Marburg variant of multiple sclerosis

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. 

Epidemiology

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

Clinical presentation

Symptoms may be preceded by fevers and are typically monophasic.

Pathology

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. 

Radiographic features

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. 

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.

Differential diagnosis

Possible imaging differential considerations include:

• Baló concentric sclerosis (BCS)
• acute disseminated encephalomyelitis (ADEM)
  • similar, although Marburg is usually more severe