Marburg variant multiple sclerosis (MS) is a rare but serious variation of MS. The condition is also called the Marburg course of MS, acute MS, malignant MS, and fulminant MS. The term “fulminant” means severe and sudden in onset.

What Is Marburg Variant MS?

All forms of MS involve demyelination — the destruction of the myelin sheath that surrounds and protects the nerves. Demyelinating diseases occur when the immune system mistakenly attacks the central nervous system, which is made up of the brain and spinal cord. This demyelination causes inflammation and damage to the nerve tissue. The damage and scarring, which form lesions, disrupt the nervous system’s ability to send messages from the brain to other parts of the body. This disruption leads to symptoms and signs typically associated with MS, including:

• Numbness
• Weakness
• Vision problems
• Bowel and bladder problems

There are several different subtypes of MS. Most people with MS have relapsing-remitting MS (RRMS). In this type, they experience symptoms for a few weeks or months and then have periods of remission — when symptoms improve or disappear. Over time (usually decades), RRMS can develop into secondary progressive MS (SPMS), in which the disease continually gets worse without remission. Some people are first diagnosed with primary progressive MS (PPMS), in which symptoms continue to worsen without remission.

Whether you have RRMS, SPMS, or PRMS, the demyelination process is slow — the disease course can take years to progress. Although living with these types of MS can be difficult, they are rarely fatal by themselves.

However, in the Marburg type of MS, the demyelination process occurs much more quickly, and the demyelinating lesions are more widespread, large, and swollen. People with Marburg variant MS experience startling, quick-onset symptoms, such as:

• Severe confusion
• Seizures
• Quadriplegia (not being able to move arms and legs)
• Motor aphasia (not being able to talk)

Symptoms start quickly, then are usually followed by more relapses and a fast decline in brain and nerve function.

Causes and Risk Factors of Marburg MS

As with other types of MS, the cause of Marburg variant MS can’t be pinpointed to one single factor. The current leading theory is that Marburg variant MS is an autoimmune disease. Scientists believe that a combination of genetic and environmental factors makes certain people more likely to develop this condition. In addition, the Marburg variant of MS more commonly affects young adults and children, compared to other forms of MS.

The quick decline that occurs in people with the Marburg variant can lead to a very severe outcome including severe long-term disability and death.

Diagnosis of Marburg MS

Marburg variant MS is very rare, and most neurologists will probably never see a case throughout their careers. As of 2024, only a few dozen case reports have been published about the disease since it was first discovered in 1906. Diagnosing the condition can be difficult because it is so uncommon and can be similar to other very rare conditions that cause demyelination. Conditions similar to Marburg variant MS include:

• Acute disseminated encephalomyelitis
• Balo’s disease
• Schilder’s diffuse sclerosis

It can be difficult for a doctor to determine whether a person’s signs and symptoms are caused by Marburg variant MS or another condition. Even with MRI scans, Marburg variant MS can look like more common serious illnesses, such as tumors or infections.

Diagnosis for any neurologic condition begins with history taking and a physical examination. Your doctor may order different tests, such as:

• An MRI to see the injury to the brain
• A lumbar puncture (spinal tap) to examine the fluid that surrounds the brain and spinal cord (cerebrospinal fluid)
• Nerve function tests
• Various blood tests to figure out the diagnosis

If standard tests don’t give a clear diagnosis, or if doctors need to see how much nerve damage there is, a neurosurgeon might do a brain biopsy. This means they will remove a small piece of brain tissue in an operating room. Then, this tissue is examined under a microscope.

Treatment for Marburg MS

Treatment for Marburg variant MS must be aggressive and should be done in a hospital. Options include:

• High-dose corticosteroids
• Plasmapheresis (plasma exchange)
• Immunoglobulin therapy
• Chemotherapy including mitoxantrone and cyclophosphamide
• Monoclonal antibodies — Alemtuzumab (Lemtrada), rituximab (Rituxan), ocrelizumab (Ocrevus)

These treatments have shown different levels of success in clinical settings. Some doctors prefer using high-dose cyclophosphamide, but there is still limited experience with this drug.

Prognosis and Outlook for Marburg MS

Most people with MS generally have slightly shorter lifespans than the general population. Unfortunately, cases of Marburg variant MS are usually fatal within one year, and many cases are fatal within a few weeks or months. This high death rate is caused by the intensity of the inflammatory process and the effects that Marburg variant MS has on the brainstem, which controls many basic functions of life, including breathing. It’s important to recognize this variant early and start quick, strong treatment.

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