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The Marburg Variant: Understanding Fulminant MS

Medically reviewed by Remi A. Kessler, M.D.
Written by Jessica Wolpert
Posted on June 29, 2021

What Is Marburg Variant MS? | Causes and Risk Factors | Diagnosis | Treatment | Prognosis and Outlook | Support

Marburg variant multiple sclerosis (MS) is a rare 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. Demyelination occurs when the immune system mistakenly attacks the central nervous system (CNS), which primarily comprises the brain and spinal cord. This demyelination causes inflammation and damage to the nerve tissue. The damage and ultimate scarring — which forms into lesions — impairs the nervous system’s ability to communicate messages from the brain to other parts of the body. This can lead to symptoms and signs typically associated with MS, including:

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

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

Whether you have RRMS, SPMS, or PRMS, the demyelination process is slow, meaning that the disease course can take years to progress. Although living with these types of MS can be difficult, they are almost never fatal in and of themselves.

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

  • Severe confusion
  • Seizures
  • Quadriplegia (the inability to move one’s arms and legs)
  • Motor aphasia (the inability to talk)

The quick onset of symptoms is then typically followed by more relapses and rapid decline in neurological function.

Causes and Risk Factors of Marburg MS

As with other types of MS, the cause of Marburg variant MS cannot be pinpointed to one single factor. The current leading theory is, Marburg variant MS is an autoimmune disease, and a combination of genetic and environmental factors make certain individuals more susceptible to the condition. In addition, Marburg variant MS more commonly affects young adults and children, compared to other forms of MS.

Whereas the disease course of most types of MS is long-term, with disability that accumulates over time, Marburg variant MS is different. It typically has a very rapid and significant decline, often leading to death. Those who survive can develop severe residual disability.

Diagnosis of Marburg MS

Marburg variant MS is very rare, and even most neurologists will probably never see a case over the course of their career. As of 2015, only 17 case reports have been published about the disease since it was first discovered in 1906. In fact, diagnosing the condition can be difficult, simply 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
  • Tumefactive MS

As such, 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 appear similar to more common serious illnesses, such as tumors or infections.

With that said, diagnosis for any neurologic condition begins with a physical examination. Your doctor may order different tests, such as:

  • An MRI to visualize lesion enhancement
  • A spinal tap (lumbar puncture) to examine cerebrospinal fluid
  • Nerve function tests
  • Various blood tests to figure out the diagnosis

If standard testing fails to yield a clear diagnosis, or if your health care team wants to understand how much demyelination has occurred, a neurosurgeon might perform a brain biopsy. This process entails removing a small piece of brain tissue in an operating room. This piece of tissue is then examined under a microscope.

Treatment for Marburg MS

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

  • High-dose corticosteroids
  • Plasma exchange (plasmapheresis)
  • Immunoglobulin therapy
  • Interferon-beta medications (immunomodulators)
  • Chemotherapy

These treatments have had varying levels of success in clinical settings.

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 brain stem. The brain stem controls many basic functions of life, including breathing.

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Posted on June 29, 2021
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Remi A. Kessler, M.D. is affiliated with the Medical University of South Carolina and Cleveland Clinic. Learn more about her here.
Jessica Wolpert earned a B.A. in English from the University of Virginia and an MA in Literature and Medicine from King's College. Learn more about her here.

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