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.”