Marburg variant of multiple sclerosis

Last revised by Rohit Sharma on 22 Oct 2022

The Marburg variant of multiple sclerosis, also known as acute, fulminant, or malignant multiple sclerosis, is characterized by extensive and fulminant acute demyelination, often resulting in death within one year after the onset of clinical signs.

Please, refer to the main multiple sclerosis (MS) article for a broad discussion on this entity. 

Marburg variant is typically seen in younger patients 1,4.

Symptoms may be preceded by fevers and are typically monophasic.

Areas of demyelination demonstrate marked macrophage infiltration, marked associated inflammation, and extensive tissue destruction, necrosis and axonal damage and necrosis 2,4

Unlike other multiple sclerosis variants, the peripheral nervous system may also be involved 2

As is the case with other demyelinating diseases, MRI is the modality of choice for assessing patients with suspected Marburg type multiple sclerosis. 

Extensive confluent areas of tumefactive demyelination are seen with mass effect and defined rings and incomplete ring enhancement 1,4

Only rarely are numerous smaller lesions seen disseminated throughout the brain 2

Marburg type MS typically is rapidly progressive, fulminant and often results in death from involvement of the brainstem or due to marked mass effect and herniation 2,4

Only rarely do affected areas remyelinate 2

It was first described in 1906 by the Austrian neurologist Otto Marburg (1874-1948) 3.

Possible imaging differential considerations include:

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Cases and figures

  •  Case 1
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  • Case 2
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