Saturday, 27 August 2011

Espresso clinical tutorial (1): Fulminant MS or Marburg's variant

In response to a comment yesterday:

Marburg's variant of MS, which is also known as fulminant MS, is quite rare.

It was initially described by the Austrian neurologist Otto Marburg

The term fulminant is a carry-over from the pre disease-modifying therapy era. Most cases presenting with Marburg's variant of MS who treated promptly with appropriate therapies, respond to treatment, and can do very well. 

In the modern-era Marburg's variant of MS is rarely fatal and responds to the more aggressive therapies such as, mitoxantrone, cyclophosphamide and alemtuzumab. These are usually given in combination with high dose steroids. In some MS centres they also use plasma exchange. 

I have personal experience of using natalizumab in 37-year old male presenting with Marburg's variant of MS; apart from some persistent weakness in his right leg and some unsteadiness of gait he is well and fully independent 3 years after starting natalizumab. 

"This is no humbug! For the cynics out there does this not represent a major advance in the treatment of MS? In the pre-DMT era he would not be alive."

Marburg's variant of MS is diagnosed using MRI. It typically occurs in the form of a single large demyelinating lesion that can be indistinguishable from a brain tumor (see figure below; taken from radiopaedia). You may see the term tumefactive (tumour-like) used to describe these lesions. Such lesions are often biopsied to exclude other diagnoses in particular brain tumours. It is important to stress that the pathology of Marburg-variant MS is indistinguishable from 'classic MS'; i.e. Marburg MS is MS. 


Marburg's variant of MS, should also not be confused with neuromyelitis optica, Balo's concentric sclerosis and Schilder's disease. These are distinct clinico-pathological or disease entities, that overlap with MS, and can also present with a malignant or fulminant course.

"I will prepare separate posts on these diseases. I suggest you register yourself as a member or subscribe to email updates not to miss these posts."

The term malignant MS also needs clarification in this context; we us the term malignant MS to describe rapid progression of disease with substantial disability within 5 years of disease onset (typically needing a walking aid). Marburg's variant may be classified as being malignant if it results in significant and sustained disability within 5 years. Patients with Marburg's variant of MS may respond to treatment and recover from their initial presentation to then do well over the next 5 years. It is important to note that most cases of malignant MS will not be due to Marburg's variant of MS. 

"I hope this post clarifies your questions regarding fulminant MS." 

4 comments:

  1. This post has confused me even more. Malignant MS describes rapid progression of disease with substantial disability within 5 years of disease onset (typically needing a walking aid). I have PPMS and needed crutches with 4 year of disease onset. Does this mean my PPMS is malignant?

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  2. Re: "I have PPMS and needed crutches with 4 year of disease onset. Does this mean my PPMS is malignant?"

    Yes, according to the 5-year definition. Unfortunately there is no consensus in the field on the definition of what constitutes malignant MS; some investigators say 5 years others 2 years. What is important is that whatever definition we use we are simply trying to identify the 5 to 10% of MS'ers with the worst disease course. Unfortunately, needing a walking aid with 4 years would put you in this category.

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  3. My husband had/has Marburg's, Thank you for letting others know it is not always fatal. At the time he was diagnosed with it, all the literature on the web stated it was. He still has problems with walking, but is doing fairly well 5 years later.

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  4. I have PPMS. When dx I was using a cane on occasion. Within 4 months it was a walker and within 6 a wheelchair. Within the first year if dx I went from being able to balance somewhat on my own to not at all. Now I spend most days in my adjustable bed. And full time in a wheelchair for mobility. Of course I have other rapid onsets with eye sight, talking, swallowing, washroom issues and severe numbness , tinging... Does this put me in the PPMS Malignant ?

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