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 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.
Only rarely are numerous smaller lesions seen disseminated throughout the brain 2.
Treatment and prognosis
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.
History and etymology
It was first described in 1906 by the Austrian neurologist Otto Marburg (1874-1948) 3.
Possible imaging differential considerations include:
- Balo concentric sclerosis (BCS)
acute disseminated encephalomyelitis (ADEM)
- similar, although Marburg is usually more severe
- 1. Raine CS, McFarland HF, Hohlfeld R. Multiple sclerosis, a comprehensive text. Saunders. (2008) ISBN:0702028118. Read it at Google Books - Find it at Amazon
- 2. Cook SD. Handbook of Multiple Sclerosis, Fourth Edition. CRC Press. (2013) ISBN:142001871X. Read it at Google Books - Find it at Amazon
- 3. Marburg, Otto. Die sogenannte akute multiple Sklerose (Encephalomyelitis periaxialis scleroticans). F. Deuticke, 1906.
- 4. Sarbu N, Shih RY, Jones RV et-al. White Matter Diseases with Radiologic-Pathologic Correlation. Radiographics. 2016;36 (5): 1426-47. doi:10.1148/rg.2016160031 - Pubmed citation
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