Marburg described a rapidly progressive demyelinating disorder that is included within the spectrum of multiple sclerosis.
Synonym: malignant monophasic multiple sclerosis
The classic disorder is a progressive disorder without remission that can cause death within weeks or months of onset.
The patient presents with neurological symptoms and raised intracranial pressure. The patient may be suspected of having a brain tumor (pseudotumor).
Imaging studies show large demyelinating plaques in the hemispheric white matter that are hyperintense on T2-weighted images and which often show enhancement with gadolinium.
Demyelination may also occur in the:
(1) brainstem
(2) spinal cord
(3) peripheral nervous system
A biopsy of a plaque shows widespread demyelination with extensive necrosis and a histiocytic infiltrate.
Death may be caused by involvement of the brainstem or widespread demyelination. However, some patients have achieved remissions after immunosuppression and/or aggressive therapy with corticosteroids.
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