- Rare acute variant of multiple sclerosis
- Seen in young adults
- Rapidly progressive course
- Frequent severe relapses
- Death or severe disability within weeks to months of onset
- Preceded by febrile illness
- Monophasic course
- Also considered fulminant form of ADEM
- Massive macrophage infiltration, demyelination, hypertrophic astrocytes, severe axonal injury
Imaging
- More destructive lesions than in MS or ADEM
- Multiple focal T2 lesions of different sizes, that coalesce, forming large white matter plaques seen in entire brainstem and hemispheric white matter
- Perilesional edema and enhancement commonly seen
Read more
Sarbu, Nicolae, et al. “White matter diseases with radiologic-pathologic correlation.” Radiographics 36.5 (2016): 1426-1447.
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