macrophages
- intracytoplasmic myelin granules
- PMNs present early on
- Mononuclear
infiltrate later on
Immunophenotype:
Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- demyelinating disease with monophasic
course
- follows viral
infection
- rapidly
progressing symptoms of diffuse brain involvement:
- fatal in up to 20%
- complete recovery
in survivors
References: