Marburg's variant of multiple sclerosis

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

Please, refer on multiple sclerosis (MS) 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. 

MRI

Extensive confluent areas 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 brain stem or due to marked mass effect and herniation 2,4

Only rarely do affected areas remyelinate 2

It was first described in 1906 by Otto Marburg 3.

Possible imaging differential considerations include:

 


Share article

Article Information

rID: 12276
Synonyms or Alternate Spellings:
  • Marburg multiple sclerosis
  • Multiple sclerosis Marburg variant
  • Acute malignant marburg type (MS variant)
  • Acute malignant marburg type (multiple sclerosis variant)
  • Marburg variant

Support Radiopaedia and see fewer ads

Cases and Figures

  • Drag
     Case 1
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.